What a Summer!!!

Greetings from the Hearls!

We have had a very eventful summer! Joey and I went to the CCHS conference in Florida--thanks to everyone that gave we were able to got to this conference and learn about new things on Dani Jo's disease. There was so much information to take in. We were able to see many kids that have CCHS from 2 years to 31 years. This was nice because it gave us hope that Dani Jo will lead a normal life. These teens were able to play sports, with many breaks; go out with friends; and lead normal lives except for knowing their limits. Most of their friends knew them and their condition and were able to tell them when it was time to slow down for a rest or take a few breaths.

Some of them had Trachs, pneumobelt, others were ventilated via bipap while they slept, and most had pacers. You all know about the trachs by reading our blog, the bipap or cpap is shown below. These are used on the less severe cases. If you look at the picture below you can see how it cups around the nose. Because some of the CCHS kids used these, there is a distinct impression on their faces. Below the bipap picture are some of the kids that used the bipap. You can see the impression on their faces. These teens used bipap early in life before the face is finished developing and because the face muscles were still growing the impression was caused. The look of some CCHS kids are only because they used the bipap, not from the disease itself.

BIPAP/CPAP
Teens at the conference!!!

A pneumobelt with a positive pressure respirator was used for sleep for three patients with chronic respiratory insufficiency secondary to syringomyelia, poliomyelitis, and Friedreich's ataxia respectively. This device was found to be effective in improving and maintaining their daily functioning in the community or institute.

Joey and I were most intrigued by the pneumo belt. This thing was the most promising without surgery! I have a picture of one of the girls at the conference that had this. All it was, was a belt that was connected around the abdomen/diaphragm and hooked to the ventilator! This belt as causes the diaphragm to contract therefore causing the patient to take a breath! This would not be available to Dani Jo until she is 4 or 5 at least.

Teen with pneumobelt

There is also the diaphragmatic pacer. Joey and I were very hesitant on this procedure. This has to do with making an internal incision just under the phrenic nerve and implanting the pacer with a transmitter. Dani Jo is able to come off the vent while awake, however if this nerve is damaged (which is a possibility) while Dani Jo would be ventilated 24/7 for the rest of her life. Just don't know if we want to take that chance. It is a very intense surgery!

We learned so much at this conference all because of you we were able to go! Thanks again for everything!

I will put some pics of other CCHS that were at the conference. It was nice to meet other families with Dani Jo disease--they all understood--and most of them had already been through what we are going through now, and were able to give us a light at the end of our tunnel. Thanks again for everything! Joey, Amanda, Jordan, Jamie, and Dani Jo