When It's a Struggle to Get Your Life-Saving Medication Because You're Rare

Recently, I was out of one of my medications and had no refills left. Of the plethora of medications I take, this is the one that keeps me alive. I have idiopathic intracranial hypertension (IH), a rare disease which affects only one percent of the population. For an unknown reason, my body stopped absorbing my spinal fluid, so it builds up in and around my brain and spinal column. Fortunately, I am one of the 50 percent of patients who respond to this medication, which was the only FDA-approved treatment in my state at the time of my diagnosis nearly 10 years ago.

I went into remission after one year of taking the medicine. “Remission” is a commonly debated term among the IH patient community, as well as with doctors and specialists. For my purposes, I use it to describe the ceasing of my IH symptoms and leveling of my spinal pressure while taking medicine. However, in 2015 my CSF (cerebral spinal fluid) levels began to rise again, causing decreased vision, headaches and pressure within my head. My dosage was increased, but I’ve since lost some vision in my left eye and still deal with headaches and pressure at times. Without the medicine, my CSF would increase until I experienced blindness, seizures, stroke or death. So yes, the medicine literally keeps me alive.

My pharmacy contacted my doctor’s office on a Friday about refilling the medication but got no response. The pharmacist was kind enough to give me an emergency supply to last until Monday morning. When Monday came, I contacted the office twice through an online messaging system, imploring them to refill my prescription that day, as I was out of the medicine. By the afternoon, my pharmacy still hadn’t received an order to fill, so I called the office. The nurse asked what I needed, and I explained to her the urgency of having the prescription called in that day. When I mentioned what the medication was used for, she replied with sarcasm, “It drains your spinal fluid?” I said, “Yes, I have idiopathic intracranial hypertension.” She said, “Huh, never heard of it. Well, I’ll send this to the doctor and tell him you say it’s urgent.” I got off the phone feeling hurt, frustrated and angry. Just because she’d never heard of my disease didn’t make it nonexistent. Her skepticism could endanger me. Sure enough, I called the pharmacy Monday evening to learn they’d still not received an order.

So, Tuesday morning I called the doctor’s office again in a near panic. I learned from the young lady who took my call that the nurse had not sent the message to my doctor — but to his nurse, and no action was taken. I again repeated how important it was that I begin taking this medication as soon as possible and why. I was told that she was marking it urgent and that if the pharmacy had not received an order within an hour, to call back and ask for her specifically. Lo and behold, I was finally listened to. She listened and acted, and I had my medicine in a few hours.

I thought about what would have happened had I not been assertive in advocating for myself. I thought about patients who may not know they have that right or feel confident enough to speak up. But when people have rare diseases, there are medications we take that literally mean life or death for us. We matter. We’re important. We deserve to speak up for ourselves. We deserve to be listened to. We deserve to live.

And, how about the health care workers? The ones who aren’t knowledgeable about every rare disease? The ones who dismiss patients as hypochondriacs or dramatic instead of listening to them and taking them seriously? This kind of attitude can potentially cause harm for rare disease patients, not to mention their family.

So, if you have a rare disease like me, speak up. And if you work in the health care system, listen up. Let’s start giving care to our rare.

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