Living with HOD, a rare degenerative neurological condition, and fighting for answers for the community: “And you may ask yourself, ‘Well… how did I get here?’”
Christina Coates was diagnosed in 2021 with a rare degenerative neurological condition called hypertrophic olivary degeneration (HOD). Only two months later Christina turned her despair into hope when she resolved to form a patient organisation to unite people affected by the condition across the world. Today, the Hypertrophic Olivary Degeneration Association fundraises, connects patients and caregivers and brings researchers and industry together to find answers to critical questions about the condition
By Christina Coates
Hypertrophic olivary degeneration: a life-changing diagnosis
Do you remember the song, “Once in a Lifetime” by Talking Heads? Specifically, the line, “And you may ask yourself, ‘Well… how did I get here?’” This phrase has been the theme of my existence since July 28, 2021. This was a day that will live on in personal infamy in my life. This is the day that I received the diagnosis of hypertrophic olivary degeneration (HOD).
Dear reader, please indulge me, and make an allowance for me to take you back in time. Way back in 2017, I was diagnosed with a sporadic cavernous malformation on the fourth ventricle on the cerebellum, adjacent to my brainstem. The lesion, often described as a small grouping of misplaced blood vessels with very thin walls that resembles a raspberry, had evidence of a previous haemorrhage. At that time, the only symptom that I had was daily migraine headaches. The consensus amongst the patient community is that most of us have migraine headaches, but our physicians all tell us that they are not linked to the cavernous malformations. Since it was fairly benign, my team of doctors agreed that we would manage it by MRI (what is known as Watch and Wait).
In the fall of 2020, during the peak of the worldwide pandemic, my most recent MRI showed the malformation to be plumping up. This meant it was time to seriously consider surgery. Craniotomy is a tricky thing to consider. On the one hand, it is a terrifying prospect. On the other hand, any neurosurgeon worth their salt is confident to the point of overconfident and can convince you that this is not a big deal. After all, how many people can say, “Well, it is brain surgery!” I had the operation in May 2021 and began the long road to recovery.
I faithfully participated in all my therapies, and I was steadily improving. Right up to the point when I was not. My progress began to slide backwards, and I knew that something was wrong. After a battery of tests, it was discovered that I had developed a lesion on my medulla that looked like a multiple sclerosis lesion. Eventually, a radiologist determined that it was HOD.
Hypertrophic olivary degeneration is an extraordinarily rare degenerative neurological disease that is not well understood at this time. Imagine, if you will, receiving a phone call from your neurologist to tell you that you have this disease that is difficult to pronounce and that they do not know what this means for you, other than: “It is not a death sentence.” Indeed, HOD will not be what eventually ends my existence. HOD is more of a life sentence with no possibility of parole.
The information that our small patient population has about our shared rare disease comes from our own lived experience with it. We often find ourselves playing a game of symptom bingo. Much of the population has constant dizziness and gait issues. Some patients have ataxia. Some have spasticity. Some have myoclonus. Some have all these symptoms and more. HOD is classified as a movement disorder, like Parkinson’s disease. Many of our patient population received this diagnosis after some sort of trauma to the cerebellum, brainstem or both. However, there are a few patients that we know of that arrived at the diagnosis with no known trauma, lesion, tumour or surgery. This disease is a truly mysterious.
Two months after my diagnosis, I made the decision that I was not going to just let this disease take my mind, my livelihood, my ambition and my quality of life away from me. After doing a small bit of research on NORD’s website, I came to understand that researchers are more likely to work on diseases that have a patient organisation established.
I woke up early one Saturday morning with determination and drive to form a patient organisation. It simply was not an option to just sit back and hope that someone else would do it for me. That morning, I could feel the tides turning. I felt a twinge of hope where there was previously a vacuum of despair.
The Hypertrophic Olivary Degeneration Association: fighting for answers
My education and livelihood are in accounting, and not social work. I am a natural introvert. I struggle with imposter syndrome daily and creating a patient organisation would most certainly plunge me into anxiety. I had to become a “hype man”, and get people excited enough to accept invitations to become board members and volunteer their time to help me get this organisation functioning. I was pleasantly surprised when I had people coming out of the woodwork that were excited to help and give up their own time and resources to give hope to this precious community. I am continually impressed with the giving and empathetic spirit of our governing board, even though their only true exposure to HOD is through their relationship with me.
In October of 2021, the Hypertrophic Olivary Degeneration Association (HODA) was born. The mission for HODA is to connect the patient and caregiver community, fundraise for research, and connect research and industry to our community to seek answers as to why this disease occurs and how we can treat and/or prevent it from occurring in the future. We have just recruited two doctors to our external medical board and are seeking one more member to round out the board. We are working with Sanford Health CoRDS to create a patient registry. The registry is essential to gather data points from the patient population for our future research team.
I truly had no idea how much energy and effort it would take. This was not the life I had always envisioned for myself. My plan was to work until an acceptable retirement age, then travel, spend time with our kids and grandkids, troll around town in matching track suits with my husband and enjoy the typical life of a retiree. Now, I must accept that I will likely be forced to retire before my 50th birthday and also accept the real possibility that I will need to be in a managed care facility long before a “normal” person. However, I have changed my legacy into something I am exceedingly proud to have been a part of. My life will never be the “same as it ever was”, as the song goes.
My hopes for HODA are to provide community and hope to this small group of people, who are separated by mountains, oceans and borders, but are united in the cause of fighting for answers. We are small, but we are mighty. I believe that HODA will continue far past my tenure as president. I have had the privilege of meeting so many rare allies and partners. I believe that these united communities can truly turn the tide for all rare communities. I know that the work we put in today will pay off tenfold in the future for all rare disease communities.
I am optimistic that we can improve the current state of our healthcare. A person diagnosed with HOD today has far more information available to them than those of us who received the diagnosis in the past, and that is exciting.
About the author: Christina Coates is the founding member and president of the Hypertrophic Olivary Degeneration Association. She works for a heavy equipment company full time as a senior credit account manager while spending her free time working for the patient organisation. She volunteers with the Arizona chapter of the Alliance to Cure Cavernous Malformation. She enjoys reading and learning about neurology in her free time. She enjoys travelling and exploring as her symptoms permit. Her favourite destinations include oceans, as the feel of the sand beneath her feet is one of her favourite sensations.