August 23, 2019

Endocrine Appointment

Azer has been on antibiotics for about a week. A nurse popped her head in to ask him how he was feeling today. He feels better than he did last week. I wouldn't say he is back to sounding his normal best, as he is still coughing more than normal even though his culture came back with no Steno or Aspergillus, and just MSSA. His Pulmonologist thought it over and believes that since his new allergy shot, Dupixent, is helping to shrink his nasal polyps, that his sinuses are draining quite a bit and causing him to cough. She wants to treat his cough like a sinus infection, and continue the oral antibiotics, to make it a 21 day course.

It has been about a year since we've seen the Endocrinologist. She would like to see him every 4 months from now on. In the past year, he hasn't grown as much as she would expect. They ran some blood tests that will tell her if his growth hormone level is where it should be. She expects that it will need to be increased. She also ordered a bone age scan, to see how many years left he will probably grow. She mentioned he may have not grown that well this year based on how many times he has gotten sick and/or hospitalized.

He put on about a pound from last week. The Endocrinologist said today he weighed about the same as he did last year when she saw him.

We discussed the possibility of continuing growth hormones into adulthood for long term health and to also keep his muscle mass stable. She said when adults are deficient in growth hormones, they can have high cholesterol and other issues. We also discussed how high his glucose tests can be before it's time to start being concerned. His last OGGT was a bit higher than he normally is, but it isn't concerning at this point.

Azer also saw his PT today, who did sort of an out of the box test that is meant for athletes instead of CF patients. He did exceptionally well.

Azer was given tickets to one of the last Rangers game at the current stadium as a reward for helping out another CF kiddo at the hospital. He got to meet a former Rangers pitcher, Martin Perez, and watch his favorite Ranger, Josh Hamilton be inducted into the Rangers Hall of Fame. He also watched Nelson Cruz hit at batting practice.


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!

June 25, 2019

Sick CF Clinic Visit

Everything had been going great, and we thought we had the summer to relax a little after doing IV antibiotics. He felt great and sounded clear.

Sunday morning, he suddenly sounded a little congested. Within hours, he spiked a fever and was suddenly coughing up brown/rust colored mucus. The CF doctor on call suggested perhaps it was transiet, but to bring him to the ER if his fever got any higher. He took some ibuprofen and the fever broke later in the evening. Yesteday, he was still coughing up some dark mucus, but seemed to feel better. Today, Azer said his legs don't hurt, but they feel uncomfortable, and he has a headache.



At clinic today they did a virus swab to see if he picked up a virus somewhere. He gained several pounds from last week. His lung function was down slightly. His lung culture from last week found that the Steno is now resistant to Levofloxin. In order to treat it, he will have to be on an inhaled antibiotic for a month. The doctor warned us that the nebulized antibiotic smells like cat pee (awesome). They put in an order for a CT scan for in a few weeks. The doctor said if they tried to do a CT scan right now, radiology wouldn't be able to tell if he has fungus spores or not because of the increased mucus.

It will take some time to receive the inhaled antibiotics since they have to come from a special pharmacy. Azer is currently laying in bed not feeling well...

Update: Azer's virus panel came back negative for all viruses, so his symptoms are from bacteria and/or fungus. He was also told not to use his IPV while he is coughing up brown or red mucus, just in case he is having a slow lung bleed.

June 17, 2019

Follow Up Admission CF Clinic

Azer has been working hard this week. To try to avoid his dressing coming off from sweating, he has been riding the exercise bike everyday instead of running. 


We did bend the rules a little bit. He actually rode his bike while hooked up to the medicine ball IV.
Medicine Infusion balls make IVs at home easier

Today marked one full week on home IV's and a complete 14 days on IV antibiotics. Azer ran this morning to help make his lungs as clear as possible. He was disappointed to see his numbers were lower than when he was discharged. He says his lungs feel completely clear. He sounds clearer than he has in a long time. The doctor said it was probably time for a good clear out of the two bacteria he normally cultures. She explained in a little more detail about the ABPA test and his high IGE. It is possible that he does have Aspergillus growing somewhere in his lungs. She said at his next CF appointment, if his PFT's continue to fall, we will start an antifungal medication. In the mean time, she will do some more research to see if an allergy shot specifically made to lower IGE levels will help him. 

The good news is he put on 5 more pounds since being discharged. He has been eating well lately. 

Both Azer and the doctor felt like he had gotten the full benefits from the two antibiotics he has gotten these past two weeks, so he was able to have his PICC line pulled today. He can stop the Reglan and go back to taking his EES and Probiotics! 



Summer can finally start for him now!


June 10, 2019

Tentatively Discharged




Azer did his second PFT since he got here, and it was a little higher than last week's. He was annoyed that it wasn't significantly higher given that he had just done a breathing treatment and he felt really clear.

Azer's ABPA and Aspergillus test finally came back, and it looks negative for the most part, but the Pulmonologist said his IGE is still high, and there were particulates of aspergillus antibiodies evident. I'm not exactly sure what that all means, but she said basically we will go home continuing the IV antibiotics. At CF clinic, if his PFTs decline, they will do a CT scan to get a definitive conclusion of if he has ABPA nor not. If it turns out he does, he will either be readmitted or start a course of antifungal through his PICC line. 



Now begins operation "Hospital at Home" featuring Azer's mom as resporitory therapist, pharmacist, nurse, physical therapist and more! 😆




The Job

This kid fights every single day. Even when he isn't in the hospital, or on IVs, he has to work every single day. Pills taken with every meal. Breathing treatments to slow the progress of this disease. Exercises and stretches to keep his body from falling apart so quickly. Running and coughing to clear the thick, sticky mucus from his lungs. Boiling and steaming nebulizer cups after every use. Waking up in sticky formula because something from his G-tube came lose in the night.

Sometimes it can be hard to see your child fight so hard to breathe at times. He just keeps pushing. He never gives up.

Don't take the health of your kids for granted. This boy of mine has never woken up a day in his life and missed a breathing treatment. He has never had a meal digest perfectly normal. He has never known a day where he hasn't coughed. If we have a simple day trip, or just a weekend stay somewhere, we can't just pick up and leave. It takes real planning to make sure he won't run out, and has everything he needs to make it just a few days.

There is no "easy day" with Cystic Fibrosis. 

June 8, 2019

Undecided

Today, another CF doctor was on the floor. Azer and I asked her what was the best decision; go home Monday, or stay until the Aspergillus test comes back. She really feels like the best decision is to stay, because if that test comes back positive, starting a new IV medication at home in addition to the two he is already on would be doable, but difficult. She said on Monday, if it looks like the test will take a long time to come back, then it's probably alright to go home. When she listened to him, she said the same thing everyone else has heard; that he sounds crackly until he coughs, then sounds clear but has some wheezing.

Azer was talking to her about his allergies, and high IGE level, and she mentioned an allergy shot that specifically helps against IGE levels. Our main CF doctor and an allergist had mentioned it before. It wouldn't help allergy symptoms, but it would help if he gets ABPA frequently. 

Azer had to have another dressing change today because he was sweating from being so active while playing. He definitely seems to be feeling a lot better. 

It also helped cheer him up to be playing Xbox with Dad today 😊 

June 7, 2019

Staying A Little Longer

So, we have been debating whether to go come today, or wait. This morning, one of the CF doctors came in and helped with that decision. She said that she would feel more comfortable with him staying at least until Monday. The test for the Aspergillus and ABPA haven't come back yet. One portion of it came back and looked normal, but that still doesn't answer the question of why his IGE was so high. She was telling us of the order they usually treat things, and that they are first treating what they can see first, which was the bacteria culture. She said if the wheezing in his lungs wasn't improving, it's possible to do a CT scan do check for mold spores. 

Yesterday, we had tentatively planned to go home today, and I guess the case manager started the discharge process, so now we have the medicine and IV supplies ready to take home with us. They also changed one of his IV antibiotics from one that is every 6 hours to one that is every 8, to help our at home schedule.

Azer says his lungs feel about the same but better after doing the IPV. 

He had a dressing change done today, and we have arrangements that if we get discharged, to have the PICC team do the dressing change at clinic. 



Other than that, he has improved his foosball game, and annoyed many by playing a mean game of whiffle ball bouncing off the windows outside!



June 6, 2019

Higher PFT

Azer had a PFT today and his lung function has improved a lot. Azer says his lungs still feel full. He is still coughing up a lot during breathing treatments. This morning, his lungs sounded wheezy and crackly, but they sound clearer this evening.

The doctors ran his IGE levels when we got here, and they are pretty high. Azer's are almost always elevated, but it was high enough where they ran a test to see if he's culturing aspergillus. They are also running a test for ABPA. It takes a while for these results to come in. 

We are debating whether to stay admitted and see what the culture says, or try home IV's and see how it goes. It's always a very difficult decision... 

June 5, 2019

Off of Droplet Caution

Azer has been on droplet precaution since he got admitted because he tested positive for parainfluenza last week. Due to this, he wasn't allowed to leave his room, because of the risk of spreading it to others. We requested a retest last night, and this morning the doctor said it was all negative. He is still on contact, because he cultured MRSA back in 2010, but at least he can walk around outside of his room now!

Azer feels like his cough is better than it was on the day he got admitted, but it's hard to say if it's better than yesterday. His ears don't feel blocked anymore. His mucus isn't streaked with blood and is a lighter color. 

If his PFTs are improving, then there is the possibility that we could go home and finish his IV course. 

An interesting thing I learned: Azer can't be on probiotics while he has a PICC line because there is a possibility of a rare infection occuring. 

June 4, 2019

Living the Hospital Life

Working hard! He will likely be here until at least Thursday when he gets a PFT test to see if there are any marked improvements.





Last night

Azer had a lot of pain for a few hours after getting his PICC line. Hot packs didn't help much, but Tylenol, ibuprofen and time did.

In the middle of the night, they are thinking the blood clot at the PICC line site got disrupted,  and blood filled up all the dressing. They had to do a dressing change in the middle of the night. There is still some blood inside right now, and they will have to do another dressing change today. This can be extremely painful. Hopefully, the site is clotted better now. 

His Relizorb cartridge also ended up breaking while he was asleep and he had formula all in his bed. Needless to say, he was really ready to take a shower this morning!

June 3, 2019

Admission

Yesterday, Azer said he wasn't feeling much better. This morning, he went for a run to try to clear his lungs and had to stop because he felt like he just couldn't breathe well enough.

His CF culture from Wednesday hasn't come back yet, so he will be on the same IV antibiotic as he was taking orally this week. He will also start a broad spectrum antibiotic as well. 

The PICC team was able to come by and put his PICC line in this evening, so he didn't even need to get one IV. One of the PICC team nurses was saying how brave he is to be able to have one put in without sedation.