'Silence like a statue' is not just a figure of speech. There is a disease that can make a person's body can only stand still like a statue. This disease is called fibrodysplasia ossificans progressiva or FOP.
Fibrodysplasia ossificans progressiva is a disease in which the soft tissues of the skeletal system, such as muscles, tendons, and ligaments, turn into hard bone tissue. This disease also has another name, namely stone man's disease.
As a result, people with FOP will have difficulty or even not be able to move their limbs at all. FOP is a very rare disease. It is estimated that there are only about 800 people suffering from this disease worldwide.
Causes of FOP Penyakit
Fibrodysplasia ossificans progressiva occurs due to a genetic mutation in the ACVR1 gene that plays a role in the process of bone growth and development. As a result of this genetic disorder, bone tissue in FOP sufferers grows uncontrollably.
Because it is caused by genetic factors, FOP disease can be passed down from parents who have FOP to their children. However, in certain cases, FOP can also occur in children whose parents do not have this disease.
Until now, the reason for the occurrence of genetic disorders that cause FOP is still unknown.
Some Signs and Symptoms of FOP
Babies born with FOP will usually experience some signs and symptoms of FOP in the first few years of life. However, the course of FOP in each patient is different.
The following are some of the signs and symptoms of FOP to pay attention to:
1. Abnormal shape of the big toe
One of the early signs of FOP that can be recognized from birth is the shape of the two big toes that are shorter and bent in the opposite direction from the other toe. For some people with FOP, this condition is also experienced by their thumb.
2. Transformation of soft tissue into bone
Another major sign of FOP is the soft tissue turning into bone accompanied by pain, inflammation, and low-grade fever. The condition called flare-ups This can last for 6-8 weeks and occurs repeatedly throughout the life of a person with FOP.
Flare-ups It usually begins with a hard lump or bulge in certain parts of the body, such as the neck, shoulders, and back. After that, the bone growth will spread to other body parts, except the tongue, diaphragm, heart muscle, and smooth muscle.
Flare-up can appear more quickly in a child or baby born with FOP if he has an injury or has an infection, such as the flu. For most people with FOP, flare-ups they will experience for the first time at the age of 10 years.
3. Difficulty moving
Bone growth in the joints and muscles will result in the movement of people with FOP being very limited. They will also have difficulty talking, eating, and drinking because of limited jaw movement. Over time, they can become malnourished because it is difficult to eat.
Because it is difficult to maintain posture, FOP sufferers will also find it difficult to move and hold their body position, so they fall quickly when standing.
4. It's hard to breathe
Not only that, FOP sufferers may find it difficult to breathe due to the formation of bone tissue in the chest and around the ribs, so that lung movement becomes limited when breathing.
Various Steps for Handling FOP
Until now, no treatment has been found that is able to inhibit, stop, or eliminate bone growth outside the skeleton of people with FOP.
The treatment given by the doctor is only intended to relieve the symptoms that arise due to FOP. To relieve the symptoms of FOP, doctors can try the following treatment steps:
- Prescribing corticosteroid medications to treat inflammation and swelling caused by flare-ups.
- Prescribing nonsteroidal anti-inflammatory drugs (NSAIDs) to relieve pain and prevent inflammation between breaks flare-ups.
- Advise the patient to use assistive devices, such as special shoes to help with walking, a cane, or a wheelchair.
- Perform physiotherapy and occupational therapy.
Most people with FOP require a wheelchair to move around by the time they reach their 20s. In their 30s, sufferers usually can no longer move completely and mostly just lie in bed. Life expectancy in patients with FOP is about 40 years.
During their lifetime, people with FOP will need to undergo regular monitoring to an orthopedic doctor for treatment if needed.
FOP (fibrodysplasia ossificans progressiva) is a very rare and incurable disease. Therefore, various studies are still being carried out to find out further what causes this disease, prevention steps, and treatment.
