So, a while back after we returned from New York City, I mentioned that we saw Dr. Micheal Ain. Dr. Ain is an Orthopedic Surgeon from John Hopkins Medical Center. I had spoken with him previously while we were in Detroit for LPA’s National conference in 2008. Dr. Ain is well respected within the dwarfism community, not only because of his patients with Achondroplasia, but also because he has Achondroplasia.

When I saw Dr. Ain during one of the medical seminars, I asked his opinion regarding children with Achondroplasia and progressive Kyphosis. Now, this question was not asking about Kyphosis in general, but progressive Kyphosis in children, specifically under 2. At the time I explained that Knoah has been in bracing therapy since he was 7 months old. When we started it, his Kyphotic curve was hovering around 30+ degrees, which is nearly unheard of in a child under the age of 7, but it does happen.

January 05, 2007

May 23, 2007

January 21, 2008

A year later, Knoah’s curve was at 65+ degrees even during bracing. So, Dr. Ain suggested I make an impromptu appointment so we could see him and he could examine Knoah. Now, before I set foot into the room, I knew what he was going to say. So, it came as no surprise when he said Knoah needed surgery. It also came as no surprise when he said Knoah needs surgery NOW. Although hearing it and thinking it are two separate things.

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Now comes the “debate”. We all know by now ALL children with Achondroplasia have Kyphosis. We also know that in 90% of cases the Kyphosis resolves on it’s own as the child starts walking. The 10% that don’t resolve will go into bracing therapy around age 4 and continue until age 7 or 8. By this point the Kyphosis should be resolved or minimized under 10 degrees or so. Out of that 10%, maybe 4% will end up needing spinal surgery or a spinal fusion by the time their 15.

Our problem. Knoah just turned 3. He is 1 out of say 2 children (39,998 have Achondroplasia) with such a progressive Kyphosis that bracing is not working.

There is no reference guide, no text books nor medical journals that chronicle a 3 year having a spinal fusion or Laminectomy. With a progressive Kyphosis, damage can occur to the vertebrae. This is called Anterior Wedging. When the vertebrae start to hit each other, bone fragments start to break off. This leads to symptoms such as back pain, respiratory dysfunction, neurologic involvement, or symptoms of spinal stenosis.

So, we are left asking, what do we do? Will and differ on opinions. He suggests we contact Ain and have the surgery done. I say get a third opinion before we throw Knoah on the table. So, where do we go? We already see Dr. Pauli and he says NO SURGERY! That’s one opinion. We saw Dr. Ain and he says SURGERY NOW! There’s two. We need a third opinion.

I understand Dr. Pauli’s reasoning. Surgery at this point is way too aggressive, especially given Knoah’s age. We would be looking at nearly 8 weeks at John Hopkins for the surgery and recovery. Another 12 weeks of sustained recovery and therapy and we are not talking the any complications that may arise or the issue with transportation, travel expenses, lodging, etc. The cost of such a thing would be insurmountable. However, if it HAS to be done, we will do it.

So, I guess, I am looking for your advice. Who do you see for an Orthopedic Surgeon? Has your child had a spinal fusion and what was it like. Who would you recommend for us to see to get a third opinion.

I have been sitting on this for 4 months because while Knoah is not showing signs of pain or neurological issues, we don’t want to wait either.