Saturday, July 30, 2011

Congenital Central Hypoventilation Syndrome (CCHS) is confirmed

July 30, 2011
It’s been a busy last couple of days. I can’t go into much detail right now because we have to leave soon to attend a NICU sibling support group… In short, Wyatt is improving and the antibiotics seem to be working to fight off the two types of bacteria in his trach. Last Thursday we received the news that the genetic testing results were in and Wyatt does in fact have Congenital Central Hypoventilation Syndrome (CCHS). This website is the best we have found so far and I already joined their network and contacted the specialist listed: http://www.cchsnetwork.org/. In very simple terms, CCHS means that there is a mutation in one of Wyatt’s genes that causes his body to not be able to recognize or respond to an increase in carbon dioxide in his blood, especially during tired or asleep periods. Therefore, he is unable to learn to improve his breathing and will require continuous mechanical ventilation. However, the prognosis is good in that most children with CCHS are able to eventually come off the ventilator at least partially while awake. There are medical advances such as diaphragm pacing that we are looking into. Most of these procedures are performed later in life though. Our biggest obstacle right now is to find cases similar to Wyatt’s. He is not the “typical” CCHS case because normally this disorder presents with normal MRI results. He has the added challenge of having an abnormal MRI and therefore it is impossible to predict what his long-term prognosis is. From a developmental standpoint, Wyatt so far is on track with movements and tracing. So that’s great news and the doctor said that based on his muscle tone and movements, he may not be at as high of a risk as originally predicted for Cerebral Pulsy. How CCHS, the abnormal MRI and their respective possible complications come together, that’s what we’re trying to research now? Only then can we move forward with indentifying what medical advances may apply to his particular case. We’re hoping that Wyatt eventually qualifies for diaphragm pacing which would be done in Chicago, IL at Children’s Memorial Hospital… Needless to say, with the remodeling of the house, the preparation to transition Wyatt to a home ventilator and then actually bringing him home (hopefully around Labor Day if everything runs smoothly), and doing all the research and contacting CCHS specialists, there is not a lazy moment in our house right now.

2 comments:

  1. Hi, my name is Travis. I just came across your blog about Wyatt and his CCHS condition. My wife and I are currently waiting for our new daughter, Keira's test for CCHS. It's been a nerve-racking and insanely anxious time. My wife is holding up extremely well given the circumstances but it's definitely taking its toll on us. We've been through all of the differential tests for all the obvious suspects but so far everything about her is normal except for her shallow breathing. The doctors (and us) are pretty sure the test will come back positive for CCHS given all of her symptoms but we're taking it one day at a time. I'd love to be able to maybe start up some sort of dialogue once we get a full picture of baby Keira's outlook. Would you be up to possibly exchanging emails every now and then? Thank you for posting this. I know how extremely personal it is but seeing that there are others out there in our situation gives us some hope in what is a pretty dark time for us. Please give our best to your family from ours.

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  2. Travis... I am so gald that my blog is able to reach out to your family and offer support. I know how difficult it is for you right now. Wyatt has only been home for a couple weeks now and the NICU stay was very difficult emotionally and physically. After ruling everything else out, one of the NICU doctors decided to send off the genetic test and I have to tell you, the best thing I did while waiting for the results was to research the disorder. I didn't want it to come true but I felt like the more I knew about it ahead of time, the better prepared I'd be if it did come back positive. You will be your child's best advocate and you need to know what's ahead because quite frankly, you'll have to set the doctors straight a lot of the times because they don't usually know enough about CCHS and you have to make sure they're not treating your child like any other ventilated child. cchsnetwork.org is a great resource. You can't join until you have a diagnosis but you can read some the stories and literature and there are contacts for CCHS specialists as well. The best advice I can give you while you're waiting is to read as much as you can and get involved in the hospital with hands on care. I know you're terrified and there are a lot of uncertainties but having only dealt with this for 3 1/2 months myself, I can tell you it's not a doomed sentence. We went through the grief of a normal child (and it still hits us every now and then) but by now we look at it differently. It could be a lot worse and I've seen a lot worse at the NICU. I don't look at Wyatt as being sick. He has a gene mutation and challenges ahead of him but he's stronger than any other person I've ever met and that makes me stronger. As scary as it is, it is a managable condition and you'll get in the swing of things. No matter what, every baby has hurdles to overcome and you'll learn to adjust and become experts on your child's needs and optimal care.
    Please feel free to continue posting comments on here as it is my hope that this blog will develop into a support group for many more families. If you have more specific questions though, I'd be more than happy to go the route of direct email, too.
    Best regards & hugs to your family.
    Nicole

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