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Christian Smith's Page

**For updates on Christian, please visit Christian's new CaringBridge site at https://www.caringbridge.org/visit/christiantsmith/

This page was created to update family and friends about Christian. It is our hope this site will also be beneficial to others diagnosed with Nemaline Myopathy.

Donations to help find a cure can be sent to:
Nemaline Myopathy Foundation
P.O. Box 5937
Round Rock, TX 78683-5937

Christian Trevor Smith
Born: November 6, 1999
Birth weight: 6 lbs 14 oz Birth length: 19 1/2 inches

Journal

Thursday, May 21, 2009 12:42 AM CDT

I feel incredibly guilty because it's been a very long time since I've posted a message on Christian's site. It's definitely in need of an update.

Last time I posted Christian had just recently had his surgery for salivary re-section and removal. They removed his sub-mandibular glands and tied off the parotid ducts. Dr. Johnson had told me that, over time, the body tends to compensate for the tying of the ducts and will grow new canals to the surface. Dr. Johnson said this seems to occur, on average, in 2-3 years. This would mean a repeat surgery to tie off the ducts to the parotids again but, in the meantime, the surgery was very successful and has been a huge improvement for Christian's ability to be upright in his chair and travel outside of our home.

Since the last time I posted we finally made the migration to a hospital bed for Christian. It's been a good move in that the bed we have allows us to elevate the head and foot of the bed for chest percussion and postural drainage, when needed. However, we are doing chest percussion and postural drainage less often these days since we got our cough assist.

Yes, that's another big change that's taken place over the past year...we changed to a new pulmonary doctor. I had struggled for years with Christian's local pulmonary doctor and had been reluctant to change to a doctor out of our area because I'd been forewarned Christian would likely have repeated pneumonias and if his pulmonary doctor were in another city that would mean a lengthy hospital admission away from home. However, Christian is 9 and has never had a pneumonia (yes, I know, we're very blessed) so I was more confident in taking this step now. Christian previous pulmonary doctor and I had many disagreements on his pulmonary management. To begin with, he never saw the need to put Christian on heated humidification which nearly cost Christian his life due to the constant and serious tracheal plugs we fought daily. In addition, I could never get him to agree to order a cough assist or vest for Christian to help with airway clearance. Furthermore, he always had Christian way over-ventilated and, because of this, Christian had become more and more dependent on the vent over time. We had discussed respiratory sprints for Christian where we would temporarily drop his breath rate for a period of time but this was challenging back when we were doing it because of the salivary issues we were fighting.

So last year we changed to a pulmonary doctor 3 hours from where we live. We had a 2-week hospital stay last summer (June 2008) to evaluate Christian and make adjustments. In the end, we upsized his trach to an adult 6.0 which helped seal some of the leak he has out of his mouth and we dropped his breath rate from 22 to 16. Christian does just fine with this new breath rate (most recent blood gas was 32) and he's been able to breathe on his own again for short periods of time while we move him from the bed to the car, etc.

Our next adventure is going to be craniofacial work. People with nemaline myopathy are born with a malformation of the jaw and, in fact, their whole oral, nasal, pharyngeal anatomy is atypical. We know this because 1) the first and only time Christian has had a full bronchoscopy the surgeon got the bronch stuck in Christian's airway and told me he literally had to get up on the operating table and kneel over Christian on his knees and struggle to get it back out. This process knocked several of Christian's baby teeth loose and we were very lucky they took root again 2)the first time Christian had botox injections to his salivary glands years ago the surgeon had booked 10 minutes in the OR for the procedure and it took them over an hour because they had difficulty locating the glands (they weren't in the typical location). This was confirmed again by the CT with contrast we had before Christian's salivary re-section and removal. 3) When we insert a suction catheter to suction Christian's nose we notice that instead of the catheter going up and then curving back down as you would see in a typical individual, you have to stick the suction catheter in going straight back and it goes back quite far before it drops.

The malformation of the jaw, coupled with the characteristic high, narrow palate seen in nemaline myopathy, causes serious crowding issues for the teeth and also prevents the full closure of the mouth. We've known we had these issues and that we were likely going to have to have something done in this regard but had been hoping to put it off until summer. Then we recently had an issue with one of Christian's teeth. It began to bleed spontaneously and bled for 2 days despite us having tried to stop the bleeding with pressure. The tooth finally stopped bleeding on its own but pushed us to go see someone sooner about the issues with Christian's teeth. We were referred to the craniofacial team at our local children's hospital. We had our initial visit last week and were told we would likely be facing a combination of craniofacial surgery and orthodontistry to correct the problem. As luck would have it the craniofacial orthodontist we saw has actually treated one other patient with NM when she had her practice in Chicago. Christian has the high, narrow palate and both his baby teeth and permanent teeth seem to be battling for position to come out the sides of his gum. He has this strange protrusion of what we thought was gum tissue coming down on both sides of his mouth inside his teeth. I thought this was gum tissue and I was always curious as to why his teeth were coming out the sides of this gum tissue rather than coming out the bottom. The craniofacial orthodontist explained that this is not actually gum tissue but bone and that, in her other patient with nemaline, they had to do surgery to go in and shave down this bone and then follow up with orthodontistry. She further explained that Christian's teeth cannot move down, in part, because they are being blocked by this bony protrusion. So, the next adventure begins!

The other adventure we're on is our constant battle with communication, or a lack thereof. Christian began trying to communicate using the Springboard by Prentke Romich and was using string switches which he pulled with his thumbs in order to scan and select. He had success with this device initially but then his school speech pathologist moved to another town and the new speech pathologist assigned to Christian is completely clueless. I've been fighting constantly with the school because of the regression we've seen in Christian's communication. Recently, Christian's home OT told me about a fairly new communication software called Brainfingers. This software is revolutionary and allows people with extremely limited or no mobility to communicate using brainwave control of a mouse or pointer. I was able to convince our school district to talk with the company and set up a trial period for us to use and evaluate the software. We're encouraged this might be a solution to our ongoing battle with communication.

I'll try my best to update with a new journal posting in a few months to provide further details on our craniofacial and communication journeys.

Until then, many blessings to you all!

-Monette

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Hospital Information:

Dell Children's Medical Center of Central Texas
4900 Mueller Blvd.
Austin, Texas 78723
(512) 324-0000

Links:

http://www.davidmcd.freeuk.com/   Official Nemaline Myopathy website.
http://www.mdausa.org   Muscular Dystrophy Association


 
 

E-mail Author: monette.smith@ni.com

 
 

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