All  too often, when I hear the term “growing older,” usually it is  accompanied by a litany of complaints, whether physical (wrinkles!),  emotional (so tired!), or regrets (I wish I had…). Every once in a  while, and more often in the CF community, I hear how amazing and  beautiful it is that we’re aging, a sentiment to which I wholeheartedly  subscribe. Growing older with CF, every decade conquered, is a victory. I  overcame the poor odds of survival that accompanied the initial  diagnosis and turned 60 (yes, 60!!) last June. Yet I feel that I’m a  twenty-something at heart and hope to have many great years ahead of me.

To me,  growing older means being more in tune with yourself—knowing what you  can and can’t handle. This is why I continually adapt and adjust what  I’m doing in order to pursue new interests and passions. It’s not always  successful but hey, life is about learning, right?

​

In 2006,  I had to leave my wonderful practice as an allergist, tending to  others’ health, and go on disability due to rapidly declining lung  function. During the next 16 years, I had to tend full time to someone  else’s health—my own. Concurrently, I reinvented myself and my life and  tried to figure out what I could accomplish while trying to heal and  maintain my health. It didn’t come easily. Looking back, I view my life  so far as an artful jigsaw puzzle, each piece an experience while not  knowing the full details of the final picture. Some experiences lock in  place better than others. I’ve learned to fine tune my experiences to  create my own masterpiece. I continue to experiment with new pieces,  throwing out those that don’t fit and keeping the best ones.

​

Spending  so much time on my health every day limited what I could do. However, I  did find I was able to try out new interests and accomplish a lot  through volunteering. Every year, I changed up my volunteering to test  the waters in different roles. For example, I started a nonprofit  patient advocacy organization to use my medical degree to help others  receive the best medical care possible. I volunteered and joined the  board of nonprofits like USACFA and CF Research Institute (CFRI). I  became my neighborhood’s Block Captain and dove into genealogy, loving  to research and investigate ancestral roots, not just for me, but for  others, too. I enrolled in research studies to advance CF science that  may benefit all those with CF. Everything is on the table and I’m open  to considering all kinds of new professions and new avenues.

​

The  beauty of volunteering is that it can be done from home, very often  during nebulized breathing treatments. It allows the flexibility to take  care of yourself and doesn’t interfere with healthcare appointments. If  it ever feels overwhelming, then cutting back is in order. The biggest  benefit of volunteering is meeting and getting to know like-minded  individuals throughout the U.S.—people who have enriched my life  immeasurably.

​

I feel  very fortunate that my health has improved on a grand scale in the past  few years with the advent of better CFTR modulators. This led to my  kicking around the idea of returning to practice as an allergist in  direct patient care. Basically, reinventing a new permutation of what  felt like a previous life in academia. I wrote down what this new  permutation would entail. I knew I couldn’t return full time, as in the  past. It would have to be part time, a few days at most per week and I’d  need my mornings free. Like that would ever happen! My mornings are the  most important part of my day—necessary for treatments, exercise, and,  if I can, even a power nap. As God is my witness, a position  materialized, as if sending the universe an outline of the best job  situation for me led to a metaphysical all-points-bulletin to local  allergists. An allergy practice responded to the universe’s request with  my near-exact specifications. What a wonder! One thing led to another  and, lo and behold, I’ve signed on the dotted line and will start soon. I  couldn’t be more excited to add this new beautiful jigsaw piece. It’s  taken oodles of preparation to catch up and get back up to snuff. It’s  been worth it.

​

While  I’ll have to put much of my volunteering aside for now, I hope to feel  settled in my new position before eventually adding it back since it’s  such an emotional salve and extremely rewarding. I believe growing older  with CF makes us value each and every unique experience, particularly  the challenging times such as being on disability, a time when I learned  so much about myself. I’m ready to tackle new challenges, regardless of  physical age, on my journey to complete my jigsaw masterpiece.

Jeanie  is 60 years old and has CF. She is a doctor, lives in Los Angeles with  her husband and has three grown children, a son-in-law, soon-to-be  daughter-in-law, and a granddaughter, all who light up her life. Not too  surprising, she did her share of jigsaw puzzles during the COVID-19  pandemic. She is also a director of USACFA and CFRI. Her contact  information is on page 2.

I. Introduction.

In the  previous issues of CF Roundtable, the first and second parts of this  three-part series appeared. The first part addressed §1619(b) of the  Social Security Act, which applies only to individuals receiving  Supplemental Security Income benefits (SSI). The second part addressed  Trial Work Periods (TWP) for Social Security Disability Insurance (SSDI)  beneficiaries.

​

This  third part addresses extended periods of eligibility and extended  periods of Medicare coverage for beneficiaries after a TWP. The rules  that apply to SSDI and Medicare coverage do not apply to SSI and  Medicaid benefits. The rules regarding continuation of SSI benefits are  different from the rules governing continuation of SSDI benefits. If you  are a SSI beneficiary, see Part I of this series on continued Medicaid  eligibility under §1619(b) in the Winter 2022 CF Roundtable issue. Part  II of this series relates to SSDI and the TWP and appeared in the Spring  2022 CF Roundtable issue.

​

Three  separate provisions in the Social Security Act allow a SSDI beneficiary  to maintain Medicare benefits while he or she remains disabled and has  work income. These three provisions are: (1) Trial Work Period; (2)  Extended Period of Eligibility; and (3) Continued Extended Medicare  Coverage. This article addresses the Extended Period of Eligibility and  Continued Extended Medicare Coverage, which only apply to SSDI and  Medicare recipients and do not apply to SSI beneficiaries. Some people  with CF think they are receiving SSI benefits but are actually receiving  SSDI benefits, and some believe they are receiving SSDI benefits but  are actually receiving SSI benefits.

​

Please  make sure you know the benefit you are receiving as it makes a big  difference in terms of non-medical eligibility criteria and the ability  to work and keep benefits or keep Medicare or Medicaid benefits.  However, medical eligibility criteria is the same for SSI and SSDI.

​

II. Extended Period Of Eligibility After A Trial Work Period.

A. Social Security Administration (SSA) Determination.

At the  end of the nine-month TWP, some SSDI beneficiaries decide their TWP is a  failed work attempt. They discontinue work activity and want to  continue receiving the monthly SSDI cash benefit and Medicare coverage.  However, the beneficiary does not determine whether the TWP is  successful or unsuccessful. SSA makes the determination. A person may  continue to be eligible for SSDI only if SSA determines the person  continues to be disabled under its rules.

​

B. Suspension Of Payments Versus Termination of Benefits.

At the  end of a TWP, SSA may stop benefits. However, not all cessations of  benefit payments are the same. A suspension of payments is different  from a termination of benefits, even though both are a cessation of  benefit payments.

​

Termination  of benefits often occurs after a TWP when SSA concludes that the  beneficiary has made a successful return to work and is no longer  disabled. When SSA determines that a beneficiary is no longer disabled,  SSA will terminate benefits because the beneficiary is no longer  eligible to receive further benefits based on that claim. If a person  wants to resume benefits after a termination, that person must submit a  new claim by filing a new application for benefits and show that he or  she meets all medical and non-medical criteria. However, the person may  not be able to show insured status if the claimant’s work credits have  expired while receiving benefits.

​

Suspension  of benefits is different. If SSA still regards the beneficiary as  disabled under its rules (despite the beneficiary having earnings from  work activity), SSA may suspend benefit payments. When benefits are  suspended, the beneficiary remains eligible to resume benefit payments  based on the claim or application for benefits. The beneficiary is still  required to show that he or she remains disabled and meets the medical  eligibility criteria, but their work credits and insured status will not  have expired or lapsed. If benefits are suspended, SSA will extend the  beneficiary’s eligibility based on the prior work record. This is called  an Extended Period of Eligibility.

​

C. Benefits During An Extended Period Of Eligibility.

The  Extended Period of Eligibility may be up to 36 months, but the  beneficiary may not continue to receive cash benefits for 36 months.  Extended Period of Eligibility means that the eligibility to receive  SSDI benefits on the prior work record continues for up to 36 months if  the individual continues to be disabled under SSA rules. During the  Extended Period of Eligibility, a beneficiary does not receive monthly  cash benefit payments, but the beneficiary continues to be eligible to  resume benefits based on their prior work record if the beneficiary  stops working.

​

However,  a beneficiary who wants to resume benefits based on their prior work  record must still show evidence that he or she continues to be disabled  under SSA Rules. The medical criteria for determining disability status  are the same during an extended period of eligibility as during an  initial application. The return to active benefit status is not  automatic. SSA must still review the evidence to determine if the  individual is disabled under the medical criteria set out in the law.  SSA often describes this review as an “expedited review.” “Expedited  review” describes SSA’s internal procedure on review, but there is  little or no difference in the speed or ease of the procedure  experienced by the claimant.

​

III.  Extended Period Of Medicare Coverage (EPMC).

A. EPMC Is An Additional Work Incentive.

SSDI  beneficiaries often believe that Medicare entitlement stops when cash  payments stop. This is true in most cases, but not all cases. It is  possible under certain circumstances for a beneficiary to continue  Medicare coverage, even when monthly cash benefits have ceased. In 2000,  Congress extended Medicare coverage for people who receive SSDI  benefits and who work while remaining disabled.

​

The EPMC  provision makes it possible for beneficiaries who continue to be  disabled to continue Medicare coverage after the TWP ends and after cash  benefits stop due to substantial gainful activity. A SSDI beneficiary  may keep Medicare coverage when the beneficiary is working as long as  the disabling condition continues to meet SSA rules.

​

The EPMC  is a work incentive for SSDI beneficiaries. EPMC is intended to benefit  individuals who remain disabled and who have lost their monthly cash  benefit due to work activity. EPMC is not intended to maintain Medicare  coverage when SSA terminates benefits due to medical improvement that  makes the beneficiary no longer disabled under the medical criteria for  benefits or for other reasons resulting in a termination of eligibility.  Individuals receiving the EPMC must still meet SSA disability  requirements, even though these individuals may be working and are not  due a cash payment.

​

B. How Long Is The EPMC?

The  current rules allow EPMC to continue for at least eight and a half years  (93 months) after a TWP ends. During the EPMC, the beneficiary will not  receive a monthly cash benefit, but will continue to be covered by  Medicare.

​

The EPMC  period typically will not begin until a person’s TWP is completed. This  is because monthly cash benefits are protected during a TWP, and  Medicare enrollment continues while cash benefits continue. Determining  exactly when EPMC begins and ends will depend on several factors. Making  an exact calculation is often difficult for an individual because the  information required to make an exact calculation is based on Social  Security’s records and determinations.

​

C. Obtaining EPMC.

EPMC is  not automatic. EPMC is an extension of a person’s current Medicare  enrollment. EPMC cannot be obtained if SSDI benefits have already been  terminated. Thus, it is important to confirm EPMC before work activity  causes SSA to terminate benefits. The EPMC and the Extended Period of  Eligibility run concurrently, not consecutively. It is best to confirm  the EPMC with SSA near the end of any TWP if the beneficiary intends to  continue work activity with income over the SSA allowable amount of  earnings.

​

D. Continuation Of Medicare Coverage.

Individuals  seeking continuation of Medicare have typically been eligible for SSDI  benefits for more than 29 months. This is because a beneficiary does not  begin to receive Medicare until 29 months after the date SSA determines  the beneficiary is disabled.

​

The  29-month delay in Medicare enrollment is due to Social Security’s  waiting period before Medicare starts. Under SSA rules, a beneficiary  must wait five full months after the date of disability determined by  SSA before the beneficiary may begin to receive the SSDI monthly cash  benefit. During the five-month initial waiting period, no cash benefits  are paid and no Medicare coverage is provided to the SSDI beneficiary.  After the initial five-month waiting period, the monthly cash benefits  may begin. However, Medicare enrollment does not begin until an  additional 24 months have passed. The initial five-month waiting period,  plus the 24-month Medicare waiting period, combine for a total of 29  months after the date of disability.

​

A  beneficiary cannot continue Medicare until after Medicare begins.  Therefore, a person cannot seek a continuation of Medicare benefits  until they are enrolled in Medicare, which occurs 29 months after the  date of disability.

IV.  Conclusion.

​

The  rules discussed in this article are complicated. Nothing in this article  is meant to be legal advice about your specific situation. If you have  questions please contact the CF Legal Information Hotline at CFLegal@sufianpassamano.com or by calling 1-800-622-0385. All calls to the CF Legal Information  Hotline (CFLIH) are confidential and free of charge to the caller. The  CFLIH is sponsored by a grant from the CF Foundation but the CFLIH  employees are not employed by the CF Foundation.

Beth  Sufian is 56 years old and has CF. She is an attorney who focuses her  law practice on disability law and is the Vice President of USACFA. Her  contact information is on page 2. You may contact her with your legal  questions about CF-related issues at CFLegal@sufianpassamano.com.

My  spiritual life is upheld by a fundamental belief: to embrace sorrow and  pain together with joy and wellbeing. This is the true meaning of yin  and yang—that life has light and darkness swirling together in every  experience, every moment, and every lifetime. In this issue focusing on  pain, I’d like to ponder how the pains in life can coexist with fun and  wellbeing.

​

The day  after cancer surgery on my eye, I discovered Audible and randomly chose a  book that popped up called The Power of Fun. Catherine Price, its  author, who also wrote How to Break Up with Your Phone, argues that  adults do not have enough fun. We are so caught up in achievement,  productivity, and capitalistic consumer culture that we devalue fun.  Cell phones and laptops have also pulled us away from sources of  creative fun, entertainment, and engagement. Price defines fun as  anything that includes these three things: playfulness, connection, and  flow. Flow is defined as a state of being fully immersed in an activity,  with energized focus and full involvement, where time seems to stand  still. It is actively engaging in fun rather than being passive, like  watching TV. It is different from a “relaxing” activity (though  sometimes those feel good), such as walking in nature, getting a  massage, or lying on the beach. Fun is good for our physical, spiritual,  and emotional wellbeing. Fun is not selfish, indulgent, or immature. We  all need to enjoy life.

​

This  book inspired me to pursue more fun in my life, intentionally and  deliberately. Because of this book, I’ve started a FunSquad with the  CFRI Retreat monthly Zoom calls. As people with CF, we have plenty of  pain and struggles, but incorporating fun is such a vital compensatory  act. When working nearly full time, I was deeply deprived of fun. My  “free” time was spent on healthcare and exercise. My father always used  to say, “work hard and play hard.” I worked hard at a job and healthcare  and then went to sleep. That was pretty much it.

​

So what  can we do to have more fun? I’m planning more. I plan to go to  pickleball practice every week or two. I’ve planned to mosaic my garden  wall with friends. I’ve planned a few trips with close friends. I make  sure to schedule things with my nieces. Even if I feel physical pain, I  still do these things, because they are healthy distractions and  inevitably I feel better afterwards. I have the kind of body where the  more I move, the better I feel. Some people have a hard time feeling  relief from pain much of the time. But fun can also be stationary: a  board game, comedy writing, karaoke, or even painting.

​

Distraction  is not the same thing as denial or avoidance. It is a deliberate  compartmentalization of our experiences. We create space for positive  emotions and sensations and set aside the painful ones.

​

I do  believe that fun is part of a healthy spiritual life. Some religions  teach asceticism, saying that abstinence from sensual pleasures can lead  to spiritual transformation. This has given fun a bad rap when it comes  to spirituality.

​

I  believe God wants us to be happy and celebrate life and laughter. After  all, many of the times we’ve had the most fun have been done with love  for the people around us. Love is the foundation of all spirituality,  after all. Fun lightens us from the heaviness of our struggles. We need  not overindulge, get intoxicated, or succumb to physical temptations to  have fun. But we do need to wake up our life-force energy that makes us  glad to be here and to feel grateful for life, no matter its challenges.  Fun helps us balance the yin and yang, the dark and light, the negative  and positive. When we endure pain, we have a choice to set it aside  (when we can) and redirect our focus on the good, the joyful, and the  fun side of life. And when our pain and fatigue are too much to have  fun, we can assume the attitude, as my dear cousin did: “I’m learning to  appreciate living through others’ experiences.” We can still have fun  vicariously.

​

Pain is  an inevitable part of living with CF, and sometimes it seems to get  worse as we get older. If pain is going to be present, how can we live a  full life, anyway? Besides having fun, I’d like to explore the idea of  wellbeing. Positive psychologist Martin Seligman’s book, Flourish,  summarizes his research to see what makes some people thrive in the  midst of adversity. He studied happiness and life satisfaction but felt  these themes did not fully capture what it is that helps us endure  through struggles. He tried to see what makes life worth living. He  decided that wellbeing is a state that helps us judge life positively  and feel good. He uses the acronym PERMA to define wellbeing: positive  emotions, engagement, relationships, meaning, and accomplishment. We  need to feel some positive emotions to be well. We need to have some  engagement in life activities—hobbies, work, volunteerism—to be well. We  need to have positive relationships with others to be well. We need  meaning—purpose, a why, a reason—to be here to be well. And finally, we  need a sense of accomplishment to feel well—some achievement  vocationally, academically, socially, or artistically that helps us feel  good about ourselves.

​

In this  CF life, I believe wellbeing is literally life-giving. Wellbeing gives  us our drive to keep going. Our PFTs can be dismally low, our futures  bleak, and our bodies full of chronic pain, but we can still reach PERMA  if the right variables are set in place. I’ve witnessed that being part  of the CF community can definitely create PERMA. Being aware of our  mortality can also push us to seek and find PERMA sooner in life  compared to the pace for our healthy peers. Seligman emphasizes that  wellbeing can be learned and calls this “learned optimism.” He has  developed specific trainings to help teach PERMA and learned optimism.

Seligman’s  work about psychological wellbeing has a direct connection to spiritual  wellbeing as well. Chronic pain can make us feel abandoned by God. In  my professional world of hospice, we define spiritual (or existential)  pain as a time when a person is “unable to find sources of meaning,  hope, love, peace, comfort, strength, and connection in life or when  conflict occurs between their beliefs and what is happening in their  life.” (Anandarajah and Hight, 2001). PERMA can help us find some relief  from this kind of spiritual suffering. Feeling the effort of life is  worthwhile is often augmented when we have connection, love, and  meaning.

​

Fun and  wellbeing help us embrace the gift of the miracle of life, no matter how  painful it is. Rather than succumbing to pain, we have agency to decide  how we carry it. Fun and PERMA remind us that we can still be well  despite the challenges of our bodies. If you’d like to join CFRI  Retreat’s FunSquad and share your fun, email me at the address below.  You can google the Penn Resiliency Program and “PERMA Workshops” to  learn more ways to boost your wellbeing. I wish you pain relief and  wellness.

Isa  Stenzel Byrnes is 50 years old and has CF. She lives in Redwood City,  California. She is 18 years post-lung transplant. You can contact her at  isabear27@hotmail.com.

It  is the middle of May and my face remains under construction. Right now,  I have an even bigger gap in my bite thanks to getting mounts built for  the permanent bridge that will get installed about two weeks from now.  That work also included some surprise gum surgery—words that do not seem  like they should go together in a sentence. But with CF, anything is  possible. So the continuing saga with getting my jawbone repaired and  filling the gap in my bite has me thinking anew about pain and how we  perceive it as people living and aging with CF.

​

I still  feel confused and frustrated about why it took so long to get adequate  treatment for an infection and cyst that had eaten through the bone in  my tooth socket when I was getting regular dental care. The short answer  here is that neither x-rays nor CT scans—the only imaging that was  available to both my dentist and my surgeon—showed the full extent of  the problems with the root of that tooth. So even though I had let my  dentist know periodically that the socket below that crown felt strange,  which seemed especially concerning since the nerve in that root had  long since been removed via root canal, she was not able to do much  until the prosthetic in that spot broke apart.

​

This  feels frustrating to me because I suspect I would have experienced much  worse pain—and thus sounded a serious alarm about needing the root  removed—years earlier if I were not so accustomed to dealing with  various kinds of pain each day as a result of CF-related infections. Of  course, those same infections are likely what caused such severe dental  issues in the first place. Understanding the origins and implications of  the pain I feel in different parts of my body challenges me constantly.  Figuring out how to respond to these experiences feels even more  difficult.

​

It  probably comes as no surprise that I have done some work on pain  management in my own career, dating back to my days in the Master of  Public Health program at Rutgers, where I focused my capstone project on  what hospitals are doing to offer patients alternatives to opioids for  chronic pain management. About two years before starting my M.P.H., I  had undergone an operation to remove scar tissue that had accumulated in  my bladder from many years of recurrent infections and my body  attacking the tissue with histamine as part of the immune response. Over  time, thick masses of scar tissue started pressing on the nerves in the  wall of my bladder, causing constant sensations of someone squeezing my  pelvis in a vise or stabbing it with a knife.

​

I first  experienced that pain when I was 11 years old. I had just turned 22 when  I underwent surgery to remove the scar tissue. Being in constant pain  feels scary no matter your age. It was also incredibly disorienting when  so much else about my body was changing. Because, at that point, nobody  knew for sure that my health issues were caused by CF rather than some  sort of primary autoimmune disease, there was little I could do to stop  the vicious cycle of infection and scarring.

​

Even  several years after identifying the genetic mutations that cause my CF,  my ability to stop what remains of my natural dentition from being  destroyed by virulent bacteria remains limited. I have already had most  of my teeth completely replaced above the gumline; I have also undergone  multiple operations to restore the gums themselves. Now my prosthetic  teeth are breaking apart as infections destroy the roots that support  them. I expect that if I live long enough, I will wind up wearing full  dentures. I would feel happy with this outcome because it would mean I  survived long enough to lose my remaining roots. What I do not look  forward to are the years of renewed pain that would likely precede that  transition.

​

What  frustrates me most is that even after 38 years of living with this  disease and six years of knowing for sure that I have it, I still do not  always know when the pain I feel is something usual versus something  requiring urgent action. My ability to perceive something harmful as  painful is already so compromised by nearly four decades of acclimating  to chronic pain that I have to exercise caution when cooking so I do not  accidentally put my hand on a hot pan. I burned the skin off one of my  knuckles several years ago because I did not realize I was touching  something hot until I smelled singed flesh.

​

I have  voluntarily had deep fillings done on some of my teeth without any  anesthesia because I know I will not feel anything from the drill except  vibration. When I had my wisdom teeth extracted, I asked for only local  anesthesia and then drove myself home afterwards. I did the same for  the surgeries to remove my diseased root and install the bone graft. I  took my antibiotics as prescribed afterward but declined pain  medication. When I had my gumline reshaped a couple weeks ago around  where the bridge will go, I again asked for the minimum possible amount  of Novocain. I took an ibuprofen after the procedure to help control the  swelling then forgot to take more later. My upper lip felt stiff and  immobile for a few days afterwards. I could feel pressure and swelling  around that part of my jaw, and some tingling in the nerves in the roots  of the teeth that had been ground away, if I drank a cold beverage. But  I cannot say I felt any real pain.

​

What do I  even consider “real” when it comes to my own pain, though? Is that even  a relevant term for people who experience pain similarly? The memory of  spending an entire night vomiting from the combination of general  anesthesia and morphine I was given during and after my bladder surgery  still lingers. It makes me averse to pain medication unless I  “absolutely need it.” For pain to distract me it has to be incredibly  severe. I would rather deal with nuisance discomfort than become  incapacitated in other ways. But does that mean my pain is not real or  that it should not be managed?

​

I grew  up in a neuroscience research lab, which gave me some good insight on  why we feel pain and how not all types of pain are alike. Some pain  originates in nerves outside of our brains and some originates within  the brain itself. This means not all pain responds to the same types of  therapies. It also means understanding our own pain can be incredibly  difficult even when we have years of experience. And as other articles  in this issue of CF Roundtable demonstrate, not everyone with CF  experiences the same things when it comes to feeling pain. Some people  experience heightened awareness and some, like me, barely register  discomfort. It may be peak neurodivergence that I get more bothered by  certain fabric or food textures than when I walked around with broken  ribs for several months.

​

Then  again, I may feel more bothered than I consciously know. My wife can  often tell when I am in more pain than usual because of my body language  and affect—even if I do not realize anything is amiss myself. The  sociologist in me thinks there is probably more to this than just  acclimation. In the distant past, I was punished for showing any  evidence of being sick. I was also constantly reminded that my health  issues made me less desirable and less valuable than my peers. This has  not been the case for a long time, but it certainly took a toll during  the years I experienced it. I think I do prize being “tough” to a  certain extent—or at least, I used to. Now I just feel lost a lot of the  time, and sad about all the opportunities I have missed to advocate for  myself because I could not voice that I was hurting.

​

I have  no easy answers, either for us as patients or for those without CF who  care for us. But I do feel strongly that we need focused attention to  pain as an integral part of living and aging with CF. This remains a  progressive disease even for people who benefit tremendously from  therapies that keep our respiratory and digestive systems clear and  functional. More of us will experience the breakdown of other body  parts—our teeth, our bones, our joints—as we live longer without going  into respiratory failure or starving to death. Pain is often part of  aging even without underlying chronic conditions, simply because of wear  and tear on the body. What does that mean for our community in the age  of CFTR modulators and other innovations in treatment? We need to ask  those questions early and often to ensure patients can receive pain  management appropriate to our unique individual needs as we grow older.

Dr.  Alexandra “Xan” Nowakowski is 38 years old and has CF. Xan is a  director of CF Roundtable, in addition to being a medical sociologist  and public health program evaluator. They currently serve as an  Assistant Professor in the Geriatrics and Behavioral Sciences and Social  Medicine departments at Florida State University College of Medicine.  They also founded the Write Where It Hurts project (www.writewhereithurts.net)  on scholarship engaging lessons from lived experience of illness and  trauma with their spouse, Dr. J Sumerau. You can find their contact  information on page 2.

Like  many CF patients, I’ve dealt with decades of chronic pain. Whether it’s  bouts of recurrent pleurisy, dislocated ribs from coughing, sinusitis  triggering migraines, or abdominal pain from Distal Intestinal  Obstruction Syndrome (DIOS) and gastroparesis, there’s always something  that hurts!

​

Chronic  pain can be particularly challenging as a parent. The physicality of  raising kids—carrying babies, being climbed on by toddlers, getting hit  by your preschooler mid-tantrum—is often unrelenting, and it’s easy for  the everyday rigors of parenting to exacerbate existing chronic pain  issues.

​

As a  mama to a wonderful nine-year-old who has always been a highly physical  kid, I’ve spent years trying to figure out how best to balance my body’s  needs with my daughter’s needs. While some seasons are harder than  others, here are a few of the things I’ve found most helpful when it  comes to parenting through pain.

1. Honor Your Limits

Just  like parents are reminded on airplanes to put on their own oxygen mask  in case of emergency before they put on their child’s, there are times  when we, as parents with CF, have to get serious about respecting our  physical needs, even if that means we can’t do exactly what our child  wants.

​

Because  sleep is key for me in dealing with both chronic pain and pulmonary  health, I have always needed to schedule time for an afternoon nap.  Often, this means setting my daughter up with games, books, or a TV show  that she can enjoy on her own, even if she’d really rather have my  company. (When she was little, I shamelessly bribed my daughter to let  me have some quiet time in my bedroom alone—we started with five minutes  of quiet time and slowly worked up until she could play or read for an  hour at a time.)

​

I also  have mobility limitations and chronic muscle pain due to other  conditions and have to be careful about how much walking I do in a day.  Sometimes this means saying no when my daughter wants to take a walk or  play an energetic game outside while other times it means finding ways  to modify the activity she wants to do so that I can do it while  sitting.

Although  it can be hard to tell a pleading kid that you can’t play because you  need to sleep, or that you can’t push them on the swing anymore, or that  you can’t go to the park—honoring your limits and making sure you’re  respecting your body’s needs ultimately gives you the tools you need to  be a more present parent.

2. Set Firm Boundaries

Because I  have fibromyalgia, I sometimes experience hypersensitivity to touch.  During those times, I’ve found I have to be explicit with my daughter in  setting boundaries about how she’s allowed to touch or cuddle me. We’ve  also had a lot of talks about how mama needs gentle hugs, because my  ever-present abdominal pain from a lifetime of DIOS and dysmotility  makes tight squeezes very unpleasant!

​

When  setting boundaries with kids, it’s important to be kind and caring, but  firm. Talk to your kid about what your pain is like and help them to  understand what kind of touch is okay and what isn’t. Practice ways they  can touch you that don’t trigger pain, and, if they forget, give a  gentle reminder.

3. Get Creative

Whether  you can’t join your kid on a hike because of the altitude, or you can’t  carry them because of chest pain, get creative to find ways to work  around your pain! Some of the workarounds I’ve used throughout my  daughter’s life include:

​

A.  Learning about babywearing when she was a baby and experimenting with  several types of wraps and slings so that I could hold her without  additional stress on my body.

B. Snuggling together on the couch instead of holding her when she got too big for me to lift safely.

C. Researching activities and games we could play with me sitting or lying down.

D.  Incorporating my need to rest into the games she wants to play. The  chair I’m sitting in becomes the soccer goal; the fact that I need to  lay down on the couch is because I have a mysterious illness she needs  to go on a fantasy quest to solve; I can’t run around in the kiddie pool  with her, but I can sit beside it and splash her with my feet.

E.  Finding engaging, kid-friendly audiobooks and podcasts to listen to  together when I don’t have the stamina to do anything more active.

​

Chronic  pain is definitely an extra layer of hard on top of the normal  difficulties of parenting…but finding ways to honor our limits, set firm  boundaries, and get creative about ways to play on bad pain days can  not only help us to manage our pain, but help us to raise children who  are empathetic and compassionate.

Cindy Baldwin is 34 years old and has CF. She is the author of several books with HarperCollins, including the upcoming No Matter The Distance (February 2023), which features a protagonist with cystic fibrosis.  Cindy lives near Portland, OR, with her husband and daughter.

Readers,  I’m thrilled to introduce one of my closest friends in the CF  community, artist and performer Dominic Quagliozzi! We met in 2017  because he messaged me after I posted in the big public CF Facebook  group. We bonded over our love of visual and performance art, our  philosophy about intimacy and relationships, and our fascination with  the evolving human body. We also have very different CF  experiences—almost seven years ago, Dominic received a double lung  transplant. He also started Trikafta this January. But that’s not even  the biggest change Dominic has made this year. He and his wife Deb  returned to Massachusetts after many years in California. Their son  Thaelo is now experiencing his first snowfalls at their new home in  Worcester. Please welcome our newest star, Dominic Quagliozzi.  Spotlight, please!

​

Age: 39

​

Home: Worcester, MA

​

What would you most like our readers to know about who you are and what makes you feel connected to others with CF?

I’ve  always been a big proponent of building communities. One of the biggest  things artists can do is build a community; it’s no different with CF.  We’ve done a great job with resources and providing opportunities for  community building over the years. That goes back to my days as a young  kid in the early 90s, well before the six-foot rule. We had camps and  events where all the CF kids were invited, so I met others my age. This  made a difference developmentally, especially in my preteens and teens.  It let me stay connected to other people with similar challenges; I felt  like nobody could really relate to me except my CF friends. So I’ve  always reached out to build those relationships.

​

You’re  an accomplished visual and performance artist; these days you’re also a  dad and a homeowner! How do you balance all of that with the daily tasks  of managing CF?

It’s all  about living these experiences in my daily life—my health life, my  chronically ill life—and then bringing them into the studio with  materials from the hospital. I love having Thaelo be part of all that.  He has a huge play space with a carpet; he loves when I’m in the studio.  Whenever I’m down here, he comes and finds me. So my studio basically  has been completely infiltrated by his toys. But that just makes it  easier to spend time with him while I create.

​

I know  you and Deb went through a pretty complicated journey to owning your  home. What was it like doing all that as someone with transplanted lungs  in the middle of a global pandemic?

I built  quality relationships, both in general and with medical professionals,  during my 15 years in LA. That reduced my anxiety and depression while I  was there. Moving away from my literal paradise was so hard. I felt  like the most like myself there; it was a huge part of my heart and  soul. I moved back here for specific reasons but I keep questioning,  “Why did we do this? Why did we come back?” But the artist in me is  saying, “These things are going to strengthen the art you make.”

​

Can you  tell us more about your transplant experience and how living with your  new lungs has shaped your art? Where have these experiences taken you  creatively?

I would  joke about feeling scared to get transplanted—not because I worried  about dying, but because I feared once my CF lungs were removed I would  lose all my artistic power. They brought me so much struggle,  perspective, and content. In a weird way, my CF lungs were very giving  toward my art career. Taking that away scared me and still does; I’m  wrestling with that and the trauma underlying it. But my new lungs have  given me seven more years of making art; now my work is getting  purchased by museums and I can reach a wider audience. My current work  has similar threads—common themes like temporality, patienthood, and  privacy. So I’m still doing those things, just differently.  Pre-transplant, I did a lot of performance-based things where I used my  body. Now I’m not doing as many performances, but I’m using a lot of  ephemera from patient life like hospital gowns, clinical tissue, and  rubber gloves.

​

You’ve  also been hard at work setting up your new studio space at home. What  will your basement studio enable you to do artistically in the year  ahead?

A major  factor in choosing this house was that Deb gets a beautiful studio  upstairs that has natural light all day long for painting, illustration,  and pattern design. Then I have the basement space that allows me to do  all kinds of projects at once, which is really how I work best. I’ve  got about 200 square feet, probably five times more than before. So I  want to do larger-scale projects like soft sculptures using hospital  gowns. I can work on multiple projects at once by setting up different  tables and hanging canvases on the walls. I have one table for drawings  and one for sewing, plus big folding tables I’ll use for other projects.  Right now I’m developing ideas around gowns or tissue and themes of  clocks and parachutes. In this space I can stretch out, get messy, and  return to the fundamentals of my art practice.

​

What are your big goals with your art right now? Where would you like to go with your work in the near future?

Starting  Trikafta has really influenced my goals for my art. One of my new  drawings is in a show at the Rhode Island School of Design Museum. The  director asked if we could extend the show until October 9, which is  amazing. So I have to network and meet other influential art people in  New England—because of that show, I can connect with more opportunities  in the area. I would also love to get a job teaching art somewhere or  develop my own program with Deb. The freelance life has been stressful.  It’s burning me out and making time management tougher. I’m missing  deadlines and feeling scatterbrained about qualifications and materials  for different job and grant applications. I would also like to do a  residency. There are some really good art residencies in New England, so  I want to take advantage of living here.

​

Besides  lung issues, what are your biggest challenges with CF? What is your  unique presentation of the disease like? How has that changed in your  experiences with CFTR modulators?

It’s  this constant flow of problems that move around my body. I’ll have sinus  issues and then start a treatment for those. Then the sinuses clear up a  little, but I’ll have something else—a severely low white blood cell  count, for example. Then I’ll need a bunch of shots and medication  changes to stimulate my bone marrow. That’ll clear up and then I’ll be  dealing with arthritis in my back, causing pain and weakness. Next, I’ll  have similar issues with my knees. My new thing is entering Stage 4  kidney disease—it’s getting pretty serious. Most days my legs swell up  so much that I have trouble even wearing my shoes.

​

You’ve  had a lot of changes with your health since starting on Trikafta. How  have your experiences with Trikafta differed from what you noticed with  previous modulators you were taking? How have your expectations evolved  as you’ve been on the triple combo longer?

In 2015,  Orkambi came out for folks with double delta F508, but that was a  couple weeks after my transplant. So I didn’t try that one or Symdeko.  But when Trikafta came out in 2019, I was excited and wanted to know if I  could get on it. I didn’t actually get approved until I moved here. I  started January 1, 2022, and, so far, it’s been hard. I’m getting  physical issues with fatigue—my muscles feel like I’ve been doing  squats. I know that’s a possible side effect, but I haven’t talked to  anyone who’s had it this badly. And I’ve been falling asleep like twice a  day. I went to my endocrinologist and my labs look fine—slightly low on  iron, but that’s it. It’s a lot emotionally and mentally; sometimes I  question why I’m doing all this. I will say, though, it’s nice to feel a  breeze in my sinuses! Breathing through my nose again feels weird but  awesome. Other things are the same—I haven’t gained any weight or  changed my diabetes regimen.

​

What  helps you cope with your CF? Are there any community resources you’ve  found helpful in living with CF—like websites, newsletters, apps, social  media groups?

Being  online, definitely. In the late 90s, when computers and AOL chat got  bigger, I stayed in touch with more CF friends virtually. I used  MySpace, but it was really Facebook groups that helped a ton. I knew  maybe a dozen people growing up with CF just from hospital functions.  But once I got on Facebook, I suddenly knew hundreds of other CFers  worldwide. That’s been really amazing. It also helped me through the  transplant process; I went through it with four people who were going  through it at the same time. We became friends and bonded very closely  because of that. Of course, there’s a dark side to that. I’ve had  friends I’d chat with every day online and then I’d see their wall with  messages saying, “RIP” and “I can’t believe you’re gone.” I’d think, “We  were just talking last night.”

​

You and I  really connected about the closeness we share with our spouses and how  that intimacy supports us in managing our health. Can you tell our  readers about how you and Deb met and how you first shared about your CF  with her?

Until  Deb, I basically withheld my CF. I’d only had more casual relationships;  there was definitely something different about Deb. Even right when we  met, I thought, “This might be a time where I need to say something.” I  needed to be completely honest—and I was hesitant because it was scary.  On our second date, she was feeling down and told me she was having some  health issues. I said, “That’s awesome because I have cystic fibrosis!  I’m used to being a patient, so whatever you’re going through, I’ll be  there for you.” We connected over this really dark subject matter over  dinner and just kept talking. Thankfully, her issue resolved, but,  shortly afterward, I had an exacerbation and ended up hospitalized. She  started coming and hanging out; she brought her pads and we would sketch  each other. I loved making art together, still do—it was a partnership  from the beginning. We’ve taken care of each other so much; now with  Thaelo here, it’s a new adventure.

​

As a  first-time dad, how are you planning to talk with Thaelo about CF as he  grows older? How does living with transplanted lungs—and now being on  Trikafta—shape what you’d like to share with your son about your health?

I just  want to share as I go. I wanted to see what kind of kid Thaelo would be  and choose a pace he could handle. Now that I know him, I think that’s a  good approach. He’s only two, but he’s completely there for me. If he  notices I’m tired or not feeling great, he’ll bring me some water or pat  me on the leg. It’s wild to see how empathetic and emotionally  intelligent he is. So I’ll go step by step with him—more in kid terms  now, then more specific language over time. He already sees me taking  pills every time I eat. I’ll bring that up: “You know how you went to  the doctor for your physical? Daddy has to go to the doctor, too.” We’ll  read books or watch shows about healthcare and talk about them. I want  him to know he might take care of me at some point—he already does, in a  way. I want him to know the expectations as we get older. But he’s a  very easygoing kid; he can adapt to change.

​

Do you have a funny CF story you’d feel comfortable sharing? Is there an experience you look back on now that makes you laugh?

I ran  into a cliché because of COVID-19! I was making all this work before the  pandemic even happened. Now people think I’m just remarking on  COVID-19—not looking at the year it was made, or the context. There’s a  weird traumatic response to that. Now everyone’s making work about  illness; they probably have paint-by-number about COVID-19! It’s a weird  time for me as an artist and I’m trying to feel my way through it. I  was using masks in my work or making portraits of my hospital team—like  those photos where my providers didn’t have the bottom half of their  faces in 2010. I feel protective and resentful; I’m struggling with  that. I want to let it go, but I can’t—this is my life. Did people  realize all these COVID-19 protocols were my transplant protocols? I  didn’t complain about them; I wore a mask literally everywhere except  indoors in my own home for three months straight. Not just a mask that  you could pull down under your nose, but a two-canister respirator.

​

Living  with CF constantly requires us to change our plans and accept difficult  things. What would a perfect day look like for you? Where would you go  and what would you do? Would your answer be different if the COVID-19  pandemic were under better control?

That’s a  hard question for me to answer right now, because obviously I would go  away from here. I’d fly to LA and spend the day there. Despite the air  pollution, I think LA is the perfect place to have cystic fibrosis  because the climate is dry. The humidity in Massachusetts gave me  pneumonia every year on my birthday in April. There’s also a daily  struggle getting from one place to another. The mobility issues that I  have with CF are common, like arthritic knees. Even though I’m  post-transplant, I do have chronic rejection plus shortness of breath  with activity. Struggling with snow and ice getting to my car, needing  to shovel out my walkway and snowblower the driveway, are very  challenging for me. Those big environmental changes make me notice my CF  way more here.

Dr.  Alexandra “Xan” Nowakowski is 38 years old and has CF. Xan is a  director of CF Roundtable, in addition to being a medical sociologist  and public health program evaluator. They currently serve as an  Assistant Professor in the Geriatrics and Behavioral Sciences and Social  Medicine departments at Florida State University College of Medicine.  They also founded the Write Where It Hurts project (www.writewhereithurts.net) on scholarship engaging lessons from lived experience of illness and trauma with their spouse, Dr. J Sumerau.

If  you would like to be interviewed for “In The Spotlight,” please contact  Xan Nowakowski or Andrea Eisenman. Their contact information is on page  2.

I  have struggled with chronic pain for a big chunk of my life just like  many of my fellow warriors, particularly those who’ve been fortunate  enough (or unfortunate enough, depending how you look at it) to hang  around for five-plus decades. At the risk of losing my audience at the  outset for committing the crime of winning, I will briefly describe the  sources and causes of my chronic pain from most to least favorite.

​

To begin  with, I have several lingering sports injuries that I am particularly  proud of: several hockey injuries—a banged-up shoulder, fractured  kneecap, and back pain from slipping too many discs—and a broken wrist  from a kickboxing tournament. I also have constant pain and tightness in  my abdomen from being stabbed during a blackout and pain in my left  elbow from being thrown through the rear glass door of the Apalachin  Tavern. While all of these injuries are quite painful, I relish them the  same way some folks lick their canker sores, because it makes me feel  alive and brings to mind memories of the full life I once lived, bad  choices and all.

​

On the  other hand, my least favorite pain is CF-related because it was not by  my choosing and it prevents me from living life more fully.  Specifically, at present, my CF-related pain primarily consists of  near-constant GI bloating and pain due to my enzymes no longer working  as well and sharp, unpredictable right-lung pain, which began about ten  years ago and has only increased in frequency and acuity ever since.

​

Quite  naturally, the seeds of pain have always inevitably sprouted anger  within me if I do not take effective action to prevent it. Beginning at  age nine, I started flying into fits of rage occasionally that seemed to  come out of nowhere, not fit the circumstances, and last way too long.  For example, one time I flew into a rage because we were out of  strawberry Nesquick, which I discovered upon arriving home after a  clinic visit in which I saw my best friend on oxygen for the first time.  I deeply regretted having that outburst and the many others that would  follow, which never failed to cast a shadow of shame for days afterward.  In their eagerness to console me, my nurse and my mom reassured me that  those reactions were normal. Their reassurance did nothing to reduce  the number of outbursts but did make me feel less guilty afterward.

​

Interestingly,  when I was nine, my dad, who rarely helped with my chest physical  therapy (PT), accidentally bruised three of my ribs while doing my chest  PT. Not surprisingly, that made it excruciatingly painful to breathe,  let alone cough, for several weeks. Prior to that, on the eve of my  first day of Christmas break, my parents got a surprise visit from my  bus driver complaining about me. At this point, you’re probably  wondering what these two events have to do with one another and, even  more poignantly, the topic at hand.

​

For  starters, a few days prior to my bus driver’s ill-timed Christmas visit,  some kids on the bus were playing with a radio-controlled plane and, as  kids often do, were teasing me by keeping it from me. After begging and  pleading with them to let me play with it, I yanked it out of one of  the kid’s hands while the propeller was spinning and it accidentally got  badly knotted into the hair of a girl sitting across the aisle. When  she burst into tears and began screaming, the bus driver flipped out,  stopped the bus, and yelled at me—even going as far as threatening to  kick me off the bus. After he finally extracted the propeller from the  girl’s hair by using another kid’s scissors, which unfortunately left a  gaping bald spot on the back of her head, he leveled another threat at  me, which barely registered at the time: “I’m going to pay your parents a  visit this Christmas to let them know what a monster you are.” I did  not try to talk back because the incident had been resolved and I never  imagined he would follow through.

​

Later,  his ill-timed visit did ruin my Christmas break and, worst of all, my  parents returned all my Christmas presents as punishment. When that  dreadful Christmas break was finally over, I was bitter due to what my  bus driver did and my whole body ached from what my dad did, so I was  completely miserable and my mind kept slipping into darkness,  involuntarily contemplating ways I could exact revenge on my bus driver.

​

I’ve  always known that whenever anyone hurts you, if you’re patient and act  at the exact right moment, you’ll have the opportunity to exact  vengeance, which is why I also fervently believe the key to not acting  in anger is letting go of the hurt swiftly and forgiving the offending  party quickly. However, in the throes of my rib pain, when that perfect  moment arrived, I did not hesitate and let loose two simple words, “it’s  clear,” that I had no doubt would exact the vengeance I sought. Several  factors contributed to maximize the impact of those words at the exact  moment I uttered them. The bus driver’s regular short bus, which had  minimal blind spots, was in the shop and a senior, Jimmy, who liked me  and took me under his wing, cajoled the bus driver into letting me sit  in the back with him while he spotted for the bus driver, who needed  help with blind spots while backing up to turn the bus around at the top  of the Jewitte Hill, where Apalachin’s upper crust lived with their  fancy extra cars occasionally parked on the streets. Unfortunately for  my bus driver, I quickly responded, “it’s clear,” when he asked Jimmy to  look out the window—just as we began rolling over the hood of Dr.  Rosen’s 1965 Morris Garages (MG) Roadster. This sounded remarkably like a  bus rolling over a frozen snowbank. Jimmy and I shot each other a  knowing look that seemed to acknowledge instantly that neither of us  ever saw the little sportscar now deeply wedged under the bus. Needless  to say, our bus ride literally came to a screeching halt that day until a  rescue bus came to take us the rest of the way to school, and our bus  driver never drove that route again.

​

In  retrospect, I do not believe that I would have been able to sustain my  anger for as long as I did, plot my revenge so thoroughly, or act in as  timely a manner if I had not been in such intense pain, which made it  hard to focus on positive things and made it even more difficult to move  past my anger. I am thoroughly convinced that this angry act, which was  admittedly devastating to many undeserving people, was as much a  reaction to my physical pain as it was to my anger at any one  individual.

As I  mentioned earlier, the frequency and intensity of my chronic pain has  once again escalated, which has made me increasingly irritable and has  made it difficult to stay focused on positive things. I am so concerned  that the seeds of pain may once again sprout anger, that I enrolled in  an online anger management class for adjudicated (convicted and  sentenced) clients. I found through experience that when traditional  counseling does not work to curb anger, anger management courses,  particularly those that meet court-mandated criteria, can be extremely  helpful. In fact, when I was a young psychologist in my mid-20s, I used  to teach court-mandated anger management courses at the drug and alcohol  treatment center where I worked, so I have first-hand knowledge of how  effective they can be when counseling doesn’t seem to work.

​

During  my six years teaching anger management and counseling adjudicated  clients, I came across one dynamic in particular that I have always  found deeply relatable. Aptly named the “victim perpetrator paradigm,”  it is a concept I believe some CF patients may find relatable as well.  This occurs when a former victim, most often a severely traumatized one,  becomes the perpetrator who traumatizes another person (but not  necessarily the person who victimized them) with the intent of inducing  in them the level of hurt they themselves felt when victimized and, in  many cases, continues to feel.

​

If I  want to prevent my pain from sprouting anger, I have to take proactive  measures to prevent it from growing into chronic bitterness and rage,  which triggers a cycle of isolation and even more bitterness. However,  if I do take proactive steps to nip my anger in the bud by initiating  counseling, enrolling in an anger management class, and/or sharing  openly and transparently about my feelings early on, I can mitigate harm  to my loved ones, myself, and at times the community at large.

Mark  Tremblay is 53 years old and has CF. He lives in Troy, NY, with his  wife, MaryGrace. He has a Master of Arts in Psychology from Marywood  University and a Master of Public Administration from Syracuse  University. Mark worked in the New York Governor’s Division of Budget  for six years and 22 years as a Bureau Director for New York State  Department of Health. He is the President of “CF Vests for Life,” which  collects donated therapy vests, nebulizers, and oxygen saturators for  distribution to CF patients around the world. Additionally, he is the  leader of the Attain Health group, “CF Warriors for Recovery and  Freedom.” Mark is also a director of USACFA. His contact information is  on page 2.

This  interview is intended for all individuals with CF, but especially for  those who might need a new liver and kidney. Lifesaving transplants of  both a liver and a kidney at the same time can be successful—never give  up.

​

Jerry  Cahill has been a friend for many years. He’s been running marathons and  biking centuries for as long as I’ve known him. He pushes hard to excel  and, as he likes to put it, “I am relentless.” He knows how to train  his body and mind to accomplish his goals.

​

I was  already transplanted with two lungs by the time I met Jerry. It became  evident he would need a bilateral lung transplant, too. I tried to share  as much about my transplant experience as I could to help him  understand what to anticipate with the surgery and what recovery might  be like. Everyone is different—even though we all have CF, there are so  many variables that come into play.

​

Jerry  proved to be a responsible and compliant owner of his newly transplanted  lungs. So much so, I knew that when he needed to have a liver and a  kidney transplant at the same time, he would be seen as a good  candidate. But there are always other obstacles and I am not on the  surgical committee. Because there is a severe shortage of organs, he was  possibly facing a long wait while feeling sick.

​

I saw  Jerry a few times prior to his recent surgeries and what I saw shocked  me. My athletically active friend was wasting away in front of me and  was the color of yellowing leather. Would he be able to survive the long  wait for these organs? I was not sure he could withstand the rigors of  two surgeries possibly taking 16 hours in total. I thought, I have to  have faith and that if anyone could do it, it would be Jerry.

​

How were you feeling during this time?

About  two years ago, I started to get a lot of itching and started ripping at  my skin. I had marks all over from scratching. I was constantly fatigued  and didn’t feel right. I was already seeing a liver transplant doctor,  recommended by my lung transplant team, because I had abdominal swelling  from my liver. The liver team did an ultrasound of my liver and they  found I had a clot in the portal going into my liver. On top of starting  daily Lovenox injections to break up the clot, I still had a lot of  swelling in my body. The injections didn’t work so I was given oral  anti-coagulants. However, the bleeding got worse and oral medications  were causing major nose bleeds. My fatigue worsened. The portal clot was  not dissolving and I kept swelling. The hepatologist eventually drained  my abdomen to combat the fluid buildup and swelling. All of my existing  symptoms worsened when I was in a car accident. I was rushed to a local  hospital in Westchester. The doctors saw I was bleeding internally so  they quickly transferred me to New York Presbyterian (formerly Columbia)  Hospital. While in the hospital, I then met with the liver transplant  surgeon who, after reviewing my CT scan, said I had to have surgery to  try to stop the internal bleeding. After a week in the hospital  recovering from the first surgery, I had more abdominal pain and was  brought back into surgery again to stem the bleeding. Unfortunately,  they could not fix the portal clot as it was too risky. They felt I  could have bled out.

​

Before  being discharged from the hospital, the oral blood thinner was adjusted.  But, if I scratched my skin, I bled. Things were getting worse. I was  still itchy and I was getting confused and not able to remember things.  The liver team told me I would eventually need a liver transplant.

​

What was your quality of life at this point?

Thankfully  my lung function was very high. And because I never think I am that  sick, I was doing a planned bike tour to raise awareness for CF and  transplant called, “Alive at 65.” The irony was that I was barely alive.  Just before the event, I was retaining a lot of fluid. And I could  barely bike and barely get into the van after biking. The support team  wanted me to stop but I would not stop—I had to finish it. I called my  doctors at New York Presbyterian and they wanted me to come in and get  diuretics.

It was  at this point, even though I didn’t realize it at the time, that my  kidneys were failing, hence the extreme fluid retention and edema. Once I  finished the bike tour or, rather, it almost finished me—my kidneys  were not filtering out toxins, my creatinine was high, and my energy was  low—I was admitted to the hospital. I was immediately moved to a  different floor to undergo dialysis and, at this stage, my memory is  spotty. At one point I woke up in the ICU and was receiving dialysis  24/7. I only have a vague memory of this time. I was blacking out a lot.  Without a functioning liver or kidneys, my body was becoming toxic.  When I was awake and aware after a few days, I was told I would be  needing both a liver and kidney transplant. I had three transplant teams  visiting me in the hospital—my usual lung team plus the addition of  liver and kidney teams. Both organs had to come from the same donor due  to antibodies matching.

​

How did you take this news?

It was  not a shock to hear I might need a new liver and then a kidney. I think I  am strange. I never think I am that sick so I push through and want to  get things done. I barrel through to get past the unpleasantness of  waiting—I just want it over.

When  they told me I would need both organs and the surgery could last 16  hours, I told them that it was ridiculous, and they were looking at me  like I was crazy. They said, “Mr. Cahill, I don’t think you understand  how sick you are.” I replied, “It can’t be that bad. What is my MELD  score?” For those that don’t know, a MELD score (which stands for Model  for End-Stage Liver Disease) ranges from six to 40, with the higher  numbers indicating a more urgent need for a liver transplant. They told  me my score and it was high. They said without these two transplants, I  would not be here anymore. I was told that I would not be going home  without a kidney and a liver. But because I was running a mild fever,  the kidney doctors would not transplant me until I no longer had one.  After 14 days, they wanted to wait six more days to be sure before  activating me on the organ donor list. I was already listed for the  liver. It then became a waiting game.

​

I was  very out of it and it was all during COVID. There were only two visitors  allowed each day. I was finally out of the ICU and on the transplant  floor, receiving dialysis daily through a machine next to my bed. I had  already been in the hospital for six weeks. And I was told it could be a  two-to-three-month wait for both organs.

​

I was  finally activated (by liver and kidney teams) and was put higher on the  list due to needing both organs. I’m claustrophobic in small places like  the MRI tube so I worried and wondered—how would I get through this,  being in a small room for months? I figured I would have to make the  best of it and try to build myself back up the way I did for lung  transplant. But...three hours later…a doctor from the liver team came  in—she said they had found a donor. I was baffled; unsure of what they  meant. The attending explained that the surgeries were happening today!  They came to get me at night and it was dark while I waited by myself  with no visitors allowed.

​

Stuff got real. What were you feeling then?

At this  point, I didn’t think I would survive as I was not in good shape. How  would I make it through a 16-hour surgery? Even before I was listed, I  was told lung transplant recipients are not good candidates for a liver  transplant. They didn’t want to list me, but the liver surgeon who knew  me said that they do not normally do it and I was at risk due to already  being a transplant recipient. He also said, “But because of your mental  state and your willpower, you are so unbelievable we are gonna give it a  shot.”

​

They  wheeled me in and put the liver in first, then they checked to make it  sure it was functioning properly, before the kidney team came and put in  a kidney (but did not remove my own and so I have three!). I was off  intubation after only four hours.

​

Once you knew you were alive, what was going through your mind?

After  surgery, after ICU, and on the transplant floor, a doctor asked me how I  was and I said, “I’m ok.” And I added, “I didn’t think I would get  through it.” The doctor confided, “Honestly, none of us thought you  would!” When I could get out of bed, I started walking a lot in the  hospital. I have learned in life to never obsess over things. I just try  to get them done. I thought back and knew I had a good life and did a  lot of things. I just try to keep reinventing myself and staying  positive. My coping mechanisms are all centered around reinventing  myself.

​

Each  time I had to stop doing something, I tried to find other ways to move  through life. When I could no longer work, I volunteered. When I could  no longer run due to my knees, I started biking. Once that became too  hard, I started hiking and walking. Any exercise is good for one’s mind  and body. So I do what I can to stay healthy. I feel I am good at  pivoting. I began walking in the hospital to build my strength back and  now I am walking to raise money for BEF’s transplant fund.

​

What’s going on now?

I am now  10 months post-liver and kidney transplants. I am not where I want to  be. I look fine and feel good, but my strength is not where I had  envisioned it. I am now 66 and grateful to still be alive. But it’s a  long haul. I am doing freelance work to survive. Living with CF never  goes away. I have two to three appointments each week for different  transplant teams. That’s a fulltime job in itself!

​

It’s not over, the journey with Jerry continues…

Andrea  and Jerry will continue their conversations about life after  transplant. If you have questions for Jerry Cahill, email us at cfroundtable@usacfa.org.

I  am 53 years old and was diagnosed when I was 13 months old. My mom  diagnosed me after looking up my symptoms in Dr. Spock’s book! I had all  the symptoms of CF: failure to thrive, sick all the time, salty skin,  etc. Despite having all the classic symptoms, my doctors concluded that I  did not have CF because we had no family history of it, but my mom  insisted I be tested. As it turns out, I did have relatives on both  sides of the family who died of lung issues, but these issues were not  called CF at the time.

​

I had a  bilateral lung transplant on July 3rd, 1998 and a living kidney  transplant from my friend Kelly on March 7th, 2006. Both transplants  were done at Fairfax Hospital in Falls Church, Virginia. I am very  thankful for my two donors!

​

I worked  full time for the U.S. Government as an Operations Research Analyst,  developing cost estimates for major government systems such as ships and  aircraft. I retired on disability a few years ago because I was having  issues with my speech and memory. I was having a very hard time  remembering numbers and talking about them, which is kind of important  when developing and briefing cost estimates! Now I am glad to not have  the job stress anymore and grateful to have time to take better care of  myself.

​

I spend  my time volunteering for Fairfax Hospital and for my church, although I  did take a break from in-person activities during the pandemic and I  have not been back yet per my doctor’s orders because the pandemic is  still ongoing. I am a Fairfax Hospital Lung Transplant Center Mentor,  speaking to pre- and post-transplant patients about my experiences with  lung transplantation. I am also a Donate Life Ambassador for the  Washington Regional Transplant Community (WRTC), promoting organ, eye,  and tissue donation so that others may have the gift of life that I have  been given. I am married to Scott Adamson and we will celebrate our  25th anniversary in June 2022. We have a furbaby named Penny; she is a  Miniature Schnauzer and brings us joy every day.

I  didn’t write about aging for the last edition of CF Roundtable, even  though I am quite qualified to do so given my grey hairs, because I was  in too much pain to think about writing. So, when I saw this time the  job is to write about pain, I laughed…a tiny bit. Here is what comes  through me regarding pain at this stage of my longer-than-expected life.

​

If you  are in pain, first of all, have compassion for yourself, because  physical pain sucks. No sane person wants to feel pain. In my body,  there is pain in the lower back and down the back of both legs. It seems  to “always” be there to some degree, and it has become the master of my  activities. In other words, unless I prefer that it be excruciating  rather than just super annoying, I cannot do the movements that up until  now have kept my body healthy and my mind relatively sane. Walking is  out. Weight training is out. Any intense form of movement that gets my  heartbeat up seems to be a memory. I don’t know if it will always be  this way. But right now, it is.

​

I’ve  learned through reading and watching videos and podcasts that chronic  pain is understood way better than it was when I trained in medicine. In  fact, pretty much everything I learned is useless, like a DVD player or  something. We now know that with chronic pain (defined as pain that  lasts longer than three months), the brain actually learns to send pain  signals as a response to increasingly smaller stimuli. It develops  supersensitivity to incoming signals over time. This creates a vicious  cycle that, in my opinion, is frankly evil. The end result is that the  pain is always there, even when the body is completely safe and the  brain could totally chill out if it just took a moment to look around.  This is obviously a very brief sketch of a complicated subject. If you  are interested, google “central sensitization.” Get a snack, because you  will be reading for a while.

​

So I  watch. I watch muscles atrophy. I watch stiffness become the new norm. I  am powerless over this. But I’m not special. All bodies decay. All  bodies die, either slowly or suddenly. If I think of this through the  lens of time, it is happening slowly in my body. It seems to me that  looking through this lens of time is what makes it sad to watch. I see  what is the case right now, in terms of what my body could do in the  past. Then I grieve about what is lost and am scared shitless of what  must certainly be my future.

​

But what  is actually here now? A body is sitting in a chair typing. There is  tingling and numbness down the legs, especially the left. Calf muscles  are rigor mortis tight. There is a tiny bit of pain in the left  hamstring, which is relieved if I stretch it a bit. That’s all. That is  what I am aware of right now. My awareness also hears beautiful music  and tastes morning coffee. Awareness looks out the window in front of  this body and sees oak trees and a vineyard in the distance. Birds  flitter by, evading a clear view needed for labeling with a name.  Suddenly, there is silence in the mind and the fingers stop typing. That  silence is so sweet, so free.

​

The  message the pain gives is that it is not me. This sounds esoteric and  unhelpful. It sounds like a really hard thing to grasp, but really it  just takes a moment of reflection. If “I” can sit back and observe this  uncomfortable sensation I call pain, then how can it be me? I am  watching it. If I am watching it, I am not it. Is it there? Yes. Is it  mine? Do I have to own it? No, I do that in a thought. There is a  thought that says, “Hell, yes, it’s my pain; nobody else feels it!” This  very convincing thought is the source of all of my misery.

​

We have  been trained to think this way. It is a conditioned pattern to think  that what my body experiences is me. Sensations in my body experienced  only by me must be mine, or so we believe, but only because it is what  we have always believed. The same is true for thoughts and emotions. It  flows like this: This fear of a future of incapacity is “my” fear.  Nobody else feels this fear. Right there, I know that is not an accurate  thought. Of course other people feel fear. All people feel fear at some  point. I haven’t been singled out. And yet my fear (or any emotion I  feel) is personal and private and, because I am the one experiencing it  so intensely, it singles me out. It is mine.

​

This  claiming of things—sensations, thoughts, feelings—this process of  identifying these things as me or mine is why I suffer. But I can very  firmly say, no, these things belong to nothing. They are simply here  being experienced, but not owned. This is where freedom is found.

​

I can  suffer if I want, but I don’t have to. All I have to do is get better  and better at catching this claiming process by the mind in action and  inquire into it. Is it true that these sensations are me? There will be a  lot of arguing in the mind by the various voices that I have listened  to over the years and believed to hold the truth . Some will be  screaming, “F*%k you and your mindfulness, Julie. This hurts like hell,  and if you don’t f#*king do something about it, it is going to hurt  forever!” Others will be shaking their imaginary heads, saying, “You  have lost your mind. This makes no sense.”

​

I am not  my body or my mind. What I really am is the witness—I can listen,  observe these voices, and firmly understand that these thoughts are not  me. I can say to the pain, “Prove to me that you are me.” Can a  sensation prove its identity? How would that work? In direct experience,  how is it that this painful sensation is mine but the sound of music is  not mine? The taste of coffee is mine but the sound of the woodpecker  on the side of the house is not mine? How? Where is the proof? Without  believing a thought, what is me or mine?

​

All of  these sensations floating around in awareness are just what they are,  sensations. Yes, they are experienced. Yes, they are uncomfortable and  it would be nice if they go away. But not claiming them is much more  comfortable than the alternative, and I don’t have to do it anymore. It  is entirely up to me.

Dr.  Julie Desch is 60 years old and has CF. She lives in San Rafael, CA,  with her partner and their three dogs. She enjoys biking, meditating,  and filling her days with joy.

Pain  is usually associated with physical pain, or at least most people I’ve  asked associate it with physical pain—like in January, when I was  folding laundry and decided to “crack” my own knee, but, in reality, I  dislocated it and then popped it back in myself! The result was  everything but my ACL being torn in my left knee (which is good). No  surgery was needed, but the pain for four months was awful and still, to  this day, the knee does not feel right. But the physical pain wasn’t as  bad as the mental pain. Mental pain always seems to hurt more to me  than the physical. Mentally, I was shot. I hated not being able to take  care of myself or my son the way I always have. I hated having to depend  on others so much for simple tasks.

​

Having  CF can physically be painful—I have coughed so hard that I have  dislocated some of my ribs and needed a visit to my chiropractor to have  them popped back in. Some amazing cryotherapy to soothe the resultant  discomfort afterward was my only relief. I have had costochondritis— the  inflammation of the cartilage adjoining the ribs to the sternum—more  times than I can remember, and I have even gone to the ER for treatment  on several occasions. My joints have the beginning signs of rheumatoid  arthritis; living in Buffalo, NY, makes this challenging because of the  constant weather changes. My sinuses, even after having had two sinus  surgeries, still give me pain during the allergy season. Again, the  frequent weather changes here don’t help. Nonetheless, my mental pain is  always there and always more pronounced than my physical pain.

​

I am in  pain mentally. That may sound super confusing but let me explain. The  fact that my mind never stops gives me pain. The constant worry I  experience makes my heart and mind feel almost as if the pain were  physical. Pain management is important, but we cannot forget to address  the pain of our mind and heart. Having a disease like CF can even  inflict pain on others, and that also weighs heavily on my mind.

​

Our  families have pain and worry because they watch us go to endless doctor  appointments and undergo endless treatments. And I worry about the pain  that may come from watching me “suffer” or die. Now that I am a mom, I  worry about my son potentially growing up without me, or having a mommy  who is too sick to participate. Even after starting Trikafta and being  the healthiest I have ever been, I still worry about all of this. In  addition, I am sure we all feel the pain of survivor’s guilt or the pain  of the cost of medications. I also worry about how much of a pain in  the a$$ I can be toward others—my boss, my friends, or my family—with my  random appointments or issues.

​

Ultimately,  the mental pain of this disease is what hurts the most. I discuss my  pain with my husband and my therapist. I take two anxiety medications to  assist with this as well, and, my gosh, that has helped tremendously. I  wish it were more “normal” to discuss this type of inner suffering.  Mental pain is a real thing, and I believe a lot of people overlook  that. I tend to resort to sarcasm and humor to cover my pain. We all  have different coping mechanisms for our pain and the main thing is to  make sure you seek help for whatever pain you’re experiencing. If you  are physically in pain, please do not just deal with it, and, if  mentally in pain, please reach out to someone who will help you through  it. Pain comes in so many forms and we have so many sources nowadays to  relieve our suffering and get the help we deserve.

Nicole  is 34 years old and has CF. She has been married to her husband Michael  for over six years and they have been together almost 11 years. They  were blessed with their son Ernest within six months of starting  Trikafta. They live in a small farm town near Buffalo, NY. You can email  her at abnormalnicole@gmail.com.

During  the pandemic lockdown of 2020, I was free from the burden of hiding my  cystic fibrosis in public for the first time. In isolation, I no longer  needed to give drawn-out explanations for my CF symptoms or lie to cover  them up. I no longer had to abruptly leave events or college classes to  cough up cups of blood or take care of another symptom. I no longer had  to deal with the embarrassment of being taken out of class in an  ambulance. Among people without CF, I’d always felt like an alien  masquerading as a human. Ironically, right before the lockdown, my worst  symptoms began disappearing because I’d started taking Trikafta. Now  largely healthy and alone, I had all the time in the world to cope with  the past and wonder about who I really was and what it all meant.

Roy is  31 years old and has CF. He lives in Los Angeles, California, and is  passionate about filmmaking that explores issues of trauma and stigma.  He is a recent graduate of the USC School of Cinematic Arts. You can  email him at royberkeley@gmail.com.

Have  you ever been in so much pain that you couldn’t breathe? This was a new  sensation for me and it had nothing to do with my lungs; rather, it was  my gut. I had no idea what was happening when these attacks would come  on suddenly, but they were debilitating and scary.

​

Initially,  the pain was mild but grew in intensity over time, warranting a trip to  the emergency room (ER) of the closest hospital. I was doubled over in  agony, the main source of which seemed to be emanating from my  diaphragm. The pain started slowly and escalated to a cramping type of  pain where it felt like a tree trunk was pushing through my core,  clamping it shut. To work through and ease this severe discomfort, I had  to get on my hands and knees and have someone rub my back while I tried  to sip the air. The person rubbing my back noted that my back felt hot  to the touch.

​

During  these situations, I couldn’t eat or drink anything, including necessary  medications, making a difficult situation if the episodes lasted longer  than six hours. If I did try to drink once the pain eased slightly,  within 10 minutes I started vomiting whatever small gulp of liquid I was  able to get down. After about four hours, I called the transplant  doctor on call at my clinic. They usually tell me to get to an ER to get  some IV fluids, which is typical. Although I’ve been dealing with this  situation for several years, I have seen it progress to the point of a  hospital admit.

​

The  random nature of these episodes was the scariest thing. I tried to note  what was setting my system off each time. What can I avoid eating or  doing so I can live preventatively? The answer, as it turns out, was  more complicated.

​

All of  this started in 2006, about a year after I had my gallbladder removed. I  was told that eating fatty foods (ribs, fried foods, etc.) would be  problematic without a gallbladder. I didn’t eat many of those items and,  when I did, I took more enzymes. I couldn’t discern the pattern—after  eating ribs I was fine, but there were a few times when I noticed that  eating smoked fish on a bagel with cream cheese seemed to set off these  painful episodes.

​

On one  occasion, while visiting my mom in Hampton Bays, I was on IV antibiotics  and the doctor on call in NYC told me to go get hydrated. When I  arrived at the local ER to get fluids, my bloodwork indicated extremely  elevated liver enzymes, so they kept me overnight and admitted me. They  did an emergency abdominal CT plus a sonogram of my liver. They were  concerned I had a blockage in my biliary tree (the gallbladder, liver,  and bile ducts, collectively). I told them no, that whenever I vomit for  several hours (and I had been vomiting for close to five hours  already), my liver numbers rise. It was so frustrating. I know my body  and this was par for the course. By then, I was hydrated and no longer  vomiting, thankfully. But if I left, I was signing out “against medical  advice” (AMA), which meant insurance might not have covered my visit.

​

Because I  was on IV antibiotics and the hospital didn’t have that particular  antibiotic in their formulary, I was put on a different one, which made  me nauseated from the change midway through the course of antibiotics.  Sadly, this happened on a Saturday and the attending clinician in the  hospital claimed that I needed to stay until Monday so the surgeon could  read the CT scan and see whether an operation was advised. So I stayed.  There was no blockage, no stone or biliary duct problem, and my blood  work returned to normal.

​

I was  still no closer to understanding what was happening. There were several  incidents after that admission, and I was becoming fearful of eating  certain foods as I didn’t want to end up in the hospital again. My  transplant doctor ran every GI test under the sun—a colonoscopy, a  stomach-emptying test, a nuclear test, and probably some I cannot  recall. I had an episode happen while I was playing tennis, fortuitously  with an opera singer. She saw I was distressed and coached me through  breath work to get through it. It was amazing—I was able to relax my  diaphragm and the pain slowly dissipated. I was convinced I had  pancreatitis, but my transplant doctor said that doesn’t go away with  breathing or just spontaneously within a day.

After a  few times, I was able to breathe through it, especially if no one was  around to rub my back when I was prone. I tried breathing like an opera  singer and sometimes it worked, but not consistently.

​

The last  time it happened was a disaster. I went into full pain and vomiting  mode. I couldn’t keep any fluids down and therefore couldn’t take my  immunosuppressants. I was slumped over in agony. This time I was in NYC,  so I went to my clinic’s hospital where I lay in the ER for 10 hours  with no medical intervention or attention other than IV Dilaudid for  pain. My blood work didn’t have the markers for pancreatitis, even  though I was certain I had that at this point. I was given a urine cup  and my pee was brown. Since no one was doing anything in the ER, my  husband and I decided I could cope with this at home, so I left. I was  still in pain and was in bed for two days. Despite being thoroughly  drained and exhausted, I could drink little sips of water, so I started  taking my meds. I called my transplant coordinator who thought I was  still in the ER! I told her what happened and said there was no way I  was going back to that horrendous place—either admit me to the  transplant floor or I am “checking out.” I cannot live this way. She got  me in but first I had to drink a bunch of liquid contrast to get a GI  CT of my abdomen.

​

I was  admitted to the transplant unit and I had never been so happy to be in  the hospital. There they immediately realized I had a full-blown urinary  tract infection so was put on IV antibiotics for that as well as a  constant IV saline drip. I was seen by a lovely GI doctor who told me I  didn’t have pancreatitis but they didn’t know what was wrong—yet.  Nevertheless, the interns treated me like I did have pancreatitis. I was  in the hospital for 10 days! I was put on a low-fat diet for  pancreatitis! It was crazy. I back slid a few times into pain, even  though I was given opiates but was afraid to take them. I was reassured I  was not going to get addicted.

​

I left  the hospital feeling better but had lost about 10 pounds and was  emotionally and physically decimated by the whole affair. I was supposed  to remain on a low-fat diet at home and I did. I was to go back to see  the GI doctor once out of “Club Med.” It was only at his office that the  doctor and I had a heart to heart and he gave me a magnificent little  blue pill which was an anti-spasmodic. I was told to only use it when I  was trying to ward off an “attack.” We decided my stomach was spasming  from an unknown trigger.

​

The  minute I start feeling twinges of gut tightening, I take the blue pill.  Sometimes I don’t quite catch it in time. But amazingly, after popping  it, the pill works in about five minutes. I haven’t been hospitalized  for this since 2018 and I make sure I always have those pills with me.

I am no  longer living in fear of ending up in the nearest ER. It was a long road  to discovery. I am grateful to have the tools, a.k.a. my  anti-spasmodic, along for the ride for the rest of my life.

Andrea  Eisenman is 57 and has CF. She recently realized that her initials are  AGED: Andrea Gail Eisenman Downey (her husband’s surname)! She lives in  New York, NY, with her husband Steve and dogs, Willie and Roscoe. Andrea  is the Executive Editor for USACFA. She enjoys cooking new recipes,  playing pickle ball, biking, tennis when possible, and staying active as  her health allows. Her contact information is on page 2.

Here  I was, 51 years old, living with CF, post-menopause and divorced. After  going through a 13-year emotionally and mentally abusive marriage, I  was happily on my own again. But who was I? Who did I want to be in my  50s? Was I ready to date again? How would someone take the news when I  had to tell them I have CF? When I was dating almost 20 years ago, it  was very different and I did not know I had CF. I was 37 years old when I  was diagnosed. Going out and meeting someone then was the norm, but now  I had to learn a whole new way of dating. Scary! After my divorce, it  took four years to heal, learn, reflect, and grow until I felt I was  ready for the dating scene. I had heard horror stories about online  dating from friends and colleagues, and here I was about to start my own  journey.

​

So the  first thing I did was talk to all my friends about which sites I should  use. The recommendations were overwhelming—go on Match.com, try  eHarmony, or see if you like Bumble. Ugh! So many choices. I settled on  Match.com and quickly had to learn about scamming. Thankfully, my  closest friend was very familiar with this and taught me all the signs  to look for when a “fake” person was texting me. I quickly learned how  to block people. Some would think this factor alone would be enough to  scare you away from dating, but I was determined to get my dating life  back. So I decided I was going to face all of this with a sense of  humor. Scammers? So, what! Terrible dates with rude people? Yes, but  there are some hysterical stories.

​

As the  months ticked by, I was not meeting anyone who interested me on  Match.com, so I turned to eHarmony. This proved to be another dead-end,  so I tried other dating apps like Bumble and Hinge, only to be equally  disappointed.

As a  year was approaching with online dating and all I had were funny  stories, my CFReSHC friends urged me to try Plenty of Fish (POF). I  thought this was a hook-up site but, after their stories of how they met  their significant others, I was intrigued.

After a  week on POF a cute message appeared. After looking at his profile I  thought we had lots in common, so I messaged him back. We did the  obligatory texts for a day and then he called me. OMG what a sexy voice,  I thought. We talked for a while and he asked if he could call me  tomorrow. Yes! And so it continued every day leading up to our first  date. I felt like I knew him already and couldn’t wait to meet him in  person.

​

Being as  this was the height of the pandemic, I knew I was taking a risk but I  just couldn’t pass this up and I’m so glad I didn’t. On our very first  date I decided to reveal my CF to him. There was something very special  about him, so I wanted him to know about my CF. I was nervous, wondering  if he would end things but knew I had to tell him. I soon found out  that he was a truly wonderful, caring person and had no intention of  ending our relationship based on my CF. In all the previous dates I went  on in the past year I did not reveal my CF. I knew these relationships  were not going anywhere so I felt it was my choice not to tell. There  were times I actually enjoyed being on a date and the person not knowing  about my illness—it  was a mini vacation from CF. But this time it was  different. We are still together and our relationship continues to grow  stronger. I am so glad I did not let age, abuse, or CF stop me from  meeting a wonderful person.

Jennifer  Kyle is 57 years old, has CF and lives in Somerset, NJ. She was  diagnosed at 37. Jennifer was a health, physical education, and dance  educator for 16 years in the New Jersey public schools. For four years  she served as an adjunct professor for Montclair State University,  served as President for the New Jersey Association for Health, Physical  Education, Recreation and Dance (now known as SHAPE NJ), and served as  the VP of Dance for the Eastern District Association for the national  SHAPE organization. Since retiring on disability, she runs a dog-sitting  business and has helped organize BreathCons, ResearchCons, MiniCons for  the CFF. She has also been a peer mentor for the CFF. Jennifer  currently serves on the Governance Board for CFReSHC as the Meeting  Coordinator and is working as a patient advisor for an outreach study  with the CFF for improving the collection of spirometry results for  telehealth appointments. She has also participated in five clinical  trials and hopes to be a part of many more.

https://www.njsocf.org/

Pharmaceutical Services For Adults With Cystic Fibrosis

Since 1990, the New Jersey State Organization of Cystic Fibrosis has administered a
special state-funded program, “Pharmaceutical Services for Adults with Cystic Fibrosis.”

​

As more  and more individuals with CF reach adulthood, they face increasing  financial burdens and difficulty financing the high costs of living with  cystic fibrosis. In response to the increasing needs of adults with CF,  NJSOCF developed a plan for an adult program, now funded by the State  of New Jersey.

​

Services  are available to New Jersey residents with CF, age 18 and over, whose  incomes meet state eligibility requirements. Direct service components  of the program include assistance with paying for prescription  medication; office visits and diagnostic copayments; home IV  out-of-pocket expenses; medical equipment and supplies; nutritional  supplements; and extra nutritious food. The program will also pay a  health insurance deductible. Adults from every county in New Jersey are  currently enrolled in the adult program and new applicants are always  welcome to apply.

​

1. Diagnosis of Cystic Fibrosis verified by CF doctor

2. Must be a New Jersey resident

3. Must be 18 years or older

4. Individual annual income less than $51,952/year

Fill out an application here: https://www.njsocf.org/adult-program/

Or you can also e-mail them at das@njsocf.org or dtimr@njsocf.org

Once you are accepted into the grant the following will be covered:

• Copayments for prescription medications related to the treatment of cystic fibrosis

• Copayments for office visits related to the treatment of cystic fibrosis

•  Copayments for diagnostic testing, i.e., routine lab work, scans, x-rays  and sputum cultures, related to the treatment of cystic fibrosis

• Out-of-pocket expenses for home IV antibiotics

• Copayments for respiratory equipment and supplies

• Insurance deductibles up to a maximum of $1,000 per year

You will also receive $125 each month for food and nutritional supplements. Here are the requirements:

• Shopping should be done every month for approved foods and/or nutritional supplements

• You  must send back your itemized grocery receipt(s) each month in the  postage paid envelope provided.  You will not get another card until a  receipt is received.

Please  note that only nutritious and non-taxable food items should be  purchased.   If for some reason you cannot shop in any one month, please  call or email us.

EXAMPLES OF UNACCEPTABLE PURCHASES

Soda and non-nutritious drinks

Coffee and regular tea

Spices and condiments

All candy

ALL TAXABLE ITEMS ARE UNACCEPTABLE

Paper and plastic products

Laundry products

Cleaning products

Grooming and cosmetic aides

EXAMPLES OF ACCEPTABLE NUTRITIONAL SUPPLEMENTS

Vitamins

Boost

Ensure products

Carnation Instant Breakfast

Glucerna

Scandishake

Power bars and equivalents

Weight gain supplements

Lupin Gets Tentative Approval For Cystic Fibrosis Drug

The drug  company announced that it has received tentative approval from the US  Food & Drug Administration (USFDA) for its Abbreviated New Drug  Application (ANDA) Ivacaftor tablets, 150 mg. Ivacaftor tablet is a  generic equivalent of Kalydeco tablets manufactured by Vertex  Pharmaceuticals. These tablets will be manufactured at Lupin’s Nagpur  facility in India.

https://tinyurl.com/vhmbfjup

New Research Could Prevent Hearing Loss For 50% Of People With Cystic Fibrosis

People  with cystic fibrosis are prone to recurring lung infections which need  to be treated with aminoglycoside antibiotics. Aminoglycoside  antibiotics are very effective against life threatening infections and  are associated with low rates of antibiotic resistance; however, they  can also cause hearing loss. Researchers estimate that it may be 50% of  adults with the condition. Aminoglycosides enter and kill the sensory  hair cells in the inner ear that are vital for hearing. Researchers are  developing new aminoglycosides that aren’t able to get into hair cells,  but still retain the ability to kill bacteria, thus making them less  toxic to hearing. By the end of three years the researchers hope to have  at least three new aminoglycosides that can be moved towards clinical  testing.

https://tinyurl.com/yu37krkn

AND

https://tinyurl.com/yuuaex85

AND

https://tinyurl.com/yuuaex85

Pilot RCT Of A Telehealth Intervention To Reduce Symptoms Of Depression And Anxiety In Adults With Cystic Fibrosis

Adults  with cystic fibrosis (awCF) have higher levels of depression and anxiety  than community samples. The Coping and Learning to Manage Stress with  CF (CALM) intervention was developed for awCF reporting elevated  symptoms of depression or anxiety. In this pilot study, awCF were  randomly assigned to either six telehealth sessions (CALM; n = 15) or  treatment-as-usual (TAU; n = Primary outcomes were depression and  anxiety. Secondary outcomes were coping self-efficacy and health-related  quality of life (HrQOL). Tertiary outcomes were feasibility,  acceptability, and satisfaction. Assessments were completed at baseline,  post-intervention, and 3-month follow-up. At post-intervention, the  CALM group had a lower mean score than the TAU group for depression and  anxiety. The CALM group had higher (i.e., better) mean scores than the  TAU group for coping and HrQOL domains of Social Functioning and  Vitality. Most treatment gains were not sustained at 3-month follow-up.  The researchers concluded that CALM shows promise as an intervention to  reduce symptoms of depression and anxiety and improve coping and HrQOL.

https://tinyurl.com/2a8cbbdp

The Impact Of Cystic Fibrosis On The Working Life Of Patients: A Systematic Review

This  review aimed to address the impact of CF on the occupational functioning  of patients. A significant proportion of patients were reported to  retain a job on a full- or part-time schedule. Less physically demanding  occupations were most frequently performed, perhaps due to CF-related  inability to sustain a heavy workload. Disease severity parameters  (e.g., lung function measurements, or personal, psycho-social, or  economic conditions) have been reported as determinant or co-determinant  factors for the development of work-related disability. Although  further research is necessary, these results may be useful to inform  interdisciplinary CF healthcare management, including the assessment of  work function, and to define career counseling plans and workplace risk  assessment and management strategies to support the personal, social and  professional lives of patients.

https://tinyurl.com/2ad5okpt

Flares, Poorer Lung Health Can Follow Becoming A Parent With CF

People  with mild-to-moderate cystic fibrosis commonly experience a decline in  lung function and an uptick in exacerbations shortly after becoming  parents, a study reports. The use of CFTR modulator therapy can lessen  the negative health impacts of newfound parenthood, findings also  suggest. With available care, people with CF are living longer than was  once possible, and more and more patients are choosing to become  parents. The researchers looked at changes in lung health by assessing  percent predicted forced expiratory volume in one second (ppFEV1). They  also assessed the frequency of exacerbations, or times when symptoms  suddenly worsen, by looking at times when patients were treated with  intravenous (IV) antibiotics. Changes in patients’ body mass index (BMI)  were also evaluated. Results indicated that lung function decreased  significantly following the birth of a child. On average, ppFEV1 lowered  by 3.19% from the year before to the year after a child’s birth. BMI  also decreased while the frequency of exacerbations significantly  increased by about 30%. The researchers reported that among people with  CF with mild to moderate disease, parenthood adversely impacted health  outcomes in the year following the birth of a child compared to the year  before birth. Analyses based on sex showed a significant decline in BMI  among women but not men. Rates of ppFEV1 decline or exacerbations did  not significantly differ based on sex. Older age was not associated with  any of the outcomes analyzed. Declines in patients’ health might be  attributed to the distractions of parenthood, from “sleep deprivation”  to less time for self-care. Statistical analyses showed that, after  adjusting for age and sex, patients using a CFTR modulator actually  experienced a slight increase in ppFEV1 after becoming parents, in  contrast to the significant decline seen in those not using these  treatments. Modulator therapy did not significantly affect BMI or  exacerbation rates.

https://tinyurl.com/222ab5r5

The Negative Impact Of Chronic Rhinosinusitis On The Health-Related Quality Of Life Among Adult Patients With Cystic Fibrosis

With  improved survival in cystic fibrosis patients, it is crucial to evaluate  the impact of chronic co-morbidities such as chronic rhinosinusitis  (CRS). The objectives were 1) To determine the prevalence of CRS with a  large series of CF patients 2) To evaluate the impact of CRS on the  Health-Related Quality of Life (HRQoL) of CF patients and 3) To compare  CRS-specific, CF-specific and general HRQoL instruments. CRS patients  reported significantly lower HRQoL with higher Nasal Outcome Test  (SNOT-22) scores and lower scores in the respiratory domain of Cystic  Fibrosis Questionnaire-Revised (CFQ-R) and physical health domains of  Cystic Fibrosis Quality of Life Evaluative Self-administered Test  (CF-QUEST) and Short Form Survey (SF-36). The physical and mental  domains of SF-36 and CF-QUEST had a strong correlation with SNOT-22.  Higher scores of SNOT-22 nasal subdomains correlated with lower scores  of SF-36, CFQ-R and CF-QUEST. Thus, CRS is a prevalent co-morbidity of  CF patients, which significantly reduces HRQoL. SNOT-22, CFQ-R, CF-QUEST  and SF-36 were strongly correlated. Severity of sinonasal symptoms have  a strong correlation with HRQoL in CF patients.

https://tinyurl.com/53defjfp

AND

https://tinyurl.com/2h6x4wyt

Cystic Fibrosis-Related Diabetes (CFRD) And Cognitive Function In Adults With Cystic Fibrosis

Diabetes  is known to cause changes in brain structure and long-term cognitive  dysfunction. This work investigated cystic fibrosis-related diabetes  (CFRD) as a mechanism for cognitive impairment in people with CF. It was  hypothesised that cognition would be poorer in adults with CFRD than in  those with CF without diabetes (CFND) or in healthy controls. Cognitive  performance was assessed using the Cambridge Neuropsychological Test  Automated Battery which provides a comprehensive cognitive assessment  with tests mapping onto specific brain regions. Demographic, clinical  and self-reported health data were documented for all participants. CF  specific clinical variables were recorded for the two CF groups. People  with CF demonstrated deficits in aspects of verbal and spatial memory,  processing speed and cognitive flexibility compared with healthy  controls, with all areas of the brain implicated. Those with CFRD had  additional difficulties with higher-level processes known collectively  as ‘executive function’, which demand greater cognitive load and recruit  the prefrontal cortex. Compared with healthy controls, those with CFND  and CFRD had an estimated 20% and up to 40% reduction in processing  speed respectively. Managing CF requires higher order executive  function. Impairments may be sufficient to interfere with self-care and  the ability to perform everyday tasks efficiently.

https://tinyurl.com/27j3mvsu

Indoor Air Pollution Exposure Is Associated With Greater Morbidity In Cystic Fibrosis

Exposure  to higher levels of outdoor air pollution is associated with worse lung  function and greater rates of pulmonary exacerbations in CF, but  limited data on exposure to indoor air pollution exists. Individuals  with cystic fibrosis who were enrolled in the Twin and Sibling Study  self-reported exposure to four known sources of indoor air pollution  (secondhand smoke, forced hot air, wood stove and fireplace). Change in  lung function, rates of hospitalizations and pulmonary exacerbations  were followed over 4 years to compare outcomes in those who were exposed  to those who were not exposed. Adults exposed to secondhand smoke had  42% increased yearly risk of hospitalization compared to those adults  who were not exposed. Questionnaire-based data suggest that exposure to  sources of indoor air pollution increase morbidity in both the pediatric  and adult cystic fibrosis populations. Future studies with qualitative  indoor air pollution measurements are needed to further quantify  exposure risks for the CF population.

https://tinyurl.com/8bh2vf8k

CFF Grants $1.6M To Study Lung Transplant Complications

The  Cystic Fibrosis Foundation (CFF) has awarded $1.6 million to support  research focused on identifying biomarkers of chronic lung allograft  dysfunction (CLAD)—a complication of lung transplants—in people with  cystic fibrosis. With this research, the CFF hopes that CLAD diagnoses  can be made sooner, leading to improved prognoses in the nearly 50% of  transplant recipients who experience the condition in the first five  years after transplant. CLAD encompasses a range of complications that  occur when one’s body rejects a transplant, leading to an inability of  the transplanted lungs to function normally. It is the most common  complication leading to death in transplant recipients, and strategies  to sooner detect it are greatly needed. One of the funded projects will  explore the relationship between transplant rejection and digestive  reflux, a condition characterized by indigestion (so-called heartburn),  chest pain, and swallowing difficulties. To do so, the researchers will  examine the bile acids—digestive fluids made in the liver—found in the  lungs of transplant recipients. In collaboration with the Cleveland  Clinic, CFF launched the CF Lung Transplant Consortium (CFLTC) patient  registry and biorepository last year. The project’s goal is to collect  clinical data and biological samples to aid scientists in better  understanding CLAD and other transplant outcomes.

https://tinyurl.com/mrxx9utw

Sustained Effectiveness Of Elexacaftor-Tezacaftor-Ivacaftor In Lung Transplant Candidates With Cystic Fibrosis

Elexacaftor-tezacaftor-ivacaftor  induces rapid clinical improvement in patients with cystic fibrosis  (CF) and advanced pulmonary disease, often leading to suspend the  indication for lung transplantation. Yet no long-term data is available  in lung transplant candidates. The authors found that in lung transplant  candidates eligible for elexacaftor-tezacaftor-ivacaftor, the rapid  improvement following initiation of treatment persisted over one year  with a reduction in treatment burden and lung transplantation could be  safely deferred in most patients.

https://tinyurl.com/2nhsr3ve

Outcomes Following Lung Re-Transplantation In Patients With Cystic Fibrosis

Compared  to their initial transplant, CF patients experience significant  clinical decline in renal, cardiac, and pulmonary function at the time  of lung retransplantation. This may indicate that an earlier evaluation  and rehabilitation process may be necessary to identify patients earlier  for lung retransplantation prior significant clinical decline.

https://tinyurl.com/3vc5xcsm

Low Body Mass Index As A Barrier To Lung Transplant In Cystic Fibrosis

Patients  with advanced CF lung disease and BMI ≤ 17 kg/m2 are less likely to be  listed for lung transplant and have a higher risk of dying without  listing, compared to those with higher BMI. Regional differences suggest  access to transplant for malnourished CF patients may be limited by  location.

https://tinyurl.com/wtwxjh34

Survival Improved For Patients With Cystic Fibrosis With BMI Recovery Post-Lung Transplant

In  patients with cystic fibrosis, lower preoperative BMI was linked to  lower likelihood for BMI recovery within 1 year of lung transplant, but  for those who achieved BMI recovery within 1 year after transplant  survival improved. Poor nutritional status as measured by BMI is an  independent risk factor for pre-transplant death in advanced cystic  fibrosis lung disease and low BMI is an indication for early referral to  a lung transplant center. Thus, having a low preoperative BMI is a risk  factor for poor outcomes after lung transplant, but this study  underscores the importance of attention to BMI recovery posttransplant.  Future research should investigate whether approaches to augment weight  gain posttransplant improve outcomes, particularly among recipients with  very low BMI at the time of transplant.

https://tinyurl.com/37v6f2x3

CF Patients With CFRD Treated With Lung-Pancreatic Cell Transplant

A lung  and pancreatic cell transplant from a single donor to people with  end-stage cystic fibrosis and CF-related diabetes (CFRD) safely and  effectively improved their lung function, metabolic control, and quality  of life. Given that CFRD increases the risk of complications after a  lung transplant, these findings suggest that a combined lung-pancreatic  cell transplant may be an effective way of improving life for this group  of end-stage CF patients. CFRD is associated with poorer lung function,  poor nutritional status (slower growth and/or weight loss), and a  greater risk of death. In addition, for CF patients undergoing a lung  transplant, its presence decreases patient survival, while promoting  infection and post-transplant pulmonary rejection. Restoring  satisfactory glucose control through a transplant of pancreatic  islets—groups of cells that produce insulin and other hormones—may help  improve outcomes of a lung transplant in CF patients with CFRD. Combined  lung-pancreas cell transplantation, however, is technically more  complex, carrying a higher risk of complications due to the simultaneous  thoracic and abdominal procedures in already very weak patients.  Pancreatic islets can be successfully grown and maintained in the lab  for up to 10 days, and transplanted into the liver through the portal  vein. This allows implantation to be delayed until the patient’s  condition has improved. To date, only case reports of combined  lung-pancreatic islets have been published, and questions remain as to  the efficacy and safety of this procedure in this particular patient  population. Researchers launched a Phase 1/2 clinical trial, called PIM  (NCT01548729), to assess the feasibility and effectiveness of combined  double lung-pancreatic islet transplant from a single donor in end-stage  CF patients with CFRD and on insulin therapy. Using the same donor for a  combined transplant is thought to reduce the risk of immune responses  against the transplanted tissues. The trial’s main goal was to assess  the combined transplant’s metabolic efficacy, measured by a composite  score including metabolic and nutritional parameters. A transplant was  defined as successful if, at one year, three of the following four  criteria were met: weight increase of at least 5%; fasting blood-sugar  levels lower than 110 mg/dL; a 30% or greater reduction in insulin  requirements; and at least a 0.5% drop in HbA1c. Secondary goals  included changes in these individual measures: insulin production, lung  function, and health-related quality of life, as well as safety  assessments. 70% of the patients had a successful combined transplant.  They showed an increase in body-mass index (BMI, a ratio between weight  and height), better control of blood-sugar levels, and a 38% drop in  daily insulin doses. One person was able to stop taking insulin.  Successful lung-pancreatic islet transplant was also associated with  better lung function and gains in health-related quality of life,  particularly its physical aspects. No one died or rejected their  transplanted lungs during the follow-up year, and no complications  related to the pancreatic islet injection procedure were reported. The  safety profile of the combined transplant was similar to that of a lung  transplant alone, with lung infection as the most common complication  and no reports of unexpected adverse events. All serious adverse events,  most commonly occurring within the first month post-transplant, were  successfully treated. These findings indicate that this is an efficient  and viable therapeutic option for patients with end-stage CF and CFRD.

https://tinyurl.com/29dpv4gs

AND

https://tinyurl.com/ynj3xamc

High Levels Of DMBT1 Protein In Lungs May Mark CF Progression

Elevated  levels of DMBT1 in the lungs, a protein previously linked with  inflammatory processes, may be a biomarker of progression in cystic  fibrosis. High DMBT1 levels impaired the movement of cilia, the  finger-like projections that help to clear mucus from the airways. DMBT1  is a protein found in several lung cells, including alveolar type II  cells that play key roles in lung function, including the regeneration  of the epithelium—which lines the respiratory tract—following injury. In  healthy lungs, DMBT1 is found at low to moderate levels that rise  during inflammation and with bacterial and viral infections. Besides its  role in inflammation, DMBT1 promotes blood vessel formation and  epithelial cell maturation, highlighting its role in tissue repair.  Researchers examined post-mortem lung tissue from a man with CF and  tissues from CF patients who underwent a lung transplant. Compared to  samples from people without lung disease, those from CF patients had  markedly higher levels of the DMBT1 protein. Results showed that DMBT1  expression was upregulated in CF which is in line with the known  functions of DMBT1 during inflammation.

https://tinyurl.com/nhzbtr92

Association Between Elevated Peripheral Blood Eosinophil Count And Respiratory Outcomes In Adults With Cystic Fibrosis

Elevated  blood eosinophil counts are linked to worse outcomes in asthma and  COPD, but have yet to be well characterized in CF. scientists  hypothesized that higher stable visit blood eosinophil counts are  associated with increased rates of lung function decline and pulmonary  exacerbations (PEx). A retrospective analysis of adult CF patients  enrolled in a prospective cohort study focused on blood biomarkers was  performed. Those with high eosinophil counts experienced increased  respiratory symptoms, but the rates of lung function decline and PEx  were comparable between the group with high eosinophil counts and the  one with low levels.

https://tinyurl.com/2p993j43

Home Monitoring In CF May Help Detect Pulmonary Exacerbations

Home  monitoring with a mobile phone-linked spirometry device may provide an  effective way of detecting pulmonary exacerbations in people with cystic  fibrosis. The findings showed more pulmonary exacerbations were  identified through home monitoring compared with standard CF care. The  use of home monitoring was generally well-accepted by patients, for whom  it gave a sense of control and independence. Several factors have been  identified that can predict whether a patient is more likely to fail to  return to baseline after an exacerbation. These include being female,  infection with bacteria such as Pseudomonas aeruginosa and being  malnourished. Another predictive factor is a large decrease, before the  start of treatment, in FEV1. According to the researchers, since  symptoms often gradually worsen during an exacerbation, home monitoring  would allow faster treatment initiation and possibly improve patient  outcomes. Researchers hypothesized that home-monitored patients would  need fewer days hospitalized and have a better health-related quality of  life (HRQoL) compared with patients receiving routine CF care. In this  pilot study (NCT02994706) adults with CF were randomly assigned to the  home monitoring intervention or to standard care. Home-monitored  patients were provided with a Bluetooth-enabled digital spirometer and a  mobile phone, and instructed to examine their health status twice a  week during the yearlong study period. Participants were required to  record their FEV1 results using the digital spirometer. Their symptoms  also were recorded with the use of the Cystic Fibrosis Respiratory  Symptom Diary – Chronic Respiratory Infection Symptom Score  (CFRSD-CRISS). A drop in FEV1 of 10% or more from baseline and/or a  total score of the CFRSD-CRISS worsening by more than 10  points  automatically triggered an alert to the participant’s care team. The  team would then discuss the symptoms with the patient and decide on the  best approach. Secondary outcome goals of the trial included assessments  of antibiotic requirements, measured by the number of days on oral and  intravenous antibiotics, and the identification of protocol-defined  pulmonary exacerbations. This was described as the presence of four or  more specific criteria, including a change in sputum, increased  coughing, increased shortness of breath, fatigue, and fever, and changes  in lung function. The mean number of hospital inpatient days was  similar for patients in the home monitoring intervention group and for  those receiving standard care. To determine HRQoL, the team used the  Cystic Fibrosis Questionnaire-Revised (CFQ-R). No differences in HRQoL  were found between the two groups. Protocol-defined pulmonary  exacerbations were detected more often in home-monitored patients during  the study period. Participants in the home-monitored group also  received oral antibiotics for protocol-defined pulmonary exacerbations  for longer compared with patients receiving routine care. However, no  differences in FEV1 over the 12-month study period were found. A  qualitative analysis showed that most patients felt that home monitoring  was beneficial. The findings of this trial confirm that home monitoring  is effective in detecting pulmonary exacerbations in adults with CF.

https://tinyurl.com/547rbm83

Method To Detect Cystic Fibrosis Infection Within Minutes

The  present methods for diagnosing acute and chronic infections are complex  and time-A multi-disciplinary team set out to develop a diagnostic tool  that would be rapid, accurate and simple-to-use for doctors. The  multi-excitation Raman spectroscopy is the analysis technique produced;  it’s a non-invasive method that emits a scattering of multiple colours  of light into a patients sample. When light is applied to a sample’s  molecules they can vibrate which helps researchers understand their  characteristics. By using different colours of light, a different set of  such vibrations can be triggered, meaning more information can be  obtained about their composition than previously possible. This then  allows ‘finger-printing’ that can be used to identify the properties of  the pathogens. In many current techniques, a reagent needs to be added  to a sample or a tag needs to be attached to the molecules of interest  to analyse their composition. This is not required under this new  approach which uses natural properties of the molecules to analyse them.  This new Raman spectroscopy based method offers many advantages over  resource-intensive, culture-based methods, allowing rapid and label-free  analysis. It avoids complex sample-preparation steps with sophisticated  equipment.

https://tinyurl.com/yzxtune9

AND

https://tinyurl.com/5n6jptmf

AND

https://tinyurl.com/yburjx49

AND

https://tinyurl.com/2h77fva5

Effectiveness Of Antibiotics Significantly Reduced When Multiple Bugs Present

In the  study researchers say that even a low level of one type of microbe in  the airways can have a profound effect on the way other microbes respond  to antibiotics. The results highlight the need to consider the  interaction between different species of microbe when treating  infections with antibiotics--and to adjust dosage accordingly. Chronic  bacterial infections such as those in the human airways are very  difficult to cure using antibiotics. Although these types of infection  are often associated with a single pathogenic species, the infection  site is frequently co-colonised by a number of other microbes, most of  which are not usually pathogenic in their own right. Treatment options  usually revolve around targeting the pathogen, and take little account  of the co-habiting species. However, these treatments often fail to  resolve the infection. The model allowed them to grow a mixture of  different microbes, including pathogens, in a stable way for weeks at a  time. This is novel, because usually one pathogen will outgrow the  others very quickly and spoil the experiment. It enabled the researchers  to replicate and study infections with multiple species of microbe,  called ‘poly-microbial infections’, in the laboratory. The three  microbes used in the experiment were the bacteria Pseudomonas aeruginosa  and Staphylococcus aureus, and the fungus Candida albicans. The  researchers treated this microbial mix with colistin, which is very  effective in killing Pseudomonas aeruginosa. But when the other  pathogens were present alongside Pseudomonas aeruginosa, the antibiotic  didn’t work. The same effect happened when the microbial mix was treated  with fusidic acid -- an antibiotic that specifically targets  Staphylococcus aureus, and with fluconazole -an antibiotic that  specifically targets Candida albicans. The researchers found that  significantly higher doses of each antibiotic were needed to kill  bacteria when it was part of poly-microbial infection, compared to when  no other pathogens were present. At present antibiotics are usually only  laboratory tested against the main pathogen they are designed to  target, to determine the lowest effective dose. But when the same dose  is used to treat infection in a person it often doesn’t work, and this  study helps to explain why.

https://tinyurl.com/4c5v2nte

AND

https://tinyurl.com/ytaaytet

AND

https://tinyurl.com/2p8c2wph

Bacteria Boosts Anti-fungal Medicine’s Killing Power In CF Study

Co-infection  with the bacterium Pseudomonas aeruginosa can increase the potency of  therapies that kill Candida albicans, an infectious fungus, according to  a new study. Little is known about how co-occurring infections affect  the response to treatment. Scientists conducted tests using fluconazole  (FLC), an anti-fungal medication that is commonly used to manage Candida  infections. In lab dish experiments, the researchers used FLC to treat  Candida fungi, either alone or in the presence of Pseudomonas. When  Candida was grown with Pseudomonas in the absence of treatment, the  bacteria had little to no effect on the fungus’ growth. Treatment of  Candida alone with FLC was fungistatic, but not fungicidal — that is, it  stopped the fungus from growing, but did not kill it. However, when FLC  treatment was given in the presence of Pseudomonas, the medication  powerfully killed the fungus. Thus, fungal-bacterial interactions can  drive an unexpected enhancement in antifungal susceptibility during  treatment of infection. The fungicidal effect of FLC during co-infection  suggests that Pseudomonas blocks C. albicans tolerance to FLC, leading  to death rather than persistence or slow growth during treatment.

Research  showed this effect may be driven in part by the two microbes competing  for iron, a nutrient that both Pseudomonas and Candida require to  survive. Supplementing the microorganisms with iron reduced, but did not  eliminate, Pseudomonas and FLC’s synergistic anti-fungal effect. This  work demonstrates that polymicrobial interactions can indeed affect  treatment efficacy and, most importantly, it highlights the importance  of nutrient availability in the environment — such as iron — and how it  modulates treatment efficacy.

https://tinyurl.com/ymrz8cb8

AND

https://tinyurl.com/2p95vzjy

AND

https://tinyurl.com/mryfryyv

Molecules Found In Mucus Can Thwart Fungal Infection

Researchers  have now identified components of mucus that can interact with Candida  albicans and prevent it from causing infection. These molecules, known  as glycans, are a major constituent of mucins, the gel-forming polymers  that make up mucus. Mucins contain many different glycans, which are  complex sugar molecules. A growing body of research suggests that  glycans can be specialized to help tame specific pathogens -- not only  Candida albicans but also other pathogens such as Pseudomonas aeruginosa  and Staphylococcus aureus. Within the mucus that lines much of the  body, there are densely packed communities of different microbes, many  beneficial but some harmful. This study, combined with previous work on  Pseudomonas aeruginosa and ongoing studies of Staphylococcus aureus and  Vibrio cholerae, suggests that different glycans are specialized to  disable different kinds of microbes. Taking advantage of these mucins  could help researchers design new antifungal medicines, or make  disease-causing fungus more susceptible to existing drugs.

https://tinyurl.com/297wnwrs

A New Perspective On Opportunistic Pathogens Of The Genus Bordetella In Cystic Fibrosis

The  Bordetella genus, closely related to the genus Achromobacter including  the emerging pathogen Achromobacter xylosoxidans, has not been studied  much in Cystic Fibrosis. The current literature suggests that Bordetella  spp. are able to colonize and persist in CF patient’s airways and a few  studies have correlated pulmonary exacerbations with their presence.  Others have shown Bordetella spp. to exhibit virulence-associated traits  compatible with a potential contribution to CF clinical pathology using  CF mice models. Regarding sources of infection, host-adapted species  are thought to be acquired through zoonotic transmission but some  host-associated species have the potential to survive and grow in the  environment and were shown to be transmitted from amoeba to mice,  suggesting that the natural environments could be transient reservoirs  for dissemination.

https://tinyurl.com/2p8b4fe8

Serological Biomarkers For The Diagnosis Of Mycobacterium Abscessus Infections In Cystic Fibrosis Patients

Culture  conditions sometimes make it difficult to detect non-tuberculous  mycobacteria (NTM), particularly Mycobacterium abscessus, an emerging  cystic fibrosis pathogen. The diagnosis of NTM positive cases not  detected by classical culture methods might benefit from the development  of a serological assay. High antibody titers against two specific  antigens were obtained in M. abscessus-culture positive CF-patients,  allowing scientists to consider these serological markers as potential  tools in the detection of CF-patients infected with M. abscessus.

https://tinyurl.com/2dk5ybpp

Changes In Airway Metabolites May Predict Likelihood Of NTM Infection

Cystic  fibrosis (CF) patients with non-tuberculous mycobacteria (NTM) lung  infection show significant changes in airway metabolites relative to  those without such infections, a study shows. Metabolites are  intermediate or end products of cellular metabolism, and some of these  altered metabolites play roles in immune responses and bacterial growth.  Combining metabolic and microbiota changes also allowed for an accurate  distinction between patients with and without NTM infection, further  suggesting that these may be risk factors of NTM infection in people  with CF. Airway microbiota comprise the community of bacteria, fungi,  and viruses that colonizes the lungs. NTM are increasingly being  recognized as common and worrisome infectious agents in people with CF,  as they cause infections that are challenging to treat, associated with a  poorer prognosis, and often become chronic. A research team evaluated  whether airway metabolites and microbiota — reflecting the lung  environment — could potentially represent risk factors for NTM infection  in people with CF. The cause of nearly 60% of NTM cases was  Mycobacterium avium complex, followed by Mycobacterium abscessus  complex; both being the most common NTM types. The NTM cases and  NTM-negative controls did not significantly differ in most clinical  characteristics. Several of the 902 total metabolites detected in these  patients significantly differed between NTM cases and controls, the  researchers found. Notably, some of these alterations were present both  before and after NTM infection, suggesting that they may represent risk  factors of such infection. Their findings highlight significant  differences in metabolic patterns between CF patients with and without  NTM infection, including metabolites that play important roles in the  host immune response and in bacterial growth. Metabolites showing  differences even before the infection may represent risk factors and  therapeutic targets for preventing and/or treating NTM infections in  people with CF.

https://tinyurl.com/eu6un7eh

Breath Biomarkers Show Potential For Identifying NTM Lung Infections

Several  potential biomarkers of nontuberculosis mycobacteria (NTM)-associated  lung disease were identified in the breath of people with cystic  fibrosis. This approach may offer a faster way of reaching an NTM  diagnosis, which has historically relied on slow bacterial culture  techniques. Breath holds great potential as a source of information on  an individual’s health status. In the breath collection procedure,  patients were asked to breathe into a bag through a drinking straw  mouthpiece. The air in the bag was pulled through a filter using vacuum  into a tube that can absorb molecules from the breath sample. While the  exact chemical identity of the biomarkers could not be definitively  confirmed in this study, several molecules had likely been previously  identified in breath samples from cows, monkeys, and humans with  mycobacterial infections. Overall, the findings suggest that the  biomarkers may be breath signatures of mycobacteria infections generally  and may not be specific to NTM, the researchers noted. They added that  while the results suggest the breath test may be a “promising” way of  identifying NTM-associated lung disease, a study involving more patients  and the use of authentic chemical standards or high resolution  analytical tools to absolutely confirm biomarker identity is necessary.

https://tinyurl.com/4cz54w7p

Unprecedented Case Series Advances Promise Of Phage Therapy

An  international team of researchers report promising results from the  largest case series yet of patients treated with bacteriophage therapy  for antibiotic-resistant infections. Non-tuberculosis Mycobacterium  (NTM) infections are increasingly common among patients with cystic  fibrosis. Treatment of NTM infections, particularly those caused by  Mycobacterium abscessus, are difficult due to growing bacterial  resistance to antibiotics. Bacteriophages are viruses that have evolved  to target and destroy specific bacterial species or strains. Each phage  species seeks and destroys only one bacterial species and the current  armamentarium of known therapeutically useful phages is relatively  small. As a result, phage therapy testing is currently constrained to  experimental treatments where all other viable alternatives are failing  or have failed. Phages were administered to the study participants  intravenously, by aerosolization through a nebulizer or by using both  methods twice daily over an average course of six months, though some  patients had shorter or longer treatments based on clinical or  microbiologic response. Patients were monitored for adverse effects,  signs of symptomatic improvement or reduced bacterial presence,  emergence of phage resistance and/or neutralization of phages by the  patients’ immune systems. The authors reported no adverse reactions to  phage therapy in any of the patients, regardless of type of bacterial  infection, types of phages used or method of treatment. The team  determined that phage treatment of mycobacterial infections shows  promise, should be explored further, and provided several insights into  how therapeutic phages might be effectively used.

https://tinyurl.com/bdz3jhjt

The Effect Of Antibiotic Changes During Treatment Of Cystic Fibrosis Pulmonary Exacerbations

Antibiotics  are often changed during treatment of pulmonary exacerbations (PEx) in  people with cystic fibrosis who have a poor clinical response. The  authors aimed to describe the reasons CF providers change antibiotics  and examined the effects of antibiotic changes on lung function  recovery. The co-primary outcome measure was absolute and relative  change in forced expiratory lung volume in 1 s (FEV1) at the end of  treatment and follow-up. Secondary outcome assessed the proportion of  patients returning to > 90% or > 100% previous baseline FEV1.  Reasons for antibiotic changes included change in antibiotic route prior  to discharge, drug reactions, poor FEV1 response, targeting additional  microbes and lack of symptom improvement. In the researchers analysis,  among non-responders, a change in antibiotics was not associated with  any significant difference in absolute or relative FEV1 at the end of  treatment or at follow-up. Antibiotic change in non-responders was not  associated with improved return to 90% or 100% baseline FEV1 at end of  treatment or follow-up. The researchers concluded that changing  antibiotics during CF PEx treatment in those with poor clinical response  was not associated with any improved FEV1 response or return to  baseline lung function.

https://tinyurl.com/2aj67nzk

Ivacaftor Withdrawal Syndrome: A Potentially Life-Threatening Consequence From A Life-Saving Medication

In 2018,  a case series of three adult patients with cystic fibrosis suggested an  ivacaftor withdrawal syndrome (IWS) perpetuated by abrupt cessation of  ivacaftor treatment. An additional case published that year proposed IWS  in the setting of rifampin use, demonstrating this may also be induced  by drug-drug interactions. While this syndrome is a rare complication of  highly effective modulator therapy (HEMT), it is important to recognize  as increasing numbers of patients are prescribed HEMT.

https://tinyurl.com/yn5pc4rs 

Laura  Tillman is 74 years old and has CF. She is a former director and  President of USACFA. She and her husband, Lew, live in Northville, MI.