Thursday, March 31, 2011

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:

     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

Monday, March 21, 2011

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 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 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. 

Sunday, March 6, 2011

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.

Wednesday, March 2, 2011

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 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.