May 13, 2018

Help Us Find a Cure - Great Strides 2018

Click here to donate to our Great Strides 2018 Walk!

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

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!

January 23, 2018

Hunger and Pain

About 3 days after Azer ended his course of Prednisone, he started to feel very hungry. It is common to feel hungry or have cravings on steroids. But this began days after he ended it, and has continued. He is eating as if he was on very strong steroids or Reglan. We are starting to wonder if it's caused by the Growth Hormone Injections. Either way, we aren't complaining.

For the past few days, his chest/lung pain as started to return. He was doing a 30 minute run Monday morning, but had to stop a few times because of sharp, right lobe pain. CPT has been also painful for him, as he says his entire chest is sore. He emailed the CF clinic, and the doctor says it might be Costochondritis, which is basically inflammation of the cartilage that connects the ribs. She said to try a ibuprofen 3x a day for the next three days. If it has been relieved, this is probably what is causing his pain. 

Azer had a tough time breathing outside yesterday, but it wasn't until the evening we realized there were some very large grass fires not too far away. We couldn't smell any smoke, but it was extremely windy.

January 18, 2018

CF Clinic

This was basically a follow up appointment after Azer's hospital visit.

So, when Azer started growth hormones, I put him on 5 cans of formula a day instead of 4 to try to keep his nutrition up, since he hadn't gained any weight in quite a while (he also wasn't growing either). He gained two pounds since then, so the GI doctor said we can go back to 4 cans a day, since he's been having reflux and feelings of fullness in the morning from the extra formula.

The GI doctor said that in the event we have to use the antibiotic, Levoquin again, and are forced to stop Erythromycin, she said it might be worth while to temporarily give Azer a G-J tube instead of just a G-tube. This would help assure that he isn't refluxing formula into his lungs. Another alternative would be to temporarily start Reglan which worked wonders for him in the past, but does come with a black box warning. 

His PFT's went up, above where they were in October. The doctor was pleased with those results. She allowed us to look at his X-ray, and explained why the radiologist's report can sound completely devastating because they really don't see the full picture.

The doctor took her time and really explained to Azer the anatomy of his lungs, and what he was actually seeing on the X-ray.

While clinically, he looks great, and he didn't culture anything serious, Azer told the doctor he felt like his lungs still feel sick and congested. She said that even though the antibiotics are really causing a lot of GI problems right now, that we should finish the course. She listened to Azer's lungs, and heard some wheeze and noises. She said it was a good thing that the noises moved around when he coughed or did treatments. She is adding a secondary inhaled steroid to the one he is using already. This is temporary until he starts feeling better.

Regarding, the continued bloody mucus Azer's been coughing up, the doctor said as long as it's mucus with blood in it, and not blood alone, or blood not mixed with mucus, this blood we are seeing is just from irritation, which is likely coming from the Hyper-sal, steriods, and the dry, winter air.

Azer hasn't been able to return to school yet, and the main problem is how many times he's been in the bathroom a day. We counted 8 watery diarrhea BM's yesterday, and the average is 6-8 times a day since he's gotten discharged. He is almost done with Flagyl, and the GI doctor said Flagyl doesn't always work immediately. They took a stool sample to check for any other bacterial overgrowth that might be causing him issues (when Azer came back from "getting the sample", he said, "I am not nurse material").

Based on this situation, we will be doing homebound school for the next few weeks, which gives more time for Azer to work on staying hydrated, and more time for extra breathing treatments (which, based on his PFT's, seems to be working!) I contacted this school this week, and the staff worked hard to get me the information needed. I was able to get all the paperwork and brought it today for the doctor's signature.

I brought up the PEP device we got at the hospital with a nurse and the Respiratory Therapist. They said they don't have them there at clinic, and really hadn't seen them used. While we were all talking, we got on the subject of the Vest. The RT was surprised that we had a SmartVest. She mentioned that most patients use the Hill-rom Vest, and was able to quickly call the company and find out if we could try one out on a trial basis. She said once we get our information settled with Hill-rom, we can be put on a waiting list for a new Vest called the Monarch. This new vest is portable, and is supposed to work more like hand CPT rather than just pressure and vibrations.

Side note - Wednesday the 17th, after he'd been on the Prednisone for about 5 days, his sense of smell started to return a little. Two days later, he can smell nearly everything. So, it's either the steroids reducing inflammation, or the antibiotics killing an infection in his sinuses.