Close to Home

Recently, as I was checking in on our fellow families with kids suffering with ROHHAD (on one of our Facebook group pages) I saw my name tagged in a post.  It was a news post about a little girl being denied a gastric bypass surgery due to her age.

The reason her doctors felt she needed the surgery, though is because she had a tumor that damaged her hypothalamus (the part of the brain that controls hunger among other things).  The reason my name was tagged, though, was because the girl is from Cibolo - that's where we live.  I have not actually met anyone living in Cibolo who has a child with a damaged hypothalamus.  We thought Josh was the only one living here whose hypothalamus was not functioning correctly.

Here is a link to that original story I saw:  Obese, but starving: Girl, 12, denied weight-loss surgery

Since seeing the news story, though, I have been curious about this family and wondered about the similarities between that girl and my son.  I've since learned that the girl, her name is Alexis Shapiro, had the trouble with her tumor that brought on her obesity, when she was 9 years old.

Alexis Shapiro before/after hypothalamus damage

 Josh was about to turn 9 when he was diagnosed with ROHHAD.  His major symptoms all came on when he was 8 and a half.

Josh (age 8) in Feb 2005, days before going into respiratory failure

 and Josh as he has grown

Josh is now 17 and in 10th grade

Josh has been screened for tumors of neural crest origin many times over the years since his diagnosis - but has never had one discovered.  There are some in the ROHHAD community who have suggested perhaps some of our kids had tumors that damaged the brain in that area but then the tumor may have disappeared on its own????  Since none of us knows for sure what causes ROHHAD, we cannot be sure one way or another.  I just find it fascinating that another child so close to our home is dealing with something so similar to my son's disorder, which is extremely rare, and we have never crossed paths.

I did learn today, that Alexis has been approved (by her father's military insurance) for the gastric bypass surgery her doctors feel she needs.  Here is a link to that update on her story: Hope for Alexis: Insurance will pay for surgery

I shared her story with Josh this morning. I also emailed a reporter covering her story to offer my contact information to the family in case they would like to meet.  I think it helps to know someone else out there (especially so close by) is going through some of the same things you are dealing with.

Pacers are ON!

FINALLY we went to the doctor yesterday, October 6th, and got to turn on Joshua's diaphragmatic (breathing) pacemakers.

What exactly does that mean? What all is involved?

Well, check out this diagram again:


It shows an electrode implanted near the neck that connects to the phrenic nerve. The phrenic nerve causes the diaphragm to contract. Then the diaphragm contracting makes air suck inside the lung. When it releases, the lung pushes the air back out.

In the diagram above, just below the electrode is a receiver. It receives impulses and sends them to the electrode, causing the stimulation for the diaphragm to contract.

Both the electrode and receiver were surgically implanted into Josh's chest back in June. His incisions and placement of the devices were a bit different than in the diagram above. Here is a picture of Josh's incisions from back in June and July.





He has two over the nipple area and two underneath. The two above are very deep where the doctor went in and placed the electrode around the phrenic nerve. The two below are where the doctor made much shallower cuts to make pockets to hold the receivers. The receivers are about the size of quarters. The electrodes and receivers look like this:



Over the receivers, on the outside of the skin, when you are going to turn on the pacers, you wear antennas. They look like this:



The antennas plug into a small box, called a Mark IV Transmitter. It looks like this:


The box sets how strong the "shocks" are to make the diaphgram contract and it controls how often it sends the shocks. There are controls for strength for both the right and left lung. And you can turn on or off each side. This box is worn on a strap that can be connected around the waist like a belt.

Well, yesterday, we went to see Dr. Kelly Smith, Joshua's lung doctor (pulmonologist) in San Antonio. He placed the antennas on Josh's chest with the circles around the receivers. We held them in place with a wide Ace bandage we bought. He then plugged in the antennas in the Mark IV transmitter box. And he turned on just the left side, to set the strength. And for the first time, we saw how it looked when the shock made Josh's chest move and take in a breath. I hope you can see this video I took showing the left side pacer working:



If not, here are some still pictures from the doctor's appointment:

The doctor gave Josh a break after setting the left side. Josh got back on his ventilator and the doctor went to see another patient. Then, he came back and set the right side. Then, he turned on both sides at the same time. He adjusted and readjusted many times the strength on both sides. And the doctor changed the breath rate several times.

All the while, we kept Joshua's trach hooked to his End Tidal CO2 monitor (a tube that connects the trach to a box that checks how much CO2 is in his breaths). And we kept his pulsox hooked up to his finger. That checks the amount of oxygen in his blood. It also tracks how fast his heart is beating.

When Josh was off his ventilator and we started the first side of the pacers, making adjustments to strength and rate, his CO2 went up to the low 50's which is a bit too high. And his oxygen saturations were down in the low 90's to high 80's. But by the time the doctor got everything set just right, the CO2 went down to the low 30's and his oxygen saturation went up to near 100 and stayed there. Both those were perfect! And his heart rate went from being over 100 beats a minute to down in the low 80's - much better.

We were very pleased with the results. But we have to keep in mind that using the pacers will make his diaphgram muscle pretty tired out and sore, since he's not used to it working so hard like this. So, he is only using his pacers for short times and then taking breaks. He will stay on longer and longer as we go, as he tolerates it. Josh gets to decide and that is great.

The only problem we have seen so far, and it concerns me a little, is a little blistering around the top outside part of the antennas on Josh's chest. I noticed them this morning as we went to put the pacers back on for today. Josh didn't complain about them, but I could see it was a bit worse on the right side than the left. The right side is set a bit stronger than the left. I'm not sure if that is the reason for them or not. But, I put ointment on them and will check on them again tonight. If they get worse, I will have to call the doctor and see what he recommends.

Well, I've gotta run now. I've got to head to work soon for my third day working in a daycare center!

Finally Officially on the Market

It is official. Our house has a realtor and a MLS listing of it's own. If you'd like to see it click here or here.

The listing includes more than a dozen color pictures of the house. They do not show all the work we did on the house, but you can definitely see the new wood laminate floors through the living room, dining room, kitchen, bathroom and laundry room.