Wednesday, March 28, 2012

Tough Decisions

So we are facing a tough decision right now.  First of all, as some may or may not know Macy was born with hip dysplasia.  It is something that is common with FA kids.  When she was 6 weeks old she had to wear a hip brace (palvik harness) for 2 months.  Below is the picture of us getting home after getting it put on...let's just say she wasn't exactly thrilled to have her legs pulled up into a "frog" position for 2 months.  We were allowed to have it off for 1 hour a day to bathe her and that was it.  That 2 months was over Thanksgiving and Christmas as well.  A Palvik harness doesn't exactly make for the most traditional looking Christmas photo op!  But, we were happy it didn't have to be on for longer than 2 months.  Especially since I was busy doing neck stretches daily with her as well.  She also had muscular torticolis on the right side which often goes hand in hand with hip dysplasia.  Anywho, here's the pic.
After she got it off she had an x-ray done when she was 7 months old (2 weeks before her FA diagnosis).  They said her hips looked great.  They were corrected and all was well.  Well...apparently that's not entirely the case.  

She had a 1 year follow up scheduled so I called to see if it was really necessary to keep following up with Ortho since her hips were corrected.  When your child sees 10+ Doctors you want to get rid of as many as you can; but only if their not necessary anymore.  I always joke that once you see a Specailist you can never get rid of them.  They will try seeing you for "follow-up's" for the rest of your life if they can.  Sometimes it's necessary, but sometimes not.  So that was last week I spoke to them and they told me that actually she is missing an ossific nuclei on her right femur.  Basically that is what keeps the end of the bone in the hip socket.  They recommended an annual xray, because they said the end of the femur can flatten and cause hip dislocation.  Her hips can become painful and then could need hip surgery.  UGH!  Why didn't they say anything a year ago?

So the problem is...xray's are dangerous for kids with FA because of the radiation involved.  Macy had numerous xrays before she was diagnosed with FA so doing them annually now makes me cringe.  

I basically conceptualize Macy and children with FA (and everyone else too) as having an "empty cup" when they are born.  When the cup gets full that is when they get cancer.  Those with FA lack the proteins to repair DNA damage from radiation and all other toxins from the environment and everything else; so their cups fill so much faster than a non-FA person.  Non-FA bodies can drain the cup by fixing the damage, hers cannot.  So most (not all) non-FA people if they get cancer it is later in life; 50's, 60's, etc. but those with FA it is single digits, teens, and 20's (if they survive transplant).  So by allowing xrays we are just filling her cup even faster, and that really bothers me.  But on the other hand we need to keep an eye on her hips.  And if an xray is the only way; we are stuck in a difficult place.  I hate making these decisions.

Her hematologist always says we need to weigh the cost vs. benefit.  Which is true; but it is not always easy to tell which one outweighs the other.  Everything is black and white when it comes to FA. 

As we move about life I always have Macy's invisible "cup" in the back of my mind.  Constantly weighing out cost vs. benefit of everything we do.  Going outside in the sun her cup fills a little more; but the benefit of all the fun we have outside outweighs the sun's exposure.  Running to the store for a minute and forgetting to put her chemical-free natural sunscreen on her so I have to use "regular" and her cup fills a little more than if I had remembered.  Getting into an elevator with someone that smells of cigarette smoke instead of waiting for the next because we are late to her appointment...her cup fills a little more.  Letting her live as normal of a life as possible all the while trying to prevent her cup from filling as much as I can.  And trying to do this as "invisibly" as possible so it's natural and not noticeable to Macy.  It's mentally exhausting; but she is totally worth it.  As time goes on I am sure it will become second nature.  And when Macy gets old enough it will be her decision to make.

Macy's appointment with Orthopedics is in the beginning of May.  The specialist she saw before moved to Wisconsin so this will be someone new.  I want to see if an MRI (which does not have radiation) will be able to see what we need to see.  She will need to have an IV and be put under anesthesia for an MRI which means more pokes...but she has to have an annual bone marrow biopsy every year to check for pre-leukemia, etc. which she is put under anesthesia for anyways.  So I want to see if they can just do them at the same time.  If they can see what they need with an MRI; I'm not taking 'no' for an answer.

Macy has an appointment with an Audiologist next week.  Let's hope her hearing comes back good!

Almost vacation time....counting the days.

take care,


  1. Jenn don't be too proud to ask for help if you need it. We're a big family and even though we don't see each other much, we come together when needed. I pray for Macy everyday and for you and your husband. She is such a beauty. Love her smile

  2. Thank you. She does have quite the contagious smile! She's a great kiddo! Almost vacation time. We'll be sure to take lots of pictures to share. Take care. And thank you for the prayers; they are much appreciated.