A crazy antibiotic October!

Last week was crazy for us!  After about 3 weeks of what I thought were allergies for Cal, we took her into see the peditrician.  She just didn't look right, overly tired, pale, not herself.  Doc diagnosed her with severe bronchitic/walking pnemonia!  Ugh, zpac (antibiotic #1).  Evie, along for the ride at the doc looked great but developed a little cough over the previous week so I had her checked too.  Bam, ear infection, amoxicillin (antibiotic #2).  hmm...this could get interesting...I was convinced we weren't going to have a tri-fecta since my docs appt was scheduled for later in the day.  One of us really had to be just affected by these allergies!  Yes, just allergies for me but the cloudiness in my head wasn't from allergies - it was from a collapsed eustacian tube.  Nice, zpac #2 (antibiotic #3). 

Fortunately we had a routine CF clinic visit for Dean (at the specialist) the day after us girls had our doc appts and all the scripts were filled.  He was on Bactrim two weeks back for a deep cough so in my mind I completely thought he was "covered" from any of these recent illnesses we brought into the house.  He looked great but actually woke with a cold that very morning.  Doc listened and deteremined it was just viral but wrote us a script in case the cough came back.  It did, 2 days later, so we filled zpac #3 (antiobiotic #4).  The pharmacists at CVS must think I'm reselling this stuff ! 

After Dean's 4th day on zpac, we (conveniently) had his 18month appt scheduled (at the peditrician).  Cold was still there and that nasty cough still lingering.  Doc told me he had an ear infection.  Wouldn't this be covered by the zpac that he was going to finish that evening?  So confused!  I thought antibiotics wiped you out and got rid of all the nasty little bugs?  Never ending learning process for me.  We got home, called the specialist to update them on the ear infection and find out if we needed to start Dean on another antibiotic.  Checking the preliminary cultures from our "well-visit" last week, we were told that Dean cultured pseudomonas aeruginosa or PA (a nasty little bacteria that lives in water and soil and to most of the population, wouldn't do too much harm).  Because the thick mucus that sticks in Dean's lungs forms a warm, moist environment, it's a great breeding ground for PA where it establishes permanent colonies causing significant damage to the airways and ultimately reduces his lung function.  PA can be resistant to most antibiotics so the normal treatment is a 28 day inhaled antibiotic called TOBI.

So, the feelings part of this blog...I was sad.  I know this is a bad one to have and we've been so great about keeping clean.  We wash our hands everytime we come into the house from anywhere.  Even Dean gets up on the stool, knows the routine and even says dry dry dry when he's done. We don't wear shoes in our house, we disinfect with our "green" cleaning products, we boil our nebulizer after every use, and on and on.  I said to our nurse, I just don't get it, how did he get it?  She said it's everywhere, water, soil, in the environment. Oh my...Can you picture an 18 month old boy in your head now?  What is he doing?  Digging the rocks out of the dirt?  Helping you pull tomatoes off the vine in your salsa garden?   Drinking out of a hose?  Splashing in a puddle?  Trying to turn on the jacuzzi in your tub?  How can I avoid this bacteria with my little boy?  Nearly impossible...  Undeniably frustrating... 

So, back to reality...after a few days of calls to figure out the details, namely the insurance bawking at the astronomical price of TOBI, we received the meds in the mail and added this to Dean's normal daily treatments.  With our new schedule in place, we're set to attack this bacteria!  For the next 28 days, Dean's treatment schedule will be:

• Pulmozyme for 10 min (inhaled through a nebulizer to thin the mucus in his lungs)
• Vest Therapy for 20 min (to move the mucus out of his airways)
• Tobi for 30 min  (to attack the psedomonas bacteria) 2x a day 12 hours apart (once in the morning and then again just before bedtime).

Besides the lung treatments, his digestive treatments include:

• 3 Creon 6 with snacks and meals (digestive enzymes)
• 2 ml zantac 2x a day (to reduce the acid and aid in the absorption of food)
• 2 ml CF vitamins (extra A, D, E and K)

I thought this was a list, as usual, God's always there to pull me up after I get a bit down.  I clicked on a link of another fellow CF mommy only to find her list for her little man more than 2x as long.  We're still blessed, always will be...my next to do...find an app to track all of this, my brain cells aren't what they used to be...

OJ, the Juice, turns 6!

Happy Birthday Owen.  Ever caring and completely conscious of doing the right thing, Owen has "grown up" this past year.  He's gone from his nickname of PS (personal space - as he doesn't like to let you have yours) to a sweet and caring and incredibly smart kindergartner.  Owen enjoys playing Wii, eating bacon and being tickled.  He's got one of the best giggles in the world, can do math in his head, dips his salami in mustard and gives the strongest hugs ever.  He loves to torment his little sister, wrestle with his little brother and follow his big sister everywhere.  He makes you smile with his smile and tears up only when a can of chicken falls on his toe or when you make him practice his sight words.  To many years of smiles and giggles buddy, we love you like crazy!

Owen James (6)

What is KALYDECO?

Today, Vertex applied to the FDA for a drug (Kalydeco) that targets the underlying cause of CF (rather than a drug to treat the symptoms).  Here is the full article.  Although, Dean doesn't have the G551D change that this drug targets, we're ecstatic for those that will benefit from this and patiently await the results as well as the drug that is in the pipeline that will treat the change (DF508) that he has.  Keep those prayers coming, they're working people!!!


http://www.cff.org/aboutCFFoundation/NewsEvents/Vertex-Submits-VX-770-Application-to-FDA.cfm

2012 Fundraising already?

Hello!  We have a lofty fundraising goal this year so we're forming our team to start brainstorming ideas and we need help!  We'll kick-off on Nov. 4 so if you're interested in joining the planning team of Eat. Pray. Dean, please let us know.  Our main fundraiser will be the Great Strides Walk in May 2012 and our plan is to see if we can host another event/two with your help.  Enjoy the cool down and a pic from our apple and pumpkin picking day!

Summer 2011



Our summer of fun...awesome...

some pics to show you the smiles...

5 college roommates; 13 offspring



our beautiful niece graduates

from my Alma Mater



2nd Annual Tea Party with Gigi



Evie turns 3



and shows the first harvest

from her Salsa garden to Papi



Owen achieves his
Yellow Belt
 in Taekwondo



and gets his first baseball
trophy as a Pirate

Hanging with the O's

 

Cali performed as a Mouse in the
Bear Went over the Mountain

 


turned 8, and got a room

makeover from Omma & Obba



Dean gets his first

knee scrape :(





reminds us that he LOVES
Trader Joe's Greek Yogurt,



  

and takes his first wave runner ride!



A weekend away with great friends in WI

Ages of the kids:  8,7,6,5,4,3,2,1



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.

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...