Guide
How I explain my brain with Chiari Malformation
Living with Arnold-Chiari Malformation Type 1. How I explain several elements of Chiari Malformation in severn words or less.
I live with a neurological condition called Chiari 1 Malformation (pronounced kee-ah-ree), formerly known as Arnold-Chiari Malformation Type 1 or shortened to Chiari Malformation. While Chiari Malformation is not a rare condition since it affects 1 in 1000 people, it is an uncommon diagnosis that has a wide range of different symptoms, so I’ve had to learn strategies for how to “explain my brain” and how Chiari Malformation can affect me in different environments, including at school, at home, at work, and in social settings. Here are some phrases and explanations that help when I “explain my brain” and describing experiences and symptoms of Chiari Malformation.
Disclaimer
As a reminder, I am not a doctor or medical professional, and all information, content and material on this website details my personal experience, is for informational purposes only, and does not constitute medical advice. You should not rely solely on this information and should seek individualized consultation, diagnosis, and/or medical treatment of a qualified physician or healthcare provider. If you believe you are experiencing a medical emergency, please call 911 or go to the nearest emergency room. Do not delay in seeking medical treatment because of information posted on this website.
What is Chiari Malformation?
A Chiari malformation is a structural defect in the base of the skull near the cerebellum, where the cerebellar tonsils descend from the foramen magnum into the spinal canal, which is referred to as a descension. This clinical definition is a great thing to share with healthcare professionals, but isn’t particularly helpful for those who don’t have a medical background. Here are some other explanations that have worked well for me:
My brain is trying to escape
Chiari Malformation is sometimes described as having “too much brain to contain” or having a brain that is too big for the skull, but a more accurate term is that the brain is trying to escape, which is what creates the descension in the spinal column. This also can better illustrate the back-of-head pain associated with the condition.
Not all in head, also in spine
A common saying for people who have their symptoms dismissed is to say that it is “all in their head”, but for people with Chiari Malformation, this isn’t true- a Chiari Malformation is when brain tissue extends into the spine. Some people may have Chiari symptoms that also affect their shoulders, back, arms, hands, legs, feet and other non-head body parts.
Descension size doesn’t matter, symptoms do
Someone with a larger descension does not necessarily experience more intense Chiari Malformation symptoms, and describing the symptoms of a Chiari Malformation is a more accurate way to determine how the condition affects them. Specific symptoms are generally not correlated to a specific descension measurement.
Chiari Malformation can be asymptomatic or incidental
For some people, Chiari Malformation is an incidental finding on an MRI and may not cause any symptoms or require further treatment. This is why many Chiari Malformation resources focus on symptoms and symptom management, and why learning to explain my brain was so important.
Related links
- Chiari Malformation Type 1 – StatPearls – NCBI Bookshelf (nih.gov)
- Supporting Patients With Low Vision In Medical Environments
- Bedding Hacks For Chiari Malformation
- Disability and Classroom Accommodations For Chiari Malformation
Understanding the diagnostic process
I started experiencing significant symptoms of Chiari Malformation when I was fourteen years old and in ninth grade but didn’t receive a diagnosis until I was eighteen and in college. As I went through the process of being diagnosed, I received questions from others about why the process was taking so long, and why it suddenly became a problem when I was a teenager. Here is some information that helped me understand this better:
Symptom onset can happen at any time
Chiari Malformation may be present at birth (congenital) or acquired as the result of another disorder; symptoms may develop gradually or evolve over time. Many people with Chiari Malformation begin experiencing symptoms as teenagers or young adults, though Chiari Malformation can be diagnosed at any point during the lifespan. There is currently no way to determine if a child will be born with Chiari 1 Malformation or if they will develop it later in life.
MRI is 2-D Image of a 3-D problem
One of my favorite ways I’ve heard Chiari Malformation described is as a 2-D image of a 3-D problem, since the MRI cannot adequately illustrate factors such as crowding and bone density. MRIs are an important diagnostic tool, but being able to describe symptoms and how they impact quality of life will provide additional valuable information that can be used to make treatment plans or identify areas of needed support.
Some people have Chiari Malformation symptoms for years or decades before they receive a definitive diagnosis on a MRI, even if they have had multiple MRIs in the past. This can be due to a number of factors, including positioning, and a healthcare professional can provide more information on this topic.
Can do a great impression of other issues
Chiari Malformation symptoms can mimic other diseases or conditions, which means that it can take a while to receive an accurate diagnosis since so many other things have to be ruled out. I gave a talk at a conference in 2020 called “Why Everyone Should Design For Chiari Malformation” that touches on how accessibility strategies for Chiari Malformation can be used with other common and uncommon disabilities and illnesses that are associated with similar symptoms, which is linked below.
In addition, Chiari Malformation can also be associated with other conditions such as POTS, spina bifida, and Ehlers-Danlos Syndrome. I was diagnosed with POTS about four years after I was diagnosed with Chiari Malformation, but I do not have spina bifida or Ehlers-Danlos Syndrome.
Related links
- Associated disorders of Chiari Type I malformations: a review in: Neurosurgical Focus Volume 31 Issue 3 (2011) Journals (thejns.org)
- Why Everyone Should Design For Chiari Malformation: My Talk At ID24 2020
- Having An Undiagnosed Chronic Illness In High School
- Disability and Classroom Accommodations For POTS
Describing Chiari Malformation pain
Pain is relative and dynamic, and pain levels can fluctuate depending on environmental factors or other triggers. Still, learning to describe common characteristics of Chiari Malformation pain has helped me better explain my brain and how pain affects my day-to-day life. Here are some ways that I would describe the characteristics of Chiari Malformation pain:
Near-constant pain in back of my head
Since the “brain is trying to escape” via the spinal column, many people with Chiari Malformation have a constant pain in the back of their head that can change with head position. There isn’t a head position that would take away the head pain completely, but certain positions can be more comfortable than others, such as laying down or otherwise changing positions.
Several sources report that the Valsalva maneuver makes headache and pain worse for many patients with Chiari Malformation (see StatPearls article).
Straining and sneezing feel like getting kicked
Sneezing and coughing can trigger a headache or make pain feel more intense, which I describe as feeling like getting kicked in the back of the head. The change in pain can be temporarily disorienting, especially after sneezing or coughing multiple times in a row. Other activities that involve straining can also trigger this same feeling of getting kicked, so things like bad seasonal allergies or a stomach bug could trigger a bad headache on top of everything.
Additional pain becomes overwhelming quickly
In addition to Chiari Malformation, some people experience chronic daily headache, chronic migraines, or other chronic pain from associated disorders. While many people adapt to functioning with the “baseline pain” associated with the condition, adding another headache, an injury, or overstimulation in general can make it much harder for someone to manage their pain and cause them to “shut down” or need time to rest and wait for their pain levels to decrease. It can be challenging to balance multiple sources of pain at once both physically and cognitively, and it’s important to schedule rest time or identify strategies that can be used for pain management.
Related links
- Chiari Malformation Type 1 – StatPearls – NCBI Bookshelf (nih.gov)
- Learning to Self-Advocate
- Managing Chronic Migraines in College
- Activity Ideas For Vision Breaks
Describing Chiari Malformation visual impairment
One of the most obvious/prominent symptoms of Chiari Malformation for me personally is visual impairment, specifically low vision. I was diagnosed with accommodative esotropia as a child, which is a form of strabismus and led to a diagnosis of low vision. I later learned that accommodative esotropia and Chiari Malformation are commonly associated with each other, and the combination of both diagnoses led to me receiving an updated diagnosis of decompensated strabismus. Chiari Malformation vision issues are another thing that varies from person to person, and some examples of how I explain my vision loss include:
There’s two of everything, and it’s blurry
Double vision and blurry vision can be associated with Chiari Malformation and require prescription lens correction; since my vision loss cannot be completely corrected by glasses, I was diagnosed with low vision. For me, double vision is not necessarily two items side-by-side, but it’s more of a shadow effect where two images blend into each other, sort of like a shadow effect, which can make it challenging to grab items off of a shelf or locate objects in general.
Bright lights are distracting and painful
Photophobia is a sensitivity to bright lights, which may also be referred to as photosensitivity. Things like fluorescent lights, glare from bright paper or reflective floors, screen displays, and sudden bursts of bright light can be painful and make eye strain feel worse, which can in turn also make head pain feel worse. Wearing non-polarized tinted glasses and minimizing glare in environments has helped me tremendously with accommodating photophobia, and I share more information in a post on tinted glasses below.
Photosensitivity may also refer to a light sensitivity to strobe, flickering, or flashing lights, which may also be a migraine trigger. I also experience this type of light sensitivity, but tinted glasses don’t help very much with it; I limit my exposure to these lights as much as possible.
Text can be harder to read
Factors such as double and blurry vision, difficulty positioning the head close to a screen/page, and light sensitivity can all play a role in making standard print more difficult to read for people with Chiari Malformation. Strategies such as increasing line spacing, larger font sizes, and simplified reading displays can all play a role in improving the reading experience.
Physical activity modifications can be helpful
While Chiari Malformation can be associated with abnormal gait and balance issues, visual impairment can also be associated with lower levels of physical activity as a whole because of limited access to inclusive or accessible physical activity options. Things like limited depth perception, difficulty with tracking objects, and similar barriers can all negatively impact participation in physical activity, or lead to feelings of discouragement or not wanting to participate at all. Physical therapy, adapted physical education activities, and modified workouts are examples of interventions that individuals can discuss with their healthcare providers.
Related links
- Learning To Explain Usable Vision
- How Tinted Glasses Help My Light Sensitivity
- Veronica’s Four Eyes: All About My Glasses For Low Vision
- Two of Everything: Living With Double Vision
- Using PicsArt To Simulate Low Vision
- A to Z of Assistive Technology for Reading Digital Text
- Gym Classes and Low Vision
Chiari Malformation treatment and outlook
Because Chiari Malformation has so many different symptoms and other conditions associated with it, there’s no “magic” treatment that can make symptoms go away or cure Chiari Malformation altogether. While I can’t offer any medical advice or treatment ideas, the following information was helpful for my family and I when making decisions and figuring out how to manage symptoms:
Not life threatening, can be life altering
Chiari 1 Malformation is not a life-threatening condition, and many people can live full lives with or without treating the descension. However, Chiari Malformation can be a life altering diagnosis as symptoms can have a profound impact on quality of life or make it difficult to participate fully in physical activities or social activities.
Medications may be contraindicated
Some medications for symptom management may be contraindicated or not effective for people with Chiari Malformation, and some people may have to work with their doctor and try multiple medications or treatment protocols before finding something that works for them. Some people may also find benefit from physical therapy and occupational therapy or alternative medicine approaches like massage or acupuncture, though these treatments should be discussed with a doctor before starting them.
Only treatment is surgery, medication doesn’t cure
While medication can be used to address individual symptoms, there is no medication designed to treat Chiari Malformation or the underlying structural abnormalities. The only treatment for Chiari Malformation is decompression surgery, and there is no guaranteed outcome for symptom remission or improvement. In addition, individuals may not be a candidate for Chiari Malformation surgery due to other factors, or may have other conditions that affect how they treat it.
Identifying symptoms is key to thriving
I’ve repeated it throughout the post, but I’ll share it again: identifying Chiari Malformation symptoms and how they affect an individual is key to understanding how to not just live with Chiari Malformation, but thrive with it too. Learning how to explain my usable vision, understanding my physical limits and when to prioritize rest, managing pain triggers, and finding workflows for different scenarios has helped me attend several milestone events and have even more amazing opportunities, including but not limited to:
- Going to my junior and senior prom
- Graduating from high school with an advanced diploma
- Being accepted to and attending my dream school, George Mason University and living on campus during undergrad
- Graduating with a degree in Computational and Data Sciences, with a minor in assistive technology from George Mason University.
- Studying for my Master’s in Education in Curriculum and Instruction: Assistive Technology through the online program at GMU
- Interning at Microsoft as a program manager at their office in Redmond, Washington
- Traveling to several places around the United States, including Austin, Reno, San Diego, St Louis, New York City, Louisville, Atlanta, Knoxville, Orlando, and many more- including by plane, train, and car.
- Developing a health technology application influenced by my experience living with Chiari Malformation
- Creating a website dedicated to educating people about low vision and assistive technology that is read by people and organizations around the world, and becoming a subject matter expert in low vision accessibility and assistive technology
Related links
- How I Play In GMU Green Machine/Pep Band With Low Vision
- Having An Undiagnosed Chronic Illness In High School
- Going To Prom With Chiari Malformation
- Disability Accommodations For Graduation
- How To Choose A Dorm And Pick College Housing
- Tips For Keeping A Dorm Room Clean With Chronic Illness
- A to Z of Assistive Technology For Low Vision
- 10 Ways College Is Better Than High School For Disabled Students
- Airport Security and Low Vision
- How To Create Accessible Workspaces For Chiari Malformation
More resources on how I explain my brain with Chiari Malformation
- Chiari Malformation Archives | Veronica With Four Eyes (veroniiiica.com)
- Migraine And Light Sensitivity Archives | Veronica With Four Eyes (veroniiiica.com)
- Low Vision Archives | Veronica With Four Eyes (veroniiiica.com)
By Veronica Lewis/Veronica With Four Eyes, www.veroniiiica.com
Updated June 2024; last update was August 2023.
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