When two isn't enough to tango

Thanks for all the support and prayers headed Keane's direction. Last week my biggest concern was my finishing my manuscript for school, starting to actually work a bit again and planning for Avery's birthday party and now...well, my whole world view has changed.

It's so crazy. He doesn't really look that ill. He gets a little pale from time to time, but he laughs, chuckles, plays with his toys, even kicks his sister when given the opportunity. Ironically, in the last couple days he started standing up on both legs, which cracks him up and obviously gives him a tremendous sense of accomplishment. When ever I am in the room, he watches me non-stop. Every time my eyes catch his from across the room, he brings his hands to his mouth and gives this sheepish grin as if to say, "Hi, Mom." 

I have good news, sort of, and less than ideal news. We spent about four hours yesterday afternoon at Doernbecher Children's Hospital to see Keane's new best friend, Dr. Kelly, his Pediatric Cardiologist. First the good news: Keane is stable enough to go home for a couple weeks. He needs to gain some weight before his surgery. I have pulled my first crazy Mom card and made him five appointments at his pediatrician's office in the next seven days. The first appointment will be today to get a baseline weight and oxygen saturation level. Dr. Kelly wants him to gain 20 to 30 grams per day and I don't want him to fall behind, so we are going in for a weight check and O2 check on Mondays, Wednesdays and Fridays. Keane was also prescribed and started taking Lasix yesterday. As many of you know, Lasix is an anti-diuretic that will hopefully remove some fluid from around his lungs. I still have a couple oxygen tanks in case my Mommy -dar thinks his oxygen saturation is too low.

The less than ideal news is that his heart is actually worse than Ben and I had understood from our pediatrician and our hours of research. When a normal heart starts to form around 8-ish weeks of gestation, it makes a series of folds that form the four chambers of the heart. It's a little like origami, first it folds in half in one direction (e.g. horizontally), then it folds in half again (e.g. vertically). Hence, four chambers are created. We were under the impression that the heart murmur indicated a hole between two of his heart chambers, causing oxygenated blood to mix with non-oxygenated blood. After reviewing the echo report ourselves and, particularly Ben studying up on all the terms contained within, we were anticipating to hear that Keane had at least three holes that needed to be plugged. That is not what we heard. I think I stopped breathing completely when I realized what Dr. Kelly was telling us - there were no holes. "What?" There were no holes. The folding of heart never happened. There were never complete chambers formed. There's just one big non-hole. There are sort of two incomplete chambers where there should be four. The murmur detected at his visit last week wasn't a murmur at all, although in kids with normal hearts, it would be a murmur. Keane's "murmur" is really blood spilling back to his lungs. Of rouse this couldn't really be detected earlier.... without walls, there is no hole, without a hole, there is no detectable sound.

Gortex. I used to work at REI when I was in grad school (the first time). I have a great sales pitch about Gortex as a superior waterproof product. The surgeon at OHSU, whose only job it is to fix kids with congenital heart conditions, is going to re-build Keane's heart using Gortex. I think if it were not my own child, I would be truly impressed and fascinated. I am still getting over the fact that Keane doesn't even have four heart chambers. When I was pregnant with Keane we were unable to get clean cardiac pictures in utero at my 20 week ultra sound. I went back at 24 weeks - still no clear pictures were obtained. My only question was "Can you tell me if there are four chambers?" The answer. "Yes." I understand now. The pictures weren't unclear. All the blood was mixing together. They thought he was moving, that he was so active, the images couldn't be reliable. They were clear. I don't think, given my risk factors, or lack thereof, that anyone ever considered the images were reliable - he wasn't moving too much, he was just missing at least two walls of his heart altogether.

In two weeks, Keane will return to Doernbecher for a repeat cardiac echo under sedation. Every measurement and every picture will be obtained. Next, Keane will be presented to the entire pediatric cardiac team - "cardiac conference." The entire team will decide how to rebuild his heart, how to make four chambers out of only two partial walls. Obviously he will require a full open procedure, go on cardiac bypass, and hopefully, when it's all said and done, his heart will accept its new structure, they will divert the blood back to his heart, off the bypass machine, and it will beat for the first time, in the right way. His surgery will be in about three weeks. If all goes well, he will only be in the hospital about a week. Then, Ben and I have decided we will stay in Portland, at my parent's house, for another week before heading home to start the next steps in a long journey.

I know many of you are waiting for confirmation of the diagnosis of Down Syndrome. We have not received any results of the chromosomal count and structure. However, we all need to move toward putting the systems and early intervention supports in place that will help Keane keep up with his age cohort in physical, emotional and cognitive development. Ben and I do not need to wait for the results of the kerotyping; kids with only 46 chromosomes almost always have four heart chambers. Our little man has a little extra something that makes him a little extra cool - one more chromosome and one more chance at a long, healthy life thanks to a doctor with a great English accent (his surgeon) and Drs. Kelly and Velarde (our pediatrician).

Of all the learning opportunities I have had in my life, exposure to children or adults with developmental disabilities has not been one of them. The medical literature and academic sites are a little overwhelming - potential health issues, barriers, etc. I have found, by far, the two best resources in my life are: (1) my sister-in-law, Amanda, who has already discovered through her network as a educational specialist for children with special needs that Lane County has one of the best early intervention programs in the State and (2) this website: www.noahsdad.com. In Noah's Dad's words, the website is "A window for you to see hope, encouragement, and normalcy [of raising a child with Down Syndrome]." 


With love and hope,
Erin