Adventures with Superkid

If you've recently stumbled upon my blog, you might have come for some of the sewing or craft ideas I've been posting (my goodness, I'm getting full of myself, aren't I?).  It's only been in the last few months that I've had the time, energy, or inclination to do that kind of stuff, and it is fun to share that on my blog.  My blog actually began as an extension of the CarePages I was keeping for my two daughters, Superkid and Lily.  (If you want to know about CarePages, click here.)  Today's post is an update on Superkid and some of her ongoing health challenges.....so no crafts today!  (But I did make that twirly skirt she's wearing.)

Some of you are aware that Superkid was born with a heart defect called mitral valve stenosis.  Her own mitral valve only worked for about a year, by that time it was causing severe enough heart failure that it had to be replaced by an prosthetic (artificial) valve during open heart surgery.  Superkid's new valve puts her at risk for blood clots, so she takes a blood-thinning medication called Coumadin.  (You can read more about that here.)  The other tricky thing about prosthetic valves is that they don't grow.  Superkid's prosthetic valve has to be replaced as her heart outgrows it.  She received her second valve in December, 2008.  It looks like this:

As if that weren't enough of a challenge for one little person, Superkid has had to deal with several others, as well.  

Her heart failure was severe enough prior to her first surgery that she had to have a feeding tube that went directly into her intestines.  We used this at the time of her initial heart surgery to help provide her with additional nutrition and calories that she didn't have the energy to ingest by mouth, both pre and post op.  We used it for several months after surgery to help her get extra calories she needed to catch up.  We also used it for a couple of years following surgery to administer her heart meds, until she could handle taking them by mouth.  All that remains of her feeding tube now is a small, closed hole on her tummy, which Superkid refers to as "my other belly button."  Up until last year, Superkid was followed by a gastroenterologist for issues related to the feeding tube and what was thought to be reflux.  (Turns out what everyone thought was reflux was actually discomfort that seems to stem from pressure changes in her heart and fluid issues.)

A few months after her first heart surgery, we started to notice that Superkid's eyes didn't always track together.  This was diagnosed as an eye-muscle disorder called intermittent exotropia.  Corrective lenses helped a little bit, but the only permanent solution for this problem is--you guessed it--surgery.  Taking a blood thinner makes even the most minor surgeries very complex, so Superkid's eye surgery in June of 2009 put us in the hospital for a good two weeks--longer than her open heart surgery six months prior.  To give you some perspective, the eye surgery that she had is normally an outpatient procedure.  We continue to see a pediatric opthamologist to monitor Superkid's eye muscles and vision.

And then there are the migraines.  Superkid gets migraines.  Severe headache can be a sign of a blood clot or hemmorrhage in the brain--two things that Superkid is at risk for.  She's had a litany of tests (from CT scans to sleep studies) with the conclusion being that Superkid gets migraines for no obvious reason.  They are awful for here, but fortunately they don't last more than four hours (so far).  We are getting better at determining what triggers them: chocolate, excessive juice, sugar, late nights, etc.  She only gets them in the morning, so at least they are predictable that way.  So, we started to work with a neurologist on those.  

Pneumonia is another issue.  If Sariah gets a cold, it more than likely turns into pneumonia.  I've become pretty reliable at knowing when the pneumonia is starting: sometimes I can catch it before there is even much indication of it in a chest x-ray.  She's never had to be hospitalized for pneumonia, although it has sent us to the ER a couple of times.  Anitibiotics and breathing treatments always clear it right up.  It's just that she gets it sooooo often: three times between the first day of school and the first of December, this last year.  This year, her pediatrician turned us over to a pulmonologist.  Everything that was easy to diagnose was ruled out: her pneumonia isn't caused by heart failure, lung damage, asthma, or any obvious immune disorders.  Our pediatric pulmonologist, at a loss for any easy explanation, is sending us on to a pediatric allergist, to see if undiagnosed allergies could be the cause of her frequent pneumonia.

In case you are having trouble keeping track of everyone, here is a list of all the medical specialties we've covered, so far:

Cardiology

Cardiothoracic Surgery

Pulmonology

Neurology

Gastroenterology

Allergy

Opthamology

Infectious Disease (did I mention that Superkid is MRSA positive?)

Nutrition (went with the feeding issues early on)

Anesthesiology

Radiology (for all our xrays and assorted other procedures)

and we're about to add

Psychology

and Child Development

because Superkid just isn't picking up reading the way I hoped she would.  The motivation and desire are there,  but Superkid's frustration is increasing exponentially as we near the end of kindergarten.  When she asked me one day, after a particularly difficult reading homework assignment, "Do the letters jump around on the page for every reader, or just me?" I could tell that it was time to get some extra help.  I've read about studies that show children who have been on heart/lung bypass have an increased rate of learning disorders, so we're going to have some educational testing done.  Whether or not they make a diagnosis, I'm hopeful that through the testing we'll get some tips for helping Superkid learn to read in a way that works for her and her unique learning style.

The other specialty that follows Superkid is 

genetics and metabolism.  

Ever since Superkids was little, and we were dealing with new diagnoses of eye muscles and reflux, I've been asking our doctors, "Is there something else going on?"  I was always told that this was typical for heart kids; many children with CHDs have accompanying other issues that most doctors blame on their hearts.  After all, inadequate blood flow or poor nutrition due to heart failure can cause so many other things to go wrong.  

When our daughter Lily was alive, she had a lot of feeding and digestive issues.  A very bright, caring third year resident was determined to find a way to improve Lily's digestion, so she called on the metabolic expert at our children's hospital, Dr. Brian Hainline, MD, PhD.  He special interests are Inborn metabolism errors, molecular genetics of fatty acid metabolism.   I've been told that Dr. Hainline is one of only about five experts in this particular area of medicine within the United States.  Don't ask me what any of that means; I already feel like I have to get a degree in Chemistry just to be able to converse with him.  Since our first meeting with Dr. Hainline, I've decided that he is one of the most caring, engaged physicians we've ever encountered--in his own, unique way.  Dr. Hainline was able to answer a lot of the questions that we had about Lily.  The answers weren't easy to get, but the knowledge made us better able to care for and comfort her during the remainder of her time with us.  

Dr. Hainline also told me that he'd like to meet Superkid.  He was the first doctor to agree with me that Superkid's various medical issues might not be heart related.  In fact, he believes that many of the children who have CHDs and accompanying health issues aren't necessarily correctly diagnosed: he believes many of them have overriding syndromes that caused their CHDs and the other problems, rather than blaming the CHD for everything.  The problem with this radical concept is that science has yet to discover most of those syndromes.  Dr. Hainline had been able to determine that Lily had some type of metabolic disorder but had not been able to pinpoint which one prior to her death.  He has been working with Superkid to discover if many of her health issues are a result of a metabolic or neuro/muscular disorder.  

We don't have any answers, yet.  Initial tests have been inconclusive.  After our last appointment with him, in December, Dr. Hainline said that he'd like to review how Superkid handled the winter.  Would she miss a lot of school?  (20 absences and 6 tardies, so far, due to illness.)  Would she continue to have pneumonia and migraines? (Yes.)  Would she have a growth spurt?  (Not a spurt, exactly.)  How would she do at school?  (Not as well as we hoped.)  These are the things he'll be reviewing as Dr. Hainline considers our next course of action.  And this will take some considering.  If Dr. Hainline feels that additional testing is needed to make a concrete diagnosis, that testing could get pretty invasive.  We're talking muscle biopsy in a patient on blood thinners.  Not fun.  Painful.  And it is doubtful whether or not an official diagnosis would be helpful.  Treatment for many metabolic disorders is somewhat behind the scientific community's ability to diagnose them.  It's a relatively new field of study.

So, that's where we're at: between a rock and a hard place.

It brings our list of specialists we've met up to 14.  Looking on the bright side, Superkid hasn't had to be treated by anyone in 

Dermatology

Endocrinology

Oncology

Nephrology

Orthopedics

Otolaryngology

Plastic Surgery

Rheumatology

or Urology.

Am I the only one who is disturbed that our list of specialists we've met is longer than our list of specialists we haven't met?  I'm afraid that I've reached a level of experience at which I am obligated to go back and get a nursing degree.  Seriously.

Well, if you've read this far, it's probably only because you are related to me or because you can relate due to your own ongoing medical challenges.  I have to tell you that the reason Superkid continues to consider all her doctor's appointments to be social calls and the reason I'm not yet completely insane is because we have such an amazing children's medical center.  It is staffed by some of the most caring, sensitive, all-around nice doctors, nurses, techs, and staff.  Yes, we've met a few people along the way who didn't make our list of faves, but they've been few and far between.  As homesick as I get for the mountains, I don't know how I'll ever be able to leave these wonderful people who have given our entire family such stellar care.  If anyone reading this needs a recommendation for any of the specialists we've seen and lives in the Midwest (or is motivated to travel to get care from one of them) feel free to leave a comment with your email address.