how it is

The last day or so, I've been working on a letter to have Liam's kindergarten teacher distribute to the parents of classmates. It's hard and it's something I wouldn't have thought I'd ever need to do, at least not six years ago, but it is how it is. And I'm taking it as an opportunity to educate and increase awareness, hopefully without being too long-winded. It's hard, though, to avoid medical terminology, but I try to get the information across clearly.

Here is the letter – maybe you're interested in reading it, maybe you aren't. Maybe you've been curious about Liam when I mention him, maybe not. Read on, skip it, I won't necessarily know, but here it is:

Dear Parents,

I’ve been meaning to write this letter since school started, but I imagine you can understand how life gets – and stays – busy. Still, better late than never, so I wanted to let you know a little bit more about our son, Liam, who is in Mr. K’s class with your child … He’s a hard one to miss – he is outgoing and talkative … and he drives a red power chair, which we call his Snap Dragon or Snappie.

Liam has a neuromuscular disorder called Spinal Muscular Atrophy (SMA) Type II. He was diagnosed when he was 14 months old and before then we had never heard of SMA, even though approximately 1 in 40 people are carriers and 1 in 6000 babies are born with it. It is an autosomal recessive genetic disease which means that both parents must be carriers and both must pass the gene on to their child. A person with SMA has a missing or mutated gene (SMN1, or Survival Motor Neuron 1) that produces a protein called Survival Motor Neuron (SMN). This protein deficiency has its most severe affect on motor neurons that affect the voluntary muscles used for activities such as crawling, walking, head and neck control, and swallowing. A compromised respiratory system can also lead to an increased tendency for pneumonia and other lung function complications. Sensation, the ability to feel and intellect are not affected.

SMA ranges from Type I (the most severe) to Type IV (adult onset). Liam is a very strong Type II. There is a lot of variation even within each type, but Liam sat up by himself and crawled within his first year. It was shortly after his first birthday that we began to notice that he was losing physical abilities. He was diagnosed in early July 2005, when he was 14 months old. What this means for our family in the day-to-day is that Liam does not walk, but uses an amazing power chair that we brought back from England in March of 2005. Liam first learned to drive a power chair when he was 16 months old at Shriners Hospital in Portland, where we lived until last June. He’s had his Snappie since he was 20 months old, so he’s a very good driver, though is sometimes prone to errors of judgment, as are many five year olds! While at school and at home, Liam also spends time in a stander which he can propel by himself while wearing braces. At Jefferson, Liam has an assistant, Kim Crane, but we also encourage his peers to find ways to help him as well – by picking heavy things up for him, moving furniture or toys out of his path, taking caps off markers, etc. There is a delicate balance between helping Liam and the need for him to cultivate self-sufficiency. As the year goes on, I think his classmates will begin to anticipate and understand Liam’s needs and they’ll find a rhythm with each other.

Spinal Muscular Atrophy is a purely physical disorder, so cognitively Liam is unaffected. He began talking at 11 months old and hasn’t stopped since! He’s a bright and curious little boy – so like many others and, at the same time, so unlike them. Liam’s Snap Dragon chair allows him to explore the world around him on as many levels as possible. His Snappie has an elevator that lets him, by himself, go down to floor level and back up again. He can get down on the floor and play. He can race around outdoors. He can go on a bumpy path to pick blueberries in the summer. He can join his peers at a little table. The Snappie does weigh 230 pounds, so it’s important that both Liam and those around him, children and adults, be careful. The Snappie is, essentially, an extension of Liam’s body and I know that Mr. K and Ms. Crane are doing an excellent job of helping the other children recognize that.

We moved to Corvallis from Portland in June 2008. We came here for several reasons, a critical one being that we were able to find a single level home that would work well for our family. Because our home is fully accessible, it is a great place for Liam to play with friends. We are also happy to meet at an accessible location, like the library, for example. If you’d like Liam to come to your house, please consider whether it is wheelchair accessible; we do have a portable ramp that could span one or two stairs. If your child and Liam are interested in playing together, let us know.

Please be aware that even common illnesses, such as a cold or stomach bug, can have severe consequences for Liam. When he was 3, he spent a week in the Pediatric ICU because a cold very quickly turned into pneumonia. As we head into flu season, and particularly with H1N1, we ask that you keep this in mind when deciding whether to send your child to school when he or she may be sick. Likewise, we would much rather reschedule a play date than risk Liam’s health and well-being. Attached you’ll also find a short note from Mardi Henke, one of the school district nurses.

We hope you’ll share any information about Liam and SMA you’d like with your child and also feel free to call or email if you have further questions. We encourage you or your child to ask questions if you have them; we will do our best to answer them. Your children have been so welcoming and friendly toward Liam – thank you. We really feel that Jefferson is a wonderful community and we’re so pleased to be part of it now.

Best,

Lynn and Ben Russell