Baclofen

Baclofen is a medication for treating spasticity that Keira has been taking for about two years now. It was initially prescribed at a very low dose by her physiatrist, who told us it was best for us not to Google it and, rather, ask him if we were worried about the risks. To put it simply, Baclofen has serious risks of overdose and/or withdrawal. Patients also tend to require higher and higher doses when using it long term. We suspected going in that if Baclofen worked for her, she may eventually need a pump.

We saw good effects from the drug pretty much right away. Keira’s muscles were looser and she was more relaxed over all. Initially, she was extra enthusiastic and giggly, as if slightly inebriated, but that part subsided. Therapists noticed that her volitional movement increased and she was less dependent on her primitive reflexes and tone. It became clearer that with the decreased tone also comes less vomiting.

Over the past 2 years, the physiatrist guided us as Keira needed a higher and higher dose of Baclofen more and more often. We eventually added a fifth dose to be given nightly between 2 and 3 a.m. We can bet that without that dose, she'll wake in the morning in a puddle of vomit and will be extra irritable.  

In April of this year, we had already reached the point where the doctor did not want to increase the prescription any further. He schooled us on the ins and outs of the pump and we decided to take the next step, an inpatient “trial” to see if the pump would be a viable option. We left that visit and waited for a phone call to schedule this one-day experiment in the ICU. 

Weeks passed with no phone call and I came to find that Keira’s physiatrist had had a “family emergency” and would be out of the office for several more weeks. Weeks turned to months. Repeatedly, there was a day set for his return and then he would not meet it. There was no other physiatrist or physician competent to handle Baclofen issues in the Boise area, even at the competing hospital.  

Keira's physiatrist was not even available for consultation or in case if emergency. I felt for his poor office staff who had to handle all of the phone calls from parents like me. I was a worried mess come July when Keira was due to have her next hip surgery. I was getting mixed messages with some clinicians advising that the baclofen pump surgery should come first, before the hip surgery so that Keira's high tone was less likely to damage the effects of the hip reconstruction. The hospital was supposedly working on credetialling another physiatrist so there would be some backup, but this was going to take months. We felt we had to do something, so we went ahead with Keira's hip surgery in hopes that the pump surgery would be close behind. The hip surgery went beautifully, but when the cast came off after 6 weeks, Keira's old patterns resumed in the way we feared, which threatens the longevity of those reconstructed hips.

The whole situation with the physiatrist felt so strange. I suspected that the doctor had either ended up in rehab or in jail. Otherwise, how could he suddenly be so completely unavailable? It also felt very tenuous. Here I am in Nampa with my child in this serious drug with no MD to help us any closer than Twin Falls. Nobody. 

Finally, in October, just as it was looking like the backup physiatrist would get credentialed before ours would return, he died. Suddenly and unexpectedly. 

This was heartbreaking. This specialist had really felt like part of our team. He was one of the first specialists that really seemed to get it. I loved him because he didn’t beat around the bush. He wasn’t afraid to call it what it was. He labeled Keira "medically fragile" and stated that we could never be certain whether she would wake up on any given morning. And at the same time, he wanted her to be happy and comfortable. He referred us to palliative care, one of the best connections we've got. He always asked how the kids were and recommended that my husband and I go on “wow” dates. One of our people, one of Keira’s people, was gone. 

I was so disappointed. I couldn’t help but think that he must have committed suicide. Or overdosed on drugs. I have no sound basis for this presumption. It’s just a feeling I get after trying to read between the lines. His obituary was sparse; little more than birth and death dates. I feel so sad for him and his family and his patients. But also, frankly, a little angry at him. You understood. You got it. And you opted out. It’s not fair! I can’t just check out; why can you? I thought we were a team. It's a strange position to be in. As a patient, or, rather, the patient’s mom, there was no outlet or ceremony or designated space for grieving. I didn’t know him personally and therefore I’m not entitled to participate in any funeral or memorial. Yet because he really understood, it felt like we lost a fairly intimate relation. 

For better or worse, the doctor’s death seemed to expedite things at the hospital. There is now a physiatrist in Boise a few days each month and available by phone. This doctor met us later in October for the pump trial. He is a wonderful doctor in his own right. He’s got a pleasant bedside manner, was comfortable with Keira and has first hand, meaning personal, experience with muscle spasticity. 

Keira was incredible on the day of the trial. Typically, kids get sedated for the procedure, but when I pointed out to the doc how little Keira actually wiggles, he agreed to try without sedation. The procedure involved the injection the Baclofen directly into her spine (Like a spinal tap, I’m told) and Keira  didn’t. Even. Cry. My guess is she was enjoying all the attention. There were probably 8-10 clinicians in the room at the time. Lots of observers because, once again, we were in territory that they don’t cover often. 

After the injection, our job was to just sit back and observe the affects. We were in the ICU primarily as a precaution in case the drug suppressed her breathing. It was actually a pretty fun afternoon. There is no better way to describe Keira than “noodly.”  She was absolutely limp and relaxed. Flaccid. Too relaxed, really, but the trial is really a firehose blast to see if the drug will work ultimately.

I was surprised and glad to see how limber Keira’s legs were. This was good news because it means that so far, the spasticity hasn’t actually limited her muscles ability to develop in length. Most astounding to me were Keira’s feet. In the past year it has struck me how more and more her feet are looking twisted and, well, deformed. With the relaxing agent on board, her feet looked utterly normal. 

Fast forward to December. Keira and I are sitting in the back seat of the van as we approach Salt Lake City. There is currently no neurosurgeon in Idaho who does pediatric Baclofen pumps, so Primary Children's Hospital it is!

Today we’ll meet the surgeon and tomorrow Keira will have surgery. She’ll have a hockey-puck sized pump put under her skin below her rib cage. Then a catheter will be threaded up her spinal cord as far as they can get it. The further up the spinal column, the more likely the drug will help her with her neck, shoulders, and arms; not just her legs. The pump works by continuously delivering microdoses of medication directly to where it needs to go, making it much more effective and efficient than oral Baclofen. The side effects are obvious discomfort from the implants, misplacement or slippage of the catheter and unplanned withdrawal or overdose. Also, we cannot adjust the dose ourselves, so we have to be in close contact with the doctor.

If all goes well, we should be able to come home on Monday. We'll keep you posted. As per usual, Keira's wonderful brothers are staying with Jeff's parents and my parents will be travelling to Salt Lake tomorrow to take some shifts on hospital duty.