PICC Line in

Azer's PICC line went in easily, but he is pretty sore. He is settling in and getting comfortable. 

He lost even more weight, about 5lbs more since his clinic visit, and the GI team is worried about it, so they will be counting his calories while he is here. He has been doing less G-tube formula, but has been eating more food, which they like, so they are trying to find the reason why he is losing weight so fast. 

He really likes ordering food while he's admitted. If you'd like to help him gain weight, he would really love some GrubHub gift cards! azerbaseball32@gmail.com

Admission

After Azer finished the antibiotics that his pulmonologist prescribed, he started to get worse. He is frustrated as he has to keep calling into work because he feels too bad to complete a shift. He has been dizzy when he stands up. His oxygen is a little lower than normal. He feels chilled everyday but hasn't had any fevers. He told clinic he needs to be admitted, as this cycle has been going on for too long. He says he will be debating the plan to get a port, as he says he isn't quitting his job, school and summer baseball and that they need to figure out another way to treat this. Hopefully they won't have trouble putting the PICC in! 

Discharged!

Azer's PFT yesterday was much better than last week's test. He still coughed up very dark mucus which they are sending for another culture. They felt comfortable enough with his progress to discharge him. They felt that if he kept the PICC line in, it was an unnecessary risk for infection, so they pulled it out yesterday before we left. The oral antibiotics are very strong. He has to take 5 pills of them at a time! The plan is if he does end up going down hill in the next week, he will be readmitted for a Port and long term IVs. At least he has some time to see if the oral antibiotics work and to be able to enjoy the holidays! 

Staying Longer

Well, Azer was supposed to be going home today on oral antibiotics. He had one more PFT before he was supposed to leave. He ended up three points lower than he has this whole time he's been admitted. He also coughed up the darkest mucus he's ever seen. His doctor felt like this is possibly old mucus, as the longer he's on antibiotics, the less irritation his lungs have, which allows old, infected mucus to come out. 

The doctor didn't feel comfortable with discharging him today, and both Azer and I felt like we weren't comfortable going from IVs straight to oral and pulling the PICC out. So, we all worked out a plan we're a bit more comfortable with. He will continue IV antibiotics through the weekend, and then will try out a few days on the oral antibiotics to see how he feels. If he feels ok, he can go home and continue with oral antibiotics, and hopefully will continue to improve. If not, he will go home on IV antibiotics just to get to be home for Christmas. After the holidays, he will be readmitted for a Port. 

The culture came back to find out what kind of NTM he has, and unfortunately he has non tuberculosis mycobacterium abscessus. This type is harder to treat, and requires long term IV antibiotics, and is associated with faster lung disease progression. His doctor is hoping that by treating the MSSA, it will cause the inflammation to go down, and that the Trikafta will keep the mucus thin enough for his lungs to clear the NTM on their own. 

Azer had a bright moment in his day. Usually the Texas Rangers come to the hospital around Christmas time, but because of covid-19, it happened virtually. He got to go down to the studio and host the virtual meeting with a few of the Texas Rangers. He had a blast and asked them of lot of his own questions, especially about how to improve his pitching. He came back a lot happier! 

Admission Again

Since his last clinic visit, his lungs kind of stayed the same, but over the last few days, they have grown much worse. They are admitting him again today. This is the 6th time this year, and they still don't have a definitive answer for this. The doctor brought up the possibility of getting a port in the near future, and possibly needing IV antibiotics every other month. This is discouraging to think of this, as it could really change his ability to play baseball. 

He has had quite a bit of stomach pain over the last few weeks. He tries to vent his stomach, but nothing comes out. He had an incident where he had extreme stomach pain and threw up just water. He lost more weight since he was last admitted. I think he's down almost 10 lbs since he got discharged from the last hospitalization. They may try a special kind of G-tube bag while he's admitted to help the air vent out. Since he's been still having stomach pain and trouble swallowing some foods, the dietician would prefer him to continue a mechanical soft diet. 

Azer also saw his endocrinologist today. She ordered some lab draws and x-rays, including a bone age scan, since it's been about a year since those were done. These are to check to see how much time he has left to grow, and how long he could continue to take his growth hormone shots. They also check to make sure he hasn't developed diabetes, as growth hormones can potentially cause this. 

He also had a chest x-ray done, and then he is going home to pack for yet another hospital stay. This is hard with it being December...

Home!

Surgery, GI and the Pulmonary teams all feel comfortable with Azer going home today. His PFTs were better than when he got admitted. He has a follow up with the CF clinic and the ENT doctor in a few weeks, as well as a follow up appointment with surgery. He needs to be on the mechanical soft diet for 6 weeks, and keep his G-tube feeds at a rate for the next 6 weeks as well. This one is tough, as his morning feed ends up lasting for 5 hours, so even though he is craving a lot of different foods, he is too full of formula to eat much at all. A friend of Azer's sent some Pho noodles which he should be able to eat!

Having the Nissen has been a learning experience for Azer. He learned he has to be mindful of how well he chews his food. He's found if he doesn't chew well enough, even soft foods kind of stay stuck and don't go down, and eventually come back up again.

GI took Azer off of one of his GI medications, as they felt like he didn't need it anymore, now that he can't physically reflux. He's been off of it for four days now, and he hasn't experienced any bloating, which is amazing. 

Overall, he is recovering very well and is so glad to be home!

Ready To Leave

Azer is very ready to go home, and has been cleared by the surgeon to go home. The GI doctor would like him to stay until Monday. Their reason is by Monday, his IV antibiotic course would be complete, and he would be able to do another PFT test. His PFT from yesterday was worse than when he came in, which is to be expected. They did an X-ray earlier in the week, which showed that he still had some gas in his abdomen, so it still hurts to take a deep breath.

They have been slowly increasing the rate of his G-tube feeds, and by Monday he should be at the rate they have been aiming for. They want to make sure that he is tolerating the speed and amount of formula. They are also taking away one of his GI medications now that Azer cannot reflux. 

Azer felt like he might have been refluxing, but he later realized that the burning feeling isn't going up to his esophogus, that it is staying lower. He said the new medication that coats his esophogus doesn't help the burning, but that tums does help. This is great news, as it shows that the Nissen is working. 

More good news is that Azer hasn't needed any pain medication for the last few days, even though he has been increasing his activity during PT. 

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. 

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. 

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. 

PICC Line Is Out & Annual Labs

 The two weeks of IV antibiotics at home went smoothly. Azer gained weight over the past week even though he had a few days of not being very hungry.

Azer had clinic yesterday to discuss how the course went and how he feels. Overall, he feels like his lungs are very clear, in fact so clear that he feels like he needs to turn down the settings of his vest. Azer said he didn't feel like another week of antibiotics would do much more. He told the doctor he has no interest in restarting the hypertonic saline, as his lungs aren't full of mucus like they used to be. Azer was able to ask the doctor to tell him exactly what she saw from the bronchoscopy. She told him that if she didn't know who she was examining, she wouldn't know that he had CF. That's how healthy his lungs visibly looked. Of course, this is just the main airways that look this good, as the X-ray from the hospital stay still shows bronchiectasis, bronchial wall thickening, and blockage of the small airways. This damage can't be fixed, but the less infections he has, the better off his lungs will be long term. The cultures still aren't showing anything other than MSSA, but he is still having coughing attacks a few times a day. He can't explain what triggers it, and the doctor doesn't really have an explanation. His reflux is well controlled, as long as he doesn't have anything acidic before bedtime. 

Azer did a PFT at home with a device the clinic has been sending out to patients. The numbers he got at home were very close to the ones he blew in clinic, which shows how accurate the home device can be. This is great for peace of mind, especially when he is doing most of his CF visits using telemed. He was disappointed with his results, but the doctor said that his lungs are probably still recovering from the bronch. 

It was time for his annual labs and glucose testing, so we were there for a few hours. All the results look great. The best news was his IGE level, which is the antibodies his body produces to allergens. When it is very high, he usually cultures aspergillus. Even when he isn't culturing any fungus or mold, his IGE is always elevated, which is his normal. Since he started Dupixent a year ago, his IGE has gradually been going down, but still out of the normal range. Yesterday was the first time his has ever been in the normal range! 

The process of transitioning from the pediatric clinic to the adult clinic begins very early for a CF child. At a young age, they learn their medication names and what they are for. There are skills that they should learn by a certain age. As Azer is getting closer to transitioning, they had him fill out a form that shows them how competent he is in different areas. They told him that he is ahead of most other 16 year olds in most things, and that the rest will come with experience and time.  

Home IV's Going Well

Home IV's have been going pretty well. The hospital staff adjusted the schedule while we were there to make it easier on our sleep schedule at home. It isn't too difficult as he only is on one IV medication. 

So far, all the cultures from both the sinuses and lungs have come back negative for everything except for MSSA. Some of the cultures are still pending for the next few weeks, but so far it looks promising.

Nasal rinses have been getting a lot easier for him. The bleeding is pretty much gone now, and he is able to breathe through his nose finally. He's not sure if the dissolvable packets are still there. He has been coughing considerably less. 

Azer had a dressing change today at clinic. The first one is always the most painful, but it wasn't too bad this time. The plan is to do one more week of IV antibiotics, and to see the ENT doctor in a few weeks to make sure everything is healing well. 

PICC Line and the Plan

Azer felt quite a bit better as the day went on. He did a nasal rinse, which was a bit painful. The oozing never seemed to end. The ENT doctor who did the surgery came to see what could be done. She carefully suctioned a bunch of blood out of both nostrils. The bleeding seemed to slow down quite a bit after that. She wants him to really try to do four rinses a day, and to hold the sinus steroids for several weeks. Later in the afternoon, another ENT doctor came and suctioned his nose again. The next sinus rinse was painful, but manageable.

So far, the cultures show a lot of MSSA, so they put in a PICC line today, and started a broad spectrum antibiotics. They are trying put in the orders to continue IVs at home, perhaps tomorrow!

He is feeling pretty good tonight and is hungry, but says he can't taste much. We are watching opening day for our Texas Rangers!

Admission Questions

Yesterday, Azer and I had a long conversation with the CF doctor for this week. She said he is on the path to a bronchoscopy, but not quite there yet. Azer asked why they are treating something when they don't know what it is. So she explained that they are treating what they see. She told us we might not see anything different culture from the bronch. We brought up the possibility that the hypertonic saline might be irritating his lungs, so she said it was fine to stop that. She said she would order a PFT for today, pre and post Albuterol.

Today Azer blew only one point higher after inhaling Albuterol. The doctor was a little disappointed with the results, as she was expecting to see a higher number after Albuterol. She thinks his lungs have a strong inflammation component and that it will take time for them to calm down. She said before gene modifiers, they would put patients on a very high dose of ibuprofen to reduce inflammation and their lung function would improve. Azer coughed out some very dark mucus that she saw, and she believes it's old mucus still coming out. She said during bronchs, they flush the lungs with saline, and tons and tons of old mucus comes out at once. She said that Trikafta is likely doing the same thing, but a little at a time, and it's coming out as if in layers. It seems difficult to get much information on whether the off and on fevers and lung infections are due to Trikafta. 

So far, the doctors are undecided on whether to continue antibiotics for a third week. His appetite seemed better last night, but his GI system as still been very upset. They may start Flagyl today.

Staying Another Week

His PFTs were exactly the same as last week. He feels like his lungs haven't improved since Thursday. They are better than the day of admission, but he said they still feel irritated. He feels discouraged that his numbers are not higher and that he's not feeling better than he expected to be by now, even with two strong IV antibiotics. They are still causing an upset stomach, and he still hasn't been able to eat more than few bites of food a day. 

We made the decision to stay in for another week instead of going home on IV antibiotics. He is getting good lung therapy with the IPV, which we don't have the advantage of using at home. We are still struggling to get formula and G-tube supplies at home, so he will have time to get his weight up with extra formula while admitted. He missed a dose of Trikafta last week, and noticed the effects quickly, so it is doing a lot more for him than we realized. There still isn't a clear answer on why he got so sick only a month later, without anything new showing up in his cultures.

In mean time, he has been entertaining himself with Legos, crafts, and has been on the Red Balloon network a few times (a channel broadcast in the hospital). He even got to meet one of the Harlem Globetrotters. Azer would appreciate any type of arts and crafts that would keep him occupied.