Tuesday, July 21, 2009

"That's what we do for PRS kids..."

I saw that on a message board last night and it got me wondering - just what was your care plan for your PRS child? It became clear to me during the first year that what happens varies a great deal regionally and what is the typical course for one area, is vastly different from the typical course somewhere else. It also became clear that sometimes, a hospital will offer you choices based on their own protocol, and not tell you that there are other options available - so lots of parents are making decisions without knowing all there is to know. Seems like there should be a database, or at the very least, a website where you can see the choices available in different areas of the country, or world.

So I'm curious --- what were the options presented to you??


Melissa said...

Hi, I just found your blog. My daughter was born with Pierre Robin last October. We did the lip tounge adhesion and she had a G-tube. Her lip-tounge were de-adheased in May and her sleep studys are greatly improved, she has had great catch up growth with her jaw. She is scheduled for Palate repair next month but I don't see that happening as she is not to interested in a sippy cup.

Jessica said...

I also just fond your blog. My daughter was just born August 15th of this year - with isolated Pierre Robin. we had a G-tube placed before we came home from the NICU but are now looking for an airway management strategy. She can clear any obstrustion herself, but is work so very hard at it. Weight gain is slow, and she swallows a ton of air while struggling to get in air and keep her food down.
We were told she didn't need a trach and distractions wansn't favorable. The thought she could manage with positioning - and we still may. They pretty much refused to do a TLA which is infuriating to us at the moment becasue we believe that is what should have been done. We're also looking into a nasopahryngeal airway now instead....
She is being treated and the University of North Carolina Chapel Hill.

Brad and Hailey said...

I too tagged along and found your blog. My daughter was born 5 weeks early May 31st of this year. She was born septic and with PRS.

While she was diagnoses with PRS by a geneticist at Primary Childrens they did not know of the link between PRS and sleep apnea. It took a RN who was a former sleep tech to recognize and confirm what we had suspected all along - that my daughter momentarily stops breathing in her sleep. In fact with her first sleep study she was characterized as "severe sleep apnea" even though the NICU didn't even recognize it nor accept it for weeks. They said that it wasn't real because her alarms never went off. What they didn't know was that there is a 20 second delay in the programing of their monitors and as her apnea happened in mere seconds so it wouldn't sound.

As soon as we had the horrible sleep study results they scheduled the tongue lip adhesion. I prayed about it and it just didn't seem right to me. With me being around my daughter when she was on oxygen in the NICU it seemed to stop the episodes, but since they didn't do the sleep study with oxygen they didn't see that. Finally I called the surgeon and told him how I felt and against the doctor's orders we canceled the surgery and sent her home on oxygen. It turns out that according to the sleep doctor/pulmonologist and the Ear Nose and Throat Specialist it wouldn't have made any difference.

Well we went back for a follow up sleep study without oxygen and somehow the surgeon didn't read the results and said it was okay to pull her off oxygen because the study had said "shown great improvement." It w asn't until after my pediatrician called freaking out about the study that we knew something was wrong. So I called the surgeon back and he after rereading the study wanted to do the jaw surgery. But after the Ear Nose and Throat Specialist saw it (which he in the NICU had said my daughter couldn't possibly have an obstruction) said she needs a trache.

So after praying about it everything felt wrong.

So we went to see the sleep doctor/pulminologist and got more of the whole picture from him. First off my daughter still has sever obstructive sleep apnea with hundreds of episodes every sleep period. The problem he sees, as though we are pushing oxygen past the blocking of her airway by her tongue through a nasal canula, her REM sleep and blood pressure in her heart have been affected. By her brain perceiving a block it has cut the amount of REM sleep she has so it can focus on her breathing which will disrupt her cognitive abilities. Also by perceiving the block it doesn't pump blood to the lungs and causes a pressure build up in the heart. He hopes that through the oxygen supplementation and a quick miracle that her body will stop doing this. In order to fix the problem we would have to do the jaw surgery as he feels that she is too young to have a trach if we can avoid it. He says that an infants chest walls are not strong enough and can cause even more difficulty breathing with a trach.

The plan right now is to see how her REM sleep is while on oxygen on her tummy (like she is at home) during this last sleep study. If it is back to normal than that is one half of the good news we need to avoid the jaw surgery, if not then we have to do the surgery. The second thing is we need to see if her heart chamber is dilated from the pressure differences. If it is then we have to do the jaw surgery and pray we don't need a heart transplant later down the road. If it's not then we can avoid the jaw surgery.

One thing is for certain though, that we will be fixing the palate at about a year of age.