PICC Line soon!

They came in and looked for a good place to thread the PICC line in. Apparently he didn't need to be NPO, so since he hasn't had any food or liquids by mouth since midnight, he is going to eat now! 

He also got a CT scan done on his sinuses to check for polyps or blockages that may be affecting his lung function.

He is also getting breathing treatments 4 times a day.

Admission

Well, since we knew this admission was coming, we made the most of the time we had after school got out. We swam to our hearts content, and he played one more baseball game Thursday. He is missing a tournament this weekend, but hopefully he will be done with IV's before his World Series tournament in a few weeks.

We got here late last night. It always seems to take a while before a bed is ready (popular place lol). He got an IV last night, and has been NPO since midnight. We are hoping to get a PICC line placed soon so he has use of both hands. He can't play his Nintendo very well with only one hand. He is hungry since he hasn't gotten his morning feed. He is on IV fluids since he can't drink anything either.

So, they have increased his antacid medicine to see if that will make any difference. They are planning to do a CT scan of his sinuses before we go home. He only cultured sensitive Staph, but they are going to temporarily treat for Pseudomonas as well just in case it's lurking somewhere in his lungs.  He is getting two strong antibiotics through his IV right now. Apparently, his IV was burning last night, but seems to be alright this morning. I hope it holds up before he gets his PICC line.

Reflux

Hey Ozzie, whatcha doing?
Azer: refluxing...

CF creates new words, apparently. The new antacid seems to not be working after all. This is the worst I've ever seen it. I feel like we're not able to do breathing treatments properly because it causes more reflux. I hope they'll be able to fix this in the hospital.

I don't know if this is part of the reflux, but he has had a lot of bloating and constipation lately. A few doses of Miralax seems to have thrown that into complete reverse. It is so hard to find a balance!

CF Clinic

After the last sick visit, he was prescribed some pretty tough antibiotics along with a brief time on steroids. His cough improved temporarily but his cough came back again. So this was a follow-up and sick visit.

He gained a few pounds this time (despite his poor appetite) which is fantastic. They are still pushing for more calories because his growth is just so slow lately, and his height percentile is tapering from where it used to be. We will be switching to a higher calorie formula, which should push a few more calories per ounce.

He has been having a lot of issues with reflux, but our GI doctor decided a few weeks ago to try a different type, and it seems to be working. We may have to try a course of Flagyl soon if he still continues to have issues. We are also increasing his Bethanechol which helps his stomach move food along faster.

His salt was a little low, and I will be adding salt to some of his G-tube feeds as it is a struggle to get him to drink one (1) bottle of Gatorade a day.

His Fev1 was 79%. I know it can be much higher. So, we had a choice. Do another long course of oral antibiotics (the last course just cleared all the bacteria out of his colon and his very hard on his stomach, which means lots of time in the bathroom), or do a course of IV antibiotics to clear him out. Doing IV antibiotics seems to be the best choice at this time. It's been a few years since his last tune up, so it is needed. The good news is he has only been culturing bacteria that responds well to broad spectrum antibiotics. The plan is to wait until school is out, go in for the PICC line and some X-rays, and then do the rest of the IV antibiotics at home. Hopefully this will clear all the junk out of his lungs so he can have a summer free from antibiotics, steroids and IV's!

We are still participating in the CF walk this Saturday. It will be rain or shine, so I hope to see everyone there!!!

Sign up or donate here! http://fightcf.cff.org/goto/joggersforazer

Sick Visit

At the last clinic visit, Azer was prescribed oral antibiotics for his lowered PFT's and increased cough. They worked quickly, but about two weeks off of them, his cough came back along with some wheezing. After increasing treatments, we weren't seeing much of an improvement. Last Thursday, I emailed the clinic, and they were able to see us the very next day. (Sometimes it feels like we are always "on call" as far as CF goes!)

His vitals were great, but his Fev1 was down to the lower 70's. We are doing a course of oral antibiotics and steroids. If there isn't improvement at his next clinic visit in a few weeks, it might be time for IV antibiotics. It's been a few years since his last admission, so we've been pretty lucky.

His reflux has been horrible lately. It has been interfering with his appetite and hasn't helped him trying to cough this junk out. We will see the GI doctor at our next clinic appointment, but in the meantime, they've switched him to a different reflux medication and  a higher dosage.

Also wanted to let everyone know that it is 24 days until the CF walk. It will be on Saturday, May 30th at the Vitruvian Park in Addison. Check in time is at 8am and the walk starts at 9am.

Click here to donate or join our team, Joggers for Azer! 

http://fightcf.cff.org/site/TR?team_id=29213&fr_id=3453&pg=team

Clinic Day

At Azer's clinic visit three months ago, the doctor noticed his growth was starting to plateau out. Since then, he has been doing 5 cans of formula a day through his G-tube instead of 4. Three at night, one in the morning, and one when he gets home from school. He gained 4 lbs in 3 months, and grew almost a whole inch. I believe it's working unless his long hair is adding to the weight!

He got a few colds over the last few months, and the last one just seemed to stick in his lungs. His Fev1 was down to 81%. He has been junky for weeks. The doctor is putting him on oral Bactrim for 2 weeks unless he has cultured something that would need different treatment. They also tested his IGE level again to see if it's up. I'm really hoping not. I feel like the last ABPA took so long to treat, and he was on Prednisone for months! Hopefully the oral antibiotics will get his lungs feeling better again. He commented recently how much relief he gets from manual CPT and running, so we have been doing both without fail! 

Sick...

Azer is still on steroids... a very lose dosage every other day, but still on them. His IGE levels are slowly dropping, and we are still doing labs every other week.

The day before yesterday, I noticed he might be coming down with a virus.  Last night, after his evening treatment, he had a wheezing noise in his upper right lobe that was so loud, you could hear it without a stethoscope. I called the on-call doctor who didn't have much advice to give other than to continue to do treatments or take him to the ER. He was coughing hard enough he threw up, so I figured rest would do him good. I know he didn't sleep well. Today, he had a fever, and was nauseous, but the worrying thing is his cough. It is very congested. Luckily, I don't hear much wheezing. I have been doing CPT every few hours to try to relieve him.

I don't know what exactly has changed in him, or his lungs lately. Perhaps there is still remaining inflammation from the ABPA. But this past year, whenever he's gotten sick, the wheezing can be terrible. It can be so loud that he needs a fan in his room full blast so that it covers up the noise so he can sleep. I've been getting a sense, for the first time, that Cystic Fibrosis and that horrible mucus is just so hideous. It's just horrible how quickly his lungs can get full and irritated seemingly overnight. That we can work for months and months getting him to gain several precious pounds, and one little virus can take it away in a week. While everyone else's kids get a cold and stay home for a few days and be perfectly fine, he has to be on antibiotics and steroids for weeks, sometimes months.

I just know we have to keep working hard, doing breathing treatments, CPT, running, praying... whatever it takes.

Clinic Day

When Azer was on the Voriconizole (antifungal medication) he had cracked and bleeding lips for nearly a month, and also his very first sunburn. The sensitivity to sunlight was so bad, that even sunscreen didn't help. The nurses were pleased to see his face and lips had healed completely. Where he had a sunburn on his cheeks, he now has freckles. He is on a pretty low dosage of steroids now, and his appetite is slowly going away too, unfortunately. Thankfully, he gained a few pounds.

We saw a different doctor today, but both Azer and I really enjoyed him. He explained a lot of things to Azer, and talked to him on his level. He also commented how smart Azer was when he asked him a few questions about his G-tube and what would he do if it ever fell out. 

His lung function was great, and he sounded pretty clear. His X-ray from October still showed some mucus in spots. During the time when the doctor was explaining the chest X-ray to Azer, he explained what bronchiectasis was to Azer in a way I had never heard it, and it was just a bit frightening. He brought up a CT scan that was done on Azer's lungs last year. He explained bronchiectasis is when the bronchi get damaged and floppy, and they also get holes in them. He showed us the tiny holes that you can apparently only see on the CT scan. 

Another area of concern was Azer's growth. I hadn't really paid attention to his height as much as his weight, but that is usually how the information is presented to me during clinic. The doctor pointed out to Azer that he used to be around 25th percentile in height, but now he is below 10th percentile and really tried to make a point to tell him that he has got to eat in order to grow. 

To know that his lungs have tiny holes and that he has scarring that damage that will never go away is a let down, but I know I have to trust God in this and work harder than ever at keeping his lungs clear. 

Clinic Day

Yesterday, his weight was up to 66 lbs. He's been eating very well on the steroids as always, so the weight gain is likely temporary, but I know this gives him an opportunity to have a normal appetite which is nice to see.

Because he is finally responding well to the steroids and anti-fungal medication, we can stop the Voriconizole next week! Also, next week we will lower the steroids further. They will check is IGE levels next week to see if we can lower the steroids or stop them.

The Voriconizole causes photo sensitivity and he has had a sunburn on his face, and his lips have been cracked ever since we started it. We are looking forward to seeing that heal.

He got a 99% Fev1 on his PFT's as well, and his lungs sounded great. We did an X-ray just to see how they look after all this hard work. I expect them to look great.

There is a new Pulmonologist added to the team, and after three weeks of seeing her name on prescriptions and lab orders, we finally got to meet her. She is fantastic and a great addition. We are blessed to have such a great team.

Labs, labs, labs

After going to Dallas Childrens every... single... week to get his blood drawn, we have been given the word that we can start to lower his steroid dosage! It is starting to feel like we're finally making progress after fighting Aspergillus for months. His IGE levels started lowering about 3 weeks ago, which shows that the anti-fungal is doing what it is supposed to. His liver enzymes have remained stable, which is a blessing.

High Liver Enzymes

So, he's only been on the Voriconazole (antifungal medication) for about a week, but at his first weekly lab draw, they saw his liver enzymes were elevated. I am surprised it happened so quickly. So, they have lowered the dosage of Voriconazole he is taking, and have added Ursodiol to help his liver out. Within a few days of starting Voriconazole, he started complaining of blurry vision, especially when it is dark, or very bright out, but this seems to have subsided. The Prednisone (steroids) seem to be making him a bit moody, but nothing to extreme at this point. It seems to be giving him redness in the face, but luckily no other side effects.

The past few days, his cough seems to be coming back. His lungs sound pretty clear, so I'm hoping this is just temporary.

ABPA Again

Two weeks ago Azer had a blood test for ABPA which is an allergic response to aspergillus. It took a while to come back, but he tested positive for it. It was also found in his CF culture that he grew another type of fungus called Scedosporum apiospermum. This is another commonly grown fungus in CF.

He will be started on a fairly high dosage of steroids and an antifungal medication. Because the antifungal medication can cause liver damage, I have to take him to have a blood test every week to make sure his levels are within a safe range. 

I am glad that at this point he can be treated without being admitted and orally!

Clinic Day

About 2 weeks before our CF appointment, Azer started to get a congested cough. No matter how much CPT I did on him, I just couldn't get all the mucus out (I always do CPT in addition to the Vest when he's sick. He has never coughed anything out during the Vest). With the stethoscope, he had diminished airflow in the lower lobes. So, the doctor prescribed the antibiotic Cipro for 14 days. Within a few days, his cough sounded dry again.

His oxygen was 100%. On his PFT, the highest he got was 93% Fev1 but the respiratory technician said that she could see he had a mucus plug. The doctor wants us to do Pulmozyme and Hyper-Sal twice a day instead of just once a day to clear up any remaining mucus. He hasn't gained any weight, but the doctor said that it tends to be hard to gain weight in the summer, especially how active he is.

It was time for annual X-rays and labs!

This machine was set up in the room where they drew his blood (it wasn't set up for him). I haven't seen it in 10 years. It is used to collect sweat to diagnose Cystic Fibrosis using electrodes.