Great Strides 2016

You may know, Azer has Cystic Fibrosis. This is a genetic disease. It affects how the body makes mucus. The mucus in Azer’s body is very thick, and causes chronic and frequent lung infections. It also causes him to have a hard time digesting fat and protein. He eats high calorie foods to try to compensate for this, but he is unable to eat enough to gain weight, hence the need for the feeding tube. He receives more than 1,200 calories through it a day.

Azer takes about 20 medications a day, and does about 2 hours of breathing treatments a day to keep his lungs clear. We also exercise and jog. Exercise is one of the best ways to keep his lungs healthy, we’ve found.

The life expectancy used to be only in the teens. But now, the average life expectancy is 37 years. The Cystic Fibrosis Foundation works hard creating new medications, and they are working to find a potential cure, too.

We are walking at the Dallas Great Strides 5k Walk, Saturday May 21st. Check in is at 8:00am being held at the Vitruvian Park in the heart of Addison.  

We are a team this year, called Joggers for Azer. If you are interested in walking with us this year, you can sign up here to join our team

http://fightcf.cff.org/goto/lilhyperfingers

lilhyperfingers@gmail.com

If you can’t walk on Saturday, that’s ok too!! Go to the website, and if you could donate a dollar, we would love it! The funds go directly to the CF Foundation, which goes to research for Cystic Fibrosis.

Dallas Great Strides

5k Walk

Saturday, May 21, 2016

Check in: 8am

Walk: 9am

Location: Vitruvian Park,
Addison, TX

Fun, Food and Much More!

Visit http://fightcf.cff.org/goto/lilhyperfingers

Cystic Fibrosis Can Be A Full Time Job

3am, hear pump beeping. Check clock. If it's earlier than 3am, go check to see if he rolled over. If 3am, it's probably ok. Go back to sleep. He's coughing now. Please don't let him throw up or reflux everywhere.

5:15, alarm goes off, Azer wakes up to go to the bathroom.
Get up, Run with him for 30 mins, or run/walk for an hour. Encourage him to try to run harder/faster.

6:30 Azer starts vest and nebulizer cups, and morning G-tube feed.

Getting Some Homework In During Morning Treatment

7:20 am As Azer is brushing his teeth, I fill the pill box. Make mental notes of each bottle and what is getting low, and need to order soon.

Take kids to school. Remind Azer to drink liquids!

Start vacuuming house. Vacuum everyday in case there is dirt, or dust that could aggravate his asthmatic component
Put mask, nose attachment, and spacer in rubbing alcohol, swap nebulizer cups in vinegar.
Rinse nebulizer cups and put in sterilizer. Make note of level of distilled water.
Take Aerobika apart, clean and put in sterilizer
Take things out of rubbing alcohol and air dry
To help me remember to take Tobi, albuterol, and spacer with me to school later, put in ziplock bag

Start cleaning kitchen. Let cleaner sit in sink drains in case there is Pseudomonas in there
Let cleaner sit on kitchen floor spot of formula dripped
sterilize dining table in case there is staph coughed on it, never know it might make him sick.
Clean their bathroom. Sterilize his toothbrush, let cleaner sit in drain, clean his toilet, notice there is remnant of fatty stool. he didn't eat much for dinner, I don't know why he's not absorbing like he should.
Pass by his room. Grab the empty formula bag and empty formula boxes. Take to kitchen to recycle, clean his G-tube extension with hot water.

10;45am Time to go to the school. Grab ziplock bag. Make sure Tobi is in there. Nurse calls him into the office. Albuterol, 10 Aerobika blows. I do a push on both sides of his chest, he sounds like crap. He does his Tobi and he goes to lunch.

I'm free till 3pm! Keep track of copay programs, insurance, what needs to be printed, and sent in.

One Week's Amount of Pills

3pm, drive to school, pick up kids. Home by 3:30. Start Aerobika. Formula time, bolus feed. Make mental note of when I need to order formula next. If the pill box has oral antibiotics, take the vitamin out or he might vomit. Ask how many times he's had a bowel movement, even though he obviously had a fatty one last night. He can get constipated in a day. Use judgement to decide if he needs Miralax or not.  Try to get him to clean  his room. (Way harder than anything else here... lol)

Bolus Feed

5pm, Make Scandishake in blender. Boost still hasn't gotten here. Why? Remember to check Live 2 Thrive program, and check status. Need more milk, using about a half a gallon a day.

6pm, Azer needs to eat dinner. Make sure dinner pills are out of the box. He eats dinner does the Scandishake as a bolus.

7:30, start evening nebulizers.

Cystic Fibrosis Weapons

About 8, start CPT. Husband has been helping me with this lately, as I have had sternum pain (from doing it so often? I don't know) Pushes on all sides of his chest while he huff coughs. Aerobika, Symbicort, Nasal Spray, Tobi. Make sure Azer brushes his teeth, or he might get oral thrush from steroids.

Make sure nebulizer cups are in vinegar.

Start night time feed. Make sure he takes his pills and they're not sitting on the night stand. Tape him up so we don't end up with two possibilities. Possibility #1, he rolls over on it, if no one hears it beep, he doesn't get the full dosage of the night time feed, and he desperately needs those calories. Possibility #2, it becomes disconnected somewhere and he may or may not wake up to stop the pump, and we end up with soaked clothes, soaked bed, floor, etc.  Make sure bed side fan is pointing at his face. Feel scared that he feels the need to have air blowing on his face, possible night time low oxygen. Hope we don't have to deal with oxygen soon.

Why do all of this? If one thing I did will make him a little healthier, help him live just a little longer, spend more time out of the hospital, have more assurance that he will outlive me, then it's worth it.

You can help make our day a little easier by helping fund CF research. You can join us at our Great Strides Walk, or help raise money by clicking here. Thank you :-)

http://fightcf.cff.org/goto/lilhyperfingers

No Pseudomonas!!

So, for the past two weeks, Azer has said he felt more congested. He also started coughing up brown to black chunks of mucus in the morning. So, Monday we went to get cultures, PFT's and an IGE level check just in case he had ABPA again. 

He gained 2 pounds in a month, but his FEV1 was down a little from last month. 

A few days ago, we got the test result back from the IGE and it is lower than was last September. We've been waiting expectantly to see if he is culturing something, but today it finally came back negative for Pseudomonas and for Aspergillus! Yay! 

Bone Density Scan

The Bone Density Scan doesn't even need it's own blog post, but based on our experience today, I felt like it did. 

So, at our last CF appointment, our GI doctor said he would need a bone age study to rule out growth problems and osteopenia. I didn't realize until today that he was actually having a bone density scan done instead. The orders to do the bone age study may be there, but I am not sure. 

Anyhow, the nurse/technician was nice enough. She was trying to explain to Azer what she was going to do and why. He was having a sort of teenager-ish time, and was kind of giving her the "I really don't care how and why, let's just get this over with" look. So, I mentioned to him that we're getting this done so we can maybe find out why you're not growing very fast. The technician hurriedly explained to me that this is not what this test is for, and that it was to find out how dense his bones are. From everything I've read before, I assumed the bone density tests would start when he was 18, so I mentioned that. Her responses felt very argumentative, so I just stopped talking. 

She first scanned what looked like his spine. She then had him sit up, and place his right arm on the table to scan it. When she was about to start scanning his right hand, she asked if he was right handed. I said that I was sorry, he is left handed in baseball, and most other things except for writing. I told her when he was about 4 years old (at that point I had no idea he could possibly be left handed) I taught him to write right handed. It wasn't until later when we began to play catch together that I realized he was left handed. By then, he was already pretty good at writing with his right hand, so it stuck. (I realized recently how left-dominate he is when we found out he is left eye dominate as well). She then told me that it can be very damaging for brain development to force someone to write with the less dominate hand. By now, I just decided to stop defending myself, and just looked around the room while the computer "measured" his bone density. 

It was a pretty quick appointment, so he was able to go back to school for the rest of the day. 

This evening, as I was going over today's visit in my head, I texted Azer. The screenshot sums it up lol. 

Vomiting

The antibiotic called Bactrim, that Azer has been on has been making him vomit, usually once a day. Last week, he vomited so hard, his nose started to bleed. Last night, we started the antibiotic Cipro. The Bactrim had to be given three times a day, the Cipro twice a day, so he ended up having to take them at the same time this morning. On our way to his baseball tournament, he threw up everything, formula, antacids, antibiotics... I knew this wasn't going to work.

So, I called the on-call doctor, and they are taking him off the Bactrim and Cipro, and putting him on Levaquin. The doctor said it treats all the of bacteria he is culturing including the Pseudomonas, which is awesome. I am glad he only needs to be on one oral antibiotic.

Azer did say that the inhaled Tobi is making him feel a little icky, like the beginning of a cold feeling. Hopefully that feeling won't last through the whole month.

So, basically, the Tobi Podhaler has these four capsules. He has to inhale 4 of them twice a day.

The little device in his hand pierces the capsule and then he has to inhale the powder (weird right) and hold it for 5 seconds. He says the urge to cough it out immediately makes it hard to hold his breath, but he manages.

He did great pitching today, and pitched into the 4th inning!

Pseudomonas

Well, the antibiotic Azer has been on has been causing some vomiting, but nothing too terrible. He initially said the Bactrim was helping his lungs, but a few days into it, he said his lungs felt tight. Unfortunately, the reason for this is that his culture came back, and it was positive for Pseudomonas. He hasn't cultured this since 2013.

Pseudomonas Aeruginosa (or PA), can be a very aggressive bacteria in CF. It can cause further lung damage and can be difficult to eradicate. PA is one of the reasons for boiling the nebulizer cups, as it can survive normal washing. They said he has 2 colonies of it right now. Apparently, we can think of the colonies of pseudomonas like little apartments of bacteria, each one living in a different complex. How they can figure that out from a sputum culture, I have no idea.

We will treat the Pseudo using the Tobi Podhaler again. This worked out very well last time. The normal treatment for pseudomonas is inhaled Tobramycin, or Tobi, which is an antibiotic which is inhaled. This is inhaled though the nebulizer and usually takes about 45 minutes. With the Tobi Podhaler, which only takes about 6 minutes to inhale the powdered antibiotic.

On top of the Tobi, he is going to have to do Cipro in addition to finishing his course of Bactrim. The PA has to be treated aggressively. I hope we can control or eradicate it as quickly as we did last time!

Clinic

Had another fairly good CF clinic appointment. He is now 100 lbs! Triple digits! The formula is working great, and he is absorbing most of it. He isn't eating a whole lot, but they aren't really concerned with that right now since our goal at the moment is to get him growing taller. Which isn't really happening. In a few months, he's only grown a tiny amount. We have not heard back from the endocrinologist since the last appointment, so our GI doctor is going to put in another referral. He is going to need an X-ray for bone age to rule out any osteopenia. While their at it, they will do an X-ray to make sure his scoliosis hasn't progressed. We are facing a real possibility of growth hormones, which can put him at an increased risk of CF related diabetes, so the endocrinologist will need to evaluate the risk versus the benefit of getting him to grow. The GI doctor said with the X-rays and all these months of aggressively gaining weight without growing will provide evidence to the endocrinologist that we have tried to get his body to grow on it's own. It is not clear why children with CF sometimes do not grow as they should, even with above optimum nutrition

Azer's Mic-key button has been bothering him a great deal lately. Apparently within a few months, he outgrew his last size, so an ostomy nurse came and put in a larger size. He said it felt so much better after. 

The erythromycin has been working very well to control his feelings of bloating and fullness, although it doesn't seem to be actually making the stomach empty faster. The CF doctor wants us to discontinue azithromycin, since they both are in the same class of drug, and he has not cultured pseudomonas since 2013. She mentioned that if he does culture Pseudo again, we can put him back on azithromycin at that time. Some studies have shown that azithromycin can prevent pseudomonas from growing a biofilm, which is like a shield that it can grow to prevent antibiotics from killing it.

During spring break, our GI doctor would like us to back the bethanechol to twice a day instead of with every meal. 

We all had a virus come through the household about a week ago, and as can often happen with Azer, the virus left, but the congestion in his lungs can stay and get worse in a short time. His cough was concerning to her, so she wants to put him on an oral antibiotic for a few weeks to help his lungs clear the junk out. His Fev1 was 86%, which both the doctor and I agreed wasn't bad, but we know he can blow higher.

Our CF clinic is beginning to test CF patients 12 years and older for depression and anxiety, which will be confidential between him and the doctors. It is good that they have started this early, so if he has issues later in life, he will hopefully feel confident enough to share any of those feelings with them before they become issues. 

Reglan

So, Azer has been on Reglan for almost a month now. It has worked beautifully. His constant constipation has gone away. For nearly the first time in his life, he has been able to eat what he wants, when he wants. He has no bloating, reflux, or feelings of fullness. All symptoms of gastroparesis have gone.

At last clinic, we decided since it was working so well, they would fax in a month's long prescription to the pharmacy.

Unknown to me all this time, it is a black box drug. In other words, potential bad side effects. The nurse called me to tell me about a potential side effect called Tardive Dyskinesia. This is basically a condition that could permanently affect the nervous system, and can cause involuntary movements of voluntary muscles. I read on a CF website about someone's daughter who had severe facial twitches after being on Reglan. This terrifies me and sorrows me at the same time. I have stopped the Reglan immediately. The possibility of him developing TD is around 15-20%. I stopped Reglan last night, and his bloating, reflux, feeling of fullness came back immediately, as did diarrhea for some reason.

I called the pharmacy to cancel the prescription. I was able to talk to our GI doctor, and we are going to try another medication over the weekend. It is an antibiotic, but can treat gastroparesis as well. We have tried it in the past, and it resulted in him constantly vomiting. I really hope this does not happen this time.

This whole thing has been very upsetting to me. I mean, I was so happy for him that he was able to eat normally, and feel what enjoying food feels like! But at the same time, absolutely terrified that he took this medication that could have had disastrous side effects. I thank God that this did not happen, but I feel like I should have been warned about the potential side effects BEFORE he took the medication for the first time.

Clinic Day

About two weeks ago, Azer was stilling having reflux issues, so the GI doctor decided to switch him from bethanechol to reglan which can be used to treat gastroparesis. While it hasn't made the reflux/vomiting completely go away, it has helped significantly. Currently, he has only been having food come up if he is running or playing aggressively. The GI doctor said there is little they can do about that, because most of what is in his stomach is usually just formula, and it is easier for liquid to come up than solid food.

So, yesterday, both the GI and Pulmonary doctors were very pleased. He is now 95 lbs, which puts him around 60th percentile for weight. The ideal weight for a CF person is 50%, so we are doing fantastic. On the growth chart, it looks like a straight line up.  His height is finally starting to creep higher above the 10th percentile. It is moving slowly in the right direction. When the pulmonary doctor came in, she said that the GI doctor was grinning out in the hallway. His weight is high enough now to have to move up to a higher strength of enzymes and he hasn't seemed to have as much constipation since we started the Reglan.

His Fev1 was only 83%. However, the doctor said they now have a new computer system they use for PFT's. She showed us based on the volume of air he blew out, that his total volume is increased compared to last time. She believes his Fev1 was actually in the 90's because of this. I was grateful for her taking the time to explain and compare the test. She listened to his lungs and she said he had great airflow and no wheezing at all. She mentioned that the pushing technique I do after CPT helps clear the mucus out of the smaller airways, so on we must continue!

Results from the Endoscopy

So, the results from the endoscopy biopsies are... he has no sensitivity to lactose or sucrose, but he has superficial gastric inflammation. They are not sure what is causing this, and so far the only suggestion is to increase his Nexium to twice a day. He has vomited once since we have done this, and seems to be still having reflux, but mostly in the morning. So, hopefully the double dose of Nexium will take effect soon! Despite vomiting, he is continuing to steadily gain weight, and he is over 90 lbs now. I hope his height will catch up with his weight, because shopping for all new clothes has been challenging!

Endoscopy

Our GI doctor wanted to do an endoscopy on Azer in addition to the gastric emptying he had a few weeks ago. This was to see if there are any visual reasons why he is having so many issues with vomiting and reflux. 

He was very calm and relaxed about it. When the anesthesiologist came in, they asked Azer if he wanted any Versed. Azer said he was fine without it, so they took him to the surgical room and started him out on gas (it was supposed to smell like bubble gum, but he said it didn't smell that great lol)

The procedure only lasted about 30 minutes. The GI doctor told me that he looked like he had irritation to his stomach. She took small biopsies of his stomach and small intestines to see if there were any issues she couldn't see with just the camera. She also put a new G-button in, as the old one had lost a lot of water, and also put a longer button in, as his old size was fitting kind of tight. 

He's had a cough for about a week, but nothing serious enough to hold back the procedure. When he woke up, he kind of gave the nurses a bit of a scare, as he couldn't stop coughing. They had to do several treatments of a Xopenex. It seemed to help him breathe better. 

When they were confident that he could breathe fine, we were allowed to leave! He was kind of woozy the rest of the evening, but he was able to hold down a little food, and tolerated his night feed just fine. Oh, and also he weighed in at 89.9 lbs yesterday! Lets just hope he starts growing on his own now! 

Gastric Emptying Study

We had the Gastric Emptying study today. We won't know the results until radiology takes a look at the pictures.

He had to be fasting for 4 hours before the procedure, so he ended up having to sit through lunch at school.

The ceiling in the room was pretty cool to look at.

We were told to bring something he would eat fast, such as pudding, yogurt or applesauce. He chose applesauce. (I find this funny because if you're a CF mom, we have fed our kids more applesauce than anybody knows! You'd think he'd be sick of it lol) They mixed some radioactive liquid with it, which is thankfully tasteless. He had five minutes to eat it.

He then had to lie still for an hour while they scanned his stomach. We got to watch the Lego movie... again!

He had to cough several times during the scan, but they said it didn't affect it that much since it's quick motion.

I'm hoping they find something we can fix!

CF Clinic and Annual Blood Work

So, today was one of those longer clinic days. He had to be fasting starting at midnight for his glucose test today. They drew blood for a number of tests when we first got there. He then had to drink a few cups of glucose solution and wait two hours.

He weighed in at 86.8 lbs today! He gained almost 8 lbs in just two months. It appears the higher calorie formula is working! On the down side, he just didn't grow much at all, and hasn't, even since increasing his caloric intake. We will see an Endocrinologist in a few months who may have some ideas as to how to handle this issue. We hope his body will kick it in gear and start growing now that it has some extra nutrition.

About two weeks ago, we had a week of vomiting only in the morning. After switching his morning medications to afternoon, it seems to have cleared up, but he is still having reflux frequently. Because of this, the GI doctor wants him to have a gastic emptying scan to see if his stomach is holding onto food for too long. She also wants to do an endoscopy to check for any other issues that could cause problems.

He got an 83% Fev1 on his PFT. It's down about 10 points from last time. Because it seemed like the Symbicort worked well last month, we are going to start it back up to see if the decline is related to his asthmatic/allergic components.

After the two hours passed, they pricked his finger to test his glucose again, which came back normal. No CFRD! (CF related diabetes). Later on this week, we are scheduled for the gastric emptying study.

After fasting and a long clinic visit, we decided tacos sounded great.