I am a  67-year-old living with CF. I retired two years ago after a 33-year  career as a social work professor and I continue activity as a professor  emeritus at the University of Kansas. I would like to share several  stories about life phases in adulthood and lessons learned that support  living and aging well with CF. I don’t assume that my perspective will  fit for others with CF. My perspective is shaped by a life and career  honoring many spiritual traditions and philosophies and applying their  wisdom to health and service.

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Diagnosis in Early Adulthood

My  childhood was marked by frequent colds, severe seasonal allergies, nasal  polyps, and low weight. My older brother Tom had a similar medical  picture, though more severe. Our family pediatrician, and later  respiratory specialist, did not correctly diagnose our condition. This  was a frustrating time of mistreatments, harmful side effects, and  worsening lung infections.

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When my  brother moved out of our parents’ home he went to see a free clinic  doctor. Thankfully, this doctor referred him to a CF clinic for  evaluation, which led to both of us receiving a correct diagnosis. Thus,  at age 18, I embarked on treatments with our outstanding physician, Dr.  Robert Stern of University Hospitals in Cleveland. He remained my  physician for about 45 years until his retirement.

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Initially,  the diagnosis was shocking. I was relieved because our strange array of  symptoms finally made sense and I knew there were treatments available.  But this also threw my life expectations askew. I was trying to figure  out who I wanted to be and how I wanted to live as I was entering  adulthood just when I found out that I might not survive for long. Dr.  Stern never claimed certainties about life expectancy or pushed  limitations on life goals. He was honest and frank about the clinical  outlook for CF, but he always individualized care. Rather than telling  me what I could not do, he helped me figure out how medical care could  help me accomplish what I wanted to do.

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I  generally had a wonderful time during my undergraduate years living on  campus, full of adventures and intellectual growth. I decided to make  the most of whatever time I had to live, while not being stuck in any  life-limiting assumptions. Still, I had many quandaries about prospects  for career, marriage, and other long-term goals. I felt awkward as  friends provided postural drainage therapy and observed my illness  close-up. Sometimes I felt despair and anger at a combination of my  personal fragility and the larger context of the Vietnam War and rampant  racial and social injustices. I started exploring what I could do to  help our society and world.

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A Second Life

My major  was cultural anthropology. I explored not only what varied cultures had  to offer about the meaning and purpose of human existence but also  various techniques of meditation, healing, and spiritual transformation.  Serendipitously, in my junior year at college, my advisor offered me  the opportunity to study in South Korea as a graduate fellow and  Fulbright Scholar. Immediately after graduation, I flew there with large  bags of oral antibiotics and preparation for self-administered  respiratory clearance therapies. When first offered this opportunity, I  was dubious that I could undertake such travel and international living.  But Dr. Stern and I agreed that the only thing we knew for sure about  my future was that if I did not try, then I would forever regret the  missed opportunity.

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For 14  months, I studied East Asian philosophies, religions, and arts. I often  visited cultural events with a Korean woman whom I later married  (Hwi-Ja). I found ways to live and work around many bouts of fatigue and  illness.

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During  my first summer there, in 1976, I traveled with a friend to beautiful  places for a few weeks. Near the end of our journey, I stood on a beach  looking out at the spectacular scene of the East Sea. Suddenly, a high  wave crashed onto shore and knocked me down. It pulled me out and  twirled me in deep water. I struggled to call for help and was crushed  down by waves many times. I felt my life was being pulled down a  bottomless drain. Yet an inner voice assured me this was not the right  time to die and an unexplainable energy kept me going. My friend alerted  a marine, who happened to be nearby, to rescue me with an inflated  tube. The marine dragged me to shore, pushed the water out of my body,  and then laid me on a cot. Someone asked, “How does it feel to come back  from hell?” I said, “Great! Amazing! Wonderful!” In that moment, I  realized the utter joy and mystery of just being alive. The sound of a  nearby radio, the bright sunlight, the faces of rescuers looking at  me—every sensation was completely wonderful.

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The next  morning, I watched a brilliant sunrise over the ocean, red-orange  ripples flowing at me and filling me with new life. It was not CF that  almost killed me, but rather a totally unexpected calamity. About two  weeks later, I was hospitalized for acute pancreatitis, to which I am  susceptible. It was probably triggered by the trauma of drowning and  resuscitation. Once I came through the nausea, delirium, and acute pain,  I passed fully into a new phase of life.

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Hwi-Ja  and I came to the U.S. in 1977 and soon married. Over the next nine  years, we formed a new life together by connecting our diverse cultural  and spiritual backgrounds. Hwi-Ja has been a constant supporter for me  in all things. I completed graduate studies, including a Ph.D. in social  work, and took my first faculty position in 1986. We both retired in  2019. During that career, much of my work focused on Southeast Asian  refugee resettlement, health resilience, disability and independent  living services, and mental health recovery. My main goal was to prepare  social workers to support people’s efforts to survive and thrive  through chronic illness, crises, and trauma, especially by drawing on  diverse religious and nonreligious spiritual perspectives, while always  respecting clients’ own goals, beliefs, and interests. Hwi-Ja was a  medical social worker for 30 years.

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This  goal has been central to my personal life as well—my brother died when  he was 41. I felt devastated. We had a final meeting that affirmed our  brotherly love and care. That helped me work through survivor’s guilt  and to make a commitment to survivor’s responsibility—the responsibility  to live life well and meaningfully and to help others. In our family  life, we missed having no children. However, unexpectedly, about 12  years ago, we became “adopted” grandparents for the new baby of close  friends, and later also for her younger sister.

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In  adulthood, I established a lifestyle that combines the best of medical  care, consistent use of medications, self-administered respiratory  clearance (such as the Flutter and autogenic drainage breathing  technique), meditation, yoga-inspired stretching, nature walks,  Christian and Buddhist spiritual retreats, and many other holistic  healing practices. About four years ago, after Dr. Stern retired, I  shifted care to a wonderful team led by Dr. Deepika Polineni at the  Adult CF Clinic at the University of Kansas Medical Center, who helped  me transition to my current phase.

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Retirement and Older Adulthood

About a  week after my retirement at the end of 2019, I was hospitalized briefly  and continued home IV antibiotics. Soon after my recovery, the COVID-19  pandemic hit and many aspects of activity shut down. I used the time to  set new priorities. I’m serving on the CF Foundation’s Adult Advisory  Council and the Access Committee. Additionally, I served on the 2021  ResearchCon Organizing Committee. I also participated in a Trikafta  clinical trial. The appearance of Trikafta, which I continue to receive  post-trial, has been an amazing and unanticipated boon. I feel healthier  than I did ten years ago.

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Retirement  presents new quandaries involving the intersection of older adulthood  and CF-based challenges. My body is changing with age and more friends  in my age cohort are dying. We shifted to a fixed income based on social  security and retirement savings. Now there are complications of  coordinating retirement-based Medicare, supplemental insurances, and  patient assistance programs. I lost access to copay programs due to  restrictions on Medicare recipients. These are issues requiring advice,  support, and geriatric medicine, all of which aren’t readily available  for older adults with CF. Fortunately, my wife and I had  employer-supported retirement savings plans for decades, without which  we would be in serious financial jeopardy at this point.

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Conclusion: Mindfulness and Living/Aging Well

Underlying my life stories are some lessons learned that have been very important for my living and aging well:

• Seek  the best of CF medical care provided by teams who are skilled,  knowledgeable, creative, sincere, and empathetic, and who address the  whole person, including CF, but not defined by or limited to CF.

• Complement medical care with a wide repertoire of holistic approaches to health and wellness.

• Establish and maintain a consistent lifestyle and daily behaviors of wellness.

• Do all this to maximize the quality and meaning of life for myself and others.

• Cherish loved ones, share feelings, and leave no cause for regret when they or I pass.

• Do not assume anything based on CF or any other situations, while realistically adjusting to each situation.

• Be ready to respond to possibilities and opportunities for learning and growth.

• Plan carefully for the future regarding life goals and retirement, but don’t be attached to expectations.

• Access older adult services, such as through area agencies on aging, and coordinate with CF-based services.

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All of  this implies living and aging in a mindful way. Mindfulness practices  can reduce symptoms related to anxiety, depression, and distress, and  they can promote positive coping and daily life satisfaction.  Mindfulness cultivates awareness that is clear, gentle, and  non-judgmentally present in each moment. It opens a space in my mind  between an immediate experience and my reaction to it. This frees me for  how to respond to pain and discomfort and how to enjoy the beauty and  wonder of living. I can be aware of my feelings, thoughts, and  sensations whether pleasant, unpleasant, or neutral, while not being  trapped in them. Then I can respond with greater compassion and skill to  myself and others.

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When  mindfulness becomes a way of life, every moment becomes an opportunity  for growing insights through the ups, downs, steady times, and doldrums  of life. For example, daily respiratory clearance and antibiotic  inhalation treatments are a time-consuming inconvenience, but they are  also opportunities for gratitude and self-care. I view my medicine and  nebulizer with appreciation for the medical community and all people and  beings and the earth itself that made this treatment possible. When I  do my respiratory clearance and then inhale the nebulized antibiotic, I  take this as a time to get centered, and to pay caring attention to  myself, my breath, and my condition.

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Mindful  living does not mean always feeling happy or having a positive attitude.  With mindfulness, even times of pain, worry, fatigue, anger, or  discouragement can be acknowledged, loosened, let go, and recovered from  more easily. We can regard ourselves with acceptance, compassion, and  care at each moment. Mindful living and aging are about appreciation for  the journey of life and experiencing growth and fulfillment along the  way.

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However,  wellness is not just about individual and family effort. We need to  have public health and social welfare policies and programs to promote a  high quality of life for everyone, including people with disabilities  and chronic illnesses. Medical services and insurance should be  affordable, accessible, and equitable to support surviving and thriving.  My personal and professional experiences have shown me that each  person’s wellness is interconnected with everyone’s wellness.

Dr.  Ed Canda is 67 years old and has CF. He is a retired professor and  lives in Lawrence, Kansas. He enjoys playing with grandchildren and  performing meditative percussion. Many of his publications are open  access at KU ScholarWorks.

Nothing  in this column is intended to be legal advice. It is intended only as  general information. If a reader has a question about the information  provided in this column, the reader may contact the CF Legal Information  Hotline at CFLegal@sufianpassamano.com or call 1-800-622-0385. All communications with CF Legal Information  Hotline are confidential and there is no cost to call. The CF Legal  Information Hotline is funded by the CF Foundation, but staff are not  employees of the CF Foundation.

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In the  previous issue (Winter 2022) of CF Roundtable, the first part 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 (SSI) Benefits.

This  second part addresses continued monthly cash benefits and continued  Medicare coverage during a trial work period for those receiving Social  Security Disability Insurance (SSDI). Before going further, it is  important to understand that the rules that apply to SSDI and Medicare  coverage do not apply to SSI and Medicaid benefits. Please take care not  to confuse the two because 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) that appeared in the  winter edition of CF Roundtable.

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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) extended period of Medicare  coverage. This article focuses on the trial work period. Some people do  not know if they receive SSI or SSDI benefits. A person should make sure  he or she knows their benefit program so that they are using the  correct work program rules when considering the effect of work on their  ability to receive benefits.

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For an  application for SSDI benefits to be approved, the claimant must show  that, because of an impairment, he or she is incapable of work or  substantial gainful activity. If circumstances change after benefits are  approved and the beneficiary returns to work, the Social Security  Administration (SSA) may terminate benefits. However, a beneficiary may  not know if he or she can return to work without trying, and he or she  may not want to try if attempting a return to work will cause them to  lose his or her benefits.

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The SSA  allows a person receiving SSDI benefits to attempt a return to work  without losing the monthly cash benefit or Medicare eligibility. This is  called a Trial Work Period (TWP). During a trial work period, a  beneficiary receiving Social Security disability benefits (based on his  or her own earnings history) may test his or her ability to work and  still be considered disabled by SSA. SSA generally will not consider  work performed during the trial work period as showing that the  disability has ended until after nine months of work activity.

1. What Months Count As Trial Work Months?

Generally,  if an SSDI beneficiary earns less than $970 per month in 2022 and works  less than 20 hours per week, the work activity is typically regarded as  less than substantial by the SSA. Earning less than the allowable  amount generally will not affect SSDI eligibility or trigger a trial  work month, provided the beneficiary continues to be disabled under SSA  rules. If an SSDI beneficiary earns more than the allowable amount (such  as $970 in 2022) during a month while on benefits, that month will be  regarded as a trial work month.

2. How Long Is A Trial Work Period?

The  maximum TWP is nine months. The nine-month period is cumulative and SSA  will count each month that a beneficiary earns over the monthly  allowable amount ($970 in 2022) as a trial work month. Each month that a  beneficiary earns more than the allowable amount is counted as a trial  work month, even if those months are not consecutive.

It is  important to note that the counting of trial work months is automatic.  If you make over the allowable amount during a month while on SSDI, that  month will be counted as part of a trial work period. Also, it is  important to note that the dollar amounts are gross monthly earnings  before taxes and other withholdings. The SSA will not necessarily give  notice to a beneficiary that a particular month is counted as a trial  work month. SSDI beneficiaries who are working must be aware of their  gross monthly earnings to avoid an inadvertent trial work month or an  unanticipated termination of benefits.

3. Trial Work Periods Do Not Preclude Medical Reviews.

SSA can  consider medical evidence that might demonstrate a medical recovery at  any time. Therefore, it is possible for SSA to conduct a medical review  and stop benefits due to a medical recovery before the end of a TWP.

4. What Happens At The End Of A TWP?

SSA may  determine the work attempt successful without additional inquiry. At the  end of a TWP, the attempted return to work will either be considered  successful or unsuccessful. SSA may conduct an inquiry at the end of a  TWP to determine if the beneficiary’s work attempt was successful.  However, SSA may believe that it already has sufficient information to  assess the beneficiary’s disability status without a further inquiry.

SSA may  make an additional inquiry—medical or earnings and expenses. In the case  of a medical inquiry, SSA may need additional information to determine  if the work attempt was successful. SSA may conduct an inquiry regarding  the beneficiary’s disability status and it may collect information  about the disabling condition, limitations in daily activities, and any  medical improvement. This inquiry is to determine if the beneficiary’s  condition is still disabling under SSA rules. When SSA determines a  beneficiary is no longer disabled, the person is no longer eligible to  receive benefits and the benefits are terminated.

In the  case of an earnings and expense inquiry, SSA may also make an inquiry to  determine if it correctly calculated earned income during the TWP and  if it correctly counted the number of trial work months. To do this, the  SSA may request pay stubs or other records to confirm that the  beneficiary’s gross earned income was over the allowable monthly amount  for each of the nine months of the TWP. To determine if the months were  properly counted as a trial work month, the SSA may also inquire about  any impairment-related work expenses (IRWE).

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5. Impairment-Related Work Expenses.

SSA  deducts IRWE from gross earned income to determine if the beneficiary’s  income was over the allowable amount in a month. IRWE are described in  detail in the SSA regulations (20 C.F.R. §404.1576), but generally  include payments made by the beneficiary for items or services that are  needed to engage in work and are incurred because of the disability.

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SSA will  consider an expense to be an IRWE if the item or service: (1) enables  the beneficiary to work; (2) is needed because of the disabling  impairment; (3) is paid for by the beneficiary and not reimbursed by  another payer (such as Medicare, Medicaid or a private insurance  carrier); and (4) is a reasonable cost for the item or service.

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While  the range of expenses that count as IRWE is broad, some common examples  of expenses incurred by a person with CF are durable medical equipment  (such as nebulizers) and payments for medications and medical services.  Even if most of a medical expense is covered by insurance, the copays  and deducible should count as an IRWE if they are costs paid by the  beneficiary, not reimbursed by another payer, and otherwise meet the  criteria for an IRWE.

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There  are limits to IRWE and the expenses must be verified. However, the  important point is that IRWE can offset gross income that is over the  allowable amount and possibly result in the countable income being less  than the allowable. Another important point is that a small amount of  IRWE can make a big difference. Even a small amount of earnings over the  monthly allowable will cause the month to be counted as a trail work  month and, similarly, even a small amount of IRWE can offset income and  prevent the month from being counted as a trial work month.

6. Grace Period.

If SSA  determines a beneficiary has made a successful return to work at the end  of a TWP and terminates benefits, then SSA will pay the monthly benefit  for the month in which the disability ceased and for the following two  months. This is known as the grace period.

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In the  next issue of CF Roundtable, Part III of this series will address what  happens if after a TWP a beneficiary remains disabled under SSA rules  and continues to have incomes from work. Part III will discuss SSA’s  extended period of eligibility and continued Medicare eligibility.

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.

I’m  going to say the F word…yep. FIFTY. Hallelujah, I made it to 50! This  is something I never expected as a child with aggressive CF born in  1972. I have lost so, so, so many peers from my generation. And though I  have many more elderly mentors with CF, I consider myself “old.” Being  18 years post-transplant means to me that “50 is the new 70.”

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Growing  up, all I wanted was a long life. I yearned to survive. Now, my youth is  behind me. My body is changing. The transplant meds are taking their  toll. Cancer has attacked my eye and I have lost my eye. But I’m still  breathing easy. The future promises more challenges—the loss of my  parents, the decline of my ability to function, and changes in  appearance. I feel both dread and anxiety, and simultaneously laugh at  my once-youthful innocence about a long life. What guides me as I reach  this age?

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One  important source of insight is my perspective of the “long view.” This  means looking at life with a pair of binoculars, seeking the details of  the expansive life that I’ve lived within the context of the wider human  experience. I’m on the top of a mountain looking down to the trail I’ve  climbed, and eyeing with trepidation the trail I must find to climb  back down. I try to take the long view of my life, as if I were hovering  above in a hot air balloon and enjoying the sights from a superior  perspective.

The long  view is the approach that no matter what I am dealing with in the  present moment, I look backwards to see how good I’ve had it earlier in  my life. Life with CF was, and is, hard as hell, but I did my very best  and it paid off in the long run. I was sick growing up but surrounded  with hope, love, compassion, and care. My community always looked at  sick kids tenderly, so I was given attention and opportunities that  never would have happened if I was healthy. I look back at all of us  within the CF community and see so many kids in my old camp photos who  are no longer here. I could’ve been one of those ten-year-olds who died.  Instead, I’m here at fifty. I feel proud that I am still here to tell  their stories.

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The long  view helps me realize I had my turn to be healthy during my  transplanted life. I had a chance to breathe easy and to feel  invulnerable. I had my chance to be a competitive athlete at the  Transplant Games. I also had the chance to be healthy enough to have a  career; to be a real professional.

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The long  view also helps me recognize how fortunate I am to be a woman in 2022,  and how far we have come thanks to courageous feminists of the past. To  be a woman of color brings more privilege today than ever; I get to  marry whomever I want to, reach my fullest academic and vocational  potential, and have worth despite being childless. And compared to human  history, we live in relative peace, abundance, and security. No matter  what I will go through, my basic needs—food, shelter, clothing, clean  water, and air—are being met. How blessed I am!

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As I get  older, the long view also means questioning my perceptions. If I think I  need to be ashamed of my illness or my physical changes, I ask from  where did these messages come? At this age, I question our cultural  norms and ask whether they are fair. Is my value as a woman based on  what I look like? What patriarchy! Was I “better” when I was younger and  prettier? What ageism! I can call BS on the social values that try to  diminish my sense of worth. That’s why I love women who don’t dye their  hair.

As I  mature, I am shifting from the desire to achieve and to accomplish—all  with a sense of urgency to make the most of my one precious life with  CF— to simply enjoying this present moment. The long view means being on  the other side of achievement and feeling content and satisfied with  the life I’ve lived. True maturity means saying yes to saying no. Saying  no so I can do more of what I enjoy and want to do.

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The long  view means looking at my past habits and patterns and shifting them to  something healthier and more adaptive to my present and future needs.  The overthinking, the need to please, the desperation to make the most  of every moment…those things aren’t helpful anymore. It means letting go  of the resistance and fear of medical issues that constantly arise;  leaning into difficulty gives us a growth mindset. My relationship with  pain and discomfort is changing.

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The long  view helps me to normalize daily pain and stiffness as a part of aging.  It helps to find compassion in myself when I can’t remember things or  can’t do what I used to do. It means trusting that my body’s got  this—the natural process of aging, menopause, and loss. I have adapted  and I will adapt to whatever change arises.

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I look  to ancient times to give me perspective. The long view helps me remember  that for most of human history, fifty was a very old age. Elders who  couldn’t see well or move fast didn’t survive in nature very long. In  nature, when animals procreate, they have completed their purpose. Death  gives rise to new life. That is the divine order. All living things age  and die. We are no different.

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As I  age, I constantly strive to live a spiritually informed life. Linda  Graham, a spiritual teacher and psychotherapist, says that “AGE” stands  for Awe, Gratitude, and Equanimity. I feel awe at still being here; awe  is really a deep love for beauty and life itself and all those in it.  This love is how I commune with God. Nothing separates us from the love  of God. Gratitude is intentionally looking at what is given to us, not  what is taken. I have had a chance to be here—to grow and learn and know  myself. How thankful I am for self-awareness and for all that life has  to offer. Equanimity is reflected in “let me taste the whole of it”  (Robert Browning). Equanimity is a goal and not easy to attain. I have  to remind myself that all struggles and triumphs are part of this  package of life; that each experience can still be blessed with  teachings and bring me closer to God.

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We know  that adversity can enhance spirituality and spirituality can be a  healing salve for adversity. English historian Arnold Toynbee says that  generation after generation, there is “opportunity to open souls by way  of the learning that comes through suffering, for getting into closer  communication with God during their brief passage through this world.”  Through all the chapters of life, human beings are linked with God.

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My  spiritual goal is to age without life’s hardships beating me down. I  want to come out of my life difficulties fully spiritually alive, even  if it kills me. I’d like to believe that hardships are polishing me;  Confucius says, “A gem cannot be polished without friction, nor man  without trials.” As I age, my spirit becomes shinier; the edges are  smoothed out.

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Another  spiritual goal is to leave this world a better place. Aging means being  aware of my continuation. What does that mean? That by being kind to  others, mentoring others, and making a difference in my community, I  will continue no matter how long this body survives. Thich Nhat Hahn, a  Vietnamese monk and global spiritual leader, says “my body will  disintegrate but my actions will continue me.” Life will continue.

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I aim to  embrace the sacredness in this bodily journey. I love that Susan  Willson, in her book, Making Sense of Menopause, says that “menopause is  a harvest, a time of life to pick the juiciest fruit, savor it and take  the best seeds you have and plant them for the next generation.” Ain’t  that the truth?

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The  older we get, the more we gain and the more we lose. As I cope with the  loss of my eye, I tell myself that I had two eyes for fifty years. What a  blessing! And the cancer was removed—how lucky I am! And, at the same  time, I mourn what I’ve lost. I am being tested now in a different way.  My eye is gone but replaced with an internalized long view, a new  insight and vision. I’ll do the best I can, like I always have.

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With all  the grief I carry, I find solace in different ways of looking at grief.  The Lakota Sioux see a person who is grieving as most “wakan”—that  means “most holy.” Grief creates an openness to that which is beyond  this everyday world. The pain of grief is seen to give access to a sense  of the timeless, to the eternal. Grieving peoples’ prayers are  considered especially strong. Lakota Sioux tradition maintains that it’s  exactly these grieving people whom we should ask for help and prayer.  So, as we age, there is potential for usefulness and purpose with the  griefs we experience.

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The long  view is accessible to anyone, regardless of age. Even a 15-year-old can  reflect on their full life or what they’ve overcome since age five. It  is a spiritual act to remove oneself from being mired in present-moment  emotions. My prayer is that everyone with cystic fibrosis can cultivate  wise vision with the passage of time. In sum, I leave with theologian  Harvey Potthoff’s wise words: “Good aging is a quest for greater quality  of life in the midst of the natural cycles of life and death, growth  and diminishment, love and loss, joy and grief. It affirms the sanctity  of existence and the continuing love of God in the midst of adversity,  diminishment and even death.” Amen to that.

Isa Stenzel Byrnes is 50 years old and has CF. She lives in Redwood City, California. She is 18 years post-lung transplant.

Remember  that new house I wrote about in my last column? About 12 hours before I  was supposed to pick up the keys, my left front dental prosthetic broke  apart in my mouth and then fell out completely. I wish I could say this  was my first experience with having my teeth—whether the original ones  or their porcelain replacements—fall apart. This story also did not  sound unusual to any of my age peers in the CF community with whom I  shared it. Welcome to aging with CF.

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Getting  older with a progressive chronic disease is alternately glorious,  bizarre, rigorous, and horrific. Having outlived the initial life  expectancy for people with CF in my birth cohort by a good margin, I  find myself in uncharted territory. Several of my clinicians have humbly  admitted that they are learning primarily from us patients about what  to anticipate and how to respond as we grow older. This is a wonderful  attitude and more than a little scary for everyone involved.

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People  with CF become pretty tough because our lives demand toughness. Honing  our ability to laugh at all kinds of terrible experiences tends to help  us live better with the physical burdens of this disease and also cope  better mentally with the internal struggles we face. When my full crown  came apart, I placed it in a little plastic jar that I continue carrying  around in my shoulder bag. I come by my morbid fascinations honestly;  growing up in a medical research lab often makes one embrace the body  and its various odd products. But underneath the dark humor and the  quirky fascination with decay, there is pain and heartbreak. My teeth  are not the only broken things.

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Growing  older with a disease that barely lets you be young can feel like an  endless spin cycle of loss and adjustment from childhood onward. The  hardest thing for me right now is figuring out what is even “normal” for  a body that did not receive coordinated CF care until its early 30s. I  still struggle to balance my heightened tolerance for discomfort and  pain with my acumen for self-advocacy. And I continue to process the  cognitive dissonance of sounding the alarm about a problem for years  before it became bad enough to merit more serious intervention in the  eyes of clinicians.

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When  that prosthetic broke after nearly eight years of continuous issues, I  felt considerable relief both physically and mentally. I knew I would no  longer have to chew each bite of food in fear of it breaking apart. I  also felt less pressure around the socket after losing the crown than I  had previously. But over time, the pressure became worse again and the  area started to ache. I was just on the verge of calling the surgical  practice back to say that I needed an urgent care appointment, when they  called me to offer an earlier consult due to last-minute cancellations  by other patients. I adjusted my work schedule accordingly and took the  next-day appointment.

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It took  several rounds of CT scanning to give a clear image of the socket. All  of the films also showed destruction in the upper jawbone. This  surprised neither the surgeon nor me, given that my entire sinus network  was essentially fossilized by congealed infected mucus until the summer  of 2020 when my ENT care protocol changed. Beneath the socket with the  diseased root, the bone was almost completely gone. Surgery was  scheduled for the first available appointment, about three weeks later.  We made a plan for how to address CF-specific infection concerns and for  early follow-up to assess my healing.

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This  initial consult took exponentially longer than the surgery itself.  Because I had worked arduously to keep my gum tissue—which is also the  product of extensive reconstructive surgery—as healthy as possible, the  socket was in good enough condition to support a combined extraction and  bone graft procedure. My surgeon consented to operate with only local  anesthetic so that I could remain as independent and functional as  possible on the day of my procedure. So during the surgery, I was wide  awake and able to follow what was happening. I even got to take a  picture of the diseased root after it came out. My sense that this would  help me cognitively proved very accurate when I saw what was attached  to the remnant.

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We  expected to see a considerable amount of infected tissue, but not  necessarily a cyst taking up the entirety of the empty space around the  destroyed bone. Even with my face numbed, I could immediately tell that  the pressure in the socket had vanished. I gave my surgeon a thumbs up  on starting the bone graft, and only got through one more song in my  headphones before he began stitching me up. The most challenging thing I  did that day was pick up my antibiotic at a pharmacy in my neighborhood  after multiple technical issues arose with the surgical team’s effort  to submit an electronic order for it. This was a welcome change after  years of voicing the same concerns about that socket being infected and  unstable.

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To age  with CF is to learn our own bodies painfully well, both literally and  figuratively. Looking back, I realize that I should have trusted my  instincts more about something being wrong and pushed harder for an  extraction. Even though I am aging with a deadly progressive disease, I  still face destructive norms about growing older that make many  clinicians and patients prioritize cosmetic interventions over other  types of care. If I had allowed my dentist to try to “save” this root by  putting a deep post into it, I could have wound up hospitalized with a  severe infection in the jawbone after having that cyst rupture.

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As I  told every provider involved with this process, I care about only two  things: quality of life and functionality. This is what aging well with  CF means to me. I absolutely notice how the continued progression of CF  changes my physical appearance. But I would rather look different or be  “missing” something than live in pain or go back to the soft-foods-only  diet that was my only option after infections ate away enough gum tissue  to expose bone below each root. Aging well with CF is about embracing  every bit of freedom and comfort I can find. As I explained to my  surgeon, there is absolutely nothing I will “miss” about this prosthetic  or the tooth that was once there.

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Of  course, I say this from a perspective of economic privilege. I am  getting treatment because I can afford treatment, both via the dental  insurance coverage I get from my employer and via my own income and my  family’s resources to cover out-of-pocket costs. Many of my peers in the  adult CF community do not have access to these resources. Because of  broader patterns of systemic oppression of racial and ethnic minority  communities, as well as queer and trans people, our adult peers who  struggle most to access basic dental care experience these challenges  within a larger context of injustice and hardship.

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Basic  dental care also does not suffice for people aging with CF. Getting the  root remnants of my other teeth stabilized has required years of working  with committed dental and periodontic professionals who embrace  learning from me about the unique oral health concerns CF introduces.  The need for provider education on CF dental issues cannot be  overstated—and neither can the barriers to conducting such training.  Because of the general classist stigma surrounding tooth loss, dental  damage remains a largely silent issue even within the adult CF  community.

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In a  world where so many of us are experiencing transformational change in  our ability to keep breathing as we grow older, we find ourselves  increasingly confronting the aggressive damage CF does to all the other  parts that keep our bodies going. The joy of celebrating each new  birthday comes with tremendous uncertainty about the new challenges we  will face in the year ahead. I just celebrated 38 years myself, my  surgery a slightly belated gift that left me smiling brightly around the  hole in my bite. I remain grateful for the integrity of my remaining  dental prosthetics and the little bit of natural enamel I still have.  But this year, I feel more grateful for that hole and for the progress  it represents toward my own goals for aging successfully.

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.

Aging.  I’d rather be tortured by aliens with a penchant for sadism than think  about it. In fact, to be fully transparent, I can barely stand when  others mention it, let alone complain about it. Sometimes I imagine that  hell is actually spending an eternity locked in an uncomfortably  cramped space listening to healthy people complain about aging, like a  bad remake of Jean Paul Sartre’s play, No Exit.

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Aging  repulses me; but before I explain why, I have to warn you there is no  sexy or compelling way to write about aging. However, to quote my  favorite professor’s advice on teaching, I will attempt to “do well, be  funny, and, failing that, at least give a good book recommendation.”  Given the topic, I will most likely fail on the first two counts, but  hopefully you’ll at least get a good book recommendation if you hang in  there until the end.

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I have  spent hours mulling on this topic, including questioning whether my  visceral repulsion to it is some sort of antisocial, inhuman,  sociopathy; however, I ultimately concluded that my reaction is quite  simple. It comes down to this: I cannot fathom complaining about aging  as someone whose entire existence has been spent striving to age. I have  spent a lifetime trying to extend my time as the sands slip beneath my  feet in the hourglass of my existence even as my hold on this life fades  and my physical faculties falter.

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However,  aging with CF has many paradoxical, easy-to-overlook rewards as well.  In five decades, I’ve survived long enough to earn many distinguished  service medals, including diabetes; hearing loss due to aminoglycosides;  sight loss due to diabetes; early-onset chronic arthritis; and my two  most recent prized trophies—chronic pain in my right lung and acute  digestive problems, which are untreatable with enzymes.

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As some  of you may know from prior columns, I lived in a halfway house in the  Alternatives to Incarceration Program (ATIP) program in Onondaga County,  New York, while I attended college and played hockey and rugby. During  rugby practice one cold fall afternoon, I got into a fight and broke one  of my teammate’s wrist. Immediately, the halfway house got a call to  have me report to my probation officer (PO) downtown where I expected  him to cuff me and escort me across the street to jail to await transfer  to an upstate corrections facility. My best friend at the time, who is  currently doing a 20-year stint in a California penal institution,  offered to drive me, which, inexplicably, the halfway house permitted.  On the way, we had what you might call a serious existential discussion.  He started the conversation abruptly with an offer to buy me an 8 ball  (a heroin/cocaine cocktail that’s taken intravenously) so I could  overdose in order to avoid the eight-year stint I was facing. I was  shocked and flattered he would consider spending nearly all his money to  give me the option, but I couldn’t take him up on it. At the time, I do  not believe my mind had the tiniest cluster of neurons in the frontal  lobes of my cerebral cortex to begin to fathom such a notion.  Considering all the fighting I had done just to age into my 20s, the  thought of giving up was cognitively walled off from consideration.  Exasperated, he exhaled loudly and said, “what the hell are you going to  do upstate with CF?” I thought about it for a second and responded much  more calmly than I imagine he expected, “I don’t know…I guess I’ll be  the only CF patient in prison…what other choice do I have?” Visibly  upset, he half shouted, “how long do you think you’ll make it up there,  man?” I quickly shut down the conversation by saying, “I really don’t  know but one thing’s for sure, I’d rather take my own road (the road  less traveled) than the easy way out you’re offering.” As you can  probably imagine, the rest of the 15-minute car ride felt like two hours  and was spent painfully, yet perfectly, silent until he let me out, at  which time we neither shook hands nor hugged, but rather parted ways  like strangers who had never so much as seen each other before. In the  end, the whole incident was blamed on a rough scrimmage and charges were  never filed—my PO had no grounds for writing up a probation violation.

More  than a decade later, after years of therapy, in the middle of a messy  divorce, bankruptcy, and a lengthy health crisis, my mind had finally  softened enough to weigh more thoughtfully the option of taking the easy  way out, but once again I did not. Due to God’s grace and mercy, one  very dark, desperate night I withdrew the gun from my mouth and veered  back onto the road less traveled, not because I feared or couldn’t  fathom giving up but because I had grown to love the fight and, even  more than that, the person the fight made me. I simply longed to age  more even though it most certainly would entail more time in the  trenches battling CF and other challenges as I aged into my 40s.

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A decade  later, I made it to yet another penultimate milestone in my life—a day I  never thought I would see. On the day I finally received my first dose  of Trikafta, I felt an unparalleled sense of personal relief, but my  sense of relief extended far beyond that. To contextualize this, I, like  thousands of other patients, had prayed my whole life for a miracle  that would lift the cross of CF from my shoulders. However, even through  many intense prayer sessions, I harbored a deeply held reservation that  I dared not admit to myself nor share with others. The truth was, in my  heart of hearts, I did not merely want a personal miracle; I longed to  see all CF captives set free so the remaining 10% of CF patients who  can’t take Trikafta aren’t left behind. On October 21, 2019, the day the  FDA approved Trikafta, I pulled over on my way home from work and  uncontrollably wept for what seemed like hours until I noticed it was  pitch dark outside. I had never experienced such overwhelming joy. I  knew that God had heard the secret desires of my heart by following  through on the impossible promise that I never actually voiced to Him.  Trikafta would not only provide me relief, but it would also provide  relief to tens of thousands of fellow warriors and prevent still tens of  thousands more young, would-be warriors from ever setting foot on the  battlefield. When I finally pulled back onto the highway again, I took  such a deep breath I coughed vigorously when I exhaled and then I softly  whispered, “okay, God, you have done more than I ever could have asked  or imagined so you can take me home now.” At that moment, I recalled  Simeon who saw his life’s desire fulfilled the moment he held the infant  Jesus in his arms: “Sovereign Lord, as you may have promised, you may  now dismiss your servant in peace. For my eyes have seen your  salvation…” Luke: 2:29-30.

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In the  past two years I have aged greatly. I can no longer read a hardcover  book due to diabetic-related progressive sight loss, my right lung hurts  so much it makes me wince if I move suddenly, and the gastrointestinal  side effects of Trikafta cause nearly constant abdominal bloating and  pain. Another unexpected side effect of Trikafta for me is that the  nominal bump in PFTs meant I was no longer eligible for disability or  early disability pension so I will be stuck indefinitely trying to hold  down a fairly demanding job while living at a functional level as bad  as, if not worse than, before Trikafta. Nevertheless, I am forever  grateful to have aged long enough to see Trikafta and how it has brought  hope to thousands of fellow warriors.

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Just a  few closing thoughts on this terminally unsexy topic—if you want to  avoid aging, at least in spirit, don’t ever let yourself believe your  best is behind you. Wear your scars and badges with humility and  gratitude and live in a state of perpetual expectancy that something  wonderful is unfolding even if you are blind to it. Finally, as  promised, if you, like me, have lived life traveling down “The Road Less  Traveled,” read the book by the same name by M. Scott Peck. His novel  spent 10 years on the New York Times Best Seller list, sold over ten  million copies and was translated into 20 languages. I promise you will  not regret it.

Mark  Tremblay is 52 years old and has CF. He lives in Albany, 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 has worked in the New York Governor’s Division of  Budget for six years and presently works full time at the 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.

We  were thrilled when Alex contacted us to be interviewed. Having been  secretive about having CF for most of her 36 years of life, Alex’s most  recent mission has been to spread the joys and benefits of talking about  CF to whomever will listen. And we obliged! She was diagnosed with  cystic fibrosis at nine months old and was raised in Austin, TX. She and  her husband of 16 years, Daniel, and their black lab, Bella, now live  in Colorado. Alex has an extraordinarily open and charming take on  sharing all aspects of life and CF. Please welcome our newest star,  Alexandrea Gantt. Spotlight, please!

Are you eligible for CFTR modulators like Trikafta?

Yes. I  started taking it at the end of December 2019. Prior to Trikafta, I was  never eligible for any modulators, so this was a game changer for me.

What benefits have you noticed while on Trikafta?

I’ve  definitely noticed less coughing! I feel so incredibly quiet now and my  friends and family certainly notice. If we get separated in public, my  husband can no longer find me by following my cough! In Texas, I was on  IV antibiotics five to six times a year. After moving to Colorado, it  was three to four times a year. Now that I’m on Trikafta I still  consider it a win with only once or twice a year!

What side effects have you had?

I’ve  experienced major weight gain plus a debilitating headache a few times a  month. I did have the rash in the beginning and had to take Benadryl  for over a week to combat it. I also had dizzy spells for over 70 days,  but they finally faded.

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Why did you move to Colorado?

For the  last few years that I lived in Texas, I was on IVs pretty much every  other month. We went to visit my friend in Denver and I could breathe  better with the dry air. It was significant enough that my husband and I  decided to move here. Now when I fly back to Austin (well,  pre-COVID-19), the humidity immediately makes my lungs tight and I  almost always have a CF exacerbation once I’m back home.

Why were you closed off about sharing that you had CF?

Growing  up, I never really had to tell anyone about having CF. In school,  everyone always just knew (mostly from another student who had CF).  Anyone I met outside of school was usually a friend of my brother so  they already knew about me. After high school, it felt nice to keep a  part of me “hidden” when I met new people and it almost became an  obsession to see how long I could go without revealing that part of me.  It usually wasn’t long though because I would inevitably get sick and  need to go to the hospital.

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In high  school, I met a few people with CF through the message boards on CFF.com  and became very close to one of them in particular. When she passed  away suddenly, I was devastated. I decided I would never befriend anyone  with CF ever again to protect myself from that pain. I truly thought I  was helping myself, but, in reality, I was actually hurting myself by  denying those friendships.

What led to you opening up about your CF?

I did a  speech about not having kids for my CF clinic, which led to someone  reaching out about speaking at a mini-con, which led to me facilitating a  session and meeting other people with CF who just “got it.” From there,  I threw myself into the community and have never looked back. I now  have many close friendships and I can’t imagine not having them in my  life.

What would you say to others who are not open to talking about their CF?

A good  place to start is by attending virtual events, such as CFF MiniCons,  offered by CF organizations. I know how scary it can be to take that  first step, but I have never regretted it for a moment. I know that  there is a chance I could lose a CF friend again, but I have finally  realized that I would rather have known them and lost them, than to  never have known them in the first place.

How else are you involved with the CF community?

I  frequently participate in the work groups for CFF’s BreatheCon and  FamilyCon. I am also a mentor for CF Peer Connect. In the beginning of  2020, I was featured on a podcast and shared my CF story, which was  completely out of my comfort zone but very rewarding! I will also be one  of the cochairs for 2022’s FamilyCon.

What profession were you in and how was it affected by CF?

I was in  HR for almost ten years. I loved it, but I was always putting work  before my health. I finally had to stop working. It was a really hard  adjustment at first and I especially hated meeting new people because I  knew they would ask the inevitable: “So, what do you do?” I don’t want  to share with people I’ve just met why I don’t work, so I found myself  just stammering and changing the subject. I kind of resent that because,  if I had kids and was a stay-at-home mom, no one would bat an eye—you  can’t just stay home as a wife in this day and age. So, I started saying  I’m a housewife because I find it amusing to see the confusion and/or  judgment in their faces. It’s the little things that give me pleasure in  life, I guess.

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What effect/impact has the COVID-19 pandemic had on you?

The  pandemic has been really hard on me, but I know I’m not alone in that,  which helps. I didn’t leave my house for 15 months and then I had the  summer of 2021, where I actually lived life again and thought everything  was on its way to being over. Then the Delta variant hit, followed by  the Omicron variant, and I have again been homebound ever since.  Luckily, I keep very busy! I have a virtual CF group that meets every  Wednesday, a monthly Bible study that I run, a writing group that I help  run, and a book club, all virtual. I also FaceTime with my niece and  nephew and watch 80s kids movies over Zoom with some of my high school  friends.

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We  recently had to put down our beloved rat terrier, Izzy, and it was  heartbreaking. After losing her, however, I realized how lucky I really  was to have had almost two full years of quality time with her because  of the pandemic. I will never regret having that time with her, and,  because of that, I don’t have the same harsh feelings over being stuck  at home anymore.

Who is your greatest support?

My husband. I know it’s cliché, but he is my best friend and I couldn’t do life without him.

Any hobbies?

I am an  avid reader! With all my treatments (and the fact that I read very fast)  I always log over 200 books a year on Goodreads. I also love jigsaw  puzzles, hiking, board games, crossword puzzles, and playing video  games, especially with my niece and nephew over FaceTime! We bought an  RV this last summer, and now my new favorite thing is taking the RV out  to camping sites and just getting away from it all.

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Do you have a funny CF story?

When I  was 16, I had to be hospitalized for a tuneup. When I returned back to  school, a boy from one of my classes came running over to me and said,  “I’m so sorry, I had no idea you had hemophilia!” (!?!) I just kind of  stared at him and said, “Neither did I.” Turns out, one of my teachers  told the whole class that I was in the hospital for hemophilia, and, let  me tell you, that was a fun rumor to handle.

How do you cope physically and emotionally?

I am a  big proponent of therapy and I also started an antidepressant about two  years ago. For so many years I was opposed to taking something for my  clinical depression, mainly because all I’d ever heard were horror  stories about SSRIs. Now that I’ve been taking it and it’s been so  amazing for me, I want to scream it from the rooftops so other people  know it’s a real option and they don’t have to be scared of it! I wish  more people had been open about it working for them so I wouldn’t have  been resistant to trying it for so long.

Who or what inspires you?

My niece  and nephew. I love watching them grow up and figure out who they want  to be in this world, and they continue to surprise me every day. They  are the funniest, smartest, and sweetest kids I know. I am blessed to  have such an incredibly close relationship with them. We get them for a  few weeks every summer and spending time with them is one of my favorite  things in the world.

Anything else you want to add?

A few  years ago, doing an interview like this would have been unheard of for  me. It’s kind of funny to look back and remember how closed off and shy I  was, because now I’ll tell complete strangers that I have CF! I wish I  could go back and tell myself that I didn’t need to hide. I hope that  anyone who has the fears that I did will hear my story and change their  mind. s

Dr.  Jeanie Hanley is 58 years old and has CF. She is a director and former  president of USACFA. She lives in Los Angeles and welcomes your  comments. Her contact information is listed on page 2.

If  you would like to be interviewed for our “In The Spotlight” column, you  can fill out the form on our website. Alternatively, you can contact  Andrea Eisenman, Jeanie Hanley, or Xan Nowakowski directly. Their  contact information is on page 2.

I  am so incredibly grateful and honored to have been one of the first two  students awarded the newly established Scholarship for the Arts. I want  to thank USACFA and the late Helen E. Eisenman for their work to  support artists with cystic fibrosis through this scholarship. This aid  has dramatically impacted my educational journey, giving me the  financial support necessary to get my Bachelor of Fine Arts. I have had  to become financially independent due to my parents not being able to  find work during the COVID-19 pandemic. It has been a struggle to pay  for college tuition; however, this scholarship has made it possible.

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As a  student enrolled in UCLA’s Department of Art, I am constantly investing  in my art because not all of our supplies are given to us for free. Last  quarter, in the fall of 2021, I made a wall sculpture related to my  identity as someone with chronic illness. The sculpture is about three  feet by four feet and made out of wood, nails, spray paint, and my used  oxygen tubing, which I dyed in different colors. I used my old oxygen  tubes as a way to repurpose the trash I create from my medical devices. I  dyed the tubes in bright colors as a metaphor for my transition from  pediatric care to adult hospitals and care teams. I wanted to make a  statement about my relationship with the oxygen machines to which I have  to be connected 24/7, in addition to highlighting the stark difference  between the art in pediatric hospitals versus that in adult hospitals.  After I switched to UCLA medical hospital for my adult care, I  immediately noticed the white, sterile aesthetic of the interior of  adult hospitals. I missed the colorful walls and playful art at  Children’s Hospital Los Angeles, which is supposed to make kids more  comfortable. I think adults deserve that as well. When I finished the  piece, I realized it helped me change my negative perception of my  oxygen therapy into something positive that makes me feel unique. The  funds from this scholarship allowed me access to the resources necessary  to build this piece of art.

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I plan  on graduating in 2023 with a major in Fine Arts and a minor in  Disability Studies. After I graduate, I want to find work and/or a  graduate program that incorporates both art and disability as well as  continue my studies and involvement with both the ASL and Deaf  Communities. I want to continue to advocate and support the greater  Disabled Community, including the CF and Deaf Communities, both in  school and afterwards. I have always enjoyed fundraising as a form of  supporting others in my various communities. I have considerable  experience with fundraising through my involvement with my Great Strides  team “Rowan’s Roses” when I was a child and, more recently, events to  help me pay for my future lung transplant. Another way I aim to show  support is by getting involved in the larger disabled art community.  Through my Disability Studies minor I plan to do an internship next  quarter working with a collective of disabled and chronically ill  artists and activists who have a goal of curating a library of knowledge  on lived experience of disability as well as creating  community-building workshops. I hope this internship will connect me to  the network of disabled artists and kickstart my career goals.

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I will  continue to celebrate my identity through my art on public social media  platforms to encourage others to embrace their differences. I have  received so much love from others in the Disabled Community because of  the importance and impact my art has in the community. I feel  accomplished knowing others are benefiting from what I love to do. This  scholarship has aided my artistic journey by giving me the freedom to  explore and establish my passions without financial burdens.

Rowan  O’Bryan is 22 years old and has CF. She is a fourth-year undergraduate  student majoring in Fine Arts and minoring in Disability Studies. Her  artwork is largely centered around her identity as a person with  disabilities. She repurposes her medical supplies and devices to offer  an alternative and creative viewpoint on what it means to be chronically  ill. She is a co-leader of a student group pushing for American Sign  Language to be recognized as a major. She is also co-signatory of the  Disabled Student Union where she works alongside other disabled students  to fight for UCLA to become a more accessible university. She hopes to  continue her advocacy and support for the Disabled Community in her  career after receiving her Bachelor of Fine Arts degree in 2023. You can  follow her on Instagram at @row.1.

College? I’m not sure I want to attend a four-year college...that doesn’t sound appealing in the least. These  were some of my first thoughts in high school when the topic of  “college” was brought up. After an entire education of homeschooling, I  knew I wanted a higher education; however, with my family’s military  career, the idea of staying in one town for four years was an extremely  new concept. After all, I had never lived in one place for more than two  or three years. It took quite some time before I realized that all I  had ever wanted to study was music, and it was the education pathway I  needed to pursue. Once I came to that realization, I ambitiously applied  to schools and scholarships. And now, thanks to scholarship programs,  like those offered by USACFA, I am able to attend college.

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I attend  Gordon College, located in Wenham on the North Shore of Massachusetts,  and am getting my Bachelor of Music in Piano Performance. I love the  school as it is a small, Christian, liberal arts college and focuses on  students and their individual achievements. It fosters a very personal  atmosphere and brags a small population, making it an intimate  experience. This was important to me as I knew my family would probably  not always live close to me. That prediction came true—the day my first  semester ended, I received news that my family would be moving from  Maine to Hawai’i in three months. My community of friends and support of  professors at Gordon, however, is a blessing, and I look forward to my  next three years here.

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The  college and career path I have chosen is rigorous and competitive. My  life currently consists of dozens of hours of practice a week, a  20-credit workload, and two music ensembles (not to mention my honors  requirements). Every school day is filled with three to five hours of  practice, an hour of rehearsal, four hours of classes, and homework!  Because of this, I rarely have free time and certainly have no spare  time to work to earn money to pay for college. This is the main reason I  am so thankful for the Arts Scholarship offered by USACFA; without this  scholarship and others, I would feel much pressure to earn extensive  money for school in order to graduate free of debt. Instead, I can  dedicate my time and energy to an extremely demanding major without the  major burden of potential college debt.

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After  college, I hope to move on to a graduate program in piano either here in  the States, or potentially in Europe. I would love to accompany for  ballet and opera company rehearsals, teach private lessons, perhaps fill  in when needed in an orchestra, and, of course, perform. With this  said, it is imperative that I continue pursuing quality education in  order to be competitive in the extremely intense industry—the world of  music.

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For now,  I stay healthy by consistently doing my treatments, attending routine  doctor visits, and taking my medications daily. Even with CF, I can  participate in “college life” and be as productive and accomplished as  ever. Additionally, I can focus on my intense workload and enjoy the  area in which I live. When I’m not studying or practicing, you can find  me having a good laugh with my friends, teaching piano lessons in town,  attending church, trying every café near me, planning another trip (it  looks like Hawai’i and London this summer!), or spending the evening in  Boston to see a show at Symphony Hall.

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In high  school, it was clear that I would be mostly responsible for taking care  of my college bill, and, thankfully, here I am, attending a liberal arts  college in New England, studying what I love. Thank you USACFA, for the  amazing scholarship, and thank you for supporting cystic fibrosis  students all across the country!

Faith  is 19 years old and has CF. She currently attends Gordon College near  Boston, MA. Faith was one of first two people to receive the Scholarship  for the Arts in 2021. When she’s not studying or practicing piano, she  enjoys traveling and exploring the local area.

Hi! I’m  Ela Castillo. I’m an adult diagnosed with CF in early childhood. I have a  passion for advocacy and fundraising within the CF community.  Previously, I worked at Vertex Pharmaceuticals in Boston. I have rare CF  mutations and I am currently ineligible for any modulator therapies.

I am  American and I come from a mixed family—my father’s side is Colombian  and my mother is ethnically Ashkenazi Jewish (from eastern Europe). In  addition to CF, I’m hard of hearing and neurodiverse. I was diagnosed  with epilepsy and ADHD in adulthood. I strive to gain representation for  all the groups I am a part of, as well as other underrepresented groups  within the CF community.

I am  passionate about accessibility and helping adults with CF find  appropriate support, especially for the deaf and hard of hearing and for  those navigating multiple disabilities. Another strong interest is  researching and recommending practical recommendations for young  professionals with CF.

At  the beginning of this year, one of my favorite radio hosts asked her  listeners what their word for the new year was. I thought about it for a  moment or two and decided that “endure” is my word. We all have had to  endure so much over the past two years. Some of us have had to endure  for all of our lives. Living with CF means learning to endure a lot.

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I was  diagnosed with CF in 1956, when I was 12 years old. At that time people  who had CF were not expected to live long enough to enter school. Since I  already was past the expected age of death, I decided to live. I am in  my late 70s and have no immediate plans to stop living any time soon.

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Because  of new ways of treating CF, young people today can expect to live long  lives. I read an article that quoted Kors van der Ent, professor in  pediatric pulmonology at the University Medical Centre Utrecht in the  Netherlands and coordinator of the multi-disciplinary HIT-CF project:  “We’re effectively shifting therapeutic trials from patients to the  laboratory. Thanks to these drugs, in some patients there’s a  lung-function improvement of 30–40% and life expectancy can increase  from the age of 30–40 to 60–80. In other words, there can be a normal  life expectancy.” This kind of thinking encourages me. I really hope  that the young people of today will live into their 60s, 70s, and even  their 80s.

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April 17  is my 78th birthday-anniversary. At that time, I will have made 78  trips around the sun totaling 45,552,000,000 miles. That number does not  even take into account the trip of 25,000 miles that we all make every  day on our earth. That is a lot of travel and I have seen a lot on those  trips. So much has changed over those years.

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When I  was first diagnosed, it was fairly easy to recognize the cough of  someone who had CF. Now, thanks to good medicines and treatments, it is  much more difficult to identify the sound of a CF cough. Many of us  cough only infrequently and our coughs sound almost like any “normal”  person’s cough. Many of us have fewer lung infections, and those that we  do have are less severe and last a much shorter time than they used to.

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There  are some changes that I have noticed in my health that I don’t know if  they are a part of having CF or just related to general aging. The first  one is my bone and joint pain. My back, neck, hands, and feet give me  discomfort. I know that I have suffered compression fractures in my  spine and neck and that those can be painful. So I guess I’d have to  blame them on age.

The  discomfort in my feet and hands may be CF-related arthritis. My thumbs  are quite painful at times and make it feel as if I don’t have opposable  thumbs. That can make it difficult to pick up or hold onto a glass or  cup. I know that I have to be cautious when I pick up or hold items. The  balls of my feet feel as though I am walking on marbles. My soles feel  as if they are completely coated in stiff plastic. The large joints of  my big toes get quite stiff and make walking a trial. I believe that  this discomfort is just another thing to endure.

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A big  change in my living as I’ve aged is that I get tired much more quickly  than I used to. I find that I need to sit down or even take a nap after  even a small amount of exertion. Some of this is just getting older and  some is having less lung function than I once had. However, I am better  now than I was for several years and I attribute that positive change to  taking Trikafta. I have been taking it for two years and it has given  me more energy than I’ve had since I was in my 50s. These new medicines  that are developed for specific mutations are doing wonderful things for  us. I hope that there soon will be such medicines for everyone who has  CF.

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My  physicians and I have good working relationships. They trust me to be  honest with them (and myself) and to let them know when there is a  problem. They are quick to respond to my requests and needs. I have seen  my PCP only twice in the last two years. In that time, I have seen my  ENT doctor twice. I no longer need to see him as often as I once did,  because my sinuses are almost normal. I have had virtual visits with my  pulmonologist every four months. I could never have managed with only  virtual visits with him before Trikafta. Can you tell that I really like  Trikafta?

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So it  seems that endure is the word for what I am living with, regardless of  whether it relates to CF, aging, the pandemic, or what is happening in  Ukraine. There is little that I can do other than hope for the best and  endure whatever occurs.

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Aging  with CF is something that I hope will continue to get easier for  everyone. Being an old person with CF who still has their original parts  (no transplants) may become the norm if we can just endure.

Stay healthy and happy.s

Kathy  is 78 years old and has CF. She and her husband, Paul, have been  married for 57 years. They have lived in the same house in Gresham, OR,  for 45 years. She volunteered for USACFA and CF Roundtable from  the beginning until a few years ago. She enjoys reading, doing word  puzzles and jigsaw puzzles, and she loves cooking and baking, especially  bread. Yum!

I  am 57 years old. I have CF and I’m post-menopause. Many changes come  with age, and we are paving a new path to figure out how CF affects  those changes. Are the changes we experience normal? How does CF play a  role in how our bodies will adapt to aging? What can we do to help  combat the aging cycle and help manage our CF at the same time? It’s  something I have been passionate about for a long time.

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I was  diagnosed with CF at age 37, yes, 37! Since then, my life has been a  series of connect the dots with past experiences, only to realize it was  CF causing all of my medical problems. The most interesting revelation  was that my doctors felt that exercise and diet saved my life prior to  diagnosis. Those two factors motivate me daily.

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When I  turned 40, I was still dealing with getting used to having CF—the  frequent infections, medications, and hospitalizations. I knew I was  well past the survival age, and, on top of this, my body decided it was  time to enter perimenopause. Along with all the changes in my menstrual  cycle, I started to experience my first issues with arthritis in my  knees and hips. I thought this was unusual but decided I would just keep  exercising and eating healthy and not give it much thought. I was soon  diagnosed with osteopenia, which surprised me, but my doctor explained  this was “normal” with CF. I knew exercise helped with combating  osteoporosis so I decided I would increase my exercise to help stop the  progression. In addition to taking prescription Fosomax, I decided to  increase my running from three to five miles. I slowed my pace so I  could get through the five miles without too much exhaustion and, after  six months, my body was used to the change. Instead of increasing my  weights, I increased repetitions to promote lean muscle mass and bone  density, again hoping I was doing what was right for the osteopenia in  conjunction with the arthritis. I ate fruits and vegetables daily, drank  at least eight and sometimes up to 12 glasses of water, and kept  chicken and fish as part of my diet.

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By 48 I  was in full menopause. Realizing the lack of estrogen was going to play  another role in bodily changes, I decided it was time to get back to  dancing and started studying ballet and modern dance with the Princeton  Ballet School. Having a degree in dance made this a relatively easy  transition, but it had been a long time since I was in a studio. The  dance was just the thing my body needed. I realized I was needing more  flexibility and posture exercises with my workouts and dance provided  the missing piece. So along with running and weightlifting, I felt I was  at my peak performance for my fitness. Still, I wondered about my diet.  It was then I decided to start following a more vegetarian-focused  diet. I stopped eating chicken—eating just fish—and incorporated  completely vegetarian days into my week. I noticed how much easier my  digestion was on those days with less gas and bloating. So, I kept that  routine and, as the years ticked by, I ate mostly vegetarian.

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It  wasn’t until a few months ago that I did some research into plant-based  eating. I had been on Trikafta for nine months and the side effects were  starting to show—increased liver counts, blood sugars, and cholesterol,  along with neuromuscular tears, were plaguing me and I was worried. My  doctor and I decided to decrease my dose and I decided to go full  plant-based eating (vegan).

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Since  making these changes I am the healthiest I have ever been. All my counts  are down in the low to normal range. My joint pain is gone and only  mildly flares up if I do an extra hard workout. The swelling in my hands  has decreased and the pain no longer wakes me up at night. My workouts  have even improved, and, for the first time in years, I am able to start  increasing weights, which is increasing my muscle mass and helping me  improve my cardiovascular exercise. My skin looks better and my gastro  problems have all gone away.

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I am a  true believer that even with CF we have control over our bodies and can  still make positive changes to help combat what this disease and all our  medications throw at us. I see exercise always being a part of my life.  I know as I age my exercise routine may change and I will gracefully  accept those changes. I will continue to work hard at keeping myself fit  and feeling the best I can feel. I know it’s because of diet and  exercise that I am the healthiest I have ever been and I am so happy for  it.

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.

I  was diagnosed in 1991 after my surgeon discovered polyps during a  routine adenoidectomy. Polyps aren’t common unless you have cystic  fibrosis, in which case they’re likely the bane of your existence. At  that time, the life expectancy for someone born in the 80s varied  between 10 and 14 years, depending on the source.

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At the  time of my diagnosis, no one told me that I likely wouldn’t survive into  my 20s. I remember finding out at camp that first summer after my  diagnosis when a cabinmate announced that she had read in her science  textbook that I would die before I’m 20. Needless to say, I came home  from camp with all kinds of anger at not knowing that and even worse,  finding out that way.

Most of  my early life with cystic fibrosis was relatively easy, comparatively. I  had high lung functions. My weight was stable, if not slightly over  much of the time. I got by with oral antibiotics when I had an  exacerbation. And treatments didn’t take nearly as long. Granted, most  of the advancements in nebulizer treatments came in my 20s. My last  semester of college, fall of 2001, I developed my first drug  allergy—Cipro. I was miserable. I had hives everywhere, and I mean  everywhere. I was on steroids and Benadryl for a solid month. That last  semester I didn’t take care of my health much and I paid the price in  the end. By December, I was in my doctor’s office (at that time, we  didn’t have a CF clinic yet so I saw my pediatrician who also  specialized in CF) every other day. Nothing was working and I had  constant pleuritic pain—I felt like someone was stabbing me in the  sternum. By April, I was admitted for two weeks for my first ever tuneup  in the hospital.

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Let’s  fast forward to my mid 20s. My sinuses are textbook CF sinuses, or  worse. Honestly, it’s a miracle I lasted as long as I did without too  many sinus issues. I was plagued by daily, debilitating headaches. Like  many with CF, my dark sense of humor saved me. I joked often that I’m a  human barometer and can feel the pressure changes in my head. I started  seeing my ENT monthly for scopes of my sinuses. I racked up quite a few  sinus surgeries and more drug allergies along the way. Finally, I agreed  to have a frontal sinus obliteration when I was 31. There aren’t too  many of us in the CF community who have had this “pleasure.” It’s a  brutal surgery with a very long recovery. I’ll skip the details here but  essentially my forehead sinuses are now filled with fat from my tummy.  This was lifesaving for me, on several levels—I got quality of life back  for a time and, during surgery, my ENT found a pocket of infection that  was sealed off but close to rupturing, causing either blindness or  spinal meningitis. In the many, many CT scans we did pre-surgery, that  pocket always looked like bone growth. Surprise! During my 20s, I  developed consistent pain that felt like 24/7 heartburn. After gobbling  down a box of Gas-X, with zero relief, I went to the ER in tears. Turns  out, I had a golf-ball-size gallstone—the doctor immediately scheduled  an emergency cholecystectomy to remove my gallbladder. I was so happy to  say goodbye to that pain!

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Let’s  fast forward again—this time to my 30s. This is where the aging bit  really started cropping up in my health. In addition to my CF, asthma,  and chronic sinus disease, I was also diagnosed with osteopenia, which  isn’t terribly surprising since my vitamin D levels, until recently,  have always been low. Sometimes, as low as five. After having a dozen or  so PICC lines and rounds of IV antibiotics, the team in IR  (interventional radiology) commented that placing a PICC was challenging  from existing scar tissue from prior PICC lines. The last PICC they  placed hit a lymph node, causing lymphatic fluid to leak consistently. I  went back to the ER within the week, after having a dressing change  daily because everything under the adhesive was wet. The ER physician  refused to place a new line saying I needed to save my veins.  Eventually, they took me to IR where they applied Dermabond to the site  and assured me that it wouldn’t leak anymore. The next day I had my home  health nurse pull the line because the Dermabond wasn’t sealing the  leak as promised. That was the impetus for my decision to have a  portacath placed—preserving veinous access and, as an added bonus, no  more PICC line snafus. It’s one of the best decisions I’ve made  regarding my healthcare. It’s so much easier and my husband is a pro at  accessing and deaccessing me for IVs and monthly flushes. In my 30s, I  was also diagnosed with CF Liver Disease (CFLD) and had two endoscopic  retrograde cholangiopancreatography (ERCP) surgeries to clear out the  debris in my biliary ducts.

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On top  of all of that, I was also diagnosed with Graves disease in my 30s. This  ranks second to CF for me as it was, and still is, a miserably long  road back to feeling normal. For about 18 months prior to my diagnosis, I  kept complaining about being hot and cold and I was lethargic beyond  what was normal for me. I was dismissed as being “menopausal” from the  outset. It took a year before my pain specialist at the time finally  heard me and ordered blood work for my thyroid. I’ve never been so  grateful for bad bloodwork! Near the end of that time period, I remember  having to sit on the floor to blow dry my hair before work because I  only had about three spoons (according to the spoon theory) for the  entire day and drying my hair used one of those up! Did I mention work? I  also still worked full time. It was exhausting, and I needed an  exorbitant amount of support from my husband to make it through a single  day. Eventually, I left my full-time job so I could focus on my health  and getting better. I had my thyroid nuked in 2017. When my TSH levels  skyrocketed from hyper (I was in the negative) to hypo (my levels jumped  to 91 in one week’s time), I again felt absolutely horrible. The  effects of a thyroid hormone imbalance are far-reaching: hair loss,  weight gain, constipation, and fatigue, to name a few. Over time, I’ve  learned how to pinpoint what feels off and whether we should recheck my  TSH levels. A lot happened in that decade of my life!

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Meanwhile,  my lady bits are also a hot mess. In the last 15 years, I’ve had a lot  of procedures down there: cervical cryotherapy to remove dysplastic  cells; a LEEP procedure to remove cancerous cervical cells; an ablation  to cauterize my uterine lining; an Essure placement (coils placed in the  Fallopian tubes to prevent pregnancy); removal of ovarian cysts; and,  finally, a hysterectomy, in which my ObGyn discovered I had a  bicornuated uterus. If this entire paragraph reads to you like the  screenplay for an obscure sci-fi film, you’re not alone.

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Let’s  fast forward to now. I was recently diagnosed with hypertension,  bordering on malignant hypertension. It’s been increasing over the last  few years and I’m now on a blood pressure medication and, thanks to  genetics, a cholesterol medication as well. I’m in the process of  finding a cardiologist to add to my cadre of specialists. I’ve also  scheduled a skin test for allergies as the blood test didn’t give the  doctor a lot of information. I can’t take my blood pressure medication  the day before or day of as it interferes with the allergy testing! This  will be my second round with allergy shots but I’m hoping that my  quality of life in the allergy capital of the world (Austin, TX) will  improve, especially with the spring pollen.

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All this  to say, I’m finally in my 40s and I wouldn’t trade it for anything—I’ve  made it! Despite the myriad health problems I’ve endured, on top of  cystic fibrosis, I have outlived several life expectancies. Do I feel  like sometimes I got the raw end of the deal with my genetics?  Absolutely. I also know that I’m fortunate—while my sinuses are some of  the worst CF sinuses, my lungs are rather resilient. As a kid, I didn’t  anticipate the additional health complications that come with aging as I  didn’t think I’d be around long enough. I’m overjoyed that I was wrong.  My medical history and complications can be frustrating, to put it  mildly. I also watch other adults with CF around me struggle with far  worse on a daily basis. I’ve reached the point where it takes me a few  hours to fill out the medical history questionnaire for new doctors and  hospital admissions. Thank goodness for online patient portals—I can’t  even fathom having to spend that much time filling out a paper form at  the doctor’s office! But, as I’ve told every single person who complains  about grey hairs and wrinkles, aging is a privilege, mostly. It’s not,  however, a guarantee that every aspect of it will be smooth sailing. As I  age and see the changes in both my health and appearance, I’m  continually reminded of how lucky I am to be alive in this time with the  medical advances, technology, and treatments I have at my disposal. I  know for a fact that my lifesaving frontal obliteration wouldn’t have  been a possibility if I had been born just ten years earlier.

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Mostly,  I’m extremely grateful that my mom lived just long enough to celebrate  my 40th birthday with me. And I am grateful for my dad, my husband, and a  milestone birthday that was never a guarantee and likely a pipe dream  when I was first diagnosed. Here’s to you, Mom.

Sydna  Marshall is 41 years old and has CF. She is the President and Managing  Editor of USACFA. She lives in Austin, TX, with her husband, Adam, and  her two furbabies, Husker and Cutty. She’s an avid reader, loves jigsaw  puzzles, firmly believes that the heart of the home is in the kitchen,  enjoys walks with her pups, and treasures her time on her yoga mat. Her  contact information can be found on page 2.

Each  quarter, when CF Roundtable lands in my mailbox, I sit down as soon as  time allows and read it cover to cover. No matter the topic, each issue  has articles that resonate with me and they’re written by people like  me. While I realize everyone has different experiences, there is comfort  in knowing that each person who is generous enough to share their story  understands what it’s like to live with CF.

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I  appreciate the honesty and insight of everyone who has generously  contributed over the years and have felt a vague sense of guilt for not  doing so myself. When I saw the topic for Spring 2022—Aging with CF—I  knew that I needed to sit down, write something, and finally contribute  back.

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I’m 57  years old. Although I wasn’t diagnosed until the age of 19, I was sick  throughout my childhood and adolescence and was misdiagnosed for many  years. I grew up in a small town without a big medical center where CF  might have been recognized earlier. I was diagnosed in 1984 and, despite  the relief of finally knowing what was wrong, it was a terrifying  diagnosis. There weren’t any treatments other than IV antibiotics and  chest PT at that time. In 1984, I had already outlived the life  expectancy of 18.

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I was  able to connect with a few other patients through an international  publication for CF adults, and it became apparent that one of our common  coping mechanisms was a dark sense of humor. A favorite joke back then  was that at least we wouldn’t have to worry about wrinkles, dementia, or  any of the other age-related problems that people without CF endure.  Every cloud has a silver lining, right?

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Even  with my occasional dark sense of humor, I have always tried to do what I  could to stay as healthy as possible, while living as normal of a life  as CF would allow. In the 90s, Pulmozyme seemed like a miracle and  helped space out hospitalizations. Hypertonic saline was another arrow  in the quiver; before I stopped working, I would get up 30 minutes  earlier to add them to my morning treatment regimen. The Flutter  provided a bit of independence with airway clearance, but,  unfortunately, I never really mastered the technique. Once the Vest was  available, I found that was much more user friendly. Although I’ve never  been one who enjoys exercise, I recognized early on the benefits in  terms of airway clearance, fitness, and mental health. I have practiced  yoga for 25 years, walk regularly, and have started doing high-intensity  interval training in the last eight years to increase strength,  endurance, and functional capacity.

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Despite  my best efforts, living with CF for this long has taken a toll on my  body. My intestines are cranky and don’t work as well as they used to.  My lungs are damaged from repeated infections. My veins are scarred. I  have both CFRD and osteopenia. I also have a portacath. In the past few  years, Trikafta has made a significant difference: fewer  hospitalizations, no cough, improved quality of life, and more energy.  This is almost as good as the cure we dreamed about back in the 1980s.  What a gift!

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My CF  symptoms may have improved, but my youthful joke of being spared  age-related issues hasn’t come to pass. I had a complete hysterectomy  after being diagnosed with uterine cancer in my late 40s. In my 50s, I  have developed cataracts and arthritis in my hip. In the past year  alone, I had a hip replacement and two cataract surgeries, both of which  have improved my quality of life immeasurably. It’s amazing to have a  surgery that can actually repair or fix an issue, not just alleviate  symptoms. I never anticipated any of this, but I’m not complaining; it  is a privilege to be able to experience getting older, despite having to  put up with age-related degeneration (and wrinkles!) in addition to CF  and its complications.

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One of  the richest experiences of having CF has been the connection with other  patients. No matter the difference in age, geographical location, or  health, there is an understanding and bond that is like no other. I am  very fortunate to have a loving family, friends, and healthcare  professionals who are incredibly supportive, but, try as they might,  they aren’t able to understand what it is like to go through life with  CF. My gratitude extends to the CF friends I have had for years, with  whom I can reminisce about the “old days” of sharing a hospital room  during admissions. I am also grateful to those who are younger and bring  a fresh perspective to living with the disease.

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I’m  incredibly grateful to be as healthy as I am at this age. Every life is  full of ups and downs and mine is no exception, but I believe CF has  given me a perspective and resilience that has

Kim  is 57 years old and has cystic fibrosis. She lives with her husband and  two cats on the coast of Massachusetts, just north of Boston. They have  a wonderful adult son who lives in New Hampshire. In the warmer months,  you can find her outdoors in her garden or at the beach. When she’s  indoors, she likes to bake, knit, quilt, and read. She’s looking forward  to spending more time with friends once the pandemic is under control.

At  47 years young, with respect to CF, I am the healthiest I have ever  been. Obviously, there were many times in this journey I would have said  there is no way I would make it to this point. And yet, here I am  attacking life with a vigorous energy: working out, raising our now  eight-year-old daughter, belly laughing, working, traveling, taking care  of the dog and three cats, volunteering, cleaning the entire house  without wheezing and hacking up green goblins, sleeping without  coughing, running around with my daughter and her friends, and breathing  deeply. And yet these happy events have, paradoxically, taught me to  expect the unexpected—because there is one thing we can count on, and  that is change. Therefore, I am soaking this up and going full speed  ahead while it lasts, regardless of age.

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Nonetheless,  growing old is proving to be interesting. Obviously there are the  annoying joint aches and the beginnings of hearing loss, possibly  resulting from years of antibiotic and steroid treatments. Lack of  circulation is also a bit concerning—my feet and legs tend to go numb  and tingly throughout the day, both during activity and while being  stationary.

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For now,  the two biggest challenges of age and CF are vision damage and  menopause. I currently wear bifocals to help with astigmatism and  nearsightedness, in addition to having regular screenings for glaucoma.  However, in the past two years, I have also developed an abnormal  neurologic pupil dilation. The right pupil dilates to an unusually large  size while my left pupil is sluggish and remains small. Unfortunately,  this impairs my ability to read as the words begin to multiply and blur  together almost daily. Fortunately, all scans for brain tumors and  pinched optical nerves are negative. The neuro-ophthalmologist I see  recommended I take beta-carotene, a precursor for vitamin A, which is  already in my CF ADEK vitamins. The cause of this optical phenomenon  remains enigmatic; however, I refuse to give up and continue to expect  the unexpected.

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I hate  to bring up the elephant in the room but, whew, perimenopause is a big  one that tends to sneak up with impeccable timing: I’m dealing with our  second grader when she’s testing the boundaries; I’m having a technology  breakdown during an important project; or I’m just hearing the annoying  sounds my husband makes when he chews (poor guy, he never knows what is  in store for him). At such moments, I suddenly feel there is a volcano  erupting in my very being and I am unable to control its explosion. Any  small annoyance and boom! It can trigger this loss of patience. It is  not pretty, just as night sweats are not sexy. When I realize I am  unable to control my inner demons, I am repelled by my own actions and  feelings. At that point, I become my own worst enemy and double down on  angry self-criticism. That’s when the psyche is its own worst enemy and  causes me to recoil. Of course, this withdrawal is not opportune as life  demands that we are always on point. This is where I struggle to  control my rage of imbalanced hormones and try to appear normal and  functioning. The aftereffect almost always results in depression. Just  when I started feeling like a confident woman who knows what she wants,  menopause slaps me in the face as if to say, “take that!”

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Of  course, I realize that as I write this, I am not the only woman who is  going or has gone through this change. I am not convinced menopause is  much different in an individual with CF as opposed to anyone else,  except that a lot of women with CF are living long enough to experience  this life transition that, is ultimately a gift. After my CF care team  determined that avlimil (a natural supplement with low-potency  ingredients like black cohosh, soy isoflavones, and valerian root) is  not compatible with my modulator medication, I chose not to take any  hormones or supplements and just try to deal with this stage of aging as  naturally as possible. Thus, for now, I am just working on my patience  (or lack thereof) with diet, exercise, camaraderie, sleep, meditation,  crying, letting go of what others think, and sometimes laughing at  myself during these hormonal episodes.

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Aging is  nothing to fight against—it will happen regardless of whether we are on  board with it or not. I believe as women—especially as women with CF—we  can grow with this transition; it may not be elegant and it’s certainly  not always fun, but we can do our best to appreciate this new  challenge. At the very least, we can expect the unexpected because you  never know what miracle might be around the corner that may open more  opportunities to continue our individual missions and enjoy this life a  little longer.

Sonya  Ostensen is 47 years old and has CF. She is the interim Secretary for  USACFA. She lives in Melbourne Beach, FL, with her husband, daughter,  dog, and three cats. She holds a Bachelor of Science in Environmental  Sustainable Resource Management from Ohio State University. After  working in environmental health, she retired due to CF complications.  Sonya has a passion for wildlife rehabilitation and loves to travel with  her family and experience new cultures. Her favorite activities include  gardening, baking, walking the beach, and climbing trees with her  beautiful daughter. Her contact information is on page 2.

Every  time I turn around, my husband reminds me that I am old. What he means  is that I can no longer expect to easily do what I did in my youth. I  understand that. I do not expect to play tennis or swim like I did in my  30s. I know that the last few years brought many new health challenges.  I did not get this far in life to say, oh, well, I guess I can no  longer do this or that. I should just give up? No way!

​

When I  complain I am tired, he says, “you are old.” When I comment that my back  hurts, he says, “it’s because you are getting older.” Yes, I am old-ish  and certainly old for someone with CF, but he doesn’t take into account  that I am being treated for a recurrence of Post-Transplant  Lymphoproliferative Disease (PTLD), which causes fatigue because my  Epstein-Barr virus (EBV) load becomes high. This is common when someome  is immune suppressed and their own body cannot fight off the EBV. The  oncology team felt that I may have gotten EBV from my lung donor 22  years ago.

​

I had a  spinal fusion at age 16 and now have resultant back pain. Most of my  back is fused, and playing tennis with the necessary torque can cause  back pain. It doesn’t help that my fusion is separating from my lower  spinal column. Plus, I also have degenerating discs and arthritis, both  of which can cause extreme nerve pain that radiates into my hips.  However, the way I feel during and after I play tennis brings me such  joy that I want to keep playing for as long as possible. I play tennis  with many women, most of whom are 20-25 years older than me, and they  always call me the young’un! Obviously, age is relative. Until recently,  they said they were amazed watching me dash around the court after  tennis balls while wearing two leg braces (I had a fractured right knee  and a meniscus fix on my left knee). Thankfully, these braces assist me  in not injuring myself further. People see the braces and immediately  assume I do not move much. I love proving them wrong. Although, I hate  to admit it, I am slowing down and do not run for everything any longer.  Tennis has been on hold for me since July 2021 when radiology placed a  PICC line in my right arm to treat the PTLD. And I am right-handed. I am  just starting to wade back into playing. I’m not back to playing a full  game, but I’m active in a tennis clinic where an instructor runs  several women through drills and eventually we play mock games or  “queens of the court.” After I played last week, I felt great mentally  but knew the blow back on my body might be intense. I am happy to write  that I was only a little sore—nothing that I don’t sometimes experience,  anyway.

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Still,  when my husband says I am old, I do what I need to do to function daily.  It is a big effort to constantly monitor how I am feeling—do I need to  call my doctors or what I can do to help myself breathe better or how I  can build up stamina to be able to play tennis again? These are things I  focus on in the moment. And I remain grateful I am still alive.

​

At my  age and with my physical limitations, I know in order to enjoy these  kinds of physical exertions without the ensuing pain, I have to put more  work into my aging body prior to stressing it. This is a change from 15  years ago. I have been going to physical therapy and learning exercises  to support my core and build up my glutes, quads, and hamstrings to  keep me strong while I was unable to play tennis or swim due to my PICC  line. Eventually, I began to focus on what I could do to get into better  shape for when I could play again. This was tedious as I was fatigued  for at least four to six months when I was in treatment for PTLD and my  EBV was high. Thankfully, I was in a clinical trial and I received  infusions of cytotoxic T-cells that were taken from donors whose own  bodies were besieged with EBV. These cells were administered via PICC  line through a 50-ml syringe push with the hope that the T-cells would  target and kill the EBV in my body. Even though my EBV was dropping,  receiving these cells each week was extremely tiring and I barely had  the energy to do much else aside from eating, sleeping, napping, and, at  times, cooking. Exercise was an afterthought and something that was  just too much to consider.

​

Eventually,  I could see that my infusions were going to be ending soon and I forced  myself to get back on my stationary bike. I was winded and discouraged.  But I made myself find the time, after a nap, as many days a week as  possible to just ride and not judge how I felt. I also forced myself to  spend time on my mat on the floor with focused muscle-group-  strengthening techniques. I was amazed at how fast my body had lost its  strength during the prolonged treatment.

​

I went  into menopause after my birth control pills were stopped in 2017 due to a  deep vein thrombosis—this likely contributed to my muscle weakness. The  birth control pills were used for maintaining bone health and keeping  osteoporosis at bay. I was put on a bone-building medication called  Alondronate (generic for Fosamax) to help improve my bone density.  However, since stopping the birth control, my muscles no longer react  the same and I feel generally weaker. I do not feel I can build the same  muscle I had before menopause. And I am more blobby in the middle—dare I  say, paunchy?

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Each  year I am alive, I see that most physical things do get harder. But do I  give in and just not do them? Or do I try to find a workaround to keep  doing what I love? No and yes. Staying active is important for me  physically but also mentally and socially. I have a lot of great friends  whom I have met through playing tennis. They keep me going.

​

Prior to  my lung transplant, I was lucky if I could walk on a treadmill for 20  minutes while wearing supplemental oxygen. Now that I am old(er) and  hopefully wiser, I try to manage my expectations and be more realistic  with physical goals. And I have to be careful with my osteopenic bones  when I am active. I don’t want to fall and fracture anything. Regardless  of what my husband tells me about being old, I am so grateful I am able  to be active and enjoy the life I have— limited as it may be compared  to my younger self and to my healthy peers. I am just trying to make the  most of it and constantly learning to adapt.

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.

The  application deadline for the Higher Education (formerly the Lauren  Melissa Kelly) Scholarship is June 30, 2022. Any student seeking a  degree in higher education, from Associate to Ph.D., is welcome to  apply. We look for students who demonstrate tremendous academic  achievement, community involvement, and a powerful understanding of how  their CF—matched with these achievements—places them in a unique  situation to gain leadership roles within the community. We believe that  any higher education is a strong foundation for advocacy and  involvement in the CF community.

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Nancy  Wech established this scholarship in honor of her daughter, Lauren  Melissa Kelly. This semester’s winners demonstrated outstanding  potential, just like Lauren years ago. Lauren was an inspiration to all  who knew her. An incredible leader and scholar, her drive and success  are the foundation of her memory. She was transformative in every aspect  of her life. She had distinguished herself as a member of the Golden  Key Honor Society, Mortar Board, Phi Upsilon Omicron, Gamma Beta Phi,  Delta Gamma sorority, and was chosen as one of ten Senior Leads at the  University of Georgia. She acted as one of the re-founding members of  the Phi Kappa Literary Society and was significant in the metamorphosis  of the Z Club into the William Tate Society. Although Lauren lost her  battle with cystic fibrosis late in her senior year, her hard work and  memory continue to live on through her inspiring involvement.

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Scholarships  are awarded each year. More information, including the application and  relevant deadlines, can be found on our website. For questions about  future scholarships or anything related to the application process,  please contact us at scholarships@usacfa.org. 

A Year In Review: Real World Evidence, Functional Monitoring And Emerging Therapeutics In 2021

This is a  comprehensive review of: 1) infection with SARS-CoV-2, 2) impact of  COVID-19 on clinical care, 3) CFTR modulators, 4) extrapulmonary  complications, 5) diagnosis, 6) early lung disease, 7) pulmonary  exacerbations, 8) microbiology, and 9) novel therapies. While lengthy,  it is well worth reading.

https://tinyurl.com/mu7r62es

CF Patients Satisfied With Care, But Not Life Quality, US Survey Finds

Most  people with cystic fibrosis responding to a U.S.-based online survey  were generally satisfied with their treatment plan and were not  considering changing their medication. Shortness of breath was the most  commonly reported symptom among CF patients, as well as the one that had  the greatest impact on their daily life. Despite overall treatment  satisfaction, nearly a third of survey respondents were dissatisfied  with their quality of life, suggesting that more should be done in terms  of symptom management and patient support/care. Shortness of breath  also was most commonly reported as having the greatest impact on daily  activities, followed by diabetes, persistent coughing, frequent  lung/sinus infections, and damaged airways. Nearly all of the CF  patients surveyed reported seeing a pulmonologist, and more than three  in five were seeing a general or primary care doctor. Others reported  being seen by a respiratory therapist, an endocrinologist, an ear/nose  and throat doctor, and a cardiologist. All patients were taking  medications to treat CF. Patients were on the same treatment plan for a  median of 25.5 years. The most common side effects were anxiety, joint  and muscle pain, sinus congestion, nasal congestion, depression, and  bloating. Joint and muscle pain, along with extreme tiredness, were the  most bothersome. Among the patients who answered a question about their  quality of life, more than half reported being satisfied. However,  nearly a third of patients reported being “somewhat” to “very  unsatisfied”. More than half expressed optimism about their future. More  than a quarter were “neutral,” and only less than one fourth of  patients reported being “somewhat pessimistic,” with none saying they  were “very pessimistic.” Also, most patients were “somewhat” to  “extremely satisfied” with their current treatment plan. The remaining  were “somewhat dissatisfied” or “neither satisfied nor dissatisfied”;  none were “extremely dissatisfied.” More than three-quarters of patients  also reported being satisfied with their health insurance provider.  Nearly three fourths of the patients who answered a question about  clinical trials expressed interest in participating. Overall, the survey  results highlight generally high rates of satisfaction with care and  health insurance among CF patients, but also indicate that a relevant  proportion of patients are dissatisfied with their quality of life.  These findings highlight areas of unmet needs and call for better care  and support to improve the lives of these patients.

https://tinyurl.com/yb56beqd

Cardiovascular Complications In Cystic Fibrosis: A Review Of The Literature

Cystic  fibrosis is a genetic disease caused by mutations in the cystic fibrosis  transmembrane conductance regulator (CFTR) gene, leading to dysfunction  of the CFTR protein. Disorders of the cardiovascular system in  individuals with CF are usually attributed to secondary effects from  progressive lung disease. However, CFTR has been localized to vascular  endothelium and smooth muscle, suggesting that CFTR dysfunction may  directly impact cardiovascular function. As treatments for CF improve  and life-expectancy increases, the risk of vascular disease may increase  in prevalence related to primary and secondary CFTR dysfunction,  chronic systemic inflammation, nutritional health and hyperglycemia in  individuals with CF related diabetes.

https://tinyurl.com/y8kdnuf9

Testicular Cancer In Men With Cystic Fibrosis

Researchers  reported on the incidence and risk factors for cancer in individuals  with cystic fibrosis in the United Kingdom. Using a case-control  approach, they observed a high incidence of lower gastrointestinal  cancers, confirming and extending results from a large cohort study in  the United States. Besides, they reported testicular cancer in six men  with CF during the 19 years of observation. Although the authors did not  mention whether this number was greater than in the control group or  than expected based on cancer rates in the general population, it is  consistent with a two-fold excess risk based on numbers and rates  observed in the US cohort.

https://tinyurl.com/yvs6dbnt

What Is Cystic Fibrosis–Related Arthritis (CFRA)?

Cystic  fibrosis–related arthritis (CFRA) is a complication of cystic fibrosis.  Pain attacks can be infrequent or happen regularly. There are two types  of CFRA: cystic fibrosis–related arthropathy (CFA) and hypertrophic  pulmonary osteoarthropathy (HPO). It is estimated that between 2% and  8.5% CF patients develop CFA. Signs of CFA include: 1) episodes of joint  pain, swelling, or tenderness, 2) limitation of movement, and 3) fever.  CFA is more common in people with more severe lung disease and can get  worse during an active lung infection. CFA is more episodic, with joint  pain lasting less than one week at a time. Symptoms arise during flares  but may completely go away. HPO is less common in CF patients, at  between 2% and 7% and generally causes severe symptoms which include: 1)  episodes of joint pain, 2) dull bone pain, 3) digital clubbing, and 4)  worsening in cold weather. It is unclear what causes CFA and HPO since  little research has been done on these conditions. The following tests  are used to diagnose CFRA: 1) full blood count (also known as complete  blood count, or CBC), 2) measuring acute phase reactant levels (APR):  These are inflammation markers in the blood that increase during an  infection or injury, 3) X-rays, and 4) synovial fluid analysis: This  test shows whether the fluid between your joints is normal. The color,  thickness, and presence of bacteria are examined. CFRA may also  increases the risk for bone diseases, such as osteoporosis and  osteopenia. Treatment for CFRA includes NSAIDS, corticosteroids or  DMARDs (disease-modifying antirheumatic drugs).

https://tinyurl.com/ycoztk69

Prevalence And Impact Of Rheumatologic Pain In Cystic Fibrosis Adult Patients

The aim  of this prospective study was to evaluate the prevalence of spinal and  joint pain, and their impact on disability, anxiety, depression, and  quality of life in CF adult patients. The investigators found that  rheumatologic pain is frequent in CF adult patients, and may affect  daily living, anxiety and quality of life. Systematic assessment of  rheumatologic pain should be included in the management of CF patients.

https://tinyurl.com/2p9esrmn

Urinary Tract Infections In Cystic Fibrosis Patients

To date,  there has not been a published report of urinary tract infections  (UTIs) in CF patients. Investigators performed a retrospective chart  review at a major academic medical center during 2010-2020 to determine  the features of UTIs in 826 CF patients. They identified 108 UTI  episodes during this period. Diabetes, distal intestinal obstruction  syndrome (DIOS), and kidney stones were correlated with increased risk  of UTIs. UTIs in CF patients were less likely to be caused by  Gram-negative rods compared to non-CF patients and more likely to be  caused by Enterococcus faecalis. The unique features of UTIs in CF  patients highlight the importance of investigating non-respiratory  infections to ensure appropriate treatment.

https://tinyurl.com/mr2xtye8

Two Anaerobic Bacteria Species Tied To Declining Lung Health In Adults

Two  types of bacteria — Porphyromonas pasteri and Prevotella nanceiensis —  were found to be highly prevalent in the mucus of adults with cystic  fibrosis and associated with a progressive decline in lung health. These  two bacteria differ from previously identified, disease-exacerbating  bacteria in being anaerobic species, meaning they do not require oxygen  to survive. Researchers evaluated anaerobic bacteria in CF sputum and  its potential association with lung function decline over eight years.  Sputum samples from 70 adults were analyzed and compared with patients’  clinical data. Among the 10 most abundant anaerobic bacteria in the  samples, P. pasteri and P. nanceiensis alone were associated with lung  function declines, as measured by yearly changes in each patient’s  forced expiratory volume (FEV). Data showed the presence of P. pasteri  or P. nanceiensis correlated with an average FEV loss of 52.3 and 67.9  milliliters each year, respectively, indicating a decline in lung  health. The greater the abundance of either bacteria in an individual’s  sample, the faster the rate of decline.

https://tinyurl.com/yc8m4kg4

Higher BMI Associated With Favorable Clinical Outcomes In Patients With Cystic Fibrosis

In a  systematic review and meta-analysis, researchers found higher BMI was  associated with favorable clinical outcomes in patients with cystic  fibrosis, urging reconsideration of the currently recommended target  BMI, which in children older than 2 years, is at least the 50th  percentile; in adults, the target BMI is greater than or equal to 22 for  women and greater than or equal to 23 for men. However, BMI does not  distinguish between the major components of the body, namely, fat mass,  fat-free mass, total body water, bone mineral density and bone mineral  content. Studies of patients aged 2 years and older with cystic fibrosis  with altered BMI or body composition were compared with patients with  measured parameters in the reference ranges. The primary outcomes were  pulmonary function, exocrine pancreatic insufficiency and cystic  fibrosis-related diabetes. Compared with those with normal weight,  researchers observed higher FEV1 among those who were overweight and  obese. In addition, likelihood for cystic fibrosis-related diabetes and  pancreatic insufficiency was higher among patients with normal weight  compared with those with overweight. These findings suggest that  nutritional status plays an important role in maintaining organ function  in patients with cystic fibrosis. Because a higher BMI is associated  with better clinical parameters, clinicians are advised to reconsider  increasing the currently recommended target BMI. The use of a  nutritional strategy that increases BMI, at least until the upper limit  of normal BMI is reached, should be included in the daily protocol. The  results suggest that careful evaluation of body composition should be  incorporated into everyday clinical practice. However, obesity is a  major global health concern and is associated with increased morbidity  and mortality due to higher rates of heart disease, hypertension and  diabetes. Thus, emerging concern over patients with cystic fibrosis who  are overweight or obese experiencing these detrimental health conditions  is justified, particularly in the era of modulator therapies. ...  Further longitudinal studies to address the consequences associated with  overweight status in patients with CF to optimize nutritional  approaches and treatment plans will be required to help guide further  clinical recommendations.

https://tinyurl.com/5hd6372m

Women With CF Need Counseling To Preserve Fertility, US Study Finds

With  continual advances in treatment, people with CF are living longer and  those of childbearing age are increasingly choosing to become pregnant.  CF itself can cause problems with fertility. In addition, many patients  eventually require a lung transplant to maintain their health, and  transplant recipients typically need to take immune-suppressing  medications for life to prevent organ rejection. A common side effect of  these medications is infertility; as such, CF patients may wish to  explore options to preserve fertility — processes like freezing eggs or  sperm, which can be later used to have children. A team of researchers  conducted a two-part study to better understand what woman with CF know  about fertility preservation. In its first part, the team surveyed 50  women with CF between the ages of 18 and 35 and recruited nationwide.  Most of the women reported being in a relationship. The majority were  not mothers, but most said that they wanted to have their own children  in the future. Despite their desire for a biological child, nearly three  out of four survey respondents reported never discussing fertility  preservation with their healthcare providers. About one in three said  that they had never discussed options for parenthood with anyone. The  women generally had little knowledge about options for preserving  fertility. The researchers noted that women who had conversations about  fertility tended to be more knowledgeable about its preservation.  Married women also tended to be more knowledgeable. In the study’s  second part, the researchers interviewed 20 women with CF, and  summarized some of the broad themes that emerged from the discussions.  One major theme was that many women with CF are not adequately  knowledgeable about fertility and its relationship to CF. The patients  commonly reported that medical resources were scarce, and they often  turned to other patients or social media for guidance. The women also  noted that fertility in general was not viewed as a priority by their  healthcare team. The women recommended that CF care providers need to be  educated about fertility in CF, and take care to bring up these issues  with patients. The women stressed the importance of clinicians viewing  patients as complete human beings with unique wants and needs, and not  as merely a collection of symptoms to be managed. The interviewees also  highlighted the importance of incorporating women’s healthcare into CF  healthcare, and vice versa. Findings from the study support that  additional education is needed for women with cystic fibrosis who are  considering parenthood. Clinical care models should include early,  regular, and thoughtful discussions about reproductive health issues,  including fertility preservation.

https://tinyurl.com/yaokkx7l

AND

https://tinyurl.com/2p8ph3dm

National  Jewish Health Pulmonologist Guides Development Of Innovative Tool To  Help People Of Color Screen Their Own Symptoms For Cystic Fibrosis

Cystic  fibrosis is most common within the white population, but it affects  people of all races and ethnicities. The recognition of CF in Black  individuals and people of other racial and ethnic backgrounds has been  markedly inadequate. Following the virtual North American CF conference  in the fall of 2020, Dr. Taylor-Cousar met Terry Wright, an Arkansas  resident in his 50’s whose symptoms were misdiagnosed as asthma in  combination with common gastrointestinal problems for decades.  After  Dr. Taylor-Cousar was introduced to the couple, they discussed the  possibility of developing a free online tool that would be accessible  for anyone to self-screen for symptoms of CF.  She offered her medical  expertise to create and refine the tool that could help reach the  broader population. Then, Dr. Taylor-Cousar and the Wrights presented  the tool to the Cystic Fibrosis Foundation’s (CFF) education committee,  which found that it meets their guidelines and standards of an effective  screening tool for public education about CF.  Now known as the Wright  Cystic Fibrosis Screening Tool©, the tool is being shared with CF care  teams through the CFF and with the general public on social media. The  new tool will help people self-identify symptoms that could be related  to CF and aid medical providers in identification of potential  individuals with CF, especially those who are Black, Indigenous, and  People of Color (BIPOC). Once the screening is completed and the  symptoms are consistent with CF, the individuals can take the  information to a doctor, who can refer them to get a test to confirm if  they have CF.

https://tinyurl.com/y6vzgv7p

How Do Patients Decide On Treatment For Cystic Fibrosis Exacerbations?

In a  study of patient decision making involving people with cystic fibrosis,  researchers found that concerns over difficulty or pain with breathing  had the greatest impact on patients’ decision making with respect to the  treatment of pulmonary exacerbations, and that gastrointestinal  problems also figured prominently in influencing treatment decisions.  Given the varying toxicities and burdens of therapeutics for cystic  fibrosis, researchers sought to determine how these patients valued  different aspects of their health status and the trade-offs they made  when choosing between alternative therapies. Toward that end,  researchers conducted a discrete choice experiment survey (DCE) in order  to quantify how those with CF and their caregivers hypothetically  viewed the relative importance of outcomes resulting from treatment of  CF pulmonary exacerbations. Participants were asked to make a series of  hypothetical decisions about treatment for pulmonary exacerbations to  assess how they make trade-offs between different attributes of health.  The outcomes studied were: breathing difficulty/pain, fatigue, pain  unrelated to breathing, lack of motivation/demoralization, reduced  appetite, presence of fevers/night sweats, high treatment burden,  inability to meet goals, sputum production and clearance, and  gastrointestinal symptoms. The study found that for people with CF,  breathing difficulty/pain had the greatest influence on their preferred  health outcome states followed by gastrointestinal symptoms, then  presence of fevers/night sweats. For caregivers, the greatest influence  on their preferred health outcome for their child was breathing  difficulty/pain, followed by pain unrelated to breathing, then  gastrointestinal symptoms. The researchers concluded that the health  outcome preferences reported here should guide decision-making when  seeking agreement on personal goals of therapy between patients and  clinicians for treatment of pulmonary exacerbations.

https://tinyurl.com/yckxrerp

CBD And Cystic Fibrosis - Can Cannabidiol Help?

Clinical  interest in cannabis and CBD is rising, and the data in favor of its  healing power continues to trickle in. CBD even holds the potential to  alleviate some of the symptoms of cystic fibrosis. CBD, short for  cannabidiol, is a natural cannabinoid that can be found in the Cannabis  sativa plant. It is non-psychoactive, meaning that CBD is devoid of the  ‘high’ that cannabis’ mind-altering molecule, tetrahydrocannabinol  (THC), is known to cause. Both cannabinoids – CBD and THC – have been  shown to have powerful therapeutic action, either when administered  alone or in combination. Cannabinoids interact with our endocannabinoid  system (ECS), a complex cell-signaling network in our body that helps to  regulate our mood, immune function, appetite, sleep, memory, pain, and  much more. CBD can indirectly modulate the function of our cannabinoid  receptors, which can be found all over the brain and body – including  within the airways. Back in 2002, a paper published in the Journal of  Cannabis Therapeutics detailed a theory that cystic fibrosis causes a  deficiency in endocannabinoids and “by elevating these levels, symptoms  may improve”.  CBD indirectly increases levels of anandamide, an  endocannabinoid that is produced naturally in the body. By this token,  CBD could supplement the ECS and subsequently improve CF symptoms. Since  this paper was published, the effects of THC administration have  harbored positive results in animal models of cystic fibrosis. There is  existing evidence to suggest that CBD can reduce pain and inflammation,  support the immune system, and relieve gastrointestinal issues in  countless other clinical conditions. CBD will not be a cure for CF, but  it could, in theory, help to relieve some of the symptoms, without the  unpleasant side effects of existing drugs. CBD has potent  anti-inflammatory action. It has been shown to reduce inflammatory pain  and reduce the severity of the inflammatory response in numerous  experimental and clinical studies. Inflammation is a major driver of  cystic fibrosis symptoms, so CBD could help to relieve inflammation and  inflammatory pain in the lungs and the digestive system in patients  living with CF. Cannabis is a well-known appetite stimulant. This is  largely due to THC, but there is some evidence to suggest that CBD can  also stimulate appetite. CBD has also been shown to suppress nausea in  animal models, which suggests that CBD could support those with CF who  struggle to eat. Cystic fibrosis is a hugely debilitating and isolating  disease that can cause anxiety or depression in many patients and their  families. Where CBD surpasses other pain relief medications is its  ability to support our mental health. By interacting with serotonin  receptors in the brain, CBD can help to support the signalling of  serotonin. This is thought to be why CBD can relieve anxiety and  depression, as demonstrated by various human and animal model studies.  Currently, the only evidence in favor of using either CBD or cannabis to  manage symptoms of cystic fibrosis is anecdotal. For now, there is no  clinical trial evidence. While many people choose to self-medicate with  cannabis-based products, there is insufficient data to support its  safety or efficacy in managing CF symptoms in the clinic. As a  supplement, CBD is considered to be safe and well-tolerated. The side  effects of taking CBD are rare and mild. These can include nausea,  fatigue, and appetite changes. This is, obviously, a potential  contraindication of using CBD to manage CF, since problems with appetite  and digestion are common symptoms. There is no definitive answer for  how much CBD to take for cystic fibrosis, or how to take it and  researchers can’t say for certain whether CBD could improve symptoms of  cystic fibrosis.

https://tinyurl.com/bdzxtp4j

Ivacaftor,  Tezacaftor, Elexacaftor (Kaftrio) In Combination With Ivacaftor  (Kalydeco): Risk Of Serious Liver Injury; Updated Advice On Liver  Function Testing

Cases of  serious liver injury with elevated transaminases and bilirubin have  been reported during treatment with Kaftrio–Kalydeco combination  therapy. In all patients, measures of alanine aminotransferase (ALT),  aspartate aminotransferase (AST), and total bilirubin levels should be  done before starting treatment, every 3 months during the first year of  treatment, and annually thereafter.

https://tinyurl.com/y8b4z5r4

Trikafta May Be Safe And Effective For Liver Transplant Patients

People  with cystic fibrosis (CF) who had a liver transplant can be safely  started on Trikafta (elexacaftor/tezacaftor/ivacaftor). For most  patients in the study, Trikafta’s use resulted in an easing of symptoms,  better quality of life, and healthier body weight and lung function.  Doctors with the CF and transplant teams should work together in closely  monitoring a patient’s progress. Regulatory approval was based on  positive results from clinical trials showing its efficacy and safety.  However, liver damage can occur as a side effect of Trikafta’s use, and  patients who have or had liver problems are advised to talk with their  doctor before starting with it. For those getting a solid organ  transplant, possible problems include a worsening of liver function and  drug–drug interactions with immunosuppressants, which must be taken to  prevent the immune system from attacking the transplant. Despite the  risks, Trikafta may offer benefits to some CF patients who have had a  lung transplant. However, there are no recommendations provided for CFTR  modulator use with patients undergoing a liver transplant.

https://tinyurl.com/234yk8rs

Cystic Fibrosis Transmembrane Conductance Regulator Modulators Decrease Hospitalization Rates

For  patients with cystic fibrosis (CF), using a CF transmembrane conductance  regulator (CFTR) modulator was associated with reduced hospitalizations  for acute pancreatitis (AP). Patients were stratified by  pancreas-insufficient CF (PI-CF) and pancreas-sufficient CF (PS-CF)  status. More patients with CFTR modulator use had PI-CF. Stratified by  pancreas sufficiency, 1.4% of AP admissions occurred among the PS-CF and  5.3% of admissions among the PI-CF groups during CFTR modulator use.  The highest AP hospitalization rate occurred among patients with PS-CF  who had discontinued CFTR modulator use. The lowest rates of AP  hospitalization were observed among the patients with PI-CF who were  currently using CFTR modulators. Thus, this study found that patients  with CF benefited from CFTR modulator use, regardless of established  pancreas sufficiency. These data justify a cost-effectiveness analysis  to better assess the feasibility of expanded CFTR modulator use for  preventing AP hospitalization among patients with CF.

https://tinyurl.com/56w47raz

Trikafta Leads To Lesser Need For Antibiotics, Fewer Hospitalizations

Treatment  with Trikafta is linked with fewer infection-related hospitalizations  and a reduced need for antibiotics among people with cystic fibrosis.  Trikafta is an oral combination of three CFTR modulators (elexacaftor,  tezacaftor, and ivacaftor) that works to improve how the CFTR protein  functions in patients with at least one copy of the F508del mutation in  the CFTR gene, a CF-causing defect found in roughly 90% of all patients.  Clinical trials and post-approval data have shown that Trikafta  significantly improves lung function and reduces pulmonary  exacerbations. However, fewer studies have assessed its impact on lung  infections and the use of antimicrobials in CF patients. Researchers  analyzed real-world data and identified 389 CF patients who started  treatment with Trikafta between July 1 and Dec. 1, 2019, and continued  using it through March 14, 2020. That timeframe allowed researchers to  analyze data collected 15 weeks before and after the start of the  therapy. The 15-week observation window was selected to avoid potential  overlap with the COVID-19 pandemic, which led to a significant decline  in healthcare utilization in March 2020. A total of 389 CF patients not  taking Trikafta during the same period were included as sex- and  age-matched controls. Specifically, the team compared the number of  total healthcare visits, inpatient visits, infection-related visits, and  antimicrobial prescriptions before and after Trikafta was initiated.  This analysis showed that a 15-week treatment of Trikafta was associated  with fewer healthcare visits, inpatient admissions, infection-related  visits, and antibiotic prescriptions. By contrast, these parameters  remained generally unchanged or worsened in control patients, although  initial data of overall infections and healthcare utilization suggested  that patients on Trikafta had more severe disease. Increased age was not  significantly associated with any of the outcomes, but being a female  was linked with a more infection-related visits. Also, co-existing  conditions were associated with worsening across all outcomes.

https://tinyurl.com/2p95yaa3

Targeted Exhaled Breath Analysis For Detection Of Pseudomonas Aeruginosa In Cystic Fibrosis Patients

Pseudomonas  aeruginosa (PA) is an important respiratory pathogen for cystic  fibrosis (CF) patients. Routine microbiology surveillance is  time-consuming, and is best performed on expectorated sputum. As an  alternative, volatile organic compounds (VOCs) may be indicative of PA  colonization. This study consisted of 1) a literature review to select  VOCs of interest, and 2) a cross-sectional CF study. The investigators  concluded that biomarkers from exhaled breath can yield high sensitivity  for bacterial infection in cystic fibrosis patients.

https://tinyurl.com/yxe6k2xc

Cystic Fibrosis Genetic Modifiers Identified In Novel High-Throughput Screening Platform

Using an  updated version of a recently developed technology called MaMTH-HTS  (Mammalian Membrane Two-Hybrid High-Throughput Screening) that allows  the detection of membrane-associating factors directly in living human  cells, scientists have identified hundreds of new proteins that could  lead to a better understanding of the cellular effects of cystic  fibrosis and help identify new drug targets for precision medicine  treatments. More than 400 proteins were identified that were associated  with either healthy or mutant CFTR, and have shown that some of them  could predict the variability seen in patient symptoms and treatment  responses. One of the CFTR associated proteins that the team has  identified is Fibrinogen-like 2 (FGL2), a protein believed to play a  role in hepatitis, liver disease and immunity. Despite tailored CF  treatments for the nearly 2000 known CFTR mutations, patient responses  vary widely even among patients who share the same mutation. Researchers  have long suspected variations in treatment response hinge on genetic  modifiers and environmental factors. The identification of CFTR  interacting proteins in the current study will help pinpoint these  modifiers. In addition to FGL2, the researchers are working on other  CFTR targets.

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Specific Biologics Inc. Announces Therapeutic Development Award From The Cystic Fibrosis Foundation

Specific  Biologics Inc., an early-stage biotechnology company developing novel  gene editing technologies, announced that it will receive funds to  support the preclinical development of its Dualase™ gene editing  platform to target a set of cystic fibrosis-causing nonsense mutations  where patients currently have fewer therapeutic options available.  Dualase™ cuts DNA differently than existing gene editors. This feature  enables the high frequency insertion of new sequences into precise  locations in the human genome. Specific Biologics Inc. two-site Dualase™  platform gene editors cut DNA in a way that optimally exploits the  cell’s naturally occurring DNA repair pathways. This enables two gene  editing outcomes, precise DNA deletions to disrupt genes or increased  repair to correct genes. Specific also develops lipid nanoparticles to  deliver the gene editor to target cells. Specific will deliver its gene  editing technology using an inhalable lipid nanoparticle carrier, which  will aid in delivering the gene editing machinery inside the cells of  the lung. It is believed that if the CFTR nonsense mutations are  corrected in enough cells, a gene editing therapeutic could provide a  long-lasting benefit for CF patients.

https://tinyurl.com/262fw3r6

Pulmocide Receives Investment From The Cystic Fibrosis Foundation

Pulmocide  Ltd. announced a $3.5 million investment from the Cystic Fibrosis  Foundation for the development of opelconazole for lung transplant  recipients. People with cystic fibrosis, particularly those who have  undergone lung transplants, are highly susceptible to invasive  Aspergillus fungal infections in their airways. Opelconazole is a novel  azole specifically designed for inhaled use to prevent and treat  pulmonary aspergillosis with potentially fewer side effects than  standard-of-care azole treatments. This profile is expected to enhance  efficacy and reduce the toxicities and drug-drug interactions seen with  systemic antifungal therapies. The CF Foundation’s funding will support a  multicenter Phase 2 study of Pulmocide’s opelconazole. The study will  assess the treatment’s safety and tolerability when used to prevent  invasive pulmonary fungal infections in lung transplant recipients.

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Cystic  Fibrosis Foundation Invests In Felix Biotechnology As They Advance New  Therapies Targeting Chronic Infections In Cystic Fibrosis

Felix  Biotechnology announced it received a strategic investment from the  Cystic Fibrosis Foundation as part of a Therapeutics Development Award.  The award will bolster Felix’s work creating a novel therapy to manage  chronic Pseudomonas aeruginosa lung infections. Felix takes a unique  approach by engineering the natural predators of bacteria, tiny viruses  called bacteriophage (or phage) into durable, broadly effective  treatments. Felix’s phage therapies are specially designed to consider  and drive advantageous evolutionary outcomes of phage treatment. This  results in a more durable therapy, which is key given the need to  repeatedly administer phage to address these chronic infections. This  therapy is currently being evaluated in a clinical trial at Yale.

https://tinyurl.com/ydfmynrh

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CSU Researchers Discover That Anti-Malaria Drugs Can Fight Pulmonary Disease

A  research team at Colorado State University has discovered that drugs  used to treat malaria are also effective at treating a pulmonary disease  similar to tuberculosis. The study is a significant development in the  fight against infections caused by non-tuberculous mycobacteria (NTM).  The research targeted an NTM known as Mycobacterium abscessus. Few drugs  are effective against this mycobacterium, and the ones that are tend to  be toxic and cause bad side effects. The researchers believe that the  bacterium is capable of sensing and responding to threats in its  environment, such as lowered oxygen levels, oxidative stress and acidic  pH, which are our body’s natural ways of fighting disease. It does so by  activating, among other things, a regulator known as DosRS which  controls many essential functions in the bacterium such as its  respiration, ability to form biofilms and ability to enter a dormant  state when the conditions are not favorable to bacterial multiplication.  They found that in mice, two existing antimalarial drugs were able to  prevent DosRS from responding to stresses, meaning that the bacterium  struggled to fight off antibiotics and the immune system’s natural  disease response. One of the things the treatment did was to lower the  bacterium’s ability to form biofilms, thereby reducing its ability to  resist killing by antibiotics. The treatment alone was just as effective  at dropping bacterial loads in the lungs as the combination of  antibiotics currently used to treat the disease.

https://tinyurl.com/2p9d77ny

Rapid Implementation Of Telemedicine Intervention For Cystic Fibrosis Shows Success

Rapid  implementation of a cystic fibrosis care model including telemedicine  was successful for monitoring lung function and identifying  exacerbations during the COVID-19 pandemic. The care model also enabled  monitoring of BMI and decreased use of antibiotics. The implementation  of telemedicine during the COVID-19 pandemic reduced patient and staff  interactions and by doing so preserved personal protective equipment.  Triple therapy (elexacaftor/tezacaftor/ivacaftor) likely contributed to  the overall clinical stability in patients with cystic fibrosis during  the COVID-19 pandemic. Telemedicine also increased access to care for  adults with cystic fibrosis living in regions remote to a cystic  fibrosis specialty center.

https://tinyurl.com/4jeaa7bp

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https://tinyurl.com/mr32sf75

A Cross-Sectional Investigation In Cystic Fibrosis: The CFTR Genotype, And Maximum Exercise Capacity Are Linked.

It was  determined that human skeletal muscle cells carry the cystic fibrosis  transmembrane conductance regulator (CFTR). Variations in CFTR  functioning among cystic fibrosis patients may be a key factor of cystic  fibrosis patients’ maximal exercise capacity. There have been few  conflicting investigations on the association between CFTR genotype and  maximal exercise capacity. This study looked at the factors influencing  maximal exercise capacity, as measured by peak oxygen uptake (V.o2peak),  in children and adults with cystic fibrosis. In an international,  multicenter, cross-sectional study, researchers collected data on CFTR  genotype and cardiopulmonary exercise tests in cystic fibrosis patients  aged 8 and up. Functional classifications I–V were assigned to CFTR  mutations. They found that in patients with cystic fibrosis, the  functional genotype class of the CFTR gene was not linked to maximum  exercise capacity; however, for those with at least one copy of an  F508del-CFTR mutation and a single class V mutation had a reduced  maximal exercise capacity.

https://tinyurl.com/v5kkxk6t

Boost In Vigorous Exercise Fails To Improve Lung Function In Cystic Fibrosis

Increased  vigorous physical activity did not improve lung function for sedentary  patients with cystic fibrosis. ACTIVATE-CF, an international,  parallel-arm, multicenter, randomized controlled trial, enrolled 117  relatively inactive patients aged 12 years and older with cystic  fibrosis. All patients were randomly assigned to an intervention group  in which patients added 3 hours of vigorous physical activity per week,  or the control group in which patients did not change their physical  activity behavior. The primary outcome was change in percent predicted  FEV1 at 6 months. Secondary outcomes included physical activity,  exercise capacity and motives, time to first exacerbation, exacerbation  rates, quality of life, anxiety, depression, stress and blood glucose  control. Compared with the intervention group, patients in the control  group demonstrated higher FEV1 at 6 months. However, patients in the  intervention group reported increased vigorous physical activity  compared with the control group. In addition, patients in the  intervention group had higher exercise capacity at 6 and 12 months and  had higher physical activity in total steps at 12 months. Researchers  observed no effects on all secondary outcomes during the study period.  These findings indicate that a steep increase in vigorous physical  activity represents the wrong approach to improve lung health for the  majority of people with cystic fibrosis who are relatively sedentary. In  contrast, the significant but counterintuitive improvement in FEV1 in  the control group possibly induced by a moderate increase in physical  activity suggests a less stringent ‘motivational approach’ may  effectively modulate an increase in physical activity.

https://tinyurl.com/4tzh2mc8

ARTICLES

Acute  ST-elevation myocardial infarction in two young women with cystic  fibrosis and cystic fibrosis-related diabetes. Sirisha Reddy Thambuluru,  Sayyad Kyazimzade, Katherine A. Despotes, Deepa Kirk, Jennifer L.  Goralski. Journal of Cystic Fibrosis. VOLUME 21, ISSUE 1, E44-E47,  JANUARY 01, 2022

Ischemic  heart disease is rarely reported in people with cystic fibrosis (PwCF)  despite multiple potential risk factors. Researchers report two cases of  ST elevation myocardial infarction (STEMI), both in young women with  cystic fibrosis (CF) and cystic fibrosis related diabetes (CFRD). These  cases illustrate the importance of considering myocardial  injury/infarction in the differential diagnosis of patients with CF and  chest pain or shortness of breath, and addressing the growing risk of  cardiovascular disease (CVD).

https://tinyurl.com/hfe9v67a

Comparison  of microbial composition of cough swabs and sputum for pathogen  detection in patients with cystic fibrosis. Dominic Fenn, Mahmoud I.  Abdel-Aziz, Paul Brinkman, Renate Kos, Anne H. Neerincx, Josje  Altenburg, E. Weersink, Eric G. Haarman, Suzanne W.J. Terheggen-Lagro,  Anke H. Maitland-van der Zee, Lieuwe D.J. Bos. Journal of Cystic  Fibrosis. VOLUME 21, ISSUE 1, P52-60, JANUARY 01, 2022

With the  continued advancement of CFTR modulator therapies there is likely to be  a burgeoning population of adult cystic fibrosis (CF) patients unable  to expectorate sputum. Consequently, the detection and surveillance of  pulmonary colonisation, previously reliant on sputum culture, needs  re-examining. The authors hypothesised that cough swabs analysed with  culture-independent analysis of the 16S gene could serve as a surrogate  for colonisation of the lower airways. Cough swabs and sputum samples  were collected from consecutive adults and children with CF across two  sites at regular outpatient appointments. The researchers discovered  that culture independent analysis of cough swabs provides an inaccurate  diagnosis of lower respiratory tract colonisation and should not be used  as a diagnostic test in patients with CF.

https://tinyurl.com/mveu4fc8

A  comparison of clinic and home spirometry as longtudinal outcomes in  cystic fibrosis. Alex Paynter, Umer Khan,Sonya L. Heltshe, Christopher  H. Goss, Noah Lechtzin, Nicole Mayer Hamblett. Journal of Cystic  Fibrosis. VOLUME 21, ISSUE 1, P78-83, JANUARY 01, 2022

The  COVID-19 pandemic has accelerated the transition to telehealth,  including the use of home spirometry in cystic fibrosis. Evaluating the  accuracy and precision of longitudinal home spirometry is a requisite  for telehealth-based research. This secondary analysis of a CF study  (eICE) evaluates whether there are cross-sectional or longitudinal  differences between home and clinic spirometry. Participants were issued  a home spirometer, and asked to complete spirometry efforts twice per  week for one year. Clinic spirometry was collected at baseline and every  three months. Cross-sectional differences between clinic spirometry and  the closest home spirometry measurement were analyzed. Home spirometry  is estimated to be 2.0 percentage points lower than clinic spirometry  cross-sectionally. Longitudinally, the estimates of 12-month change in  home spirometry varied by analysis method from -2.6 to -1.0 ppFEV1/  year. In order to leverage the potential cost, feasibility and  convenience of home spirometry, the differences with clinic spirometry  must be acknowledged. Significantly lower ppFEV1 in home devices shows  that direct comparison to clinic spirometers may induce a spurious  change from baseline, and additional variability in home devices impacts  statistical power. The effect of coaching, setting, and equipment must  be understood to use and improve home spirometry in CF.

https://tinyurl.com/yc6u6jtj

Quality  of home spirometry performance amongst adults with cystic fibrosis. Jody  M Bell, Sheila Sivam, Ruth L Dentice, Tiffany J Dwyer, Helen E Jo,  Edmund M Lau, Phillip A Munoz, Samantha A Nolan, Nicole A Taylor, Simone  K Visser, Veronica A Yozghatlian, Keith KH Wong. Journal of  Cystic  Fibrosis. VOLUME 21, ISSUE 1, P84-87, JANUARY 01, 2022

Spirometry  is usually performed under the supervision of a trained respiratory  scientist to ensure acceptability and repeatability of results. An  observational study was conducted to evaluate the quality of spirometry  performance by adult cystic fibrosis patients with and without  observation by a trained respiratory scientist. Patients were instructed  to perform spirometry without supervision within 24 h of their remote  CF clinic consultation. Spirometry was repeated at their consultation,  supervised by a respiratory scientist using video conferencing. The  majority of patients achieved grade A (excellent) or B (very good)  spirometry quality with and without supervision independent of lung  function severity. Similarly, forced expiratory volume in 1 second  demonstrated no significant differences with paired spirometry performed  within a 24 hour period. For a large proportion of adult CF patients,  unsupervised portable spirometry produces acceptable and repeatable  results.

https://tinyurl.com/5d226ej2

Clinical  characteristics of Pseudomonas and Aspergillus co-infected cystic  fibrosis patients: A UK registry study. Dominic A. Hughes, Olga  Archangelidi, Matthew Coates, Darius Armstrong-James, Stuart J. Elborn,  Siobhán B. Carr, Jane C. Davies. Journal of Cystic Fibrosis. VOLUME 21,  ISSUE 1, P129-135, JANUARY 01, 2022 Pseudomonas aeruginosa (Pa) and  Aspergillus species (Asp) are the most common bacterial and fungal  organisms respectively in CF airways. The researchers aim was to examine  impacts of Asp infection and Pa/Asp co-infection. They determined that  co-infection with Pa and Asp was not associated with reduced lung  function compared with Pa alone, but was associated with additional use  of IV antibiotics. Asp infection itself is associated with several  important indicators of disease severity.

https://tinyurl.com/tvwb9du5 s

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.