June is Cerebral Cavernous Malformation Awareness Month!
Cerebral Cavernous Malformations (CCMs) are vascular lesions made up of clusters of abnormally dilated blood vessels. These lesions can mostly be found in the brain or spinal cord. Neurosurgeons often refer to these lesions as having a 'raspberry' like appearance due to their composition of bubble like structures called caverns. There are defects in the building blocks of the endothelial cells and other components needed for normal blood vessel walls. Patients and their families often refer to CCM's as ticking time bombs, as there is no way to predict when these lesions will hemorrhage or cause symptoms.
It is estimated that CCMs occur in approximately 0.2% of the general population. For at least 20% of those with CCMs, the illness is hereditary. The hereditary form of the illness is often associated with those patients with multiple CCMs and is considered a more aggressive form of the illness. Each child of someone with the familial form has a 50% chance of inheriting the illness. CCM symptoms vary among individuals, in some cases no symptoms may be present. Cerebral Cavernous Malformations can cause seizures, many different neurological deficits depending on the area of the brain impacted, headaches, hemorrhage and overt hemorrhagic stroke. The only treatment for CCMs is surgery, if lesions are in operable areas. Due to the complexity of surgery to the brain or spinal cord, this sole option comes with a moderate to high risk of complications as well as varying outcomes.
My family and I are sharing our story of CCM's to raise awareness, raise funds for research and share the amazing work of non profit organizations Angioma Alliance and Angioma Alliance Canada. These organizations have been paramount in their mission to inform, support and empower individuals affected by CCM's as well as driving research for a cure. Because quite frankly, #brainsshouldntbleed
Approximately 22 years ago, I was diagnosed with Cerebral Cavernous Malformations after experiencing headaches and some unusual dizziness. First, I was told I had Multiple Sclerosis, however, that was later corrected. At that time, very little was known about CCMs and the prognosis was that I had 3 lesions of which one was in my right cerebellum. At that time, it was considered inoperable and I could only follow medical guidelines that were not yet supported by research to manage the illness.
Over the next 14 years I experienced several Transient Ischemic Attacks, headaches and minor neurological concerns.
Approximately 15 years ago I experienced a 'thunder clap' headache, the worst headache of my life. As I lived in a small town with no MRI, emergency room physicians that were not familiar with CCMs thought I may have West Nile Virus. Fortunately, the day prior I had undertaken a monitoring MRI in the neighbouring city where my neurologist was. It turned out that I had a small hemorrhagic stroke in the left periventricular area of the brain. At the time I was working full time, the President of a Board of Directors and a student in University. I still have the clinical note from the day I was told "You won't work again and will be on disability for the rest of your life". Even though recovery was difficult, I was determined that I would work again; and, I did! I have had a very successful and fulfilling career in a field that I am deeply passionate about.
For almost 9 years I had very little concerns and symptoms related to the CCMs. Almost 5 years ago I moved provinces and this resulted in an interruption to the MRI monitoring program I was following. However, I was doing great! Therefore, I really wasn't concerned about not being monitored. I was very wrong.
In November 2017, I had a headache that lasted for 12 days. Every one of those days, I made it to work, but it was persistent and by day 9 was keeping me up at night. On day 12, I felt nausea and had an earache. My family physician called me at work to see how I was doing and told me to go to the ER immediately. I was still not very concerned as I was talking and walking just fine. At the ER I was told I was having a large hemorrhagic stroke in my right temporal lobe and that I would require emergency brain surgery. Shockingly, I was still not accepting of the situation. I kept telling them that it was not possible as I did not have a CCM in the temporal lobe. A short time later, I was told that it was not medically possible that I had any level of consciousness and was in fact, gravely ill. I learned that night that the brain can perform miracles.
Unless you have experienced it, it is hard to put in to words what happens when you know you may have minutes, to say those last words to those you love. As a single mom, waiting for my son to be brought to the hospital, I was filled with "have I said and done enough?" does he knows how proud I am, how loved he is, that he is never alone and that his courage will get him through anything? As he walked in the room and I reached up to touch his ear to tell him the news he had to be told, everything about my life changed in that moment. I understood in that moment that life is both precious and fragile and I was given an opportunity to be grateful for every single second that I was able to spend living it.
My recovery has been challenging and continues to be full of twists and turns. I am now acutely aware of what it feels like to be marginalized, while at the same time, my family has been nourished by the kindness, understanding and care of the most amazing people that we will never forget.
In January of 2018 I found out that I no longer have 3 CCMs. I have "innumerable" CCMs in various locations that can bleed at anytime. I don't know if it will be tomorrow or next week, in another 15 years or in a few minutes. I do know that I have one in the area that allows us to breathe, have a beating heart and live- the medulla oblongata. Following all medical advice is critical for me now, with this illness in which brain surgery is my only option. Except with this particular little 'raspberry', the surgery is life threatening so will not be considered until that moment comes. The fear that I work hard at keeping at bay each day can be understood by so many CCM patients and their families, young children to adults, many of whom inspire my family and I every day. As I wait to find out the results of genetic testing and the chances of my family having this disease, I ask that you consider a donation if you can, towards a cure, research and more information for those living with Cerebral Cavernous Malformations and those that have not yet been diagnosed. I also ask that you share information about this illness so others can have the education required to consider it when symptoms come to light. Thank you.
For more information on Cerebral Cavernous Malformations: www.angiomaalliance.org