FOP turns a muscle into bone. The pain is unbearable [LETTER TO THE EDITOR]

FOP – this disease is both fascinating and terrifying. Man has 30 thousand. genes, only one mutation is responsible for FOP, turning healthy muscle into hard bone. My name is Asia. I’m 35 years old. I live in Kalisz. I have FOP. I will tell you my story.

1. FOP, or progressive ossifying myositis, is one of the rarest genetic diseases in the world. One in two million people is born with it
2. FOP turns muscles, ligaments, and tendons into bone. This process leads to the immobilization of the body and disability
3. One of the people who lives with FOP is 35-year-old Asia. In a letter to MedTvoiLokony, the woman described her story
4. Asia: it took a long time to get used to the thought that I had one of the most horrible genetic diseases in the world
5. April 23 is the International FOP Awareness Day
6. You can find more such stories on the Onet homepage.

Diagnosis of FOP. “I did not understand why my body becomes a living statue”

After birth, only valgus of my big toes was diagnosed, and then no one had linked this fact as an early symptom of an ultra-rare disease. The midwife told my mother that if she massaged these fingers, they would straighten over time. However, they did not straighten up … The disease showed itself to me with all its might at the end of the third year of life.

While learning to ride a bike, I fell down and injured my left knee. A few days after this event, I was vomiting, I had a low-grade fever and swelling of my left leg. Within a week, the leg in the knee was immobilized. Nobody was able to correctly diagnose me. I had three operations aimed at, among others, removing the extra bones that blocked the left knee. Unfortunately, after each such operation, these extra bones grew back and there were even more of them … Only after eight years of incorrect treatment, the correct diagnosis was made at the Institute of Paediatrics of the Medical University of Poznań: progressive ossifying myositis (however, the English name for this disease is more often used: Fibrodysplasia Ossificans Progressiva, abbreviated as FOP).

I received the diagnosis without any guidance on how to proceed. All I knew about FOP was what the disease was doing to my body – I felt that my movements were more and more limited – I couldn’t put on a hat, for example, because my hands only reached up to my eyes, I had difficulty putting on my shoes because I couldn’t get low enough bend down, I felt pain while running because my left knee was stiff. At that time, I didn’t understand why my body was becoming a living statue.

“FOP turns muscle into bone”

It was only when I had a computer and Internet access that I found out that FOP is one of the rarest genetic diseases in the world (one in two million people is born with it). FOP causes what appears to be contrary to nature – the eye does not turn into a kidney, but the heart becomes the lung. No organ becomes different. FOP turns a muscle into bone. Doctors have not yet experienced a similar process.

I realized at that time that all these blockages in my body are bones that must not be removed, because the bone tissue will then renew and limit mobility even more. Surgical intervention often makes things worse, as I was painfully aware of during the three operations I had in my childhood.

Photo Private archive

Everything started to fall into one coherent whole (I realized why I have blockages in various parts of my body). FOP attacks the neck, spine, chest, shoulders, elbows, wrists, hips, knees, ankles, jaw and many other places. The disease bypasses the diaphragm, tongue, eyes, face and heart. FOP does not ease over time. The letter “P” in the name of the disease means “progressive”. FOP progresses, or exacerbates, over the years.

FOP is in my genes, so I have had it from birth, even though the extra bone didn’t show up right away. This means that I cannot outgrow FOP and the extra bone tissue cannot disappear.

“Living in bone armor is extremely difficult”

My body doesn’t produce extra bones all the time. Months or even years can pass between the flashes of ossification. However, there is always a risk that extra bone will build up – either without warning (spontaneous flare-up) or following an injury such as a hit, fall, muscle strain, injury, injection into a muscle, surgery or even some viruses.

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It is not clear why the disease becomes active at certain times and remains dormant or stable at other times. It took me a long time to get used to the idea that I had one of the most horrible genetic diseases in the world. There is currently no cure for this. However, I am aware that it is also up to me whether an effective FOP therapy will be created. I participated in a clinical trial of a potential FOP drug. I want my body to contribute as much as possible to progressive ossifying myositis research because I know that there is nothing worse than a disease for which there is no effective cure.

Living in bone armor is tremendously difficult. FOP is often pain that is unbearable. FOP is like a ticking bomb in my body – I never know when it will explode and immobilize another part of my body. FOP turns my own body into a prison. Currently, I no longer raise my hands up, I cannot sit or stand up on my own – my knees are stiffened, my hips are immobilized, I am not able to perform basic everyday activities myself, such as preparing a meal, getting dressed or using the toilet. I am in a wheelchair.

Living with FOP. “What is everyday life for one person, is a dream for another”

The disease creates a second skeleton in me, the so-called FOP skeleton, which in turn becomes a prison for a fully functional intellect. The disease taught me humility, patience and perseverance. My physical limitations aren’t all me yet. I am more than what FOP does to my body.

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My mother, sister and people who support me in making my dreams come true help me in my daily functioning. My dreams? I have three – a drug for FOP, an adapted electric wheelchair (the price of the wheelchair is PLN 45) and something that I do not want to pronounce out loud at the moment. I hope that my story can help other people to look at their own life from a different perspective, because what is everyday life for one person is another’s dream.

Movement is freedom, independence, something priceless. This is how you can help me in everyday life and overcome FOP:

Transfer name of the recipient: Fundacja Avalonn Number of the recipient’s account: 62 1600 1286 0003 0031 8642 6001 payment title: GIERZ 6629

1 percent KRS: 0000270809 detailed target 1%: GIERZ 6629

My fanpage: DwaSkkieletyW JednymCiele

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