Spina bifida is a birth defect. There are three types, and my sister was born with the worst kind. myelomeningocele. There was literally a hole in her lower back. She was born in 1970, before there were ultrasounds and much prenatal screening that happens today. These days the evidence suggests that most of the damage done to babies with spina bifida comes from the amniotic fluid getting into the spinal canal. Nowadays, fetuses are operated on to close the hole to minimize damage in utero. There can be mental/learning disability defects in addition to physical ones. My sister was lucky. She eventually learned to walk by age 3, and was never bound to a wheelchair. She did not have hydrocephalus. She had frequent bladder infections, but those faded with age. Then in 1987, she started having something that looked like seizures or spasms. Back then doctors called them “myoclonics”, but they are not called that today. They started in her legs. Would last for a few minutes and then subside. They kept getting worse, eventually involving her whole body. It would contract violently and then release. Contract and release. It was horrifying to watch. I’m sure it was even worse to endure. She eventually saw a neurosurgeon who had seen this before, and first heard of a “tethered spinal cord.” That’s when scar tissue develops around the spinal cord and puts pressure on the nerves. You can imagine, in a growing child, scar tissue pulling on things is not good. The surgery was a success, and her symptoms were relieved.
Unfortunately, any kind of surgery leads to the formation of more scar tissue. To date, she’s had 3 more untethering surgeries. Also unfortunately, the more you operate on the spine, the greater the chance of nerve damage. Especially back in the 90s, before they had the kind of monitoring they do today that can make sure they’re not cutting nerves. She now can no longer feel her feet or the back of her legs. She had to go through physical therapy to learn how to walk again. She walks with a cane, but still walks. Back in July, they were trying for her 5th untethering surgery… but the monitoring revealed that now all the scar tissue was attached to nerves. If they had cut anything away, she would be paralyzed from the waist down. So they closed her back up and did nothing. She would have to move forward with pain management to alleviate her symptoms.
Naturally we were all devastated. But it seemed her symptoms did initially get better on their own and she was able to make to the pain clinic. But now she had a new problem… Because of her multiple surgeries, the dura that surrounds the spinal cord and the skin over her back are both very thin, fragile, and they were hard to close after the incision in July. She was still suffering from low CSF headaches, and there was a bulge in her lower back. It was determined that she has a CSF leak. Meaning, there was a hole in the dura, and CSF was leaking out of the spinal cord into her back. It was still held in by the skin until last Thursday, where a tear finally formed in the skin. Leaking CSF fluid out your back is a very scary thing! Her clothes were soaked. It was the middle of the night and she hasn’t been able to drive since April, and her live-in boyfriend is wheelchair bound and also can’t drive. She had to call an ambulance to get to the hospital. Fortunately, we have been reassured by numerous people that a CSF leak, while serious, is not life-threatening nor something that will lead to nerve damage or loss. But she’s in intense pain with headaches.
It’s been an agonizing week with neurosurgeons trying this and that to close the hole now in both the skin and the dura. Nothing has worked so far. Today they are finally going to implant a shunt in her brain to try to get the leak to reverse flow so it stops filling into her back, which should allow the dura to dry out and heal closed on its own. It’s a gamble. We know. But at this point, there are no other good options.
Tensions are high. My mother has been driving me crazy with questions and googling answers and thinking she knows everything and the doctors know nothing. We had a big fight last night. We’re not speaking. I’m feeling pretty low…
Swimming against the tide 