Changes Needed

So, it's nearly been 7 days since Azer's been on three IV antibiotics. Azer was feeling discouraged last night. Even though his lung function test Thursday was up, he feels like his lungs haven't gotten better and says they feel full and irritated. He was worried that if he went home, his lungs would just nose dive.

 He did a great job this morning explaining to his Pulmonologist how he felt. Azer said that using the IPV has done a great job clearing the mucus out, and the bronciodilator is opening his lungs, but feels that the antibiotics he's on are not killing whatever is making his lungs sick. The doctor agreed with Azer and told him that the PFT is just one indicator in how his lungs are doing, and they really take into consideration how he feels.

So, the staff will present the data from this week to Azer's main pulmonologist and she may change his antibiotics, or he may have a bronchoscopy this week. Either way, it's not looking like he will go home quite as soon as we had hoped.

Worsening skin

Dermatology was contacted again. They said that this was not unusual, but they can't explain why it started nor when it will stop or how far it will progress. They are not concerned because it is just affecting the top layers of skin. They suggested that he puts vaseline on and wear socks and gloves. (I will not be offended if everyone replies to this post with "it puts the lotion on its skin" memes)

Dressing change and Frisco Rough Riders

It would be normal to think inserting a long term IV would be painful, but dressing changes are very painful. Azer had to have his dressing changed a few days earlier than normal as it was coming off. This can be dangerous as it can lead to bacteria entering the blood stream right near the heart. 

Child life surprised Azer today. A few players from the Frisco Rough Riders were here, and came to visit him. He was so excited! They talked baseball, about him being a southpaw, and watching him play an MLB game on the Xbox. Azer's eyes lit up when one of the players mentioned that he met Barcelona Colin (a Texas Rangers pitcher). It was a wonderful experience for him!

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.

IPV and Possible Antibiotic Reaction

Last night, Azer noticed a spot on finger where the skin was peeling. Today, more finger had peeling skin. This afternoon, it started to appear on his toes. The doctors say it could be from the antibiotics. They will watch it to see if it gets worse.

Today, Azer tried IPV for the first time. The RT said he had great technique. It takes quite a bit of effort to use it, but Azer did great, and said his lungs felt very open after using it.

Hospitals are boring

Azer has to be hooked up to the IVs at all times, unless he wants to shower. He had a friend come visit for a while which helped to ease the bordeum. 

We were rebels and escaped outside when the rain died down. 

He says if anyone wants to text him or FB message him, that would be cool. 

Got the PICC line!

They were able to put in a PICC line without sedation. They also did a chest X-ray.

He will have three different antibiotics running through it. Several can affect the kidneys so they have to test the levels periodically. Another medication is in the same family as tobramycin and could affect his hearing. They have to use these medications as he has vomiting with an antibiotic he used to be fine with. Another possible medication that could have worked could cause an adverse side effect with one of his GI motility medications.

He will also be starting an inhaled steroid while he's here.

So, here's to hoping that they help his lungs feel better soon!

CF Clinic and Admission

Today was a follow up visit for after our vacation. We knew coming in that we might be facing a hospitalization.

Azer gained weight from last visit. He's been very hungry from being on Periactin and Prednisone at the same time.

Azer did three treatments yesterday and ran for 30 minutes today to try to get his lungs as clear as possible for PFT's today.

His first try ended up 20 points below his normal. After 8 tries, he was still lower than last week. His culture from last week only came back with MSSA, he's been on oral antibiotics for two weeks, and steroids for over a week, and it feels like nothing is working. The doctor listened to him, and said his lungs sounded clear, oddly enough. His cough sounds productive and irritated. He said when he ran this morning, he had lung pain. He says his lungs feel full, like the mucus is stuck.

The doctor said we can start IV antibiotics and see if he feels better after a few days. If not, he will get a bronchoscopy, which would help them get a culture from deeper in his lungs. She said about 70% of the time, a bronchoscopy causes a fever. The stain for mycrobacterium came back negative, but the doctor said sometimes the stain can be negative, but the culture could come back positive. If it is mycrobacterium, it could be a hospitalization up to three months. 😶 It is frustrating to not know what his causing his lung function to decline when he is doing what he can to improve.

They will try to put in a PICC line in today without sedation. Previously, he has had light sedation. The technician came in to check his veins and put numbing medicine on his arms.

The antibiotics he will be taking are Ceftazidime, Nafcillin, and Gentamicin. He started inhaling Pulmicort with his breathing treatments.

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.

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.

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.

Help Us Find a Cure - Great Strides 2018

Click here to donate to our Great Strides 2018 Walk! http://fightcf.cff.org/goto/lilhyperfingers

I recently had the opportunity to attend a meeting and heard Bruce Marshall, the VP of Clinical Affairs for the Cystic Fibrosis Foundation speak. He played this video, which of course, drew tears from everyone in the room. My own tears came from gratefulness for the people who have been helped with these new medications, and also sadness, because those pills aren't available for Azer yet. I feel like we've been told he will have them soon, for many years, but it wasn't until this meeting that I knew, it will be very soon.

This is an extremely costly endeavor for the CF Foundation, and they will continue to need money to fund further research for mutations like Azer's, and for new medications that will continue to treat the symptoms of CF.

Great Strides is the largest fundraiser the CF Foundation holds annually. As a family, we stride every year, and raise money so that we can soon, have these life saving medications. Will you join us in raising money and lacing up for a cure?

Why the Cystic Fibrosis Foundation Strides for a Cure

Watch Joe Flacco, QB for the Baltimore Ravens Speak about why he makes Great Strides for CF

Endocrinology

It looks like the Periactin is working! Azer gained about 4 lbs in only a few weeks just from an increased appetite. From his last Endo appointment, he grew about 1/3 of an inch. He told the doctor that he's had hip and knee pain lately, and he feels like he keeps banging his ankles into things frequently. These are signs that he is growing. Another sign we may see soon is he will need bigger shoes.

Azer told the doctor that the pharmacy recently changed needle sizes, and the longer needle is causing the injections to be a little more painful. She said the needles aren't a prescription, so the pharmacy themselves may be able to change this. He also mentioned that the most painful injection site is in his stomach. She told Azer he doesn't have to use the stomach if he doesn't want to. He does need to change injection sites each time, but scarring from injections happens more often with insulin, not so much with growth hormones.

The physical therapist came and looked at him for his scoliosis, since there is a risk of it worsening now that he's growing. She noticed that his left scapula area is higher than his left, and we were all concerned that his scoliosis got worse, so the doctor sent us to get an X-ray.

The results from the X-ray look good. His scoliosis hasn't worsened, which means that the changes in his posture are muscular from growing, and possibly from pitching. He has been working on pitching quite a bit lately. So, it looks like over the summer, he will be working with the physical therapist every other week again to correct his form. If we don't correct it now, it can eventually be permanent.