A Conversation with Ben's surgeon

Mark and I didn't get a heck of a lot of sleep last night. We'd set the alarm clock for 6:30 because we had a scheduled phone call with Ben's surgeon at Shriners, Philadelphia, at 7 a.m. Pacific time. I love my "happy pills," though. Xanax. Little peach colored tablets of peace and tranquility. Can't recommend 'em highly enough. Got me through Ben's two months at Children's Hospital four years ago and they're going to get me through this, too. Suffice it to say, I got more sleep than Mark.

So, out of bed at 6:45. Even brushed my teeth. Practiced talking so we wouldn't sound like cavemen when the phone rang. Woke Ben up. (He was adamant about listening to the conversation.) He joined us in the office. (Didn't last through the conversation, by the way. TMI.)

Thrum...thrum...that's our fingers drumming on the desk. 7:12 a.m.

Thrum...thrum...7:22 a.m. Checked emails, no last minute changes from the nurse.

[We first found out about the change in Ben's upcoming surgery a week and a half ago. The worst week to find out something that you need to talk to a doctor about immediately! He was away for the holidays, so we've been waiting patiently for this day to arrive, for our scheduled conversation in which we can ask the 57 questions we've written down (and the few we didn't think to write down). So, you can imagine how hard it was to wait this morning for the phone to ring.]

Thrum...thrum...7:28 a.m. Time to give the hospital a call.

He had it on his calendar for 10 p.m. (Philly time) Glad we took action, it's the kind of people we are. Otherwise we'd be sitting here at the computer picking our noses for 12 hours. Not really productive.

But I digress. (It's a technique. Called "diversion." Get used to it.)

Thankfully, Dr. Cahill was apologetic, patient and had some time to talk. We asked every single question on the list, and a few more. (Reminiscent of our trip to Shriners in May when we spent 3 hours at the spine clinic, all three of those hours being seen, x-rayed, checked, talked with, etc. Definitely not being ignored or quickly moved out the exit door as we had at Children's Hospital.)

Before the talk we'd done as much research as possible. But the technique is new and there isn't much out there on it. Most of what I got was from Susanne, Dr. Cahill's nurse, and from the VBS forum I am a part of. We actually didn't find out much we didn't already sort of know, except one BIGGIE: He will only need to have 2 or 3 lengthening surgeries, not the 14 we feared. (More on that below.)

So here's (finally) the skinny on what Ben's in for, ask questions in the comment area, if you have any.

Surgerical technique: Vertebral Body Stapling with Hybrid Rod

     **This is all very new, innovative and experimental. The staples have been used for about 8 years and the rods for about 1 1/2. Only 12 kids right now have the VBS/Rod combo.**

Purpose: The staples pull the vertebrae straighter, and don't allow the spine to curve more in their predisposed direction, while the rod pushes up on the concave side of the spine to give additional support to the staples/spine. Also, to allow for spinal flexibility long-term without fusion. The rod is adjustable, so it will be lengthened as he grows.
Why the rod?: Ben's curve is greater than 35 now and at that degree of curvature the staples can't do the work on their own.
Rod details: Titanium. It's about 14" long (!) and about as big around as a pencil. It's place about an inch or two to the side of his spine, hooks onto a rib and the lamina of one of his lumbar vertebrae.
Staple details: Titanium nickel memory metal. Open up when iced and go back to their intended C shape when warmed up to body temperature.
Surgery details: 6 hours all together. Rod first. Inserted from the back. One small incision in the small of his back. One longer incision (4") in his upper back, over his ribcage. VBS next. Ben will be turned on his side, his lung collapsed, and several small incisions will be made in his side. Staples inserted from there. A chest tube will be placed at the end of the surgery to reinflate his lung.
Recovery: 5-7 days in the hospital. Chest tube removed day after surgery (this is apparently the worst part). Catheter removed day after surgery. Breathing exercises are the most important part of in-hospital recovery.
Lengthenings: Every 6-9 months for a couple years. At that point his spine will hopefully have acheived the majority of its growth. We'll assess it at that time. Remember, this is a VERY new technique, so "we'll just have to wait and see" is going to be the answer for a while.
Lengthening surgeries: 2 hours in and out. Overnight stay in the hospital and then home.
Long term goals: No more brace wearing. The rod is removed at the end of the growth. (No fusion occurs.) Staples are left in. Ben's spine is straighter and he didn't need fusion and has flexibility and full growth.
Risks, overall: The rod breaks after it's been in for a while (several years) and needs to be removed or replaced. Infection, especially with repeated surgeries.
Limitations in activities: NO sky-diving, bungee cord jumping, trampolines, off-road vehicles, jumping on horseback, rock climbing, ice hockey, contact football/sports, possibly Aikido.
What if it doesn't keep his spine from getting more curved?: Fusion surgery.
What if we get to Shriners in February and it's already at 45 degrees?: Fusion surgery.

Plan for February's surgery:
Susie, Mark and Ben fly to NY and stay with Grandma Joyce Feb. 11-13.
Go to Philadelphia Feb. 13.
Check-in Feb. 14 for x-rays and pre-op stuff.
Surgery Feb. 15 7:30 a.m.
Check out Feb. 21-23.
Stay at Cousin Hana's until post-op follow up visit, around Feb. 28.
After that, Mark returns home and Susie and Ben go up to NY to stay at Grandma's for another 5 or so days.
Susie and Ben return home around March 4.

That's it for now!
(Gotta go feed some very hungry animals outside...poor guys!)

Morning, Sebastopol

sunrise behind the treetops on our ridge

Post holiday blur

I can't seem to get my head around what day today is. I keep thinking we've already passed through New Year's and are into the new decade. Christmas seems like ages ago...but it was just a long weekend. I'm not really sure what's up with this faux-time-warp, but maybe I'll just accept the fuzz and go with it.

The holidays went by in a blur, but with much happiness. I decided a few months back to have a Handmade Hanukkah (or "Chanmade Chanukah" depending on how you like to spell it). So I knitted, sewed, and papier mached, practically every gift I gave. I also got the boys busy with gifts for family that took some energy and creativity. Harry made ducktape match boxes and Toby and Ben made a watercolored matching game for their little cousin Hannah. (I seem to have deleted those photos from my camera and can't find them on the computer, but if they turn up I'll post them here!) Whenever we get invested in the creating of the gifts the focus of the holiday goes from GETTING to GIVING and it makes all the difference.

Our Hanukkah party was cousin-ful and terrific. We ate (latkes, roast chicken, homemade applesauce, salad, and cheesecake) and were merry and then spent a lot of time opening presents and oohing and aahing over each gift. I made purses and market bags (I was a crafting lunatic in the days and nights before) for several folks. They were received with delight, I'm happy to say. The big surprise present wasn't handmade, though. It was a trip to Disneyland that Mark and I gave the boys (January 8-11). It was out of the blue and we're all excited! We decided that with everything coming down the pike these days we need a little F.U.N. to look forward to.

Another joy from the holidays was that I was asked to teach my sister, Mara, and my cousin, David, how to knit. Now, I still consider myself a beginner, but I could at least show them how to cast on and do a garter stitch scarf. We'll see where it goes from there. I was happy to at least pass on my enthusiasm for the yarn-art.

Over the weekend we saw some old friends, new friends, and family. Mark's sister Sharon and her husband, Alan, came up for a visit and we took them out to dinner for a belated 50th birthday celebration for Sharon. Here's Sharon in the capelet I made for her. It fit perfectly, I'm so happy! She loved it!

Harry went off to his winter teen meditation retreat today. A welcome respite for him and for us. I appreciate the time away from each other and he comes home transformed. Having a teen is, um, challenging!

The rest of our week is looking calm, mostly because I didn't plan anything. I am scheduled for jury duty, though it's unlikely that I'll have to serve. Hopefully, it will be painless and we'll just get to enjoy the open time in our calendar. Then next week we'll blink at the bright and shiny new year and say, "How did it get to be 2010?!!!"

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.

New Bends in the Road

(Photo at left is of Ben in his ice hockey helmet!)

Oddly enough, I feel the desire to rev up this blog's engine again. (Never really got it going before, but my previous posts were pretty sweet...) We are going through some challenging times in our family now/again and rather than repeatrepeatrepeat myself on email, on the phone, in person, I think it will be easier to have you come here to check in and find out the scoop.

The challenges are medical and with Ben again. In February, a new x-ray was taken of his spine and we discovered that his scoliosis (spinal curvature) has progressed from 28 degrees in 2007 to 35 degrees. This is not happy news. Since then we have been searching for a treatment plan and it's still not clear what we will do.

To back up, in late 2005 we discovered Ben had scoliosis at his annual checkup (8 years old). We went for x-rays and were sent on for MRI's (to rule out worse conditions). Turned out we had one: Chiari I Malformation and Syringomyelia. This means his cerebellum was too big, blocking the flow of spinal fluid from his spine to his brain. They Syringo part means that there was a pocket of fluid (syrinx) trapped in his spinal cord and as it expanded it pressed on the nerves of his spine and created a weakness there. Hence the scoliosis. Or so we thought.

We went to Children's Hospital, Oakland, for treatment: a decompression surgery (brain surgery) on February 1, 2006 and after way too much trauma and drama (i.e., infections on the incision site, infection on the brain tissue, fevers that wouldn't go away, leaking spinal fluid, wounds that wouldn't heal, four surgeries instead of one, etc.) finally returned home (to stay) on April 1, 2006, a full two months later.

He was monitored by the neurosurgeon at intervals throughout the following year. And at the last MRI (April 2007) we were told no need to come back to neurosurgery for at least 2-3 years. BUT, we were not told to monitor the spine and so, it wasn't until we went in to see our neurosurgeon, Dr. Sun, in February (because Ben was having recurring headaches) that we were asked to do an x-ray.

Now that we look back we really wish we'd had more information. Like, the fact that since there's so much scoliosis in both sides of our family it's quite possible that Ben has a genetic type of scoliosis. That we should have been monitoring Toby and Harry as well. That Ben should have been monitored with x-rays more frequently over the past two years so that we would know if this has been a gradual change or a rapid, recent one. That perhaps Ben should have been in a brace since after the Chiari surgeries.

It's hard to know.

We've now taken MRI's as well. The Chiari has not returned (it can), thankfully. The headaches have gone away (not sure why). What we know now is that there are several paths we can take to address the scoliosis (whatever it's genesis) and none of them has a clear outcome and only a few are palatable. After what he's been through. After what we've been through.

Anyways, enough for now. Later I'll post our options as we know them now, and you can always check back for updates.