The Unique Challenges and Lessons Imparted by the Cystic Fibrosis Community in the Time of COVID-19 Pandemic

摘要

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.