August 16, 2019

CF Clinic - Sick Visit

This year is Sophomore year!



So, the day after his clinic visit last week, Azer started to sound congested. It seemed to get worse through this week. His culture from last week came through, and the Steno wasn't present, but a rare growth of Aspergillus was there. Yesterday, he and I came down with a mild virus, which didn't cause many symptoms and seemed to only last a day. However, he ended up coughing hard all night long. He said at school, he was trying to breathe shallow so he didn't end up having a coughing fit in the middle of class. He was exhausted this morning, and sounded even worse. He emailed the clinic, and we started heading in that direction, not knowing whether they could see us in clinic or not. Luckily, they had an opening in the afternoon, which was worth waiting for.

He lost 3 pounds in one week. He has been a little more active since school started, but has been eating fairly well. He had some difficulty doing his PFT today, as he was holding back coughing. He was only a point down from last week, but several from a few weeks ago.

The doctor is still trying to determine if his cough is coming back from not being on the Ceftaz, if it's from the fungus, which may or may not be colonized, or just allergies. She listened to his lungs, heard no wheezing and said they sounded so good, that she didn't see the need to order an X-ray, and she didn't feel the need for him to be on steroids right now. She looked in his nose and saw some polyps and the evidence of a lot of post nasal drip. She wondered if since the Dupixet is helping his sinuses so much, if the inflammation going down has allowed other junk that has been stuck up there to come out. Either way, he is going to try some oral antibiotics for a few weeks to see if he starts feeling better. If he's not much better, we might start to treat the fungus. In times past, it has taken months and months to treat him for ABPA and aspergillus because they were waiting for his IGE to get lower. It is almost always high whether or not he is culturing fungus. The doctor said if we have to treat it, she will treat him until his symptoms improve.

August 7, 2019

CF Clinic


Azer gained three pounds from last clinic visit. He really struggled and worked hard to get a good PFT today. It was a few points down from last month, and he was really frustrated about it. He has been running and feeling pretty good, but the high heat and humidity has been very rough on his allergies.

Last week, we received Duplixant, a new shot that helps treat the inflammation of asthma. The first dose is two shots, and the needle was intimidating looking, but he was brave and gave both to himself. He asked the doctor how soon he might see any improvements, as he still feels like he's having lung issues due to allergies. It may take a few more dosages, but he has already stopped having sinus headaches, which the doctor said by itself may be worth it. She listened to his lungs, and said that almost every year around this time, Azer's lungs sound wheezy, but that he sounded clear today. So, she thinks the shot may be helping already.

At last clinic, his culture came back clear of the Steno. If this one comes back with the Steno again, and he's not feeling that great, he will start the Ceftazadime again, and continue to cycle it every other month. Hopefully, his lungs will remain clear for the start of school!

July 18, 2019

CT Scan and CF Clinic

Today, was a long CF clinic day. Azer ran first thing in the morning to clear his lungs. 

We checked into the main hospital for a CT scan.



Azer was coughing a bit while following their instructions, but it was done pretty quickly.


Azer had an OGTT (Oral Glucose Tolerance Test) today, so he had been fasting since midnight. When we first got to clinic, they drew his blood to test his fasting level. After, they gave him the sugary drink, and he had to wait two hours until the next blood draw. 


We  recently went on vacation to New Mexico. Azer took the week off from his night feeds and boluses. Over the week, he lost about 7 lbs. He managed to put most of it back on by today, but he was still a few pounds down from his last clinic visit. The dietitian was a little worried about his ability to keep his weight up, and reminded him of the importance of being responsible about eating well eat meal times. 


Azer's lung function was much higher than last time! He was almost near his baseline from last year. The doctor wants him to finish up the Ceftazadime, and then take a month off. In a month, they will see if his lung function remains stable or drops. If it drops, we may have to do alternating months on Ceftazadime for a period of time. The doctor explained that treating Steno through IV caused it to become resistant to Levofloxan, but that by inhaling the antibiotic, it attacks it more aggressively. Azer asked why the medicine has to be prepared with a needle, and can't just be in a normal vial like his other inhaled medications. The doctor explained that we are basically using the Ceftaz "off label", meaning he is inhaling what is meant to be administered through IV. 

While we were on vacation, we quickly realized how good the air there was for him. The very first day he woke up, his mucus was clear, and easy to cough out. It was an eye opener about how much the allergens in the air affect his ability to breathe. The doctor couldn't say definitively whether his allergies can damage his lungs overtime, but she told Azer they are working on getting insurance to cover an allergy shot that would hopefully make his lungs feel as good as they did in New Mexico. 

Radiology hadn't made a report on the CT scan at the time, but the doctor did show it to us. She said nothing was particularly worrying about it, but she was able to show us multiple bronchiactasis areas. This is damage to areas of the lungs that cause  thickening and scarring. 


Azer didn't get PT today since he was fasting, but his PT spent some time lovingly measuring (torturing) his form today. She noted areas that he needs to spend extra time stretching, which will ultimately help his ability to cough. 

Azer was very hungry by the time we left. We were there for more than 4 hours, so it was a long day. He had Taco Bell which is his most favorite place to eat! 






June 29, 2019

Ceftazadime

Azer's new inhaled antibiotic has taken a little learning. An exact amount of saline has to be withdrawn by needle and mixed with Ceftazadime powder. Then, a different amount has to be drawn from the Ceftaz vial and inhaled at the end of his breathing treatment. The doctor was right, this stuff does smell similar to cat pee. Azer says it tastes awful, and he has to use a nose clip and a mint to get through it. 

We all really hope this will finally kill the Steno! 

June 26, 2019

CPT, The Vest and IPV

So, January of last year, I posted about our Respiratory therapist bringing up whether Azer tried the Hill-rom vest before.  I realized today I had never followed up with a post about whether anything came of it.

It seems most people in the US who have CF ended up with the Hill-rom vest. Back when Azer was sized for a vest, the only one he was big enough for at that time was the vest by Electromed. Nothing against that particular vest (what works for one person with CF may not work for another), but it never seemed to work for him. He would never cough or bring up mucus, ever. So all these years, I have been doing manual, hand CPT. It was helpful when my husband began helping me with CPT, as it lightened the load. We invested in a massage table that could adjust in height to make it more comfortable to stand for long periods of time. We also bought plastic ramps that helped him lay at a decline for autogenic draining.



Doing manual CPT is very effective for Azer, but it can be physically exhausting for us, and also sometimes it is uncomfortable for Azer, especially if he is sick and his chest and lungs are sore. 



We also do assisted exhales (Azer likes these). As CF creates obstructions than can be difficult to move by huff coughs alone, these help him bring up some of the bigger mucus plugs. 
So, last year when our RT brought up the possibility of trying out a different vest on a trial basis, I thought, what do we have to lose? Our RT put the call in, and soon a nurse came to our home and showed Azer how to use it. Within seconds of him putting on the vest and turning it on, he began to bring up mucus. We were both so surprised. After so many years of trying to get his old vest to work using every setting it had, here he was effortlessly bringing up mucus. 


What a weight off our shoulders! We were now able to leave the house and not worry about getting back in time to do a half an hour of manual CPT. Azer felt more independent and confident that he could do his breathing treatments on his own. He was also able to leave on youth trips and sleep overs for the first time, knowing he would be to get effective mucus clearance without depending on us. He can get consistent breathing treatments over 4x a day when he's sick without worrying about putting a huge strain on time and our physical abilities. 



It took some time, but eventually our insurance told us it would be paid in full! 

Last summer, when Azer was admitted, he was able to try out the IPV for the first time.  The IPV (Intrapulmonary Percussive Ventilator) works like a powerful nebulizer, and pushes percussion air into the lungs. He liked it a lot, so while we were admitted, we asked about the possibility of getting one for home use. This can be incredibly difficult to get insurance to pay for one. It can also come with an increased risk of getting a lung bleed, due to it being quite aggressive. The machines for home use aren't nearly as strong as the ones used in the hospital, so they are safer. 



The company that we were supposed to get the IPV from informed us that we wouldn't find out IF the insurance company would cover it until we received the machine and started using it. I felt that it would be sad that Azer would receive the machine, and benefit from it, but then we would possibly need to send it back if the insurance company wouldn't pay for it. I went ahead and said yes, despite the risk. Our RT and the company said they would fight for us to keep it if we were denied. 

Azer used the machine for several months, and really liked it. He would use his vest in the morning, and the IPV at night. One day, the tubing started leaking air around where it connected to the machine. The tubing typically is replaced every 6 months. When I looked at the tubing closer (it's also called the circuit) I noticed mold had been growing in it! Azer was annoyed when he saw it, and said, "great, so it's basically been pushing moldy air into my lungs". I called the company to see how we could get a new circuit. Since the insurance had been continually denying the machine, they wouldn't pay for the circuit either. The company told me that the replacement circuit would be $500. I told them I could not afford this, so unfortunately, Azer was unable to continue to use the machine. 

At our next CF appointment, we told the RT what had happened. She had an idea to try out another medical supply company. It took quite a few months, and a LOT of work on her end, but eventually one day the new company called us and brought a new IPV machine over. 



The machine itself is much lighter and comes inside this cool carrying bag. The circuit is much less complicated, as it only has two tubes. 




The handset is also very different. It is a new style, and we noticed on Azer's most recent admission that they are using this handset in the hospital as well. Azer says this IPV isn't has strong as the one in the hospital, nor is it as strong as his previous IPV machine, but it still moves the mucus out. 

So far, it seems like the insurance company is willing to pay for this IPV machine!