February 27, 2020

Home & Red Balloon Network Fun

Azer was a little disappointed in his PFTs today, but his airflow was pretty good, and he feels like his lungs are really clear. He wondered if the IPV was causing inflammation as he has very little mucus right now. Azer said he almost didn't care what his numbers were today as he was so ready to go home. He was happy to say goodbye to his PICC line today! This was one of the longest hospital stays he's ever had, but he is very relieved to be home.

He will continue inhaled antibiotics for a few weeks. He is glad to be done with IV antibiotics as for nearly three weeks now, he has had constant GI problems, even with Flagyl. We all hope that he continues to stay healthy and that whatever made him so sick two months in a row is gone.

Azer has had a lot of fun with the Red Balloon network for the last few weeks. He's been down in the Seacrest studios a few times. They have been doing more for the kids who are on contact, which is great for getting out of their rooms. 

Azer had so much fun doing a sports type show the first week, that they let him plan out his own show the next week. He found the videos and figured out where to start and stop them. 

Azer even did a few promotional videos for the Red Balloon network. He said even though this was a long hospitalization, that this was the most entertaining stay.  

February 24, 2020

The Current Plan

Azer has wrestled with the decision over the weekend of what to do, whether to stay for another week on IVs or try the EES again.

His PFT was the same as last Wednesday. His airflow was good and he had very little coughing during the test. He was disappointed with the number and felt like it should be better.

One of the fellow doctors that works with his main pulmonologist came in and Azer told her that he feels a lot better, but not completely at his best, and that he just wasn't sure what to do. He mentioned that before Trikafta, he seemed to do well when he was on inhaled Ceftaz, and that his lung function would drop on the off months. She said that in theory, he has been sufficiently treated for whatever has made him sick, but that a little more time might help. The plan is to switch him back to an IV antibiotic that covers MSSA the best, and to start inhaled Ceftaz. If his PFTs are higher on Thursday, we'll know it's working, and he can go home. If he starts to get sick again, he can start the EES and Trikafta on a modified schedule to see if he is aspirating. 

February 22, 2020


Azer thought that the Flagyl was helping, as he was going to the bathroom less, but apparently there was an issue with the pharmacy and he was off of one of his IV antibiotics for a few days. When they restarted it, his symptoms started up again.

They are trying to test his mucus again to see if there is fungus, as it still might be a possibility, but Azer's main Pulmonologist said that the culture from clinic was already tested for everything and it only came back with MSSA. 

Azer talked to his main doctor bluntly today and said that he feels like none of the doctors know what they are treating, and everything is just a guess. He said he was frustrated that he feels better, but that his lung function isn't at his year's best, or even at his baseline. She gave him some options of what could be going on, and that it would take time to figure out what is working and what is not. They agreed that things need to be changed or adjusted one at a time to see what happens. She thinks one of the possibilities is reflux and aspiration. Azer stopped EES a few weeks before starting Trikafta and it could be causing fevers and lung infections. It could still be a virus affecting the lower areas of the lungs, or it could still be some bacteria or fungus. Given that his lung function didn't change with the Albuterol on Wednesday, she doubts that a course of steroids would change anything. 

One of the biggest things that has been worrying is that we all expected his PFTs to be significantly higher after two weeks of antibiotics and Trikafta. The doctor did some looking back at previous tests and noticed the computer changed his expected lung capacity when he turned 16, as it now is basing it's calculations off of the adult stats after his birthday. She did some math and figured that his FEV1 should have been at least a few points higher. She told Azer that it's better to decide if the antibiotics are working based on how he feels rather than be focused on a number.

We also both got off of our chests the expectations doctors and nurses seem to have about Trikafta, including what others experiences with it have been. It seems that most of the doctors we've seen recently don't understand how it works, or what to expect. Obviously, everyone is expected to respond to it differently, but he shouldn't expect his X-rays to look different, and it will not prevent him from having lung infections, nor will it heal his pancreas. She said it is extremely unlikely he would ever have to stop taking enzymes. There has just been a lot of confusion this hospitalization between doctors saying different things, the pharmacy being delayed, and general inconsistency of the staff and schedule. 

So far, Azer decided with the doctor to stop the IV antibiotic that's making his stomach upset, and to change the remaining antibiotic to one that would be easier to manage at home. Aside from the numbers Azer wanted to see, he is feeling better. The doctor said that there is no guarantee that a third week of antibiotics would prevent him from getting sick in a month, but she is leaving it up to him to decide what he wants to do on Monday. 

February 19, 2020

Admission Questions

Yesterday, Azer and I had a long conversation with the CF doctor for this week. She said he is on the path to a bronchoscopy, but not quite there yet. Azer asked why they are treating something when they don't know what it is. So she explained that they are treating what they see. She told us we might not see anything different culture from the bronch. We brought up the possibility that the hypertonic saline might be irritating his lungs, so she said it was fine to stop that. She said she would order a PFT for today, pre and post Albuterol.

Today Azer blew only one point higher after inhaling Albuterol. The doctor was a little disappointed with the results, as she was expecting to see a higher number after Albuterol. She thinks his lungs have a strong inflammation component and that it will take time for them to calm down. She said before gene modifiers, they would put patients on a very high dose of ibuprofen to reduce inflammation and their lung function would improve. Azer coughed out some very dark mucus that she saw, and she believes it's old mucus still coming out. She said during bronchs, they flush the lungs with saline, and tons and tons of old mucus comes out at once. She said that Trikafta is likely doing the same thing, but a little at a time, and it's coming out as if in layers. It seems difficult to get much information on whether the off and on fevers and lung infections are due to Trikafta. 

So far, the doctors are undecided on whether to continue antibiotics for a third week. His appetite seemed better last night, but his GI system as still been very upset. They may start Flagyl today.

February 17, 2020

Staying Another Week

His PFTs were exactly the same as last week. He feels like his lungs haven't improved since Thursday. They are better than the day of admission, but he said they still feel irritated. He feels discouraged that his numbers are not higher and that he's not feeling better than he expected to be by now, even with two strong IV antibiotics. They are still causing an upset stomach, and he still hasn't been able to eat more than few bites of food a day. 

We made the decision to stay in for another week instead of going home on IV antibiotics. He is getting good lung therapy with the IPV, which we don't have the advantage of using at home. We are still struggling to get formula and G-tube supplies at home, so he will have time to get his weight up with extra formula while admitted. He missed a dose of Trikafta last week, and noticed the effects quickly, so it is doing a lot more for him than we realized. There still isn't a clear answer on why he got so sick only a month later, without anything new showing up in his cultures.

In mean time, he has been entertaining himself with Legos, crafts, and has been on the Red Balloon network a few times (a channel broadcast in the hospital). He even got to meet one of the Harlem Globetrotters. Azer would appreciate any type of arts and crafts that would keep him occupied.

February 13, 2020

Still Not Feeling Great

Azer had his first PFT today since admission. It is close to his baseline, but he said lungs still feel very irritated. The hyper-saline caused so much irritation this morning that he coughed for hours. He was exhausted by the time they came for PFTs. He asked the doctor if he could either stop doing it, or do a lower percentage.

The night of admission, the nurse who tried to put in his IV didn't do it quite right, and it hurt him so bad, he was sweating. The IV team was able to put it in quickly and painlessly. The PICC team had to put his PICC line in on his other arm this time, as the arm they usually use is still sore from the PICC line last month. He had an IV and PICC line on the same arm that day.

Most of the tests for Aspergillus came back, and they are all lower numbers than he's had in years, so the Dupixant is working wonderfully. His viral panel was negative, so they are just treating the bacteria he has cultured in the past. This doesn't really give a clear answer on why he is so sick a month after his last admission.

Everyday, it seems he's had less and less energy. He has had no appetite at all. The antibiotics are upsetting his stomach very badly this time. He says he hasn't been sleeping well. Even though his lung function is up, he isn't feeling well overall. He just seems to be feeling worse since he got admitted. We are hoping that the Trikafta isn't causing his decline.

February 10, 2020


After last clinic visit, Azer felt like his lungs were gradually feeling worse. He missed two doses of his Dupixant shot before insurance finally approved it. He feels it has taken a while for it to build up in his body. Last week, he feels like he caught a virus a school. His legs hurt, he had an off and on fever, he was exhausted, and he said his lungs felt really irritated. He said he had to breath shallow in classes because otherwise he would have a bad coughing attack. He would have coughing attacks so bad he was doubled over, having difficulty taking a breath in. He was doing Albuterol way too often just to stop coughing. He was waking up with his ribs and scaps aching. His mucus has also been brown. 

We called the on call doctor yesterday who urged him to come to the ER for an admission since he was doing Albuterol so often. He decided to wait for clinic today. He had difficulty trying to complete his PFT without coughing. He was lower than his clinic visit and has also lost 4 lbs. The doctor believes that the Trikafta is not to blame for his decline, but that it is just a bad combination of viruses. They did a virus swab and CF culture today. Azer had the option of going home on oral antibiotics for a few days to see if it works, but he decided he has been too miserable to wait and see. He says he feels like he's had a fever off and on for two weeks now. He started getting a fever again at the end of clinic.