January 21, 2020

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!

January 10, 2020

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! 

January 7, 2020

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. 

January 4, 2020

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. 

January 3, 2020

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!