Acute disseminated encephalomyelitis (ADEM) was actually easier to spell than it was to say. I still struggle when it comes to the myelitis part. Even more difficult: trying to understand how this could have happened to our then 8-year-old daughter, and exactly what it would mean for her and for our family.
The truth is, we still don’t have a lot of answers. Through the talented physicians and teams at Children’s Medical Center in Dallas, Texas, we have learned that there is so much more to learn about this disease, especially in children. Our little Greta is one of only a handful of children on several continents who have active cases of ADEM.
Greta’s journey began with lethargy. A girl who normally raced out of the car to get into the classroom with her friends every day couldn’t even make it through one day of school. After a second appointment with her regular physician and more tests, Greta tested positive for mononucleosis. We went through the lists of kids at school, at the two churches my husband pastors, and those in the neighborhood. No one had mono.
The diagnosis came with a monitoring order. It was supposed to be a formality only; just a once-a-week appointment to make sure Greta’s spleen didn’t enlarge as it did its job of ridding her body of the virus.
Everything was going well at her first checkup until we were asked that inevitable question: “Have you noticed anything else with Greta?”
No one warned me that when I mentioned my daughter had complained of vision problems in her left eye that I would experience nausea, helplessness, and shock as our little super reader was unable to see anything on the eye chart. How could this be? The girl who helped other children in her classroom couldn’t make out the single large sailboat at the top of the eye chart with her left eye.
We have a great relationship with Greta’s pediatric nurse practitioner; so when she recommended a CT scan, I agreed to it. It was the kind of numb agreement that comes when you’re dealing with way more than you ever thought you could, and you just allow yourself to go along with the recommendations of those you trust.
The next day she and her husband (also a nurse practitioner) met with my husband and me. It wasn’t hard to notice the tension in the room. “It’s not good, is it?”
She shook her head. “No it’s not.”
The three-centimeter mass on the right temporal area of Greta’s brain got us sent straight to the hospital. It was like a nightmare version of Monopoly. Go to jail (Children’s Medical Center in Dallas), go directly to jail, do not pass go, do not collect $200!
Children’s Medical Center is one of the best places in the world if your child is sick. They specialize in making everything as child-friendly as possible, but it’s still a hospital. Greta endured two MRIs, a spinal tap, dilation of her eyes, and various vision tests, numerous blood draws, and physical exams by physicians of numerous specialties. We were relieved to get the final diagnosis of ADEM; which has the best prognosis of any of the demyelinating diseases. It was an answer to prayer. But what, exactly, is ADEM, and where do we go from here?
I joined the Transverse Myelitis Association (TMA) on the advice of one of Greta’s specialists, which provides monthly newsletters and directories for patients and families of the lesser known demyelinating diseases, including ADEM. I read articles about transverse myelitis, neuromyelitis optica, and optical neuritis, but the literature about ADEM is still very limited.
What we do know: ADEM is rare in general and even rarer in children. It is a onetime occurrence of a disease in which the body starts to fight against itself, often as a response to the body’s fight against some other disease or virus. Like all demyelinating diseases, lesions are formed that attack the myelin sheath and can appear in any part of the brain, brain stem, and spinal column.
Treatment almost always includes an intense round of steroids until the ADEM begins to resolve, as well as a two-to five-year plan of monitoring through MRIs and regular appointments with the specialists. Since ADEM can strike in any part of the brain/neurological system, it will manifest itself differently in each patient, depending on where the lesions form in that particular patient.
Greta’s mass was a right temporal, which affected the vision in her left eye. So she had to see the specialist team at the hospital as well as a pediatric ophthalmologist that continues to monitor her left eye. Greta was released from the hospital after nine grueling days and a round of steroids that left her more puffed up than a Hollywood celebrity. Most of her pants didn’t fit, and we had to monitor her food intake. We were sent home with instructions to watch for any strange symptoms, such as uncontrollable hiccups, tingling in her hands, abnormal behaviors, and, of course, changes in her vision.
I remember the surge of adrenaline that went through me the first time Greta came and told me her thumb hurt and tingled. Before ADEM I would have shaken my head and wondered what crazy thing she had done, but now I was on instant alert. Sleeping on a cot at the hospital all those nights must’ve made me a little jumpy. Was this normal? How cautious should I be about the tingling? Didn’t she have hiccups yesterday? And what is abnormal behavior anyway?
It would’ve been nice to be in touch with someone else who has been through this. But ADEM is rare. There are no local support groups in your neighborhood. With no one to help I felt overwhelmed.
Then one day the TMA newsletter arrived with a story about Donny.* His father had written a detailed but brief summary of their experience that left me in tears. While the story was different, I could relate to the many similarities. He provided his e-mail address, in case anybody wanted to contact him. Donny’s parents responded to my e-mail with encouraging pictures of Donny, now a healthy child almost the same age as Greta.
I showed the pictures to my daughter, and she recognized the pictures of Donny on steroids that mirrored her own. “Greta,” I said, “Donny knows what you’ve been through. Look at him now; he’s OK.”
Greta began calling him “my ADEM buddy.”
A few days later I was driving home from errands, relieved that we had finally found another ADEM family. Now we had the connection we so desperately needed. Donny’s parents continued to encourage us, sharing their own concerns such as the first time Donny did something different and how they responded to it. Was it still the original ADEM, or was his diagnosis going to get worse? Was this something to report to the doctors or not? These were questions I had asked as well. Now I knew I was no longer alone.
We keep Donny and his family posted on Greta’s latest MRIs and exams as she has four more years of monitoring before she can be declared fully recovered. It’s a long journey, but now we have friends who have been in our shoes and can give us encouragement along the way.
Like Greta, our pretty blue planet has a rare disease. We are the only planet in the entire universe that has succumbed to its death and destruction.
But Jesus knows what it’s like to walk in our shoes. He knows how difficult it is to survive day by day. We don’t have to go through life alone. We have a Buddy who has scars to show us that He made it. And because He did, we can as well.
This painful journey with our daughter taught me to realize how precious is the grace of God that Jesus came to share our journey so we don’t have to go it alone. Those scars in His hands and feet are more precious now than ever before.