What We Hope For
I have found myself chasing a deferred hope waiting for my health to change, while watching it steadily decline. The release of Kayldeco by Vertex Pharmaceuticals Inc. in 2012 was a demonstration that the next generation of medications in cystic fibrosis therapy could begin to stabilize and even improve the health of CF patients. However, Kayldeco is only available to roughly 4% of the CF population leaving the vast majority of patients without the option to take such medicines.
This may soon change with a new combination drug, Orkambi by Vertex, which will be available to just under 50% of the CF population to those with two copies of the F508del variant if the drug receives FDA approval. The decision date is set for July 5, 2015 and it will likely be approved following the recent positive vote by a Food and Drug Administration advisory panel of 12 to 1.
Although promising, Orkambi is not the final answer in CF treatment for those eligible to take the medication. Critics of the drug remain skeptical of its implementation due to the modest phase III clinical results which improved FEV1, a metric of lung function, by a mere 4%, well short of the 10% gain by those taking Kayldeco.
As the July 5 deadline approaches, I have a tingle of excitement marred by a continual doubt that anything will change. Those of us with the double F508del mutation find ourselves in a situation that is known to only a fraction of a percent of the world’s population; those who are sick, but on the brink of a medical breakthrough. Our whole lives we have thought the disease was unconquerable and that it was only with time that we would succumb to its fateful course. However, scientific breakthroughs have begun to challenge this fate with the possibility, however small, that we may see a day of healing.
So as I watch my lung function seep from my lips I wonder if orkambi is the answer that will begin to reverse the progression of this disease, or if not orkambi, will another medication come in time for my benefit? It is in this see-saw of hope and doubt that the CF community continues to wait.
My Encounter With Hope
Although the outcome of Orkambi and my health are uncertain, I retain hope. I believe hope is only possible in the midst of struggle or suffering and that is why it is such a powerful emotion within the CF community. We have all experienced its devastation through hospitalizations, lung transplants and the death of those we love and that is why we respond so strongly with a feeling of hope when we are presented with the chance of healing through science and medicine.
For the past several years I keep returning to the latin phrase, ‘Dum Spiro Spero’ when I need to voice my hope and remember that hope is at hand. I happened to stumble upon this phrase as an undergraduate at Wheaton College when I made the decision, after my sophomore year, to take a year away from my studies and attend L’Abri Fellowship in Greatham, England the following spring.
L’Abri is a wonderful, old, English manor quietly ensconced in the English country. It was beautiful and as my time passed and I passed the days in study and discussion with those at the manor my appreciation for the home grew.
As you entered the manor, passed the greeting room and ascended the tiered, wooden staircase, there spanned from the first to second floor a beautiful stained glass window with an inscription on the center pane reading, ’Dum Spiro Spero.’ When it came time for me to leave the manor and L’Abri, the window left me with an indelible impression.
Time passed, life continued and I returned for my fourth and final year at Wheaton with renewed vigor for what lay ahead, but on the first day of class that vigor was slain. I became sick with the wheezing, heaving and coughing known all too well for those with CF. I experienced continual shortness of breath and climbing the stairs to class would leave my chest heaving, extremities weak and mind uneasy as I reached the top.
The problem of being sick with CF as an undergraduate is not easily resolved. Infections require two weeks of IV antibiotics in the hospital to be suppressed and my life to resume. The problem is that classes don’t stop with interruption. They continue in your absence and they continue fast and I knew that I wouldn’t be able to keep pace the remaining semester. So I sat alone in a Taco Bell with only my gordita for comfort as silent tears slipped down my indignant face when I made the decision to drop out for the semester.
I returned home in a quest for my health and a vision to return the following semester. While home, I often spent late nights talking with my now fiance, Chentell, discussing plans for her senior art exhibit in which she included ceramic representations of windows that had a special significance to those close to her.
One evening we talked of the beautiful stained glass window in England with its inscription, ‘Dum Spiro Spero’ and how I had never translated the phrase. That same evening I heard my grandfather in the midst of a loud dinner conversation say, ‘Dum Spiro Spero.’ I leaned in and asked what he meant and he held in his hand a card from a woman he had met and written upon the card was, ‘Dum Spiro Spero,’ and the translation under it read, ‘While I Breathe I hope.’ My inspiration, latent in mind, had been revealed through a small green card.
Now, as I venture into the unknown years later I remain blessed by these words. In the spectrum of the disease I consider myself fortunate for the health I retain. Although when I am sick, and as I write these words sitting on my gently humming hospital bed, I retain hope, because I have the breath to do so.
Orkambi and the current drugs available to CF patients are not the final word in the search for the complete healing of CF, but they have given me light and a continued reason to hope.
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