Strange disease: Muscles turn into bones

At the Doctors Association’s Mutter Museum in Philadelphia (USA), there are terrifying displays, notably two human skeletons, each of which seems to melt tightly together.

Visitors visit two special skeletons at the Mutter Museum. They belong to people with strange diseases, to this day medicine is still incurable. Rare syndrome These two skeletons belong to people who have had an extremely rare condition, known as the Fibrodysplasia Ossificans Progressiva, or FOP syndrome. FOP patients have muscles, tendons and ligaments that gradually turn into bones, causing the whole body to become stiff and unable to move. FOP syndrome is so rare that there are only about 700 known cases worldwide. FOP syndrome was first described around the 17th century. Until 2006, researchers discovered that FOP was caused by a mutation in the ACVR1 gene and that the condition could be hereditary. However, in most of the cases detected, FOP patients spontaneously developed genetic mutations, with no family history of the disorder. There is currently no cure for FOP. Normally, when a person dies, the connective tissue that holds the bones together is broken down, the skeleton collapses into a discrete mess. In order to display a human skeleton, usually in museums and laboratory specimens, they must be assembled with fine wire and glue. But the two specimens at the Mutter Museum are naturally fused and adhered skeletons. Carol Orzel in the 1990s. Two typical cases The most famous of these patients is Harry Raymond Eastlack, whose skeleton is on display at the Mutter Museum. Harry Raymond Eastlack was born in 1933 in Philadelphia (USA) with a characteristic defect in the big toe, but obstetricians and obstetricians do not recognize this sign. When he was 4 years old, he was hit by a broken leg in a car while playing on the street with his sister. His injured leg was cast in a cast, but the fracture did not heal completely. Months later, the cast was removed but his leg became inflamed and swollen. Before long, Harry found it difficult to move his hips and knees. An X-ray showed that the bones in the boy’s thigh were abnormally developed. The abnormality then spreads along the back, neck, and chest. Harry underwent 11 surgeries in total, but these invasive procedures only exacerbated the boy’s condition as the body attempted to “repair” the damaged tissue with more bones. Despite suffering from a strange illness, Harry still had a relatively happy childhood. Like friends of the same age, he listens to music on the radio, reads books, plays cards with his sister and often goes to the movies. At the Hamilton Theater in Philadelphia, Harry has a special seat in the middle of the 7th row, which can recline and straighten legs. When Harry’s condition worsens, he has more difficulty with daily activities and needs help with toileting, eating, and dressing. At the age of 15, Harry’s jaws got stuck, preventing him from eating solid food and talking with clenched teeth. He had difficulty sitting, as the hips were one of the first motionless parts. Before long, the bone forms through the upper arm and extends onto the sternum, pressing the arm to the chest. Bone fragments stretched down his back and the bones extending from there to the skull caused his neck and head to “lock” hard. The skeleton of Harry Raymond Eastlack seen from behind. Over the years, new bone growth caused his pelvis and thighs to form. One time, Harry accidentally bumped his butt into the fireplace, leaving a bruise, then the smooth tissue there was destroyed, making room for new bones to form. In the end, the boy’s movements remained only in the eyes, lips, and tongue. Harry died in 1973, just six days before his 40th birthday. Near his death, Harry told his sister that he wanted to donate his body and medical records to medicine to study and learn more about the disease. In 2018, Harry Eastlack’s skeleton was combined with another skeleton, belonging to Carol Orzel, who also suffered from FOP, on display. Carol was born also in Philadelphia, in 1959. With FOP, she needs help with eating and dressing because she cannot bend her elbows. Despite her disability, Carol enjoys painting and drawing with a stick-like medium that replaces her motionless elbows, wrists, fingers and neck. She paints the landscape and makes a very beautiful greeting card. This stick also helps her to put on makeup. Carol loves fashion and has a spacious wardrobe that has been modified for convenience, making it easy for her to take them out and wear. Carol died in a nursing home, where Harry spent his final years. She also decided to donate after learning about Harry and seeing his skeleton at the Mutter Museum. Currently, both Harry and Carol’s skeletons are recognized as invaluable artifacts in FOP research.