May 13, 2018

Help Us Find a Cure - Great Strides 2018



Click here to donate to our Great Strides 2018 Walk! http://fightcf.cff.org/goto/lilhyperfingers

I recently had the opportunity to attend a meeting and heard Bruce Marshall, the VP of Clinical Affairs for the Cystic Fibrosis Foundation speak. He played this video, which of course, drew tears from everyone in the room. My own tears came from gratefulness for the people who have been helped with these new medications, and also sadness, because those pills aren't available for Azer yet. I feel like we've been told he will have them soon, for many years, but it wasn't until this meeting that I knew, it will be very soon.

This is an extremely costly endeavor for the CF Foundation, and they will continue to need money to fund further research for mutations like Azer's, and for new medications that will continue to treat the symptoms of CF.

Great Strides is the largest fundraiser the CF Foundation holds annually. As a family, we stride every year, and raise money so that we can soon, have these life saving medications. Will you join us in raising money and lacing up for a cure?

Why the Cystic Fibrosis Foundation Strides for a Cure

Watch Joe Flacco, QB for the Baltimore Ravens Speak about why he makes Great Strides for CF


April 13, 2018

CF Clinic

Saturday night, Azer started to feel like he was getting sick. Sunday morning, he was very sick. He had a high fever, lower back pain, and a sore throat. Fearing the flu (again), I went to have him tested, and luckily he was negative for the flu, but positive for Strep throat. He was prescribed an antibiotic, and initially felt better, but Monday evening, the back pain returned with vomiting. He was able to stay hydrated thanks to the G-tube, and by Tuesday, most of his symptoms were gone. As with just about any virus or bacteria, it made his lungs a little junky, so his PFT's were down yesterday. His lungs sounded pretty clear otherwise, so most of it was drainage from his sinuses.

Speaking of sinuses: It seems like the nasal rinses with the added vial of Pulmocort are working. He noticed he was able to smell a little bit a few days ago. He also noticed that he's been flushing out some blood from his sinuses, but apparently, while steroids reduce inflammation, oddly enough, sometimes they can cause a little as well. The doctor explained that the tissue in the sinuses is very thin and fragile and it doesn't take much to cause them to bleed, and not to be alarmed, that this is very normal, and to keep going!

None of the doctors were able to really explain the back pain for sure but, sometimes Strep can irritate the kidneys. It could have also been pancreatitis, which apparently does not occur often with Azer's CF mutations, but it does happen. Even though pancreatitis typically causes abdominal pain, the doctor said some CF kids only feel it in their back. The doctor said it also could be from kidney stones, which are much more common in CF.

Yesterday, they measured Azer's height, and he is actually growing! The growth hormones are working, and he is also putting on muscle. Unfortunately, he isn't putting on weight to go with it, so his BMI went down. The GI doctor said we can try adding Periactin to spur on his appetite a little bit. The Pulmonologist also agreed it would be great, as Periactin is also a type of allergy pill. When it comes to allergies, Azer is pretty much symptomatic to most things during the spring. The GI doctor said we can also try to add a calorie additive to his overnight feeds to try to increase caloric intake without increasing volume (which sometimes can cause his reflux to worsen). Azer has been wondering lately what he would have to do so he wouldn't have a G-tube anymore, so he spoke with the GI doctor about it. She said he would definitely have to eat three meals a day, and make sure to add fatty additives to them (like butter, heavy cream, extra cheese, ranch). It's also difficult to consume thousands of calories through food alone, and he'd likely have to supplement with several shakes a day. She also said that right now is a critical time to keep his nutrition high because of the growth hormones, and if he's not taking in enough calories, the growth hormones just won't work. This is something we can revisit in a few years.

Azer told the Pulmonologist that his ears have been blocked and hurting since Monday. She looked in his ears, and saw that he has double ear infections. Because of this, she is changing the antibiotic he is currently on for the Strep, to a different one that should be better at targeting his ear infections. Azer had a baseball game yesterday evening, and he reminded me of a game he played when he was 6 years old and he also had a double ear infection that day. That day, he was standing on the pitcher's mound crying because his ears hurt so bad, but he wouldn't get off the field because he wanted to play so bad!



Azer also brought up a concern he's had since December. He told the doctor that when he sprints, he has pain in his right lung, and that he was worried the pain was from the partial collapse he had from the flu. The doctor assured him that from his last X-ray, that area has recovered, and that the pain may be from allergies. She said she was fine with him doing Dulera and Qvar twice a day (two inhaled steroids) for the next 6 weeks. She says she has some asthmatics on this combination and they do well (she said the Endocrinologist might not be as pleased with this, as steroids can inhibit growth).

Azer met the clinic's genetic counselor for the first time yesterday. She discussed the genetic side of CF, how someone acquires it, and also what the chances are of his possible children having CF. We talked about what his CF mutations are. He has one common gene, and the other is very rare, and isn't even in the database. With all this talk of treating CF on the DNA level, I asked her about the possibilities of treating his common gene, but unfortunately, there isn't any drugs in the pipeline to treat his variance at the moment.



It's been about a year, since Azer was first evaluated by the clinic's physical therapist. She measured his form in several places, and saw that he was digressing again. She said he may be degrading from puberty and growing. In order to correct this, he will be needing to see her again frequently this summer.

Azer's G-tube has been bothering him for a few months now. The skin around the stoma has been cracking and irritated. An ostomy nurse came and looked at it. She found he only had a little over 1 cc of water in the balloon. Azer had removed some water for comfort a few months ago, but since then, he's lost a little weight making the button fit loser. The nurse but the recommended amount of water back in, and so far, it seems to be fitting much better!

April 5, 2018

ENT

Today, Azer saw ENT a few months sooner that an already scheduled appointment, mainly because he cannot use a nose clip during his nebulizer treatments anymore. He says it feels like the nose clip is squishing something in his nose, and it hurts. He also has had no sense of smell for the past few months, and allergy season isn't making it any better.


 The ENT doctor looked with the scope today, and saw a few polyps, and some inflammation, but nothing bad enough to schedule surgery yet. She wants Azer to try to put a full vial of an inhaled steroid medication in his nasal rinse, and to do it consistently four days a week. She wants him to try this for the next 6 weeks, and if it hasn't worked by then, to try another course of steroids, to try to shrink the polyps.



Azer sent these screen shots to me the other day from Instagram. I thought it was so cool that this Rangers Fan's page shared Azer's website and is helping raise awareness for CF! Thank you #txrangersfanatics!

February 15, 2018

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.



January 30, 2018

ENT and Going Back to School

Azer says his lungs are feeling a lot better, so we backed off of the extra Qvar (additional inhaled steriod). After three days on the ibuprofen for the costochondritis, he said the pain left the lower area of his rib cage, got a little better at the top, but didn't go away completely. He let the doctor know, and she said he could restart the ibuprofen and continue it as long as it doesn't bother this stomach. The pain during CPT is gone now, thankfully.

Since he's been out of school three weeks, this week being his fourth, he is very ready to get back to school. After speaking to his homebound teacher and our social worker, we found out he isn't necessarily required to finish out the 4 weeks of homebound instruction if the doctor feels he is ready to return to school. The paperwork was completed in only a few hours, and he will return to school this week! He is excited, because this means he can also participate in baseball, as practice starts in just a few weeks. He has been feeling well enough to practice nearly everyday on his own.


As I said in a previous blog post, two weeks ago, Azer finished a short course of Prednisone after which he regained his sense of smell. We told this to the ENT doctor today. She said that this is common as the steriod causes the polyps and swelling to go down. I asked how long we could put off surgery with the steroids. Obviously there are numerous side effects from being on steroids constantly. She wouldn't recommend it more than a few times a year. She said in the mean time, while the swelling is down, that he should go back to doing nasal rinses once or twice a day to keep the mucus clear. She said he can help keep his olfactory nerve active by smelling strong smells twice a day, such as coffee or oregano, and to change up the smell every 4 weeks. He's already been doing this on his own, enjoying smelling many things he's missed out on. It's pretty remarkable how he comments on how things smell, as if he's never smelled certain things before. Having a sense of smell isn't necessary to live, but losing it does diminish quality of life, and it's fun watching him enjoy smelling and eating!