Little's Life

Little's Life

Wednesday, June 29, 2011


Andrew answers the phone

knows his name

and starts to get the hang of using a spoon!

Saturday, June 25, 2011

A break from the usual gloom and doom

That's my brother in the red hoodie on an episode of Castle   :) :)  No worries, Eminem.

 When Mister concentrates on something, he puts his tongue into his bottom lip.  Looks like Little is picking it up, too.
 GREAT improvement in his ability to hold his own weight while standing!  Still can't get there by himself, but his legs sure have gotten stronger.

 In his Smartrike striking a pose.

 Writing on the wall instead of his father's day card.

 Heading out to Mister's promotion ceremony
 nap hair!
 The moment I knew I no longer had to hold his bottle for him.

Wednesday, June 22, 2011

The EMG and Nerve Conduction explains an EMG like this:

Electromyogram (EMG)

A needle electrode that is attached by wires to a recording machine is inserted into a muscle.
When the electrodes are in place, the electrical activity in that muscle is recorded while the muscle is at rest. Then the technologist or doctor asks you to tighten (contract) the muscle slowly and steadily. This electrical activity is recorded.
The electrode may be moved a number of times to record the activity in different areas of the muscle or in different muscles.
The electrical activity in the muscle is shown as wavy and spiky lines on a video monitor and may also be heard on a loudspeaker as machine gun-like popping sounds when you contract the muscle.

Nerve conduction studies

In this test, several flat metal disc electrodes are attached to your skin with tape or a paste. A shock-emitting electrode is placed directly over the nerve, and a recording electrode is placed over the muscles controlled by that nerve. Several quick electrical pulses are given to the nerve, and the time it takes for the muscle to contract in response to the electrical pulse is recorded. The speed of the response is called the conduction velocity.

DocNeuro told me to bring distractions, so I took Tot to BabiesRUs for a Mehmo (Elmo) and well........

we went home with something else!

So that's the super fun day we had today.  Poor Andrew.  He screamed his face off during the nerve conduction even though it wasn't painful (Nurse put it on my arm first to show me just how unpainful it was).  I believe he was just scared.  Here's his reaction to it -

That last one is the worst!  It really didn't hurt him, I promise.  He was just scared and didn't like his leg being held still.

The EMG, on the other hand, was definitely painful - and no I did not take pictures of it.  DocNeuro administered that one.  My poor little angel just screamed and cried and it broke my heart.  Andrew's thigh was purple and knotted up before we even got him redressed. 

The nerve conduction showed nothing, so his nerves are all fine.  The EMG showed that whatever desease or disorder he has is, for now atleast, only in his upper arms and shoulders.  These are the muscles that we already knew were the weakest on him, but hearing that his legs read normal was great news.

Unfortunately we are no closer to a diagnosis yet.  There are hundreds of possible causes for his muscle damage including muscular dystrophy, certain enzyme defficiencies, myositis, dozens of different myopathies - seriously HUNDREDS of things can cause his symptoms.  We have an EKG scheduled for Thursday, as well as a consult with a Geneticist.  If his EKG comes out fine then a muscle biopsy will likely be the next test.  If there is damage to his heart, then the testing will go a whole different direction. 

On top of all this, Little has to have hernia surgery!  This poor baby boy just can't seem to catch a break.  Fortunately Vanderbilt is wonderful at coordinating care and DocNuero said that we can most likely take the muscle biopsies at the same time as his surgery with the Urologist. 


Please keep us all in your prayers - this is just a nightmare.  I want each one of you to praise God in His graciousness for giving you healthy children.  I don't ever, EVER want you to know what this feels like.  We've been told Andrew's condition could be terminal, could be a simple fix with a daily pill, may be a lifelong struggle, or may just be a small hurdle in what will otherwise be a long and healthy life.  The wait to find out what it will be is absolute torture.  Rosie, Jessica, and the rest of you with your own medically challenged kiddos - you're in my prayers.

Saturday, June 11, 2011

Anyone need some CPK bc Tot's got about 9,700 extra

CPK: Creatine phosphokinase (CPK) is an enzyme found mainly in the heart, brain, and skeletal muscle. Your body produces it when the muscles have been injured or diseased.

On Wednesday, Little had his consult with the Neurologist at Vanderbilt Pediatric. Doc Neuro listened to all of our concerns, examined Little, then sent us for bloodwork. Doc Neuro told us that since nothing is jumping out at him as a definitive indication of any certain syndromes, that it could be a long road to figure out why Little is weak and delayed. We already knew that, so that wasn't hard to hear.

On Friday, Doc Neuro called me with the results of the bloodwork. Here's what he had to say:
In a normal 17 month old, the body's levels shouldn't be above 300. Little's are over 10,000. What this means is that he has some form of muscle disorder or disease. There are literally HUNDREDS of conditions that can cause high levels of cpk, so now we must begin the process of ruling them out/narrowing them down. The 1st of these tests is called an Electromyogram (EMG). Basically what will happen is DOc Neuro will insert needles into Little's muscles and send currents through his body to contract his muscles. As this happens the flow of proteins and other impulses will be recorded to see which muscles are damaged the most and what proteins seem to be defficient. It will be painful and it will be scary. He told me to bring anything I can think of to distract Little with. Fantastic.

After the EMG comes the EKG to see if his heart is the damaged muscle. It's basically just an ultrasound, so that's a relief.

After the EKG comes a muscle biopsy from an area that the EMG pointed to as most affected.

After that (or before since we're already scheduled for our consult at the end of the month) comes genetic testing.

If we are lucky, we will get a diagnosis that tells us how best to help our baby boy. Some of the possible diagnosies are mild, treatable and some are downright terrifying. We are trying not to dwell on the wors tof them, but it is impossible to fully clear them from our minds.

It's going to be a long few months...