It’s Time to Rewrite the Definition of Porphyria Pain

There is a general lack of understanding and awareness when it comes to pain associated with acute porphyria attacks. Porphyria pain is not well defined in research. It’s misunderstood by doctors and industry professionals, and it’s easy to see why.

Pain cannot be objectively measured or seen, and everyone responds differently during an attack. Someone with acute intermittent porphyria (AIP) may be writhing in bed, howling, and clutching a barf bag. Or, if they’re like me, they might be curled up in a fetal position, crying silent tears, and trying to keep breathing.

For far too long, the community has been focused on finding a cure rather than learning about the pain porphyria patients endure. It’s time to get patients and members of the medical community on the same page with porphyria attack pain. It’s time to rewrite the definition of porphyria pain, in hopes we can inspire new research.

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Descriptions from research

Let’s start with assessing what we know from medical literature. Porphyria pain is underresearched, and there’s a lack of evidence-based recommendations for management and treatment. Digging into published studies, I found only vague descriptions of porphyria attack pain. For example:

• An excerpt from the journal Patient, referring to acute porphyria, describes “diffuse severe pain affecting the abdomen, back, or limbs.”
• An excerpt from The Netherlands Journal of Medicine, referring to cutaneous porphyria, describes “severe pain or blistering of the light-exposed areas of the skin.”

While straightforward, those descriptions were limited by perspective. A doctor or researcher may be able to summarize aspects of physical pain during an attack, but only someone who’s lived through attacks can detail the mental and emotional depth of the experience.

Descriptions from patients

Describing pain in a more useful and holistic way seemed like the easiest job for a patient; after all, we are the true experts in attack pain. So, how do I describe porphyria pain? Here’s my definition:

“Porphyria attack pain is like routinely being tortured and betrayed by my own body.”

As I am only one person representing one porphyria subtype, I asked others who have porphyria for help in describing attack pain. Some agreed the pain was worse than childbirth, kidney stones, and broken bones. Some called it “torture.” Following are selected quotes from some of these conversations:

Emerald, hereditary coproporphyria

Porphyria pain “feels like I’m getting crushed inside a red hot iron maiden that’s also being hit by lightning. I often feel traumatized with attacks when I go to an ER and no one knows what porphyria is.”

Betsy, variegate porphyria

“One of the worst attacks I’ve ever had, the pain intensified until it was like someone had impaled me through my abdomen.”

Jessica, AIP

“It felt like all my organs were in a vice grip and it was very hard to breathe.”

Robert, AIP

“The pain begins as a toothache, like pain in my long bones of my arms, legs. It then progresses into severe abdominal pain that feels like I have a red hot poker stuck into my liver from the front and back [that] is being moved all about through my abdominal area.”

Maree, porphyria cutanea tarda

It’s “like walking through a fire and you can’t get away.”

Cortney, erythropoietic protoporphyria

“I ran an entire half marathon with a fractured pelvic bone. Prior to the race, and even after, I swore I just had tendinitis because I don’t know how to interpret pain levels correctly. ”

***

The difference between descriptions in the literature and those written by people living with porphyria were striking. Accounts from real people made it clear that attack pain is an experience far beyond physical sensation, it’s also emotional, traumatic, complicated, and evolving.

As people with porphyria, we serve a critical role in raising awareness. But I also think of the depth we could provide research, too, if we were recognized for our expertise and sought out in designing studies that investigate attack pain.

Most of all, I dream of the day porphyria pain is better understood, when patients know how to advocate for the care we need, and physicians associate porphyria attacks with extreme pain.

Note: Porphyria News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Porphyria News or its parent company, Bionews, and are intended to spark discussion about issues pertaining to porphyria.