I Was Diagnosed With Idiopathic Intracranial Hypertension at 27

I used to be first recognized as nearsighted round 12 years outdated. After years of secretly wanting to wish glasses (my cool older brother had wanted them since earlier than I used to be born), I discovered myself struggling to see the characters on stage at a efficiency of Depraved on Broadway, so my mother and father took me to the attention physician the place, certain sufficient, I had delicate myopia, or a slight distortion of imaginative and prescient from a distance.

Sensing my pleasure, the ophthalmologist jogged my memory to solely put on my glasses when taking a look at far distances—for instance, the chalkboard at college or the tv in our front room. Basically, the extra I wore them, the extra my eyes would get used to them, he stated, and I’d probably develop to wish a stronger prescription.

Regardless of his finest efforts, by the point I used to be sufficiently old to drive, I wanted to put on my glasses on a regular basis (which, to be honest, probably would have occurred anyway, as myopia has a strong genetic component and each my mother and father had it).

All through highschool and school, I alternated between sporting my contacts and glasses. I used to be routinely getting eye exams not as a result of my imaginative and prescient was altering so often, however as a result of I cherished getting new frames and wished to ensure my lenses have been updated in them.

However then life obtained busy. I graduated school and began working as a magnificence editor in New York Metropolis, and I didn’t have as a lot time for (okay, I didn’t make as a lot time for) myself as I used to. Over the subsequent two or three years, my imaginative and prescient obtained worse and I knew I wanted an up to date prescription. However I by no means imagined the information I would get the subsequent time I stepped into an eye fixed physician’s workplace.

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The primary signal that one thing was flawed

A number of years after school, one thing unusual out of the blue occurred to my imaginative and prescient. Image this: I’m at a yard barbecue at my aunt’s home in New Jersey. It’s summer season 2020, so we’re all social-distancing outdoor (but additionally so pleased to be again collectively after months of indoor quarantining). She had moved simply previous to the pandemic, so this was our first time seeing her new residence. I’m sitting on the desk on the again deck admiring the home after I discover the siding seems off—it doesn’t seem straight on the highest of every panel.

I rise up to take a more in-depth look and as I’m approaching the home, the panel seems to flatten out. The easiest way I can describe it’s that what ought to’ve gave the impression to be a flat, even line on the prime of every panel of siding had a curve protruding from it, however as I obtained nearer, the curve went away, nearly like an optical phantasm.

“Dang,” I believed to myself. “I actually need to get my eyes checked.”

Lastly within the fall, after some encouragement from my mother, I dragged myself (and mother) to an eye fixed physician appointment. At this level it had been fairly a while since my final eye examination, so I went to an area physician that was new to me, close to my household’s residence on Lengthy Island.

When the physician requested if I had any adjustments in my imaginative and prescient, I defined to her the visible distortion I had been seeing. She advised me to not fear, however after operating by way of a typical eye examination, not solely did I would like a brand new prescription, however the physician observed one thing else that gave her pause. She stated my optic nerves seemed enlarged and inspired me to see a neuro-ophthalmologist for a second opinion, for the reason that optic nerve is what connects the eye to the brain. The neuro-ophthalmologist that took my insurance coverage at her observe wouldn’t be capable to see me for a couple of weeks, so we made an appointment for good measure, however she urged I attempt to get in someplace else extra rapidly.

I stored my composure till we have been out of the workplace, however then the panic set in. Having misplaced my father to metastatic mind most cancers after I was 14, I used to be certain this was it for me. I’m past blessed to have an incredible help system in my household, although, and my mother, aunt, and uncle hit the bottom operating searching for a neuro-ophthalmologist in my space on my plan. They got here throughout Robert Rothstein, MD, who, after listening to about my signs, squeezed me in for an emergency appointment the next day.

Getting a reputation for my signs

Dr. Rothstein carried out a complete eye examination, agreed that my optic nerves appeared swollen, and urged a prognosis of papilledema, a situation outlined by the Cleveland Clinic as “the swelling of each optic discs in your eyes as a consequence of elevated intracranial hypertension,” or strain across the mind.

Intracranial hypertension might be brought on by head damage, stroke, a ruptured mind aneurysm, tumors, and even sure infections reminiscent of meningitis or encephalitis. As a result of strain across the mind might be life-threatening, Dr. Rothstein instantly ordered a collection of assessments to find out the trigger, together with blood work to search for illness and an infection, mind imaging to scan for tumors and abnormalities, and a lumbar puncture (or spinal faucet) to test my ranges of cerebral spinal fluid (CSF), the clear, watery fluid that surrounds and protects the mind and spinal twine.

The latter take a look at was carried out by Srikanth Boddu, MD, an endovascular surgical neuroradiologist within the division of neurological surgical procedure at NewYork-Presbyterian Queens and an assistant professor of radiology in neurosurgery at Weil/Cornell Medication.

Fortunately, the blood work and imaging got here again clear. However the spinal faucet confirmed that my CSF ranges have been elevated, and different assessments confirmed that the massive veins in my mind have been narrowed and underneath strain (a situation known as venous sinus stenosis).

Collectively, these confirmed the prognosis of intracranial hypertension, however as a result of there didn’t seem like an underlying trigger, the prognosis was up to date to idiopathic intracranial hypertension (IIH), that means “elevated strain on the mind of unknown origin.”

IIH was as soon as known as pseudotumor cerebri as a result of its results mimic these of a mind tumor. Frequent signs can embrace complications, tinnitus (a whooshing or pulsing noise within the ears), and visible signs like I used to be having, but additionally blurring, floaters, and black spots. Complications are usually worse within the morning if you get up and might get extra intense if you lean ahead. Some individuals expertise extra signs, too, like dizziness, reminiscence points, cognitive impairment, and steadiness issues. In extreme instances, the swelling of the optic nerve could cause imaginative and prescient loss, per the Mayo Clinic.

Though I used to be recognized with IIH at 27, I’ve struggled with complications my entire life. Drs. Rothstein and Boddu have each stated it’s potential I used to be coping with signs of IIH lengthy earlier than my prognosis.

“A lot of my sufferers have been advised ‘you’re effective,’ ‘there’s nothing flawed with you,’ and ‘that is all simply in your head.’ The truth is, these medical doctors are right—the issue is within the sufferers’ heads, it simply didn’t get recognized!” —Srikanth Boddu, MD, endovascular surgical neuroradiologist

A rising downside

Penn Medicine studies that IIH impacts roughly 2 in 100,000 individuals annually, making it a comparatively uncommon situation. However that quantity will increase considerably for girls between the ages of 15 and 44 who’re categorised as having weight problems, to 22 in 100,000 individuals (for what it’s value, I match that demographic).

And the numbers are on the rise. Based on a February 2024 research in Neurology, IIH is a quickly rising well being care concern within the U.S., particularly for Black and Hispanic ladies. And, sadly, the biggest soar in prevalence up to now decade has been in women ages 11 to 17.

What’s extra, Dr. Boddu says underdiagnosis and misdiagnosis are widespread with IIH, which suggests the true variety of instances might be increased than what’s been reported. “IIH is closely underdiagnosed,” he says. “Lack of publicity to the prognosis, restricted consciousness of the whole spectrum of signs, and categorizing the continual refractory complications as migraines, mixed with affected person ignorance and restricted entry to extremely specialised well being care are all key contributing elements for the underdiagnosis of IIH.”

Athos Patsalides, MD, MPH, neurosurgeon and chief of neuro-interventional surgical procedure at North Shore College Hospital, has been treating individuals with IIH since 2000 and sees upwards of 200 sufferers annually. He has seen a rise in IIH instances in recent times, and he says there’s a direct correlation with one other well being situation that’s on the rise (and in addition more pervasive in minority groups): “As weight problems turns into extra prevalent, so does IIH.”

The hyperlink between weight problems (that’s, having a physique mass index of 30 or increased) and IIH isn’t utterly understood, however there are a couple of theories in regards to the relationship. One theory² suggests IIH occurs when extra fats places strain on the physique’s delicate tissues and the veins that run by way of them, in the end lowering the movement of blood to the mind. Researchers are additionally finding out how genes and hormones might play a job.

Managing my IIH

I’ve been taking 500 mg (the standard beginning dose) of acetazolamide (a diuretic) twice a day since my prognosis. Acetazolamide is a carbonic anhydrase inhibitor believed to cut back the speed of CSF manufacturing. I’ve been lucky that I’ve been capable of cope with the remedy unintended effects (most notably for me was neuropathy or tingling in my lips and extremities), however as dosage will increase so too do unintended effects, which frequently change into overwhelming for individuals treating IIH.

Surgical therapies can be found for extra extreme instances of papilledema and IIH, in addition to for people who find themselves delicate to medicines. Earlier than 2010, extra invasive therapies reminiscent of a cerebrospinal fluid shunt (a surgically implanted system that drains extra CSF from the mind or spinal twine and redirects it to a different a part of the physique for absorption) and optic nerve sheath fenestration (a surgical process through which a slit is minimize into the optic nerve to empty extra CSF) have been the commonest surgical choices for IIH.

In 2010, Dr. Patsalides pioneered a minimally invasive surgical procedure for IIH known as venous sinus stenting. The process is completed by way of a small minimize in a vein within the higher a part of the leg or the arm. A small mesh tube often called a stent is positioned into the narrowed part of the venous sinus utilizing a skinny versatile tube known as a catheter, guided by way of X-ray imaging. As soon as the catheter reaches the narrowed space, the stent is positioned into the vein and it expands to carry the vein open. By bettering blood movement, the stent helps scale back the strain contained in the cranium, relieving signs of IIH and comorbidities like papilledema.

It also needs to be famous that weight loss is a proven effective treatment for IIH³. Whereas not everybody with IIH who loses weight will see an enchancment, research have proven a direct correlation between weight problems and elevated IIH prognosis in addition to weight reduction and IIH remission.

With every physician’s go to, I am reminded of the energy I’ve gained from confronting this situation head-on. I’ve realized that whereas IIH presents its challenges, it’s additionally manageable with the fitting remedy and understanding.

What occurs subsequent?

Luckily, IIH is totally treatable, significantly when you’ve got an early prognosis like I did. Left untreated, IIH can result in everlasting imaginative and prescient loss in addition to a poor high quality of life as a consequence of persistent complications and tinnitus. In some excessive instances, untreated IIH can result in a spontaneous leak of mind fluid, which causes low strain within the mind which will result in an entire new slew of issues.

“The primary barrier to efficient remedy is delayed prognosis,” says Dr. Patsalides. Dr. Boddu agrees.

“Having the preliminary prognosis and getting referred to a neuro-interventionalist are the 2 fundamental challenges in a affected person’s journey,” Dr. Boddu says. “In my expertise, nearly all of IIH sufferers start by seeing totally different specialist physicians for every of their signs when actually all they’re having is a single underlying downside with a number of totally different manifestations.”

In different phrases, the identical points that result in IIH being underdiagnosed—primarily, lack of information in regards to the situation amongst each sufferers and medical doctors—additionally imply that fewer individuals with the situation find yourself getting the remedy they want.

My IIH is at the moment secure because of remedy and quarterly monitoring visits with Dr. Rothstein. Ought to my situation worsen (or if I select to cease taking acetazolamide), my medical workforce has suggested I go for venous sinus stenting, as that, alongside weight reduction, is what’s more than likely to cut back the CSF buildup round my mind.

Regardless of the remedy, I nonetheless have the visible distortion the place straight strains seem curved—a reminder that IIH is part of my on a regular basis life now. However with every physician’s go to, I am reminded of the energy I’ve gained from confronting this situation head-on. Via the experience of my medical workforce and my household’s unwavering help, I’ve realized that whereas IIH presents its challenges, it’s additionally manageable with the fitting remedy and understanding.

What to do when you suppose you’ve got IIH

First, be aware of your signs. IIH can manifest in plenty of methods: visible distortion; complications; a ringing, “whooshing,” or pulsing sound in your ears; and cognitive and neurologic impairments, amongst others.

In case your medical workforce has dismissed any of those signs up to now, take into account seeing specialists like a neuro-ophthalmologist or interventional neuroradiologist for a second opinion and to check your eyes for papilledema and your CSF for elevated strain.

With the dearth of training and consciousness surrounding IIH, advocating for yourself is extraordinarily necessary.

“The vast majority of my sufferers have had continual complications for years, usually recognized as migraine complications, and in some instances they’ve even been branded as ‘loopy’ by different medical doctors,” Dr. Boddu says. “Following a number of workplace and emergency room visits, lots of my sufferers have been advised ‘you’re effective,’ ‘there’s nothing flawed with you,’ and ‘that is all simply in your head.’ The truth is, these medical doctors are right—the issue is within the sufferers’ heads, it simply didn’t get recognized!”

Briefly, when you suspect one thing isn’t fairly proper together with your imaginative and prescient or well being, don’t hesitate to hunt solutions and advocate for your self. In any case, information and early intervention are the keys to dwelling nicely—particularly with a situation like IIH.