December 12, 2018

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!

November 19, 2018

November 13, 2018

Sort of Sick

Azer has still been noticing every time he takes a week off of Periactin, he starts feeling worse, his lungs start to sound more congested, and his allergies are pretty bad. He contacted the clinic, and they pushed the referral to an allergy clinic. It has been scheduled. Last week, it happened again, and he sounded pretty bad. The clinic prescribed 5 days of steroids, but the only thing it helped was his appetite. Azer is hoping that the allergy clinic agrees that the allergy shot that the Pulmonologist suggested will work for him.

October 6, 2018

15 Years

It seems so long ago, and yet like yesterday, 15 years ago. I was handed a medically fragile baby, overwhelmed with what it would take to keep him alive. I had so much responsibility so early on. But, oh I did it; everything those doctors told me to, and beyond.

I held him down when he was only a few months old to force that nebulizer mask on his face. I did hours upon hours of CPT so he could breath.

Eventually, he learned to hold the cups himself, learned to start his Vest on his own, learned to fill his own nebulizer cups.

At such a young age, he knew so many medical terms, and knew what all his medications were for and what they did for his body.

He has learned so much over these past 15 years, but over this past year, he has taken on so much of his own care, it boggles my mind.

All that responsibility that was given to me 15 years ago is now his. Most people I know struggle to get their teenagers to brush their teeth before they rush out of the house to High School. I also struggle to get him to brush his teeth, to prevent a mouth infection caused by multiple steroids. He wakes up extra early to go run to clear his lungs, keeps track of medications that need to be refilled, sterilizes his nebulizer cups, fills his pill box, works hard on his breathing treatments, communicates on his own with the doctors about any changes in his health... This is one tough, responsible teenager y'all.

Happy 15th Birthday Azer! I am so proud of you, and everything you have become.

September 27, 2018

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

September 22, 2018

Sick Clinic Visit

Last week, Azer had a sore throat that wasn't really improving. Tuesday, at school, he texted me that his shirt was soaked with sweat and he was feeling really cold. We went to CVS to test for Strep throat, but seeing that his throat looked fine, she concluded that it might have been some other infection and prescribed antibiotics.

By Thursday, his cough was worsening, so Azer emailed the clinic about what to do. He was able to be seen that morning. His PFT's weren't as low as he was expecting them to be. The doctor said his lungs sounded fairly clear, although airflow in the lower lobes is diminished. She changed his antibiotics to one that will provide better coverage for the bacteria he usually cultures. She told him that if his lungs don't feel better over the weekend, to let them know, and they will put him on oral steroids.

If he's still sick by next week, which is when we have our usual clinic visit, we will skip his annual OGTT, as when you're sick, your blood sugars can be elevated.

It's been an unusually wet ending for summer here, and unfortunately because of all the rain we've had, it's been like a second spring as far as allergies go. His cough has been kind of on the wet side for over a month. This is despite him being on a prescribed antihistamine, OTC allergy pills, Benadryl at night time, daily nasal rinses, and two inhaled steroids. The doctor asked if we had ever seen an allergist, and that our main pulmonologist was considering allergy shots, which I'm honestly surprised has never been suggested before. We will bring this up at his clinic visit next week.

July 30, 2018

Admission Follow Up

Azer blew a few points higher on his PFT than he did the day he got discharged. He still felt like it was harder that it should have been to get there. The doctor felt like he could still get back to his base line if the weather and his allergies were optimal. The great news is that he feels like his lungs are very clear, and he has been running in the mornings and has not been out of breath. So we will try to make the most of the rest of summer before high school starts!!

Azer also felt that dermatology was wrong about his skin peeling. He said that as soon as they had switching him from Gentamicin to Tobramycin, the peeling stopped progressing. The doctor said that it was a possibility it was from the antibiotic, but that the reaction he had was just undocumented.

He ate very well while in the hospital. His appetite hasn't been great since getting home. He has had an upset stomach and diarrhea for the past week. The good news (sort of) is that I also ended up with these symptoms, so it may be a virus.

While the IPV did great for him in the hospital, and we are persuing getting one for home use, he felt like getting back home and using his vest helping clear out some mucus that wasn't moving otherwise. Of course, him getting back to running has helped him feel 100% back to normal!

July 23, 2018

PICC Line Out!

This guy is so brave, y'all...

Discharge and the Plan

Azer using a resistance band the wrong way 😆

Yesterday, Azer ran around outside for a while. He said he felt like he shouldn't have gotten so out of breath so fast.

He had a repeat X-ray last night, which looked unchanged from two weeks ago.

Today, it was refreshing to see our main Pulmonologist. Azer told her how he felt, I told her my concerns. We have three options: 1. Stay in another week on the same antibiotics, 2. Go home with IV antibiotics, 3. Take out PICC line and see if his lung function goes down over the week.

Going home today sounds like what we all want regardless of what his PFTs look like today. Azer and I both wonder if there is something growing deep in his lungs that they aren't picking up on the culture. So, being off antibiotics for a week or so will give time to see if the bacteria in his lungs will come back. If in a week's time, his lung function has dropped, we know there is something there. If it has remained stable or gotten better, it is likely the air quality and allergens that are giving him problems.

July 20, 2018

Going Home Monday

Azer's PFT results were the same as Monday, so not as bad as yesterday. The doctor is comfortable with sending him home Monday on maintenance antibiotics. The fear? It took him 8 hard tries to reach his goal today. It shouldn't be that hard for him to get there after being on harsh IV antibiotics for two full weeks. He's been running, doing stairs and PT. His cough sounds clear, but according to the test, he's not breathing in as deep as he's been able to before.

Another Dressing Change

So, the doctor said if his lung function goes down over the next few weeks, that we will do a bronchoscopy to find out what else is lingering in there. At least we will get a few weeks of summer swimming first!!

July 19, 2018

A Little Discouraged

Azer says his lungs feel nearly 100% better. He did a PFT today, and since he's been here, each one has been getting better. Today, it was 5 points down. He tried 6 times to get it higher. The doctor looked at the results and said compared to previous times, the numbers are accurate.

She said we can retry the PFT tomorrow to see if it was a fluke. If they aren't better, they will do a bronchoscopy. She said often, a bronchoscopy can make a person's lungs worse for about two weeks, (which means an even longer hospital stay) but it might give them a better culture of what else might be in his lungs. He might be able to do home IV's at that point.

Azer isn't used to long hospitalizations, but here are some fun things we've done.

Playing foosball

Kendra Scott Jewelry

Assembled and painted a wooden dinosaur

Playing games with the Red 🎈 Network

Pretty much the entire time we've been here, Azer has been requesting the most silly, unusual songs on the Red 🎈 Network. Most of the songs are by Parry Grip. If you've never heard of them before, Azer says you should probably take a listen. You can thank him later.

He requested "It's Raining Tacos" so many times, I'm fairly certain everyone here is very familiar with it now.

We ran this IV pump's battery down so low, it died

Playing games

Snapchat filters lol

Physical Therapy Inpatient

Our out patient PT has been trying since last week to get Azer some physical therapy while he's here. He would definitely be able to get more mucus out if he could get up and run around. So, Tuesday Azer was evaluated by an inpatient PT, and we might be able to get to the gym some time this week.

Azer did a few minutes of exercises yesterday morning. But, in the evening, one incredible general practitioner doctor took it upon himself to get Azer some exercise. He took Azer and I to the stairs, and had Azer do push ups, jumping jacks, and then together, we all did about 10 minutes of running up the stairs. It was so much fun! Azer said his lungs felt totally normal after that. Exercise!!!!

July 18, 2018

A Sick Doctor

I think I pissed off our attending pulmonologist. She was in contact with a flu positive child yesterday.  She tried to come in our room to listen to Azer. She was a bit sweaty and obviously wasn't feeling well. I said, do you have to listen to him? He's already had the flu twice this year, and been admitted for it. Granted she was gowned and masked, but come on. He's already fighting off something that they can't identify. He doesn't need something else!!

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.