Friday, August 26, 2011

A bathless summer

In the past 3-1/2 years, we have never had the chance to be excited or hopeful about any of the medical aspects of Nathan's life. When you hear such things as "no cure", "progressive", "degenerative" and "life shortening" attached to the diagnosis of your child, it's difficult to be encouraged by medical interventions. We don't have a treatment that will keep the disease tolerable, nor a drug that will put it in remission. We simply treat each symptom as it surfaces, throw supplements and cofactors in that we hope will help in the long run, and pray that better treatments or a cure will happen in the lifetime of our children.

Its the life to which we have become accustomed to, but there are still times where it all seems too overwhelming to process.Even though no 2 kids with mito are the same, the treatment options and plans seem to follow the same route. Each medical intervention to give help and relief to your child is met with heartbreak at the realization of what has been lost. Its hard to be hopeful in these circumstances.

So when our surgeon presented a potential new treatment for Nate a few months ago, it was the first time in quite a while that I truly felt excited and hopeful about this intervention in the life of my son. The question was if it would work.

Nate is doing amazing...simply amazing. He feels great, has incredible energy, and is eating more than he has ever eaten (on top of still getting a ton of calories through his tube feeds...he has a lot of catch up growing to do). This GI stimulator has truly been life-changing for him, and it has been incredible to witness this transformation.
We know it works, but we really wish we knew why it works. The inquisitive part of me has an innate desire to know why things work, so it has been difficult knowing that we simply don't have the data or research showing us how and why it works exactly. The docs and researchers are fairly confident that it changes something in the interstitial cells of cajal, which are the cells found in the GI tract (esophagus, stomach, small intestine, and colon) that are the pacemaker cells of the gut. I find it no coincidence that these cells also contain an abundance of mitochondria (usually meaning that the function of the cell requires a lot of energy) and to quote this journal article, "mitochondrial Ca2+ handling may underlie the dependence of gastrointestinal pacemaking on oxidative metabolism." In my very unprofessional opinion, I wonder if dysfunction in these cajal cells are truly the catalyst behind mitochondrial disease-induced gastrointestinal dysfunction and failure, especially since they work as the intermediaries between the nerve cells and smooth muscle cells in the GI tract. As these cells succumb to apoptosis, it seems that the gut will lose its pacemaking abilities, thus creating the failure to move food through.

And to further that thought process, I wonder if the high frequency/low energy stimulation Nate is receiving from this device could be reducing the workload of his mitochondria in his cajal cells, thus decreasing the oxidative stress in these cells and allowing them to perform more optimally. If this is the case, the patient would need to still have a good amount of cajal cells for it to work, as they know through past biopsies that the stimulation does not work if the cells are depleted or severely low in number. Obviously, these are just theories circling around in this rattly ole' cranium of mine. We need research...we need understanding.Pending insurance approval (this is a very, very, very expensive device that will be implanted internally...think in the ballpark of a Hummer with blinging rims and a deafening sound system), Nate will have the permanent GI stimulator placed on October 11th. The device will lie in a subcutaneous pocket in the abdominal wall, and the leads will be screwed into the stomach muscle.

Right now with the temporary stimulator, the leads are screwed into the mucosal lining of the stomach, but since food hits them and there is so much movement at that part of the stomach, the leads migrate and come loose easier...thus the reason why it is a temporary placement. The leads of the permanent one will be in a place where this shouldn't happen, although due to this, the response of the stimulation usually takes a lot longer than the temporary one, and it isn't usually quite as effective. He more than likely won't get as good of symptom control and treatment as he is currently getting with this temporary stimulator...but it will still be a whole lot better than it was before. Its another one of those questions that don't have answers to yet...why does the temporary vs. permanent placement of these leads give varying reactions? Hopefully we can get answers as research continues and tailor the treatment as we know more.
This summer shall be known as the summer when Nate didn't have a real bath for a full 2 months. Believe me, that kid can have all kinds of funky smells emitting from his body. It will be nice to plop him in a sudsy bath again and give him a good washin'. There's only so much you can do with sponge baths.

But I also hope that when we look back over the past few months, we also realize that it is the summer where Nate made a turn-around. I pray that this treatment continues to work and that my little man continues to improve and can get several more years of use with his stomach and intestines. We have been blessed with many incredible opportunities in regards to Nate's medical care, and we are abundantly thankful.


Kyla on August 27, 2011 at 9:54 AM said...

HOORAY! I hope the insurance approval comes through and I'm so, so glad this is working so well for Nate!

Nicole on August 27, 2011 at 10:08 AM said...

I am praying that insurance approves this procedure and that the permanent pacemaker does wonders for him!!

Dianne on August 27, 2011 at 10:15 AM said...

Amber, I feel your uncertainties. Wanting to shout but experiences past causing you to be wary. I've heard those grim terms (no cure; shortened life span; crippling bone deformities) for our GRANDson with Job's Syndrome. I KNOW though that God put a spark of hope in my heart and it has NEVER gone out and I know it never will. Your faith is evident in EVERY post. Your strength divinely supplied. Your love for God and this little family of yours...exemplary. I continue to pray and LOVE seeing Nate responding so beautifully to this new treatment. May God continue to infuse you with strength and hope.

Mighty Mito Mom on August 27, 2011 at 11:31 AM said...

Praise God it is working. And thank you Nate and the Ferrells for pursuing this and adding to the data on this new treatment! Hugs!

julie on August 27, 2011 at 12:25 PM said...

So thankful to Christ that this treatment is working. As our family undergoes evaluation of our youngest daughter for Mito, I always find wisdom in your posts. May God continue to move in the life of your family and those that are touched by your journey.

Chris Krafft on August 27, 2011 at 2:59 PM said...

Hurrah, so good to hear that it is working well for him - would love to put some weight on him this summer!

Dani on August 27, 2011 at 3:06 PM said...

It's an answer to many prayers from many people for the little feller and your family.

Carol on August 28, 2011 at 10:16 AM said...

What a blessing this is! So good to hear it. And I love the first paragraph...sums up life with mito perfectly. God bless!

Cheryl and Bricen on August 28, 2011 at 2:53 PM said...

I'm so happy to read the pacemaker has had such a positive affect on Nate's life. What a blessing to all of your.

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