Saturday (11-03-07)

The past two days have definitely been a roller coaster of emotions. Two weeks ago I had never heard of Congenital Central Hypoventilation Syndrome (CCHS). Now, I know more than I probably need to know. Dani Jo began to really struggle keeping her oxygen saturation up over the past few days. Yesterday, she desat (dropped her oxygen levels) numerous times. They tried to put her on CPAP (a stronger ventilation system through the nose, but not as serious as the ventilator). She did not respond well with this. Yesterday afternoon, they had to put the ventilator tube back in. She is scheduled to have her tracheostomy surgery on Monday. The doctors would have had to put the vent tube back in for the surgery, so they went ahead and did it yesterday. The hope was that she could get a couple of days of good sleep and rest before the surgery. This has not been the case. She desatted several times last night and today (even while on the ventilator). She has struggled today. They put her on morphine last night to try to keep her calmed down. She fights the ventilator a lot when awake. This afternoon we experienced our scariest moment with Dani Jo. She dropped her oxygen levels. Typically the nurses come over and up her oxygen and she comes out of it. She didn't today. Her oxygen levels dropped to zero. She had turned purple. After a few seconds (it seemed like an eternity to us), she finally snapped out of it. They pumped her full of oxygen and she regained her color. The doctor said that she appears to be frustrated with the vent tube down her throat and that she is tensing up trying to fight the tube. When she does this, the air can't get into her lungs from the ventilator. He said she had actually passed out with her episode this afternoon. They are stopping her morphine and giving her verset (sp??) . This is a more effective form of sedation. Doctor Parsons said that since she has regained a bit of strength, she appears to be stronger than the morphine she was getting. The hope is that the verset will completely knock her out so that she will relax and let the vent do the work for her. They are currently trying to put a PICC (permanent IV) line into one of her arteries. She will need an IV for the surgery and to feed on for a couple of days after the surgery. They tried to put a PICC line in her last week, but were unsuccessful. If they can't get it in this time, they will have to surgically implant a line prior to the trach surgery on Monday.

Now for a bit more about CCHS. CCHS is a genetic mutation of a specific gene. Amanda nor I had no impact on her getting this gene. It was a spontaneous mutation. We could have other children and not be affected by CCHS. However, if Dani Jo has children, there will be a 50% chance of that child having CCHS. If all goes well with the trach surgery, recovery time is usually 2-3 weeks. It will take a week or so to determine what type of ventilator settings will work for Dani Jo. As an infant, she will be hooked to a ventilator via the trach tube 24/7. As she grows older, we will be able to further determine if she can come off of the vent while she is awake and breathing on her own. Basically, her respiratory system shuts down when she falls asleep. About 70% of CCHS children are able to breath on their own while awake. They only need the vent while sleeping. 30% of CCHS children require the vent all day long. There are currently about 200 children and infants in the world today with CCHS. There is no known cure for this rare disease. (Yet!!) When Dani Jo comes home, we will have 24/7 nursing care at the house for a while. As we become more comfortable with caring for her with the trach and ventilator, the nursing care at home will be reduced. Dani Jo will not be able to make any sounds for the better part of a year. The trach tube initially will take up her entire windpipe. As she grows and her windpipe enlarges, there will be room for air to bypass the trach tube, thus causing sound. So we won't be able to hear our girl cry for about a year. We were fortunate to get to hear her cry two days ago when we gave her a bath. It was her first bath since birth (21 days earlier). If Dani Jo progresses well over the first few years of her life, she may be able to have the trach tube removed in lieu of a diagphragmatic pacemaker. This is a surgical implant into the diaphragm which causes it to move, causing breathing to take place. We met with a CCHS specialist yesterday. He gave us encouragement and hope with a lot of the new technology coming for CCHS kids. He made the statement that he would like to say one day that "Dani Jo had CCHS." CCHS kids can live relatively normal lives. Many are active in school and athletics. They just sleep and speak a little differently than other kids. Please don't feel sorry for Dani Jo or our family. We are blessed that we may have the opportunity to take her home in a few weeks. This is the third time we have been in the NICU with a new baby and we can say that we have been blessed. We have seen and talked with several moms and dads that never got to take their babies home from the NICU. What seems like a terrible thing to many in this world will become our new way of life for Dani Jo and our family. We know that God is in control and that He will take care of Dani Jo. He may choose to heal her today, tomorrow, in ten years, or never. He may use Dani Jo just the way she is to touch many people's lives. She has definitely already touched our lives. For those of you wanting to learn more about CCHS, there is information out there. The best website for CCHS info is www.cchsnetwork.org This website was created by Mary Vanderlaan, mother of a CCHS kid (now 19 years old). The website touched me a few days ago. I saw a picture of a CCHS kid on the website at her senior prom. This picture gave me hope and inspiration for Dani Jo.

We are all very tired, stressed, etc. We miss not being able to bring Dani Jo home right now. We miss not getting hardly any time with Jordan and Jamie right now. But we know that God will not give us more than we can handle with His help. We want to thank all of you for your prayers, support, gifts, cards, visits, etc. You will never realize the impact it has had on us.

Please remember Dani Jo in your prayers as she prepares for surgery on Monday afternoon. We will post more when we get a chance.

In Christ,

Joey, Amanda, Jordan, Jamie, and Dani Jo Hearl