Selective dorsal rhizotomy update

Well, we finally got a decision on Alex’s selective dorsal rhizotomy. The Mayo team has decided not to proceed with the SDR at this time. An SDR will remain “on the table” as an option to consider over the next year or two, but only if we see significant improvement in the strength and control of Alex’s torso and head.

Here’s what we understand about the no-SDR decision. Right now, the Mayo team is observing significant weakness in Alex’s torso and neck; they also note that he fatigues very rapidly when he is walking. By relaxing the muscles in his legs and lower torso, an SDR would make it even more difficult for him to maintain an upright posture. He would definitely recover some strength and control over the year following an SDR, but the concern is whether he would be able to recover enough strength and control to be functional. In the words of his doctor, Dr. Brandenburg, his physical medicine and rehabilitation (PM&R) doctor: “The concern is that if we take away the tone in his legs and we don’t have the tone in his head or trunk, he won’t be able to stand in his walker.” In order to be more comfortable that he would function better, not worse, after an SDR, the Mayo team wants to see a higher baseline of torso and neck strength prior to giving an SDR a green light.

Will he ever have an SDR? Maybe; the option will remain on the table over the next year or two, but it is definitely not a sure thing. The feeling is that he would have to demonstrate a lot of improvement. Toward that end, his doctor is suggesting that we really focus on seeing how much stronger he can get in the next year, and especially over the summer. She notes that most kids have more than a year and a half of therapy to build up their strength prior to an SDR; Alex hasn’t had that, nor has he had the benefit of being active when he was younger, so it is possible that with continued intensive therapy over the next year, we will continue to see good improvement. But again, it doesn’t seem “likely” at this point.

Will he ever walk? Based on what we’re seeing from the gait lab analysis, it seems unlikely that he will ever walk without a walker. Even an SDR or other intervention would probably just improve his function in a walker, not get him out of it. Moreover, especially as he gains weight, Alex’s preference is probably going to be to use a wheelchair. As Dr. Brandenburg said, for him, walking uses the same amount of energy as running does for us. (We’re also aware that improvements in robotic prosthesis technology over the next couple of decades will probably see him walking at some point down the road; see this article for where that technology is at now.)

What’s next? The short version is that we will be zeroing in on strengthening the torso and neck muscles. We’re also going to be having conversations with Dr. Brandenburg and his school and Winona Health medical team about how to move forward to improve Alex’s daily functioning, so that he can be “the best Alex he can be,” as his doctor put it. He is going to get back on Botox and phenol (he will probably go for that on May 8), and over the summer we will be ramping up his baclofen. We will be looking at his equipment needs, too. Down the road, we will revisit the possibility of an SDR; other options that Mayo is interested in considering include a tendon release (cutting tendons to reduce scissoring) and a baclofen pump (implanting a pump to deliver baclofen directly to the affected muscles).

I want to say that Susan and I are not disappointed by this outcome. We have known from the beginning that walking independently was not a sure thing for Alex, so this doesn’t come as a surprise. Our goal has always been to help Alex realize his full potential: to participate in the life of community as much as possible, to contribute his gifts to the world, to love and to be loved. That is our focus, and the particular way that happens is less important. One of the chapters in Susan’s new book looks at the theology of disability, the upshot being that even disability can be used by God to disclose the divine. Not that God wants Alex to be disabled, necessarily, but his plan is to take that disability and turn it upside down and inside out, so that something good comes out of it. We are privileged to be able to participate in that plan, and to witness all the “good fruit” coming from Alex’s life.

Gait analysis clinic

"This is not as fun as it looks."

Earlier this week Alex went to Mayo for a gait analysis clinic. It was a LONG day. "LOTS of appointments," he kept saying.

The big one was the actual gait lab, which involved four adults (not including me) working to make Alex the bionic boy for a few minutes. After taking lots of measurements where he tried to stretch, extend, and bend as far as he could, they began attaching microphones to his legs and little silver balls all over his body, but especially his legs. Cords were attached to the microphones, and when he moved a leg, the computer registered the strength of the movement in a variety of muscles. By the time has was all "hooked up," they picked him up and asked him to walk (with support on each side). He would walk two second, then one of the silver balls would pop off (because he is scissoring so badly all this electronic gear is getting bumped). They would redo, set him up to walk, and we'd get two-four seconds, and boom--stop, lost a ball, or lost the signal on this microphone, etc.. I honestly thought it was a bit touch and go, because Alex was getting tired, and they were having a hard time getting the readings they needed. In the end, they said they didn't get everything they wanted, but they got what they needed. Then they took bionic gear off Alex, and asked him to walk (with one person support) for videotape. He did better on that. So, two hours after we started there, we were off....

Reattaching electrogear in the walking....

After lunch, we went to "Mayo 16 East" for appointments with the social worker, his PM&R doctor, and an assessing PT. Oh, and a pelvic xray. The xray was short, but the appointments were all an hour long. Alex tends to be very good at these appts, but he was really flaming out by the end of this day.

Anyway, our PM&R doctor spoke to us more about the selective dorsal rhizotomy, and if I get anything wrong here, blame me, not her. The way it is done at Mayo is that these surgeries occur on Fridays, and total going into surgery and back in recovery time is about 4-5 hours. The surgery involves going into the spine and isolating nerves that send messages to legs. They want to cut the nervelets that send the spastic messages. They also cur sensory nerves, not motor (?) nerves. They test each nervelet as they do this. They do not cut more than 70% of the nervelets--and in general, as few as they can. But even if an area has 100% spasticity, they only cut 70%. After that point, it is very hard for a person to recover strength. You can read about selective dorsal rhizotomy in great detail at the St. Louis Children's Hospital -- pictures and everything.

She said he would initially seem much worse--kids who have this done are very "floppy." The initial recovery is to mend the spine, but she said they do light physical therapy the day after surgery (very light). And epidural is helping a lot Saturday and Sunday. Monday, barring complications, we go into a 3 HOURS OF REHAB routine 5 days a week for two weeks. Weekends are off. Then we get busted loose and we continue 5 days a week PT at home through Winona rehab services...although 45 minute sessions then. She said the daily PT (except weekends) is to be expected for the first three months, and the full effect of the rhizotomy would not be seen for a year.

In terms of surgery recovery, this is shorter than the rhizotomy that is done at Gillette (6 week in hospital recovery). She explained why and the pros and cons of each procedure, and I could see a great debate among medical professionals about which to do (she explained them very objectively, I must say). We asked if she was confident with the results she sees at Mayo, and she said absolutely.

They have not decided to do this yet. March 20, the doctors there meet to discuss whether he is a candidate for the procedure. (Basically, if he is too dystonic--that is, he has too much mixed tone--this will not help and will make things worse.) The assessing PT was friendly but concerned that he has very low tone in his trunk. She volunteered that she was seeing him at the end of a long day, never a good time to make assessments, and said she would check the video that the gait lab produced. We also talked about how this has been an issue since we adopted him, but despite appearances, he has made real improvements. So on March 20, the PM&R doctor said she would call us and let us know what the medical staff decided. And then would ask us whether we wanted to proceed. If we say yes, we get to visit Mayo A LOT in the next few weeks for pre-op procedures and another MRI. Surgery would be on a Friday in May; we would hope for May 2, but we are not guaranteed that date, as these things are difficult to schedule. After the surgery, one of us will stay with Alex in Rochester for 2.5 weeks.

They are all talking like it will be worth it in the long run. The advantages of this surgery for Alex is that it will help prevent (more) muscle contractures, decrease spasticity pain, and give him a better shot at walking well in a walker (when Jerry raised walking with crutches/canes, she said she didn't really see that in his future, which doesn't surprise me). And so, we wait. We're depending on the doctors here to make a good decision about whether this works for Alex or not. If he is not a candidate, we're back to Botox injections and maybe a baclofen pump (a move they and we are not thrilled about perusing).

If you want to see more of the gait lab, see the video below.
--Susan