The Big Picture

It has been a tough couple of weeks…but I cannot be distracted from the big picture.  Brady had been having a hell of a time breathing and sleeping because of swollen, chronically infected tonsils and adenoids.   He had surgery to remove them on July 28^th.  I know lots of people have had their tonsils removed, but I think it has been a little tougher for Brady.  He has to swallow a ton of pills every day, no matter how much his throat hurts.  His breathing treatments have definitely been a challenge also.  One inhaled treatment is concentrated salt water, and while probably very therapeutic, seems to burn quite a bit.  He has been complaining during his vest treatments too.  I suppose being shaken like crazy doesn’t do much to soothe raw surgical wounds.  I feel so bad.  CF just sucks.  It makes his life so much harder.  And my heart breaks when he sees me coming toward him with another handful of pills and he starts to cry.  Or when I am sitting next to him for his treatments and all I hear is a muffled, “mommy my throat hurts” from behind his nebulizer mask.  Fortunately, we are now over a week post-surgery and things should start getting a lot better soon.  That is worth repeating…Things should start getting a lot better soon.

The day Brady had his surgery; Vertex announced their plans to apply to the FDA with VX-770 in October, 2011.  This October.  Just a few months away.  And while I know there are a million variables that could affect the outcome, VX-770 does have fast-track status with the FDA.   Vertex Pharmaceuticals managed to get another one of their new drugs (Incivek for Hepatitis) approved earlier this year in less than 2 months!  Vertex was prepared and was able to make Incivek available to patients 3 days after the approval.  VX-770 has superstar clinical data with only a few minor adverse side effects reported.  Honestly, I think they are in a good position for a hasty approval.  I am trying so hard to think about this objectively.   I can’t even guess how many scenarios I’ve played out in my head concerning when and how Brady will get his drug.  My latest goes something like this(cue dreaming sounding background music)…Vertex submits their new drug application in October and is approved by the end of year.  2012 starts off with a bang and VX-770 drops from the sky with a little silken parachute into my hands.  I’m pretty sure the bottle will be encrusted with jewels.  I rub the bottle and a genie pops out.  He doesn’t even ask what my wish is, he just hands me the little blue pill.  At that point, I drop to my knees and give it to Brady(very dramatic, tears streaming down my face).  He swallows it and runs off to play, as if nothing ever happened.  But for Brock and I, there is a palpable shift in our universe at that very moment(I know, soooo cheesy right?  Big deal, it is my fantasy!) 
Back to reality, I’m so curious to know how I will feel right then.  I try to be thankful on a daily basis, but this is new.  This is something I would gladly give my life for.  It must feel awesome to receive a gift like that.  It is something I’ve dreamed about every day since his diagnosis.  I have never wanted anything more.  Never.  And now it is practically here.  It is happening.  There are still thousands of people with CF that won’t benefit from this right away so it seems like an even more personal miracle.  When I first read about Vertex’s research, Brady was an infant.  I dreamed up my first scenario that day.  As the years passed and further trial results were published, I obviously became more and more obsessed.  This couldn’t be better if I had scripted it myself.  Why the hell am I so lucky?  I have been trying to stay busy and focus on other things for my sanity.  Trying is the key word there.  My detox diet has been a nice distraction.  I want to look my best for all the television interviews I will be doing when Brady is all fixed up and the world wants to marvel at him ;)  I hope Brady’s pharmacy will agree to deliver via silken parachute(horse drawn carriage would be a second choice)…just this once! 

Why I need coffee

It is impossible to look at Brady and imagine what his day is like…but here is what he actually does most days.  It is a demanding schedule that is sometimes very difficult to adhere to, for everyone.  We are happy that all of Brady’s medicines are in pill form now.  He used to have to swallow some disgusting liquid vitamins, and other liquid medicines.  Getting the prescription filled is one thing…getting the medicine inside the child is quite another thing.  We are lucky that Brady is very compliant with his treatment schedule.  As you will see, Brady has to do something CF related every few hours throughout the day.  It makes things like traveling and having a social life difficult sometimes, but with planning it can be done.  It is a lot of work for Brock and I, so it is a good thing that Brady is so darn cute.  I have Disney Pixar movies and white Russian cocktails to thank for getting me through some difficult times. Keep in mind that the schedule below is the “healthy” schedule.  If Brady is ill, breathing and vest treatments are added.  Let’s just say that I will be smiling from ear to ear as Brady is able to be taken off several of the medicines and treatments in his schedule after VX-770 is in his system for a while!  That is our hope and what I truly believe will happen.  Anything is possible! 

7:00 a.m. Wake up—The first thing I do when Brady wakes up is give him his Prevacid capsule in a spoonful of applesauce.  He used to swallow his enzyme beads in applesauce and was used to just gulping it down without chewing, so I started sticking pills in the spoon whole when he turned 2.  He stilI swallows his pills this way, which means he eats a ton of applesauce.  Fortunately, he loves it.  I get him some OJ and spike it with a liquid fiber supplement.  He needs a drink nearby for treatments.  If I have time, I begin preparing his morning treatments before he wakes up.  Basically, I have to wash hands, prepare nebulizers with correct medicines, take Brady to potty and get him in his vest, and get something sweet on the TV to occupy the next 45 minutes of our time.  Brady inhales his Albuterol (about 6 minutes) and then his Hyptertonic Saline solution (about 18 minutes), and then we turn on his Vest to shake the crap out of his lungs for 20 more minutes. 

8:00 a.m. Breakfast. While he is finishing his vest, I begin cooking his breakfast and sterilizing his nebulizers.  After every single session, his equipment gets cleaned.  First, wash nebulizers in warm soapy water and rinse.  Next, sterilize them in an electric baby bottle steam sterilizer.  Finally, air dry them in a little device called a “Germ Guardian” that uses dry heat to kill germs.  Boring.  Moving on… I cook something special for him every morning.  I strive for a minimum of 15 grams of fat and 15 grams of protein at meals.  He is usually pretty hungry and goes to town on Pediasure and eggs scrambled in tablespoons of butter. His other breakfast faves include: frozen fortified whole grain waffles soaked in butter and pure maple syrup, rice with raisins, cinnamon, and ½ n ½, and double fiber toast with PB, honey, and butter.    At breakfast, Brady takes 5 ½ more pills: 3 Zenpep, 1 Ursodiol, ½ Source CF chewable vitamin tablet, 1 Singulair pill.  All this crap takes a while. The time is extended by the fact that Brady is the slowest chewer in the universe. 

9:00 a.m.  Party time.  Put on sunscreen and go to the park, library, etc… I usually try to eat some breakfast and wash my face around this time.    Brady is occasionally kind of pissed that I’ve been making so many demands of him and wants to do something fun.  If the weather is permitting, we go outside.  He needs exercise every day for his lungs and this is usually the best time to get it. 

12:00 p.m. Lunch time.  Brady’s favorite lunches right now are: elk burgers and chips, or creamy cheesy pasta with bacon sprinkles and a side of black olives.  Can’t say I blame him.  He takes 5 ½ more pills at lunch: 3 Zenpep, a fish oil pill, an antioxidant Juiceplus capsule, and ½ a chewable vitamin(I break his vitamin in 2 because it is super strong and has a tendency to hurt Brady’s stomach and make him puke).  I always offer him Pediasure or Boost at mealtime.  He doesn’t need enzymes to process juice, so I mix juice with water and give that to him between meals. 

1:45 p.m. Nap time.  Brady usually sleeps between 2-3:30 or so.  He loves his nap and I am pretty strict about it.  I feel the extra rest helps to keep his immune system humming.    

4:30 p.m. Dinner time. Prepare meal and give 6 more pills: 3 Zenpep, 1 fishoil, N-acetylcysteine capsule, and Juiceplus antioxidant capsule.

6:30 p.m.  Breathing treatment time again.  In the evening, he does the same as in the morning, plus one additional breathing treatment (Pulmozyme—takes about 6 extra minutes). It takes an about an hour to do it all. Usually Brock does his evening treatments with himJ  There are 2 nebulizers to sterilize again just like the routine described earlier.  *Brady spends about 1 hr. 45 min total every day doing treatments for his lungs.  I bet we spend 20 min sterilizing equipment every day plus time to set up and put away all the machines. 

8:00 p.m. Night time snack plus 6 more pills:3 Zenpep, calcium pill, antioxidant Juiceplus, Ursodiol.  We have to try to cram a few more calories in before bed.  Hopefully they go straight to his tiny butt!  Buttered graham crackers, buttered salty popcorn, and vanilla milkshakes are favorites for this snack.  We can never let up on the calories or his weight gain completely stalls out or he starts losing!  It is a huge source of stress for me.  

8:30 p.m. Shower. We try to bathe or shower Brady every evening to keep allergens and bacteria off his skin.  It is pretty annoying right now because he fights it.  If we don’t do it, his nose gets all stuffed up and he snorts and snores all night.  Boo allergies! 

Medicine Guide

This is just a brief description of the medicines he takes. (Brady takes an average of 23 pills a day, That works out to 690 a month. 8280 in a year.)  Prescription drug coverage is of supreme importance for us!  Brady’s medicines retail approximately $4500/mo.

Prevacid—Acid blocker.  Assists action of enzymes.

Zenpep—These are the digestive enzyme pills that Brady must take with everything he eats.  He generally takes 3 at each meal, but the dosage gets adjusted based on what and how much he is eating (which is sometimes anybody’s guess!).

Ursodiol—This pill assists his liver function.

Singlulair—Asthma pill

Fish Oil Supplement—helps body with inflammation, which is a concern in the lungs in CFers

JuicePlus Antioxidant Supplements—These are a whole food supplement made with a variety of fruits and veggies.  There are 3 different pills: fruits, veggies, and berries.  That is why he takes 3 of them each day.  Brady is a very picky eater and I feel like this is at least something I can do to help fill the gaps in his diet.  I was skeptical, but there has been a decent amount of clinical research regarding the effectiveness of raising antioxidant levels in the blood when using this product. 

N-acetylcysteine—powerful antioxidant.  The body uses NAC to synthesize glutathione.  I try to get Brady to take the effervescent kind from Bioadvantex, but he doesn’t like the taste right now so we are doing caps for a while.  NAC is a readily oxidized compound and pills often lose a lot of their potency by the time they are ingested. 

Albuterol—bronchodilator.  This medicine is used by a lot of people with asthma because it opens the airways.  Doing this medicine first allows all his other medicines to get deeper into his lungs. 

Hypertonic Saline—This is a 7% salt water solution (about twice as salty as seawater).  Depositing salt on the inside of the lungs draws water to the lung surface.  Hydrating lung mucus allows Brady to get a couple of good juicy coughs out and move some of that junk loose.  I’ve heard from adults that this is very irritating to inhale.  Sort of like sandpaper.  Brady doesn’t seem to mind. 

Pulmozyme—This is a super expensive inhaled breathing treatment that breaks up mucus in the lungs. 

thick skinned

It is fundraising season...probably the most heart wrenching time of year for a CF parent.  To put on the golf tournament that I hosted recently, I had to solicit silent auction items and business sponsorships from people and places in my community.  In total, I received a ton of support, but I was also faced with lots of rejection.  One woman yelled at me for even asking!  I approached the manager of a local garden nursery to see if she would donate a plant to my silent auction.  Her response was, "What is it with YOU people this year?!" "I've already donated to the school and I don't donate to churches and it seems like everyone WANTS something this year!"  I stood there sort of shocked for a second before I replied, "well, I don't have anything to do with any of THEM.  I'm just trying to save my son's life."  Then I walked to my car and cried.   The thing is, I learned very early that the CF Foundation would be having a huge impact on my life.  I feel it is in the best interests of my son, and everyone with CF, that I devote the rest of my life to supporting them.  Brady was diagnosed early because of their work. He has the benefit of preventative medicines because of their work. His illness will soon be largely controlled by an upcoming pharmaceutical(VX-770) because of their work.  No job or project has ever come close to motivating me to work the way fighting CF has.  So I allow myself to sob in my car for a while...then I have to shake it off and keep going.

When I was just beginning my chemistry studies in 1996, scientists were stumbling upon the first Vertex compounds VX-770 and VX-809 in a lab.  A series of events and investments followed those discoveries to bring us to where we stand today...at the door of the FDA with a groundbreaking new treatment not only for CF, but for all genetic diseases.  The fact that Brady is part of the tiny population(1200-1500 people is what I have heard) that will be able to benefit from this drug right away, is something that I give thanks for every day.  It is not lost upon me for a single second that when VX-770 arrives to help Brady, thousands of others with CF will still be suffering.  Bittersweet.  I would be lying if I claimed that I wasn't so involved in supporting the CFF for completely personal reasons.  I NEED my baby to be alive.  At the same time, I feel a connection to this community that is more powerful and compelling than anything I've ever experienced.  I love several of the people, especially other moms,  that I have met through facebook etc... that make me feel like I am not alone.  For those of us that live with CF every day, I don't need to explain to you how much the support from friends, family, and community at Great Strides time means.  People frequently tell me that I seem "dedicated" to supporting the CFF.  My response is always, "there is a fine line between dedication and desperation." 

I am really just a mother, who is not about to stand idly by while their child is destroyed.  I remember the day I learned that Brady had CF as the worst day of my life...but it is also the day that things came into focus.  Absolutely nothing has changed my personality or outlook on life the way dealing with CF has.  Before CF I based my life on "plans."  The life I have today is certainly not the one I had planned, but I feel more focused and determined than I ever have before.  At this point, I see life as the wildest ride I could ever imagine and I am just trying to hold on.  When I decided that fundraising for the CFF would become my mission, I basically committed to sharing the journey with everyone in my path.  People don't want to donate to a disease that they know nothing about.  Be warned that sharing your CF story can be a painful road that will leave you vulnerable, wounded, and frequently sobbing in your car.  You have to place your battered heart out there constantly and hope that the reaction you get doesn't just finish you off.  I can't begin to explain how important it is to me that people stand up and get behind me when I do an event like the "2nd Annual On Par for a Cure Golf Scramble," or the Great Strides Walk.  The money contributed to the CFF is going directly to the research that is controlling CF.  That research could be drastically changing our lives within the next year.  How much closer to the action can I get?  I must remain focused.  For me, fundraising is one of the only things that feels really good, like I'm doing something to help.  I would be lying if I said that I don't take it personally if my friends and family support the CFF.  If you aren't interested in helping me keep my child alive, well, I'm not sure we have much to talk about.  Is that fair?  No. That is sort of like saddling the rest of the people that love me and Brady with CF too.   But is it fair to try to pretend like I don't mind if people who claim to be my friends are suddenly too busy and broke at Great Strides time to contribute?   Hell no.  The people that attend Great Strides are more important to me than those who attended my wedding.  I don't know how else to put it. 

I feel an enormous responsibility to repay the work that the CF Foundation has already done and make sure that everyone suffering from CF gets to benefit from a genetic modifier like VX-770.  It is emotionally draining to be so angry at CF and so ecstatic about VX-770 at the same time.  I know a local man who underwent a double lung transplant a week ago because of his CF.  He very nearly died. The clock never stops ticking when CF is in your life.   Every day I tell myself that once Brady has his new medicine, I will have so much more time and energy to devote to fundraising.  It is a surreal feeling to know his medicine is coming so soon.  I feel "guilty" being one of the first CF moms to be staring this breakthrough in the face.  Why are we so lucky?  We haven't paid our dues the way so many other families have.  God, I will try to pay it back.  I will never stop fighting.  As much as I hate it, I will keep approaching everyone I know (and don't know) to ask for help to cure CF.  I absolutely despise asking for money and sometimes I swear I would curl up and die if the person I beared my soul to decided to reject me.   

So thank you friends.  Thank you family.  Thank you dozens of businesses that donated to my golf tournament.  Thank you stranger who decides to volunteer.  Because of you, I won't go completely crazy today.  Because of you I will put my old beat up heart out there again tomorrow.  and thank you also to the mean lady that yelled at me for asking for her help.  Because of you, my skin is thicker and I will try to never allow "no" to get me down.  What an extraordinarily lucky life I live. Who knew that giving your heart an absolute beating could be so rewarding at the same time?

Little Tweaks

When you are dealing with cystic fibrosis, bacteria and viruses suddenly take on a whole new significance.  I know that fighting germs is a losing battle.  No matter what you do, people with CF will acquire infections and viruses.  Still, because I am Brady's mom, I have to do everything I can to minimize their devastating effects on his little body.  Think back to a 7th grade science class, when you were learning about different types of cells in the body.  People with CF have dysfunction in their epithelial cells (mucous membrane cells).  Normally, there is a fairly thick layer of water on the surface of these cells, creating an environment for cilia(the little hair like projections on the cell) to sway freely in the liquid, sweeping away any foreign bacteria or particles out of the lung.

People with CF have a salt imbalance in these cells, which creates a water shortage on the surface.  Instead of swaying freely in the liquid, the cilia are bogged down in a thick sticky mucus, as can be seen below in the image on the right.  The lungs become unable to clear normally occuring bacteria like staph, or pseudomonas aeriginosa out of the lungs and chronic infections develop.  Over time, it is these infections, and the inflammation and scar tissue that they cause that lead to respiratory failure for CF patients.  The introduction of a respiratory virus almost always complicates issues further. 

Anything that causes further impairment of the respiratory system, and creates even more thick mucus becomes a recipe for worsening lung infection.  It is not an easy job to fight an enemy that you will never defeat.  Bacteria and viruses are everwhere.  I realize that I will never be able to control Brady's environment completely, but I feel that it is my job to give him the healthiest environment possible for his lung health.  We have made some little tweaks to the way we do things around our house and I think they have gone a long way toward keeping Brady healthy.  It is tough to really put these things out there because I will inevitably be judged.  I realize I am paranoid.  I realize that the way I think is not "normal"...and I don't care.  If any of my other CF moms take away a few tips, then it is totally worth it. 

1) Hand Hygeine.
 This is the obvious choice for my #1 tip, and the importance cannot be stressed enough.  I can tell you with 100% certainty, that paying close attention to hand hygeine works to prevent illness.  I have been sick less than once a year since I had Brady because I started paying attention.  That is a significant decrease for me.   I preach thorough hand washing, use of hand sanitizers and antibacterial hand wipes, and ALWAYS USE PAPER TOWELS TO DRY HANDS!  I know that tip is not going to be popular with the environmentalists, and I try to compensate in other ways as much as I can, but the action (actual friction) of using a paper towel to wipe your hands dry removes the greatest amount of bacteria and viruses from your skin.  Discarding the towel eliminates the chance for spreading.  I installed paper towel dispensers in my kitchen and bathroom and I ask everyone that comes into my house to wash or sanitize.  A company called Brick House makes paper towel dispensers that don't look so industrial and come in many colors: http://www.brickhousedispensers.com/.  I get boxes of multi-fold paper towel refills from Costco.  I don't even own hand towels anymore.  They remain damp for long periods of time and can be an ideal breeding ground for bugs.  Do I take Brady places and let him touch things...absolutely!  I just santize his hands afterword.  Does he play with other kids and at playgrounds etc...Yes!  But he can expect a bath when he gets home.  This behavior has been ground into him practically from birth.  He knows exactly what to do with "hanitizer."

2) Clean with killing on your mind.
Cleaning your house takes on new significance when you have a child with CF.  It become less about tidying up and more about killing.  You have to pay close attention to the products that you are buying, making sure they kill bacteria and which kinds.  Use cleaner in the appropriate strength and for the appropriate length of time.  Using diluted solutions or rinsing off early has actually been shown to promote bacterial resistance and is a big no-no.  I actually hate most of the best killing products, but they have a harsh smell and are very irritating.  There are some great natural products that work just as well.  I like Seventh Generation: http://www.seventhgeneration.com/Disinfectants?sub-cat=botanical-disinfectants.  We have hardwood and ceramic tile flooring in the upstairs of our house.  We clean our floors weekly with our trusty Steam Shark.  I love it because it cleans and disenfects with water.  No stink and no chemicals. 

I am also a big fan of cleaning with UVC technology.  UVC is a wavelength of light that has been implemented in appliances like vacuums, air purifiers, sanitizing wands, etc...  Exposure to this light kills bacteria, viruses, mold spores, and dust mites in seconds.  It leaves behind no chemical residue, has no smell, and can be used on virtually any surface.  There are some very convincing studies on the efficacy of UVC light in fighting bacteria and the technology has been used in the healthcare field for years:
http://www.esmagazine.com/Articles/Feature_Article/d843d8e109da8010VgnVCM100000f932a8c0____ .  Here is a little video I made about it:  Since I made this video, we have installed another UVC product in our home--a whole house air sanitizer by UV-aire mounted in our heating/cooling ductwork.
http://www.ductdusters.com/uvair.htm

3. Get rid of bacterial traps in your home.
Cleaning sinks and toilets is a no-brainer...but unexpected things can be dangerous for CFers in the home.  For example, this article discusses how harmful bacteria can lurk in your showerhead: http://healthmad.com/conditions-and-diseases/shower-heads-harbour-dangerous-bacteria/
When we finish showering, we leave the shower head dangling down so all the water runs out of the hose to keep the insides as dry as possible.  Every few weeks we soak the shower head in bleach water. 

Houseplants can also be a culprit.  The moist soil can grow nasty bugs.  Fortunately, I had already "taken care" of most of my houseplants by killing them with a lack of water ;).  Now, I just keep them out of Brady's reach, or skip them altogether. 

We have a dog in our home and I try to keep his bacterial contribution to a minimum.  First of all, he is a tiny, hypo-allergenic Yorkie.  I bathe him weekly and keep his food/water dishes and dog bed clean with UVC light and frequent washings.  Fishtanks are known to grow some nasty things, so we don't have one. 

As part of our cleaning regime, we also wipe down most hard surfaces and electronics in our home at least weekly with a disinfecting wipe.  I'm talking about: remote controls, light switches, door knobs, computer keyboards, cell phones, refrigerator door handle, buttons on microwave, kitchen chairs, you get the picture.  We also do countertops, tables, and wooden furniture with a disinfecting wipe and use the UVC wand on the couch, toys, and dog bed.

Finally, I wash all of Brady's clothes in HOT water.  I buy his clothes a little big because I know they will shrink.  If it can't be washed in hot water, I usually don't buy it.  I also wash all towels, rugs, blankets, and underwear in hot water.   http://abcnews.go.com/Health/Wellness/washing-machines-loaded-bacteria-dirty-clothes/story?id=10751420  Once again, the laundry is a place where things can be damp for long periods of time.  Make sure to switch laundry over to dryer promptly and dry until thoroughly dry on high heat. 

4.Avoid bacterial traps in public.
Here is where things can get tricky.  What can you do to fight germs when you are not in your own home?  A lot.  First of all, I always carry disinfecting wipes, hand sanitizer, and antibacterial hand wipes in my purse.  Most grocery stores carry disinfecting wipes at the door, but I carry my own...just in case.  This article refers to shopping carts as, "rolling fecal bacteria Petri dishes."  Enough said.  http://articles.nydailynews.com/2011-03-08/entertainment/28686369_1_carts-bacteria-flu-bug
I also carry my own pen and keep it handing to avoid using the one everyone has been touching all day.  I will turn around and walk the other way if I hear someone coughing, and I think my head would explode if someone sneezed in my face. 

My most important piece of advice about avoiding public germ traps is TIMING.  I try to run my errands and make my appointments early in the day.  I take Brady to places like the library first thing on Monday morning (after a weekend of no kids and probably a cleaning).  I do my grocery shopping on Friday or Saturday night, or super early in the morning.  The store is usually empty then.  Making small tweaks to scheduling of your outings can greatly reduce the number of germs you come into contact during cold and flu season, when it seems like everyone around you is ill.

5. Limit exposure... to an extent.
When you are hit with a virus and you have CF, treatment time often doubles, reaching 3-4 hours a day.  It is not uncommon for people with CF to be hospitalized several times a year for IV antibiotics resulting from exacerbations from viruses.  For these reasons, I avoid people that are obviously sick and will steer Brady clear of anyone that is snotty or coughing. End of story.  All of our friends know about Brady's CF and avoid us if they are sick.  When we take Brady to the CF clinic or pediatrician's office, I take the first appointment of the morning, to minimize exposure.  If Brock or I get sick, we are quarantined to the basement of our split-level house to try to prevent spreading germs to Brady.

6. Take care of yourself.
I have no business talking about this subject because I am not very good at it.  The thing is, to keep your child healthy, you must remain healthy yourself.  That means that no matter how depressed or frustrated you are, you must continue to eat and sleep and try to exercise once in a while.  When you get stressed or sleep deprived, your immune system suffers.  Coffee is not a food.  When you eat properly, your immune system functions better.  The only reason that I care remotely about this is because I am one of Brady's main sources of exposure to bugs.  If Brock or I wind up sick, chances are much better that Brady will get it.  Also, we ask so much of Brady to remain healthy.  I need to be a reasonably good example for him of show him that everyone has to work a little to stay healthy. 

These little tweaks make us a little different from some families...and that suits us just fine.  Thinking this way doesn't just happen overnight, Brock and I have been in training for 3 1/2 years now.  Most of these things are changes that we have made, as parents.  Brady has absolutely no ill feelings about using hand sanitizer and will grow up thinking that cleaning the house top-to-bottom every Saturday morning is just something that all families do.  My husband has been supportive of all of these changes and is an absolute cleaning machine.  Nothing is sexier than seeing him hike up the legs of his sweat pants and fire up the Steam Shark.  I know...I'm lucky.

Are you going to watch or are you going to play?

     People with CF are always being told what they can't do.  Adults with CF say that when they were small, their parents were told by doctors to, "enjoy them while you can...because they probably won't live past 8 (or some other ridiculously low arbitrary number)."  Then when the child's 8th birthday would roll around, they would say something like..."maybe he will make it to see 12."  When kids with CF began living long enough to graduate from high school, they had some decisions to make: do I keep listening to this BS and sit around and wait to die?  or do I go about my life as if I AM LIVING and I'm going to continue to live?

     Parents of children with CF have to make their own tough decisions right out of the chute: How are we going to incorporate cystic fibrosis into our lives?  What is our new "normal" going to look like?  Some families keep the illness to themselves, and try to make cystic fibrosis on the sidelines of their lives as much as possible.  I met one family at a CF parent education night organized by our clinic, that wasn't aware that The Cystic Fibrosis Foundation operated a website!  These families seem to strive for the traditional definition of "normal."  They do not want cystic fibrosis to change them or their lifestyle. In my opinion, they seem to often have a more peaceful acceptance of the disease. Then there are the families that fail to accept that fate...

     Cystic fibrosis has tormented me relentlessly since the day I heard those awful words.  For almost 9 months after Brady was born, I was sort of in shock.  I could barely say the words "cystic fibrosis" without breaking down.   Then one day I learned that our State was considering legislation to cut a program that provided benefits to adults with CF, to balance our budget.  Brady would not be directly affected, but he was on a similar state operated program for children with CF.  Would it be cut next?  Something inside me snapped.  I suddenly realized that other people--crooked politician type people--were going to be making decisions regarding my child's health and access to medical care and treatments.  Most of them didn't even know what cystic fibrosis was.  I woke up from my CF coma and started writing letters to Senators, House Reps, and the Governor.  I even did a TV interview for our local news. The politicians were claiming that pharmaceutical companies would step forward and pay the cost of the super expensive CF meds that the patients would no longer be able to afford.  I strongly disagreed.  No matter how much I hated talking about CF, I realized that I had to...and loudly!  I had to be an advocate for Brady.  The adult CF program was actually saved that year ('09), only to be cut one year later.  Adults with CF in Idaho suffered greatly because of the budget cuts and I was devastated. My news bit can be viewed here:
http://www.krem.com/news/local/62685687.html

     Keeping me going was the fact that VX-770 was progressing through clinical trials and beginning to look very promising.  The non-profit Cystic Fibrosis Foundation was funding the research and I knew it relied on donations to keep investing in these types of drugs.  I realized that curing this disease was going to boil down to money.  Raising awareness about CF and working to support the Cystic Fibrosis Foundation became my mission.  I can't let my son die because of something as stupid as money.  Brock and I decided that we weren't going to stand on the sidelines.  We want to be in the game. We want to fight.  There is no price tag that could be put on the gift that they are giving us by bringing this miraculous treatment to market.  How can we ever repay that?--keep fighting. Every morning I am on pins and needles as I turn on my computer.  Recenly,  I've been seeing headlines that turn my world upside down!  From what I hear, things are going to be very different for our family when Brady is able to take VX-770.  As I watch this miracle unfold, certain people flash in my mind that have told me things that Brady CAN'T do. 
*The first contact I had with another CF mom was over the phone and she was very "gloom and doom."  She made it seem like CF only had one horrible inevitable fate.  It was traumatizing and I haven't spoken to her since.
*The respiratory therapist that prepared us for doing breathing treatments for the rest of Brady's life.  It felt like we were being sentenced.
*The Hill-Rom Vest representative that mentioned to me that, "it would be something he would need for the rest of his life."   

     I guess what I want to drive home is that I have new-found belief in the idea that anything is possible.  When Brady gets all fixed up, I am going to call that grumpy mom back and thank her for being such a poor example for me...because it made me want to do such a better job for other moms reaching out for some hope.  It has helped me form some of my most cherished relationships, with people that understand exactly what I am going through.  Then, when it is time, I am going to demolish Brady's compressor and give his Vest machine to someone who needs it...and smile smile smile. And keep fighting. 
"Those who say it can't be done should not get in the way of those doing it."
Chinese Proverb

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Brady's guts

I let out a huge sigh as I sat down to write this because feeding Brady has been the most exhausting endeavor I've ever undertaken.  The thick mucus caused by cystic fibrosis clogged the ducts in Brady's pancreas sometime between 2 and 3 weeks of age.  He lost the ability to metabolize food on his own because the pancreas typically releases digestive enzymes into the small intestine, to allow the body to break down food and absorb calories and nutrients.  Shortly after Brady's first positive screen for CF, he began to have some troubling symptoms.  He was tired, and had stopped gaining weight.  He was also pooping every single time I fed him, which was every few hours since he was small and losing weight.  A few more days past and he started having the weirdest blueish green poops constantly.  I will never forget the visit we had with the pediatrician that day...The results of the genetic blood test had not come back yet, but the Dr. suggested we start him on enzymes(to me this sounded like admitting he has CF and I was totally against it.)  After she explained to me that if he didn't have CF, there were no negative side effects, we decided to try.  I hoped so hard that the enzymes didn't make a bit of difference.  Isn't that twisted?  But they did help.  A lot. 

This is how we fed him the enzymes as a baby

Brady has had to take enzyme pills/beads since he was diagnosed, with every single meal or snack.  When he was tiny, we opened the capsules and sprinkled the beads on applesauce to get them down.  He learned how to swallow pills whole right when he turned 2.  He has been an expert at swallowing horse pills ever since.  I never leave the house without enzymes.  In fact, if a cop ever had to search my purse or car for some reason...he would definitely think I was a dealer.  I have little bags of pills stashed everywhere!  Currently, Brady takes about 3 enzyme pills (Zenpep 10) with each meal, or about 12 a day.  I have to adjust his dosage based on a number of factors: how much fat the meal has(more fat=more pills), and how slow Brady eats(the enzymes last for 45 min or so, and Brady is the slowest chewer in the history of mankind.)  I try to get him to eat a full meal at designated meal times, and I am a huge stickler for keeping to our routine.    Brady has always been considered "severely pancreatic insufficient" by his Dr. and actually takes a second prescription (Prevacid) to help the enzymes work better.   He also takes special vitamins with mega-doses of the fat-soluble vitamins A,D,E, and K.  If he didn't take the enzymes and vitamins...he would become malnourished, no matter how much he ate.  From what I have heard from adults, the stomach/digestive pain associated with the disease can get pretty severe.  Perhaps that is why Brady has never had any appetite to speak of from the day he was born.  I think I have heard him say he was hungry a total of about 3 times.  When kids with CF fail to gain weight properly, it is not uncommon to surgically place a feeding tube into their stomach so they can be given a special "formula"(like extra strength boost or ensure + enzymes) through the night.  I've always been terrified of that and it is has been a constant struggle to keep his weight up. 

Some of Brady's pills and supplements

 I heard an adult with CF speak the other day and he compared staying healthy with CF to training as an elite athlete.  Your job becomes making sure you are doing everything you can to help your body work at its optimum performance.  You have to be constantly aware of how much sleep you get, how many calories you eat, and how many workouts you get it...no matter how you feel.  I typically cook special high calorie meals for Brady.  Either Brock or I sit down at the table for a meal with him 4 times a day.  Mealtime also means swallowing about 5 pills and several supplements, so we can't just turn him loose yet or half of his pills would probably end up in the dog's mouth!  I feel so bad poking food down his throat when I think his stomach hurts...but he has to have the calories.  Also, many of the pills/medicines that he takes have potential side effects of "upset stomach, nausea, diarrhea, etc..." so I can only imagine what his poor gut feels like.  In addition to the problems with his pancreas, his intestines are also affected by the CF mucus.  I saw some funky things in Brady's diaper before he was potty trained.  "The snotty diapers" were a real pleasure to change ;)  All that digestive dysfunction gives Brady what I call "the power of stink."  When you don't digest food very well, and your system is filled with mucus...it apparently makes the stinkiest poops and farts on the planet.  I can only hope that, as a teenage boy, Brady uses these powers for good rather than evil!  He will likely be regarded as some sort of hero among his peers for his ability to clear a room! 

At Brady's last clinic visit, he surpassed the 50th percentile for weight for the first time in his life.  I hear that most parents worry about their kids being good at sports, smart in school, and well liked by their friends.  All I worry about is keeping Brady growing and breathing...just growing and breathing.  It is actually a bit of a luxury to never sweat the small stuff.