Follow Up Clinic

Azer finally had a good clinic day! His weight is stable, and his PFTs were only a little lower than they were when he got discharged from the hospital. The doctor said his lungs sounded clear and not crackly or wheezy. The very dark mucus that he coughed up before he got discharged came back, and there was no bacteria in it, which was interesting, as it was the darkest stuff he has ever coughed up. 

Last week, a few days after Azer went fishing, he started to go downhill again. He began to cough up dark mucus, was exhausted and felt chilled. He didn't have a fever, and it seems that whatever this was went away after a few days. It seems lately that this has been a trend, that he feels worse after spending time around water, or outdoors for an extended period of time.  

His doctor wants him to continue his antibiotic treatment, which would last three more months depending on how he feels. The timing on taking them is only three times a day, so it isn't too burdensome. It also hasn't been hard on his GI. He still hasn't had much of an appetite since the Nissen, but his stomach pain is getting better a little at a time. 

A few months ago, the CF clinic's psycologist brought up the suggestion for Azer to go back on homebound instruction with the school. Normally, this is only an option when a student can't attend in person due to illness. Over this school year, it was becoming increasingly difficult for Azer to stay caught up. He spent so many weeks with a fever, and being so ill he was in bed all day, he fell so far behind, we didn't know what to do. He was also struggling finding the right ADHD medication that worked best for him, and doing Connected Learning while unmedicated was extremely difficult. We are grateful that the psycologist brought up the homebound suggestion. Since then, the school has been more than helpful in decreasing his workload, especially when he's been sick, and modifying his classes to help him get the credits he needs to graduate. They also helped further modify his 504 plan. This has greatly reduced Azer's stress. 

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). 

Sick CF Clinic Visit

Everything had been going great, and we thought we had the summer to relax a little after doing IV antibiotics. He felt great and sounded clear.

Sunday morning, he suddenly sounded a little congested. Within hours, he spiked a fever and was suddenly coughing up brown/rust colored mucus. The CF doctor on call suggested perhaps it was transiet, but to bring him to the ER if his fever got any higher. He took some ibuprofen and the fever broke later in the evening. Yesteday, he was still coughing up some dark mucus, but seemed to feel better. Today, Azer said his legs don't hurt, but they feel uncomfortable, and he has a headache.

At clinic today they did a virus swab to see if he picked up a virus somewhere. He gained several pounds from last week. His lung function was down slightly. His lung culture from last week found that the Steno is now resistant to Levofloxin. In order to treat it, he will have to be on an inhaled antibiotic for a month. The doctor warned us that the nebulized antibiotic smells like cat pee (awesome). They put in an order for a CT scan for in a few weeks. The doctor said if they tried to do a CT scan right now, radiology wouldn't be able to tell if he has fungus spores or not because of the increased mucus.

It will take some time to receive the inhaled antibiotics since they have to come from a special pharmacy. Azer is currently laying in bed not feeling well...

Update: Azer's virus panel came back negative for all viruses, so his symptoms are from bacteria and/or fungus. He was also told not to use his IPV while he is coughing up brown or red mucus, just in case he is having a slow lung bleed.

CF Clinic - sick visit

On Sunday, Azer was by himself watching a movie when suddenly, he was coughing non-stop and was struggling to breathe. He did an albuterol breathing treatment to see if it would stop. Unfortunately, he continued to cough for two full hours. He was exhausted by the time it started to get better. He coughed up the most mucus I've ever seen at a time. At the end, he coughed up some brown mucus. He said his upper airways hurt after this happened. The on-call doctor said to take him to the ER if another attack happened. Luckily, it hasn't happened again.

He went to clinic yesterday to get a PFT and an X-ray to see if there were any significant changes. His lung function was down slightly, but within normal range, especially for it being allergy season. The doctor said his X-ray looked about the same from last time. The doctor wondered if the attack was a mucus plug coming up. Just to be safe, he will be on a course of oral antibiotics.

Azer lost even more weight from last month, and a total of 8 lbs so far. The dietitian came in to discuss what he's been eating, and what else we can do to change this trend. The appetite stimulant medication he is on seems to help, but with evenings being so busy, he isn't able to take it consistently. Even though his appetite has been pretty good, he hasn't been making meal times a priority. They would like to try another appetite stimulating medication and see if it helps him at all. This medication has the possibility of interacting with other medications. They had to do an EKG first before they could prescribe this medication. It took a while to get a good EKG result, because he wouldn't lay still! 😄

In addition to possibly starting the new medication, he has to start doing an extra can of formula. The doctor warned him that people with CF tend to lose lung function along with weight.

No Pseudomonas!!

So, for the past two weeks, Azer has said he felt more congested. He also started coughing up brown to black chunks of mucus in the morning. So, Monday we went to get cultures, PFT's and an IGE level check just in case he had ABPA again. 

He gained 2 pounds in a month, but his FEV1 was down a little from last month. 

A few days ago, we got the test result back from the IGE and it is lower than was last September. We've been waiting expectantly to see if he is culturing something, but today it finally came back negative for Pseudomonas and for Aspergillus! Yay!