Improvements for my migraines, but what about for Batten disease?

I’ve suffered from migraine headaches since childhood and cluster headaches for most of my adult life. While migraines and cluster headaches are often confused, they’re different conditions. However, both types of headaches can have a significant impact on quality of life.

But how have chronic headaches affected quality of life for me, a generally healthy person, compared with Batten disease‘s quality-of-life effect on its patients? I’ve considered the question often since my sister, Taylor, began to lose her abilities to CLN1 disease, one of the diseases collectively known as Batten.

Living with constant pain

The first time I had a migraine, I was 7 years old. I can still clearly see the ceiling of the sunroom in my grandparents’ beach house. There, I writhed on a blue, flowered loveseat while my grandmother rubbed my temples and spoke in soothing tones.

From that point, headaches were an unwelcome but near-constant companion. I’ve missed soccer, school, and social activities. Lain in a dark room for hours at a time. Gone for ambulance rides with intractable pain and nausea. I recalled one especially bad episode in my 2018 memoir, “Run to the Light“:

“I’d spiraled down some psychedelic rabbit hole, and when I first opened my eyes in the hospital room I thought maybe the sheets covering my cold legs were clouds and the nurses hovering over my bed were angels in heaven. I didn’t feel in control of my own body. A strange sense of calm washed over me as the pain squeezed my brain like a clamp. I realized I didn’t care what those nurses did to me as long as I didn’t hurt anymore afterward. I said a silent prayer that I’d just drift off into a dreamless sleep.” 

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Focusing on Better Quality of Life in Batten Disease

I’ve become so accustomed to life with brain pain, I often keep at whatever I’m doing — from working to running — when an attack strikes. Otherwise, life as I know it would stop. I’ve tried plenty of possible solutions over the years, from over-the-counter and prescription drugs to essential oils, foam rolling, and massage. I’ve given up alcohol. Cut out caffeine. Cut back on screen time.

But in November, my headache frequency increased until the condition became too much to bear. Finally, I saw a neurologist last month, my first such visit in nearly a decade. My new doctor, a respected headache specialist, put me on a cocktail of physical therapy and medications. The prescribed regimen included Emgality (galcanezumab-gnlm), a fairly new antibody migraine treatment. Emgality binds to CGRP, a substance in the brain, and prevents it from binding to the receptor.

A light at the end of the tunnel

Emgality is wildly expensive, and my insurance provider initially denied coverage. But I put up a fight and, with my doctor’s support, won approval. This week, my local pharmacy filled my first monthly (loading) dose of two injections. When I unpackaged the medication at home, I joked to my husband that Emgality, at greater than $800 per 120-mL syringe, might as well be liquid gold.

I thought of my sister before I injected the contents of the two syringes into my thighs. I’ve never handled needles well, so thinking of Taylor’s unflappable courage helped me feel brave. But I also thought of how, if effective, this drug will have a significant impact on my overall quality of life.

I thought about how, once Taylor stopped showing measurable gains from physical and occupational therapy, my parents’ insurance stopped paying for sessions. But anyone who knows anything about Batten disease understands that it’s a neurodegenerative condition, meaning symptoms get worse over time.

Maintaining quality of life was the core purpose of the first clinical care guidelines for CLN1 disease, which Taylor’s Tale, our nonprofit, spearheaded and funded in 2019, not long after Taylor’s death. The guidelines were published in Pediatric Neurology in 2021, as reported in a 2022 Batten Disease News article.

In it, my mother, Sharon King, recalled an expensive overnight hospital stay that could have been avoided if the doctor who saw Taylor in an after-hours clinic understood how to care for her. “If only I had had access to this expertise … it could have been a huge change-maker for Taylor’s best quality of life,” Mom said.

It’s still too soon to know how Emgality will improve my condition. But at the time of this writing, I’ve gone more than 48 hours without a headache — an encouraging sign. My sister, on the other hand, was repeatedly failed by a system that decided her quality of life was no longer important once she’d been diagnosed with her fatal disease.

I’m grateful that innovations in science and medicine give me more options today than I had a decade ago. But I’m equally disappointed that we aren’t doing more for people like Taylor. We can do better. We must do better.

Note: Batten Disease News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Batten Disease News or its parent company, Bionews, and are intended to spark discussion about issues pertaining to Batten disease.