"Don't Do My Nose"...(by Michael)

Just recently Ella was complaining of her nose hurting while taking her bath.  At the same time she was also having rough nights, one after another.  Her bipap machine was also going off throughout the night...much more than usual.

We put two-and-two together and figured out that her overnight mask was too tight.  For a few nights we experimented with different "tightnesses" until we found a happy medium.  So far things have been back to the normal nightly routines.  With one exception...

During the time before we figured out what was bothering Ella she would always say to me, as I was bathing her, to "not wash my nose when you wash my face".  Of course, I obliged.   Once we figured out the bipap mask issue I figured this request would stop, yet she continued to say the same thing night after night.

As time rolled along I gradually began "skimming" over her nose to make sure it was, in fact, getting clean...at least somewhat.  She never whinced but always prefaced the face washing with, "Daddy, don't wash my nose when you wash my face."

This has gone on for a good month or so.  I think now it is simply a matter of habit.  She tells me not to wash her nose, I say, "oh, ok...no problem."  She closes her eyes tight and scrunches up her mouth as to not let any soap enter either place and I proceed...I wash the sides of her face, wrap around the ears, clean her jawline and chin, and whip, ever so gently, over her cheeks and the sides of her nose while saying at the precise moment, "Ok...now we got it."  She waits patiently for the stream of shower water to wash over her face as I count to three and the ritual has ended.  Her nose is clean and she's none the wiser.

As I worked through this process one evening a realization washed over me.  I realized that I will be bathing my child, most likely, for the rest of her life.  This is not something that most parents can say.  At some point, quite naturally, the bathing becomes a personal responsibility.  The child grows and becomes independent.  

I thought about this briefly as I was washing Ella.  And I was awestruck at how much she trusts me as I moved her from one position to another, making sure not to miss any part of her in the cleansing of her body.  She "rolled" with me; she allowed me to adjust her accordingly; she put her trust solely in my knowledge of her physical limitations.  Through contractures, weakened joints, and "floppiness" we work together to make sure she is cleansed with dignity.

I've been bathing Ella since her birth.  I've watched, with a sad yet watchful eye, how SMA really does affect the human body.  I have also watched, with amazement and pride, how well my three-year old accepts and works with what she has.

"Don't do my nose" she says...     

The Same Breath of Thought...(by Michael)

I woke one morning this past week to read on my Facebook page that a young boy, a very young boy, died early that morning.  SMA took his life.  The post was a mixture of an author dazed and confused, angry and despondent.  

The young boy was the fifth child lost to SMA (that I know of) in as many days.  Five days.  Five children.  

The posts, when they appear, quickly fill with comments from all walks of life.  The thread grows longer every minute as more and more people chime in.  Many of whom have lost a child to SMA or some other disease.  Expressions of sorrow and support echo through the writings.  Sympathy and empathy abound.  

The SMA Community is a tightly knit community bound together unwillingly by the strings of a disease most had never heard of.  Yet bound together we are.  As I have written so many times before, we celebrate our children's triumphs together, extend a helping hand when possible to one another, and mourn at the loss of every person.  It's a community that hopes together while at the same time walks in disbelief of the "how's" and "why's" of life itself.

There is one aspect I have taken particular notice of as I clumsily walk this path of SMA.  I notice that every parent recognizes the amazing strength people with SMA possess.  The disease itself, by its very nature, is debilitating and as we know, deadly.  Yet children of all ages battle against it with pride, dignity, self-confidence, and grace.  Adults that survive their childhood exemplify what it means to be a survivor against the odds.  I feel that the spirit that resides in those afflicted with SMA is the spirit that drives the hope for a cure.  That spirit is strong.  That spirit is alive.  That spirit cannot be quelled.  

As we take the moments during our days and nights to think of those who have lost their earthly life to SMA, to think of those left behind in the wake of their death, we can also, in the same breath of thought, allow the undying spirit of our SMA warriors, each and every one of them of all ages, enter into our consciousness and fuel our hopes for the future of those whom still fight every single day.

Sometimes I'm Puzzled...(by Michael)

Sitting across from Ella while on the hardwood floor, her back resting against the wall behind her, I see my three-year old concentrating on a game.  Her focus is sharp, her intentions are calculated, her body unnaturally fighting her desire to manipulate not only herself, but the world around her.  

She sits in front of me, void of any braces, as she relies on her own devices to keep herself balanced. A considerable amount of energy on her part is used simply maintaining her sitting position as she plays the game in front of her.

The game is a wooden board covered in a strong but thin plastic.  Pathways are carved into the board, intertwining and intersecting, and at the ends of the pathways are circles (almost like cul-de-sacs) which are of various colors.  There are tiny magnetized balls that are colored the same as the pathways' end circles, and a magic magnetic wand is roped to the board.  The object: using the magic magnetic wand one must move the little colored magnetic balls to their rightful colored circle-spot.  Patience and dexterity are required.

The board, at first, rested on her lap.  This presented a problem, though.  Every time she shifted her weight the little balls would roll out of the places she wanted them to stay. 

We spread out her feet and placed the board on the floor, in between her legs.  Now it was stable.  But now she had to grapple with gravity as she needed to reach across the board to move the tiny balls around.  

I watched.  I coached.  I helped just a little.  She did wonderfully.  Challenging her own comfort level, reaching farther and crossing mid-line.  Leaning on her one arm in an appropriate position and then switching; transferring the magic magnetic wand from one hand to the other.  

A simple game--yet riddled with a host of challenges for Ella. 

Real Challenges.  
                          Difficult Challenges. 
                                                           SMA Challenges.

I'm sometimes puzzled by this disease...

I'm sometimes puzzled by how one can lose their muscle functioning yet still take on the challenges that are part of everyday life.  

I'm sometimes puzzled by the sheer determination exhibited by a three-year old when often, I feel defeated.

I'm sometimes puzzled by how Mother Nature orchestrates our lives--providing us with everything we need despite our lack of seeing it right in front of us.

I'm sometimes puzzled by the strength found within a frame of a person so delicate and fragile, so young.

I'm sometimes puzzled by the courage I find by simply watching my daughter overcome a disease--each and every day and every night...

When she finished her magnet game she asked for a puzzle. With gratitude for her persistence and an eagerness on my part to engage her intellectually once more, we puzzled through the puzzle, despite SMA.  All the while, as I was with her, I myself was puzzled...simply puzzled.

She's quite a person...

Our Little Henry (by Lindsay)

Our kids are small.  They're petite. They always have been.

But Henry's size has caused a little concern with us and his pediatrician.  

Henry has a disorder called Renal Tubular Acidosis (RTA).  He was diagnosed by a blood test shortly after he turned one year old. 

Basically, having RTA means that Henry's kidneys are not working properly and he has too much acid in his blood.  Having this disorder can also cause stunted growth (which is why he was tested in the first place).

However, there is an easy fix.  He's been taking a medication twice a day since he was one year old and has had normal blood tests ever since.  RTA is typically outgrown by the age of 5 or 6, so this spring we will be taking Henry off of the medication to see how his kidney function reacts.

But Henry is still quite small.  He is at the 5th percentile for both height and weight and at his 5 year checkup last week, his pediatrician suggested that we take him to a pediatric endocrinologist to see if there is something else going on or if there is something more we could be doing.

So this past Monday, I brought Henry to see Dr. Levin.  To be honest, I really had no idea what to expect at this appointment.  

Dr. Levin had an incredible bedside manner and was very knowledgable regarding growth issues.

She took an accurate height and weight for Henry and ordered blood tests and an x-ray of his hand (to determine his "bone age").  We will take Henry back to see Dr. Levin in 5 months to do another height and weight to see what his "growth velocity" is (his rate of growth).

Looking at Henry's medical information as well as Michael and my heights, it is projected that Henry should be around 5'9" as an adult.  However, at his current rate, he will not even come close to that mark.  

So what will we do?

We're not quite sure at this point.  But Dr. Levin was discussing the possibility of administering growth hormones to Henry.  

But administering growth hormones is a little controversial these days.  There are possible side effects from the daily injections.  And some professionals have mixed feelings about artificially giving the growth hormone to children.  And the growth hormone injections are very costly.  

So for now, we wait and see.  We'll take a look at Henry's lab and x-ray results and then wait for his appointment in 5 months to see his growth velocity.  

Hopefully there will be a clear choice for what actions, if any, we should take.

Being so brave for his blood test :)