Fibrodysplasia Ossificans Progressiva (FOP) is a rare genetic disorder that primarily affects the skeletal system. The disease causes bone to form in soft tissues, such as muscles, tendons, ligaments, and even organs. This abnormal bone formation, called heterotopic ossification, can lead to severe limitations in movement and can even affect breathing, swallowing, and other vital functions.
While FOP primarily affects the skeletal system, it can indirectly affect other organs and systems due to the progressive ossification:
Organs and Systems Affected by FOP:
- Musculoskeletal System: The most prominent impact of FOP is on the musculoskeletal system. The new bone formation in muscles, tendons, and ligaments restricts movement, leading to stiffness, pain, and ultimately, immobility.
- Respiratory System: Ossification in the chest muscles and rib cage can restrict chest expansion, making breathing difficult. This can lead to respiratory complications, such as pneumonia and sleep apnea.
- Digestive System: Ossification in the muscles of the jaw and neck can affect swallowing and chewing.
- Cardiovascular System: While FOP does not directly affect the heart, the restricted breathing caused by ossification in the chest can put strain on the cardiovascular system.
- Skin: People with FOP often have malformations in their thumbs and big toes, as well as skin abnormalities like a webbed neck.
It's important to remember that FOP is a complex and unpredictable disease. The severity and progression of ossification can vary greatly from person to person.
