Some of you may recall a season four episode of House called “97 Seconds.” The patient, Stark, had a genetic condition called spinal muscular atrophy (SMA). The season before, House (Hugh Laurie) had an encounter with a little girl in “Merry Little Christmas” (he stole her French fries and had his patented epiphany moment with her arguing about whether her stuffed animal was a bear or a dog). She too suffered from SMA. Coincidence that two Princeton Plainsboro patients have come to our attention with this rare disease? Nope.
House executive producer/writer Garrett Lerner has a son with SMA, and we spoke by phone this morning about SMA, his son Zeke, and what people can do to help find the cure for this terrible (and sometimes fatal) disease.
SMA is a neuromuscular disease affecting the voluntary muscles used for crawling, walking, head and neck control, swallowing, and other activities. According to the Families of SMA website, “it is a relatively common ‘rare disorder’: approximately 1 in 6,000 babies born are affected, and about 1 in 40 people are genetic carriers.”
Lerner had never heard of the little known disease until Zeke was diagnosed at about one year old. “He never stood. Other kids were walking around and he wasn’t. It took around three to four months to get a diagnosis and the doctors were baffled.” Lerner related that it was years before his work on House and he was going through “a real-life diagnostic conundrum of my own. [Zeke] became weaker and weaker and then he finally stopped crawling. He was unable to crawl anymore and they finally figured out what was going on.”
Lerner explained that SMA is broken down into several types. “Type 1 is the most severe and that is characterized by children who are not even able to sit on their own as babies and the prognosis is horrible; they don’t make it out of childhood. Type 2 is what Zeke has. They are able to sit on their own, sometimes crawl on their own at a point, but never gain the ability to stand or walk. Type 3 comes on a bit later. Some of the kids are normal until they’re 5, 10 years old—even into their early teens."