Wednesday, December 23, 2009

Time to let this blog out into the atmosphere

I tried before and couldn't get this off the ground. 2009 has been a long year, a year in which the miniscule amount of time I had free, the time I could have been blogging and updating our friends and relatives on what's going on here, I mostly spent trying to escape from the reality I had to deal with. I learned to knit though...that was excellent. Having something to do with my hands and now having lots of beautiful hats and scarves is fantastic! But, I also feel it's time to get this rolling, so that when life gets really complex in a month or two, I'll have a place to write it down and you can all easily access the information you want.

I have to say, 2009 didn't zoom by for me. Illness, travails, much to tend to. I still can't believe we'll be writing 2010 on all of our checks starting in a week. That seems to be something pulled out of a piece of science fiction. Nope, 2009 was not one of those "blink and you'll miss it" kinds of years.

Right from the start we were dealing with Ben's health and we are still in the thick of it 12 months later. Back in January we were seeing our wonderful therapist, beginning to process (again) the neurosurgies Ben went through four years before. And then, suddenly we were looking at a new x-ray that showed a serious progression in his scoliosis, something we'd actually tried to forget in the intervening time. Since then, we have visited three hospitals, had 10 more x-rays, talked to several orthopedic surgeons and a slew of alternative practitioners, spent many hours discussing the options available, and are again looking at multiple surgeries in Ben's immediate future. Sigh...

Ben's scoliosis was discovered at the end of 2005, when he was eight, and was 28 degrees at the time. It was the key to our discovering his Chiari Malformation. At the time, we were focused on the neurological issue. Our neurosurgeon told us it was likely a result of the Chiari. But it didn't resolve after that surgery and the doctors at Children's Hospital in Oakland didn't monitor it. Hence, our surprise this year when we found out it had progressed. Can you say "denial"?

Juvenile scoliosis can be a somewhat more difficult affair than adolescent scoliosis. AS shows up during the big growth years and is usually treated with bracing. My cousin Julie was identified with it when she was 14 and wore a brace 23 hours a day for 2 1/2 years of high school. It worked for her, she tolerated wearing it and kept her from needing spinal fusion surgery. Our nephew, Brandon, though, had aggressive AS that had was identified in his early teen years and required emergency fusion surgery when he was 15. Bracing sometimes works better with late onset AS, since the curve tends to get worse when the spine grows (which is why it so often appears during puberty) and later in adolescence a child has fewer years of growth left. The brace is not tremendously effective, though, since it is purely external and because it's hard to get a child/teen to wear the thing for practically 24/7 year after year, especially during those self-conscious years.

JS shows up during early childhood and has many years to progress. As the spine grows the growth tends to go into the curve, rather than height. During those years doctors again usually prescribe bracing or casting, the same routines they've used for decades to treat this mysterious condition. And when it gets bad enough they resort to fusion.

Ben's curve was already at 28 degrees when he was 8. It didn't improve after the Chiari surgeries. But it apparently moved slowly. A year after surgery it was still the same. But by January '09 it was 35 degrees. Fusion is recommended at 40 degrees. And once fusion is done no growth happens in that area of the spine. Fusing a pre-adolescent spine is NOT a good idea. Think of the issues down the line.

We read as much as we could on the topic in the early part of this year. We saw doctors at Children's Oakland and UCSF. We were told to put him in a brace 22 hours a day until he stops growing or until he reached 40 degrees. We were told that the brace was not greatly effective with cases such as his. He hadn't even begun his adolescent growth spurts as far as we could tell. Brace him until he stopped growing? What is that 7, 8, 9 years? That seemed impossible to adhere to. You see the trouble with treating juvenile scoliosis. You're talking about years of someone's life spent in a hard plastic corset.

One piece of research we saw said that children who have reached 30 degrees + by age 11 have a 98% chance of needing fusion before they reach full growth. Sobering numbers.

Our research took us to Shriners Hospital in Philadelphia. The doctors there are doing numerous innovations with scoliosis techniques. When we went for an evaluation in May the nurse and doctors spent hours, literally, looking at a variety of information about Ben, including 7 x-rays, and answering our questions. (One piece of very important information we got was that Ben's score on the skeletal maturity Risser scale was a 1 out of 8, indicating that he has maaaany years left of growing.) At the time we were told he was an excellent candidate for VBS (Vertebral Body Stapling) which is titanium-nickel staples inserted into the vertebrae to act as an inner brace. They give support and allow for growth and flexibility and at the least stave off the need for external bracing and fusion surgery during the growth years. The surgery is major, but not as difficult as fusion, and the result more optimistic for a child. We were also told his situation wasn't a "red alert" situation, meaning we had time to wait, see how he did with a brace (16 hours a day--not to solve the problem, but to slow the curve down), and still be able to do the VBS before the curve was too far gone.

VBS is a new technique. They innovated it at Shriners Philly about eight years ago. It's had a lot of success, but it's still having the kinks worked out of it and most orthopedic surgeons don't do it, recommend it, or even know about it. We felt that the fact that it gives Ben time to grow and live brace-free was positive enough put him through it.

From April until recently Ben has been seen by an osteopath and a Feldenkrais practitioner as we attempted to work on the situation from a non-invasive perspective. We were hopeful that those treatments were doing something positive, we hoped even that they could reverse the progress of the curve.

But, earlier this month an x-ray showed that the lower part of the curve had indeed worsened. And last week our surgeon at Shriners told us that their research now shows that with curves as high as Ben's, VBS isn't enough. He is now recommending VBS/Adjustable Hybrid Rod surgery and we are tentatively scheduled for it on February 15, 2010.

The hybrid rod is a titanium rod that is literally hooked onto one of his ribs at the top and then placed into one of his lumbar vertebrae. It hangs onto the ribcage in concert with the spine to keep it from curving over more. The strength of the rod and the staples seems to work and the kids who have had this surgery are (so far) successful at keeping the curve from progressing.

It's a VERY new technique. Only about 2 years old at this point. And it has a huge disadvantage. The rod needs to be lengthened as he grows. It's an adjustable rod, remember? As Ben grows he'll need to have the rod lengthened, so that it doesn't restrict his spine and ribcage. This means having a small surgery every six months or so until he stops growing. Remember the Risser score of 1? And the fact Ben's only just turned 12? That means potentially 14 surgeries in the years ahead.

14. One. Four. I can't quite get my head wrapped around it. But my heart is really feeling it.

Ben has already walked a hard road. He's being stoic right now, but we're working on feeling the feelings that come with this story. (He brings up the happy fact that all those trips to Philadelphia mean we can eat a LOT of cheese steak sandwiches!) If we had a crystal ball, we'd know how it ends, but alas, that's not the way life works.


Jenny said...

Oh my. Fourteen? Wow. Please let me know if we can do anything to help.

offshore said...

thank for the post.chiari malformation scares me when i first know it on