I may have Doritos on my face, but no PA in my lungs!



Pseudomonas aeruginosa



Back in October, we found out Dean had cultured Pseudomonas aeruginosa, a common bacteria found in water, soil, etc. Normally the bacteria doesn't bother anyone but for Dean with CF, his lungs form an ideal breeding ground for this bacteria that likes to grow in warm, moist environments.  This is dangerous because repeated lung infections from PA are the leading cause of decreasing lung function in people with CF. Most oral antibiotics cannot attack the bacteria so Dean's treatment consisted of 28 days of an inhaled antibiotic, Tobramycin (called TOBI) 2x a day.  The inhaled TOBI is concentrated in the airways and targets the area infected with the bacteria.  It made Dean tack on an additional 20 min inhaled/nebulized anitibiotic after his morning routine and then again in the evening.  I thought getting a 19 month old to sit somewhat still for 30 minutes was hard (his normal daily treatment time); 50 minutes seemed excruciating.  I stressed over it all...sometimes we only lasted 17 min, other times the full 20...once only 13 minutes (ugh)!  I felt sure that the few minutes we missed were going to cause us to not get rid of the bacteria.  I stressed about him having his binky in during the treatments but it's really the only way we can get him to stay there without flailing all around. 

This was our first experience with PA.  It broke my heart when I heard.  I had previously read that about 40% of CFers between 6 and 10 have had PA infections in their lungs.  How in the world could our 1 year old get it?  Was that so bad that it will damage his lungs? Will the TOBI work?  Sometimes I forget that the data for the little ones just isn't there.  Newborn screening for CF is relatively new in many of the states (within the last 6 years for many of the 50 states) so the data just doesn't exist for effectiveness for some of the treatments that we will be faced with.  However, for the strain of PA that Dean had (non-mucoid), this treatment was proven to work in kids 6 and above.  We were told it could sometimes take a 2nd or 3rd round of TOBI (coupled with a separate oral antiobiotic) to combat this bacteria.

In typical Dean fashion, he adjusted quickly and went with the flow.  He had his moments but overall, did great.  He...amazes...us.  After the 28 day cycle, Dean had a throat swab and the culture came back psudeomonas free.   

We are in the clear.  No cough, cold, congestion.  I quickly learned to stop complaining about the normal 30 minutes of treatments that he has daily and I am believing that they too have to be working.  I constantly learn each step of the way, not only medically (learning new terms and what questions to ask, new precautions to take) but also have learned to be more content.  I can't predict and plan what is going to come up so I've learned to appreciate the little things (so cliche right) but it's true...

...the silly comments that the kids make, the crazy loudness that a Sunday conga line brings, the fascination and wonder about how in the world our ELF got into the fishtank, the smile that only one of your own can bring.  They live in the moment, I am so trying to get there but as a start I'm more content than ever, more thankful than ever, more blessed than ever.

No more PA video message from Dean!

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Boomer Esiason said my Chicken Salad is tremendous!

Abbot Laboratories sponsored a contest, the CF Chef Challenge, to launch a new online nutrition resource that allows those of us touched by cystic fibrosis to share recipes.  CFers need to maintain a high calorie, high fat, high protein diet because they burn more calories than normal because their body is working hard to deal with all of that sticky mucus.  So those low fat, low calorie recipes that many deal with daily just won't do! 


To launch the site, they held a CF Chef Challenge, where you submitted a recipe (and your CF story) and a winner was chosen in the Breakfast, Lunch, Dinner and Snack categories. 


The judges (Michael Symon from the Food Network, the legendary Boomer Esiason, Ali Christensen from America's Got Talent and a CFer herself, and Suzanne Michel a registered dietician) held a taste test and chose the winners.  I can't claim to be a cook, but my chicken salad did take first in the Lunch Category!


Here is a link to the main website Chef4CF. 


Click here to see the videos of the taste test and the recipe.



One of the prizes!
My very own Chef hat
signed by the Judges!

Chicken Salad





And the media props:

http://www.multivu.com/mnr/52460-cfchef-recipe-challenge-winners (videos of the taste test)

http://idmanagedsolutions.usatoday.com/news/story.idms?ID_NEWS=210792987 (USA Today)

http://www.frankfortstation.com/Articles-Story-of-the-Day-c-2011-11-14-231600.114133-Cooking-for-Dean.html (Frankfort Station)

http://southtownstar.suntimes.com/lifestyles/food/8849352-423/frankfort-mom-wins-cf-recipe-contest.html (Southtown Star)

Halloween 2011

Happy Halloween from our bunch (a witch, WaLuigi, a pumpkin and a monkey).  Fun times with all the Halloween activities and way too much candy.  I'm having a hard time not swiping a Snickers everytime I pass the large purple bowl!

We're all on the mend over here, feeling a lot better, adjusting to the new treatments and looking forward to the launch of our 2012 Fundraising committee - our kick-off is Friday, November 4th at 6:30pm.  Contact us if you'd like to be a part of it.  We need all the help we can get!

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!