orkambi

I had another CF appointment, out-of-state, on Monday. My lung function is maintaining. This is fabulous news because, for the past year, my lung function decreases rapidly shortly after steroid and IV antibiotic treatments.

I have had difficulty swallowing for almost a year now, choking and aspirating (food goes now the 'wrong pipe') at times.  At my appointment on Monday they told me that this should be temporary. They said if I continue to vomit less, coupled with doubling my acid reflux meds, it should go away. What a relief!

My weight is on track so that's great news too. Less vomiting is helping with that issue. For any one that doesn't know, keeping weight on in CF is important so that your body better fights and recovers from lung infection. Also, when it comes time for a lung transplant (hopefully I will NEVER need one) I hear that many transplant centers cannot accept patients whose BMI (body mass index) falls below a certain level. So, again, great news!

Lastly, and most excitedly, I qualify for the new CF medication, Orkambi! I filled out the paperwork while I was at the CF center and now it's a waiting game while the CF team works hard to get the drug for me. The drug works at a genetic level. Essentially, it's meant to repair the mutation that causes my type of CF. Which is the most common mutation and is called F508 delta. My out-of-state CF team explained that, generally speaking, they are not seeing the positive results that they initially anticipated. However, they feel that trying the Orkambi is worth a shot since my personal CF is a much more global type-meaning I have more than just the lung stuff. In short, it may do nothing but it doesn't hurt to try as the side effects are supposed to be minimal. Orkambi it is!!!!

Love, Kristen