December 26, 2013

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!

November 14, 2013

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has shown promise in CF studies is when it is compared to percussion and Postural Drainage - a technique that is now outdated and no longer used (Bradley 2010)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!

October 24, 2013

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.

October 11, 2013

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.

October 6, 2013

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!

October 1, 2013

Follow Up Clinic Visit

So, it's been a few weeks since he's been off of the broad spectrum antibiotic prescribed last month, and his cough has come back, and it has been productive. Surprisingly, his PFT got up to 93% Fev1. I know it can go higher, but the doctor was pretty pleased with it. He also gained a pound, which always amazes me, because he hardly eats some days.

On the sputum culture from last month, he only grew Staph again, but the doctor is realizing that it seems to be causing more symptoms than previously thought. So, he will be on a narrow spectrum antibiotic, which pretty much will treat only the staph. His chest x-ray compared to last years is actually improved. There will always be bronchiectasis, which is widening of the airways and it causes them to become flabby. So far, it is only in the upper lobes and the goal is to prevent further damage.

The doctor noticed a lot of post nasal drip, which he seems to always have. We are going to try OTC allergy medications again to see if it will clear up. The doctor wants to do a CT scan on his sinuses in a few weeks to check for nasal polyps. These are quite common in CF, and relatively easy to treat.

August 29, 2013

Scoliosis and lower PFT's

Well, today was our annual big CF checkup. Once a year, they do basically a head-to-toe exam on him. This includes yearly blood work and X-rays.

He gained a pound since his last appointment in May. I really don't know how since his appetite has been next to nothing lately. He grew a whole inch, which makes his BMI just a little lower, but overall, they are not too concerned with his weight.

His PFT (pulmonary function test) showed that some of the smaller airways are constricted. His FEV1 was only 74%. That's down from 92% in June. He definitely has some infection going on. He has been coughing up nearly green mucus. Not quite Nickelodeon slime green, but about that thick! He has been on oral antibiotics about once every 3-4 weeks. They are going to try another course of oral antibiotics to try to clear this up. Lets pray it works. If not, it's IV antibiotics. Yikes.

We had a few concerns with trying to work out keeping him hydrated at school as well as snacks and restroom time. Whatever he needs was written in a letter signed by the doctor himself. It's so nice to have a team backing me up and fighting for what he needs.

We waited about an hour to get his labs taken. He had been fasting all morning so they could get a glucose level. They took quite a bit of blood this time. He said he felt weak afterwards...

So, at his recent pediatric appointment, she noticed possible scoliosis. The doctor today took a look at it, and did see some minor curvature, and said we would keep an eye on it with periodic X-rays. Orthopedic X-rays were done on a different floor than we're used to. I couldn't help but feel just a bit sad, even though it is minor and quite common. I didn't know this before, but apparently it's quite common among people with CF.

After 5 long hours there, and fasting since this morning, we went out for our lunch date afterwards. He definitely deserved it!

June 20, 2013

Better PFT's

The doc wanted us back to test for PFT's after 3 weeks of antibiotics. They wanted to see if they could get his lung function back higher where it used to be. The first time he blew, it was Fev1 71%. Yikes! They are allowed to try 8 times, to see if it will get higher. Thankfully, it did. And the technician allowed him to blow a 9th time, which resulted in 92%. Better. I know it can get higher, so I am praying that being extremely active through the summer will help raise it up more.

May 31, 2013

Great clinic day!

Well, he has lost another pound since his last clinic visit. His Fev1 is down to 81%. I should note though that I recently found out that he has not been taking some of his medications, and trying to do the Vest for a short amount of time. So, all of the responsibility of treatments is now on me. I have been extremely blunt about the future of his life if he keeps short changing himself.
The doctor had a good talk with him about why it's important to do his treatments. The doctor brought up the new Vertex medicine, and then the social worker and the nurse came in and talked about what they had learned at the recent CF conference. We all had goosebumps and I am completely amazed at how it is already changing people's lives. I am also amazed that CFers weight becomes normal when taking it. The doctor said it would be a few more years before it would be available for Azer to take. But the one thing they stressed over and over to him was, when he starts taking it, they want his lung function to be the best it can possibly be. It is possible that when he begins the new medicine, it could help him keep normal lung function for the rest of his life. If that isn't a motivator, I don't know what is!!
Also, they saw how extremely important running and being athletic helps CF in the long run. They had several adults with CF, and that was one thing they all had in common, being athletic and consistently exercising.

May 17, 2013

Why Do I Run?

Running isn't pretty. Running outdoors tends to be even ickier. Cold wind cutting through my clothes. Blowing my hair into my mouth, trying to blow snot out of my nose, not always going the direction I intended. After work, as soon as they get home from school, I run. In the summer, waking up early, before it gets sweltering hot, I run. I won't lie. Often, I want to stop running and walk the rest of the way. Especially when there is a hill up ahead. Another hill. I want to wimp out.

I broke my little toe recently. Even while it is still sore, I run. Getting over a cold, I run. Irritated lungs from asthma, I run.

He runs beside me. Through lung infections, with IV's in his arm. Last fall, he ran coughing up brown mucus. Trying to battle fungus growing in his lungs. But, we run.

We aren't hard-core runners. We don't run very fast. Only 3 times a week. We only run 2.5 miles at a time.

The changes I have seen in his lungs the past few years we have been running, have been incredible. Even after his lung function decreased from a nasty infection, he bounced back faster than even the doctors expected. The amount of air he can inhale as increased. During our runs, he coughs up the junk that would otherwise stay at the bottom of his lungs.

We run for his lungs...

For him...

April 30, 2013

Joggers for Azer 2013

It's that time again! Less than 20 days till the Great Strides walk in Dallas! We have already raised more than $300!

This past year has been challenging CF wise. We did home IV's for the first time, and he battled a fungal infection for several months, and was on steroids to combat the allergic responds his lungs had to the fungus. Thankfully, his lung function has been relatively stable through all of this and has been extremely physically active!

The link to our Great Strides page is

January 31, 2013

Clinic Visit, Doc is happy!

So, when he started his first few blows on the PFT test machine, he was blowing at mid 80's. But, thankfully, with each try, it got higher. The highest was 94% Fev1. Not the very best I was hoping for, but definitely great, for not being on antibiotics for about a month. So, hopefully we have killed off enough of the Staph to keep it from causing symptoms for a while.

They did labs again, and if his IGE level (allergy response) comes back lower, they will stop the antifungal medication, and will wean him further off the prednisone.

He lost a few pounds, but they aren't terribly concerned about it, since he's appetite has gone back to normal  since he's been on the lower dosage of prednisone.

The doctor mentioned that in her study and research, she found that CF people that are on the antibiotic, azithromycin long term have better lung function and less lung infections. She said it also prevents psuedomonas from growing a biofilm barrier. Sounds great. We just have to make sure it doesn't cause excessive lose stools. If it doesn't, he will be on it as long as she deems necessary.

So, things are looking up. Baseball will be starting soon. He has been doing great on our runs. I'm the one huffing and puffing, and he barely even coughs after 30 minutes of running. I am thankful that things are getting back to normal!