Question: Did you guys know before he was born?
Answer: No, they counted fingers and toes during the ultrasound but did not see that his arms were short. It was a complete surprise when he was delivered, even to the doctor. We had no idea what it even was for about 48 hours, given how rare it is.
Question: How long was the Labor?
Answer: Jenn had 18 hours of "Transition Labor" This is the stuff that hurts. She stayed at home through most of it as they sent us home from the hospital when we first came in at 9:00pm on April 27. She was not happy about being sent home. By the time we came back to the hospital at 7:00am of the 28th, she was 5 cm dialated. The only word she was capable of saying was "epideral" At about 9:00am she recieved the epideral she had been requesting and proceeded to sit up and put on makeup. Once the actual pushing began it was only about an hour and a half and spencer was born. Delivery went smoothly although at the last minute, the doctor used the "Vacuum assist" to help Spencer on his way.
Question: What is wrong with Spencer?
Answer: Nothing
Question: But why are his arms the way they are?
Answer: Spencer was born with a rare genetic condition called T.A.R. Syndrome.
Question: What Causes TAR?
Answer: They have ruled out all environmental things and believe it is caused by a double recessive gene. That means that both Jenn and Coburn carry a recessive gene that doesn't show up anywhere in their family but get the two of them together and TAR is a possibility. (Possibly as high as a 25% chance) Jenn has since had another child with her new Husband Doug. That child does not have TAR syndrome.
Question: How did he crawl and start move around?
Answer: He started by rolling around on his belly. He couldn't "crawl" as his arms are not long enough or strong enough to support his weight.
His first form of locomotion was hopping. He would sit and bounce striaght up in the air and move forward in little hops. He loved doing this on the bed or the couch where he got extra spring. He perfected this technique until he was able to move with astonishing speed and really catch air. You could hear him thumping across the floor from a long way up.
He was eventually able to stand and walk like a little cowboy. He could do this for short periods but as his body weight increased, this became painful. Then even his hopping was too hard on him. At the age of seven, both of his legs were amputated at the knee in a series of operations. He now walks on two prothetic legs. He also has "Stump socks" that allow his "hopping 2.0" version of locomotion which is a sideways version.
He can also stand up and balance on his stumps with effort. This allowed him to participate in Karate and other activities.
Question: Why was a double amputation necessary?
Answer: Spencer's legs were very bowed when he was born (he looked like he is always in a meditation pose) due to his knees. His lower legs were rotated in about 80 degrees (at the knee) and may have been the result of the top of his Tibia bones not forming correctly. His knees were very... intersting (as the doctors told us) and there was no way to realign them as we had hoped.
Amputation was an option that was not ideal, but ultimitely proved to be the best alternative. The original plan was a single amputation and a rod that locked the other leg straight. However, after a long series of tries, the remianing leg could not be straightened without cutting off the blood supply. Double amputation proved to be the best option.
Question: How many Platelet transfusions has Spencer had?
Answer: Spence had a total of 10 platelet transfusions before he was one. He has not had any whole blood transfusions. He did not require any more transfusions after he was one. He did have a few more to prep him for his amputations.
Question: What special precautions have you had to take?
Answer: Many (but not all) Children with TAR have allergies to cow's milk and dairy products. In addition to cow's milk allergies (the protein in the milk), soy is also an allergen because of the protein. Since Jenn is breast feeding she has modified her diet, eliminating both dairy and soy.
Also, due to his low Platelets, he bruises very easily. Even a little bonk on the head can turn into a real knarly bruise. We may have to get him a helmet when he is a toddler. I am very excited to put cool graphics on his helmet!
Question: How are you guys doing?
Answer: Spencer, Jenn, Doug (Spencer's step-dad), and Owen (his step-brother) live in Colorado and are doing great. His Dad Coburn still lives in Los Angeles and visits his son and his god-son Owen every chance he gets.
Once you meet Spencer, you realize that there is nothing wrong in his world. He is bright, alert and quietly thoughtful. He laughs at everything and makes you wonder why you worry so much.
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