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.

Advocate

ad·vo·cate

  v. ad-vuh-keyt; n. ad-vuh-kit, -keyt] verb,-cat·ed, -cat·ing, noun

–verb (used with object)

1.

to speak or write in favor of; support or urge by argument; recommend publicly: He advocated higher salaries for teachers.

–noun

2.

a person who speaks or writes in support or defense of a person, cause, etc. (usually fol. by of): an advocate of peace.

3.

a person who pleads for or in behalf of another; intercessor.

4.

a person who pleads the cause of another in a court of law.

-from Dictionary.com

Excuse me for a moment while I climb atop my soapbox.

If you have ever loved someone, then you are aware that with love comes responsibility.  The responsibility to care for and protect the ones you love.  During the different times and seasons of love, sometimes this responsibility can be a heavy burden to bear.  Trying to care for and protect an adult suffering from the consequences of addiction is a very different experience than caring for and protecting a curious toddler who is constantly climbing and exploring.

I believe very strongly that part of this responsibility includes standing as an advocate for the ones you love.  As a mother, specifically, it is my responsibility to speak in support and defense of my children in regards to their needs.  While there are many social, clerical, and government organizations out there who share this mission, none of them love my children and know my children the way I do.  Therefore, none can be a better advocate for my children than I can.

Let me share two examples with you.  

Example A:  I had a conversation with a fellow mother who shared some of the frustrations and difficulties she was having with one of her children.  During the course of the conversation, we bounced ideas off of each other.  I voiced a few suggestions.  By the end of the conversation, the other mother had a plan of action.  She was ready to make phone calls, gather resources, find a helpful doctor.....she was ready to take action.  

Example B:  I had a conversation with another mother.  This mother also had frustrations and concerns about the behavior of one of her children.  I listened, and agreed that her situation was difficult.  I consoled her.  I voiced a few suggestions of things this mother could try and resources that she could seek in order to help her child.  For every suggestion I gave, the other mother had a reason she couldn't use it.  A book to read:  why should she read it knowing her husband wouldn't and then she'd be the only one using the disciplinary techniques?  A trip to the doctor: the child hated doctors and would scream the whole time, why bother?  A conference with the child's teacher: she just knew the teacher hated her child anyway, what good would that do?  By the end of the conversation, I felt as down in the dumps as my friend.  She thanked me for listening, and sighed, "I guess I'll just have to keep hoping and praying that my child will change."

Which of these mothers was ready to be an advocate for her child?

I hope you answered the first one.  Don't get me wrong, I believe in the power of prayer.  But I also believe that an integral part of faith is action.  Pray all you can for help as a mother, but for heaven's sake, jump up and DO!  

If you walked into the kitchen and saw your diapered darling climbing onto the top of the refrigerator, would you kneel in prayer and hope for the best?  Of course not!  You'd say that prayer as you rushed towards the refrigerator, arms outstretched, and you'd pull that baby down from his precarious perch and you'd tell him no and then you'd come up with some very creative way to keep that child from ever climbing up that refrigerator again.

I feel strongly that God has not left us to fend for ourselves.  He has given each of us gifts that give us insight and inspiration when we need them most.  We can access these gifts particularly in our roles as mothers.  Sometimes you just know that your child has a need that is not being met.  This is the time when you are called upon to use those God given gifts and act as an advocate for your child.

Talk to the teacher.  Go to the doctor.  Meet with the therapist.  Plead for the resources.  Join the support group.  Change the babysitter.    Look for the clues.  Research the diagnosis.  Bring concerns to the school board.  Learn the lingo.  Find the right doctor.  

These are the things that no one can do for your child better than you.

A fellow heart mom, Stefenie, shared a story on her blog she'd heard about another family.  Their son was born with a complex heart condition that eventually necessitated a heart transplant.  As a baby, however, he was misdiagnosed.  The original diagnosis?  Allergies.  Allergies.

Science is not perfect.  Doctors do the best they can, but they aren't able to spend 24-7 with each of their patients.  That's something only a mother can do.  And doctors don't have those God-given gifts that allow them to serve as the perfect advocate for each of their patients.  That's something else only a mother can do.

I'll admit, it took a while for me to step up to the plate and take on the role of advocate.  It's a skill that I'm still developing.  But I've improved.  

Before Superkid was born, I knew there was something about her that was different than my other children.  I was told, very clearly, that she would have physical challenges.  My husband received the same message.  We were so prepared for her arrival this way, that both of us were a little shocked when she received a 10 on her APGAR score, was handed to us pink and tiny and perfect, without any evidence of anything that set her apart from her healthy siblings.  For months, Superkid seemed fine--she just didn't get chubby the way my other babies had--but the warning I had been given was constantly there: something was different.  The pediatrician chalked it up to her having a different metabolism than her siblings......until her nine month well-child visit, when he realized that Superkid hadn't gained weight since her six month visit, and heard a faint heart murmur.

At that point, things happened very quickly, and my husband and I often look back on some of our initial experiences and decisions during the first few months of her diagnosis and second guess ourselves.  We think we could have been better advocates.  We sometimes wonder what effect different actions on our part would have had on Superkid's condition, and we've had to learn to forgive ourselves and recognize that we're all on a learning curve.  Some lessons have to be learned the hard way.

I guess my point is this:  if you have a genuine concern about one of your children, take action.  Pray for support and guidance and then get up and go find the help you need.  Don't wait for things to change or things to get worse or for someone else to come and diagnose and fix the problem.  Advocate.

The following video really doesn't illustrate how to be an advocate for your child.  I just feel like I'm supposed to post it on the blog, so humor me.  I came across it yesterday, while I was looking for another file.  It is 19 seconds of footage that my husband shot of Superkid and I, soon after she was diagnosed with her CHD.  At that time, we'd been trying to explain to our doctors brief "episodes" that Superkid was having.  They were very frightening to us, but never occurred in a doctor's office and the portable heart monitor Superkid wore for several days didn't detect any problems.  We shot this video to show to our doctors so that they could see what we were seeing at home.  As we later learned, the little noise Superkid was making is something called "grunting", a classic sign of respiratory distress and heart failure.  I believe that Superkid was admitted to the PICU of our children's hospital just hours after my husband filmed this.  If you ever see your child or another child breathing like this, you need to get them to a ER very quickly, and urge the staff there to take a chest xray to look for fluid in the lungs.  

Love: 400 Extra-Ordinary Days

At this time, three years ago, I was pregnant.  Expecting our fourth child.  We were thrilled to be having another baby.  We love babies, at our house.

During the twentieth week of pregnancy, my husband went with me to the obstetrician's office.  It was ultrasound day.  My husband was supposed to be on a business trip, but it was cancelled early that morning.  That was our first miracle.

The ultrasound showed that we were going to have a baby girl.

The doctor came in.  We smiled at her, and she didn't smile back.  Instead, she began to gently explain to us all the problems that our sweet baby's developing body already had.  It was a long list.  Two vessel umbilical cord.  Hydrocephalus.  Hypo-plastic left heart syndrome.  A possible fatal chromosome abnormality.

My husband gripped my hand.  We tried to ask questions.  We cried.  We already had one child with a heart defect--we had already experienced long days in the hospital and an uncertain future for one baby.  We had been confident that this baby--our last--was to be our miracle baby.  

We named our baby Lily, for the lilies of the field that Christ used so eloquently to teach his followers of our Father's love for His children.

We prayed.  We tried to make difficult decisions without the aid of concrete evidence and information.  We moved forward in faith, sometimes not sure if faith was enough.

Our Lily was born alive, another miracle.  She was tiny and perfect, without the facial deformities or extra fingers or toes or enlarged head that we had been prepared to love along with her.

I was able to see her, pink and soft and wiggly, before she was taken to the NICU.  In the NICU, she grew, her little body preparing for the first of three surgeries her heart needed to function.

For three weeks, we loved and fed and held our baby in the NICU, not knowing if her sweet, tiny body would be able to handle the complex surgery.  

It did.  Lily spent many days in the hospital.  What would be unusual for some families became the norm for us.  Picnics on the floor of a hospital room.  Walks through the halls with IV pump and monitor in tow.  Saying goodbye to one child so I could go home to do laundry for three others.

Things that others might take for granted, we celebrated.  Like Lily's first trip outside.  Wires, and tubes, and all.

Two months after her birth, we brought Lily home.  Another miracle.  One we hadn't dared to hope for.

The following days were not ever easy.  Caring for Lily at home, as joyful as it was, was also complex and exhausting.  She had frequent trips to doctors' offices, more surgery and more procedures, more hospital stays.  I learned to insert a feeding tube and carry a baby in a carseat, a preschooler, and an oxygen tank across a snowy parking lot and through the grocery store.  

We were so grateful for the people who stepped in to help us.  Family, friends, neighbors, doctors, nurses, therapists, strangers...all of them became part of the fabric of each miracle.

Lily's siblings could have been envious or resentful of all the time that it took their parents to care for their youngest sister.  But they weren't.  Their unconditional, unwavering love for Lily was nothing short of miraculous.  And they way she returned our love so enthusiastically was yet another miracle.

 

We had been told, when our doctors first diagnosed Lily's condition, that we should be prepared that our baby girl might not survive the pregnancy.  There were so many unknowns, so many steps into the dark. I realized, as my baby grew and kicked inside me, that each day she was with us was a miracle.  

There may be some skeptics in the world that say miracles have ceased. 

They have not.  I've seen 400.

Lily lived for 400 days.  

Many of those days were difficult, for her more than anyone.  

Many of those days were spent in a hospital.  But many of them weren't.  Despite the 200+ days Lily spent with her friends at the hospital, she managed to be at home for Halloween, Thanksgiving, Christmas, New Year's Eve, St. Patrick's Day, Easter, Memorial Day, and the Fourth of July.  She was at home for the birthdays of each of her family members.  She went on a family vacation.  She spent Valentine's Day and her own first birthday at--appropriately--the Heart Center.

More days have passed, now, since Lily returned to her Heavenly Father, than the 400 she spent on the earth.  Some days have been really hard.  We miss having Lily in our home and in our arms.  But we are convinced that we will be with Lily again one day, and we've learned that joy can still be present in our lives.  

I'm so grateful for each of the 400 extra-ordinary days that we spent with Lily.  

I'm also grateful for each day since.  Ordinary days that become extra-ordinary when I look at how far we've come, how far we have to go; how much joy we've tasted, and all the joy we have yet to taste; how much love we've felt and how much love we have yet to give.

This week is Congenital Heart Defect (CHD) Awareness Week.

This post has been linked to Tuesdays Unwrapped at Chatting with the Sky.

Soapbox

Good morning friends!

I'm going to speak to you from atop my soapbox today.  I promise to keep it short.  If you have absolutely no concern for the welfare of children with special medical needs, feel free to skip this post. 

If you have read my blog before, you know that I am the mother of two children born with congenital heart defects.  Li'l Angel's combination of defects were incompatible with life, so she only lived on this earth for a year.  Superkid is six years old, and lives and thrives today thanks to a prosthetic mitral valve, a blood thinning medication, our beloved team of medical providers, and many miracles.

Chances are that we are not the only family that you have heard of who has been affected by congenital heart defects (CHD).  You may not have realized, however, that congenital heart disease is this country’s number one birth defect and kills twice as many children as childhood cancer. 

Although many children--like my Superkid--now undergo successful heart repair surgeries, most will require special life-long care and face high risks of developing additional heart problems. Up until now there has been virtually no federal investment to address the research and education needs of the 1.8 million Americans now living with congenital heart disease.

One adult CHD survivor is Paul Cardall, a talented musician living in Salt Lake City, Utah.  Paul is a husaband and father who recently received a life-saving heart transplant at Primary Children's Hospital.  Paul is also the author of an inspiring blog, Living for Eden, and has become an activist in raising awareness about CHD and those who are affected by the #1 Birth Defect.  Today he posted information regarding his efforts to educate our country's lawmakers about a new bill coming before the Senate and House, The Congenital Futures Act. 

Paul Cardall meeting with Utah Senator Orrin Hatch.

Thanks to Paul for his courage and efforts to bring understanding to this overlooked issue. 

You can help families living with CHD by voicing your support for this bill to your own senators and state representatives.  By visiting the Adult Congenital Heart Association website, I was able to find the contact information for my own representatives and jet off emails to the three of them (there is a handy sample letter you can use to create yours) in less than thirty minutes.  I strongly encourage you to do the same. 

Please, please, help to provide a brighter future for the millions of children in America who are living with heart disease.  The risk factor for CHDs in any pregnancy is that 1/100 pregancies will result in a baby with a congenital heart defect.  With statistics that high, it is likely that if you aren't already the parent, grandparent, sibling, aunt, uncle, cousin, or friend of someone who has a congenital heart defect, then you will be someday.  I urge you to take a few minutes to voice your support to your legislators to improve the lives of children and adults with CHD.

Thank you!