It's been a while...

Sorry about the absence ,but cf hasn't been the only thing that I’ve been battling for the past few months. Starting in the pandemic the mental strain that was thrown at me was something I had never experienced before. I’ve always been able to take whatever surgerys, picc lines, and tough bacterias that were thrown my way, but nothing prepared me for what I would experience coming out of the pandemic. On top of what seemed like the never ending hospitalizations and being accused of not taking care of myself throughout the pandemic, I had baseball, friends, and any kind of social interaction snatched away in what seemed like a few weeks. Lashing out in anger at random times and always feeling numb while I was being admitted drowning in my own mucus every other month because no answers could be found. It takes a toll on a person and especially on a person that shook off mental awareness as a joke up until it wasn’t. Fast forward to the fall of 2021 I took up a new hobby. Doing weed. The hospitalizations were not going down and I was being asked to work so much I was coming home and skipping treatments. The more sick I got the more weed I did. It helped me ignore the very prominent mental and physical issues that had surrounded me for the past 2 years. My parents didn't know how to help and I was a ship going down much faster than I had realized. Skipping meds unless I was in the hospital ,and struggling even more the closer I got to the end of the year because weed was beginning to not be enjoyable. It was just another thing I did. My mental health was killing me fast. Not taking antidepressants and my adhd meds, skipping breathing treatments on the daily. It took a toll on the people watching me do it everyday, my parents. January 2022, I was kicked out in hopes of a much needed wake up call. It took me until February to realize what I had done. It was like I woke up. I Started taking my Lexapro and Strattera consistently again and finally realized what I had been doing. Almost half a year of fog because my dependency had become so great that I didn’t know what to do without it. Couldn’t sleep without it, couldn’t function or concentrate, couldn’t feel healthy. Looking back on the entire mental struggle I will say that I would like to see the CF community and foundation do something about this. I’d like to think that I’m not the only person with a chronic illness that struggled with these obstacles. I wish there were more resources for cf families so these issues could be troubleshooted faster and addressed. A much needed change needs to happen otherwise the mental health of people with chronic illnesses will continue to suffer. I hope that we can create a plan so less people have to go through this experience.

Discharged

Azer's PFT yesterday was the same as Monday, it hasn't gotten any higher or lower. The doctor thinks that perhaps with exercise, he could get it back. He gained over 20lbs during this admission. He will be on two oral antibiotics and one inhaled for the next month to continue to treat the achromobacter and pseudomonas. 

He was so glad to get home and be with his kitty! 

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. 

18 Years Old

It seems like a long time ago, and at the same time it seems like only days ago that I had a tiny, medically fragile baby that was my whole world. Although it was the biggest responsibility and challenge I have ever had in my life, I wouldn't have changed it for anything. You changed my life in an instant, and I vowed to always be there for you, even though I was terrified of losing you. I grew up with you, we became a team fighting CF. We have shared so many good times and the bad times.

Even though you are 18 now, and legally independent, I will always be here for you in any way you need me. You drive me up the wall, push my buttons and annoy me, and I do the same to you. I look forward to continuing. 😜 I love you. 

Clinic Visit

Yesterday was Azer's follow up visit. As is common for any one, even without CF, Azer has struggled through the summer with keeping a schedule. Now that school started again, he's been trying to figure out how to balance school, work and CF at the same time. This is an issue for adolescents with a CF, gaining independence and managing life and CF throughout highschool and college. Unfortunately, his weight has fallen quite a bit over the last month as he's hasn't been doing his overnight feeds consistently. His lung function also fell again, so they want to see him back in a few weeks. The clinic is trying to work with him to be more consistent with everything to prevent him from a possible admission. His pulmonologist is trying to make breathing treatments easier on him by allowing him to do some of his nebulized medicines once a day, if necessary. Overall, he is still doing well with the Tobramycin, and hasn't had any fevers. 

Azer has requested several times during hospital admissions to get a new G-tube button. For whatever reason, they couldn't, or wouldn't do it. We've struggled to get them from the DME company as well. He has had the same button for well over a year. They sent him to the GI clinic after he was done with the CF clinic. They helped him put in a new button, which was unnecessarily painful due to the button being so old. The GI nurse said she will be making sure that he gets a new button sent asap. 

Azer has started to do a clinical study that studies breathing treatment adherence. It is a long term study that lasts months and keeps track of how many nebulized treatments he misses. He completes surveys from time to time throughout the study, and he even earns money for completing tasks! 

Last First Day!

Azer starts his senior year of High School today!

CF Clinic

Today, Azer's PFT was quite a bit higher than two weeks ago. This is despite being off of Tobramycin for several weeks due to pharmacy issues. 

He lost several pounds over the past two weeks, mostly due to not being consistent with his overnight feeds, but he plans to start getting them ready before he goes to work so he doesn't have to push himself to do it late at night. 

Azer was invited to do another study, this one being for breathing treatment compliance, if he qualifies. 

Bone Density Test and CF Clinic

Today Azer had a bone density test to test for osteoporosis that can affect people with CF. We'll find out the results later this week.

Azer has been working hard and taking a lot of shifts at work, sometimes working up to 40 hours a week. His pulmonologist is a little concerned about how many hours he's working as at times he's too tired to wake up on time and do his complete treatment. He is still working on trying to adjust his schedule and work out a routine to help remember his medications, and give himself enough time to do his breathing treatments.  

Azer has been been more vigilant about doing his overnight feeds, and gained several more pounds from last week. His lung function fell a little more from last time, but the doctor is going to continue him on Tobramycin instead of doing a month on and a month off. They did another virus test just in case, but it seems most of his coughing is coming from drainage. Two weeks ago, he did test positive for rhinovirus, and viruses are starting to pick up as more people are passing them around now. 

Since he's been working so much, he has been saving up money to pay for car insurance. He passed his driver's license test and is finally a licenced independent driver! He has been enjoying his freedom and has spent a lot of time fishing on his days off!

ENT

Today was a follow up to March's ENT appointment.

Azer was a few pounds heavier than be was at CF clinic a few weeks ago. He's been better about doing his night feeds. He hasn't been doing his nasal rinses that frequently, but the ENT doctor said there weren't any polyps and that things looked pretty good. She wasn't aware that he's been on Dupixent for a few years, and she said she wished that more kids she sees could get insurance approval to be on it, since it really helps slow the growth of polyps, even to the point of delaying the need for surgery. 

Azer has been having drainage issues, but so far his lungs have been pretty healthy. 

Since he's been on inhaled Tobramycin for about a month, she wanted to do a hearing test, just to be sure that it isn't affecting his hearing. His hearing test was perfect, thankfully. 

Results from the sweat test: the results still show that he has Cystic Fibrosis, so either the Trikafta isn't lowering his salt in his sweat enough to see a change, or perhaps it's because he was fasting that day and didn't take it that morning. 

CF Clinic and Sweat Test

For about two weeks, Azer has been experiencing symptoms of either a virus or allergies, with worsening CF symptoms. He called his doctor on Monday and she told him to take a high dose of OTC allergy pills. It has helped some, but most of his symptoms are related to his sinuses. He felt well enough to wait for a clinic visit for today.

Today Azer was 10 pounds less than he was when he was discharged from the hospital. He hasn't been able to do all of his night feeds as he his stomach has been filling up with mucus. There weren't too many changes in his routine so far. They are running a virus panel, but will likely put him in steroids for about a week. His lung function was lower than it was when he was in the hospital, but they want to see him back in two weeks to make sure it doesn't start tanking. 

Today, the clinic researcher scheduled Azer for a sweat test. This is a Trikafta study to see if people on Trikafta have a lower salt value in their sweat than before they were on Trikafta. People with CF have an abnormally high amount of salt in their sweat. Azer had this test when he was born to confirm he had CF before his mutations were found. It will be interesting to see if his salt concentration is still high. 

Discharged!

Azer came home last night! His lung function was holding steady from last week. His Tobramycin got delivered in time, and the clinic ordered a new nebulizer machine to hopefully help his treatment go a little faster. Adding Tobi to his morning and evening treatment will make his 30 minute treatments quite a bit longer. 

He got out just in time for his Ortho apparently. He should be getting is braces off by fall! 

Staying Through the Weekend

The inpatient doctors and the ID doctors were ready to send Azer home Thursday or Friday. The ID doctors had a few options other than to put in a port. They said they could send him home with the same oral antibiotic combined with inhaled Tobramycin, or with a different oral antibiotic, Minocycline. The problem with the Minocycline is it will take two weeks to find out of his type of staph is susceptible to it. 

Azer made the decision with his pulmonologist to stay through the weekend, and aim for discharge on Tuesday which will give him a full two weeks of IV treatment. He may need to stay a little bit longer past Tuesday, as they are working on insurance coverage of the Tobi. He would rather have the Tobi Podhaler instead, as it is much quicker, but it is unlikely to get the insurance to cover it. The regular inhaled Tobramycin takes 30-45 minutes via nebulizer, which he will have to do twice a day, but the added benefit is that he would be able to inhale it with a mask to target some of his sinuses where he is culturing abundent staph. 

The hospital staff and his pulmonologist noted that he seems much more like himself now that he's on the antidepressant. Azer has been enjoying Uno games with the nurses. Several will come in at a time and play. They have been keeping score on the whiteboard in the room through the two weeks, and the list is quite long now!

Improving & Mental Health

So, Thursday, Azer's PFT was about 20 points higher than it was the day he got admitted. Today, he blew the best he has in two years. He cultuted MSSA again. This is all great news, but he has been getting annoyed with the doctors as they asked to send him home on IVs, but they haven't come up with an explanation as to why this is happening over and over again, since last year. Azer is supposed to see the Infectious Disease doctors tomorrow. 

We have been noticing since last year that Azer has become increasingly irritable, and he stated that he would begin to feel numb when he was in the hospital. His psychiatrist said that adolescents, especially males, tend to exhibit depression in anger. His pulmonologist mentioned that there have been some possible cases of Trikafta making people irritable, but this isn't certain. Azer spoke with his psychiatrist who told him he would benefit from an antidepressant added to his ADHD treatment. It will take time for it to start working, but this is a good reminder that May is Mental Health Awareness month, and that CF affects families and patients in many unseen ways.

Thank you to all who have donated to the walk, GrubHub gift cards, snacks, and games!!!