Brady's Status--Pre-Kalydeco, Age 4 1/2
Kalydeco was approved by the FDA on January 31st, 2012. Brady took his first dose on February 10th, 2012.
Lung Health: Brady had very good lung health. He had always presented with "clean/normal" cultures, and did not have any baseline cough. A chest CT scan performed 1 month after beginning Kalydeco showed "no visible structural damage" to his lungs. The hope was that initiating a treatment like Kalydeco at this earliest stage of lung disease may actually be able to PREVENT disease progression. Prior to beginning Kalydeco, Brady's nebulizer schedule was:
Morning-Albuterol, Pulmozyme, and Hypersal (7%), followed by 20 min. in the Vest machine, for a total treatment time of 45-60 min.
Evening- Albuterol and Hypersal (7%), followed by 20 min. in the Vest. Total time 30-45 min.
Upper Respiratory Health: Brady may have enjoyed good lung health at that time, but had a really tough time with upper respiratory inflammation. Brady had undergone 2 surgeries as a 4 year old--tonsil/adenoidectomy, and an endoscopic sinus clean-out. He had aggressively growing polyps that had completely shut off the air flow through his sinuses. The polyps grew so large that they had begun thinning and shifting his delicate facial bones. A CT scan of his sinuses showed that there was a risk of the polyps actually breaking through the eye socket on one side, and causing potential eye or brain damage. Not cool. The sinus polyps had also completely robbed Brady of his sense of smell. He snored, snorted, and struggled to breathe at night and woke up frequently. Brock and I could hear every breath he took at night, and we also struggled to sleep. Brady never complained about any pain associated with the polyps, but I can't imagine that this felt good.
He took frequent bursts of Prednisone and antibiotics to deal with upper respiratory inflammation/infection and we used a device called "The Nasatouch" to irrigate his sinuses with steroids 3 times a day.
I think the Nasatouch is an extremely useful device, but irrigating a 4 year old's sinuses, 3 times a day, is honestly just one step away from full blown HELL. We basically had to hold Brady down like a criminal and shoot steroids into each nostril--10 seconds on each side. I'm sure it burned and wasn't very much fun...but we did it. Even with all our efforts to keep his sinuses open, the polyps began growing back just a few months after the surgery to remove them. When Kalydeco was approved, Brady had just finished a Prednisone burst for inflammation, but it wasn't helping enough. His sinuses were definitely getting bad again. We had an appointment with his ENT coming up, and we were all dreading the thought of going through another sinus surgery.
Digestive Health: Like many people with CF, Brady had severe pancreatic insufficiency. I can pinpoint the exact day that his pancreas became completely clogged with mucus, and he stopped being able to digest food on his own (when he was between 2-3 weeks old). He stopped gaining weight and began having all sorts of digestive distress. He had already tested positive on the newborn screen for CF by this time, and was diagnosed with CF and put on digestive enzyme replacements within a week. Brady was not born with meconium ileus, but he did have several soft/partial bowel blockages in the first 4 years of his life. Like many with CF, I think he suffered with some low grade stomach pain and irregularity, as a rule. Still, Brady was doing pretty well nutritionally, and his Dr. was pleased with his weight and height at age 4.
Brady's gut functioned best when he was taking approximately 2000 units of lipase/Kg body weight, at meal times.
Then it happened. Our family was on vacation in Arizona when Kalydeco was approved by the FDA, ahead of schedule. We were in a small town called Verrado, just west of Pheonix when we heard the news. Within a week, we had gotten a prescription and insurance approval. We rushed home for a baseline visit with Brady's Dr., so he could begin the drug immediately.
|We looked at dino bones before Kalydeco...|
|Then we did it again 2 years later.|
|We took desert hikes in 2012 before Kalydeco...|
|And did the same hikes in 2014.|
|He fed Giraffes before...(age 4 1/2)|
|And then did it again. (age 6 1/2)|