Rare Disease Day: Idiopathic Intracranial Hypertension

Today is Rare Disease Day, an international day of observance and awareness raising event for rare diseases. While there are over 6000 existing rare conditions, I live with one of them. Idiopathic Intracranial Hypertension (IIH), also known as pseudotumor cerebri affects 1 in 100,000 people globally. As with other rare diseases, lack of scientific knowledge and quality information results in a delay in diagnosis, and treatment is often a guessing game.

Everyone can understand and sympathize with the seriousness of a brain tumor. However, many people can’t understand or sympathize with IIH, in which your body and brain reacts as if you have a brain tumor. Instead of a tumor, there is an excess of cerebral spinal fluid in the head which puts pressure on the brain and backs up into the optic nerves.

IIH can cause a variety of symptoms and issues including:

Vision disturbances
Seizures
Memory loss
Gait issues
Cognitive function loss
Nausea
Extreme pain
Tremors
Complete blindness
Cerebral spinal fluid leakage from ears and nose
and more

Although women are more consistently diagnosed with Intracranial Hypertension, men can be affected as well. Women are often diagnosed and put into a box, with doctors blaming weight or diet when the true underlying cause of Intercranial Hypertension remains unknown, hence the ‘idiopathic’ part of IIH. Unlike suffering from a physical brain tumor people with IIH don’t have advocacy or support. There are no awareness walks or well funded studies for a cure. In fact, there is no cure for Idiopathic Intracranial Hypertension. You can manage the diagnosis with a variety of medications, none of which are specifically formulated for this disease. You can try to lose weight, to avoid being fat shamed by your physician. You can travel to see neurologists and specialists; but there is not any one regimen for successful treatment. If you happen to respond well to a special blend of medication and there is no presence of head pain or optic nerve swelling you can go into remission, just like cancer. However, there is never a point in time which you can be declared cured – and it will always rear its head again.

People who are severely affected by IIH often undergo surgeries. Stents to open up the arteries in an attempt to get the spinal fluid to drain more easily can be attempted. Often patients undergo shunt surgery, where tubing is snaked from head to abdomen to have the cerebral spinal fluid drain more freely. However, these surgeries are not cures. Oftentimes, they aren’t even effective – with a 40% failure rate. Many people I know that suffer from IIH have brain surgery an average of once a year and they still suffer debilitating head pain.

Living with IIH can be crippling. Depending on the severity, a person with IIH can experience daily headaches or migraines. Bending over, sneezing, coughing, or sitting up suddenly in bed can cause sharp sudden pain – even to the point of vomiting or blacking out. It is not uncommon for vision to get fuzzy, or see “floaters” or have a decreased line of sight. Sometimes, spinal taps can temporarily relieve high pressure and alleviate symptoms, but cerebral spinal fluid replenishes in 24 hours.

How rare is Idiopathic Intercranial Hypertension?

It is 8 times more common to be diagnosed with a malignant brain tumor, than it is to be diagnosed with IIH
It is 6 times more common to die from a brain tumor, than it is to be diagnosed with IIH

I have headaches on a daily basis. A headache to me is defined as being a 3 or under on the pain scale, assuming 1 is little to no pain, and a 10 is the worst pain imaginable. Anything above a 3 I describe as a migraine, although technically my pain is not a real migraine it’s just due to a buildup of cerebral spinal fluid. One of the worst parts of having IIH is the fact that it is an invisible illness. Unlike with cancer – which people can easily understand the severity of and grasp – there is no outwardly visible signs of the symptoms of IIH. This leads to regular questioning from people who simply don’t understand. “But you don’t look sick.” “What’s wrong with you? I can’t see anything” or “Oh, I had a headache yesterday. Here, take some Excedrin, it helps me.” “Why are you so tired all the time? Admit it, aren’t you just a little lazy?”

Imagine waking up with a headache. Working with a headache. Becoming increasingly sound and perhaps light sensitive. Going to sleep with a headache – and then waking up with that same headache, day in and day out. Most people after experiencing a day’s worth of head pain get cranky, irritable, and tired. Some people may take day off of work to recuperate, or increase their caffeine intake and take some over the counter medication to lessen the pain and continue on with their routine.

People with IIH can’t find relief in medications or caffeine – or if we do, its temporary. Typically, there is no relief from that headache. I’ve had a headache every day for 3 years. A good day to me, is when my headache is low level enough to be able to function “normally”, or at least within my modified range of normal. I can drive, I can run errands, I can complete work. When the pressure – and pain – increases, it starts to go downhill. I’m a lucky case – I’ve successfully avoided brain surgery so far, and have found a mixture of things that help relieve my pressure and keep things manageable. I haven’t lost my sight, and I don’t leak cerebral spinal fluid. I’m one of the lucky ones.

The unsettling thing is, at any point in time this could change for me. My condition could get worse and I could be forced to increase my medication, increase my doctors visits, undergo brain surgery, or have regular spinal taps to relieve the pressure. There is never a guarantee of maintaining my current state despite my best efforts. I look at those I know who suffer with IIH, who are shaving their heads in preparation for brain surgery #9, or who are in the hospital having their shunts revised, a bolt stuck into their head to monitor their pressure. Those I know who can’t work, but struggle to apply for and be given disability because “they don’t look sick,” or because “headaches aren’t enough of an excuse.” I have acquaintances who are steadily losing their vision, or are watching their children or spouses suffer through this diagnosis and are helpless to change the outcome.

Living with Chronic Pain

Just like any other permanent rare disease, Idiopathic Intracranial Hypertension changes the course of your life forever. You have to weigh out the number of activities you can participate in, and struggle to find the balance between pain management and happiness. Normal people can’t begin to understand the number of choices people with chronic pain make every single day.

If I wash the dishes, will I have enough energy to make the bed?
I feel good, my pain level is manageable – can I walk the dog, or do I risk overdoing it and causing my pain to increase?
On a bad day, do I miserably stay in bed, or do I try to go to work, and just suffer through it?
Should I eat healthier and make dinner tonight, or should I reserve my energy and order out?
The floors need to be mopped, but can I do that without bending over, which causes dizziness and increased head pain?

Chronic pain causes you to continually adjust your life to maintain an acceptable level of pain. As sad as that sounds, its true. Those of us with chronic pain have determined what is considered an acceptable level of pain, and a good day typically means that your pain level is below what you consider acceptable. An excellent day may mean a day with little – perhaps even temporarily no – pain. Excellent days may cause us to take advantage of it and overdo our activities, which we then pay for with increased pain later. It’s a delicate balance that you can only learn through trial and error.

If you are interested in learning more about my rare disease, I recommend visiting the following website:

The Intracranial Hypertension Research Foundation, the world’s only not for profit devoted to supporting the research of chronic Intercranial Hypertension: http://ihrfoundation.org.

Do you or someone you know suffer from intracranial hypertension? Reach out to me – I enjoy meeting fellow IIHers!