“I know my appearance can generate rejection, but I never pay much attention to it”: what it is like to fight against the rare Günther porphyria

Fide Mirón is 46 years old and knows no other life than to fight Credit: BBC

Fide Mirón He was 18 years old the day he suffered the teasing and harassment from a group of teenagers, in the Barcelona metro. They chased her, took her as their entertainment for the day, she says. They followed her because of her appearance, a face marked by congenital erythropoietic porphyria, a rare and aggressive disease with which he has lived since he was a baby.

Fifteen years later, that young woman received an unexpected message. It was from one of those who had bothered her, asking sorry for the “girlie” -as she describes it now- that she did when she was a teenager. The repentant recognized Fide in an interview with the press, in which he recounted his fight against the also known as times de Günther.

But she doesn’t like to talk about “unpleasant experiences.” They no longer matter. What motivates her is that her ailment receives attention and efforts to find a cure or treatment that mitigates its effects. It is a devastating disease. It causes liver damage, anemia, and skin ulcers. The tissue ends up rotting and falling, and that is why they also tell you the “mutilating porphyria”.

Mirón is now 46 years old and this is his account of a whole life with her.

“A continuous, excruciating, heartbreaking pain”

“It hurt a lot. The wounds appeared at six months, when he was just a baby. My parents took me to the doctor, but at that time very little was known about the disease. All they were told was his name. There was no treatment – nor is there now – and it was not known how it might evolve.

The first wounds appeared at six months and then very little – even less – was known about the disease Credit: BBC

What was known was that it was tremendously hard, so my life was not going to be normal. I would have to avoid hurting myself at all costs, I would not be able to expose myself to the sun. Since the disease made me anemic, I needed blood transfusions every 15 days. I spent my childhood receiving them.

This porphyria attacks by outbreaks, day after day. I would wake up in the morning and discover a new wound. Due to the injuries I lost my hands, my facial features. It was continuous, excruciating, gut-wrenching pain.

At 14, my body suddenly stopped needing transfusions. The disease subsided. It’s still there, but it’s like it’s giving me a break. It continues to hurt, but not as hard as in my childhood. It’s more sporadic. “

What is and how does Günther’s erythropoietic porphyria develop?

“It is a very rare disease. To develop it, both parents of the patient must have a destabilizing mutation “, explains to BBC Mundo Dr. Óscar Millet, specialist in hereditary diseases. For symptoms to appear, you have to have both halves, the mother and the father. Just one is not enough. It behaves, but does not develop, it indicates. What Millet describes seems highly unlikely, but in her case it happened.

“Mutation niches formed in different regions and by coincidence it happens that two distant relatives marry, have offspring and inherit the gene,” says Millet. Worldwide, the Spanish Porphyria Association points out that it is estimated that there are between 200 and 300 cases of the “mutilating” type.

The mutation causes the enzyme responsible for producing the heme group, the one that gives blood its color, to be defective. “Hence the anemia and the release of porphyrins, a series of highly toxic compounds that, in the face of stimuli such as visible light, they burn the skin and rot it. They also damage the liver and spleen, “adds the specialist.

In addition to causing skin ulcers, porphyria mutilans destroys cartilage and bones, especially in the hands and face.

In addition to causing skin ulcers, porphyria mutilans destroys cartilage and bones, especially in the hands and face. Credit: BBC

The Spanish Porphyria Association describes that the first symptom is usually the appearance of red urine in babies. Then, there is an extreme sensitivity to sunlight that manifests itself with very intense, painful and repeated injuries, which ends up taking away bones and cartilage, especially in the faces and hands.

The reason the illness has given Fide a break is because the body changes and it doesn’t always work the same way. “This has a mechanism of excretion of porphyrins. When more are expelled than are produced, a steady state arrives.”

But if Mirón is stressed, is exposed to too much light or his metabolism changes, the porphyrins are relaunched, symptoms appear and the wounds return.

A marked face

“My appearance is different because of the sequels. It is very visible. I know it can generate rejection. But I never pay too much attention to it. Anyone who has wanted to know me knows how I am and what I am capable of. Whoever has remained with the sickly and delicate Fide has lost that this life and the pains have strengthened me.

Fide has no time for those who have remained on the surface and have not met the woman she has become now

Fide has no time for those who have remained on the surface and have not met the woman she has become now Credit: BBC

Of course I felt bad when those guys from Barcelona harassed me on the metro. One tries to go around without being messed with and nobody is looking at you too much. That afternoon I was his distraction. But look, that was a minor childish and having one of them apologize to me so many years later was something nice. “

No answers

“The worst is not the pain, it is loneliness. I am not referring to personal loneliness, but to the lack of answers, of guides. I have doctors who support me, but there has never been a specific treatment. These are the moments that weigh the most and hurt, when you feel that the roads are closed and you don’t know where to go.

When I ask for answers, it is as if it creates problems. My case is very complex and since we all go so fast, sometimes we only pay attention to the things that we think we can solve.

Fide loves traveling with friends, it is one of her favorite hobbies

Fide loves traveling with friends, it is one of her favorite hobbies Credit: BBC

We are not many like me. From a young age I began to look for more people in my situation to promote research and find a treatment. I know of a case that I visited in Cuba, another in Brazil, and another in the United States. I have eight people located in Spain. Most are stable, but last summer a friend passed away. His illness got complicated. You never know how it will evolve. That is why I am focused on giving it visibility and looking for a solution.

In one of my investigations, I came across Dr. Millet and we started walking together to find a medicine. That path has paid off and Two years ago a compound that acts on the disease was discovered. It is approved by the European Medicines Agency and we are now waiting for clinical trials to see if it evolves in patients. It is a door of illusion and hope “.

“I feel I can fly”

“My family has been a very important link. My father gave me the blood I needed to survive, but when I was nine he died in a traffic accident. His absence marked us all, but especially me. My support, my reference, the person who gave me his blood left.

“I feel like I can fly,” says Fide today Credit: BBC

Life is too fragile. You are here today and not tomorrow. I had to fight a lot for my life, to get ahead. My mother, on the other hand, has always supported me and carried much of the burden of the disease. He is very strong and has never set goals for me.

Obviously I have to take care of myself a lot, but in my free time I love to travel, meet people and share. Now I have turned my life around. As a young man I could not study due to the aggressiveness of the disease and the harshness of the treatments, I have started a career, that of Social work.

Everything that happens in life can become a lesson. I feel like I can fly. “

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