DeKALB – It has been almost five years since Shelby Johnson received the diagnosis that profoundly affected her life.
“We went to one doctor on Halloween when I was 14, and then within a week we went to another doctor and he referred me to a doctor in Chicago,” said Johnson, a 19-year-old student at Kishwaukee College. “When we saw the doctor in Chicago he said he had an opening the next day for surgery.”
Johnson was diagnosed with Chiari malformation, a potentially debilitating brain condition caused by the cerebellum and brain stem developing improperly in the upper part of the spinal column, putting pressure on those parts of the brain and restricting the flow of fluid between the spine and brain.
Chiari malformation is often associated with hydrocephalus and spina bifida. The Chiari Institute estimates anywhere from 200,000 to 2 million Americans have the condition.
September is Chiari Awareness Month. Johnson visited with MidWeek reporter Curtis Clegg to discuss her condition and her plans for the future, and to share advice for others who find themselves facing the same diagnosis.
MidWeek: Tell me about Chiari.
Shelby Johnson: I can tell you it’s really stressful. …It’s definitely a life-changer because you don’t really know what’s going on inside your head but then (after diagnosis is confirmed) it makes sense why some of these issues are happening.
MW: Were you born with Chiari, or is it something you developed later in life?
SJ: I was born with it but it never really reared its head until I was older. We found out about it when I was 14.
MW: What were your symptoms?
SJ: Really bad headaches in the back of my head, and then in the front by my temples I get a raging headache. I got a lot of fatigue and weakness and I shook a lot and I started not being able to see as well.
MW: How many surgeries have you had?
SJ: I only had the one surgery, about three weeks after I found out. I had a large blockage in my spinal cord.
MW: What is your day-to-day life like?
SJ: Chiari still affects me emotionally; it drains me when I stop to think about it. Most of the times I’ll get a headache or I’ll feel really weak or busy if I’m walking around school a lot. I’m one of the lucky ones that had the problem fixed and I maybe have 10 percent of the symptoms from before I had my surgery. It’s a blessing because I was really not OK for a while after the surgery.
MW: Are you in any extracurriculars?
SJ: Not really so much anymore. The day before I found out about my Chiari I was at traveling softball practice. We used to have tournaments every weekend, I was really serious.
MW: Do you find that people don’t take your diagnosis seriously?
SJ: Since I was a freshman during my diagnosis and surgery and recovery, trying to explain it to other kids and teachers (was difficult) because they didn’t take me seriously. …I only had one little scar across the back of my neck but I hid it with my hair.
MW: I notice your engagement ring. Is that a recent development?
SJ: I got engaged to a person I met right after my surgery in high school while I was recovering.
MW: It must be a good feeling to move on with your life and do normal things.
SJ: I have my job and I have my fiance and I plan on getting married and doing normal things which is nice, because Chiari is not usually passed down.
MW: If someone who was just diagnosed with Chiari malformation came to you for advice, what would you tell them?
SJ: I would say definitely keep the people who are supporting you close to you. I’m really lucky I had my family around. …Make sure you are honest with your doctor about how you are feeling. …Make sure to always talk about it and don’t keep it bottled up inside because even if you’re a private person, dealing with it head-on really helps.
MW: Are there any misconceptions about Chiari that you’d like to address?
SJ: One misconception people have is that people think “you don’t really look like you have anything wrong with you.” Like if someone has depression, you can’t look at them and know, and if someone has Chiari you can’t look at them and know. There are people who will think you’re being lazy even when you’re fighting such a big battle.