Several weeks ago I wrote my one year review on Orkambi. I said that I felt it was helping, but did not have the clinical proof to back up the way I felt. My PFTs are unchanged and even though I had a health streak that was unheard of for me following Orkambi, I fell into a typical health slump around the nine month mark. Although Orkambi didn't feel life altering, I also did not feel any worse so I continued to take Orkambi with hope of slowing the decline of the progressive nature of Cystic Fibrosis. Well, I now officially have proof that Orkambi is working for me!
I want to preface this post by saying CF has taught me to be cynical and I am not a blind believer in anything. So many times I have believed that certain procedures or medicine would benefit me only to be left disappointed. Antibiotics that my cultures are sensitive to (meaning they should knock down an infection) have failed me, I have left hospital stays with numbers just as horrible as I entered with, I have gotten worse with no rhyme or reason and I don't fully trust anything medically based until I try it and see it working for me. The medical field isn't fool proof and CF doesn't seem to follow any rules.
To explain my proof I need to back up a few years (this will be short, I promise). In 2014 I was going through an exhausting and frustrating health slump. My doctors, perplexed by my inability to get better despite everyone's best efforts, ordered a CT scan. The CT scan didn't show anything overly telling despite mucus plugging, scar tissue, bronchiectasis, and other typical CF problems in someone with more advanced lung disease. I continued the roller coaster of a CF slump for a few months and eventually stabilized.
Fast forward to summer 2016 (now post Orkambi) and I was experiencing another CF slump. After IVs and months of oral antibiotics we weren't getting anywhere. My doctors were once again stumped, this seems to be a pattern with me, we decided a CT may be needed to see exactly what was going on. I didn't anticipate it to show much and didn't even bother to call for results knowing if something horrible showed up (say a collapsed lung or pneumonia) my doctors would notify me. Honestly, I didn't want to hear about how much more my lungs progressed because there is nothing more depressing than proof your lungs are dying.
When I did get the results they shocked me! Remember, this is a CT scan taken while doctors couldn't get my lungs to stabilize so clearly, I was not in great health. This CT was a comparison to a 2014 CT scan and CF is progressive in nature meaning things usually just get worse and worse. My thirties have been extraordinarily hard on me and good news in regards to my CF just doesn't happen anymore.
When my results were finally sent to me I skimmed through the write up, holding my breath and feeling that uneasiness that comes with impending disappointment. And yet words like stable, significant improvement, markedly improved jumped off that page at me. Wait, what?! There were improvements in my CT over that past two years? And these improvements are showing up despite how sick I was when the CT was taken? My progression of bronchiectasis has stabilized (it is impossible for bronchiectasis to improve so stability is the best case scenario). Nodules in my lungs were shrinking? There was significant interval improvements in my right lung (which has always been my problem lung)?
I had to read through everything several times before it sunk in. My CT scan did not get worse over the past two years and in fact it actually got better! Although I have not seen the miracle results many people have seen on Orkambi including increased lung function, increased energy, decreased exacerbations, I have seen an improvement in my CT scan. When your disease is progressing at a dizzying rate seeing any improvement can feel nothing short of miraculous.
Yes, I wish I had seen an improvement in my lung function especially since I sit so very close to transplant now. Yes, I wish I had increased energy to make each day count just a little bit more. Absolutely, I wish these infuriating slumps in my health that seem to be nonresponsive to antibiotics would disappear forever. However, at this stage in the game I will take any improvement I can get and even if those improvements are only noticeable through an improved CT scan then I can't complain. Without Orkambi I am positive that CT scan would have continued to look worse. Honestly, I don't even want to think about what my life would look like today without Orkambi.