September 21, 2019

CF Clinic Follow Up

So, this week Azer has been off of Periactin, and his allergies are in full swing. His PFT's were down today, but we weren't surprised about that. The doctor listening to him, and historically he has a lot of wheezing and asthma symptoms this time of year. Sometimes it's so bad he needs to be on antibiotics, because the nasal dranage ends up in his lungs. This time, she heard no wheezing, and his lungs sounded totally clear. We all think the Dupixent is working well for him. Azer told the doctor that he doesn't think the Periactin is helping his appetite that much right now. He said he is so hungry lately after working out in baseball class at high school. So, she told him he can stay on Periactin, and not take a week off, at least until it gets cold and everything outside is dead. All the cultured on his last sample was MSSA, which is great!

His weight has remained exactly the same. Azer feels like he has lost some fat on his body, and was worried he was losing weight. Luckily, his weight has remained stable from him putting on so much muscle.

He brought up a concern to the doctor, social worker and physical therapist. Apparently they use some sort of powder in the gym for griping the weight bars, which isn't just bad for healthy people's lungs, but very bad for him. Azer is going to try to advocate for himself at school first, but if nothing changes, the CF team will try get the coaches and school to take action about this.

For over a week, Azer has had lose stools several times a day. He was very dehydrated yesterday, to the point of having a headache, and neck pain. Between the diarrhea and the nasal drainage, he was pretty miserable. The doctor figured out the time line of when his three weeks of antibiotics ended, and believes it might be from bacterial overgrowth, so she put him on Flagyl to see if it helps get his stomach back on track.

Azer has been noticing more and more of the possibilty of ADHD. I personally have it, and have recently started taking medication for it. He has seen such an improvment in my life, that he decided he wants to persue a diagnosis and treatment. Since a lot of the ADHD medications can affect appetite, we wanted to ask the CF doctor about this first. She said the primary thing for him is she wants him to feel better, be able to function in school and life as best as he can. She said that if it does affect his appetite, we will roll with it and figure out options for him. This was a big relief!

Another concern we had was about the Zantac he takes 3-4 times a day, and the concerns of the cancer causing ingredients that has been in the news. The doctor said it is only certain types of Zantac that are concerning, and that the levels are so low, that it isn't a cause to disrupt Azer's delicant balance of his GI system right now.

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!