CF Clinic and Sweat Test

For about two weeks, Azer has been experiencing symptoms of either a virus or allergies, with worsening CF symptoms. He called his doctor on Monday and she told him to take a high dose of OTC allergy pills. It has helped some, but most of his symptoms are related to his sinuses. He felt well enough to wait for a clinic visit for today.

Today Azer was 10 pounds less than he was when he was discharged from the hospital. He hasn't been able to do all of his night feeds as he his stomach has been filling up with mucus. There weren't too many changes in his routine so far. They are running a virus panel, but will likely put him in steroids for about a week. His lung function was lower than it was when he was in the hospital, but they want to see him back in two weeks to make sure it doesn't start tanking. 

Today, the clinic researcher scheduled Azer for a sweat test. This is a Trikafta study to see if people on Trikafta have a lower salt value in their sweat than before they were on Trikafta. People with CF have an abnormally high amount of salt in their sweat. Azer had this test when he was born to confirm he had CF before his mutations were found. It will be interesting to see if his salt concentration is still high. 

CF Clinic

It's been about 4 weeks since Azer's surgery. He has been throwing up pretty much with each meal. The GI team said he should make sure everything he eats is basically moist or wet and that he should try to chew longer and take smaller bites.

He has lost 7 lbs since getting discharged from the hospital. His weight loss is due to him not being able to eat as much as he usually does. He also had a braces adjustment where they are correcting for a cross bite, and the elastics are causing a lot of pain, even weeks later. Azer is already off of one of his stomach motility medications. Today they want him to stop one of the antacids he's been taking for a few years. They said the goal is to stop taking most of his GI medications. 

About a week ago, Azer started having an increased cough and was coughing up darker mucus, pretty much all day. After increasing treatments, he started to feel a little better. His PFTs were pretty much at his baseline today, and a little higher than when he got discharged from the hospital. The doctor said his lungs sound pretty clear except for a few crackles. They wonder if most of it is draining from his sinuses and think a visit to see ENT might be helpful. In the meantime, they want him to use an anti-inflammation nasal spray before his nasal rinses. 

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! 

CF Clinic Sick Visit Follow Up

This was a follow up to Azer's sick clinic visit last month. He hasn't had another asthma attack since then, and has been breathing fairly well seeing as we are deep in the heart of allergy season. He felt like his PFT's could have been higher, but that he is a little bit more mucusy than normal.

Adding the extra half a can of formula to his night feeds has help. He gained two pounds in three weeks. The dietitian and doctor gave Azer lots of good ideas on other ways to add calories to things he already enjoys eating, including some new snacks he might like to try.

Time management has been difficult this school year, seeing as it's his first year of high school, and first year playing sports in school. I've always told Azer when he was little, because of the CF, you're going to have to work harder than others at everything you do. The social worker today basically said the same thing. Having CF is hard, and makes it harder to have extra time to do what you want. Azer is still learning about what works for him, what time of day works best for him doing treatments and homework, and how exhausting it can be getting to everything late everyday because of practice. He is so passionate about baseball, and we are all doing everything we can to help him continue to play!

CF Clinic

Azer had an early CF Clinic yesterday, followed by two games an hour away, so needless to say, he is pretty tired today!

Azer lost a few pounds since his last CF appointment, and hasn't grown enough to see a change. The dietician came in to discuss what he eats and how to add calories without disrupting his G-tube feeds too much. He is eating snacks and his appetite has been pretty good lately. He packs his own lunches and fixes his own dinner since he knows what he is craving at the time. So, the only change is to increase the Liquigen added to his night feeds for a small increase in calories. Since he has been lifting weights several times a week, and has had baseball practice nearly everyday, he has been burning quite a few more calories than usual. He has also been visibly putting on a lot of muscle. The dietician mentioned that sometimes the CFF focuses so much on the number of the BMI, without taking into consideration muscle mass and body fat. It would interesting to see the long term effect of a healthy BMI with the a measurement of muscle mass versus a higher BMI and a higher percentage of body fat.

The doctor said to not worry too much about the growth just yet, as the Endocronologist looks at the long term trend.

Azer's Fev1 looked pretty good today. His next CF appointment will be in only a few months instead of longer because of allergy season coming up. We discussed with the doctor that Azer hasn't been able to be consistent with his Periactin as sometimes he gets home too late to take it. If he takes it right before bed, he is so sleepy the next morning, he can barely function. Whenever he misses a few dosages of Periactin during allergy season, he gets so symptomatic, that he feels like he's getting a lung infection.  One of the assistant doctors suggested a prescription allergy medication that is not an anti-histamine so it shouldn't make him drowsy. Hee can also take it with or without Periactin.

About two weeks ago, Azer started to have a sinus headache. Nothing seems to touch it. One morning, during his breathing treatment, he started getting a nose bleed that lasted an hour. He tried to not blow his nose that day, but it started oozing again that night. The weird thing was after the nose bleed, he said he could smell a lot better, but that it was very sore. We let both ENT and his CF doctor know.

Update: ENT said that the nose bleed was possibly caused by winter time dryness, and to use the antibiotic ointment to keep it moist.

CF Clinic

A few weeks ago, Azer went to the allergy clinic for a consultation. He felt like he didn't get much out of it. We mentioned the allergy shots that target IGE that his pulmonolgist said helps some CF patients. He didn't feel like it would help Azer, but did recommend restarting his nasal spray and prescribed some eye drops for eye itchiness. The allergist said that if/when Azer gets ABPA again, that there is a shot that would benefit him at that time.

At clinic, we told the pulmonogist about how the allergy clinic visit went, and she still believes that the shot would help Azer, so they will do some discussions on how to proceed.

Azer lost a little weight, but his BMI is still great. However, the doctor really wants to see him work on packing a better lunch for school, and trying to eat within the time range of his enzymes working. She also recommended that he should consistently add Liquigen to his night feeds, as this is a ready to absorb fat that is easy for his body to use. He should also consistently use Relizorb, which helps him absorb the fat from the formula all night long. She would like him to continue to gain some weight, to have a cushion for whenever he gets sick, as he can lose a lot of weight within a few days when he gets sick enough.

Azer felt like his PFT's weren't going to be great, but managed to do better than he expected. He sounded fairly clear, despite his wet sounding cough. He continues to feel sick when he takes a week off of the Periactin. The doctor suggested taking more OTC allergy medication and increasing nasal rinses during the off weeks. The Periactin has been working great for his appetite, but makes him very drowsy in the morning. The doctor suggested taking it earlier in the evening, so it would perhaps be out of his body sooner.

He has had a lot of nasal drainage over the past week, to where he describes it as feeling like a stream going down the back of his throat. The doctor looked and saw a visible polyp in one nostril. She wondered if the polyps are bad enough to be occluding his sinuses forcing the drainage down the back of his throat instead of draining out of his nose. We may be going to see ENT a bit earlier than expected.

Azer decided that Mr. Glass Head needed some protection this winter

I would like to take this opportunity to remind everyone to please get their flu shots. It not only protects you, but also protects those in our community that could potentially die from flu complications. If you need proof that it works, take a look at how many died last year, when the flu shot was much less effective than usual!

CF Clinic

Today was a long clinic day, time wise. It was time for Azer's annual blood draws and OGTT (Oral Glucose Tolerance Test). He had to be fasting, and had his blood drawn as soon as we got there. He then had to drink the nasty, sweet glucose liquid (he drank it like a champ). They test his blood again two hours later, so we had plenty of waiting time today. 

We were lucky enough to have a large and comfy clinic room today, which was nice.

There was some confusion on his PFT results today. His height was entered into the computer incorrectly today, which changes the margins of the lung function numbers on the computer. So, initially his numbers were way low. When his height was corrected, he was one point higher than last week. So, he isn't as high has he wants to be, but he is within his normal range for this year (it's generally expected that lung function decreases as the years go on).

We spoke some more about possibly seeing an Allergist and an allergy shot. The doctor said generally, allergy shots don't help people with CF, specifically those whose IGE levels are in normal ranges. However, since Azer's IGE is always high, that the allergy shot she wants to try specifically targets IGE levels. This could also mean it could help him out the next time he ends up growing Aspergillus. 

For the past year, Azer has been talking with our Social Worker about Make-A-Wish, researching different possibilities, and reading about other's wishes. He feels like he has come to a decision about what he wants (he really wanted something that he could enjoy with the family, rather than something solely for himself, because he felt like that would be selfish. I told him he can be selfish in that this is his once in a life time wish, but his mind is made up :) So, he brought it up again to the Social Worker, told her what his wish is, and she will make the request! (He or I will share his wish at a later time)

I really can't believe he's old enough for this, but in a little over a week, he will be 15 years old, and he will get his Learners Permit. I asked the doctor if there were any CF restrictions on driving (there aren't any), I asked the Clinic Physiologist about it, and she recommends that I go on some calming medication, our social worker merely wished us good luck, and when I asked the group of them if they thought Azer was mature enough to drive a car, he proceeded to dab. 

Dabbing in his Dabbing Shirt

CF Clinic Follow Up

Today, Azer weighed about the same as last time. It's hard to tell on the growth chart, but we believe he grew some. Because his height is measured at Endocrinology, ENT, and the CF Clinic, and entered into the same growth chart, there are small differences between the measurements at the different clinics (as in, it appears he shrinks lol). But compared to his last CF clinic visit, he has grown a little. One noticeable difference (much to our PT's delight) is that he definitely putting on muscle because of the growth hormones. Another wonderful side effect of the growth hormones (and probably because of puberty as well) is that he has been truly hungry and eating full meals.

One concerning symptom he has had for the past few weeks is hip pain that seems to be worsening. The PT gave him a few stretches that might help, but when she looked and felt him over today, she was concerned the pain was not muscular, that it was in the joint. Hip pain is one side effect that we were told to watch for with growth hormones. We were sent to do a pelvic X-ray to see what is going on.

He also did a chest X-ray to see if that portion of lung that was plugged and collapsed has recovered.

His PFT was fantastic today, likely because he is on Qvar as well as Dulera. The doctor said it's fine short term, but long term being on two inhaled steroids can inhibit growth, and can also affect adrenal glands. So, when he feels bad, she said he can do Qvar for three days at a time.

The doctor also felt like when he had all that chest pain after the flu, that it might have been pleurisy, which she said has been very common with the flu this year. After recounting all the things to the doctor that happened after Azer got the flu has been remarkable.

He was put on an antibiotic that forced him to stop one of his GI motility medications.
This caused him to reflux and vomit, and made it harder to eat and stay hydrated.
They switched his antibiotic to one that would allow the GI medication.
He ended up with severe diarrhea, which forced us to do home-bound education.
He was put on a medication for intestinal overgrowth.
All the congestion in his lungs caused inflammation to the point of pain.
An area of his lungs ended up plugged and partially collapsing.
He was put on steroids, both inhaled and orally.
He coughed up bloody mucus, and had to stop an Hyper-Sal which made him feel congested.
A week later, he experienced chest pain either caused by inflammation of the cartilage of the chest wall, or the lining of the lungs.
A week of ibuprofen, and everything was back to normal!

The flu is something no one wants, and having it twice on top of having CF is potentially deadly. This is just a friendly reminder that if you are ill, please stay home. Get the flu shot if you can. You could save someone's life.