By: Mark Tremblay
As someone diagnosed with CF 50 years ago when patients slept in mist tents and weren’t expected to live to the age of ten, my life always seemed destined to be “poor, nasty, brutish and short” as Thomas Hobbes described in his book, Leviathan. Not surprisingly, through the years, whenever I’ve had the opportunity to help a fellow warrior struggling with trauma, anger, depression, or anxiety I’ve found it helpful to acknowledge that life with CF is unfair, relentless, unforgiving, and often ends badly. I’m a firm believer that the first step in emotional healing is to acknowledge the full extent of the hurting. However, for many who have benefitted from recent treatment innovations including modulators, this stark outlook may not resonate as much as it once did. Nevertheless, for a significant number of you and our fellow warriors who have rare gene mutations which are untreatable by modulators, and/or little to no access to care, including many of the state-of-the-art interventions we take for granted in the US, Hobbes’s words ring true.
Last year at a CFF virtual conference, I met a young warrior by the name of Diamini Tengetile Nompilo, who lives in Swaziland, South Africa, who opened my eyes to living beyond the reach of modulators, home IVs, transplants and other first world medical interventions. Needless to say, nowhere on earth are the lives of CF patients more poor, nasty, brutish and short than her corner of the world where the Atlantic meets the Pacific on the horn of Africa.
South Africa (SA) has fascinated me since I first read about it in my dad’s Encyclopedia Brittanica (before Google) while soaking wet sitting in my mist tent to when I later volunteered to help orient South African exchange students at LeMoyne College. Like many people, I have long known SA is internationally renowned for its transformational leaders, including: Nelson Mendela, Mahatma Ghandi, and Elon Musk, but I did not know it’s also known for its medical breakthroughs: the first Computed Topography (CT) scan in 1963, the first successful heart transplant in 1967, and the first surgery using three-dimensional printed bone in 2019. Unfortunately, SA also leads the world in food insecurity with 45 million people experiencing caloric deprivation and 2.5 million people dying of starvation annually (www.wfp.org). Additionally, the country struggles under crushing poverty which impacts 50% of the households and unemployment rates which rank it the highest in the world, which is nine times that of the US (www.statssa.gov.za).
Whenever I’ve had the opportunity to help a fellow warrior struggling with trauma, anger, depression, or anxiety I’ve found it helpful to acknowledge that life with CF is unfair, relentless, unforgiving, and often ends badly.
An estimated 700 of the 59 million people in SA have CF (449 patients in the 2018 South African Cystic Fibrosis Registry). Despite hearing first hand from patients, family members, and doctors, it is nearly impossible for me to fathom what it’s like to have CF there. So rather than focusing on individual patient experiences I’m going to highlight just a few of the metrics in the 2018 Cystic Fibrosis Patient Registry between the US (which is similar to most Northern nations) and SA.
US SA
Ø Median mortality age (years): 30.8 15.0
Ø % on inhaled Pulmozyme (rhDNase) 90.7 27.4
Ø % on inhaled Hypertonic Saline 75.4 47.0
Ø % on inhaled Tobramycin 69.4 0?
Ø % that use airway clearance vest * 76.6 0
Ø % with access to modulators (adults) 73 0
*Covered by nearly all public and private insurance in the US
As this summary reveals, CF patients in SA as a whole face long odds and tall giants. If that weren’t bad enough, there are vast inconsistencies in patient access to quality CF care among the fourteen treating hospitals and clinics as well as large disparities between patients who rely solely on public healthcare versus those who are fully or partially privately insured. Approximately 40 percent of the CF patients in SA rely solely on public healthcare which means many lack access to even the most basic CF care, including pancreatic enzymes as well as inhaled and IV antibiotics. To illustrate this, one parent I know has to ration her child’s enzymes because the public clinic does not have enough. Another patient told me the last time she was admitted to the hospital the doctor told her that, “a sputum culture wasn’t necessary because the hospital had only one type of IV antibiotic anyway.”
However, this story does not lack mighty heroes. A small handful of doctors, parents, volunteers, and patients who call SA their home and have chosen to make a stand in this lonely outpost at the end of the world are doing an incredible job against all odds. I’ll highlight just a few who particularly inspire me: Dr. Marco Zampoli, who published the first patient registry in 2018; Mr. Allen Dunn, who leads an all-volunteer army at the SA Cystic Fibrosis Association and established the Medical & Scientific Advisory Committee for Cystic Fibrosis; and Ms. Nompilo, the fellow warrior, who first drew my attention to the plight of CF patients in SA and who continues to share her struggles and her indomitable smile with us daily through social media.
I am immensely grateful that I’ve lived long enough to benefit from improvements in care that have enabled me to play a small part in helping the SA CF community and even consider making a trip to SA when the pandemic lifts to see the people who inspire me firsthand and continue to help them in this battle we share.
A since deceased mentor of mine once told me, “the key to long life is to find the thing or cause that makes your heart break and strive every day to mend the wound.” The beauty of following this simple advice is that it automatically puts me in a space of gratitude, imbues my life with meaning and purpose and propels me to spread healing and light in the world at large despite the limitations of my illness. That said, what makes your heart break and what can you do today to make a tiny difference?
Mark Tremblay, M.A., M.P.A., was diagnosed with CF at 18 months, currently is 51 years old, and 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 for United States Adult Cystic Fibrosis Association, the publishers of CF Roundtable.