July 17, 2018

The Plan So Far

So Azer and I spoke with the attending pulmonologist. He says his lungs still feel full and is still having more of a productive cough than he expects to have at this point. She told him that his PFTs are still going up, and he sounded better while performing the test, and isn't bringing up as much mucus as before.



 She said that this infection might just be harder to fight but that it doesn't necessarily mean that the next time it will be this hard. We are used to finishing the course of IV antibiotics at home. She said the IV antibiotics given in the hospital are given more frequent and are stronger, and she'd rather him finish a full 14 days of aggressive therapy. She said if he still feels bad after Monday, they will regroup and try something else. If he feels better by then, we can go home with oral antibiotics.



The skin peeling has slowed down, but in some areas, it has continued. It is annoying him, but otherwise it isn't harmful.



Overall, he just wants to go back to having a normal summer, spending time with his friends and go swimming.

Sleep Study

A few months ago, Azer started using a Fitbit. Since he can track his sleep with it, he was noticing that he is far more restless when he sleeps than he should be. He also has been noticing that he will have periods of sleeping with his eyes open during the day. Since sleep apnea can happen as CF progresses, we decided to try to schedule a sleep study while he was inpatient.

They were able to schedule it for Sunday night. They came in, attached sensors and electrodes to his head, nose, arms and legs. The results came back and he does not have sleep apnea, but he sleeps so restless, he does not obtain REM sleep. I haven't heard if there is any help for this, but at least we know he is breathing well while he sleeps.

July 15, 2018

Changes Needed

So, it's nearly been 7 days since Azer's been on three IV antibiotics. Azer was feeling discouraged last night. Even though his lung function test Thursday was up, he feels like his lungs haven't gotten better and says they feel full and irritated. He was worried that if he went home, his lungs would just nose dive.

 He did a great job this morning explaining to his Pulmonologist how he felt. Azer said that using the IPV has done a great job clearing the mucus out, and the bronciodilator is opening his lungs, but feels that the antibiotics he's on are not killing whatever is making his lungs sick. The doctor agreed with Azer and told him that the PFT is just one indicator in how his lungs are doing, and they really take into consideration how he feels.

So, the staff will present the data from this week to Azer's main pulmonologist and she may change his antibiotics, or he may have a bronchoscopy this week. Either way, it's not looking like he will go home quite as soon as we had hoped.

July 14, 2018

Worsening skin





Dermatology was contacted again. They said that this was not unusual, but they can't explain why it started nor when it will stop or how far it will progress. They are not concerned because it is just affecting the top layers of skin. They suggested that he puts vaseline on and wear socks and gloves. (I will not be offended if everyone replies to this post with "it puts the lotion on its skin" memes)

July 13, 2018

Dressing change and Frisco Rough Riders

It would be normal to think inserting a long term IV would be painful, but dressing changes are very painful. Azer had to have his dressing changed a few days earlier than normal as it was coming off. This can be dangerous as it can lead to bacteria entering the blood stream right near the heart. 

Child life surprised Azer today. A few players from the Frisco Rough Riders were here, and came to visit him. He was so excited! They talked baseball, about him being a southpaw, and watching him play an MLB game on the Xbox. Azer's eyes lit up when one of the players mentioned that he met Barcelona Colin (a Texas Rangers pitcher). It was a wonderful experience for him!





July 12, 2018

Reason for Skin Peeling

So, the dermatology team came in to look at his skin.


They determined that this was not a reaction to the antibiotics, but was instead a skin peeling condition called keratolysis exfoliativa which can happen to people with sweaty hands, but is apparently very common with CF. Basically they said now that he has this condition, he will always have it, but they can prescribe a lotion that can help the dead skin come off faster.

They changed one of his antibiotics, Gentamicin to Tobramycin. They will continue to watch out for any hearing loss from the medication.

July 11, 2018

IPV and Possible Antibiotic Reaction





Last night, Azer noticed a spot on finger where the skin was peeling. Today, more finger had peeling skin. This afternoon, it started to appear on his toes. The doctors say it could be from the antibiotics. They will watch it to see if it gets worse.

Today, Azer tried IPV for the first time. The RT said he had great technique. It takes quite a bit of effort to use it, but Azer did great, and said his lungs felt very open after using it.

July 10, 2018

Hospitals are boring




Azer has to be hooked up to the IVs at all times, unless he wants to shower. He had a friend come visit for a while which helped to ease the bordeum. 

We were rebels and escaped outside when the rain died down. 




He says if anyone wants to text him or FB message him, that would be cool. 

July 9, 2018

Got the PICC line!

They were able to put in a PICC line without sedation. They also did a chest X-ray.




He will have three different antibiotics running through it. Several can affect the kidneys so they have to test the levels periodically. Another medication is in the same family as tobramycin and could affect his hearing. They have to use these medications as he has vomiting with an antibiotic he used to be fine with. Another possible medication that could have worked could cause an adverse side effect with one of his GI motility medications.

He will also be starting an inhaled steroid while he's here.

So, here's to hoping that they help his lungs feel better soon!

CF Clinic and Admission

Today was a follow up visit for after our vacation. We knew coming in that we might be facing a hospitalization.

Azer gained weight from last visit. He's been very hungry from being on Periactin and Prednisone at the same time.

Azer did three treatments yesterday and ran for 30 minutes today to try to get his lungs as clear as possible for PFT's today.



His first try ended up 20 points below his normal. After 8 tries, he was still lower than last week. His culture from last week only came back with MSSA, he's been on oral antibiotics for two weeks, and steroids for over a week, and it feels like nothing is working. The doctor listened to him, and said his lungs sounded clear, oddly enough. His cough sounds productive and irritated. He said when he ran this morning, he had lung pain. He says his lungs feel full, like the mucus is stuck.

The doctor said we can start IV antibiotics and see if he feels better after a few days. If not, he will get a bronchoscopy, which would help them get a culture from deeper in his lungs. She said about 70% of the time, a bronchoscopy causes a fever. The stain for mycrobacterium came back negative, but the doctor said sometimes the stain can be negative, but the culture could come back positive. If it is mycrobacterium, it could be a hospitalization up to three months. 😶 It is frustrating to not know what his causing his lung function to decline when he is doing what he can to improve.




They will try to put in a PICC line in today without sedation. Previously, he has had light sedation. The technician came in to check his veins and put numbing medicine on his arms.


The antibiotics he will be taking are Ceftazidime, Nafcillin, and Gentamicin. He started inhaling Pulmicort with his breathing treatments.

June 29, 2018

Update on Sick Visit

So today was mostly just a follow up for PFT's to see how his lungs are since he's been on steroids and antibiotics for a few days. His first few blows were exactly the same pre-albuterol last visit. They tried a different blowing technique which apparently works well for him when he's sick, and he blew just a bit lower than his post-albuterol test on Wednesday.



So his numbers look good, but numbers look good on paper, but don't always reflect the patient. Azer told the doctor that his lungs still do not feel good. He said they feel the same as they did before steroids. Usually, steroids make him feel a lot better quickly. The doctor did note that he's only been on this treatment for a few days, and it may take a little longer to start feeling the effects. She told him to have fun on our family vacation, and that if when he gets back, even if his PFT's are up to nearly normal, if he still feels like his lungs are sick, she can admit him to get his lungs back to normal before school starts. The doctor added that she's adding a test to his lung culture to test for non-TB mycrobacterium (we really hope it's not this, as it can take over 6 weeks for the culture to come back and the treatment is very intense and long. This bacteria grows very slowly, and treatment for it can take up to a year).


CF can be overwhelming and can really take over your day if you're not careful. Azer definitely felt those pressures when he was away for a week. Even at home, with not much else going on, it can be tough. I thought this picture really demonstrated him trying to fit everything in so he could go be a teenager. He was doing his inhaled Tobi podhaler and giving himself his growth hormone shot at the same time.

June 27, 2018

Sick Visit / CF Clinic

The Friday before Azer left with his youth group on a mission trip, he woke up that morning, and told me his tongue hurt and felt scratched up.

I was able to send pictures to the clinic that morning, and a few hours later they confirmed it was oral thrush, and sent the anti-fungal medication to the pharmacy.

Azer had a fantastic time and did as well as he could to keep up with his treatments during his mission trip. He told me mid-week that the schedule was not easy for a CF person to keep up with. He missed quite a few vest treatments due to how late at night they got in. By Thursday, he told me his lungs felt pretty sick, but he wanted to push through and finish the week. He didn't get enough time to do his Tobi or oral thrush medicine, but did most everything else and ate really well. He took the week off from his steroid nasal rinses, and ended up losing his sense of smell this week. He's hoping it comes back quickly now that he's back on it again.

When he got back home, Azer said his lungs felt pretty sick. When I listened to him, he didn't sound too bad, but had a few wheezes. Of course, perfect timing, his Vest ended up making horrible noises and needed to be replaced the day after he got home. Luckily, the Vest company was able to overnight us a new replacement machine!

Clinic: Azer had to get his annual Oral Glucose Tolerance Test today, so he was fasting since midnight. His height was the same as last time (he missed a week of growth hormones), and he lost about 3 pounds (not very concerning given how active he was last week. He was getting about 30,000 steps a day!) They drew his blood during vitals to test his fasting glucose level, which was normal.


Azer has been eating very well with the Periactin and as such has had changes to his stool and hasn't been absorbing his food as well as he could, so the dietitian came in to go over enzyme dosaging.

His PFT's were considerably lower than his baseline and lower than last month. The doctor listened to him and wanted him to do an albuterol treatment and then re-do PFT's and see if there was any difference. His FEV1 did go up about 7%. Azer told the doctor his cough never completely went away after the virus last month, but that his lungs felt much worse on his mission trip, but since he's gotten back home, they've gotten a little better.

The best course of action would be an admission to the hospital at this time, but we have already planned to be on vacation next week. The doctor felt like a quick admission for a PICC line would be pointless if he had to go back in to the hospital when we got back home. So, we are doing the next best thing: Hospital at Home: breathing treatments every 4 hours, antibiotics and steroids. The doctor warned Azer he needs to "get his butt in gear" (her actual words). We will redo PFT's before we leave, and if he hasn't improved when we get back, he will probably be spending a few weeks in the hospital on IV's.

The doctor told Azer to not feel like he didn't do a good job over his week on his own. She said the work he was doing combined with the weather and the environment he was in could be contributing factors to how his lungs are right now. They are running another virus panel, as well as a lung culture to see if he's growing anything other than MSSA. I will update as I find out the results.

June 5, 2018

CF Clinic

About 2 weeks ago, Azer suddenly started to sound congested. Oddly enough, it started during his week off of Periactin. The week off of Periactin, his seasonal (year round lol) allergies hit him hard. During Memorial Day Weekend, he began to sound so congested, we all wondered if it was time for an admission. He also began to cough up brown mucus. The on-call doctor prescribed some oral antibiotics to see if that would help. Since then, his cough has improved slightly, but he says his lungs have stayed full.

Not surprisingly, his PFT's were down today. The Pulmonologist said that his small airways were the same as they were last time, so whatever is going on with him is unlikely to be ABPA (our worst fear, as it would put a damper on summer plans). She said he should finish his course of antibiotics, and start Tobi Podhaler while we wait for his culture to come through. Even if it isn't Pseudomonas, the Tobi should also help out with the Staph he normally cultures. She heard some mild wheezing in his lungs. They also took a nose swab to see if he's culturing a virus.

He put on some weight, so the Liquigen is helping him out. His appetite is very good when he's on the Periactin, and he has been enjoying a healthy appetite, although it has come and gone since he's been sick. He is also growing!

Azer is going on a week-long trip with his youth group. This will be the first time he is fully responsible for all of his treatments and medications. The doctor went over things that were the most important (such as staying hydrated and ways to add sodium) and she went over a few ways to make his treatments easier, such has medications he doesn't have to take during that week.

Azer is still taking Dulera and Qvar. Well, apparently Qvar has stopped making the inhaler type, and is now coming out as an inhaled powder. Since it's already been ordered, and the doctor feels like he's old enough to be able to inhale it properly, he can try it out and see if it works for him. If not, we have to go back to Flovent.

- Update: Azer's viral panel came back positive with metapneumovirus. His lung culture only came back with MSSA. He is doing great with the new Qvar, and doesn't really notice a difference in how it feels, other than this Qvar is not taken with a spacer.

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


May 3, 2018

Endocrinology

It looks like the Periactin is working! Azer gained about 4 lbs in only a few weeks just from an increased appetite. From his last Endo appointment, he grew about 1/3 of an inch. He told the doctor that he's had hip and knee pain lately, and he feels like he keeps banging his ankles into things frequently. These are signs that he is growing. Another sign we may see soon is he will need bigger shoes.

Azer told the doctor that the pharmacy recently changed needle sizes, and the longer needle is causing the injections to be a little more painful. She said the needles aren't a prescription, so the pharmacy themselves may be able to change this. He also mentioned that the most painful injection site is in his stomach. She told Azer he doesn't have to use the stomach if he doesn't want to. He does need to change injection sites each time, but scarring from injections happens more often with insulin, not so much with growth hormones.





The physical therapist came and looked at him for his scoliosis, since there is a risk of it worsening now that he's growing. She noticed that his left scapula area is higher than his left, and we were all concerned that his scoliosis got worse, so the doctor sent us to get an X-ray.



The results from the X-ray look good. His scoliosis hasn't worsened, which means that the changes in his posture are muscular from growing, and possibly from pitching. He has been working on pitching quite a bit lately. So, it looks like over the summer, he will be working with the physical therapist every other week again to correct his form. If we don't correct it now, it can eventually be permanent.


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.