Week 6 and Clinic

Azer still felt good even through the switch to oral antibiotics. Going home without a PICC line was worth it, as the very next day he got discharged, it actually snowed, and he had a blast sledding and playing in the snow.

He hasn't been coughing as much, and isn't bringing up much mucus during treatments. He has gone back to doing hyper sal twice a day. He coughed up sort of bloody mucus one day and other days, dark mucus. His cough continued to be clear even after he finished his antibiotics. He hasn't been able to laugh without having a cough attack in a very long time, but this week, it has gotten so much better. Through out week 6, he got off and on pain in his chest, and he couldn't tell if heart pain or lung pain, but it was most likely a mucus plug moving out.

Insurance is requiring us to switch his Dupixant to another pharmacy or they won't pay it. We have been fighting to get it, and unfortunately he ran out of it and has been experiencing increased mucus and allergy symptoms, which proves how much it was actually helping. When he was admitted, they tested his IGE levels, and they were lower than they have been in 8 years. The Dupixant has helped immensely, so it is frustrating to not be able to have it right now. They are also requiring us to switch his Trikafta as well, and he has a little over a week left, so I am really hoping it gets transfered before he runs out. The doctor assured us that other people with CF that have struggled getting their Trikafta due to insurance have done pretty well being off of it for a week. We are also having a hard time getting his growth hormones covered, as he hasn't grown much at all in the past year, and according to the insurance, there is no reason for him to continue being on them. 

Azer has been continuing to do 6 cans of formula a day, and he has slowly been regaining the weight he lost. He feels like the Remeron is helping, as he actually has an appetite now, even when taking his ADHD medication. He has had some painful reflux since he started eating solid food though. The GI doctor is increasing his enzyme dosage and also temporarily doubling his Nexium dosage.

His PFTs were lower than when he was discharged. This is most likely due to his allergy symptoms from not having his Dupixant shot. One thing that was different on the test was that normally the slope of his exhale shows lung restriction. This time, it was showing less lung obstruction. The doctor was so pleased with how he sounds, she said he can stop taking one of his inhaled steroids. There is a study starting soon that will show what other medications people with CF can stop taking. They are doing a virus panel to see if his dark/greenish mucus is from the rhinovirus still lingering. The doctor said the virus is presenting in a flu-like way this year, making even healthy people pretty sick. If his lung culture shows anything other than MSSA, she wants him to start I

inhaled antibiotics for a month. 

Azer had his annual PT evaluation, and his capacity on his test was lower than last year. He also has regressed on his posture so, we may need to start out patient PT again. Azer has had some tendon issues in his left wrist. It's not too severe, but he does have to hold off on some pitches for a while. The doctor said it's possible that the antibiotic he was on in the hospital caused this to happen. 

Azer mentioned to the doctor that he has had numb and tingly fingertips since starting Trikafta. The doctor figured out that it is aquagenic wrinkling, since the tingling is happening when his hands are sweaty. In some people with CF, aquagenic wrinkling can even be painful, which Azer has experienced when swimming. The doctors took note of all the Trikafta experiences, good and bad, as it helps Vertex see how it affects everyone differently!

Going Home!

Thursday, Azer's PFT results was three points higher than Monday. He was expecting it to be higher, but the weather has been warm and humid, and he figured he's being affected by allergies. His lungs have been sounding pretty clear, and he isn't coughing up very much now. He can laugh without being doubled over in a coughing fit. The doctors were surprised at how fast his lung function went up after only a few days on antibiotics.

Since they stopped the Miralax, he's been going less times a day, but still lose. They did a stool viral panel, and it was clear. They are running a test to see how well his enzymes are working  He still is having no appetite after increasing his Periactin dose. The GI doctor had suggested trying Remeron a while ago, and did an EKG, but at the time, Azer's appetite came back before we started it. They suggested trying it out this time, so they redid the EKG which was normal. So far he hasn't seen any increased appetite, but Azer is pretty sure his lack of appetite is from his ADHD medication. His BMI is still around 50%, and the 6 cans of day of formula is keeping his weight stable, so they aren't too concerned. 

They decided that he is doing so well, he can go home today, and to just to a week on oral antibiotics. It is unusual for him to have his PICC line taken out before even one dressing change! Both Azer and I asked the staff again if they could ask our main CF doctor if this plan is ok with her, just because this is not normal for him.

Azer is very happy with how good he is feeling and how quickly it was for him to recover from this illness, especially for how bad he was feeling for several months. He is ready for baseball season! 

Week 5 and Admission

PICC team said that because Azer has had a PICC line over and over in the same area for years, the vein has become scarred and this may be why he has had pain and bleeding at the site last time. He has had minimal pain with it this time. He really tried to move his arm around while it was healing.

Because of the positive virus test, he is on droplet precaution, so he can't go to the playroom or walk around outside the room, but it's been very nice outdoors, so he goes out as much as possible. His favorite outdoor activity is wiggle ball (swinging only with the non-picc line arm of course 😉).

Azer really hated being continuously hooked up to the IV fluids, so he has been working hard to drink lots of fluids, and got off of continuous fluids, so he is only hooked up three hours at a time for antibiotics.

His diarrhea started to come back last night, probably due to the antibiotics. They are backing off of the Miralax twice a day. The GI team came by to work out a plan for getting his appetite back, and want to do a fecal test to make sure there isn't any unusual intestinal bacteria and check for how well he is absorbing fat. They are increasing his Periactin to help his appetite. It seems his ADHD medication may be dampening his appetite during the day, as around bedtime, he gets a little hungry.

His PFT test yesterday went very well. He is only a little lower than his normal baseline. The doctor wants Azer to stay at least until the end of the week to see if his GI symptoms improve. 

Day 1 of Hospital Stay

Last night it was too late to place a PICC line, so he had an IV placed to start antibiotics. Since he is off of EES, he can now use Levoquin in addition to the other antibiotics to treat the bacteria he usually cultures. Some of the IV antibiotics burn going through the IV, so he was happy to get the PICC line today.

He is still struggling to eat anything. They did an X-ray of his abdomen last night and noticed he might be getting backed up, so they added Miralax again to avoid any blockages. He is also not taking in enough fluids, so he is on constant IV fluids right now.

The respiratory therapist changed up his vest settings a bit different, and it seems to be shaking the mucus up. He is also able to use the IPV that he likes so much, since it brings a lot of mucus.

Azer is concerned as baseball tryouts start next  are week.We hoping for good PFTs next week. This is the lowest lung function he has ever had in his life. 

Week 4 and Admission

The week started well, with Azer feeling more energetic but still coughing a lot and zero appetite. Some days, he only had G-tube formula and milk. As the week progressed, his cough became worse, to the point where every time he coughed he would have a coughing fit.

The diarrhea continued until Thursday, when it seemed to finally end. Thursday, he had a low grade fever, and felt much worse, so he contacted the CF clinic. They made an appointment for him Friday. 

He was miserable when we arrived. He said his lungs felt uncomfortable, and he had to breathe shallow just to keep from constantly coughing. 

At clinic, they confirmed his rapid weight loss. The dietician recommended that if he just can't eat anything, his weight loss will continue. She prescribed an extra can of formula during the day, which would make it 6 cans a day, and should meet his daily caloric intake. 

His heart rate was elevated, and he had a low grade fever. His first PFT test was about 30 points lower than his baseline. After an Albuterol treatment, it was slightly higher. It was very difficult for him to complete the PFT test, as he could barely make a complete exhale without coughing. Both the doctor and Azer felt like he never fully recovered from his lung infection that began before he started Trikafta. Two weeks of antibiotics and steroids hadn't touched it, and we were waiting to see if the Trikafta would clear it up, which did not happen.

The doctor said his lungs sounded fairly clear. She said his symptoms and color of mucus really pointed to the possibility of a virus that could be making a lung infection even worse. Azer decided it's best to be admitted for IV antibiotics, to clear up his lungs and also to judge if any of his symptoms are being caused by the Trikafta. The floor was very full, so they sent us home to wait for a bed to be available.

Hopefully they get him feeling better quick he's not in the hospital too long! 

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. 

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. 

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.

Trikafta

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. 

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! 

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). 

16 Years Old

I cannot believe I brought this amazing person into the world.

In so many ways, I feel like I have grown up with him.

Through the years, we have both learned about CF together.

I have watched him develop a strong passion for baseball.

He has grown into a funny, caring, loving person. He has matured in many ways. He has become responsible in taking care of  himself and all his treatments.

Azer is strong in more ways than one, and advocates for himself.

Happy 16th Birthday Azer!