Thursday, January 29, 2009

Working on things

I've shot a lot of video and pictures related to where Elena is now, hopefully to compare to where she will be in the future. I'll also be adding/fixing the timeline at the bottom of the page. Just wanted you all to know I haven't forgotten about that stuff. Being on bedrest prior to a crazy delivery and returning to work after Elena's sister Vivian was born was a big blur. Anyway, I should be updating weekly from now on.

To SDR or not to SDR, that is the question

Parents of kids with spastic diplegia know that fighting to get into their shoes/AFOs is a daily struggle. This was ameliorated for a while with Botox injections. Botox allowed Elena to have her feet flat for the first time, which allowed her AFOs to form an arch in her feet (previously she pronated so badly she didn’t have one) as well as give her a better foundation for balance. We had three rounds of Botox in total. Her last one (July 2, 2008) hardly took effect, despite the fact that more muscle groups were targeted (hip flexors and adductors in addition to the usual calf and soleus). We still had to struggle to flex her feet to put them in her AFOs—it was only marginally easier post-injections, and only for a month or so. Her AFOs were on the verge of being to small, and they were “old and worn”, as far as Velcro fasteners and plastic molding goes, so it didn’t take long for Elena to be back on her toes after putting on her AFOs/shoes.
I knew SDR was an option for Elena, I have since our orthopaedist told us after Elena’s second botox injection. The question for me was when to do it; I didn’t know (at the time) when Botox would stop working, and I was pregnant with our second child. Our doctor said to consider SDR when Botox was “off the table”. That was August of 2008. He amended that to “when Botox was off the table and AFTER the new baby is born”.

What is SDR?

Selective Dorsal Rhizotomy, for those who don’t know, involves severing nerve “rootlets” from the spine responsible for overstimulation to the muscles in the legs. Effectively, it can “permanently” (this has been debated) reduce spasticity. It is not a cure for cerebral palsy, and it is not recommended for all people who have CP. Good candidates for this treatment have all or most of the following: relatively symmetrical spastic diplegia, muscularly strong (not to be confused with just being “spastically tight”), easily motivated for PT, and still be young (preferably between ages 4 and puberty), and have parents that are committed to an intense PT schedule post-op. Elena is/has all of these. St. Louis Children’s has a great site.

SDR is not performed at every hospital—the percentage of patients who get this surgery is quite small, so even if a surgeon is trained, they may not have lots of experience with this procedure. Since it involves a judgement call, experience is a plus. Since it is “permanent” (quotations b/c spasticity can return, but not as great, and it takes a long time), there is a real risk of trading strength for mobility—kids can have the range to move, but be left without enough nerves to stimulate the muscles to support the desired movement. The ideal is to have a normal range of motion with the strength to support it, and that is not always achievable.

Team Opinions

My first course of action was to poll everyone who had anything to do with Elena as to their opinion of her as an SDR candidate:

Orthopaedic (surgeon): Strongly Pro. (Of course! He cuts into people for a living!) He said she’s a great candidate, and it would reduce her need for future surgeries (not all).
Neurosurgeon 1: Pro. Probably strongly pro, but he’s not pushy. He thinks she can really benefit from the procedure, and it should alleviate the need for some (probably not all, but maybe) future surgeries.
Neurosurgeon 2: Pro. Probably strongly pro, but also not pushy. Said that he thought E would benefit tremendously, but reminded me—cutting nerves is a very painful procedure. Said that E was a natural with PT and her movement patterns were good, and he has never had a patient that wasn’t the same kid (psychologically) post-SDR as they were pre-. The longest it took a kid to mentally recover, in his practice, was 2 days.
Main PT: Cautiously Pro. She understood Elena was a good candidate, but she was concerned about interrupting her current progress (PT and school) and wondered how she would cope psychologically. Wanted her to be older/stronger.
Orthotist: Pro. He said future bracing can’t keep her in her shoes, and SDR would allow her to have a good support for balance, and would help keep her soles/feet in line, therefore preserving her knee and hip joints as well as possible.
Auxiliary PT1: Cautiously Pro. Had lots of experience with kids pre/post SDR. Strongly Pro if using the head surgeon from her former location.
Auxiliary PT2: Con. Worried about the permanency of the procedure. Elena was already so functional, why mess with what works (albeit, works not as well as those without CP). Didn’t think it was worth the risk.
Auxiliary PT3: Pro. Thought E was a great candidate and would benefit tremendously. Not worried about E’s psychological state, said she was one of the best prepared she’d worked with.
Auxiliary PT4: Con. Thought it was better to work with what we had then try to give Elena a new body to learn to move. Said there was never surgery without risk. Interestingly, he is also impaired (including legs) from a brain injury during adolescence.
Developmental Pediatrician: Pro. She’s distant/impartial, but seems to think it’s a good idea.
Regular Pediatrician: Pro. He thinks we’ve weighed all options and thinks SDR is the right call, given her tightness and strength and motivation.

Finding a good neurosurgeon

Before her last Botox shots (July 2008), I decided to go on a hunt for a neurosurgeon do perform SDR.
I checked here, at UVA first. Dr. Elias does SDR. He does about 3 a year. He is one of the top neurosurgeons in the country, but his specialty is epilepsy. I liked him, but wanted a second opinion—how was I supposed to figure out if he was the best person for the job?
The recommendation from one of Elena’s PTs was for Dr. Tae-Sung Park. She used to work with him, and he has seen every type of CP kid there is. He is located at the Children’s hospital in St. Louis. He has “pioneered” what is the modern-day SDR procedure (smaller incision site, etc) and hands-down has the most experience. This seemed just fine, except for the fact that I am absolutely scared to death of flying, and our-in town neuro (Elias) was taught by Park. I wondered about Dr. Park’s age and notoriety—he could retire, and Elena isn’t exactly a high-profile case. She’s not going to be some poster-child for impairment-to-normal-gait type of change, so I wasn’t sure if Dr. Park would actually be the one to operate. If not, well…maybe his team would be better than other surgeons, since it’s Park’s team. How was this different than Elias? Hmm. St. Louis also does NOT take my insurance. The procedure runs about $50,000. I didn’t want to make this about the money—we would find a way to pay if Park is the best person for the job. But I wasn’t sure if that was true.
Our orthopaedic surgeon didn’t seem to recommend Dr. Park. He said he had changed his criteria for selecting SDR candidates, and had become a little more lenient in his selection. Meaning, if you weren’t as strong or symmetrical or whatever, he’d still consider SDR even though other surgeons wouldn’t. This was a red flag for our ortho. He had a lot of confidence in Elias. I still wasn’t convinced that there wasn’t some bad blood or something between our ortho and Park, so I continued to look for information.

I made an appointment at Duke neurosurgery. I spoke to Dr. Fuchs, who does more SDR procedures/year than Dr. Elias does. He’s very easy to talk to and spent a lot of time with me. He echoed our orthopaedist’s concerns regarding Dr. Park. Not because he had any personal problem with the man, but because he considered Park to be more aggressive of a neurosurgeon than he used to be. For a child like mine, this was a big deal—Elena is highly functional, and I wanted a more conservative surgeon (that’s not to say that Park wouldn’t do an excellent job). Fuchs had knowledge of Elias, and had very good confidence in him and the UVA staff. He also mentioned the truth of having everything Elena needed in my home town, and the benefits of staying local. He also said the best way to find out about a surgeon for something like this isn’t to ask the surgeon or their staff—it’s to ask the patients. That’s slightly problematic, given doctor-patient confidentiality, but some centers will contact former patients to see if they would be willing to talk with me. So that was my next job.

Over the next few months, I had the opportunity to talk with 5 mothers of SDR patients. 3 of them had their procedure done by Dr. Park, and couldn’t be more happy with their experience. One child was 2 yrs old when she had SDR, and her legs were “scissored” all the time—she’s going to college this year, and was on the cheerleading squad in high school, and was skiing at the time I talked to her mother. She uses loftstrands for long distances, and that’s it. Another was a boy who had it when he was 6 or 7. Now he plays baseball—granted, he’s not the best on the team, but he’s ON THE TEAM. He’s had 2 heel cord lengthenings post-SDR. Another was a girl who had it done when she was 5 or so. She uses loftstrands now also, but can run and jump and all that. They all did warn me of the immediate aftermath of SDR, with their children slumped over in wheelchairs, drooling, due to weakness/change in balance. These candidates seemed more impaired than Elena, although the two older children could already walk before the procedure.
The other two had their surgeries with Elias. They did not experience any of this extreme “floppiness” post-SDR, although there is no way to determine if that is due to the child or the surgeon (or anesthesia). The first child was a boy, he was 3 at the time of the procedure, and he could not walk at all independently and was crouching very very badly which made using a walker very difficult. He was officially GMFCS 3. A year later, he is taking a few independent steps at a time (5-10) and no longer crouches. They have been doing PT (or some other athletic activity) 5x/week since the surgery. The second child, also a boy, had the procedure done when he was 5 and his feet turned so badly inward (pigeon-toed) he fell constantly and could not walk on any surface that wasn’t perfectly flat and was still in diapers. Now, even though his feet are still turned inward, his balance has improved amazingly and he can run up hills, curbs and run and jump and is now potty trained.

All the parents had intense praise for their respective surgeons.

Now What?

Here’s what I know: Elena is a good candidate. She’s great with PT. She’s had to get used to new legs twice already (post-botox) and did fantastically. She’s had three surgeries already (eyes), and isn’t nervous about doctors b/c we are honest with her when she asks questions about her operations. I was on bedrest for 2 months, and she saw in the hospital and understood why I was there, why I couldn’t move, and that I’m fine now. I know that if we do NOTHING, Elena will be alright (mentally). I also know that she absolutely will have future surgeries, to lengthen her tendons, or fuse her ankle bones, or fix a hip dislocation or something like that if we don’t do anything. She’ll probably need surgeries even if we do SDR; but SDR has the potential to alleviate the spasticity that would cause lots of future problems. I’m pretty sure that it’s not going to get easier for her to psychologically deal with the surgery or PT afterwards for many years—and then, it may be too late to avoid/lessen issues related to tendon length or joint stability. She is strong now, motivated now, young now, and her body (jointwise) is in great shape now. girl Elena is a wonderful child. She’s happy, even though she knows she doesn’t move like the other kids. But she gets tighter all the time.

My anxieties revolve…

E's a great kid.
She moves pretty well for a kid with CP.
She's getting worse.

I have a chance to give her the best, most normally functional body she can have. If there is always a risk with surgery, I have a chance to reduce the number of surgeries=reduce the number of risks. Now is a good time.

I scheduled her surgery date for February 18th, 2009 with Dr. Elias. I picked him for several reasons: he was local, he was well recommended, he was conservative, he’d seen Elena several times before her procedure so he knew her functionality well, he was plugged into the PT/children’s hospital network here, UVA takes my insurance, and Elena’s friends and family and therapists are all close by.