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!

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.

January 14, 2018

No Pneumonia

So, the official results of Azer's X-ray came back. He doesn't have pneumonia, but he does have a small area of atelectasis. Basically he has a small area of his right lung that is collapsed. It is blocked from inflating by mucus plugging. Apparently, this is a common thing that happens in CF, and sometimes it may not be accompanied by symptoms.

Twice when he was in the hospital, he coughed up blood streaked mucus. This morning, he initially had coughed on his hand and noticed a drop of bright, red blood on his thumb. He then coughed up some pink, blood streaked mucus. I called the on-call CF doctor who said we should stop doing Hypertonic Saline, and if this doesn't resolve, we have to be readmitted...

January 12, 2018

Still Recovering from Flu

Azer's lung/shoulder pain has been off and on today. CPT this morning was relatively painless. However, the second and third breathing treatments hurt him a lot. He says his lungs are feeling less congested than yesterday.

While the strong antibiotics he's on are helping his lungs out, they are not helping his intestines. He's had strangely colored, constant watery stools. With the fear of possible C-diff, he is now on a third antibiotic, this one to treat bacteria flourish in his intestines.

By the way, if anyone knows where to get a large, 4x a day pill box (I mean large, like it has to hold many, horse pills) please let me know. I bought one off Amazon thinking it was as big as the one we have (which is falling apart to the point we're taping it closed), but it barely holds all his enzymes.

January 11, 2018

First Day Home

We've been trying to do breathing treatments every 2-3 hours. Azer can only tolerate 2 minutes at a time on his chest. CPT is causing shooting pains going to his shoulders. Today, he says that it is getting harder to breathe that yesterday, and feels like his lungs are getting worse. His lungs definitely sound much noisier than yesterday, and he is producing more. I really feel like the largish spot on his X-ray may have been pneumonia instead of just simply mucus plugging.

After sending a semi-panic stricken email to the CF clinic, he will be starting steroids for the next week. Hopefully by the end of the course of steroids, the antibiotics will really start to kick in! 

January 10, 2018

Discharged

Azer had a very hard time doing a PFT test. He was refluxing and nearly vomited several times. Thankfully, the technician was very patient with him and let Azer blow when he was ready. He is, not surprisingly, about 10% lower than normal.

They switched him to a different antibiotic so that he can go back on the erythromycin. He was practically begging for it earlier.

His cough sounds pretty wet, and his x-ray showed some mucus plugging, which is probably why CPT hurts so much right now. Hopefully it will clear up soon, but we are very happy to be discharged!!



Day 3

Yesterday evening, Azer's IV really started to bother him. It flushed fine, but just running fluids through was causing pain. The doctor said as long as he drinks plenty of fluids, they could take it out and not replace it.

The most painful part of the removal is the tape. The nurse was very patient and let Azer remove it slowly himself a little at a time.



He hadn't drank much all day, so a resident doctor came in and essentially threatened Azer that if he didn't drink enough Gatorade, he wouldn't be discharged. It worked, and Azer managed to drink and hold it down.



He's had reflux issues since we've been here. It's difficult to keep track of what medications he's taking when they're given on a different schedule than we do at home. This morning I realized he hasn't been taking Erythromycin, which he takes for GI motility. Apparently, it reacts badly with the antibiotic he's taking right now. There is another antibiotic he could take, but he has experienced chronic vomiting with it. So, they are trying to figure out what antibiotic would work for the bacteria he cultures without causing an interaction with his other medications.

CPT has been painful for him, especially the upper loves, and we've only been able to do a few minutes at a time. He's been trying out a different device called PEP which is different than the Aerobika in that instead of using positive and negative airway vibrations, it only uses positive pressure to open the airways. It has been helping bring up a lot of mucus. Unfortunately, last night, his mucus had some bloody streaking in it, so we are keeping an eye on that.

The doctor ordered an X-ray to see how his lungs look. She said if we did a PFT, it would be kind of pointless because we know it would be abnormal between his refluxing and congestion. After she looks at the X-ray and figures out his medications, we might be able to be discharged today!


January 9, 2018

Admission Day 2

Yesterday, they were able to start Azer's IV easily. His electrolyte panel turned out to be normal when he was admitted, despite how little he has been drinking. He has had hydration fluids running all night long. He was able to eat some dinner before his night feed was started. They also started an IV antibiotic to help his lungs out.

Azer's night was rough. Around 7pm, his fever and headache came back. His head hurt so bad it was difficult for him to cough all the mucus out during his breathing treatment. They had to get in another breathing treatment for the day, which unfortunately ended up being around 1:30am. His fever spiked again around 4am, and had to stop his night feed just a little early due to nausea. He has been holding down his formula despite having reflux this morning.



The doctor is happy with how Azer presents this morning. The decision of when to go home has been left up to us. The doctor agreed it can be scary to deal with these fever spikes at home while worrying whether he's hydrated enough or not. We have opted to stay a few days to make sure he's getting better before going home on oral antibiotics.


Watching Dude Perfect to pass the time

January 8, 2018

Admission


This morning, I emailed the CF clinic out of concern. Azer still had a fever, and it wasn't going down with ibuprofen or Tylenol. His headache was very painful, and he was also experiencing vomiting and diarrhea. He was having a hard time keeping hydrated. Our CF doctor recommended admitting him.

A few hours later, around the time when the bed was ready, his fever finally broke and he was feeling a lot better. He still wasn't drinking much, but was holding down his formula and his headache was gone. I asked the CF clinic if it was still necessary to admit him. The CF doctor still wanted him admitted, especially since he's had the flu back to back now. His cough is also sounding pretty wet.

They are starting IV fluids to get him well hydrayed. Hopefully this will be a quick hospital stay!

January 7, 2018

Influenza Type B

Yesterday, Azer started to feel exhausted and his cough started to turn wet again. Today, we can barely keep his fever down and he says he feels worse than two weeks ago when he first got the flu. So, we had him tested... again. He now has Type B Influenza. The doctor said there are a few different strains of each type, so even though he has now had Type A and Type B, we still have to remain cautious. He is starting Tamiflu... again, but will not be going to school for at least a few days. He is very congested as well, so we are again working hard on keeping his lungs clear.