October 17, 2020

Doing Better




Azer is doing ok today. He is very sore, especially where the incisions are. They are keeping his G-tube venting to relieve some pain in his stomach. They are trying to switch him over to oral pain medications from IV medication, but his pain levels are still high. He isn't using the vest yet during breathing treatments, but he was able to walk around a lot more than yesterday. They are restarting his Hypertonic saline. 

He had to wait a while to try out some soft foods. They had to clear it with the speech therapist first before he could have anything beyond ice chips. He isn't very hungry, even though a lot of things mentally sound good. His G-tube feeds seem to be causing pain, but he was actually able to eat some mac and cheese this morning. 

Overall, he's doing a lot better than yesterday, even though he's still in quite a bit of pain. 



October 15, 2020

Surgery Done

The ENT doctor finished his part quickly. He injected some gel in the area where the cleft is to temporarily swell it up. This gel will get absorbed in a few months. If the problem returns, Azer may need further surgery.

The Nissen surgery went well. He has 5 incisions but the surgeon didn't need to move his G-tube, which was Azer's biggest fear. He won't be able to eat solids for a while, but he can start on liquids and soft foods tomorrow. 

He is in recovery and doing well. 

Swallow Study and Surgery

Azer had fun with trick shots yesterday.





So the swallow study results came back. It showed that he isn't aspirating when he swallows, but an ENT doctor will be doing a temporary repair on it during the same surgery as the Nissen. If Azer starts to go downhill again, the ENT doctor can perform a more permanent repair. 




Azer was put NPO last night tentatively for surgery today. It looks like it will happen for sure very soon this morning. 


October 13, 2020

Surgery Not Done

So, in one of my previous posts, I said that the surgeon explained he would do a bronchoscopy before surgery to check for any birth defects. It turns out Azer does have a small birth defect, called an LCET, a Laryngeal cleft, that could cause what he is swallowing to go down into his lungs. He said the type he had was Type 1. The surgeon consulted an ENT doctor to confirm visually this is what he was seeing. The surgeon is holding off on doing the Nissen for now, as he isn't completely sure if Azer needs it.

Azer is waking up from the bronch now. The plan is to do a swallow study to see if the cleft is causing him to aspirate. If not, the surgeon do the Nissen. 

Here is a link explaining what Azer has.




Surgery Happening This Morning

It was very unpredictable when Azer's surgery would happen, as the surgeon also does emergency surgeries, so it wasn't even known what day they would be able to fit him in. They were really trying to push the surgery earlier than later because his lungs got bad so quickly. Luckily, the IV antibiotics worked fast, and his PFT yesterday looked pretty good, and the CF doctor felt confident that Azer's lungs were healthy enough for surgery. 

Azer is glad he has a PICC line in for this surgery, as last surgery he only had IVs, which can fail often. Unfortunately, the PICC team told him that his vein could only handle 2-3 more PICCS before he would need a port. Hopefully this surgery is the key to keeping him healthy for the rest of the year! 

October 10, 2020

Admission - Again

So originally, Azer's surgery was scheduled for the end of October. He had a telemed visit with his pulmonologist. Azer has been doing an inhaled antibiotic which still hasn't helped the chills, increased cough or the fatigue. She opted to push the surgery date to next week, and to admit today. She wanted to admit Azer several days before surgery to start IV antibiotics, to get his lungs as healthy as possible. He will likely have trouble coughing after surgery. 

It feels strange to be back in the hospital so soon after last time. Azer had his third Covid-19 test today. There isn't an exact date or time for surgery yet. The main worry Azer has is about the possibility of the surgeon moving his G-tube to a different location. Azer remembers that the surgery for his G-tube was pretty painful and he doesn't want to experience that again. 

October 1, 2020

Met With Surgeon

Today we met with the surgeon regarding Azer's reflux surgery, called a Nissen Fundoplication. Since medication has failed to control his reflux and aspiration, surgery is the only other option. The surgeon answered a lot of the questions we had about the operation, recovery and life after.  He said he will be doing a bronchoscopy at the time of the surgery to check for any rare birth defects in the esophagus. Apparently, there are two rare defects can happen, one around the voice box area, and another between the bronchial tubes and the esophagus, that could allow reflux to enter the lungs. There is a possibility of Azer getting a new G-tube stoma if the surgeon has a hard time operating through the existing hole. Azer may have a hard time eating and drinking for a few weeks after the surgery, but he will be able to get plenty of nourishment through his G-tube while he is recovering. He might have to be admitted before the surgery if the CF team feels like he needs IV antibiotics for his lungs. 





It's been just over a week since Azer got off IV antibiotics. He already has been coughing up darker mucus, has been experiencing fatigue and chills. He messaged the clinic who wants to start him on inhaled antibiotics to hold him over until the surgery. They said if the antibiotic are helping him feel better, he can stop taking the antifungal, since it's obvious that it isn't helping his lungs at this point. 

September 25, 2020

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!
















September 22, 2020

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. 

September 21, 2020

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!

September 18, 2020

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. 

September 15, 2020

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. 

September 14, 2020

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!!