Two Suitcases: Emotional Awareness In The Journey To Transplantation
An esteemed colleague with CF, who is also a post-transplant recipient survivor, has described to me, “Pursuing a lung transplant is like carrying two suitcases…one preparing for death, one preparing for life.” She elaborates, “End-stage disease means saying goodbye to who we wanted to be, to what we wanted to do, to our expectations and beliefs, to independence, normalcy, our sense of control, our assumptive worlds, to hopes, dreams, goals and longevity; transplantation means holding on to hope for these things.” (Christensen, Tiffany, NACFC, 2018). I am reminded of this analogy as I offer a perspective on the emotional and psychosocial considerations of lung transplantation (LT) in adults with CF.
The 2018 North American Cystic Fibrosis Conference (NACFC) provided opportunities for numerous presentations and networking sessions concerning lung transplantation. Abstracts highlighted scientific, therapeutic and clinical aspects of providing care to persons with CF living with advanced lung disease, including the importance of advanced care planning, inclusion of palliative care approaches to engaging in difficult conversations and, importantly, the relationships between mental health (depression, anxiety) and health-related quality of life for persons with CF and family/caregivers (Quittner, AL, et al. Thorax, 2014; 69). Thank you for your willingness to share your stories, adding depth and meaning to our professional presentations.
The “suitcase” that is packed for living with advanced lung disease, “preparing for death,” weighs heavily on the scale in managing behavioral health concerns. Depression and anxiety are common mental health problems in this population with research efforts focusing on whether pre-transplant behavioral health problems are predictive measures of mortality and morbidity post-transplantation (Dew, MA, et al. Transplantation, 2015 May). For example, meta-analysis of studies demonstrates no effect of pre-transplant anxiety on post-lung transplant mortality (Courtwright et al. Psychosomatics, 2016) and similarly, a prospective study of lung recipients (n=66; 18% CF) found pre-transplant current or past depression was not associated with post-transplant mortality (Smith et al. Clinical Transplantation, 2017). Treatment interventions to assess and treat behavioral health needs require consistent use of validated instruments thereby assuring reliability when identifying potential contraindications in candidate selection and promoting outcomes.
Experiencing depression and anxiety in CF lung disease contributes to decreased quality of life, decreased adherence to therapies and directly affects health outcomes with increased healthcare costs and increased mortality (Riekert et al. Chest, 2007; Smith et al. Ped Pulmon, 2010, 2014). Individuals with advanced lung disease must balance treatment choices and share decision-making with families/caregivers and their teams about their hopes, goals and wishes in planning for their futures. Experiencing increased respiratory distress, with accompanying added treatment burdens, can exacerbate worry, fear and social isolation. Forced dependence on caregivers, decreased ability to maintain social relationships and changes in levels of employment can contribute to feelings of isolation, financial stress and changes in self-esteem (Gilbert, CR, and Smith, CM. Mt Sinai J Med, 2009 Feb). Emotional distress for individuals with advanced lung disease and their caregivers/families is commonly elevated when addressing preferences for end-of-life care. Effective treatment may include consultation with palliative care providers, pain specialists, social workers and mental health providers. In addition, religious professionals can address distress and suffering based on an individual’s values, preferences, beliefs, culture and religion. Counselors and psychiatrists can be instrumental in enhancing one’s coping and, contrary to perceptions, use of anti-anxiety agents and anti-depressants prior to LT does not adversely impact one’s candidacy. Appropriate pharmacologic interventions, including medically prescribed narcotic and opiate use, can be valuable therapeutic support in improving survival and quality of life and complements psychological support.
Adults living with advanced disease describe fears and uncertainties that may contribute to hesitation in committing to the transplant journey. They perceive that living with CF is “predictable” — they know their bodies and what to expect with disease progression. It is common to experience situational anxiety and panic attacks related to coping with the losses and uncertainties when choosing to proceed with transplantation. One adult expressed, “Psychologically, the scariest issue I faced pre-transplant was panic attacks. Before I was in end-stage CF, I thought panic attacks were fictional and only happened to weak-minded people. I was wrong — really wrong. The panic attacks started a year before my transplant and didn’t cease until after the transplant” (WL, adult post-transplant). Numerous social media sites exist to engage with others in seeking personal stories and/or support. Some LT programs require that all potential pre-transplant candidates and their families meet with post-transplant recipients through a structured mentoring program. Professional, peer and family community support can be helpful in exploring the range of emotions, sharing both negative thoughts as well as hopes and expectations of one’s new potential.
Consideration of lung transplantation “is an explicit acknowledgment that a patient has a limited life expectancy without a transplant and an expectation that the risk-to-benefit ratio favors lung transplantation rather than conventional medical treatment” (Weill, D, et al. J Heart Lung Transplant, 2015; 34: 1-15). This becomes the “second suitcase” — “preparing for life.” Choosing to proceed with lung transplantation and managing post-transplant mental health is critically important to health outcomes and quality of life. Transplant teams are experts in providing education to potential recipients to better inform their decision-making about the risks and benefits of LT. Information provided about behavioral health problems can include neuropsychiatric complications post-lung transplant with focus on post-operative delirium and neuropsychiatric side effects of some post-transplant medications (Sher, Yelizabeta MD, NACFC, 2018). Other studies, citing depression and anxiety post-transplant, include diagnoses of Major Depressive Disorder (30 percent), Adjustment Disorder with Anxiety (22 percent), Panic Disorder (18 percent), transplant specific Post-Traumatic Stress Disorder (15 percent) and Generalized Anxiety Disorder (4 percent) (Dew, MA, et al. Gen Hosp Psychiatry, 2012). Transplant teams offer clinical expertise in providing psychopharmacological and psychotherapy support throughout acute and chronic post-transplant management.
Awareness of the complexity of mental health across the transplant spectrum is also very important to your social, peer and family support systems as their mental health can be directed impacted. Caregiver networks are as integral in the selection of potential candidates as candidates themselves. Evaluation tools, such as the SIPAT (Stanford Integrated Psychosocial Assessment for Transplant), are utilized by some transplant programs to assess not only the potential recipient’s readiness, psychological stability and psychopathology but carefully considers the strengths, availability and functionality of the support system in assessing one’s transplant candidacy (Maldanado et al. Psychosomatics, 2012). Unfortunately, not all potential recipients are deemed appropriate candidates for listing. Contraindications for transplantation can include both physical and psychosocial factors; most relevant to this are past or current behavioral patterns related to adherence to medical therapies; psychiatric or psychologic conditions that would adversely impact patient-family-team partnership indicating inability to work with the transplant team effectively; substance use/abuse and absence of an adequate or reliable social support system (Weill, D, et al. J Heart Lung Transplant, 2015; 34). Although most caregivers of transplant adults do not report significant impairment in their own emotional functioning, assessment of caregivers› coping strategies and caregiving burden can help identify those who are at risk for experiencing increased emotional distress and affect recipients’ outcomes. (Goetzinger, AM, et al. Clin Transplant, 2012).
Consideration of transplant is a critical transition in one’s life story and respect for individual choice — whether to proceed with transplant or partner together to maintain life as fully as possible without transplant — is unique. Is undergoing transplant “trading one disease for another?” Many in the community use this phrase to frame appropriate hopes and expectations with potential recipients. One adult articulated his perception of this very clearly: “I don’t look at transplant as the disease. CF is the disease. Transplant is a way of bettering your current disease” (M.D., adult pre-transplant). While focused on a cure, the CF community is actively engaged in promoting a broadening awareness of transplant and altering perceptions of transplantation as a “failure” of conventional therapy or as “a last resort.” Individuals with CF balance the tightrope between hope within CF and working towards transplantation “Before the transplant, mentally, I always wondered — should I be on the transplant list? It seems so simple but, with medical advances and my eternally optimistic nature, I held out hope for a medical miracle. I am now post-transplant for one year…the CF pipeline is healthy, but no CF treatment for my genotype currently exists. If I had waited any longer to go on the transplant list, I certainly would have died” (W.L., adult post-transplant).
Throughout my experience providing comprehensive psychosocial care to individuals with CF and their families in both CF and lung transplantation, I remain humbled by the psychological strength to mobilize resources that contribute to resilience; resilience not being the absence of difficulties but the capacity for personal growth under difficult and stressful circumstances. Living with advanced disease and navigating the transplant journey present opportunities to mentally and emotionally find meaning. Expressing grief for the losses, facing the uncertainties, reaching for hopes and dreams that can be achieved with this gift of life encompasses only some of the profound circumstances you confront in living with advanced disease and embracing transplantation. Current efforts by the Cystic Fibrosis Foundation focus on convening professionals in both CF and transplant centers, with input of the voices of those with CF and their caregiving networks, to develop guidelines on treatment and management of advanced disease, These efforts enrich our knowledge as care providers and help us design process improvements between CF Centers and LT programs to enhance outcomes. We are partners in your care, working with you to build a foundation that enhances your coping and adjustment to navigating the complexities of living with advanced disease, including transplantation.
With gratitude for sharing stories in carrying both suitcases:
Isabel Stenzel-Byrnes, LCSW, MPH; Mathias Dougherty and W. L., all adults with CF.
Ask the attorney: Questions From Our Readers, Answers From Our Attorney
CF Roundtable readers asked questions related to the Affordable Care Act and the Ticket to Work program. No reference made in this column is to a specific situation. Nothing in this column is meant to be legal advice about a specific situation but is meant to be only information.
The CF Legal Information Hotline can answer questions related to Social Security benefits, Medicare, Medicaid, health insurance and rights in employment and education. If you have questions, please e-mail CFLegal@sufianpassamano.com to schedule a time to speak with an attorney.
Question 1 — What effect does the recent case Texas v. United States have on the Affordable Care Act?
I have received many e-mails and calls this month asking about Texas v. United States, the lawsuit that held that the Affordable Care Act (ACA) is unconstitutional. Before we discuss the details of this case, it is important for everyone to know that, despite the court’s ruling, the protections the ACA provides to people with CF have not changed. The ACA remains in effect, and all policies purchased on a healthcare exchange are still valid and in effect.
To understand Texas v. United States, you must remember that the United States Supreme Court upheld the ACA as a constitutional exercise of Congress’s power to tax and spend in the case National Federation of Independent Business v. Sebelius, 567 U.S. 519 (2012). The Court reasoned in Sebelius that the ACA’s individual mandate, which required individuals to have health insurance coverage and which imposed a penalty on those who did not, was a tax that was within Congress’s constitutional authority. However, last year, as part of the tax reform bill, Congress reduced the ACA tax penalty to zero. Consequently, anyone who failed to have health insurance coverage would have no tax consequence.
Shortly thereafter, a group of Republican state attorneys general and governors challenged the ACA in court. The case they filed reasoned that if the ACA is constitutional because it levies a tax, then it must be unconstitutional if that tax is reduced to zero. Ordinarily the United States Department of Justice would defend any action challenging a federal statute. However, the Trump administration did not choose to defend the ACA. Rather, the Justice Department agreed with the lawsuit and said that some or all of the ACA should be struck down as unconstitutional. Because the Trump administration would not defend the ACA, a group of Democratic state attorneys general intervened in the case to defend the ACA.
On December 17, 2018, a United States District Court in Texas ruled in favor of the plaintiffs claiming the ACA was unconstitutional. The court struck down not just the individual mandate, but the entire ACA. The court ruling even struck down the ACA’s ancillary provisions that are not directly related to health insurance reform, such as Medicare prescription drug benefit regulations, the establishment of a new bureau in the Center for Medicare and Medicaid Services and a provision requiring restaurant menu labeling.
The decision in Texas v. United States is significant. However, the court did not issue an injunction to halt the operation of the ACA during an appeal. So the ACA remains in effect for now. Texas v. United States will now be appealed to the United States Fifth Circuit Court of Appeals.
While no one can know for certain what the outcome of the appeal will be, many legal scholars believe that the District Court’s ruling is simply too broad to be upheld. There is some disagreement, however, about exactly what will be the result on appeal. In any event, whatever decision the Fifth Circuit makes, the case will most likely be appealed by the side that loses, and then the case will be taken up by the United States Supreme Court.
Of course it is difficult to know how the United States Supreme Court may rule on this case given the new composition of the Court. Although a majority of the justices upheld the ACA on a previous challenge, the composition of the Supreme Court has substantially changed since then. In any event, the ACA remains in effect for now. People with CF who need insurance should be thankful that there are people defending the ACA and that its mandates are still in effect.
Question 2 — What is the Ticket to Work program?
Social Security’s Ticket to Work program provides assistance in finding a full-time job to individuals who are receiving Social Security Disability Insurance benefits (SSDI) or Supplemental Security Income benefits (SSI). The Ticket to Work program is free and voluntary. The SSDI or SSI recipient who participates in the Ticket to Work program receives a paper ticket that allows the person to access vocational services at no cost. The person can look online and see the different entities that can provide vocational services under the program. One type of service provider is an Employment Network. The Employment Network can help a person with career counseling, job placement and ongoing support services.
Some people with CF report difficulty with the Ticket to Work providers in terms of the types of jobs available. Some people with CF report that some of the vocational providers suggest job placement only in food service or retail jobs. Sometimes these types of jobs are difficult for people with CF to perform full time because the jobs involve a lot of standing and walking.
Recently, Social Security representatives have been calling people who receive SSDI or SSI to ask if the person wants to participate in the Ticket to Work program. A person does not have to agree to participate in the Ticket to Work program. Some people with CF have said they want to participate in the program but did not realize that participating in the Ticket to Work program involves returning to full-time work. The goal of the Ticket to Work program is to find a full-time job for the SSDI or SSI recipient, and then SSDI or SSI benefits stop entirely. A person who does not think he or she is capable of working full time should not agree to participate in the Ticket to Work Program.
If a person wants to return to full-time work, then the Ticket to Work program may be helpful. If the person is enrolled in the Ticket to Work program, once the person finds a full-time job, his or her SSDI or SSI benefit stops. However, the person continues to receive Medicare benefits for eight and a half years. SSA counts the nine-month trial work period as part of the eight-and-a-half-year extension of Medicare.
A person who receives SSDI can work part time (less than 15 hours a week is the safest) making less than $1,180 a month before taxes are taken out of the work check. If a person with CF wants to work part time the person does NOT need to enroll in the Ticket to Work program. For those receiving SSI, the part time work limits are different. The hours a person can work are the same, up to 15-20 hours a week. However, for every $2 earned from work activity in a month, SSI will reduce the SSI check for that month by $1.
If you have questions about working and Social Security benefits, please contact the CF Legal Information Hotline.
Spirit Medicine: T For Transplant; T For Trauma
In this Spirit Medicine, I’m compelled to write about a subject that is seldom discussed when it comes to transplantation. Having a lung transplant is an extraordinary miracle. We are so fortunate to have the kind of disease where we can take out the sick part and put in a new part — all thanks to our organ donors.
But transplantation is the most difficult experience of a CF person’s life — apart from the end of life itself. And while we want to express gratitude and joy for surviving a transplant, I’d like to write about the trauma of having a transplant and how that affects our spirits.
Trauma is defined as an overwhelming amount of stress that exceeds one’s ability to cope or integrate the emotions involved with that experience. Trauma affects the body as well as the emotions. Having a transplant can be an overwhelming experience that exceeds a CF person’s ability to cope, despite all of the coping skills one has developed over a lifetime. Trauma overwhelms coping instincts and instantly propels the body and brain into survival mode — the sympathetic nervous system’s animal instincts of fight, flight and freeze. With trauma, we can’t turn that off easily and feel constantly on edge. Transplantation is filled with uncertainty and unpredictability and can push us into survival mode. Survival mode can mean shutting down our emotions.
What are the symptoms of trauma? Some include trouble sleeping; nightmares; feeling numb or detached; withdrawal/depression; difficulty with concentration; mood swings; forgetfulness; being disorganized; sensing something is wrong; feeling different, like you are outside looking in and not in your body; anxiety; panic attacks; a need to maintain control at all times; rigidity; a need for order; being judgmental of self or others; body image issues; chronic pain/migraines not explained medically; high alert; a vigilant, watchful and exaggerated startle reflex. There are many other symptoms around relationships, such as feeling mistrustful or suspicious of others, or difficulties with intimacy that are very common in people with abuse or sexual trauma. If trauma symptoms last a long time and interfere with our normal functioning, then we call this post-traumatic stress disorder.
Some of you know that I was at the very end of life when I was transplanted. I spent my final days gasping for breath like no other time of my life. My emotions shut down. I lost control of my body, and, as I was slipping into a coma, I had a poignant near-death experience. I saw a bright light and people who had died; I called out their names. I remember the noise and drama of being intubated. I remember that fight for life like it was yesterday. I also remember waking up, with new lungs, my family surrounding me. I was beyond grateful to my lung donor. I was alive but terrified of going through that again.
It took almost a year or so for me to stop reliving that experience (called flashbacks) and to not wake up in a panic that I couldn’t breathe. When I started swimming, it took a long time for me to learn to hold my breath longer without sheer terror. It took weeks after transplant for me to feel like I was really alive and this wasn’t a dream. And I was so fortunate that my recovery was relatively smooth. Others have had more traumatic events after transplant as well.
It’s important to understand that trauma is recognized as exposure to actual or threatened death or serious injury. When I first learned about this definition, it occurred to me that our entire lives with cystic fibrosis consist of threats to life— not just at the end. Difficult experiences such as a lung collapse, pneumonia, detached port, hemoptysis, intubation or bowel blockage can be felt as if they were threatening our lives. And yet, with CF, most of us endure and persevere and keep going despite these threats. How do we do this?
My life with CF has been threatened numerous times— pre- and post-transplant. Thankfully, there was always an intensive procedure to save it, and drugs to help me relax, and I returned to baseline. I would relive those scary moments for a long time. However, telling my friends and family, having their support and grounding myself in the present helped. Believing in the intrinsic power of my body also helped. Redirecting my fight/flight/freeze instincts towards exercise or other activities helped give me back control.
I believe there are long-term consequences to this chronic threat to life. It’s the little things, like my inability to see violent movies, or movies about water and drowning, without getting viscerally upset. I admit I live with fear every day, even if it’s mostly low grade. I am often forgetful, cloudy headed, easily overwhelmed and need time alone. I am constantly vigilant about people coughing around me or lawn blowers or cigarette smoke…as if I inhaled, it would be the beginning of the end. I notice my body and it feels tense, with shallow breathing, heart racing and tightened muscles until this threat has passed. I feel this way even when I am an inpatient and walk by the room where I almost died. I don’t feel like I’m crazy, or that I’m the only one who does this. I believe this is a consequence of trauma.
I have seen so many examples of resilience in overcoming trauma among my CF friends. How we do this depends on our personalities, age, gender, relationships, and what happens after the traumatic event. But little by little, with each small trauma we face — with each threat to our lives — we learn to cope. We deny death; we maintain a warrior stance, take risks and seek thrills; we do yoga and run marathons; we lean on others; we surrender to God; we believe it will get better; we vent on Facebook; we write; we serve others who are in more dire straits. We learn to calm our bodies despite the struggle to breathe. How ironic, since breathing is the best way to calm, or down-regulate, the survival mode we get into when we are threatened.
The antidotes to traumatic stress are many, especially having good support and people who understand you. There is also hope. After transplant, though we have a threat to our lives, we carry hope that things will get better and we will survive. We may set goals and work slowly but surely towards those goals. Sometimes there are many small traumas on the way to healing, such as an unexpected intubation, sepsis, pain or arrhythmia. But there’s always hope things will get better.
It is well known that being exposed to a traumatic event is itself a form of trauma. Watching one of our peers decline and die on Facebook can be traumatic for us. The potential trauma of transplant can also impact caregivers who witness these experiences. One of my friends repeatedly had her airways collapse and her mother had to bag her multiple times until the ambulance came. Our caregivers often don’t get enough attention to acknowledge how hard this journey has been for them.
Lastly, I know CF doesn’t happen in a vacuum. On top of CF, if you have a history of other traumas, including early loss, sexual abuse, physical or emotional abuse, or witnessing violence or addiction, you are juggling more risk factors for a trauma stress disorder. I hope you’ll consider talking to a therapist if you notice symptoms getting in the way of living a full and happy life. When it comes to trauma, there is nothing to be ashamed about.
I acknowledge having a transplant is an incredible blessing. I can breathe, work, play and live! But how do we take the traumas of CF and learn to live with them? How does this trauma define us or not define us? Having survived a lung transplant is, above all, my greatest accomplishment in life. I feel stronger as a person for enduring this struggle. Being able to move forward to set goals and find meaningful ways to appreciate this life has been incredibly important for me. Some of the questions that might help us see how we’ve recovered from a traumatic transplant might be: “Who am I now that I’ve gotten through this? What does what I have been through say about me?” This is our truest spiritual medicine. The ideal situation in thriving with CF is being able to learn survival strategies and then see ourselves in a different light, having overcome a very difficult experience. I love my friend James, who says, “I’m a badass.” Yep, that’s how to do it. Similar to James, I remind myself, often, if I can survive a transplant, I can overcome anything.
Speeding past 50: Help Where You Can
In this Spirit Medicine, I’m compelled to write about a subject that is seldom discussed when it comes to transplantation. Having a lung transplant is an extraordinary miracle. We are so fortunate to have the kind of disease where we can take out the sick part and put in a new part — all thanks to our organ donors.
Not all of us will have an organ transplant. Some of us are fortunate enough to live our entire lives with our original organs. Our organs may be scarred and damaged, but still we may not have a transplant. I hope that my lungs and other body parts last my entire life.
Now, don’t get me wrong. I don’t have everything that I started with. I have had removals and a form of amputation, but I never have had anything added. I guess that I am a ROLWMOP, that is Retired Old Lady With Mostly Original Parts. The mostly refers to mastectomies and such.
Even though I will not have a transplant, I still am interested in helping people who need a transplant. Since I don’t have a lot of money, I wondered how I could help. Let me tell you a story about how many of us who are not able to give money still can offer help.
In the early 1990s, one of my friends who had CF received a heart and bilateral lung transplant at Stanford University Hospital in California. That was standard procedure back then. The thinking was that it was easier to transplant the heart and both lungs as a unit, rather than doing separate surgeries.
About a year later, another friend had the same surgery, also at Stanford. His mother was his support system, and she moved to Palo Alto to be with him. She didn’t drive and she needed a place to stay. Stanford had a housing system that was outstanding. I believe that it cost only about $10 per day to stay in a nice apartment. The apartments were right across the road from the hospital and were fully furnished.
It happened that an apartment was available to her from the day of his surgery. Usually, the apartments were not available until the patient was discharged from the hospital. Things just happened to work out this way for them. When he got out of the hospital, they both lived in the apartment until they reached the allotted six weeks. (At that time, the apartments were available for only six weeks per patient.) When the time was up they, again, needed a place to stay.
Someone from their church here in Oregon contacted a nearby church to find a place for them. A member of the congregation offered a place at their home. As it happens, the man making the offer had a niece whose young son had CF and he felt the need to offer help. (The man’s brother was one of my husband’s bosses and neither he nor we were aware of the connection until later.)
The offer of a place to stay took a load of stress off my friend’s shoulders. He knew that he had a place to stay as long as he needed it and he didn’t have to worry about safety or cleanliness. Surely it helped him heal more quickly.
After my friend was back home, we had the opportunity to talk about the importance of a place to stay while recovering from transplant. He explained how that kind offer of help made such a difference to his recovery. I stored that bit of information in my mental file cabinet.
Later that year I read, in our newspaper, of a couple from Eastern Oregon who were raising funds and looking for a place to stay in our area while he waited for a heart transplant. Their story recalled that bit of information in my mental filing system. I showed the article to my husband and asked him if he thought that we might be able to offer some assistance. We knew that we didn’t have enough money to be of any great help, but we did have a studio apartment above our detached garage. Maybe that could help.
We contacted them and explained what we had to offer. They were going to be in our area the following week for doctor appointments so we asked them to come and look at what we were offering. They did and they liked what they saw. During the visit, we also found out that he was just a few months younger than I, and he had contracted rheumatic fever when he was about seven. That illness damaged his heart and had severely limited his activities ever since. We each understood about chronic illness and chronic fatigue.
The couple returned to their home to wrap up all the business they needed to before being able to leave for an unknown length of time. They moved into our apartment in February of 1992. He didn’t get his new heart until January of 1993. During that time, he had suffered a couple of strokes and was failing fast. The transplant definitely saved his life.
They finally were able to return to their home after being with us for 18 months. We never could have given the equivalent of 18 months of rent, but giving them a home for 18 months was an easy way to help.
Perhaps you have an extra room at your home that could provide someone a place to stay while waiting for “the call.” If not, maybe you could provide some transportation to and/or from medical appointments. Maybe you could do some grocery shopping or cook some meals for someone who is waiting. I am sure that there are many ways to offer assistance, if you live in the area of a transplant center. Even if you are not near a center, you still might be able to help someone who is waiting for a transplant. A person might be able to stay at his or her home while waiting but still need help with routine chores. We all can understand how exhausted we can get as we lose vital capacity. All help will be appreciated. Surely most will need some help with fundraising. Just helping to get the word out about fundraising may be a way to provide really big help.
It probably is not realistic to think that any of us could provide all that any person needs before transplant, but we might be able to provide some assistance. If each of us does what we can, life may be easier for someone else. It is important to remember that even people who don’t have CF (such as the man who stayed with us) can require organ transplants. There is little need to worry about “catching” something from them or giving some of our bugs to the person waiting for transplant. Just use good cross-infection-protection techniques and wash your hands a lot.
I hope that 2019 will be a good year that is filled with love, laughter, good health for all and definitely with peace.
Stay healthy and happy,
The Role And Impact Of Lung Transplantation In Cystic Fibrosis (CF)
As a general rule, people with CF are referred for lung transplantation when one of the following occurs:
• FEV1 <30-40% predicted
• Hospitalization with ICU stay
• Escalating pattern of pulmonary exacerbations
• Need for oxygen or BIPAP device
People with CF need both lungs transplanted due to the presence of infection. If just one lung is replaced, the infections in the old lung could spread to the new lung.
Major Determinants Of Lung Transplant Wait Time Are:
• Lung Allocation Score (measure of waitlist urgency balanced with expected one-year survival after lung transplant)
• Blood type
• Height range
• Donor availability
The number of lung transplants done in this country is limited most by the number of suitable donor lungs.
•Donor has one or more risk factors for increased risk of transmission of HIV, hepatitis B and C based on their behavior or history (criteria created by the U.S. Public Health Service).
• Increased-risk donor status has nothing to do with the quality of the lungs.
• The donor has tested negative (normal) for these three
infections,but there is a very small chance that he/she could have gotten one of these infections a few days before death and the tests wouldn’t show it yet.
• Lung transplant recipients are informed that the donor is considered an “increased-risk donor” at the time of the offer and recipients have the right to refuse the offer.
• There is special testing for a year for HIV, hepatitis B and hepatitis C to make sure the infections were not transmitted. Treatment is available for all three infections.
• Accepting an increased-risk donor increases chances of getting a transplant by opening up more potential lungs.
Ex Vivo Lung Perfusion (EVLP):
• Treatment of lungs outside of the donor body to improve their quality; it can last up to six hours.
• Towards end of EVLP, the transplant team makes a final decision if the lungs are ready for transplantation.
• Not FDA approved in this country yet, but there has been extensive experience with it worldwide, particularly in Toronto.
• Requires written consent from recipient prior to transplant because it is technically considered investigational.
• Using EVLP increases the number of available donor lungs because some lungs may be turned down without it.
Types Of Lung Donors:
• There are two different types of donors depending on the way in which the donor dies. Using both types of donors increases the number of donor lungs available.
• Donation after circulatory death (DCD) = life support is removed (family decision) and organs are removed after donor dies (usually within 60 minutes.)
• Brain death donor (BDD) = patient meets criteria for brain death (legally declared dead) and organs are removed.
• Out of respect for donor family privacy, recipients are not told details about their donor (age, gender, location, etc.) by the medical team but have the opportunity to write a letter and connect with the donor family after one year.
Life After Lung Transplantation:
• Generally, people with CF have the greatest improvement in health-related quality of life of any patient group. Most people with CF can stop doing routine airway clearance sessions after lung transplant, which is huge! But nothing good comes easy…and lung transplant is not without its issues. Below is a brief summary of the most common complications after lung transplant:
• Lung transplants have the highest rejection rates of any solid organ transplant, likely because the lungs are in communication with the outside world. As a result, lung transplant recipients require the most immunosuppression of any solid organ transplant recipients. This can lead to mediation side effects and more complications, such as infections or the development of cancers, especially skin cancers.1
Adapted from The Registry of the International Society for Heart and Lung Transplantation: Thirty-fourth Adult Lung and Heart-Lung Transplantation Report-2017. Journal of Heart and Lung Transplant. 2017 Oct; 36(10): 1037-1079.
• There are three forms of rejection that occur after lung transplant:
• Acute cellular rejection: occurs mostly in the first one to three years after transplant, treated with high dose steroids and increasing immunosuppression medications.
• Antibody mediated rejection: can occur at any time. Donor develops an antibody protein that specifically targets a protein from the donor; treated with plasmapheresis and special immunosuppressant medications that target the immune cells that make antibodies.
• Chronic rejection also called chronic lung allograft dysfunction (CLAD): historically called bronchiolitis obliterans syndrome (BOS); generally occurs three years or more after lung transplant; describes the situation where lung function has been lost without a clear reversible reason, does not significantly respond to treatment in most cases.
• Despite the complicated nature of lung transplantation, people with CF generally enjoy full and rewarding lives after transplant and have the longest survival of any patient group after lung transplant. The median survival for people with CF after lung transplantation is 9.2 years overall, and increases to 12 years for those who live to the one-year mark (which ~90% of patients do.)
Cutting Edge: New Technologies Being Investigated For Use After Lung Transplant:
• The role of pharmacogenomics (information about a person’s genetic makeup to choose the drugs and drug doses that are likely to work best for that particular person) is being explored to help customize the selection of immunosuppression medications for each patient.2
• New non-invasive ways to detect injury to the transplanted lungs are being investigated one of which is a technology called “cell-free DNA” that can detect donor DNA material in blood and bronchoscopy samples.3
1. The Registry of the International Society for Heart and Lung Transplantation: Thirty-fourth Adult Lung and Heart-Lung Transplantation Report-2017. Journal of Heart and Lung Transplant. 2017 Oct; 36(10): 1037-1079.
Focus Topic: Time For A Transplant Evaluation
In my opinion, one of the most difficult conversations you will ever have with your CF care team is: is it time for a transplant evaluation? This can be both devastating and emotional. Hopefully, I can help make this time and process a little less difficult.
My PFTs had been declining for years. I was on IV meds and in and out of the hospital at an increasingly frequent rate. I was feeling worse and worse. My energy level was declining and my body was requiring more and more sleep. So I started doing research. I was 25 years old and wanted options. I was calling other cystic fibrosis (CF) patients and even started calling different transplant centers, trying to find out additional information. I began reading anything I could find about lung transplants. I wanted as much information as I could get on the subject of lung transplantation.
Upon finding out all the information that I thought was pertinent and relative to me, I approached my family. We discussed it and prayed about it. My family and I were convinced it was time to have the talk with my CF doctors. While I waited to get an appointment with my CF doctor, I continued to research transplant centers in my area so I could be as prepared as possible.
When my appointment came with my CF team, I presented all my research, data and my thoughts on a future transplant. During this discussion with my CF doctors and my parents, it was determined I should get a transplant evaluation. My CF doctors would put in the paperwork with my insurance company and I would have to wait for approval from that company to pay for the transplant, before being allowed to schedule a transplant evaluation. The wait was nerve wracking and emotional.
This has been my experience, I have been evaluated for transplant three times at two different hospitals, and each and every time it is both nerve wracking and emotional. But for the purposes of this article, I am going to focus on the first time I was evaluated for transplant.
I was living in and being treated for CF in Orange County, CA. But for a transplant evaluation, I would be going to Los Angeles, CA. Not exactly far (40 miles away), but still out of my comfort zone. I would be meeting my new transplant care team. I had heard good and bad things about the evaluation process. How would I get along with the team? Are there new recommendations for pre- and post-transplant? Would I have to relocate? What are the costs? My head was spinning.
Next came what I call “The Alphabet Test.” This basically meant I would do every blood test, procedure and PFT known to the CF world, and even some I didn’t know. As CF patients, we all have had plenty of these through the years, but now I had to repeat them in a period of three to four days prior to seeing my transplant team. One of the benefits of doing all the required testing and procedures is that I quickly became familiar with the hospital where I now was.
After all the tests were done, it was time to meet the new team. I started with my pre-transplant nurse coordinator, followed by the transplant dietician and then the social worker. Next came a transplant nurse practitioner followed by one of the transplant doctors. We talked about my above concerns and questions. Tests were reviewed, along with the recommendations on what needed to be improved upon and then finally the reason I was there. Was I a candidate? Was I early or was I in my transplant window? It was determined that I would be listed on the UNOS listing for a bilateral lung transplant.
When this all happened to me, I was working part time. I had already had to stop working full time. I was also going to grad school part time. But the idea of transplant hit me like a load of bricks. Shortly after being listed I then stopped working and going to school. This new reality didn’t help my mental health. While talking with family and my care team, we decided I should try some anti-depressants to help get me through this difficult time.
My recommendations for anyone getting a transplant evaluation are:
1. Research and make a list of any and all questions for your transplant team.
2. Bring loved ones with you to your evaluation meetings with the team. An extra set of ears comes in handy.
3. If you want, reach out to a CF transplant support group in your area; some are online, others are at the hospital. Usually the transplant team or social worker can put you in contact with a support group that works for you.
4. Remember, you will get along with some team members better than others. Use those team members as a point of contact.
5. Pray about it.
6. Never give up.
Second Wind: My Lung Transplant Propelled Me Toward Happiness
The first time I had The Talk, in 2015, it felt as though Atlas had finally surrendered his crushing burden — to me. The atmosphere collapsed upon my already weary shoulders, and a violent buzz invaded my ears.
I heard nothing but that buzz and saw nothing but the wretched pink brochure resting casually in my doctor’s outstretched palm. Splashed across the cover: LUNG TRANSPLANT.
The Transplant Talk is infinitely more awkward than The Sex Talk and a Shakespearean tragedy that dwarfs The Breakup Talk. The doctor might as well have handed me a scalpel and shovel and said to start scooping my organs out.
The cystic fibrosis community’s greatest treatment is hope. Hope in a cure or another game-changing, disease-modifying therapy. But that hope has given root to a dangerous problem: the transplant taboo. We don’t like talking about the T-word. We hear all of the horrific stories and hope that a cure is found before we get to the point of transplant. For many of us, once we get to the point of needing a transplant, we aren’t prepared for The Talk, and so we choose death. The procedure is that scary or, at least, the idea of it is. As Albus Dumbledore once wisely said about the Voldemort taboo, “Fear of the name increases fear of the thing itself.”
The taboo crammed fear into every crevice of my trembling spine. When my doctor uttered the T-word, I nearly gasped. Did he just say that? I was revolted that such a thing would be mentioned. What about my cure? I always bounce back in my health! Always!
I didn’t. Well, not until 2017. We’ll get back to that.
For months, I lived in frantic paranoia, like the big T-word was stalking me, waiting for its prey to finally plummet into end-stage CF. I villainized what could liberate me.
I did “plummet” in 2016 when the fungal Candida slipped its way into my bloodstream and shattered my body with septic shock. After four days of hallucinating and, well, dying, I awakened to loved ones’ teary eyes and the sobering reality: It was time to gamble with the highest stakes.
Okay, side note: I’m not the biggest fan of the descriptor “gamble.” I hear it often from people afraid of transplant. A gamble implies results aren’t obvious. When it comes to transplant, the outcomes are obvious: you get the transplant or you die. Maybe you’ll get the transplant and still die in the process, but the lack of trying has proven results. Sorry if that gives you the jitters. Stick with me, this story gets brighter.
My CF care team sent my medical records to lung transplant teams across the United States. I had Mycobacterium abscessus, which is antibiotic resistant and very unfriendly, so transplant docs didn’t wanna mess with me. In 2016, I also became completely deaf (long story), so recovery would be logistically complicated. I got rejection after rejection before, by some miracle, The University of California, San Francisco, picked up my case.
I moved to California from Hawaii and fought through the rigorous and cheerfully fun transplant evaluation until I joined the waitlist for transplant. My new CF doctor sat across from me on the eve of being listed and said, “You’re at the end of your rope.” It was an easy message to lip-read. She asked if I was ready.
Sink or swim? I was at the end of my rope, but doctors offered me a new rope. Not really a gamble. “I’m ready now.”
I waited a mere five weeks on the list, six months after my septic shock, before I ended up on the operating room table in January 2017. Nurses scrawled notes on deaf-friendly, dry erase boards, updating me on what was up as they slid needles into my skin. They slathered orange antiseptic war paint on my chest, and my heart beat drums in my deaf ears — badoom, badoom, badoom. I felt the flush of sedatives course through my veins, cool as the Rubicon’s waters. I closed my eyes. Badoom, badoom, badoom.
This is the part in the script where I wake up taking deep, full, glorious breaths. But I’m talking about real life: I woke up wheezing into a ventilator. Yet, while the plastic tubing made me feel like I was choking, I no longer had the drowning sensation of gooey globs clogging my airways. That was glorious.
Within 24 hours, I was walking. Within 24 months, I traveled to four countries and a dozen states, picked up three new sports, won two medals in the Transplant Games of America, worked four wonderful jobs, founded a chapter of the Lung Transplant Foundation, laughed loudly without coughing and cultivated the most beautiful memories of my entire life — so far.
I told you things got brighter. Many people view lung transplant as an extension of misery. For me, it was a reset button on my life that has propelled me into extraordinary joy. I feel as though I haven’t lived until now. I am thriving where, before, I was surviving.
I won’t lie to you by pretending my case is representative of all transplant cases. People die painfully, have torturous medication side effects, lose bodily abilities and are fatigued. So, okay, maybe transplant is sort of a gamble in that we don’t know how it will turn out. But I think the jackpot is a lucrative temptation worth gunning for. To date, I’ve met only one person with a lung transplant (out of many dozens) who said they regret getting transplanted.
I still require medications (though they’re all oral pills), have the occasional doctor visit (every six months now) and need to be careful with my artificially compromised immune system, but I’d say that this life is a fourth of the CF burden. That makes me feel like Superman.
Of course, I have the kryptonite of potential organ rejection (when an immune system attacks transplanted organs due to them having different DNA), but I’ve found solace in my old friend, hope. Hope that a rejection cure is around the bend so I no longer have to suppress my immune system or plan for “early” death (maybe a late death, since I should have died long ago!). Hope that I will continue to survive against the odds like I always have. Hope that my most incredible memories yet await. But if those hopes don’t become reality, I could still die tomorrow fully content because the past two years have been my best yet.
I didn’t think I had it in me to be adventurous; to travel the globe and pursue daring experiences like rock climbing, competing in national competitions and delivering sermons at churches. But transplant has provided both life and a lust for more life.
I’m proud to say that CF does define me. In a good way, it motivates my current lifestyle. After living more than two decades in fear and strength against the fear, I now fervently attack life in gratitude for what I’ve lost and gained. I’ve been intensely joyful, my decade-old anxiety disorder seemingly evaporated, and the best part of all is that I’ve used my second wind to help other people through nonprofit work, motivational speaking and urging others to consider transplant as a friend rather than as the Bogeyman.
I hope that you get your cure or life-changing therapy. But, in the meantime, chase the lovely stories about transplant. Trust me, I’m not the only one.
Lungs4Lovey: The Journey
For years I was imprisoned by this monster choking me out. CF, the invisible demon. Not only did it affect me physically, it ate at me mentally, emotionally and spiritually. It held me captive with bitterness, and I felt alone in my fight. That all changed about four years ago.
With lung transplantation on the table for discussion, I knew a shift was required. I didn’t want to wait until friends saw me with oxygen tubing stuffed in my nostrils to explain what was going on with me. And so I started on my journey of becoming brave — a journey that I chose to live out loud. I opened up about CF and going through the evaluation; shared the good, the bad, the ugly. My story was met with grace, love and encouragement. Some even called me an inspiration. Who knew?
My lung disease was always aggressive, ever since the day I was born. My first year, they didn’t expect me to survive. I was in and out of the hospital, and the medical staff didn’t think I was going to make it. My parents stayed in my room, praying over their baby girl, the only control they had. Through the years, I persevered through repeated respiratory infections, coughing continuously and hawking up buckets of mucous from my drowning airways. I was always consistent and compliant with my treatments…that, sadly, wasn’t enough to save my tired lungs.
I first got the “transplant talk” at 20 when my lung function dipped below 50%. At the time, I quickly dismissed the idea, never thinking that I would get to the point of needing transplant surgery. Overwhelmed by the magnitude of a transplant, I didn’t think it was something that I would choose. My sister, who has always been a vital part of my journey and a bright light in my life, gave me a book that completely changed my perspective. The Power of Two, was written by Isa and Ana Stenzel, twins who had bilateral lung transplants and who were thriving in their new life. I looked to them and thought: if they can do it, so can I. In that moment, I decided that transplant was an option for me so that when necessary, I too could have a new lease on life.
Fast forward less than a decade…In my mind and those of my support squad for that matter, transplant was Plan B. Something that we would prepare for, but weren’t planning on anytime in the near future. Life, however, had other plans for me.
From May 2015 up until August 18, 2017, my life was a whirlwind to say the least. During that time frame, I was: forced to resign from a position that I loved; hospitalized continuously with minimal breaks in between; evaluated for transplant; the sickest I had ever been in my whole life on hi-flo oxygen, had an emergency listing for transplant and actually was transplanted.
Benjamin, as I fondly referred to my oxygen, was the most challenging adjustment. I had to release my ego, slap some tubing in my nose and bravely approach public places with confidence, while enduring stares and insensitive comments. I witnessed the best and worst of humanity during those times. It drew out the pride I had about showing my invisible disease and grew me into the person who now lives out loud in every aspect of CF and life in general, — a much needed life lesson.
At my sickest point, I was on 55 liters of oxygen and 60 liters of air-flow with an HF cannula. Admitted to the ICU for the first time ever was alarming. I was transported to Boston where I was an emergency addition to the list for transplant; I was number one on the list for a month, with a Lung Allocation Score of 86 out of 100. During this time, my family created a fundraiser for me, Lungs4Lovey (named after “Aunt Lovey,” as my nephews call me). Miraculously, I bounced back to requiring only a few liters of oxygen and was transferred back to my home hospital in Vermont, where I waited five months total for new lungs. This was one of my most favorite times of my life: I witnessed a community that rallied around me, love from those who supported me and the unsung heroes who kept me alive. It was a radical time.
I was hospitalized for six weeks leading up to transplant, however the antibiotics…had stopped working. I was getting worse rather than seeing improvement. Unsure of when the call would come, I decided it was high time to start running again, even though I couldn’t breathe. I became known as the chick running around outside the hospital, while towing a tank with 6 liters of oxygen! I ran every single day. It served as airway clearance and emotional therapy and, once again, proved to the world that whatever I set my mind to accomplish, I was unstoppable.
I was (and still am) unbelievably blessed to experience life on both sides of the coin. My precious parents taught me the true meaning of unconditional love. My mom never left my side and my dad made sure that I was always provided for. This is the true definition of caregivers — the ones who are in the trenches with you, fighting right alongside of you and never giving up on you. I was also blessed with hospital staff who became my family, friends who sat with me in my struggle and those I don’t even know who prayed for me all around the world.
It has been an adjustment transitioning to post-transplant life. I struggled with depression and anxiety something fiercely for the first several months. My life had completely changed in every area, leaving me with an identity crisis. New medications bring unfamiliar side effects; learning my new routine and how my body reacts was challenging. Talking to others who “get it” has been essential to my success. Additionally, I took a cross-country trip in May for two months by myself, driving across the top of the states, all down California and swooping the South. I knew that I had to find out who Lara was in this new life, to venture out by myself and be brave in a new way rather than fighting for my life every day. It was a healing journey and one that has been monumental in my restored hope.
This past summer, I attended the Transplant Games of America in Salt Lake City, Utah. They offer so much more than competition between transplant recipients. The games were a source of healing for me: camaraderie to the fullest extent and learning more about how to navigate post-transplant life. I met numerous recipients, living donors and donor families during the week-long event. I connected with a donor dad who taught me how to write my letter to my donor family when I am ready to do so. My hope is that I will meet my donor’s family and become best friends with them, fulfilling all the items on their life list. Each day that I get to live, I thank my donor for the gift.
My new life consists of regular adventures: Hop in my car at a moment’s notice to take a road trip. See friends and family I haven’t been able to travel to see in years. Belly laughing (without hawking a loogie) with my loves. Visit national parks that are on my life list. Drink in every moment. Revel in the deliciousness of each breath that comes easy and strong, devoid of mucous and pain. It’s a beautiful life that I lead. I am so grateful that I have been afforded this opportunity. The kindness of a stranger saved my life; because that person said yes in time of tragedy, my life began anew.
There’s a formula for living your life with purpose when everything else is out of control, when the largest capacity of your existence is out of your hands. You have to believe in your future with every single breath you take. You can’t allow the forces of darkness to take root or else they’ll suck you into a pit, to the point of no return. The battle is between your ears, not in your body. I conditioned my mind to fight. I knew that if my mental, emotional and spiritual game was strong, then I could handle anything physically. That was the easy part. And it still proves true today.
My journey has taught me life lessons that I wouldn’t have learned otherwise. Being so close to death changes you in the best of ways. It has given me an unshakable faith. I’m intentional in all of my diverse relationships. I rid myself of toxic ties. When you experience that point in your life where matters are life and death, everything else seems trivial in the grand scheme of things. And it has given me the ability to be completely authentic. With every fiber of my being I can say that if given the choice, I would not change one thing about my journey. I’m stoked to be alive — each day is a gift to live life with purpose!
Running On Love And Donated Organs
I was diagnosed with cystic fibrosis (CF) when I was 13 months old. I was born in 1984; the average life expectancy for someone with CF was then still in the teens. Growing up, whenever I divulged my health information, I always insisted that people not look up CF because my case was different. I didn’t want people to feel bad for me or treat me any differently than my healthy friends. Besides waking up at an alarmingly early hour to make sure that I had time to take all of my medications and do my nebulizers and vest treatment before school each day, I tried to be as normal as possible. Of course, as I got older, and CF reared its ugly head, being “normal” became even harder. Oftentimes I felt like I would never have a career or find someone who would want to fall in love with me and spend his life being burdened by CF. Luckily, I was very wrong.
I went away to college, not letting CF limit me. I graduated with a bachelor’s degree and then decided to go to nursing school and in 2008, became a registered nurse — a career that was definitely inspired by all of my interactions through the years with so many amazing nurses who cared for me throughout my hospitalizations. In 2008, I also started dating the most amazing guy in the world. Life was really good. I worked as a nurse on a cardiac floor gaining experience that ultimately led me to where I felt like I was meant to be, the neonatal ICU, caring for the tiniest of patients.
In 2011, my doctor suggested that I stop working. It was then that the word “transplant” was mentioned. I was extremely scared of the entire process. I knew of many CF patients who had passed away while waiting on the list for lungs. I was 27 years old, and all I wanted was to be healthy, work full time and spend time with my family and boyfriend. But if I didn’t have a transplant, I knew that my time with Joey and my family was severely limited. Having a transplant is scary. In a matter of months, I went from working full time, to being home bound, attached to oxygen 24/7 and needing help doing almost everything. I was on the list for six months before I got the call that there was a match for me. Right before I got that call, I really didn’t think that I had much more time left on earth.
On November 11, 2011, I ventured into the city with my boyfriend and his family to see the Radio City Christmas Spectacular. Just being out for a few hours wore me out completely. When I got home, I passed out. At 11:11 p.m. my phone rang with the call that we were all waiting for. I was extremely lucky that the first call was a go. The lungs were perfect and surgery was scheduled to begin very early the next morning. When I finally woke up and my breathing tube was pulled out, I was in so much pain but, despite that, I still felt how amazing it was to be able to take a deep breath in and not struggle. Growing up, I never realized how difficult it was to breathe at times, because I didn’t know anything else. Breathing with healthy lungs was absolutely amazing.
In 2012, Joey surprised me with a day in the city. He literally made it absolutely perfect in every way and ended up proposing. Here I was, healthier than I ever had been and about to spend the rest of my life with the absolutely best person I had ever met. I also went back to work full time. I didn’t think life could possibly get any better.
In December of 2012, I was admitted to the hospital because I was having trouble breathing and running fevers on and off. I was diagnosed with ARDS (Acute Respiratory Distress Syndrome). A few days into my hospital stay I was sedated and put on ECMO life support and dialysis. My lungs basically shut down and my kidneys stopped working. My family and my fiancé didn’t know if I would pull through this. With the grace of God, lots of prayers and the best medical team, I was taken off all machines and released from the hospital just in time to ring in the 2013 New Year.
I started to make improvements. Recovery was slow, but I was getting stronger day by day and I even gained back some lung function. I returned to work and continued to look forward to my upcoming wedding. In August, my sisters and I participated in a race; I noticed that just walking at a fast pace had me extremely tired and really short of breath. My doctors started to run many tests and said that it could be a lot of different things. One of those things was chronic rejection. All of this was happening with my wedding just weeks away.
I married the man of my dreams on October 11, 2013, and we went on a fabulous tropical honeymoon. The day after we returned home, I went straight to my doctors. They scheduled me for a Nissan fundoplication to help stop the acid reflux that could have been causing my lung issues. I stopped working again and put everything I had into figuring out what was wrong with me and trying to get healthy again. In January 2014, I started photopheresis treatments to try to stabilize my lung function but, after six weeks of treatments, there was no change. My doctors decided that campath, a treatment to completely wipe out your immune system was too risky because of certain bacterial growth that my lungs were currently culturing.
I was officially in chronic rejection and my medical team decided that my only option left was a second bilateral lung transplant. However, with my kidneys not fully functioning, I was told that I needed to have a kidney transplant before I could be a candidate for a second lung transplant. Two of my sisters immediately signed up to be tested to see if either of them was a match. They both ended up being matches for me and they actually fought over who would get to donate her kidney to me. I certainly don’t have the best luck in the health department but, between my family and friends, my cup is overflowing with love. In July, my sister, Christine, graciously donated her kidney to me. About a month later, I was then listed for a second bilateral lung transplant. I was on the list for a second lung transplant before I even celebrated my third transplant anniversary.
Because of the long wait times in New York, my team suggested that I look into Duke. My husband had just started a brand new career and couldn’t take time away. My sister and mom joined me in North Carolina for a few days while I was being evaluated at Duke to possibly be double listed at Duke and Columbia Presbyterian. I didn’t believe that I would survive the six-plus month wait here in NY. I had been married for only one year, and I thought that I was going to die and leave my husband and puppy all alone. Duke decided that I was ready for transplant, but that I was way too skinny. They wanted me to go home and work on trying to gain weight so that I had a higher BMI. I went home pretty defeated because trying to gain weight was impossible. I already had a feeding tube. I had lost my appetite completely and was barely eating anything by mouth. On December 4, 2014, I started to need more oxygen than my machines at home were capable of providing. I was admitted to Columbia Presbyterian, my transplant center here in NY. After a few days it became clear that I was too sick to continue waiting for the call at home. I was told that I was to stay in the hospital until a call for organs came. I honestly didn’t think that a call was going to come. I thought that I was going to die in the hospital waiting for that second call.
The days were long and scary. I had someone from my family, either my husband, my mom or a sister stay with me every single night because I was convinced I was going to pass away, and I didn’t want to die alone.
My friends in Florida who also suffer from CF started an online campaign called “Oomphforkaty.” When I was going through my first transplant, my friend Kasey had friends and family praying for me. She had asked me what it was like to have people who didn’t personally know me, praying for me. I told her that it gave me the “oomph” to keep fighting. She took that word “oomph” and turned it into a movement. People started taking pictures holding signs that said “Oomphforkaty” and posted them on social media. It ended up going viral; even celebrities took part. The posts made me smile every day and gave me something to look forward to during those long, dark, painful days in the hospital. After 77 days in the hospital, two dry runs for lungs that didn’t end up being good, and more “oomph” posts than you could ever imagine, I went into surgery and had my second bilateral lung transplant on February 19, 2015.
I waited for both of my lung transplants about six-and-a-half months from the time I was listed, to the time I went into surgery. My life post-transplant is so different from pre-transplant. My day used to begin at the crack of dawn; I would wake up so early to make sure that I had time to do all of my nebulizers and a good long vest treatment before I was able to even get in the shower. Now, I wake up, jump in the shower and go. I still take a ton of pills and am followed extremely closely by my doctor. Being on high doses of anti-rejection meds leaves my body severely immune compromised, and I am always extra careful around sick people.
In 2016, I decided that as much as I loved my job working in the NICU, and the fact that the unit I worked on was very clean, going into the hospital every day for work and travelling around to different floors as I sometimes had to do, was nevertheless just not worth the risk anymore. I am extremely lucky and blessed that I have had two lung transplants. I know that thirds aren’t really done and are extremely rare. If this set of lungs fails me, I don’t have many options left.
My husband, Joey, has been my absolute rock. In our first year of marriage, I was so sick and frail that often he had to shower me and do things for me no young couple should have to go through. Emotionally, I get really down sometimes, knowing that I had a career I loved and now I am staying at home just trying to stay healthy. I feel like I am a burden on my husband, who works full time as a firefighter and part time as a nurse to just keep us afloat. I have to constantly tell myself that he would rather have me alive and healthy than have me working and possibly exposing myself to unnecessary germs and limiting our time together on this earth.
Survivor’s guilt is something that I also struggle with, often. So many people with CF have passed away before they were even transplanted for a first time, and here I am, three transplants later, feeling great. I don’t understand why I am still here and others no longer are. I am still alive and breathing because of my donors. I know that it is normal to feel so many different emotions; I need to just be thankful for every breath that I take, and any extra days I am alive. And I want to do my best to hopefully make everyone proud.
I think about my donors every single day. I have written to both of my donor families but unfortunately never heard back. I just hope that they read my letters and realize what a profound impact their loved one’s organs have made in my life. I don’t know where I would be today without my donors and the constant love and support of my husband and my entire family. I don’t know what trials and tribulations the future will bring, but I am positive that with the amount of love I have in my life, I will be able to make it through whatever comes my way.
lung story short
Years ago I came to the decision to go ahead and, basically, “swap one disease for another.” The first time the idea of transplant came up was my sophomore year of college, which was in 2009. My lung function was 30 percent and lower. After my first evaluation I was deemed “too well.” I do believe that I was referred on time, and maybe a little early. But, hey, everyone’s body is different.
Fast forward to 2015, when I became very sick with several hospitalizations in four months, at which point my team got everyone together. It was for a very important meeting about how I needed to get on the transplant list asap. This meeting, which took place while was I was an inpatient, coincided with a planned move to Colorado.
The time from when I left Massachusetts was a whirlwind of settling in at the hospitals in Colorado and trying to get used to the elevation. The transplant team at University of Colorado Hospital decided that they didn’t want to do my transplant after they already had said, “Yes,” the day before when I had first met them. The reason was that the “bugs” I had were too resistant to all antibiotics, and the outcome after surgery would not be good if I had gotten an infection.
My sister, Laima, who became my caregiver, came to the rescue. She used her resources to find a different center. All the while, she was still living in Brooklyn and working two jobs so she could move to Colorado to help me. At that time, I was quite sick. I was in and out of the hospital every few months, on oxygen 24/7.
The University of Minnesota hospital in Minneapolis agreed to have me do an evaluation with them. So we went, and they accepted me in the spring. That following fall, I got listed after a long summer of many hospitalizations and bad infections. During one of my admissions, my sister and I decided it would be best if we moved to Minneapolis while I was listed rather than doing a med flight, which would have been expensive if I had dry runs.
So, we did it. Laima and I drove 13 hours with enough stuff to last us for our stay.
After eight months and two dry runs — one on Halloween, consisting of actually being sedated and intubated, and the second on April Fool’s Day — I finally got the real call on Mother’s Day.
Post-transplant life — what does that look like? To me, it looks like a lot of pills but no more vest or nebulizers! No more coughing basically every waking minute and throughout the night. No more gasping for each breath while walking casually anywhere. I’ve been able to hike mountains, go camping and see places I haven’t and couldn’t before! But this new life doesn’t come without its issues. Luckily, my issues haven’t been anything too major and I am thankful. I have had several admissions into the hospital and visits to the ER. I had a lot of issues with my white blood cells dropping low because of a medication for my CMV, which is a virus my donor had and which I now have. It took a while to balance being on and off the medication. I did eventually need several injections of Neupogen which helps the body with making more white blood cells. While it helped with that, its side effects weren’t so pleasant to me. I also had an episode of sepsis, which can happen to people post-transplant who have portacaths still in. It may be your center’s policy to encourage getting those out shortly after transplant.
My most recent ongoing battle is with my platelets as well as the level for one of my anti-rejection meds. Tacrolimus, also known as Prograf, is not stable. The level is always above or below the target level where they want me to stay. This often can happen, especially for the first to second year. As for my platelets, they were dropping due to the CMV virus, but that has been under control with a new medication and they still are low. Platelets are something that helps the clotting factor in your blood. This causes a lot of bruising and hard-to-stop bleeding. My legs now, most of the time, look like those of a darn Dalmatian dog. Having low platelets prevents me from being able to do certain things, such as trying out snowboarding this season.
My transplant has basically, so far, lived up to my expectations. I’ve had the inevitable speed bumps here and there, but it’s nothing I can’t handle. Having CF has prepared me for the worst and the constant anticipation of things going wrong. It has also taught me to adapt to situations quickly and to not get too upset when plans don’t work out due to illness.
I have not gone back to work yet. My transplant team wanted me to hold off longer than usual because I was having many issues and they wanted to keep a close eye on me — so close, in fact, that I have to fly to Minneapolis every two to three months, depending on how I’ve been doing, as well as having labs done every single week.
Last winter, I eased into a little part-time work by joining rover.com to watch dogs. I had looked after several dogs and, of course, I was ill while I was watching a few of them. I even had to cancel one assignment because I had to get admitted. I also got sepsis in the middle of watching a dog for a week and had to rush to the ER.
Since then, I have been quite hesitant to commit to watching people’s dog’s again. I want to find a part-time job, soon. Not working is starting to drive me a bit crazy. But when I do start looking, which will be in the upcoming weeks, I will try first in something in the field I went to school for, which is environmental science. I want to be able to use my degree, the degree I worked so hard for and that possibly had made my lung function worse. I need to make it “worth it.”
A topic that gets brought up now and then is children. Will I want to have kids? No, thank you. I’ve never had the desire to have kids myself. I actually try to avoid being near them, due to the fact that they are walking petri dishes. Having children post-transplant is frowned upon due the risks involved. Also, being around any children is a risk as well. Unless they are a friend’s or family’s child, I want nothing to do with them.
All in all, having a transplant is a choice and it comes with many risks and challenges. It’s a choice that I’m glad I made. It has given me more time for friends, family and adventures.
How My Sibling’s CF Journey And Death Affected Me
The flu was supposed to be just another setback. Although we knew how serious the flu could be, Alyssa’s prior experiences showed us she could handle it. We had been there several times before. Alyssa would get the flu, then maybe an exacerbation would flare up, but both would be taken care of quickly and Lyss would bounce back. Then she got sicker, having to go on bi-pap while in a near-unconscious state. At one point, we would learn a new word, “bacteremic” — though at first they thought she was septic.
As a freshman in college, I was like most other freshmen: I believed I knew more about the world and life than there was left to learn. It wasn’t until I had a conversation with my parents after they had just spoken with Alyssa’s doctors that it occurred to me that things were about to change drastically:
“There’s nothing else they can do.”
“What do you mean? Surely there are other options. There always are other options.”
“As of now, there aren’t.”
The perspective of that young Tré, who believed there was no way this could be the end, wound up being more youthful naïveté than resilience, persistence or intelligence. Things would change quickly in the days after that conversation. Alyssa would make a turn and come off the mechanical breathing assistance, recovering just well enough to be eligible for a bilateral lung transplant. This was typical of Alyssa: things would look hopeless, then the tides would turn abruptly and Alyssa would make a recovery.
My sister’s relentless spirit transformed my view of the world and our lives. Her story is one that I will write about and tell until my final days. It was impossible for me to know it then, but the winter of 2013 — just at the end of my first year of legal adulthood — would set the stage for a turbulent five years that would culminate in my sister’s death.
The life of a person with CF is a unique experience, even compared to the community of people with CF at large. Every CF situation is different: genetics, environment, personalities, even mere fortune all play into the progression and significance of disease severity. The life of CF siblings also is unique. Alyssa was born five and a half years before me. One of my favorite stories about Alyssa comes from when I wasn’t even a couple of months old: My mom told my big sister that her little brother also had CF and Alyssa’s response was a euphoric and innocent “YES!!” in the charming sort of way that only a child could muster. To this day, I love telling this story. As much as CF has been a burden in my life and through all of the suffering Alyssa endured, our relationship was grounded in our shared camaraderie borne out of CF.
I had hoped for a long time to go to medical school, with the ultimate goal to further CF research and be an influence in the CF community. It was during my collegiate years that my passion for learning, science (mostly biochemistry), medicine and so much else fully came to fruition. During this time, my sister was in the hospital frequently, further motivating me to understand CF disease progression and transplant medicine, so I could help both of us. I became the understander, researcher and translator for Alyssa and my parents. I believed that Alyssa’s fate was partially in my hands, so I made it my responsibility to best understand everything.
When Alyssa first was evaluated for a bilateral lung transplant in April of 2013, I don’t recall her voicing any reservations. Then, I couldn’t have imagined any reservations. In my mind, if there were literally any possibility of prolonging life, I reasoned that we should and, arguably, were obligated to figure out what we needed to do. Death is unquestionably a worse fate than a life of chronic disease, was one of my guiding principles.
A couple of years later — after more hospitalizations for rejection and GI issues, after my sister lost her hair and had to wear a wig (and did so admirably), and a laundry list of other problems — Alyssa was in chronic rejection. Once it was decided that she may be eligible for another bilateral lung transplant, that thought flashed through my mind again: Death is unquestionably a worse fate than a life of chronic disease. Looking back, I don’t know if I ever asked her properly, if she wanted to go through it all again. I just accepted she would. Her desire to live was something that helped her through all of the trauma she endured.
It wasn’t until after her second bilateral lung transplant, when she immediately had several instances of acute rejection, and then after she was in chronic rejection again within a year, that it occurred to me: This fate of constant uncertainty of life is a fate that just may be worse than death.
This is when I started to reevaluate my thoughts on bilateral lung transplants. For the five years before my sister’s death, I believed bilateral lung transplants were just a part of life with CF. After we do everything we can to sustain the lungs we were born with, modern medicine has evolved to the point where we can have another chance at life. This was the way it was, and no matter how terrifying the procedures themselves are, I viewed my sister’s two-time experiences with transplants as just a product of the hand she was dealt. Though it hit so close to home — twice I waited in a hospital room, unsure if I’d ever see my sister awake again, as she had her rib cage opened and a stranger’s lungs placed into her body — I managed to still view it as something that was a distant, therefore unrecognizable, part of my future. I neglected to put myself in her shoes. I failed to consider how a post-transplant life is so much different from a life with CF, where exacerbations are mostly treatable and decline is episodic. Before her bilateral lung transplants, it’s true that Alyssa’s daily life was a struggle, but it was a struggle she knew. She traded a chronic disease she knew for a chronic disease she didn’t.
After officially being diagnosed with chronic rejection (important to note here: she had been in chronic rejection for probably eight or nine months before the actual diagnosis came) in January 2018, she died on March 12, 2018. Toward the end, she told us she wanted to be evaluated for another transplant, but we all knew that to be incredibly unlikely. After two bilateral lung transplants, my sister got an extra 58 months. Those months were full of constant worries, dozens of hospitalizations, weekly blood draws, and a life that never resolved into the sort of enlightened acceptance that comes with having CF. Approximately 50 percent of people who receive a bilateral lung transplant survive five years after the transplant; my sister didn’t hit that mark with two bilateral lung transplants.
I would never say or claim my sister didn’t believe she made the right decision. Based on her constant advocacy for organ donation on social media and how she talked to us, I think she believed in her heart that she made the right decision. I’m immensely grateful Alyssa got more time. In those four years and ten months, she touched thousands of lives and accomplished a lot. She got to witness her little brother graduate college, run a marathon, become a published scientist and become a formidable advocate in the mold she developed.
Five years ago, I believed so strongly in my reasoning skills that I encouraged my sister to get a transplant because I told myself that’s what I’d do. And yet, five-and-a-half years later, I’m not so sure I’d be as quick to get one. My sister taught me a lot about toughness and resilience in the face of adversity. She embodied everything I hope to embody when I’m going through a hard time.
If I could talk to my sister, I think I know what she’d say if I had to get a bilateral lung transplant. I think she’d tell me that it was worth it because she got to spend more time with us and we got to spend more time with her, that it was worth all of the pain.
When — no, if — that day comes I’m almost certain I’d get a transplant. I just hope that I’m able to muster the strength that she so effortlessly personified.
My Journey To A Bilateral Lung Transplant
It seems very fitting that I should be writing this article about my transplant journey while on my way to Kauai. I had traveled to the Hawaiian Islands before my transplant, but what a different and challenging experience that was. I had a bilateral lung transplant on September 17, 2013. I recently celebrated my fifth “lungversary,” which is a major milestone in the transplant world.
I was diagnosed with CF when I was 3 years old and will be turning 60 in January. Along the way, I went to undergraduate and graduate school in urban planning, got married and worked until we had children. Both of our children, now 25 and 21 years old, were adopted. We also now have a new grandson! Who would have thought I would live long enough with CF to become a grandmother! I have always said having CF was like having a third child. At many times over the years, my CF required as much, if not more, attention than my family. They have always been very supportive, endlessly loving and caring, despite the challenges of living with a mom/wife with CF.
I truly believe having a family gave me the internal motivation to work as hard as I could to stay on top on my CF. It takes a strong partnership in your marriage to manage the CF roller coaster. Along with being incredibly compliant with all of my breathing treatments, I have always been an avid exercise enthusiast. My daily routine consisted of doing my nebulizer treatments/chest percussion therapy/vest, to jumping on the treadmill or Nordic track machine. I followed up with light weights afterwards. This routine sustained me for many years, even as my lung function slowly declined with age and chronic infections. I was hospitalized two to three times per year for many years. I was able to downhill and cross country ski, swim and bicycle (at Elyse’s pace) up until I caught RSV in November 2012. This was a game changer for me and ultimately led me down the path necessitating a bilateral lung transplant.
Post-RSV, my lung function FEV1 hovered around 35 percent. My world got much smaller, and doing ordinary tasks became more and more difficult. I was getting more frequent infections that required much longer hospitalizations. My children were in their late teens at this point, but I could tell the toll this was taking on their lives. By January of 2013, I was on oxygen full time. I still pushed myself to exercise daily, but it was incredibly exhausting and challenging. My husband was extraordinarily helpful and kept our home life going with some semblance of normality.
During a hospitalization in March 2013, a former doctor of mine came to visit me. She was the first to bring up the idea that I needed to start considering transplant. She told me a critical factor in transplant is that you need to be sick enough to need a transplant but WELL enough to survive it. At my next clinic visit, the CF team brought up the subject of transplant. The emotions I went through, realizing I had reached end-stage CF, were daunting. I knew I had to give this serious consideration as I wanted to live and still be here for my family, friends and, of course, my loving dog, Latte. My FEV1 was hovering around 28 percent. I thank my medical team for not waiting any longer to bring up the idea of transplant.
Very soon after deciding to consider transplant, I was hospitalized again for a CF exacerbation. I asked if, while I was an in-patient, could the required testing begin for the evaluation process. That was a whirlwind of medical testing! Gratefully, I passed all of the tests and was accepted into the Stanford bilateral lung transplant program. Then the waiting began.
In my mind, I had so many emotions ranging from fear of the surgery and post-surgery pain to the challenges recovery might present. I am a fairly optimistic person, so I tried to stay away from the negative what ifs that I could not control and focus on what I could control. Once again, exercise was a key survival component. A friend, Julie Desch, who also has CF and is an exercise coach for individuals with CF, gave me key exercises to strengthen my quadriceps and core. I literally trained for my transplant! This proved to be incredibly beneficial.
One of the key medications used for immunotherapy is Prednisone. This medication at high doses causes terrible muscle fatigue and wasting. Additionally, for three months post-transplant, sternal precautions are in place and you cannot use your arms to lift yourself from bed, from a sitting position, bathroom duties, etc. Strong quadriceps are so important. I immediately noticed mine declining while in the hospital and on high-dose steroids. Having prepared for this surgery, however, which is not always a possibility, I was able to stand up in the ICU within 24 hours post-transplant.
I was on the transplant list for two months. When I got the call, I was playing Mahj Jong with friends. It was a blur of activity getting home to pick up the items that I had pre-planned to take with me, to call family and get to Stanford. My surgery lasted seven hours. That first breath after being extubated was unbelievably amazing!!!! Almost immediately, I was off any supplemental oxygen. Those first few days post-surgery were uncomfortable. Pain management was a top priority, but I’ll admit it hurt! I was discharged 10 days after my surgery. What an amazing feeling coming home to find all of the medical equipment I needed to breathe had been removed from our home.
Coming home was somewhat daunting on several levels. First of all, I was discharged on Lovinox, an injectable blood-thinning medication, as I had three blood clots in my right arm from the PICC line. Secondly, with transplant, the necessary medications are almost entirely different from pre-transplant life. The number of medications and their schedules take some time to get used to. The first three months post-transplant, at my medical center, a patient has to have 24/7 care by a family member or friend. This is in part due to the enormity of the surgery, but also the post-surgical pain medications and transplant medications can cause some brain fog. Having CF-related diabetes is also impacted by being on continuous Prednisone. With the assistance of an insulin pump and the DEXCOM continuous glucose monitor, I have been able to keep my blood sugars under relatively good control.
Immediately after settling into being home, I was aware that my life had taken a 180 degree turn for the better! With not having to spend six to eight hours a day doing breathing treatments, I gained so much time in my day. As my pain diminished, I began rehabilitation. This started with very short walks in my home to then venturing outside into our neighborhood. One item that had become my new best friend was my Vog mask. Post-transplant, a mask becomes a part of one’s life. During those first six months, I wore my mask most of the time when away from home. Over time, I have let my guard down but always keep my mask close at hand. I will wear it if I hear someone coughing or sneezing in an enclosed area and always when traveling on an airplane.
The biggest challenge post-transplant I am facing is squamous cell carcinoma skin cancer, which is a result of immunotherapy medications. I have had several surgeries and now always wear sunscreen, a protective hat and often SPF clothing. As I have become an avid hiker, I try to find shadier trails and stay out of the sun between 11a.m. and 4 p.m.
Have the gifts of transplant outweighed the risks? DEFINITELY! Today my life is so full with family, friends, travel, volunteering and, of course, exercising. To celebrate my gratitude for my donor and donor’s family, seven months post-transplant, my two friends on my care team and I did a half marathon. At my first “lungversary,” I wrote to my donor’s family expressing my eternal thankfulness and gratitude for their loved one’s gift of life. I have not heard back from them but hope they have found some comfort in hearing from me.
Transplant life has far exceeded my greatest expectations. When I reflect on what this gift of life has given me, I cherish the time with family, friends and one of the little things, spontaneity.
Mental Health Post-Transplant
When it comes to chronic illnesses it’s no surprise that the discussion of mental health isn’t usually too far off. When it comes to transplant evaluation, transplant teams even do a mental health exam to make sure you’re in the right mental state to get you through the process, but no one ever really warned me about the problems that come post-transplant. The process to receive a transplant isn’t often easy and, though every person is different, the recovery can be just as complicated.
I received a bilateral lung transplant on September 2, 2016. It was a true miracle as I had been on life support and in a coma for three weeks. I knew the recovery process was going to be long and hard, especially since I had to learn to walk and use all my muscles again. What I didn’t realize is how much of my recovery time would be spent handling anxiety.
I was very well educated pre-transplant, I knew that new lungs weren’t going to be a fix-all for my problems, but they were going to help me live a longer life that I hoped would be healthier. I still wasn’t prepared for the depression that accompanied the surgery. In my mind, I had no reason to be depressed. I had already survived the hardest part of transplant, and I really felt things would be much easier from then on out. However, as my psychologist shared — you don’t need a reason to be depressed — it just happens.
My first year post-transplant was mentally straining; while I worked with the psychologist on my transplant team to find a good combination of medications that would allow me to function well. I learned that part of the reason I was such a mess was the Prednisone.
Steroids, such as Prednisone, mess with your emotions, and it’s pretty common for people on steroids to experience “roid rage.” I had a different problem: I cried. In the beginning, the tears were a problem. I even had a list of things no one should talk about around me, because they would instantly set off the water works. Three months out from transplant, the tears became a joke and my family really celebrated the first day I made it through without crying.
The tears were inconvenient, but another thing I was truly unprepared for was how overwhelming it was to be back in public. I was in the hospital from the beginning of August to the end of October, and I’m fortunate to be friends with many members of the hospital staff. So, when I left the hospital, it was sort of like leaving behind part of my family.
Adjusting to being back at home wasn’t easy either. The first time I went out to breakfast with my mom at a local Coney Island shop, I had a severe panic attack. I was so overwhelmed by all the people and the noise that I just couldn’t function or focus on anything. It was a scary experience for me as I’d never had a public panic attack before, and I didn’t know how to cope with it. It proceeded to happen almost every time we went out and, eventually, I learned how to breathe through it, but it took time to manage. I had to focus on one thing, take deep breaths in through the nose and out through the mouth and work to slow my mind down. Eventually, the panic attacks became less and less frequent. Even now, two years-post-transplant, I still have them on occasion and I don’t think I’ll ever be free of them permanently.
So, what are the best things you can do for your mental health pre- and post-transplant? Be prepared. What I’ve experienced isn’t guaranteed, but many people deal with a form of anxiety and depression post-transplant. Not being blind-sided by it will help you prepare just a little bit more.
It’s also important to remember it’s not your fault. A lot of these feelings are caused by medication side effects and there’s nothing you did to deserve them or earn them. The best thing you can do, though, is talk to your transplant team about any concerns you have and about what you’re feeling. They have the power to help and you don’t have to suffer.
getting a new kidney
I have been lucky enough to not only receive a bilateral lung transplant (on July 3, 1998) but also a kidney transplant (on March 7, 2006). Both were done at Fairfax Hospital in Virginia, 15 minutes from my house. I am very fortunate to have this hospital so close to where I live; I know many people have to drive long hours to get to their hospitals.
Unfortunately, one of the medications I was on for the lung transplant (Prograf) essentially killed my kidneys. In early 2005, I noticed my feet were swelling, the first clue that my kidneys were failing. I started seeing a nephrologist and she was surprised how quickly my kidneys failed, within a year of the first signs of an issue.
I started dialysis, three times a week. I was put next to an older man my first time doing dialysis, and the next time I went, he was not there. He had passed away; not a good sign! Doing dialysis made me very sick, which was surprising: since my blood was getting cleaned, how could that make me so ill? I was nauseated and exhausted after every treatment.
I lost 30 pounds in one month of doing dialysis, and I could barely walk. The dietitian at the dialysis center tried to put me on a dialysis diet, which meant no salt! That was the only time I cried, as I’m sure people with CF can understand! I didn’t even cry when I was told my kidneys were failing. A dialysis diet also means no potassium, so I would have to soak potatoes overnight to get the potassium out and cook them the next day. Don’t even bother doing this; the potatoes tasted like cement.
After trying to follow this horrible diet and losing weight, I asked the dietitian to create a more CF-friendly diet for me, and that worked. I got my salt back! No more soaking potatoes! I did have to work my way back to real eating though, starting with Cheerios, then crackers, then eggs, Ensure Plus, etc. Stuff that was easy to eat and drink. It took a while, as you can imagine. I never felt sorry for myself because, at the dialysis center, there was a teenager who sat across from me.
I couldn’t even imagine having to go through dialysis as a teenager, missing school, being different, not seeing her friends. She seemed to have such a good attitude about it. I sucked it up and dealt with it, too. At least they had little TVs at everyone’s chair to keep your mind off why you were there.
The nurses were very nice, but the doctor who, shockingly, was the head of the dialysis center was a real jerk. He prescribed an antibiotic for me without even looking at my chart. I suggested he look at my chart before prescribing a drug for me. His response was, “If you want another doctor, feel free to get one.” I was livid. I made sure the head nurse, and everyone else I could tell, knew about this. Not that it mattered, since he was the head doctor of the dialysis center, but at least I made my concerns about him known.
I did dialysis for about six months. My dad was going to donate a kidney to me, but he was not eligible due to his high blood pressure. My best friend, Kelly (we met in college and were roommates for two years), insisted she donate a kidney to me because, as she put it, she “had to save [my] life.” I could not talk her out of it.
She lived in upstate NY and I was down in Virginia. She was a very busy lawyer with a 4-year-old daughter. I could not upset her life like that! We went back and forth on this so many times that I lost count. She won me over, eventually, and she got tested. Luckily, she had to make only one trip down to Virginia to be tested. The rest of the testing was done in upstate NY near where she lives. As it turns out, she and I were a very good match!
My mother-in-law and her husband came down for the surgery and sat with me to keep me company for my last three hours of dialysis. It meant the world to me that they would do that. My family and Kelly’s family also came down for the surgery to support both of us. We are so lucky to have so much love and support in our lives.
The operation was done the day before my birthday — a great birthday present! The operation went fine, except the anesthesia made Kelly very sick (she knew about this issue before the operation and still went through with it). We ended up leaving the hospital the same day (three days after the surgery); she had to stay an extra day because of her issue with the anesthesia. We talked every day on the phone since we were both at home and bored! I was home for about three weeks before I could go back to work; I think she was off for two weeks. I had to wait until my surgical site healed before I could drive.
The new kidney went in my front, lower abdomen, and they kept my two original kidneys in, since to take them out would have been unnecessary surgery. I always have to remind my doctors when they are feeling my abdomen that my new kidney is there, otherwise they freak out when they feel a mass.
Kelly is doing great and so am I!
Kelly is the most selfless person I know. She could not understand why my friends and family were thanking her and sending her flowers. She actually thinks what she did for me was selfish on her part. She says she had to save me, her best friend, because she would miss me too much if I wasn’t around. Am I lucky or what??
Tale Of My Transplant
So you want to know about lung transplant? Well, I have quite a story to tell. When I was 18 I moved from the children’s hospital to the adult hospital. I was not eager to leave the hospital I had known my whole life. Right after my 18th birthday, however, I got a lung infection and ended up hospitalized where I knew no one, with doctors I had just met making important decisions about my health.
After IV antibiotics, I went home and was back at my baseline, which was not very good but stable in the 40 to 50 percent range. It was at my next clinic appointment that the team brought up lung transplant. That was something I knew of, obviously, but chose to more or less ignore and put zero thought into. They stressed that it was something I needed to look into because even though I was stable, it was not something you wait till you need it to decide on — or else it may not happen in time.
It was at that point I was referred to Mass General. Being 18 years old, the last thing I wanted to think about was cracking open my chest and replacing my failing lungs with new ones that, from what I had read in the short time I had been bothered to look, may not even work. So I more or less blew it off when the transplant people called to set up an appointment. I was a senior in high school and was more interested in girls and the next party. I was going to drink my body weight in alcohol, which was the best way I knew to not constantly think about my failing health. After blowing them off twice or cancelling appointments a few days before the date, they sent me a letter saying I was rejected from their program for not being timely with responses. Time went on and I continued to be hospitalized every few months for exacerbations. I would do a round of IV antibiotics and be done with it.
Fast forward to age 23. At this point, I was living with my girlfriend, working full time at Aetna doing computer work and still routinely going on antibiotics to fight off lung infections that were happening more and more frequently. One day, while working and feeling better than usual, I was walking across the building as I did multiple times a day and suddenly could not breathe. I ran back to the room more or less gasping for air. My coworkers called 911, and I somehow managed to call my mom and frantically tell her I couldn’t breathe and to meet me at the hospital. When the EMTs arrived, they did their thing and my oxygen saturation was in the 70s, which is NOT good. They immediately gave me oxygen and it went back up to a healthy range in the 90s.
I spent a couple of days in the hospital while the medical team tried to figure out why that had happened. They were originally concerned about a possible pulmonary embolism. After several tests and a sleep study, they saw that my oxygen dropped regularly while I slept, which is common with CF. At that point I started using oxygen at night.
This time they also stressed to me that I really should start taking the transplant process seriously. Because I had a girlfriend, an apartment and all that stuff, I decided I actually did have a reason to live and should look into this more. So began my adventure with Brigham and Women’s Hospital in Boston, MA.
Soon after this, I had my first meeting with Boston, where I would eventually have my transplant. At the first meeting I had to bring my “support system,” which consisted of both my parents and my girlfriend. We were taken into a room and made to watch a video giving us a brief summary of the entire process. During this appointment, my mind was spinning 100 mph and I wasn’t sure if I was terrified, confused or just ready to go for it and either feel better or feel nothing.
It was at this point I started testing. I was healthy (in the sense that I wasn’t actively dying), but I was far from healthy compared to my peers. I would do some testing and, by the time I got most of it done, the first tests I had done would expire and I would have to redo them. This went on for over a year until I finally was “approved for transplant.” My physical health was baseline so I opted to be “too healthy to list.” That meant I was approved but put on hold from the actual list.
The team monitored my health closely and I would travel to Boston, every two months to do basic tests and meet with them. While this was happening, I was 24 to 25. I was facing one battle with my health declining faster than anticipated and my mental health was at an all-time low. My four-year relationship was failing with my health playing an important factor. I couldn’t go out and do much without getting out of breath. I was constantly exhausted just functioning and since I had to maintain a full-time job to stay on healthcare to get the transplant, I rarely had the energy to do anything.
One morning, I woke up, coughed hard as I normally did and, all of a sudden, blood was pouring out of my body. It was beyond terrifying to me and was basically the nail in the coffin of that relationship. At that point I was extremely sick, and most of my friends had cut me off. As I have said, I was known for some out-of-control partying. I was 25 and it took me six minutes to walk to my car, which made me extremely irritable and depressed. I was eating almost 5,000 calories a day between shakes and night feeds via a g-tube, yet I still was 105 pounds soaking wet. It’s safe to say this was an all-time low in my life.
I can’t pinpoint exactly what happened, but it was probably a year or so after my life more or less fell apart that it started to move in the right direction. I decided I was going to live and I was determined to get this shit done. I turned 28 on November 26, 2016. A few weeks after that, I was listed and, on February 2, 2017, I got a call that changed my life.
I had been out that night until about 10 p.m. with the motorcycle club of which I am a member. I did my nightly routine and, around 11:30 p.m., was finally getting in bed. I was on O2 24 hours-a-day, and my phone went off. As I said, I was a pretty miserable person at this point and didn’t have many people in my life, so I planned to ignore my phone. I figured I would look at it and saw it was an out-of-state area code. When I answered, a woman calmly confirmed who I was and then asked, “How soon can you get to Boston? We have a match for you.”
The adrenaline took over and I actually drove myself and my parents to Boston, since my dad can’t see well at night. The next morning, after a night of testing and making sure everything was safe to go ahead, I went in for surgery. I hugged my parents, told them I loved them and off I went. Well, I like to think after the past I had had, and the fact I had almost died more than a handful of times from CF and non-CF-related events, I knew this was go time. Best-case scenario I wake up and have a second chance at life; flipside, I never wake up but wouldn’t be in pain and never have felt anything.
Well, here I am a few months short of two years later writing this and feeling better than I ever have in my entire life. To say I have done a 180 is an understatement. I went from taking 10 minutes to walk to my car and on oxygen 24 hours a day to working out and doing jujitsu multiple times a week and working two jobs, basically going nonstop.
I don’t regret anything as certain events have made me the person I am today. However, if I could go back, I probably would have taken it more seriously the first time around and not hesitated. I had actually been to the clinic the day before I got the call and my FEV1 was at 13 percent, and I was living in end-stage lung disease. Having a doctor look you in the eyes while alone in his or her office and tell you that if this transplant doesn’t happen, “You don’t have very long and you should get your things in order” isn’t anything anyone that age should have to hear, but the past is the past.
Now-a-days, I have a wonderful girlfriend who supports me mentally no matter what and without being overbearing, thinks of my health and will not hesitate to ask or tell me I probably shouldn’t do certain things. We have been able to do so many things I never thought I would be doing again or things I had never even been able to do in the past. I didn’t know her pre-transplant and, while she may never fully understand how sick I was and how bad things were, I sometimes think it is for the better. I am not sure the recovery process would have gone so well if it wasn’t for her and my parents helping me a LOT.
This has no doubt been the best decision of my life, but I won’t lie and say it has been all sunshine and flowers. The first few months were rough, and I had a chest tube in for at least four months as I kept having fluid around the lungs. Physical complications were a minimum for me as, aside from my lungs, my body was extremely healthy.
The biggest downer of the entire ordeal is the monetary impact. In order to get discharged from the hospital I had to get a home PFT machine and special wedge pillows, since I couldn’t lie flat. I ripped up all the carpets in my condo in exchange for wood floors and got rid of old couches because I wanted to ensure my living space was as healthy as possible. This, combined with the insane medical bill from the original hospital stay put a large financial burden on me.
While my parents have been great about helping me out when they can, the copays alone when I first got home were over $300, which was for a TON of meds but still, when you’re out of work that hurts. As I said, I lived by myself at the time so I had a mortgage, all the bills that come along with that and now all these new medical bills. I went back to work a few months later and eventually, as I got healthy, took a second job doing security at a local concert venue at night. Things haven’t been easy, but I am slowly making my way out of debt.
I wanted to know everything about the donor of my new lungs but was told you had to wait six months at least to write to the family, and even then they don’t always respond. It was right around that time that I got an e-mail from a woman at the transplant registry saying the mother of the girl whose lungs I had would like to reach out and was that okay. I agreed and we started exchanging letters. After a few months of back and forth, we actually met. I am far from an emotional person, but it meant a lot to meet the mother and sister of the person whose lungs now have given me a second chance at life. I enjoyed hearing all about her, and I think it gave them some closure for what had been a terrible thing to happen to them with the loss of a daughter and sister. It turned into something so amazing for me. We met only once, but we continue to e-mail back and forth every month or so.
Easy As 1, 2, 3
I was diagnosed with Cystic Fibrosis (CF) at 5-and-a-half years of age, which, as most of you familiar with this disease know, is pretty late in the game. I don’t remember ever not knowing that there was the possibility of a lung transplant down the road, if only because I can’t count the number of times I heard people asking my family, “Can’t she just get a lung transplant?” — as if it were a cure all. Oh, the things we hear, but that’s another story for another time. I did know one thing for sure, though: it wasn’t going to be me. I wasn’t going to need a lung transplant. I would beat this on my own, and if that time ever came, I didn’t want it. I was given this life, these lungs and I’d go out with them.
I also remember the first time I saw CF described as a “terminal” illness. I was doing a report for my seventh grade language arts class, and I was on this new thing called the Internet. Yes, I may be aging myself here: if you want me to help you out, pagers were the “in” thing, and children walking around with the Internet on their cell phones was something straight out of a sci-fi novel. Yes, we had movies! I’m a reader, okay? I put cystic fibrosis in the search engine and countless medical definitions popped up. I clicked on the first one, “cystic fibrosis, a terminal illness,” but I knew that was wrong. CF killed some people, but not everyone. It wasn’t terminal. So I clicked on the next “cystic fibrosis is a terminal illness that causes…” again and again. I clicked on definition after definition describing my disease as “terminal.” I think that’s the first time when having CF ever scared me. In the past, it had been hard and annoying, but not scary. I felt like those lines on the Internet created a shift in my thinking from this disease could kill me, to this disease will kill me. I never talked about that. I didn’t ask questions of my family. I knew they knew, and I started to understand in that moment the fear in their eyes. It was also, at that moment, that I learned to keep my fears about this disease to myself. Don’t do that. It’s not healthy. Find someone you can talk to about this.
I’m going to fast forward now to about age 24, to the hospitalization when my doctor finally suggested I go for lung transplant evaluation. Never in my life, up to that point, had I ever felt like such a failure. I had spent every single day leading up to that point fighting to stay on the other side of transplant. It had become a life goal and, at that moment, the goal had shattered. I had failed. And, to top it off, I still wasn’t certain that a transplant was something. I wanted.
Don’t get me wrong: I had no death wish; I just wasn’t sure that this was how I wanted to stay alive. I went to that first appointment, though, because of a silent promise I had made, in the dim lights of the NICU, to a 5-hour-old little girl as I held her for the first time and fed her a first bottle of sugar water. She certainly wasn’t my first sister and, as it turns out, she wouldn’t be my last, but she was my baby. I looked into that face, and the amount of love that swelled in my heart was something I hadn’t known was possible. I knew, in that moment, that I would jump off a bridge for her if it meant keeping her from pain. And in those first moments of holding her I swore, “I will see you graduate,” “I will see you get married,” “I will see you have children.” It was that promise that led me shaking into the first appointment at Stanford University Medical Center. I was accepted as a candidate but told that I was not quite ready for transplant and to come back in six months. Friends, let me tell you: I ran from that appointment. I felt like I had escaped a prison sentence! And every time I made it out after another evaluation it was the same.
On November 12, 2013, my grandpa’s birthday, I texted him from the ER something like “Happy Birthday, oh, by the way…” I thought that I was there just to get some Lasix. My kidneys had been weak since I was 17, when a local doctor had made a grievous error in the dosage of a strong IV medication called Vancomycin. I had been dealing with swelling, edema, ever since. This time was bad, though, and I could tell that it was affecting my breathing. I had no Lasix at home, so I grudgingly went to the ER. My absolute last resort in any situation is the ER. This time would be quick, though. Get Lasix. Get out. You know that line in the song, “Life is what happens to you while you’re busy making other plans.” Yeah, that. I had obvious edema, but I also had pneumonia in both lungs, and my blood work was awful. It was decided that I’d be flown to my hospital five hours away, in Sacramento. Once there, antibiotics were started. I was further assessed and also needed a blood transfusion. Things were happening fast and not in the direction I needed them to go. After a week in Sacramento, my doctor made the decision that I was not stable enough to stay there. I needed to be transferred to Stanford, where the lung transplant team could more closely monitor me.
After 22 years of care, our doctors, our nurses, our team, our hospital, became our home, our family. I was leaving everything behind to put my life in the hands of strangers. That scared me most of all. Once there, things continued to go steadily downhill. My goal to get out by Thanksgiving came and went. I had always been told that when people were ready for transplant, they were ready. Even then, in as poor condition as I was, I couldn’t foresee being ready. So you can imagine my surprise, one day in early December, when the staff came in with paperwork for me to sign to present my case to be listed. In between an almost constant stream of panic attacks I gasped, “I’m ready!”
The surprises, however, were not over. Sometime in that first two weeks at Stanford, a yeast infection was found in my blood stemming from my central line, my port-a-cath, and it had to be removed. The blood infection had affected my heart, but doctors were hopeful that it would come around. After weeks and weeks of constant monitoring, my bilateral lung transplant turned into the probability of a heart/lung transplant.
While that news was being absorbed, the doctors had to sit me down and explain that going to heart/lung changed my status on the list. For a lung transplant, we now have an amazing “point system” that allows people most in need a higher place on the list, and my status on just the lung transplant put me right near the top. But when listed for a heart/lung transplant, you are listed by the status of your heart. Mine was just not bad enough to warrant immediate transplant, so I was told that it would probably be six months to a year before one became available. I knew my lungs didn’t have that long. I saw in everyone’s faces that my lungs didn’t have that long. But see here is where my personality kicks in. I’m stubborn, like really stubborn. Telling me I can’t do something is almost guaranteeing that I will do everything in my power to prove you wrong. As time went on, it became obvious that I wasn’t leaving by Christmas, I wasn’t leaving by New Years, I wasn’t going home before transplant. And then one more bombshell: my kidneys, which, had been struggling since admission, didn’t appear to be getting much better. It was possible that they, too, would be added to my growing list of needed organs.
That aforementioned little sister turned 4 years old on January 21, 2014, and, as per her request, she traveled the six hours to spend it with me in the hospital. It was painful to get my hair washed and pulled up, and get me into a chair with a mask on so that maybe it wouldn’t scare her to put hers on as well. She wasn’t scared. She gowned, gloved and masked like a pro. I could write an entire article on that amazing child with just this one hospital stay, but again another story for another time. The night after her birthday it was my mom’s turn to stay the night in my room.
As I mentioned before, this hospital stay was the first time in my life when I had suffered from panic attacks that were almost constant, so I had her lying in my bed until I fell asleep. That night I woke up and knew that my breathing was worse than ever. I put my hand on my chest and could no longer feel it rise and fall with each breath. I woke my mom up to tell her that I needed to be intubated. This would not be the first intubation of the stay, but it would be the last before transplant. It’s also my last clear memory, minus a few flashes, before waking up sometime in early February being told that I had had my surgery.
After that night, my body started failing quickly, which, in the long run, ended up being a blessing because my heart failed as well, again putting me at the top of the list for organs. I was also officially listed for a kidney transplant. I have a brief flash of being told that I was “at the top of the list.” I also distinctly remember thinking that they were lying to me to keep my spirits up. I also have bursts of memory of walking the unit with my walker, intubated and the nurses clapping. I now know that they were clapping because most of them had never seen someone accomplish this feat and because the doctors had come in and told me that, if I was really serious about getting this transplant, that I needed to go for a walk. I’m told that they had to stop me from getting out of my bed right then and there. That sounds like me. During this time of having no memory, 30-40 of my closest friends and family made the six-hour drive to come see me one last time and to say goodbye. Because of this many of them were still there when the call came in that my organs had most probably been found!
My surgery officially started in the wee morning hours of January 28, 2014, and ended up being about 11 hours long. The surgeons went in with the plan of doing my heart/lung transplant first, giving my body a break to see how it was handling surgery, then continue on to the kidney transplant if all was well. My family was lining up ready at a moment’s notice to see if they were a match, just in case the kidney transplant was unable to happen. In the end, however, the doctors were able to go ahead with the surgery, and I received all three organs from the same donor — a woman whose name I may never know, but, to whom I will forever be thankful.
I wish I could say that from the moment I woke up life was like a fairytale, but it wasn’t. After a little less than a week post-transplant, I went in for a tracheotomy because my pneumonia-filled lungs and body were still much too weak. I was just not able to breathe on my own, and I was so angry. I was still on dialysis and would be until late June. I had what is known in the medical community as “ICU psychosis” and suffered from extreme anxiety, confusion and hallucinations. My muscles were so atrophied from being so long in bed that not only could I barely move my hand to my own face, but I was completely unable to move my legs. Because I had a trach, I was unable to eat, drink or speak. I had never felt so utterly hopeless in all of my life.
Then, another blow: my feeding tube had slipped out of place and formula and intestinal fluid had infected my entire abdominal cavity. Because of malnutrition, my tissues were falling apart, so I was rushed into emergency surgery where a seven-inch incision was made in my abdomen to remove all of the fluid and do an antibiotic rinse. That incision was left open and covered with a wound vac to heal from the inside out because closing it posed more risk of infection. About a week after that, my new temporary feeding tube had slipped as well, and they had to go through the wound in my abdomen once more. This time, they added retention sutures to keep my abdominal wall together as the wound once again started the healing process without being closed.
During this time, it seemed that for every step forward I made, I ended up taking 10 steps backwards. Slowly but surely, though, I made those steps forward. I started doing breathing trials and spending more and more time off the ventilator. After hours and hours of physical therapy, I was starting to gain some strength. As extra fluid came off from hours of dialysis, I could start bending my legs on my own in bed. I was able to start helping a little as I was moved from my bed to my chair, and I started walking a few tiny steps at a time, with help from my amazing physical therapist and a special walker.
On March 27th, almost two full months after surgery, I was able to move from the ICU down to a lower level of care. I was breathing on a trach collar with just oxygen and started walking with a normal walker. Eventually my trach was capped and, after several excruciating swallow studies, I was released to a liquid diet. Then I stepped up to pureed foods and on that day, in true CF fashion, I met my fellow transplant friend for an outside meal and snuck a Costco hot dog. Let me tell you, a hot dog never tasted so good — although, it would take several months for my taste buds to really normalize.
The moment they capped my trach, my voice came back almost as if it had never left, and I was able to make my first phone call in months. On the day that they removed my trach, I half-expected, irrationally, that I would suddenly not be able to breathe. I must have watched my oxygen saturation for a solid 15 minutes as it stayed at 100 percent on room air with just my own lungs and no crutch. When I was released from the hospital to a hotel, I still was not able to walk on my own or get up or down without help but, like everyone said, the progress I made being out of the hospital went exponentially faster. And as was always my wish, we pulled into my driveway, my HOME, shortly after midnight on June 24, 2014, my 27th birthday!
I’m quickly approaching my five-year lungiversary (heartlungkidneyversary??), and my life has been incredible. I am living like I never have before. I’m a foster parent. I’m a MOM! My little love came to me at 8 days old. She turned 3 this past September and, boy, is she a spitfire. Somehow this child has some of my DNA and I am getting a run for my money! I want to share my story because I remember going through my transplant evaluation, during which I was shown picture after picture of people traveling and living. The nurses told me how they get people out of bed and walking within 24 hours, and when things didn’t happen even a little like that for me, I thought for sure that my transplant had failed. I had been told that the first year after transplant would be one of the most difficult of my life, but I don’t think that anyone could have predicted just how difficult it would be. I want to share my story so that maybe someone who is currently going through a less-than-textbook transplant has hope that their life will get better. I want to share my story for people who are on the fence about transplant because for me not only has it been worth every single day that I fought, but I would/will do it again in a heartbeat for the life that I am able to live now. I want people to know that no matter how many setbacks you may have, you can still be a success story.
Every Beautiful Breath
Hi gorgeous people!!
There is really so much to say to put this transplant journey into words that are relatable. It started really five years ago when my CF team at University of Nebraska told me I needed to get listed and we needed to pick where. The choices they gave or recommend were California, (too far from family), Colorado or Minnesota. Aside from my husband’s family being only 45 minutes away from the University of Minnesota hospital, all our research and my team really pushed us to go to Minnesota. I was listed in 2012. We were traveling back and forth from Nebraska to Minnesota for appointments, while my husband looked for a job up north so we could just relocate and wait for that call.
I know many centers are so different from each other. I was listed at 42 percent lung function. At Minnesota, they match you as well and precisely as possible; they want people listed when they are healthy enough to recover, but not so unhealthy they don’t have time to wait for that perfect match. That call took two and a half years to get but, when it came, it was meant to be.
We moved to Duluth, which was still two to three hours away from the hospital but closer than Omaha. This is the part of the story where I know without a doubt a higher power (aka the universe) was in charge. All in 24 hours my husband found a job closer to the hospital, we found a place to live 30 minutes away from the hospital and signed the lease.
That evening at 8:45 pm, I got the call. We got to the hospital after dropping my beautiful ladies off at their grandparents’ house. We ended up sleeping overnight in the hospital while they tested and got everything prepped for surgery. The next day, January 16th, I received my new lungs!
The biggest thing I want people to understand is it’s not a cure, and sometimes you are trading one issue for a whole set of different issues. The point of the matter is, I still have CF. I’ve had two surgeries post-transplant and am about to schedule another one, all side effects of medications or CF related to some degree. That will never change. BUT, I ran my first 5k, I can hike in the mountains and the biggest blessings are some that could seem so trivial to some. Playing in the snow with my kids, chasing them and giggling until I cry, volunteering and giving back to my community here in Colorado — those are the biggest, most precious benefits.
Life is different because I am able to participate way more than I used to. Going on any adventures I want outside is so amazing. I truly feel like a kid somedays doing things I never was able to do. And yet I am still not able to go out into the workplace because of my suppressed immune system and millions of doctors’ appointments.
I did find my passion in Plexus supplements (aka gut health) that I am able to do and support people from my phone while helping my community. Even though it’s not the most conventional, it’s been the best thing I have done for myself. These people in my life have supported me for 35 years and now is my time to give back and support everyone I can. I care so much and want to help as much as I can. Give good back any way I can.
My medical regimen is really different in the fact that I don’t spend four hours a day doing treatments, but now my pills have doubled. I’m okay with that! To be honest, my regimen is a lot different, but I also found the awesome benefits of healthier eating and supplements. I cut out as much sugar as I can: the soda habit was out of control prior to transplant. After watching my Pops fight cancer and going through what I did, I was so ready to make a healthier life for myself and family.
My support system is everything and I would not be here if it weren’t for them all: my family and friends, together with the medical professionals I haven’t thanked enough — my CF and transplant teams since birth. All of my CF teams from Omaha to Minneapolis have been family and have saved my life more than once. I picked Minnesota for their success rates, but they have been truly phenomenal. Minnesota boasts one of the only clinics that integrates CF and transplant teams into one unit. I appreciate the fact they can work with both my CF and transplant issues and tweak things to help with both — pretty impressive and so reassuring! They understand that you have to be vigilant with routine and meds but also really understand the importance of quality of life. That’s huge to me. If you don’t try to live a somewhat “normal,” life you are not going to make it. Balance! I also have to give the biggest shout out to my mom, always by my side from day one, always held me up and always will. She’s a saint!
Emotionally, pre-transplant was a struggle. I always questioned my decision to have the transplant. I questioned whether I was toying with my fate, but my kids and family always brought me back to earth and made me realize they need a mom. I struggled the worst with anxiety and insomnia — mostly due to meds, but also the lack of breath always caused attacks. My husband is the one to thank for getting me through it all. He always could calm me down and really stepped up to the plate no matter what.
I never wanted to go for prescriptions to deal with these issues, I don’t like adding chemicals that add side effects that add more chemicals, so I did yoga and meditation to get through pre-transplant. I really believe in natural and holistic remedies but also understand I need western medicine to stay alive, so I have always tried to find that healthy balance.
Twenty-three months post-transplant I still have these issues but they are very, very few and far between. I do have a bit of PTSD and have flashbacks from ICU and recovery. How I deal now is supplementation; pro and probiotics have truly made my life so much better after transplant. And my team, of course, fully supports and pushes me to stick with it.
I did write a note to my donor’s family. I want them to know that the loved one they lost did an amazing thing, and I am not taking this gift for granted. As odd as this is, I have to share: I was still in ICU recovering, on pain meds, and I was asleep when I saw my Pops, who had passed away four years earlier from cancer, holding a little girl. Whether it was medicine-induced hallucinations or something else…either way, it stuck with me and I will never forget it. Guardian angels? I do hope to hear from the donor’s family someday, because my gratitude is so huge. I want to make sure I am doing everything to not take this for granted and live out the rest of my time not regretting, loving and living this to the fullest.
There are so many words in my head, and I am not the most captivating writer, but I have learned that life is so precious — the light and the dark. I don’t think I would appreciate the light if I didn’t have to fight and struggle through the dark. I am grateful for every single thing I have gone through. It has made me completely empathetic, understanding and STRONG. There is nothing I can’t get through, no matter the outcome, I can make it through anything, and that’s everything!
ten years after
My name is Renée. I’m 43 and was diagnosed with CF in 1982 at age 7. My CF always was mild. I’m the only one in my family with CF.
In the seventh grade, when I was 12, I started IVs. I worked until I was 25, when my illness started getting worse. To make things easier for me, I had surgery to have a Mediport® placed so I could do IVs anywhere.
Then I qualified for SSDI so I didn’t have to work and I could take care of myself. I continued to get worse.
In 2008, I arranged a meeting with my doctor and immediate family to discuss transplant. We decided as a team to go forward with the transplant process. I located a transplant center and the doctors moved very quickly to get me set up with my team at the Cleveland Clinic. I began the necessary testing and it was finalized two months after the first meeting with the Clinic.
My PFTs were at 25 percent and going down fast. As of August 2008, I was approved and put on the waiting list. I had been on the list two months and two days when I was called in October, but I’m fortunate that it was a dry run. The lungs they harvested had lesions on the backside that initially they could not see. So it was time to go back home and wait.
One week later to the day, on October 27, 2008, I got the call and it was a go. I was so scared that if it didn’t take, I would die on the operating table. I woke up, they took the tube out and no cough. I could breathe easily. It was AMAZING! It took! Now I have a longer life. No more insane coughing or mucus or IV antibiotics. The only thing about transplant is you trade one set of issues for another — such as rejection, medications for life, risk of colds or infections, etc.
Unfortunately, I experienced rejection and was in ICU for two and a half weeks in my local hospital and then a month in the Cleveland Clinic ICU. Then I was moved to a regular room for three months before I could leave. During those hospitalizations, I dealt with feeding tubes, breathing tubes, IVs, pain meds and being paralyzed. Since then, things have been up and down with doctor’s appointments, etc.
I had a kidney transplant in 2017 due to rejection medications, kidney failure during rejection and kidney stone surgery. My kidneys were very delicate. But since then, after the lung and kidney transplants, I feel great.
I started back to work this year and have so much energy now. Life is busy with work, friends and family whom you would definitely call dear after these kinds of ordeals. There still are follow-up appointments with the transplant center every six months and a CF follow up once a year, more if necessary. One thing to remember is your lungs don’t have CF any longer, but the rest of your body still does. So there is still ongoing care for those issues.
Transplant was as I expected since I had done research beforehand.
I chose not to have kids with the fear that it might negatively impact my health.
I did write to my donor family eight years ago and have received no response per Ohio Life Banc.
Lastly, transplant is a great option as a choice for a more fulfilling life: to do the greatest things you can think of doing for yourself. I’m very glad now that I had the chance and the opportunity to receive a second chance at life. Without my donor, I would not be here.
I wrote this poem a couple years ago when I was in the hospital because of a lung infection. It was a gift of clarity that poured out and helped me define so many feelings I had around gratitude and support.
Now, as I write this, it will be eight months since I’ve been listed for a bilateral lung and liver transplant. Eight months of being in this in-between space, away from home, holding the tension between “sick but not too sick.”
This poem feels more poignant than ever as the emotional work involved in this process is a full-time job in and of itself. I am acutely aware of our cultural aversion to waiting when, after telling someone why I’m living in Seattle away from my home in Alaska, the first thing they exclaim in shock is, “You don’t know when you will get a transplant…?” Nope, sorry.
Because waiting is so uncomfortable, whenever a friend is able to sit with me in this space and really allow herself or himself to accept all the unknowns and not try to fix anything, it means the world. When friends take the time to match their pace with mine and be still for a few hours and just enjoy the day with me, it makes me feel like I can breathe again. And that gift of presence gives me the resilience to keep waiting.
The knot rises in my chest
My heart plummets
Like an elevator in free fall
I try to move away from the
At all of these feelings —
A friend comes to visit
intending to give comfort.
They see my emotions lathered
up and agitated,
My body a knot of tension.
Unsure what to do
They sit and take my hand.
“Remember,” They begin,
“God is in control.”
“Don’t worry,” they sigh,
“Everything will work out.”
After a while they finally say,
“It could be so much worse,
You should be grateful.”
My shoulders tighten and I wince.
My mind rebels at the implied
From pain to joy.
I know it is right to give thanks and praise,
I have been in the presence of
She has come to me in the
unlooked for moments,
Like the sun breaking through
Warm and embracing.
Like a crashing wave
Spraying me whole again.
Her presence feels
Like a passionate kiss
Rocking me to my core
Leaving me speechless,
And knowing something I almost
She is a visitation,
A sacred moment shared only
By her and the one she seeks.
An angel of sorts.
The temptation to summon her,
To reduce her presence to mere
trick of the imagination
Incanting, “Thank you God I
Is to diminish the sacredness
She travels through sunsets
Not through the public transit
To compare your burden to others
Does not ground you to the path
you are called to.
To feel grateful is a fullness,
A lightness of being
Connecting you to the Source
And to This moment.
Another friend comes to visit.
They sit and they listen,
Saying, “I’m so sorry.”
Earnestly they tell me,
“It’s okay to feel these things.”
After a while they gather their
breath and invite me to be here,
Suddenly there is room
for me to ask, “What is here?”
As we sit together they reach out, take my hand and whisper,
“No matter what, you are not
I pull this reassurance into my
My body relaxes and my mind
Like a horse released from a frantic
I take in this moment and realize,
I am seen.
And I am so grateful.
Family matters: Talking About Illness With Children
How do you talk to kids about illness? CF? Mortality? This is a question I have been wrestling with this fall as my health has taken a turn. While I don’t have kids, I have two nieces, ages 3 and 5, a nephew, age 2-and-a-half, and another nephew due in May. I struggle to explain to them why I cannot carry them anymore or run around with them in the park. As hard as this is for me as an aunt, I’m sure parents feel this much more acutely, being around kids 24/7.
Aside from my nieces and nephews, I have a plethora of kids in my life. Some of my best friends have children who also call me “aunt Molly,” and I have nine little cousins. Being around so many children necessitates talking to children about CF and illness and teaching everyone good hand-washing techniques. Unfortunately, it means I miss birthday parties and have to have awkward conversations about sick kids not attending family holidays, because I cannot risk getting sick right now.
My nieces have seen me on IVs multiple times, and call my port my “owie.” We have told them “Aunt Molly’s lungs don’t work very well” when they want me to carry them, but they haven’t really processed it yet. They know that the first rule of cooking is to wash your hands and to not lick your fingers until the end (well, okay, the 3-year-old isn’t great at that one, but we’re working on it). They also know to be cautious around me on IVs and the 5-year-old will even assist me up by handing me flushes and alcohol wipes. They are especially snuggly when I’m hooked up and cuddle on the couch and ask to watch “that baking show you like” (aka The Great British Baking Show). But I have never talked to them about transplant and what that might mean for the future.
My health has started to take a decline in the last few months, so after yet another IV course, my doctor sat me down and told me it was time to start considering a lung transplant. She explained that I am not necessarily ready for one yet, but that my numbers have been ping-ponging so much, we want to get the conversation started in case I cannot revive them after a future IV course. I am grateful my doctor is thinking ahead for my health and want to take a moment to share a bit about that first transplant conversation, since I had no idea what to expect.
The first appointment with the transplant team is a big “getting to know you” session. The doctors and nurses are evaluating whether they think you need a transplant, and you are deciding whether you want them to be your care team for the rest of your life. My husband and mother accompanied me to the appointment, and the team made sure we all understood the implications of life post-transplant. New meds, new doctors, pulmonary rehab and, if all goes well, a new chance at life, work and starting a family. Because these doctors will follow you for the rest of your life, with your ups and downs and life decisions, it is critical that you have a good rapport with your team.
While the team agreed I am not quite ready for transplant, my husband and I decided on some major life decisions in the process of considering it. We agreed that right now is not the time to have kids — if even a few hours with our nieces wipes me out. But that doesn’t rule out the possibility of having our own kids post-transplant. Many people adopt, use surrogates, and some even become pregnant! It is tricky to manage with transplant meds, but definitely doable. We have always said that if we don’t have kids, we want to live within a ten-minute drive of my sister and nephew so that he can come to our house after school and think of us as a second set of parental figures. So we will be moving out West in the spring.
Support is one of the biggest factors a transplant team considers in evaluation. They want to make sure both that you have a strong support network and that your support network has its own support network. We have a robust support system in California, from parents and siblings, cousins, aunts and uncles, to friends and their kids. My best friend’s son is the same age as my nephew, and we are already planning outings to the aquarium and zoo for the four of us. The wealth of friends and family in California will make up for our diminishing support in NYC. As is typical with New York, our 30-something friends are having children and fleeing to the suburbs.
With the initial transplant meet and greet done, I am still left with the here and now, and the tensions of letting the kids in my life see me sicker. This fall, my 5-year-old niece visited me in the hospital for the first time. I was recovering from a pneumothorax and pleurodesis, so was hooked up to a chest tube with suction, 02 (which she has never seen me on), and lots of pain meds. I was nervous to have her see me connected to so many tubes. The nurses made her “gown up,” with gloves and a mask, just in case she was a vector for kid germs. When she entered my room, my normally outspoken and rambunctious niece was extremely quiet. She pulled up a chair next to my bed and just sat there with her gloved hands on top of mine. My heart nearly broke. We kept calling her “doctor” and telling her how much her love was helping me heal. I hope seeing me in the hospital did not scare her too much. I believe that nurturing empathy in children and exposing them to illness and loss, so they learn how to cope, is an important part of being human.
Unlike my nieces, my nephew has never seen me on IVs or in the hospital. This fall, however, when my Dad was an inpatient, my nephew was able to visit him. I am secretly glad I was not the first person he visited in the hospital. My dad’s hospital stay was short, and my nephew’s new favorite game is playing doctor. He tells my sister, “I’m the doctor and you’re Papa Rick and I make you feel better.” I hope he carries that positive outlook of hospital stays through to visiting me, and that, by the time he is old enough to understand what is going on, I will have figured out a way to explain CF.
In the Spotlight: With Micah Baker
We were asked by a previous CF Roundtable writer, Janie Davies, if we would talk to one of her acquaintances who had gone through treatment for Mycobacterium avium complex (MAC.) We thought this would be a good example to highlight: someone who successfully was treated for MAC and worked during his treatment. Probably many factors keep him going strong; some of them are his exercise routine, his belief in religion, his loving wife and a very motivating motto. Please read on to get more details on an inspiring and creative man who handles his CF with aplomb.
Meet our newest star, Micah Baker. Spotlight, please!
Hometown: Dallas, TX
Married and how long?
We will celebrate our sixth wedding anniversary in December 2018. Crazy!
How did you and your wife meet?
Laura and I met at the hospice where we both worked. She was a social work intern and I was a program assistant. We became friends She finished her internship. We ran into each other about two years later at a restaurant, and that was the end of my single life.
Did she know you had CF, or did you tell her?
Well, only a couple of people knew I had CF at my work place and apparently the social worker she was interning for told her about me having it. I don’t think Laura was very shocked by it. She is a very level-headed and calm person.
Do you want children? Did you discuss before marriage?
We kind of live with the thinking of, if children happen, they happen. I did let Laura know fathering children wouldn’t be easy for me and she didn’t mind a bit. For a while we were open to doing IVF, but we had a change of heart and decided, rather than putting our money into that, we would prefer to adopt. It’s not anything we are actively pursuing at the moment. We love our time together just being us.
How is your health regarding CF?
I would say my health is pretty great. My PFTs are currently at 70 percent (last appointment in August) so I hope they are higher at my appointment at the end of this month (November). I am 5 feet 7 inches, 145 pounds and I am keeping on weight and running a lot to get my lungs clear. I rarely am sick and always at work. I can’t remember the last time I called in sick. I also would like to add that I gave up fast food last spring and started eating a lot healthier. Laura became vegan a few months ago, and so I eat what she makes for dinner and eat whatever I want for lunch. I eat meat (but only beef) and seafood. I pretty much stay away from anything junky and sugary.
Where do you work and what do you do?
I work at Firewheel Christian Academy, and the list of what I do goes on and on. I teach a yearbook class, lead worship for chapel, manage the lunch program, handle all social media and troubleshoot technology issues. I do enjoy it! I used to bounce around a lot subbing and assisting teachers. Now I work more in the office and have a schedule, so that’s a better routine for me.
Tell us about the class you teach and why.
Yearbook is a twice-a-week class for seventh and eighth graders. It was just kind of handed to me as an option and seemed like a creative outlet. So I said, “Sure!” My favorite thing I do, though, is leading worship for middle school chapel. I love music and hearing the students sing along is an amazing experience.
Tell us about your experience having MAC?
I had been culturing Mycobacterium abscessus off and on since age 10, but it had never bothered me. In 2015, however, it was showing up more on my cultures and I started having symptoms. I started coughing up blood and having terrible night sweats. They were so bad they woke up Laura. My PFTs were down, but only slightly, and I also was losing weight and not really gaining. My doctor gave me the option of doing a fine needle aspiration biopsy of one of the nodules found on the CT to help me make a more informed decision about starting treatment. I agreed to go through with it and it didn’t show anything. Dr. Jain doesn’t typically just jump to things. She always looks at every detail, and since she had a really positive feeling that it was the mycobacterium, we decided to treat it.
I took a combination of three drugs daily: Zyvox, amikacin and clarithromycin. The only issue I had was slight high-frequency hearing loss, and I still suffer with a bit of tinnitus (the perception of noise or ringing in the ears). I consider myself very blessed that these were the only side effects I suffered. The hearing loss bothers me only in places with heavy background noise.
How long did you take the combo medications and what were the side effects?
I took them for a full year. However, I had to switch the IV amikacin for the inhaled because it was messing with my hearing. I had moments where my ears felt muffled and I was experiencing ringing as well. As for liver toxicity, I did not experience any. The doctors were amazing at watching my numbers closely and adjusting my dosages. They did adjust the dosage based on the level of the drugs in my system. I want to add how thankful and grateful I am to Dr. Jain and the CF team at UT Southwestern in Dallas.
Who supported you through it?
My wife and my family (both mine and hers) were great support. Laura took care of me really well. It was a pretty hard year, but it made our bond so much stronger. Christ also really sustained and held me during this time. I wouldn’t have made it through without Him.
Were you able to keep working?
Before starting treatment, the first thing I told my boss, when I told him the news about my having MAC, was “I HAVE to work, absolutely HAVE to.” The work distracted me and the students elevated my spirit so much. They had no clue of me about going through anything, but the silliness and love day-to-day really allowed me to put aside my suffering and put them first.
Can you explain what MAC is?
MAC is a group of bacteria related to tuberculosis, but it is not tuberculosis, more like a cousin. It’s found in water and soil. I believe most people carry it in fact, but it doesn’t present any kind of issues. MAC stands for Mycobacterium avium complex, however Mycobacterium can have a number of strains—almost like the flu. Mycobacterium abscessus is one of the harder bacteria to fight and is usually not eradicated, but it can be knocked back to being dormant.
How are you now?
I feel great now. After the treatment, I did continue to culture Mycobacterium abscessus but, at this time, it is dormant and my doctor doesn’t expect me to have any issues for a while. So, it comes up on my culture but not on the smear (which would indicate a lot of it is in the lungs and could cause symptoms).
Absolutely! I mainly run only on the treadmill. I used to do only a mile, but now I am trying to do 30 minutes of running without stopping. So far, I have made 25 minutes, stopping after every five minutes for 20-second water breaks. The longest I have gone without stopping is 17 minutes.
Religious or spiritual?
Yes, I am a Christian. I truly believe Christ has remained ever so present in my battle with CF.
Have you ever considered a transplant if you needed one?
This is a tricky one for me. I have always been of the mind that, when it’s my time, I am ready to leave this earth. A transplant honestly always seemed like a chance to just delay death and I did not want to experience near death more than once. However, having a wife and a greater knowledge of medical advances, I think I would really consider it as an option now.
Do you know your genetic mutations, and are new corrector drugs available for you?
I do know. I have Delta F508 and G542X. I don’t believe they are available for my mutations yet but, while I am waiting, I will continue to stay as active and healthy as possible.
What do you do for fun?
Well, I like trying different restaurants, discovering new fashions and designers, customizing hats, playing guitar, listening to music and I LOVE traveling. I really enjoy creating things like accessories when I can. I enjoy going to antique stores and finding one-of-a-kind objects. It’s always fun to search for old things and bring them back to life. I recently have gotten into buying Stetson hats (fedora and cowboy style) and customizing them by lighting them on fire for a vintage patina look, sticking random objects in them or creating different bands and ribbons to wrap around them. I also enjoy listening to and playing music.
Do you have pets and why?
Yes, we have a black cat named Hugo! We have three strays that we have been feeding since we’ve lived at our place. We always have been fond of cats. One day a neighbor said, “I saw one of your black cats…the little one!” I was like, “Um, we don’t have a little one.” Right then, this little black kitten came from around the corner of the house and that was it. We had Hugo inside after we got her shots and had her fixed. We just couldn’t put her back outside. She really makes Laura and me happy and we enjoy her presence. Laura started working from home and was going through some things when Hugo showed up, so we see her as a sign of God’s love and presence.
What helps you cope?
First, God. I couldn’t live thinking everything is just by chance and random. I also have been through so much with CF, that I don’t think it is humanly possible to go through alone. I am not that strong a person, nor do I believe my spirit would be so at peace and calm without having Christ within me. Second, Laura. She is such a source of strength and motivation for me. When I am weak, she is strong and vice versa. We make the most incredible team. She has such a loving soul about her that I see whatever she does or says coming only out of love. She always encourages me but, at the same time, respects my feelings and always comes from a place of humble acknowledgment that she can’t completely understand what it means to live with CF.
What is a perfect day for you?
Honestly, the most perfect day for me is just being with Laura without interruptions. This past Saturday, we had no plans and it was perfect. We woke up, had our espresso; she did her morning reading; I did my treatments, we went for lunch and some shopping; went for a quick run at the gym; came home and had a nice quiet evening. She loves to read, and I love sitting with her and Hugo. It is the happiest place for me.
Funny story about CF?
My wife and I, along with her parents, went to London a couple of years ago (my dad is Scottish so I have family over there). We got to the flat where we were staying, and I decided to do my treatment right away. I plugged my compressor into the converter I brought and, the second I turned it on, it blew. After panicking, I called hospitals and my clinic and then started Googling. Every option was either not present or would have taken way too long. I had almost given up when I came upon a website called Argos. They had a compressor for only $80. So I started the ordering process and noticed I could pick it up in a store that had it in stock. Fortunately, the store was about a mile and half from where we were staying. So, by the next morning, I had a brand new, UK-compatible compressor. I took it with me the next time my wife and I went to England, and I still have it as a souvenir!
A motto you live by?
My motto is simply “Fight” or “Fight Micah Fight.” This is something my mother used to always tell me when I was sick or hospitalized. I would often fall asleep while she did CPT when I was younger and she would whisper it to me. It has always stuck with me and recently I had her write the word “Fight,” and I got it tattooed on my left-inside wrist in her handwriting.
Transplant Talk: I Get By With A Little Help From My Friends
As the Beatles’ song goes, I wish I could get high with a little help from my friends, too! But we all know that is not an option. As a transplant recipient, I am not allowed extracurricular drugs, only ones by prescription, sadly. But my friends do help me get by in the way that they offer their opinions and suggestions on what works for them. And I listen. Please note, I always run whatever I learn from others and want to try myself by my transplant pulmonologist. If she okays it, I will try it and report back.
Not sure if it is because I vent to my friends about my health woes, they vent back and we then discuss the many issues we have to deal with. But I have found that we all, at times, have similar problems and get frustrated that we cannot always solve them easily.
For instance, I was continually getting sinus infections with horrible headaches and pain in my cheeks and gums. It was Kathy Russell, our Managing Editor here at CF Roundtable, who suggested I try a glacial acetic acid (GAA) solution in my nasal lavage. I consulted my ENT at the time and he concurred it was worth a try. Kathy and her ENT had discovered that GAA, which is similar to vinegar, helps eradicate pseudomonas. I had tried regular white wine vinegar with disastrous effect. I got sores in my nose and it burned like heck. But the GAA in solution didn’t burn. I started with small doses and worked my way up to an effective amount in two cups of saline. I am grateful to Kathy and her ENT. I have experimented with using and without using it. I sometimes I forgot to pack it when I visited my mom, resulting in a difference I could feel and an outcome that was not beneficial.
Editor’s note: It is imperative that no one tries this without consulting with a qualified physician. My ENT doc is willing to talk with other ENT docs about the proper dosages and methods of use of GAA. Also, tap water and table salt cannot be substituted for sterile saline.
Jerry Cahill’s suggestion is another example of how I have benefitted from seasoned advice. Jerry, as you may know, is a relentless exerciser and has pushed himself physically for success both pre- and post-transplant. I asked him if he ever got dehydrated or lost electrolytes. He said he used Salt Stick pills and then eventually Nuun tabs, which are low in glucose. I buy both of these items through Amazon. Prior to Jerry’s recommendations, I was hospitalized for hyponatremia post-transplant a few times. And then my friend Carri, whom I knew from college, told me to drink coconut water. This was before the craze that started a few years ago. It did help, but it did contain more carbohydrates than I liked, and I had to use insulin to correct for it. It was effective because coconut water does contain a lot of potassium, an element that diminished in my body from sweating so much. Potassium, it turns out, can help a body retain sodium, calcium and magnesium. And I was already taking calcium and magnesium pills three times a day.
I never had electrolyte or low-salt issues prior to transplant. My levels may have been on the low side, but not enough to create the sensation of hyponatremia — anxiety, frequent urination and, in extreme cases, checking out. It probably was the immune suppressants plus the tertiary medications to keep my lungs from rejecting that caused these symptoms.
As an avid biker and tennis player, I became frustrated, when trying to keep my lungs active and clear by exercising. I started to fear the low sodium and resultant hospitalization, which happened mainly in the summer, when sweating outside was a peak issue. I would come home after two hours of doubles tennis with salt streaks covering my legs and arms.
My doctors suggested taking sodium chloride pills that were 1,000 mg twice-a-day. They were hard to find for a period of time, so I was frustrated. I used a combination of those items listed above until I could buy the salt pills. Recently, I stopped taking the sodium chloride because the Nuun tabs worked so well in conjunction with the Salt Stick pills. Also, I noticed some hypertension when checking my blood pressure at home, so my doctor told me to discontinue the additional sodium tabs.
Originally, when I was hospitalized several times for low sodium and loss of electrolytes, the doctors covering my care wanted to put me on Florinef, a medication that corrects for adrenals not working properly and the low-sodium resulting from that. A nurse who was caring for me urged me to read up on it and gave me a printout in my hospital room. It seemed that this medication had so many side effects, I was terrified to start it. This irritated my attending doctors because I was going against their orders. I was looking for a more natural, non-body-altering way to correct the loss of salt and electrolytes. It was why I was so open to trying coconut water and looking for other remedies.
For the last few years after transplant, because my sinuses were so infected, mucus was dripping down into my transplanted lungs. I was coughing up, or trying to cough up, thick colorful mucus. I had three bronchoscopies in six months and my lungs kept filling with this viscous phlegm. This was new. I felt I needed chest PT and a good percussor to help me cough the phlegm out. My ancient machine had recently died. I asked Jerry, whom I now call my guru, what he recommended. He suggested an Electro Flo 5000 percussor. I looked it up online and called to get more information from the company. My doctor felt it was a good idea and sent in a prescription. I have a feeling not many patients asked her if they could do MORE therapies! This device was small and had a strap that easily accessed one’s back and sides. It fits into an over-the-shoulder bag and is easy to travel with. And it packs a wallop. I currently use it because, even though my mucus output is barely there, it does a good job of clearing what is lingering.
There are probably other things I have shared and learned with other friends who have CF and a transplant. I find we can be good resources for each other, if not just good support and a sympathetic ear. There are many overlapping experiences we all share. I understand when people tell me about specific things they are going through, even if I have not experienced them personally. I learn from others’ experiences, be they positive or negative. Conversely, when approached by people who have similar issues to mine, I am happy to pass along my relevant experiences. For me, it is the benefit of being part of the CF community — being part of something bigger than myself, giving and receiving help. With this disease, we are all just trying to get by (with a little help).