The physical, mental, and emotional toll of Charcot-Marie-Tooth disease: Joe Hogan’s story
Joe Hogan is a 62-year-old man living with Charcot-Marie-Tooth. He had a 25-year career working in television as a TV movie script analyst, development executive and is now a director of standards and practices at Disney ABC Television Group. As an amputee and leg brace wearer, Joe also enjoys acting in parts representing the disabled community. Joe spends his free time volunteering to bring awareness to CMT
Charcot-Marie-Tooth disease (CMT) is a progressive, degenerative disease involving the peripheral nerves that branch out from the brain and spinal cord to other parts of the body, including the arms, hands, legs and feet. In CMT the problem with the nerves results in numbness and muscle weakness, and over time the muscles weaken and deteriorate. Symptoms may begin as early as birth or during adulthood, and they gradually worsen. There are currently no treatments or cures for CMT.1
I am here to tell you about the effects Charcot-Marie-Tooth disease (CMT) has had on me and my family for generations, while being repeatedly told by the medical community there is little to be done in terms of treatment.
Since I was very young, I have had to live my life with this spirit-wasting, energy-draining disease. My mother said she first knew I had the “family disease” at three years old when I kept falling over and couldn’t stand up. I was also walking on my tiptoes (an early indicator of CMT).
I was relentlessly bullied on the playground because I didn’t walk, run or jump like the other kids. In the 4th grade, on an eye/hand coordination test, I received the worst score in the entire elementary school. The humiliation of having a hard-to-see disability started early and has persisted throughout my life. It made me feel like there was something wrong with me, beyond just my physical limitations——that I was somehow deficient as a human being.
I did painful leg exercises each night to stretch my hamstrings and ankle tendons so that I could fully set my foot down. I had high arches and hammer toes that affected my gait and balance. You could push me over with barely a tap. My Aunt Lois had the worst symptoms of CMT. She was misdiagnosed with Frederich’s ataxia when she had her first leg surgeries at Shriner’s Hospital in the 1940s. In the early 1960s, my mother had both ankles fused and hammer toes amputated. In her later years, she suffered head injuries after two CMT-related falls resulting in stroke-related dementia. She lived the last ten years of her life confined to a nursing home bed. My Uncle Jim wore leg braces and walked with the familiar CMT unsteady gait. In his fifties, he had his left leg amputated below the knee. I saw what was coming for me even when I was very young.
Every part of my family has been compromised by CMT: my cousin Pam, Lois’ only child, has undergone ankle fusions, tendon transfers and hip replacements. Although my older sister as well as my uncle’s first two children do not have CMT, his four children all do, one of the boys to the point of complete disability. And now, those cousins have children of their own, also with CMT. I chose not to have kids; how could I risk passing this disease to my child?
My point is that it’s a family disease that affects many, many people. The physical, mental, and emotional toll CMT takes is far greater than the considerable financial costs. If I had to guess, I’d say I’ve spent well over $200,000 dollars on healthcare directly related to CMT. I couldn’t even calculate what my insurance companies have spent and what the providers have written off. I have had more surgeries than I can count. The financial burden of the surgeries and lost work is just one component of CMT.
In my twenties, I moved to Los Angeles from the East Coast to be an actor. After booking one of my biggest jobs up to that point, I broke my leg on the day of taping. I didn’t step off a curb or trip but was simply walking when I felt and heard a loud pop. I thought I’d sprained it for the umpteenth time. CMT causes a loss of feeling in the lower limbs and I couldn’t feel the severity of the pain so I kept walking on it for two weeks until the chills and swelling wouldn’t go away. My tibia had fractured in two places. So, when they tell an actor to “break a leg,” I took it literally. My leg took months to heal and subsequently was more crooked than ever. It only got worse as the years passed.
The muscles in my lower legs atrophied because the electrical signal sent by the nerves had ceased to function due to demyelination. I began wearing awkward AFOs (ankle and foot orthoses) and leg braces in my thirties. Over two decades, I had pressure sores, bone infections, broken metatarsal bones, multiple rounds of intravenous antibiotics and more surgeries. The near constant use of crutches resulted in carpal tunnel pain, thus requiring more surgeries.
After the broken leg, I gave up my work as an actor due to health insurance concerns and the lack of roles at that time for less obvious disabilities. Once again, CMT would dictate major life decisions. Five years ago, after three failed attempts to reconstruct that left leg by a surgeon at Cedars Sinai, I had it amputated below the knee. Before my amputation I had a pre-eminent CMT expert rudely ask me “How did you ever let it get this bad?” as if I hadn’t done everything possible to avoid amputation. My balance and ability to walk improved with the prosthetic leg to the point that I’ve seriously considered having my other leg amputated.
I wear an AFO brace on my right leg to address severe foot drop. Due to my lack of balance and instability, there have been numerous falls. I’ve had both hips replaced, one of them twice. Some time ago, I tripped outside my house and used my right arm to protect my head from crashing into a cement pillar, thus causing a massive tear to my rotator cuff. This resulted in a reverse shoulder replacement (an operation to replace damaged joint surfaces in the shoulder) last year.
I’ve abandoned dreams for my acting career, but I’ve also had to abandon other dreams: dreams of being a father, dreams of living an active lifestyle, dreams of being seen as a man rather than as a someone who with a disability. And as my CMT progresses, I’m still giving up dreams. One of my early memories is of my Aunt Lois giving me a bath and seeing her gnarled hands as she gripped the washcloth and gouged at my ears to get them clean. I say “gouged” because she couldn’t use her thumbs or easily grasp the washcloth. I was both fascinated and scared of those hands because of how they looked. For the last ten plus years, I now have those same hands. They drop most of whatever they try to pick up. Buttoning buttons unaided is now a distant memory and anything that requires the use of my thumbs is no longer possible.
“I’ve said numerous times that I’d gladly give up my other leg if I could get the use of my hands back. Over the years, I have been told repeatedly there was nothing to be done. This consistent message of hopelessness that the CMT community hears all the time is soul crushing. If we could approve treatments for CMT, families with CMT could change the outlook of their lives.”
Imagine telling an adolescent—who is terrified because he sees in his family what’s coming for him—that at last he doesn’t have to fear his future. Imagine telling a newlywed with CMT that she doesn’t have to be afraid of passing this curse to her children. Imagine telling a grandfather that his legacy of disability, ridicule and hopelessness is over. Imagine the productivity of the CMT patient community instead of the lost wages and drain on social security. I’m here to share my story with you not for pity, but so that you can truly understand the emotional, social, financial and physical burden of CMT.
While I have dealt with the effects of CMT for most of my life, mine is far from the worst cases of the disease. There are many young people——confined to wheelchairs or wearing leg braces from early childhood——with more aggressive types of CMT.