Monday, October 7, 2013

the other shoe

When Bailey was born, we thought she was "perfect."  I remember shortly after we brought her home, we needed to stay the night at a different hospital because her bilirubin level was still too high.  We brought her in and they couldn't get a vein anywhere but her sweet little round head.  My hormones must not have helped, but I cried like there was no tomorrow.  I felt helpless, scared and all I wanted to do was take my baby home and hold her in my arms, not sit next to an incubator.

Once again home, I counted my blessings to have a healthy, beautiful baby.  She ate, slept, grew... she really was "perfect."

When Bailey was a few weeks old, I remember laying on the floor in our condo living room, as she laid on her sheep pillow mat beside me.  The phone was right there, as I often talked to my mom.  Our pediatrician's office called, and told me Bailey's heel prick test came back "flagged" for CF.  I sat up, my heart tight, my stomach immediately sick.  What is CF?  Is that like Cerebral Palsy (CP)?  Is my baby ok, what is going on?  I had not even really registered that she had a heel prick test, as I wasn't even conscious when she was born...literally I had been under general anesthesia due to complications. So to say I hadn't even given it a thought is an understatement.

The days were a blur of doctor visits, panic, late night research on my computer.  I remember licking her trying to see if she did in fact taste like salt, as that is how they used to diagnosis CF.  I couldn't salty is a baby supposed to taste?!  Bailey had 3 sweat tests all yielded lower borderline results.  A sweat test is when they strap a band around your arm which sends electrodes through the skin to make it sweat, they then measure the salt content in the sweat and high salt is indicative of CF.  In the meantime Kevin and I got tested to see if we were both carriers, which we were.  Knowing our mutations then, we had them do a blood test on Bailey to see if she carried both those genes.

In early December 2003 we found out Bailey did in fact have CF.  I paced the condo crying out.  I held her tightly to me and begged God to please please please take it from her and make her healthy.  Please don't let her be sick, please don't let her die.  Please please please.  Please let her be perfect, this sweet little baby who doesn't deserve this.  Please.

CF is a disease that affects the lungs and surrounding organs.  It's progressive but usually right from the start a CF person displays symptoms.  At birth many CFers need surgery for bowel obstruction, as the mucus is already so thick in their body they can't pass their bowels.  Then, most CFers need to take enzymes each and every time they eat because the thick mucus surrounding their pancreas prevents their natural enzymes from breaking down their food so they can digest it.  Instead, food goes right through them and they suffer from severe malnutrition very quickly.   Many CFers are diagnosed from their failure to thrive as infants.  Even with these fake enzymes a CFer will have many gastro/digestion issues.  Then, they start harboring harmful bacteria in their lungs...the bacteria gets trapped in their tar pit mucus, instead of easily leaving like it does for us because we have thin spit around our lungs.  The bacteria leads to lung infections.  Over time a CF person's lungs get weaker and weaker and more inflamed from constantly trying to fight the bacteria, which of course grows resistant to antibiotics.

I spent those early diagnosis days reading all I could about CF.  And it scared the shit out of me.   But the days, weeks, months were passing and my girl didn't have so much as a cold.  As a preventative measure we went to CHOP every 2 months and did manual chest percussion therapy on her (bopping her with little cups to try and thin the mucus) and also gave her an inhaler treatment a couple times a day.  Now that was hard.  Thankfully, Kevin really helped with treatments because I just about couldn't take the way Bailey would hold her breath when her little mask would get put on her face and her eyes would flare, she'd kick her legs and I just knew she thought we were trying to suffocate her.  I'd sing to her trying to calm her as my tears fell right on her head.

Around her first birthday, I remember telling someone that she is just the BEST baby but I was in a constant state of anxiety because I kept waiting for the shoe to drop.  The CF shoe.  When my baby would suddenly start starving to death and not be able to breathe.

The years went on and we adjusted.  We did preventative care with her but really she was healthier than most "perfect" kids.  We treated her like any other child and went to Disney, waterparks, sat her on Santa's lap, let her play in a sandbox, went on hayrides in the fall.  Why wouldn't we?  Well many CF parents are very scared about these kinds of things, as there have been places that have been proven to grow certain harmful-to-CF bacteria.  Santa sees tons of kids in the winter = germs.  Hay has spores with bacteria.  Waterparks...where do I begin.  Not to mention many CFers are homeschooled and off Bailey went to public.

I never forgot Bailey had CF.  I knew it was in there.  But we enjoyed life and I took such comfort in her more mild mutation of CF, hoping maybe even she would always be mostly asymptomatic.

Last October her FEV1 dipped into the 90's.  FEV1 can go up to 150.  Bailey's baseline was always over 100.  The dip continued...continued...different antibiotics were tried and by then it was summer.   No cough, no sickly appearance.  She always improves over summer due to the ocean and increased swim time so come August we were all expecting to see over 100 again.  Down more.  Over the year she had decreased more than 10% of her lung function when a normal CF decline is 1-3%.  We found out she had finally cultured a dreaded CF bacteria.

The CF shoe dropped.

Since August we have been consumed with Bailey's CF.  More treatments, meds, vitamins, machinery.  CHOP visits.   On Friday we found out it wasn't enough.  Tomorrow Bailey checks in for a hospital stay to get powerful IV meds administered.  The meds will be a 2 week course...we're hoping at the end her FEV1 has shot up and if not they will try something else.  I PRAY this will work and I PRAY she has no ill side effects from these powerful drugs.  I am back to begging God, as I was at the time of diagnosis.  Please please please please please.

So no, I'm not handling it well.  I cry any time I'm alone, and sometimes when I'm not because I just can not help it.  But how is Bailey doing...well, she is scared.  She woke the other night and it was very hard getting her back to sleep. They tried to explain how the meds will be given through a PICC line in her body, which she will be sedated for when it is placed.  I think she is most nervous about that.  But thankfully she sees the good in most things and has planned for a special project she and I could do together.  Bailey is amazing.

Pray for us is your faith that is helping me have faith.  Much love and thanks to you.

1 comment:

  1. Oh, Brynn! I am crying reading this! How hard this must be for you and your family. Bailey sounds like such an amazing little girl. For her to have to go through this, and for you as her mother to see it all unfold, must be just beyond hard. I am thinking of you and wish with all my heart I could just hug you.