key: cord-1005131-zrekh26c
authors: Ladores, Sigrid
title: The Unique Challenges and Lessons Imparted by the Cystic Fibrosis Community in the Time of COVID-19 Pandemic
date: 2020-05-19
journal: J Patient Exp
DOI: 10.1177/2374373520926360
sha: 3c6c6c55181c64a154ea4134d552e30e1fb2af11
doc_id: 1005131
cord_uid: zrekh26c

nan

My professional and personal lives collide in cystic fibrosis (CF), a genetic, progressive, multisystem disease that primarily affects the lungs and gastrointestinal systems (1) . There are approximately 70 000 people with CF worldwide, of which 30 000 are in the United States (1) . Due to advances in science and clinical care, average survival age is now in the late 40s (1) . As a scientist, my research is on CF. As a professor, I teach nursing students about CF. As a clinician, I have provided care to children with CF. Lastly and perhaps most importantly, my experience and expertise in CF stem from living with someone with CF. My husband of 20 years was diagnosed with CF as a newborn and he has experienced (and continues to experience) the harshness of this incurable disease. He had recurrent pneumonias and severe pulmonary disease that required a bilateral lung transplantation in 2011, and while the surgery saved his life, in no way is it a panacea. Life post-transplant is not easy, and he battles many side effects from his life-preserving medications. The most worrisome of these side effects is immunosuppression. Coronavirus disease 2019 (COVID-19) is highly transmittable and aggressively virulent especially to the lungs. As a result, our family has escalated protective measures to prevent potential exposure of my immunosuppressed husband. We try to not be paralyzed by fear and yet my worry never stops as I plan how to best meet the needs of everyone in my family, lest my husband gets sick. In the past, when he was ill and needed to be hospitalized, I was always there next to him, and I became his voice and advocate especially when he was intubated and on a ventilator. If he were to fall ill now, he would have to go through the terrifying experience alone because I would not be permitted to be by his side. Additionally, with ventilators in low supply to fully meet the needs of everyone affected by COVID-19, 25 states in the United States have instituted policies that discriminate against people with disabilities like CF, policies deemed "Crisis Standards of Care" wherein hospitals ration out life-preserving machines like ventilators to those who are more likely to survive and those who do not have chronic illnesses (2) . The CF Foundation has released their official position on such discriminatory triage plans (3). Disability advocates and bioethicists are wrangling with the implications of such policies. The injustice keeps me up at night.

Another aspect of CF that keeps me up at night are the anecdotal reports I hear from other individuals with CF who fear being attacked because of their characteristic cough that is pathognomonic for CF. The hallmark of CF is the production of excessively thick mucus that plugs different parts of the body but especially the lungs, causing the individual to cough, sometimes incessantly and paroxysmally, in order to expectorate the trapped mucus (1). Cystic fibrosis' clinical presentation of cough and shortness of breath mimics COVID-19's signs and symptoms. As a result, people who do not know about CF or understand its manifestations may be wary of those who exhibit any cough or shortness of breath. There have been reports of individuals with CF who have been verbally attacked and told to go home and not spread COVID-19 because they heard a cough. The widespread fear, lack of knowledge by the general public about CF, and hypervigilance when out in public makes for a potentially discriminatory, threatening, or dangerous situation for people with CF.

Despite the multilayered challenges faced by the CF community during this time of COVID-19 uncertainty, there is 1 School of Nursing, University of Alabama at Birmingham, AL, USA one positive dimension that must be highlighted. Individuals with CF have been well-conditioned to do self-quarantine and social distancing. Most of the planet is currently under lockdown or have "stay-at-home" orders, and while the general population has a difficult time adjusting to the activity restrictions, people with CF have been participating in these health-preserving behaviors for most, if not all, of their lives. They understand the importance of preventing crossinfections through the "6-feet-apart" rule, frequent and aggressive handwashing, and using appropriate personal protective equipment (4) . They are used to hunkering down in a small space of a hospital room for 2 to 3 weeks (if not longer) at a time, 2 to 3 times per year, when they experience a CF exacerbation and need intravenous antibiotics and rigorous chest physiotherapy. There are poignant memes shared on CF-specific social media groups about how they have "been training for this [social isolation]" all of their lives.

In conclusion, while COVID-19 leaves behind an indelible imprint on humankind and places additional anxieties among individuals with CF, perhaps the world can also learn some important lessons from the CF community about patience (with the lengthy time line for quelling this novel coronavirus), adherence (to the mitigation measures in place to "flatten the curve"), and resilience (to come out of this pandemic stronger and more prepared for whatever else may come). Maybe then we can say that "CF" also stands for "Coronavirus Finished."

The author acknowledge that the work is her own. It has not been previously published and is not under consideration for publication elsewhere. The author has obtained written informed consent from her husband who is described in the manuscript.

The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

The author(s) received no financial support for the research, authorship, and/or publication of this article.

Sigrid Ladores https://orcid.org/0000-0003-2867-5619

Cystic Fibrosis Foundation (CFF)

Coronavirus and inequality: state policies may send people with disabilities to the back of the line for ventilators

Statement on COVID-19 triage plans

Daily-Life/Germs-and-Staying-Healthy/How-Can-You-Avoid-Germs/How-Can-You-Reduce-the-Risk

Sigrid Ladores is an associate professor at the University of Alabama at Birmingham School of Nursing. She is a pediatric nurse practitioner, certified nurse educator, and scientist.