November 27, 2019

Got Sick, E-rapid and Trikafta

Over two weeks ago, we all ended up with the same virus. Of course, it ended up making Azer's lungs and sinuses all full and congested. He asked the clinic for advice. They asked him if he wanted to come in for a virus panel, but he asked for just oral antibiotics to see how it goes. He started doing more frequent breathing treatments.

After being on the oral antibiotics for about 3 days, he felt like he hadn't improved at all, and was still struggling with his lungs. He asked to be seen, but they didn't have any openings. He asked the doctor what she thought about starting steroids, and she agreed. They said if he got any worse, to go to the ER. He started doing 4 treatments a day. 

Today is the last day of steroids. His cough is still congested and frequent. He has been coughing so frequently, that his ribs are sore. We both feel like we should have pushed for admission at the beginning of the week, since school is out. 

Azer's pharmacy worked out a way for him to get a new nebulizer, called the E-Rapid. This nebulizer works differently than normal compressors. It's virtually silent, and makes the inhalation of medications much faster. He is very happy about it, as he will be able to do nebulizer treatments in the car, or bus quickly. He said that even the baseball coach wouldn't know he was doing a breathing treatment. It has the option of running on regular AA batteries. The nebulizer cups are kind of fragile, and it isn't recommend to use them for hyper-sal as it can degrade the cup quickly. After Azer uses it, he has to clean the cups immediately so medicine doesn't get stuck in the micro-small pores. When the treatment is done, it does leave a small amount of medication at the bottom, which is normal. The "fog" of medicine that comes out is more concentrated looking. 

Now, about Trikafta! So, the insurance approved it and Azer received it yesterday. He hasn't started it yet for several reasons. The first reason is the doctor would like him to finish his antibiotics and steroids first. The second issue is that Trikafta has an interaction with one of Azer's medications he uses for stomach motility and reflux, EES (erythromycin). The doctor said if he stays on the EES, he will take Trikafta on a modified dosage schedule. She said if Azer decided to stop the EES, he would need to wait 2 weeks to start Trikafta on a normal dosage. Azer decided to stop the EES and see if the reflux comes back. If it does, his GI doctor will suggest a different medication. 

This is a difficult thing to wait for, having this life changing medication in your hand! 

November 7, 2019

CF Clinic

A few days before Azer's 16th birthday, he started to feel like he was getting a lung infection. It wasn't improving with allergy medication. The day before his birthday, he said he was so tired of coughing every few minutes. We made the decision to restart Ceftaz. Within a day, he was feeling so much better. For the course of this inhaled antibiotic, he said his lungs felt very clear. The day after the course ended, Azer seemed to have caught some virus. He didn't have a fever, but he had a bad headache and it seemed overnight, his mucus turned very dark brown.

Luckily, he had clinic this week, so we held off on calling the doctor. He started to feel better, and his mucus color was lighter. His PFTs were down today, but the doctor didn't think oral antibiotics would do much right now, but we may need to restart Ceftaz in a month. 

Because of the issue of Zantac being possibly cancer causing and the fact that it will be harder to get it, the GI doctor switched his prescription to a different antacid pill. Azer has been lifting weights and doing off season conditioning with the baseball team. His appetite has been alright. He says he feels thinner, but he has put on almost 10 lbs of muscle. He also grew taller, and is officially taller than me now! 

It was Cape Day at the Hospital today :)

Some other good news is the doctor said Azer qualifies for Trikafta, the new triple combination therapy approved for people with CF who have at least one copy of the Df508 mutation. To be cleared to take it, he needed to have a liver test to make sure all his levels were normal. When he starts taking it, he will need to have his liver tested every three months. Azer also needs to have an eye exam first, as there is a rare possibly of him getting cataracts on the medication (I learned today that cataracts can happen in children who are on high doses of steroids).