a different kind of perfect

i'm a mom...on a mission...to enjoy life everyday...appreciate the little moments...try not to get so easily annoyed...raise my kids with a faith legacy that leads them to continue to be amazing people...and to make my husband feel as special as he makes me feel.

What is Cystic Fibrosis?

CF is an inherited chronic disease that affects the lungs and digestive system of about 30k children and adults in the US (70k worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that both clogs the lungs and obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food. [www.cff.org]

Tuesday, August 23, 2011

Dean's first few weeks of neb and Vest treatments

Everything was delivered in record-speed and we were up and running only 3.5 days after the doctor ordered the new treatments.  Saturday morning (August 6, 2011) marked our first neb treatment.  We had given neb treatments to our older kids before so had an idea of what to expect but it's been a while.  We decided it best to do first thing when Dean wakes up - give him a head start on the day, get everything done with first thing and start our routine off that way.

Pulmozyme takes a whole 5 minutes to go through the neb so we were fortunate that we only had to hold him tightly for a short amount of time as he wriggled in our arms.  First I tried holding him, but I didn't make the mask tight enough so he was easily able to pull it off.  He's 25 pounds too so he has a little punch to him as he writhes and wiggles all over.  Jim had to take over and secured his arms down as he held the mask to his beautiful face.  The five minutes went quick in retrospect, but seemed to take forever at the time.  Dean was confused, agitated and wanted nothing to do with all the new gadgets in his room.

Immediately following the Pulmozyme, we started the vest for 10 minutes (Doc ordered 20 but we were told we could ease into it for a few days).  Dean didn't like it for the first 2 minutes, but we had a tip from our Respiratory Therapist to save a new game/toy for this time so we had some stacking blocks that distracted him and kept him "busy" for the next 8 minutes.  Along with Evie who kept cheering him on and telling him what a good boy he was.  Dean was sitting on my lap during the 10 minutes and I was truly shocked at how strong the vibrations were against my chest, I could only imagine the feel on his little body.  He did make a few sounds during the treatments and his little voice vibrated as if he was talking into a fan.

That first weekend, Dean just coughed and coughed, spit up a little mucus and continued to cough all through the next week, which isn't typical of him and we were told not typical at all.  However after meeting with the doctor again on the 16th, he explained that the cough receptors are near the larger airways so as we're "moving this mucus" from the smaller airways to the larger airways so he can expel it, it's triggering some cough receptors.  Absence of the sign of any other symptoms of a cold, we left the office that day with no further meds (whew!) but instructions to wait 30 minutes after the pulmozyme to start the vest treatment (in order for the meds to work). 

Changing our routine so soon after getting established proved to be a setback and then the 18th proved another interesting day for us.  After finishing his treatments and setting him in his highchair to eat, Dean gobbled his oatmeal as he was starving from waiting to eat.  After finishing, and setting him down out of his chair, he couldn't walk straight or normally; he resorted to crawling, became irritated, clingy, grumpy and just not himself.  After a trip to the pediatrician and some back and forth emails with the specialist, it was concluded that he had neuropraxia but we all couldn't agree on whether it was from the vest or from a compromising position. Either way, he's now better and it was a one-time issue. 

The hard part now is finding out the timing of the treatments.  First thing in the morning works well but Dean is sometimes starving so it doesn't work and now with new school routines, we're in a constant state of change.  Each day gets subsequently better.  We learned he likes to sit on our laps, lay his head against our chest, keep in his binky and is fine as long as we don't put the mask string around his head.  Just keeping our Bubbles the Fist Neb Mask against his face is enough.  I think he just doesn't want us messing with his curls!

Cali, Owen and Evie are so attentive and helpful and want to hold him during the vest treatment.  They talk to him and tell him what a good "buddy" he is - I think it calms him to be near them.

We'll get it all figured out.  We're blessed enough to only have to figure this out once a day....for now.

Monday, August 8, 2011

Our CF Clinic Checkup (August 2011 - 16 months old)

Last week, we had our routine checkup.  Dean meets with his CF Specialist team every three months (barring any illnesses) to make sure that he's gaining weight and doing well.  I walked in with Dean knowing it was going to be a long day.  First we went for his yearly chest x-ray to determine if there was any mucus buildup.  After a registration/insurance snafu, we finally got to the radiation lab.  Dean cried but sat still for the four pictures as long as I stayed close.

Next we went for his routine appointment.  This is a team which includes about 10 people all dedicated to work with us to manage the CF.  Dean's weight and height and head circumference are measured (just as it is at a pediatrician visit) and his pulse ox level is taken (to measure the oxygen saturation of his blood). After routine questions, the doc then showed me Dean's x-ray from May 2010 compared to this one and expressed his concern over the mucus buildup in one of his lungs.  I was devasted.  Honestly, I walked in knowing we've attacked this CF thus far from a digestive point-of-view and he's looks so great, hasn't been sick/on antibiotics in over 3 months, is well above the 50%ile in head, weight and far above in height, and is developmentally where the other kids were at this age!  To me, all was good, I completely expected a "clean" bill of health.  I didn't forget that CF affects both the digestive and respiratory system, I just prayed that since we've triumphed over the digestive part, it was only right that the respiratory would be the same.  The doc explained that we needed to put Dean on Pulmozyme (an inhalation therapy drug) administered through a nebulizer daily which would break up the thick mucus in the airways allowing air to flow better and preventing bacteria build-up.  In older kids with CF, it has proven to reduce the number of lung infections and to improve lung function. The next part almost made me cry.  He said, as soon as he's ready for the Vest, we need to get him on that.   The Vest is just that, a life-jacket like vest that Dean wears that attaches to two hoses that are connected to a generator-like machine which thumps on Dean's lungs for a set amount of time at a set pressure and frequency. I took it as a sign that I failed in doing the manual thumpers.  I know it wasn't true, it's the natural progression of the disease but it's hard to take.  And, everything we've heard is that babies as early as 18 months go on the vest.  We didn't even think it was a possibility that we'd go on it, Dean's only 16 months!!  We've got this little man growing so his chest measured 21" (2 inches above the minimum for the Vest).  After that measurement, all the rest of the visit was a blur.  Instructions on how to get a nebulizer, how to clean it, how to get the Pulmozyme, how to store it, when to give it, the cost, process for ordering the Vest, when to use it, how the insurance deals with it, how to clean that, what type to order.  I was trying to absorb it all while trying not to cry.

More routine things went on but I'll save that for another post.  Finally, after leaving the appt 2.5 hours after arriving at the hospital, we still needed to have blood drawn for labwork.  12 ml lighter, and another 30 minutes later we were in the car and Dean was sleeping before we had driven a block. 

Our little man, so tough and awesome, continues to make us smile, now it's mom's turn to learn how to toughen up.  I feared life would change when the respiratory therapy had to start and I walked around in a daze for two days after the appt.- making and answering calls, filling out forms, figuring out how this is all going to work.  I think we're getting there though (and I know I think too much!).  It'll all be fine.  Jim's perspective is that we can't look at it as if we did anything wrong, just that we're blessed that we can get him started on this earlier than we thought- and we'll attack this mucus buildup just as we attacked his pancreatic issue.  Well said...