Kalydeco is a life changing drug for cystic fibrosis patients with the G551D mutation. Only 4% of the 30,000 people with CF carry this mutation too, so we are definitely blessed. This drug is the first one that actually fixes the root of the problem, doesn't just help a symptom! It is taken every 12 hours. I've chosen 7:30am/pm. We finally got the ok from the doctor to crush the pill since we have had no luck with Lily swallowing pills, and need this miracle NOW. The price of this drug is a whopping $294,000 a year. Why so expensive? It was just FDA approved January 31, 2012! Since Lily is only 29 lbs, she only needs half the dose right now.
Ok, so February 1st at 4pm, the truck arrived and we finally got it! It was such an amazing feeling to hold it in my hands. At 7:30pm Lily took her first dose. Since it has to be taken with fatty foods and Lily doesn't eat much, I came up with a special concoction... 2 ounces of her Pediasure 1.5 & a tablespoon of peanut butter. It equals 12grams of fat which meets the required 10grams. I crushed the pill, mixed it into applesauce & shot the whole recipe through her g-tube. So easy!!!
The next day (yesterday), she got her 2 doses at exactly 7:30 & 7:30. After her last dose for the day we went over my boyfriends parents house for dinner. She ate some dinner by mouth, and started to play. She played, jumped, flipped, wrestled, and ran around for hours. It was incredible to watch. She didnt cough and she wasn't out of breath one bit! I don't know if this was the Kalydeco already working or if her recent hospital stay & PICC line antibiotics just have her feeling really good. I'd like to think it was the Kalydeco though :)
This morning, February 3rd, she just got her 4th dose and I am so excited to see how the day plays out :)
Lily Brooke on K start date:
Age: 5 years, 7 months
Weight: 29 lbs
Height: 40 inches