December 28, 2019

Week 3

Azer said before Trikafta, pulmozyme felt like a lotion to his lungs after hyper sal, which can be irritating. He noticed lately that when he uses pulmozyme in his E-rapid nebulizer, that it's very irritating and makes him cough a lot. It's almost like it's too strong. He is ok using it in his normal, slower nebulizer. He says that he can actually tell the Albuterol is working. Before Trikafta, he didn't feel any effects from it at all. 

Azer says it's so weird to feel like his lungs are hollow, not dense. It feels like weight has been taken off his chest. That there is space there that wasn't there before. 

Monday, Azer went for a run. He did an Albuterol before, as normally the cold air is irritating to his lungs. He was able to run without having a coughing attack, and felt like his lungs were calm!

When it gets close to the time for the next dose of Trikafta, Azer said his mucus starts getting thicker and he feels sinus pressure starting to come. Oddly enough, he also feels like his lungs feel better around this time as well.

This week the skin on his fingers started to peel, and his lips were pretty cracked. Now that it's the end of the week, his lips are getting better, but almost all his finger tips have peeling skin. He says that it seems to happen if he sweats a lot, and also when his lungs are getting sick. 

Azer is still continually rehydrated. His is sweating so much, and is still having watery stool, sometimes several times a day. Some nights he has a hard time sleeping due to constant coughing. His sides and ribs hurt from coughing so much. The upside is his energy level is nearly normal now. 

December 22, 2019

Week 2 of Trikafta

Day 8-9 he has had bloating so bad, that he can't run without pain. Still having constant coughing. He wakes up with burning lungs. He slept with a wedge pillow and didn't wake up coughing or with burning lungs. Hypertonic saline has been making him cough a lot more.

Day 10 he has had a fever off and on at school. It was difficult for him to finish his finals today. The doctor prescribed Flagyl three times a day to see if it helps the bloating. They suspect his constant coughing is from nasal drainage, since cough drops help. 

Day 11 The flagyl might be helping. This morning, he had a lot of bloating and fullness, but this afternoon he ate lunch and said the bloating feeling was gone. He isn't coughing quite as often. He says stopping the hyper-sal has helped a lot. He wants to continue taking one less enzyme per meal, until his digestion gets more consistent. He has ended up losing 7 lbs so far, which is a bit discouraging.

Day 12 His stomach has still been bloating, but not as bad as before. He is still feeling full after eating only a little. He made it through his last day of school. It was early release, and he slept most of the afternoon. His cough sounded much more wet and congested, but his lungs sounded very clear, with no wheezing sounds. 

Day 13 he has slept quite a bit during the day, and has had a mild fever off and on. He has coughed up some brown mucus. He has been drinking Rehydrate and water continuously, and still feels dehydrated. His lips have been dry and cracked. 

Day 14 He slept for hours during the day, but says he can feel himself breathing deeper, that it's easier falling asleep, and that he feels that the Albuterol is more effective than it's ever been. His nose has been bleeding and dry. He said before, it's always been gunked up with mucus during the winter, and he isn't used to it being dry. He is back to his normal dosage of enzymes because since last Saturday, his stool continues to be watery. 

December 15, 2019

Day 7 of Trikafta

Azer was woken up by severe pain in his upper right abdomen. He had a normal bowel movement, but just in case there was a blockage, he had a cap of Miralax and plenty of liquids. This didn't seem to help, so he took some ibuprofen and put a heating pack on his abdomen. The pain was so bad he wasn't able to finish his Vest treatment. The pain got better but didn't go away completely. He didn't feel well enough to go to his baseball lesson. After lunch, the pain came back as bad as it was before. It seemed to ebb and flow, it would come on sharp and fast, and he'd be doubled over with pain, and couldn't sit still, it was so bad. This time ibuprofen and the heating pad didn't seem to help much at all. I called the on call doctor who said it might be gallbladder, and that he should avoid fatty foods tonight. She also called in a stronger pain medication. Unfortunately, 5 minutes after the medication was ready to be picked up, the pharmacy was closed. I called the doctor back, and by this time Azer said that the pain was so bad, he was starting to sweat. The doctor felt like the best thing was the ER. We always have had the joke that the double doors at the hospital are magic; that as soon as he walks through them, he suddenly feels better. It didn't work this time.

We were taken into a room fairly quickly given the risk of CF patients being in a waiting room with sick people can be life threatening. They put in an IV to take blood samples. Azer got a sonogram and an X-ray. His organs visually looked good. One of his liver enzymes qasy a little elevated, but not high enough to be a concern. All his labs came back normal, except for his potassium, which they had to draw again for. This is when the IV started to not want to work, and they had to do a lot of wiggling around on it to draw blood out of it. They eventually had to poke him again in a different spot.

The X-ray showed he has a blockage in his small intestines. Azer said with the diarrhea he had earlier in the week, he was wondering if his enzymes weren't working as well, and increased them slightly. Trikafta can also cause enzymes to work more effectively, so this might have been another reason for the blockage to have happened. 

They consulted with a surgeon who said it didn't look like a blockage that needed surgery but more like an impaction and felt like it could be remedied by medication. It worked very well, and within a half and hour, he had no pain at all, and was starving. 

He will cut back on enzyme dosage for a few days to gauge how many he needs, which is unusual as he usually has to increase enzymes! This wasn't a great way to spend a Saturday, but he is grateful to be feeling almost 100% and that he didn't miss school due to this. Azer says his lungs are feeling so much better, and that he feels like he can take a deeper breath and he could before. He is coughing frequently and it is productive each time, but the mucus is so thin, it comes out easily. Even though this hasn't been the best week, he still thinks Trikafta has been worth the side effects.


Azer finished his antibiotics, and it has been two weeks since he stopped his EES. He has had very little reflux since stopping the EES, so that's been a nice change.

Sunday was the first day Azer took Trikafta. He didn't see much change other than he felt like his mouth was full of spit. 

Day 2, he thinks his mucus might be a little more watery, but he says his mouth is coated in mucus/spit. He woke up without having a doubled over coughing fit for the first time in about a month. He started to feel dehydrated at baseball practice. He had a headache when he went to bed. 

Day 3, mouth still coated in spit, he feels like he doesn't have to work as hard to being mucus up. Everytime he coughs, he feels like some mucus comes up. He says his lungs feel agitated from nasal drainage today. His nose is running a lot. He said his mucus has been very clear. He says if he did a PFT today, it would be a really good number. Eating seems to be different for him. He says it's hard to eat quietly as his mouth is full of spit when he's eating. He also says his eyes feel weird, like they are wet. 

Day 4, Azer feels like "the Purge" has officially started. He is coughing constantly and it is sounding wet and congested. His nose is running constantly, and he was worried he was annoying people in school with his constant cough and sniffing. 

Day 5, woke up in the middle of the night coughing so hard he almost puked. He blew his nose and something black came out. Everything else has been pretty clear. He's had diarrhea for the last two days, but it seems like it's from a gastrointestinal virus. 

Day 6, he is coughing constantly, bringing mucus up every time. He is coughing so much and is very tired, he decided to stay home from school. He has a low grade fever and lower back pain. He said he's had numb finger tips off and on since yesterday and he feels weak. It seems like he is dehydrated and maybe low on potassium. He ate several baked potatoes and drank several bottles of Rehydrate and felt back to normal.