June 29, 2018

Update on Sick Visit

So today was mostly just a follow up for PFT's to see how his lungs are since he's been on steroids and antibiotics for a few days. His first few blows were exactly the same pre-albuterol last visit. They tried a different blowing technique which apparently works well for him when he's sick, and he blew just a bit lower than his post-albuterol test on Wednesday.

So his numbers look good, but numbers look good on paper, but don't always reflect the patient. Azer told the doctor that his lungs still do not feel good. He said they feel the same as they did before steroids. Usually, steroids make him feel a lot better quickly. The doctor did note that he's only been on this treatment for a few days, and it may take a little longer to start feeling the effects. She told him to have fun on our family vacation, and that if when he gets back, even if his PFT's are up to nearly normal, if he still feels like his lungs are sick, she can admit him to get his lungs back to normal before school starts. The doctor added that she's adding a test to his lung culture to test for non-TB mycrobacterium (we really hope it's not this, as it can take over 6 weeks for the culture to come back and the treatment is very intense and long. This bacteria grows very slowly, and treatment for it can take up to a year).

CF can be overwhelming and can really take over your day if you're not careful. Azer definitely felt those pressures when he was away for a week. Even at home, with not much else going on, it can be tough. I thought this picture really demonstrated him trying to fit everything in so he could go be a teenager. He was doing his inhaled Tobi podhaler and giving himself his growth hormone shot at the same time.

June 27, 2018

Sick Visit / CF Clinic

The Friday before Azer left with his youth group on a mission trip, he woke up that morning, and told me his tongue hurt and felt scratched up.

I was able to send pictures to the clinic that morning, and a few hours later they confirmed it was oral thrush, and sent the anti-fungal medication to the pharmacy.

Azer had a fantastic time and did as well as he could to keep up with his treatments during his mission trip. He told me mid-week that the schedule was not easy for a CF person to keep up with. He missed quite a few vest treatments due to how late at night they got in. By Thursday, he told me his lungs felt pretty sick, but he wanted to push through and finish the week. He didn't get enough time to do his Tobi or oral thrush medicine, but did most everything else and ate really well. He took the week off from his steroid nasal rinses, and ended up losing his sense of smell this week. He's hoping it comes back quickly now that he's back on it again.

When he got back home, Azer said his lungs felt pretty sick. When I listened to him, he didn't sound too bad, but had a few wheezes. Of course, perfect timing, his Vest ended up making horrible noises and needed to be replaced the day after he got home. Luckily, the Vest company was able to overnight us a new replacement machine!

Clinic: Azer had to get his annual Oral Glucose Tolerance Test today, so he was fasting since midnight. His height was the same as last time (he missed a week of growth hormones), and he lost about 3 pounds (not very concerning given how active he was last week. He was getting about 30,000 steps a day!) They drew his blood during vitals to test his fasting glucose level, which was normal.

Azer has been eating very well with the Periactin and as such has had changes to his stool and hasn't been absorbing his food as well as he could, so the dietitian came in to go over enzyme dosaging.

His PFT's were considerably lower than his baseline and lower than last month. The doctor listened to him and wanted him to do an albuterol treatment and then re-do PFT's and see if there was any difference. His FEV1 did go up about 7%. Azer told the doctor his cough never completely went away after the virus last month, but that his lungs felt much worse on his mission trip, but since he's gotten back home, they've gotten a little better.

The best course of action would be an admission to the hospital at this time, but we have already planned to be on vacation next week. The doctor felt like a quick admission for a PICC line would be pointless if he had to go back in to the hospital when we got back home. So, we are doing the next best thing: Hospital at Home: breathing treatments every 4 hours, antibiotics and steroids. The doctor warned Azer he needs to "get his butt in gear" (her actual words). We will redo PFT's before we leave, and if he hasn't improved when we get back, he will probably be spending a few weeks in the hospital on IV's.

The doctor told Azer to not feel like he didn't do a good job over his week on his own. She said the work he was doing combined with the weather and the environment he was in could be contributing factors to how his lungs are right now. They are running another virus panel, as well as a lung culture to see if he's growing anything other than MSSA. I will update as I find out the results.

June 5, 2018

CF Clinic

About 2 weeks ago, Azer suddenly started to sound congested. Oddly enough, it started during his week off of Periactin. The week off of Periactin, his seasonal (year round lol) allergies hit him hard. During Memorial Day Weekend, he began to sound so congested, we all wondered if it was time for an admission. He also began to cough up brown mucus. The on-call doctor prescribed some oral antibiotics to see if that would help. Since then, his cough has improved slightly, but he says his lungs have stayed full.

Not surprisingly, his PFT's were down today. The Pulmonologist said that his small airways were the same as they were last time, so whatever is going on with him is unlikely to be ABPA (our worst fear, as it would put a damper on summer plans). She said he should finish his course of antibiotics, and start Tobi Podhaler while we wait for his culture to come through. Even if it isn't Pseudomonas, the Tobi should also help out with the Staph he normally cultures. She heard some mild wheezing in his lungs. They also took a nose swab to see if he's culturing a virus.

He put on some weight, so the Liquigen is helping him out. His appetite is very good when he's on the Periactin, and he has been enjoying a healthy appetite, although it has come and gone since he's been sick. He is also growing!

Azer is going on a week-long trip with his youth group. This will be the first time he is fully responsible for all of his treatments and medications. The doctor went over things that were the most important (such as staying hydrated and ways to add sodium) and she went over a few ways to make his treatments easier, such has medications he doesn't have to take during that week.

Azer is still taking Dulera and Qvar. Well, apparently Qvar has stopped making the inhaler type, and is now coming out as an inhaled powder. Since it's already been ordered, and the doctor feels like he's old enough to be able to inhale it properly, he can try it out and see if it works for him. If not, we have to go back to Flovent.

- Update: Azer's viral panel came back positive with metapneumovirus. His lung culture only came back with MSSA. He is doing great with the new Qvar, and doesn't really notice a difference in how it feels, other than this Qvar is not taken with a spacer.