CF,  Trikafta

Cystic fibrosis Update

I wanted to jump on here to share a little bit about a medicine I am on for Cystic Fibrosis.

I’m going to throw it back to 2015. 2015 was the year when a drug combination of Lumacaftor and Ivacaftor came out, also known as Orkambi. This medicine helps treat patients with 2 copies of F508del-CFTR mutations, which is the mutation I have. This medicine allows CFTR proteins to open correctly and get to the cell surface, where they are normally located. I tried Orkambi twice. The first time my doctors, parents, and myself thought I was getting sick. I had a rash, flu symptoms, you name it I had it. We stopped the medicine, I went into the hospital for a good clean out, and tried it again. Realized I was highly allergic after that.

In February of 2018, Symdeko was released. A drug combination of Tezacaftor and Ivacaftor. I was in the hospital during this time, I had an infection in my lungs that needed 2 weeks of IV antibiotics. My doctors and I decided to start this medicine after the hospitalization because I was allergic to the IV antibiotics, SURPRISE! I tried the Symdeko twice before my doctors came up with a plan. Because it was just a rash and the medicine wasn’t affecting anything else, my doctors came up with a desensitization plan. This plan was something that no other doctors or CF patients had tried. This medicine was crushed up and mixed with cornstarch in small amounts 3 times a day for 12 days. I got on the full dose and my lung function went up from mid-seventies to mid-eighties.

In October of 2019 Trikafta was released. Trikafta is a combination of Elexacaftor, Tezacaftor, and Ivacaftor. I received the medicine mid November and a week later after being on full dose I started to develop a rash. Once the rash appeared it covered quickly and bad. It took about 2 weeks for the rash to disappear. On January 10th, 2020 I started a desensitization. This time my doctors were trying to introduce my body to the new drug (Elexacaftor) and the old drug (Ivacaftor). Guessing those were causing my body to have a rash. On the last day of the desensitization I developed a rash and once again it covered quickly and bad when it appeared. On February 25th, 2020 I tried it a little different and was also taken off of a medicine that maybe could cause the Trikafta to react. Third time’s a charm, because I finally got on the full dose. I am doing extremely well on this medicine. I have less coughing, more energy, and all around just feel better. 

Today was my first time going to the doctor since Covid-19 broke out and I was a little nervous walking in because doctor offices are where more sick people are, I had to get blood taken (which I am not good with needles or blood), and it’s my first time going to the doctor since the new medicine. With the new medicine, I feel and look really good, but still didn’t know what to except with my lung function test. About 3 months, when I was on no medicine not even the Symdeko, my lung function was at a 79% and today with the new medicine I am at a 93%. I have not seen a 93% in a long time. I honestly have goosebumps typing this out.

Leave a Reply

Your email address will not be published. Required fields are marked *