Kluber's Kid

Last njght, Azer got an awesome planned surprise. The Texas Rangers pitcher Corey Kluber has a foundation called the Kluber's family foundation. They made our family into DoppleRangers, brought us dinner from a restaurant of our choice, brought all kinds of Rangers goodies and even showed us a video recorded especially for Azer from Corey Kluber and his wife!

Unpleasant Surprise

Well tonight Azer's main GI doctor called saying that she reviewed the results of his impedance test. She said 50 or under events of reflux is normal. Azer had over 90. A large percentage of those events were when he was refluxing all the way up his esophagus, meaning he is very likely to be unknowingly aspirating. She believes the next step is a Nissen surgery. She is setting up an appointment for him to meet with a surgeon that is familiar working with CF patients. 

He's Home!!

Today was a day of a lot of uncertainty. It was doubtful he'd come home today, as they were still waiting for his Voriconizol levels to come in. They came in late this afternoon. The levels are too low, so they are increasing the dosage orally. 

A lot of the decision to discharge also rested on how his lung function looked. His PFTs today were 6 points higher than last week, so that was the big improvement we were all hoping to see. His oxygen has still been lower than normal at times. 

Azer's psychiatrist worked with the doctors to try to find another non-stimulant medication that wouldn't interact with the antibiotics or antifungal he's taking. They agreed upon one, with the only possible interaction being a rare effect on heart rhythm. The risk is very low, but they did an EKG prior to make sure his heart beat looked normal. They checked it again before increasing the dosage, but so far he feels like it's beginning to work.

The Voriconizol has still been messing with his vision, particularly his perception of colors, but it has been infrequent luckily. He has had no other side effects thankfully. The main downside is he can only take Trikafta twice a week while on the Voriconizol.

The impending study also came back late this afternoon. It shows that he is refluxing and that he is at risk for aspirating. Unfortunately, the medication they'd like to put him on has a conflict with his ADHD medication and his antifungal. He will have an appointment with his GI doctor to discuss this further. 

Azer has been gaining weight well, even though he hasn't been very hungry at all. He thinks it's partly due to how hard he's been working in PT, and that he's been putting on muscle. 

After three weeks of being in the hospital, he is so happy to be home, ready to put this hospitalization behind him, and baseball ahead!

Treating for Fungus

 The doctors started Azer on an antifungal to treat the aspergillus. The ID said that if the aspergillus turned resistant, that he would be ineligible in the future for a lung transplant, so they need to make sure they get all of it. It still is a mystery why it hasn't shown up before. The blood test for aspergillus was negative but different type of test came back positive for a fungal infection. They stopped one of the IV antibiotics and added an oral one. He is staying admitted for sure through the weekend. 

PFTs on Thursday were a few points higher than Monday. The antifungal medication is already causing his vision to be blurry, so they are having to run it slower. 

Azer had another appointment with his psychiatrist yesterday to discuss if there was another ADHD medication he could try that wouldn't interact with the antibiotics or antifungal. They have to run it by the pharmacy first to make sure. 

Impedance Study & Confusion

 Yesterday, since Azer's lung function was not better, they made the decision to move ahead with the impedance study. It was very uncomfortable for him when they put it in, and for hours after. They did an x-ray to make sure it was in the right place, but they had to adjust it again. It made breathing treatments harder, as coughing made it move around and he could feel it. After he ate something, he said it felt a lot better, but he could still feel it when he swallowed. The device attached to the catheter had buttons on it, and he was instructed to push one when he took medication, another when he ate, and another one if he ever experienced reflux or heartburn. The pH levels were real time, so he was kind of having a bit of fun seeing if what he ate or drank changed the numbers. 

It was in for 24 hours, and they pulled it out this afternoon. The results should be ready tomorrow.

He couldn't do anymore school work yesterday, as it had been a difficult enough experience. School work and even doing fun activities like crafts has been a struggle for him without any ADHD medicine.

We saw Infectious Disease today, who went over what all this could possibly be from their perspective. ID doesn't want to treat the aspergillus, because they said it would guarantee it would cause it to become resistant in a few years, if not sooner. The ID doctor also said that Azer's CF doctor should have done a biopsy during the bronchoscopy to physically check for fungus. The CF doctor came in soon after and said she really wants to treat the aspergillus, and that they don't do biopsyies during CF bronchs. 

The downfall of treating for aspergillus is they may have to completely stop the Trikafta for the 6 weeks of treatment with the antifungal. Also, the levels of medication have to stay the same, which usually means weekly lab tests. The last thing is the antifungal medication can cause the liver issues, so they have to monitor that and sometimes have him take Ursodial with the antifungal. ID is very against treating the aspergillus, and says many more tests need to be done before going ahead with the treatment because of all the risks. They said that from the CT scan, at first it looked like it could be either fungus or mucus. After looking at it closer, they said that the areas had improved in some places and gotten worse in some, so it moved around, meaning it was mucus plugging, not fungus which does not move around. The ID doctor said that his blood tests are all negative for any signs of ABPA, and this is likely a big benefit of the Dupixent he's been on for over a year.

The CF doctor also said that this could be his new baseline, but Azer really disagrees with this. 

Lung Function the Same

 The doctors wanted to start Azer back on Hypertonic saline. They saw some plugging in his lungs on the CT scan, and wanted to try to work on clearing it out. They started him slow on 3% hyper-sal and then tried the 7%. He is doing well with it. Luckily this time it's not causing him a major coughing fit. He is continuing to do the IPV three times a day. 

They added a possibility of a sensitivity of tegaderm to his medical file, because of sudden itching he had to it. They also added his reaction to the Levoquin. His right wrist has improved, but his left still hurts pretty bad. Ibuprofen and heating packs have helped, but the doctor has mentioned she might make him wear a wrist brace. PT has been great with working around his wrist pain, and keeps him motivated during every session. 

Both of his PFTs last week were exactly the same as today, so there has been virtually no improvement in his lung function, except that he feels like his lungs are clear. 

They may start Voriconizol for the rare growth aspergillus. 

Thank you to HopeKids for bringing Azer some goodies and crafts!! 

Change in Antibiotics

 Azer had a CT scan yesterday, and it didn't show any changes from last year. They plan on keeping him on IV antibiotics until his PFTs are back to normal. 

Azer ended up with bad itching from his PICC line dressing. They had to change it to a kind with a different type of adhesive. They suspect his body developed an allergy to the usual type because of overuse. 

The last time Azer was on the antibiotic Levoquin, he ended up with tendon pain in his wrists. It happened again this time. He told the doctors about it, who told him if it got worse, they'd change it to something else. It got worse, and he really noticed it in PT. They gave him some ibuprofen, but it didn't improve. So today, they switched him to a different antibiotic. It took them a while to get it, as it isn't a common medication. 

He says he's really been enjoying PT. They have added a lot of exercises, and made it more intense, which he loves. He has been sore after working out, so he knows he's getting effective therapy. 

Getting There

 Azer feels like his lungs are almost perfect. His PFT does not reflect that, as it is only a few points higher than last Thursday. 

It sounds like they will for sure do a CT scan sometime this week.

This morning, the entire GI team came in to listen to Azer's concerns. They gave him the option to do the impedance study here or at home. He hasn't decided where he wants to do it, but the important thing for him is that for the 24 hours he has it in, he needs to try to eat as normally as possible. This is difficult right now because his stomach is still very upset. 

He is frustrated trying to tell multiple doctors why he isn't hungry, but they may start another antibiotic to help his stomach settle down. 

The impedance study will show whether he is silently aspirating. If he is, his GI doctor said the best next step would be a fundoplicatin surgery, to physically stop the aspiration and reflux. She explained that he has been on so many GI medications through the years, and that if he is refluxing this bad, that surgery might be his best option. 

Azer asked them that if it turns out it isn't reflux, what could it possibly be, and what the next steps would be. They are unsure right now, but they are trying to work on crossing things out, if what it might not be. 

Azer got a surprise today from some fellow CF friends today! They sent some warm cookies! Thank you!! 😊 🍪 ♥️

Clear Lungs, But Not Back To Normal

Azer's lung function is improving, but his oxygen is only optimal right after breathing treatments or physical therapy. Azer says his lungs feel clear, but that his lungs or body are sick. He isn't confident going home with IVs and feels like it's better to finish out the course at the hospital. We are still hoping they can figure out why he keeps getting sick over and over. 

Blue Caboose brought Azer some goodies and this 3D puzzle that he really enjoyed putting together. The WiFi at the hospital has been slow, so gaming on the Xbox has been difficult. 

Feeling a Little Better

Azer is feeling a little bit better today. He has gone to PT twice so far, and he feels like that is making a difference. His oxygen was stable last night, so they are only spot checking it today. The antibiotics are making his appetite pretty poor. His cultured showed rare growth of some type of fungus, so he's probably not going it, but the cultures are still pending. His IGE is again at an all time low, and is within the normal range for someone who doesn't have CF. We are definitely not comfortable with going home on IVs at this point, not knowing what is going on.

Azer had a video appointment with his psychiatrist today. He has had a very difficult time with any stimulant he has tried for his ADHD. It seems to cause a cough, excessive sweating and an upset stomach. The psychiatrist prescribed a non-stimulant, but he hasn't had a chance to start it yet, because he got admitted. She was able to video chat with the doctor in his room, and ask that he could be started on it while he's in the hospital, since he still needs to stay up to date with his schoolwork.

Schooling online has been difficult, as he is often too busy with treatments to check in at the scheduled time. After calls with about 4 different high school staff, it sounds like he will be counted absent until the end of the semester, when they can officially enter the paperwork from the hospital. 

Day 1 of Hospitalization

 

Azer's oxygen was low last night, so they put him on supplemental oxygen over night. He said the nasal cannula was really uncomfortable. His oxygen saturation has gotten better during the day today. 

The GI team came by, and they doubt that this is reflux or aspiration. Just to be extra sure that it isn't GI related, the day before he goes home, they will do a 24 hour esophageal ph test. This is where they will insert a special NG tube in to monitor reflux. A benefit of this is, even if it isn't reflux, they will be able to see how acidic his GI is, and might be able to taper him off of some of his GI medications. 

His sputum cultures are still pending, but all of his blood tests so far look really good. So far, there are no answers as to why he's so sick. He is trying to stay caught up with his classes, but it is difficult with so many treatments going on. 

Low Oxygen

Azer's oxygen is low, and lung function is almost 30 points down from his year's best. His lungs sound congested and crackly. The RT said he's breathing shallow and fast. She's never seen him look this bad. They are continuously monitoring his oxygen saturation, especially while he sleeps tonight. If he's not able to keep his oxygen up, they may put him on oxygen tonight. 

The doctors put in an order for two IV antibiotics, but they are to treat bacteria he used to grow. They are getting a culture today to see if he's growing anything new. He tested negative for all viruses including Covid 19, so it's not a virus making him so sick. He shouldn't be this sick after doing two weeks of antibiotics a month ago. 

They don't know what it is that's making him so sick over and over. The PICC team tried to see if they can use his left arm, as his right arm has been used so much this year, it is causing the vein to scar much quicker. Unfortunately, they can't use the vein in his left arm. The PICC team was very patient and showed us that on the ultrasound, the vein disappears further up the arm. I asked a lot of questions about what would factor into the decision of getting a Port, since it was mentioned today, because of the fact that this is his 4th PICC line this year. Hopefully the PICC line goes in easy. 

Admitted Again

About two weeks ago, it seemed like Azer was going down hill again, the same way as has been happening all this year. He began becoming very fatigued in the morning to early afternoon, very cold, and increasingly congested. Saturday, he had a fever, and his lungs felt very full. His heart rate was high, and oxygen was low. The on call doctor said if the fever got worse to go to the ER, but he was stable through the weekend. This morning, his lungs felt so full, he could barely speak without a coughing attack. He has been coughing up a lot of mucus. It is extremely frustrating to feel like he is back in the same place as a month ago, with no answers at all. His cultures from his sinus surgery and bronchoscopy still don't show any slow growing bacteria or fungus. It was always that the bronch would be the final try to see what was really going on, and we feel stuck now. We are all hoping for relief for him and some insight.