Compliance, Virus and Admission

This has been quite the difficult month. Azer ended up getting a virus which made him pretty sick with fevers. He went to clinic to get checked out. Unfortunately, he hasn't been doing breathing treatments as much as he should. It is difficult to make time for treatments when he wants to be out with friends and when he comes home from work late at night, especially when he says he doesn't feel that bad. The clinic has been trying to work with him on ideas on how to fit it all in, and what can be cut out as long as he tries to stay compliant. His lung function has been steadily declining because of not taking medications and doing breathing treatments consistently. 

Unfortunately, this is what many adolescent/young adults with CF go through. They choose life, friends, work over CF, and ignore their health until sometimes it lands them in the hospital not realizing what they are doing to their body. Ronnie Sharpe went through this as well. His blog post here is especially helpful. 

http://runsickboyrun.blogspot.com/2016/01/being-parent-to-teenager-with-cystic.html?m=1

He hasn't put on any weight in quite a while. It has been stable, but they believe his body has been compensating by using his muscles as fuel. He has been eating very well, but the doctors don't believe that he can gain weight without using his g-tube. They said he has been experiencing "starvation brain" from not doing his G-tube feeds. 

The doctors recommended that he get admitted to the hospital, as his lung function fell even more this time. He felt that he might be able to avoid the admission with oral antibio

tics and steroids, but late that night, his fever spiked, and he went back to get admitted.

They had an EKG done as one of the medications can rarely cause heart rhythm issues. The EKG results seemed to indicate that the right side of his heart was enlarged. They next day, they did an echocardiogram. It showed everything was perfect with his heart. The pulmonologist said that the likely reason his EKG was interpreted that way was because Azer has lost so much weight, that it makes his heart appear too large for his body. 

The hospital started him on the usual antibiotic for his MSSA. His lung cultures came back that he is now culturing two new bacteria: psuedomonas and achromobacter. They added two more IV antibiotics. Shortly after they added the new IV meds, Azer began to have mental confusion. He has trouble finding words, is having trouble remembering many things. They had the psychiatric team come and evaluate him. They ran a few tests. They thought perhaps he was showing signs of depression again. They have started him back on his ADHD and depression medications at low doses to slowly taper him back to theraputic doses. This hasn't helped. He couldn't spell a simple word backwards, or say the months of the year in order. He is having trouble sending text messages, and has to make phone calls. He doesn't remember sending some texts, or some conversations. Doing school assignments has been impossible, as he can't comprehend the words he's reading. After a week of feeling like his concerns were being dismissed, I finally stepped in and said this is not depression or normal for him at all. After looking up medication side effects, I found out that one of the IV meds he's on can cause temporospatial disorientation. The CF pulmonologist that is on the floor this week finally recognized what Azer is going through and validated our concerns. It seems to be caused by basically a perfect storm of the IV medications he's on. It seems they want to continue both of them for double coverage of the pseudomonas, even though it is causing distressing side effects.

His glucose levels have been a little high, as well has his liver and kidney enzymes. He has been working hard on keeping his fluid uptake up, he is even above 2x what he should be drinking, but all the levels are still kind of high. They aren't really concerned about it as it's temporary from the medications he's on. 

His lung function has been slowly going up over the week since admission, but yesterday it went down a point. 

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

The Plan

As with the last 5 hospitalizations, Azer has been putting on weight again, despite still having stomach pain and no appetite. He has been eating a very small amount at night, and just his G-tube feeds overnight and throughout the morning. The doctors said that the reason he gains weight whenever he's on IV's is because his body isn't working so hard to fight the infection in his lungs. They changed one of his IV antibiotics to a different one to better treat his MSSA. They are avoiding the antibiotic that gave him joint pain last time. 

His main pulmonologist has been working hard trying to figure this all out. She feels like the Infectious Disease doctors might be correct in believing that the MSSA he's been culturing is just overgrowing rapidly whenever the IV antibiotics are stopped. The ID doctors feel like treating it long term might be a good idea. Their plan is to treat it orally over several months. His pulmonologist is trying everything first before we have to resort to the port, as putting in a port will limit his opportunities to be athletic and play baseball. Treating the staph over a long period of time could potentially give other bacteria a chance to grow. 

The culture from clinic only showed MSSA, but the one they ran from after he was admitted showed possible NTM (non-tuberculosis mycobacterium). It takes weeks to identify what strain of this bacteria might be growing. 

The plan is to go home on long term oral antibiotics. If he ends up getting sick yet again, he will be needing a port, and will be treated with IV antibiotics. If he needs to be treated for the NTM, it will depend on what strain he is growing. Some strains can be treated with oral and inhaled antibiotics, others with strong IVs and can take over a year to combat. 

The bone age scan came back, and it shows that Azer's growth plates are almost fused. His Endocrinologist said that he needs to stop his growth hormone injections, as he isn't expected to grow much more, and won't benefit from them at this point. It is a little disappointing that he won't be much taller than he is now.  

This has all taken a toll on us, both mentally and emotionally. We are all weary from this long year of constant hospitalizations and the worry of needing long term IVs. The good news is he should be home in time for Christmas.  

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!! 

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. 

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. 

Home & Red Balloon Network Fun

Azer was a little disappointed in his PFTs today, but his airflow was pretty good, and he feels like his lungs are really clear. He wondered if the IPV was causing inflammation as he has very little mucus right now. Azer said he almost didn't care what his numbers were today as he was so ready to go home. He was happy to say goodbye to his PICC line today! This was one of the longest hospital stays he's ever had, but he is very relieved to be home.

He will continue inhaled antibiotics for a few weeks. He is glad to be done with IV antibiotics as for nearly three weeks now, he has had constant GI problems, even with Flagyl. We all hope that he continues to stay healthy and that whatever made him so sick two months in a row is gone.

Azer has had a lot of fun with the Red Balloon network for the last few weeks. He's been down in the Seacrest studios a few times. They have been doing more for the kids who are on contact, which is great for getting out of their rooms. 

Azer had so much fun doing a sports type show the first week, that they let him plan out his own show the next week. He found the videos and figured out where to start and stop them. 

Azer even did a few promotional videos for the Red Balloon network. He said even though this was a long hospitalization, that this was the most entertaining stay.  

The Current Plan

Azer has wrestled with the decision over the weekend of what to do, whether to stay for another week on IVs or try the EES again.

His PFT was the same as last Wednesday. His airflow was good and he had very little coughing during the test. He was disappointed with the number and felt like it should be better.

One of the fellow doctors that works with his main pulmonologist came in and Azer told her that he feels a lot better, but not completely at his best, and that he just wasn't sure what to do. He mentioned that before Trikafta, he seemed to do well when he was on inhaled Ceftaz, and that his lung function would drop on the off months. She said that in theory, he has been sufficiently treated for whatever has made him sick, but that a little more time might help. The plan is to switch him back to an IV antibiotic that covers MSSA the best, and to start inhaled Ceftaz. If his PFTs are higher on Thursday, we'll know it's working, and he can go home. If he starts to get sick again, he can start the EES and Trikafta on a modified schedule to see if he is aspirating. 

Expectations

Azer thought that the Flagyl was helping, as he was going to the bathroom less, but apparently there was an issue with the pharmacy and he was off of one of his IV antibiotics for a few days. When they restarted it, his symptoms started up again.

They are trying to test his mucus again to see if there is fungus, as it still might be a possibility, but Azer's main Pulmonologist said that the culture from clinic was already tested for everything and it only came back with MSSA. 

Azer talked to his main doctor bluntly today and said that he feels like none of the doctors know what they are treating, and everything is just a guess. He said he was frustrated that he feels better, but that his lung function isn't at his year's best, or even at his baseline. She gave him some options of what could be going on, and that it would take time to figure out what is working and what is not. They agreed that things need to be changed or adjusted one at a time to see what happens. She thinks one of the possibilities is reflux and aspiration. Azer stopped EES a few weeks before starting Trikafta and it could be causing fevers and lung infections. It could still be a virus affecting the lower areas of the lungs, or it could still be some bacteria or fungus. Given that his lung function didn't change with the Albuterol on Wednesday, she doubts that a course of steroids would change anything. 

One of the biggest things that has been worrying is that we all expected his PFTs to be significantly higher after two weeks of antibiotics and Trikafta. The doctor did some looking back at previous tests and noticed the computer changed his expected lung capacity when he turned 16, as it now is basing it's calculations off of the adult stats after his birthday. She did some math and figured that his FEV1 should have been at least a few points higher. She told Azer that it's better to decide if the antibiotics are working based on how he feels rather than be focused on a number.

We also both got off of our chests the expectations doctors and nurses seem to have about Trikafta, including what others experiences with it have been. It seems that most of the doctors we've seen recently don't understand how it works, or what to expect. Obviously, everyone is expected to respond to it differently, but he shouldn't expect his X-rays to look different, and it will not prevent him from having lung infections, nor will it heal his pancreas. She said it is extremely unlikely he would ever have to stop taking enzymes. There has just been a lot of confusion this hospitalization between doctors saying different things, the pharmacy being delayed, and general inconsistency of the staff and schedule. 

So far, Azer decided with the doctor to stop the IV antibiotic that's making his stomach upset, and to change the remaining antibiotic to one that would be easier to manage at home. Aside from the numbers Azer wanted to see, he is feeling better. The doctor said that there is no guarantee that a third week of antibiotics would prevent him from getting sick in a month, but she is leaving it up to him to decide what he wants to do on Monday. 

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.

Still Not Feeling Great

Azer had his first PFT today since admission. It is close to his baseline, but he said lungs still feel very irritated. The hyper-saline caused so much irritation this morning that he coughed for hours. He was exhausted by the time they came for PFTs. He asked the doctor if he could either stop doing it, or do a lower percentage.

The night of admission, the nurse who tried to put in his IV didn't do it quite right, and it hurt him so bad, he was sweating. The IV team was able to put it in quickly and painlessly. The PICC team had to put his PICC line in on his other arm this time, as the arm they usually use is still sore from the PICC line last month. He had an IV and PICC line on the same arm that day.

Most of the tests for Aspergillus came back, and they are all lower numbers than he's had in years, so the Dupixant is working wonderfully. His viral panel was negative, so they are just treating the bacteria he has cultured in the past. This doesn't really give a clear answer on why he is so sick a month after his last admission.

Everyday, it seems he's had less and less energy. He has had no appetite at all. The antibiotics are upsetting his stomach very badly this time. He says he hasn't been sleeping well. Even though his lung function is up, he isn't feeling well overall. He just seems to be feeling worse since he got admitted. We are hoping that the Trikafta isn't causing his decline.

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. 

Staying A Little Longer

So, we have been debating whether to go come today, or wait. This morning, one of the CF doctors came in and helped with that decision. She said that she would feel more comfortable with him staying at least until Monday. The test for the Aspergillus and ABPA haven't come back yet. One portion of it came back and looked normal, but that still doesn't answer the question of why his IGE was so high. She was telling us of the order they usually treat things, and that they are first treating what they can see first, which was the bacteria culture. She said if the wheezing in his lungs wasn't improving, it's possible to do a CT scan do check for mold spores. 

Yesterday, we had tentatively planned to go home today, and I guess the case manager started the discharge process, so now we have the medicine and IV supplies ready to take home with us. They also changed one of his IV antibiotics from one that is every 6 hours to one that is every 8, to help our at home schedule.

Azer says his lungs feel about the same but better after doing the IPV. 

He had a dressing change done today, and we have arrangements that if we get discharged, to have the PICC team do the dressing change at clinic. 

Other than that, he has improved his foosball game, and annoyed many by playing a mean game of whiffle ball bouncing off the windows outside!

Reason for Skin Peeling

So, the dermatology team came in to look at his skin.

They determined that this was not a reaction to the antibiotics, but was instead a skin peeling condition called keratolysis exfoliativa which can happen to people with sweaty hands, but is apparently very common with CF. Basically they said now that he has this condition, he will always have it, but they can prescribe a lotion that can help the dead skin come off faster.

They changed one of his antibiotics, Gentamicin to Tobramycin. They will continue to watch out for any hearing loss from the medication.