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

Hope For Brady at OneTrueMedia.com

Make video montages at www.OneTrueMedia.com

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. 

Noise and Expensive Machines

I am honestly so grateful to be sitting here today rehashing this, because I truly believe that one day it will all be part of our past and we will have a wonderful story to tell.   In my last entry, I wrote about incorporating manual airway clearance therapy into our lives.  This time, I'm going to cover the rocky start we had with the introduction of breathing treatments into Brady's regimen.  One of the toughest things about parenting a child with CF is trying to make the right health care decisions.  There are varying schools of thought on the best way to approach this.  Some parents adopt a "deal with problems as they arise" approach.  This can, in some ways, allow the child to live a more normal life by minimizing the amount of treatment time in their early years.  Brock and I are more inclined to put in the treatment time now and try to prevent the deterioration of his lungs as much as we can.  When Brady was 6 months old, we decided to start him on an inhaled drug called Pulmozyme.  Pulmozyme breaks up the mucus in the lungs, allowing for better mobility.  There is no clinic trial data for Pulmozyme in infants, so it is "at your own risk."  After doing our research, we decided to speak to Brady's Dr.  He was open to the idea, but left it totally up to us.

I asked, "Dr. M...Please just be honest with me...what would you do if it were your child?"
"If it were my child...I would have started them on it just after birth," he responded.

That is one of the many things I love about Brady's CF specialist--he will cut to the chase if you ask him and he respects a parent's right to participate in the treatment plan.  Three days later, a respiratory therapist came to our home with all our new equipment.  I was shocked at how loud the compressor was when it was turned it on for the first time.  We decided to wait until later that night to try the first treatment.  Brady sat on my lap and I held a tiny mask over his mouth and nose while a thick mist of medicine spewed out.  The first treatment did not go well.  At all.  Brady screamed, kicked and cried the whole time.  I cried.  Hell, I'm pretty sure we all cried.  For the next two weeks we would attempt again and again to give him the treatments we had CHOSEN to start.  Sometimes he cried so hard he puked.  The first two weeks of breathing treatments were pure hell, but failure was not an option.  It honestly felt like I was torturing my baby...but my gut told me he needed this medicine to stay healthy.  Then one day, just like magic, he realized that it really isn't scary and stopped crying.  He finally figured out we would let him watch whatever he wanted on TV during that time ;)

Breathing treatments have been a breeze ever since.  Currently, Brady inhales two medicines each morning (albuterol and 7% saline) and three each evening (albuterol, 7% saline, and pulmozyme).  When Brady turned 2, he was big enough for his Vest.  The Vest is a $16K machine that consists of a special compressor connected to an inflatable vest with hoses.  The vest inflates and oscillates Brady's chest wall at a particular frequency to loosen the mucus in his lungs.  Basically, it shakes the crap out of him for 20 minutes after he finishes his breathing treatments.  Brady's Vest machine is covered in dinosaurs.
The idea is to first loosen it up with drugs, and then shake it out.  Brady spends about 90 minutes a day doing treatments and vest.  The Vest is awesome for us.  No more pounding on the baby--thank you!  The vest shakes him hard and sounds sort of like a helicopter.  My mom actually cried the first time she saw him wear it because she thought it looked too rough.  Brock and I still sit with Brady through treatments, even though he does almost everything by himself.  For me, it is a time to snuggle him, sip my morning coffee, and be so thankful that these medicines exist to keep him breathing.  I will, however, really enjoy taking a baseball bat to that loud compressor one day when Brady doesn't need it anymore.  Since Brady began treatments so young, it is the only thing he has ever known.  In fact, I'm sure that one of these days he is going to realize that not all kids have to do this.  I talk openly about CF with him, but there is only so much that a 3 yr. old can comprehend.  If he is able to take the new drug VX-770 by age 5, I wonder what he will even remember about the treatments.  In some ways I hope he forgets.  In other ways, I really hope he remembers...because it will make all of us appreciate our lives more everyday.

Back To The Beginning: CF Sucks Part 1

Rewind 3 1/2 years.  Brock and I welcomed Brady to the world on July 26, 2007.  The pregnancy had been planned and we were thrilled to start a family of our own.  I remember being a little bit surprised when I first saw Brady.  He was beautiful and perfect...but he was so skinny.  I had been such a fat baby, I was just sure that I would have one too.  I brushed it off.  Brady was very fussy, but I never suspected that his pancreas was becoming clogged and his stomach probably hurt from digestive problems.  At Brady's 2 week appointment with the pediatrician, I heard the words "cystic fibrosis" mentioned for the first time.  Well, it may not have been the first time I had heard those words...but it was the first time they applied to my child.  The nurse came in and started to repeat the heel stick test they perform on newborn infants at the hospital.  I asked why she was doing it again and she casually said, "he tested positive on a screening test for cystic fibrosis so we need to repeat it."  Whoa.  Wait, what?  I was by myself at the appointment and still very sleep deprived.  At that time I didn't have any idea what the hell cystic fibrosis was.  It sounded bad.  The Pediatrician tried to answer all my questions and keep me calm, but the screening test was brand new in Idaho and she wasn't exactly sure what the results meant.  The freaking out begins here.  When the second test came back positive, I got the call that we needed to make an appointment for a sweat test at the hospital.  The sweat test is the gold standard diagnostic test for CF. In the meanwhile, Brady had begun to have some pretty serious digestive problems.  He had stopped gaining weight all together and his inability to digest food on its own started becoming apparent.  I started to have this horrible feeling in my heart.  I knew something was terribly wrong...but it couldn't be this.  They were unable to collect enough sweat off his skinny little body to get conclusive results on the test so we decided to run a genetic blood panel on him.  The results would take a couple of days.

Brock usually came home from work for lunch, but he walked in the door early that day...followed by my brother and sister-in-law.  I knew what he had to say to me the moment I saw him.  Brady was wearing a tiny yellow onesie with a duck on it.  That moment will be suspended in my mind for the rest of my life.  I think telling me that news was probably the hardest thing Brock has ever had to do.  All I really remember is hearing myself screaming.  I still wasn't sure what cystic fibrosis meant for us...but I knew that it had suddenly changed everything and that things were going to get hard.  I knew that CF was the #1 genetic killer of children.  I immediately began thinking about having to watch Brady suffer and potentially die.  That was the day that the words "cystic fibrosis" began assaulting my brain like a machine gun.

The next several months were very hard.   Managing Brady's medications and feedings became my full time job and there was a lot to learn.  Unfortunately, many of the things you discover about CF scare the shit out of you.  One of the first horrible things we learned was how to do percussion therapy on our baby (a.k.a. pounding/beating on the Brady's chest and back to loosen the mucous in his lungs). I'm trained in massage therapy, so I was familiar with the techniques already.  This would be a required therapy, indefinitely...or until he was big enough for a Vest.  I cannot describe how horrible it is to have to beat on your child...twice a day...for years.  Brady was actually fine with it.  He often fell asleep during the 15-20 minute sessions.  Brock and I hated it with a passion.  You have to pound hard.  I cried after every time for months. We both developed a form of "carpal tunnel therapy arm syndrome" from the repetitive action of the therapy. We did everything we could to make therapy good and fun and special, but it was still so hard.  I guess I feel that to understand why curing CF is so important...you must first understand why having CF is so crappy.  It has been such a humbling experience for me to see Brady breeze through the things that cause my heart to just collapse.  He is very special and very strong.  I am very lucky.

A Miracle and a Latte Please

This is my first blog entry.  It terrifies me, because getting personal about CF makes me want to barf.  The people reading this first entry likely know me, so I'm going to get right into it.  If you have spent more than 5 minutes with me, or read my posts on Facebook, you know my 3 1/2 yr. old son Brady was diagnosed with cystic fibrosis just after birth.  For me (completely type A personality) to feel so out of control of this disease has been emotionally devastating for me.  For about 9 months, I just didn't talk about it.  Obviously I've gotten over that!  I remember when I read about the first stage trials of VX-770.  I was already excited because it was a drug to treat the root cause of CF rather than the myriad of symptoms.  This drug is designed to treat a very small percentage of people with CF--those who carry the G551D mutation.  I had just learned Brady's mutations recently and had to pull out some paperwork to find them. I remembered it was a jumble of letters and numbers. Could he possibly be one of the 4% of the CF population that VX-770 might work for? YES!! Brady has the G551D and DF508 mutations.  When the phase II trials were published, I just about lost it!  I couldn't stop researching, calling, trying to find participants in the study.  A complete obsession.  How could it not be?  I am watching my son's own personal cure making its way to him.  In the meantime, he is staying amazingly healthy (fingers crossed!).  Phase III results were released on Feb. 23rd.  We knew the results were going to be published in "the first quarter of 2011" so we have been very anxious the last few weeks.  I turned on my computer and saw it immediately on Facebook.  I sputtered something urgent out to Brock and he hurried over.  "Click on it!!," he nudged me.  I had frozen, staring at the headline. I don't think I took a breath until I finished reading.  You know when you have one of those days that you immediately know you will never forget?  Brock and I both had tears streaming down our faces.  We tried to stay sort of quiet, so we wouldn't wake up Brady, but it was pretty intense.  I swear I felt a shift in the Universe.  Like, maybe it was the beginning of the end of this CF nightmare.  Then Brock went to work and I have been trying to function ever since.  I sort of swing between celebrating my ass off to being exhausted and super emotional.  No wonder Brock is ill!  It is just so surreal.  "Hey, how are you today?"  "Oh fine.  The specific strain of my son's rare genetic disease is getting fixed with a new medication soon and could you make my latte sugar-free please?" I thought that maybe if I typed some of these things out, that they would quit swimming around so much in my head.  How am I supposed to figure out what to make for dinner with this going on?! I LOVE that the rest of the world is finally starting to get excited about it!  I was getting really sick of getting that, "oh you poor delusional mommy" look when I talked about it before!  I have heard an estimate of 18 months until VX-770 hits the market.  I realize there are numerous variables, but if all goes well with the FDA, Brady could have this treatment the summer he turns 5, just before he goes to Kindergarten.  Are you starting to believe in my luck yet?  I'm not going to take a second of this for granted...