High Liver Enzymes

So, he's only been on the Voriconazole (antifungal medication) for about a week, but at his first weekly lab draw, they saw his liver enzymes were elevated. I am surprised it happened so quickly. So, they have lowered the dosage of Voriconazole he is taking, and have added Ursodiol to help his liver out. Within a few days of starting Voriconazole, he started complaining of blurry vision, especially when it is dark, or very bright out, but this seems to have subsided. The Prednisone (steroids) seem to be making him a bit moody, but nothing to extreme at this point. It seems to be giving him redness in the face, but luckily no other side effects.

The past few days, his cough seems to be coming back. His lungs sound pretty clear, so I'm hoping this is just temporary.

ABPA Again

Two weeks ago Azer had a blood test for ABPA which is an allergic response to aspergillus. It took a while to come back, but he tested positive for it. It was also found in his CF culture that he grew another type of fungus called Scedosporum apiospermum. This is another commonly grown fungus in CF.

He will be started on a fairly high dosage of steroids and an antifungal medication. Because the antifungal medication can cause liver damage, I have to take him to have a blood test every week to make sure his levels are within a safe range. 

I am glad that at this point he can be treated without being admitted and orally!

Clinic Day

About 2 weeks before our CF appointment, Azer started to get a congested cough. No matter how much CPT I did on him, I just couldn't get all the mucus out (I always do CPT in addition to the Vest when he's sick. He has never coughed anything out during the Vest). With the stethoscope, he had diminished airflow in the lower lobes. So, the doctor prescribed the antibiotic Cipro for 14 days. Within a few days, his cough sounded dry again.

His oxygen was 100%. On his PFT, the highest he got was 93% Fev1 but the respiratory technician said that she could see he had a mucus plug. The doctor wants us to do Pulmozyme and Hyper-Sal twice a day instead of just once a day to clear up any remaining mucus. He hasn't gained any weight, but the doctor said that it tends to be hard to gain weight in the summer, especially how active he is.

It was time for annual X-rays and labs!

This machine was set up in the room where they drew his blood (it wasn't set up for him). I haven't seen it in 10 years. It is used to collect sweat to diagnose Cystic Fibrosis using electrodes.

CF Walk

What a time! We raised $335!

Azer and another boy from his baseball team were the the first people over the finish line.

Thank you to all who donated and thank you for those who came today!

CF Clinic

Well, he's been feeling pretty good the past 3 months. No real increases of cough. His oxygen was excellent, as always. He gained a few pounds. I've been grateful that he has had a fairly good appetite most days. He has been having floaty, fatty stools lately, which had the doctor and dietitian a bit puzzled. He is on a pretty high dosage of enzymes right now. The only way they could increase the dosage was if he gained more weight. They try to keep the enzyme dosage under a certain limit because if he was on a too high dose for too long, it could cause erosion of the intestinal wall. 

His PFT's were down to 87% Fev1 today. They are running a culture to see if he is growing anything that needs to be treated. The main reason they think his lung function is down is due to allergies. If allergies can affect a normal person's eyes, nose, ears and throat, they most certainly can affect the lungs of a person with CF!   

He got a new Acapella today! It seems to work quite effectively as he was coughing up all kinds of lovely things, and guess who got to hold the specimen cup ;-). The Acapella works by creating positive air pressure in the lungs to force open the alveoli which helps push the mucus into the bigger airways where it can be coughed out. In fact, he was still coughing up mucus this evening!  

The doctor noted how clean his G-tube stoma is. Azer said he keeps it clean, and I muttered how he cleans his stoma better than his room!

It is 15 days until the Great Strides CF walk! We have raised $80 so far! If you are interested in donating or walking with us, just click here! 

Great Strides 2014!!

Hello all!

We are Team "Joggers for Azer" again this year. Please register to walk with us!


This is the walk information for this year:

Date: 05/31/2014
Check-in: 8:00am
Walk: 9:00am
Distance: 5k
Chapter: Northeast Texas - Dallas
Event Location: Vitruvian Park,
Addison, TX

The walk is held at a different place this year, so a change of scenery sounds nice! 

Here is the link for our page. 

http://fightcf.cff.org/site/TR?px=1527229&fr_id=2245&pg=personal

Look forward to seeing you there! 

Azer putting in a new Mic-key button

Azer has been getting better about recognizing when the balloon may be getting a little low on water. I noticed he has way less anxiety about pulling out the button if he does it himself, since only he knows how it really feels. I am really proud of him for being able to do so much on his own! 

Here is a picture of the Mic-key button with the balloon filled with water. That is what holds the button inside the stomach wall. We usually replace the button about every 6 months.

Clinic day!

Yesterday, Azer had his first fasting and glucose test. I learned that they start doing these annually after they turn 10 years old. He wasn't the happiest about having to fast all night and not getting his morning feed. However, his blood sugar was completely normal fasting. They then had him drink a very sugary liquid, and then tested his blood two hours later, and again it was normal.

He was down about two pounds yesterday. The dietitian was kind of concerned, but I really think it had something to do with not getting his night and morning feed. He tends to hold a lot of liquid in his stomach. On the plus side, as of late, he actually has had an appetite. That is going from having nearly no appetite, ever. The only exception to that is when he is on Prednisone, which nobody wants.

He scored 94% Fev1 on his PFT's yesterday. He had great air intake, despite having a mildly wet cough. The doctor said he sounded very clear. As usual, the doctor said he has the cleanest G-tube stoma he has ever seen. That's always funny because he keeps his button so clean, but it is a constant struggle to keep his room clean. Boys are strange I guess!

After clinic, we had our obligatory lunch together. Azer chose Subway. The kid ate nearly an entire foot long meatball sub. Very impressive!

After which, he was very energetic. So, yay for a good clinic day!

No Psuedo!

So, the sputum culture from our last clinic came back clear of Pseudomonas! Yay! I called the clinic last week to ask them if they wanted us to do another round of Tobi, but they said nope! They will test for Pseudomonas again when we have clinic in a few months. But, thank God we cleared it out!

Clinic Day

Well, it was a long day, but it turned out well. Early in the morning, Azer got the CT scan off his sinuses to check for nasal polyps. The machine he used was brand-new, so he was the very first person to try it out!

The CT scan revealed there are no visible polyps, and just typical CF mucus thickening. So, that's a relief!

He managed to gain 3 lbs in a month. The Tobi isn't supposed to make you hungrier, but oddly he has had a bit more of an appetite lately. His FEV1 was 92%, which is around his normal baseline. His lungs sounded clear, and overall, the doctor was quite pleased. We haven't received the results of his culture yet, but the doctor said that the colonies of pseudomonas aren't resistant to antibiotics, so there is hope for possible eradication of it.

I wanted to share a little information I was reading recently on another CF mom's page.

This article in the CFWA RED Magazine, summer edition, lists several studies done and have found the Vest to be much less effective than Positive Airway clearance methods.

Here is a segment of the article.

"Another study in Thorax by Osman et al 2010 compared the Vest to the usual airway clearance techniques (such as Flutter, PEP, active cycle of breathing technique, autogenic drainage) performed by the participants. This was a short term study, and looked at sputum production during the physio session and for 24 hours after using either the Vest, or the participant's usual technique. When participants used the Vest they produced less sputum in both the physio session and the w24 hours afterwards. The majority (55%) of participants also said they preferred their usual technique over the Vest. in fact, the only time the Vest has shown promise in CF studies is when it is compared to percussion and Postural Drainage - a technique that is now outdated and no longer used (Bradley 2010)

There may be a couple of reasons why the Vest is not as effective as other physiotherapy techniques for CF. One is that it provides no positive airways pressure, which helps to splint floppy airways open to allow sputum to travel from the small airways up into the larger airways, where it can be coughed up. If you have damaged, floppy airways, and they are not being splinted open by positive pressure during chest physio, they can close down and trap sputum deep in the lungs.
Positive airways Pressure also helps to get air behind trapped sputum, forcing it out of the small airways. Positive Airways Pressure techniques include PEP (including Bubble PEP), Flutter, and Acapella - but can be produced whenever you blow out against resistance (blowing out under water, blowing up balloons, blowing through pursed lips). The Vest may also not be as effective because it is a passive technique. Whenever you have to blow out against resistance, or use techniques like ACBT or autogenic drainage to control your breathing, you engage and strengthen your muscles of breathing - it's a workout for the muscles that help your lungs work! you can't get this with the Vest."

I have always felt that the Vest hasn't been as effective for Azer has Chest Percussion Therapy. While I am not considering eliminating the Vest from our treatments, I will be throwing in more CPT sessions along with using the Acapella. After a few days of using the Acapella in addition to his normal breathing treatment routine, I have heard a difference in his cough, and he says his lungs feel clearer. Just another weapon in the arsenal!

Tobi Podhaler

After waiting a few weeks to receive it, the Podhaler finally arrived.

It is quite odd sounding, I think, to inhale a powdered capsule, but that's what it is.

 It is not used with the nebulizer. It is inhaled with this plastic thingy.

 It took a few minutes to figure it out, with help from a video sent by our CF clinic, but it isn't too complicated.

Amazing that this little wonder just got approved by the FDA this year. It used to take about 45 minutes to inhale the traditional Tobi by nebulizer. Tack that onto a 30 minute Vest treatment and two other nebulized medications, do all this 2-3x a day and we don't have much "day" left to do other things. I'm grateful that we have this, since fall baseball is still in full swing, and we have tournaments coming up.

Cultured Pseudomonas

Well, the first time Az cultured pseudomonas was back in 2010. At his last CF clinic, they did their usual sputum culture, and on Wednesday the nurse called to tell me that he cultured pseudomonas again. 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 3 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. What I do know about PA, is that it can form a bio-film to protect itself against antibiotics. The good news is, they have found his PA isn't resistant against any antibiotics at this time, so that is really good news!

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. What a drag, right? Well, the good news in all this is that he will be able to take his inhaled Tobi with the Tobi Podhaler, which only takes about 6 minutes to inhale the powdered antibiotic. The last time Azer did Tobi, he experienced ear ringing so bad it frightened him. With this method of taking it, it decreases the risk of ear damage.

On top of the Tobi, he is going to have to do Cipro, another antibiotic on top of the one he is already taking for the Staph in his sinuses. He has already been spending a lot of time in the bathroom because of the antibiotics, so needless to say, this isn't going to help that situation much. However, the PA has to be treated aggressively. I hope we can control or eradicate it as quickly as we did last time.

10 Years!

Today marks the day when I first started raising a child with a life threatening, chronic illness. Many people, regardless of what illness it is, always say, focus on the person, not the disease. The person is separate from the disease. This is difficult to do, as a parent, because many times our children are the embodiment of our hope, our future, our fears. When you have a child with a fatal illness, it is difficult, at times, to look at them and not see the face of the disease. Each cough can be a reminder of the next medication, the next breathing treatment, the next doctor's appointment, the next meal, the next G-tube feeding. Some days it's business as usual, and some days it's overwhelming. It is heartbreaking and rewarding. It can feel like I am fighting against the waves of mucus and infection, and it can feel like we are making progress in keeping his body healthy.

Today marks the day when I started raising a child, my son. I always knew I'd  have a boy. Before he was born, I had an image in my mind that we'd be friends. Exploring nature trails, looking at bugs, catching frogs. The day he could finally walk, I'd take his hand and we'd walk as far as his little legs could manage. Taking him to the park and watching him run around was so thrilling. Watching him observe other children, learning to socialize, and make friends. Teaching him to play catch was one of our favorite activities. Being outdoors, and both of us trying to get better at catching the  ball with our gloves, and not our faces, shins, etc. I knew very early on that throwing a ball was something he couldn't live without. It has been such a joy watching him develop in baseball. His passion and motivation is always there, regardless of how he feels. He reminds me a lot of my younger self, always with a smile and ready to laugh off the days troubles and just play. I am so proud of the young man he is developing into.

Happy 1st decade birthday Azer!