I live with a neurological condition called Arnold-Chiari Malformation Type 1, also known as Chiari Malformation. While Chiari Malformation itself is fairly common, many of these cases are asymptomatic, and I frequently get asked questions about how symptomatic Chiari Malformation affects me. One of my favorite strategies for explaining chronic illness is to do so in as few words as possible, so today I will be sharing descriptions of what it’s like to live with Chiari Malformation that are seven words or less.
Brain is trying to escape
Chiari Malformation is often 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, as the brain tissue extends into the skull. The size of this descension does not necessarily increase over time, but measurements may vary depending on the angle the neurologist or neurosurgeon measures the descension at.
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Sneezing feels like getting kicked in head
One of the distinguishing features of Chiari Malformation is that head pain gets worse when sneezing, coughing, bearing down, or other activities that can put pressure on the head. A sneeze can feel like getting kicked in the back of the head and cause someone to become temporarily disoriented if they sneeze multiple times in a row.
There’s two of everything- and it’s blurry
While Chiari Malformation symptoms can vary from person to person, double vision and blurry vision are commonly associated with the condition, which can contribute to low vision. I was diagnosed with accommodative esotropia as a child, which did not improve as I got older, and it turns out that this was due to the presence of a Chiari Malformation which caused additional complications related to low vision.
Related links
- How I Respond To Questions/Comments About My Eyes
- Two of Everything: Living With Double Vision
- Ten “Odd” Things I Do With Double Vision
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. So, it is not all in someone’s head, it’s also in their spine, and that’s why they are dealing with symptoms!
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.
MRI is a 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. This is why MRIs are not used as the sole way of determining if someone is a candidate for Chiari Malformation surgery and it is important to discuss the impact of symptoms on a person’s quality of life.
Can do great impression of brain tumor
Chiari Malformation can mimic many different conditions, including brain tumors, which can lead to people not being diagnosed for months, years, or even decades, since Chiari Malformations do not always show up on MRIs. It took me four years from symptom onset to be diagnosed with Chiari Malformation.
Related links
- Why Everyone Should Design For Chiari Malformation: My Talk At ID24 2020
- Going To Prom With Chiari Malformation
Constant sharp pain in back of head
Another defining symptom of Chiari Malformation is a constant pain in the back of the head that changes 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.
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Every surface is like walking on ice
Balance issues are also common for people with Chiari Malformation, and my gait issues resembled someone who is trying to walk on ice. Some people may be at an increased fall risk due to these balance issues
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Some get migraines every day
Chronic migraines are another Chiari Malformation symptom, and some people may deal with the effects of a migraine every day, or have another form of chronic daily headache.
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Symptoms can start during puberty- yay teenagers!
Chiari Malformation is a congenital condition, but may not cause symptoms right away. 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. I began experiencing definitive Chiari symptoms when I was fourteen years old.
Can be asymptomatic for some people
I talk a lot about Chiari Malformation symptoms, but there are many people living with Chiari Malformations that are asymptomatic, or that have symptoms come and go. For many people, Chiari Malformation is an incidental finding on a MRI and may not require further treatment.
Diagnosis comes years after symptoms begin
It took four years for me to be diagnosed with Chiari Malformation, so I went through the entirety of high school dealing with then-undiagnosed neurological symptoms. Some people are fortunate enough to receive diagnoses much quicker, and I think it’s awesome that more progress is being made for others to get treatment more quickly.
Related links
- Having An Undiagnosed Chronic Illness In High School
- How To Create Accessible Workspaces For Chiari Malformation
Affects one in one thousand people
While Chiari Malformation is considered a rare disease by some, it is actually fairly common, affecting one in one thousand people. However, many people with Chiari Malformation are asymptomatic, so it may affect even more people.
Coughing can trigger a migraine
Dealing with airborne allergies or an illness involving a lot of sneezing and coughing can lead to me getting a migraine due to the fact coughing and sneezing makes the pain in the back of my head worse.
Related links
- Assistive Technology For Medication Labels
- Managing Chronic Migraines in College
- How I Attend College Classes Remotely With Chronic Illness
Comoribity with other chronic illness
Chiari Malformation is a known comorbidity for other chronic illnesses, including 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. However, the dual diagnosis of Chiari Malformation and accommodative esotropia led to me receiving the diagnosis of decompensated strabismus, which affects my vision.
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My neck and back is hurting me
Chiari Malformation can cause back and neck pain, and this was one of the most difficult things for me to get under control. Physical therapy, acupuncture, massage, and other pain relief tools have been incredibly helpful.
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Not life threatening, can be life altering
Chiari Malformation is not a life threatening condition, and many people can live full lives with or without further treatment. However, Chiari Malformation can be a life altering diagnosis as symptoms can have a profound impact on quality of life- I have had to miss out on several popular experiences and events for people my age due to living with Chiari Malformation, but I find other fun things to do like writing, working on projects, and other hobbies.
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. The only treatment for Chiari Malformation is decompression surgery, but that does not necessarily cure all of the symptoms and many people are not candidates for Chiari surgery for a variety of reasons.
Hands and feet randomly vibrate
The effects of neuropathy from Chiari Malformation are the reason why I can’t read braille or use several tactile aids for people with low vision- I don’t have enough sensitivity in my hands. I also have to be mindful of shoes and ensure that they provide adequate support.
Related links
Migraine medications may be contraindicated
Some migraine medications may be contraindicated for people with Chiari Malformation or other comorbid conditions, and many patients often have to try out several different medications or treatments before finding something that works well for them.
Summary of explaining Chiari Malformation in seven words or less
- Brain is trying to escape
- Sneezing feels like getting kicked in head
- There’s two of everything, and it’s blurry
- Not all in head, also in spine
- Descension size doesn’t matter, symptoms do
- MRI is 2-D image of 3-D problem
- Can do great impression of brain tumor
- Constant sharp pain in back of head
- Every surface is like walking on ice
- Some get migraines every day
- Symptoms can start during puberty- yay teenagers!
- Can be asymptomatic for some people
- Diagnosis comes years after symptoms begin
- Affects one in one thousand people
- Coughing can trigger a migraine
- Comorbidity with other chronic illness
- My neck and back is hurting me
- Not life threatening, can be life altering
- Only treatment is surgery, medication doesn’t cure
- Hands and feet randomly vibrate
- Migraine medications may be contraindicated
