Imagine over time parts of your body become frozen, like stone. Your neck, then your back, your chest, your arms, your legs. It would be a living nightmare. More nightmarish is the fact that this is no myth nor a work of fiction, it is an actual disease.
It is called Fibrodysplasia Ossificans Progressiva and it is pronounced (fie-bro-dis-PLAY-shuh) (ah-suh-FIH-kans) (pro-gruh-SEE-vuh). If you think it is a chore to say it, try living with it.
For the second time this century, the Roselle Park governing body recognized Fibrodysplasia Ossificans Progressiva – or FOP as it is better known – in order to help a man with a mission to bring awareness to the rare disorder. So rare that right now only a dozen New Jersey residents along with 250 Americans suffer from the devastating condition.
Council-At-large Joseph DeIorio, before presenting the proclamation to Garden State resident and crusader Gary White, said, “Gary many years ago started on a quest to get towns across the State of New Jersey aware of this terrible disease.”
Back in 2002, a year after Mr. White started on his mission, Roselle Park was one of a handful of municipalities that adopted a proclamation to make the public aware of the disorder. Back then, Gary had over 550 towns to convince to adopt a resolution or proclamation about FOP awareness. Now, 17 years later, the borough once again adopted a proclamation – this time to coincide with the state recognizing April 23, 2018, as the first FOP Awareness Day – and Gary only has eight municipalities left to finish his undertaking.
As for Gary, he stated last Thursday night that it happened to a friend’s daughter when she was nine-years-old. It started with a burning sensation in her neck with doctors not able to diagnose her condition. She and her family finally found their way to the University of Pennsylvania (UPenn) where they diagnosed her with FOP.
Gary said, “Due to trauma which they attribute that she fell during a skiing trip, the muscle in her neck turned to bone and then the bone grew all the way down to the hip and it fused with the hip and when it grew in her body it left her body to her right side tilt for life . . . [For] the first nine years of life she had no symptoms of the disease at all except for a telltale sign that there was a malformation of the big toe but no one knew that. So then over time another bone grew in her arm and locked her arm in place and another bone grew in her leg and locked her leg in place.”
The woman, now in her mid-20s can no longer stand and is locked into a wheelchair in an upright position.
Back in 2001, Gary decided to do two things for FOP. One was to start an awareness initiative and the second was to raise funds for research. He stated, “I think we’ve raised close to a half a million dollars for FOP research. It goes right to the lab at the University of Pennsylvania.”
Five years after he started on his mission, on April 23rd in 2006, researchers at UPenn found the gene in the body that causes FOP. Currently, there are three FOP drug trials going on in the United States with one of them in Phase III, the final stage of clinical trials. Gary remarked, “It’s the best news for these families and the Mayo Clinic just started their clinical trial this week.”
Earlier this year, on the last day that New Jersey Governor Chris Christie was in office, he signed a bill that permanently designates April 23rd as FOP Awareness Day in the Garden State.
Councilman DeIorio read the proclamation into the record:
Whereas Fibrodysplasia Ossificans Progressiva is an extremely rare connective-tissue disease in which a mutation of the body’s repair mechanism causes soft connective tissue including muscle, tendon, and ligament to ossified spontaneously or when damaged causing joints to become permanently frozen and producing a second skeleton resulting in the loss of mobility to the affected areas of the body, and;
Whereas FOP is inherited in an autosomal dominant pattern in which one copy of the altered gene in each cell is sufficient to cause the disorder and most cases of FOP result from a new gene mutation, and;
Whereas the symptoms of FOP usually appear in the first or second decade of life with a majority of patients diagnosed by the age of ten depriving children of normal development, and;
Whereas most children born with FOP tend to have malformed toes which helps distinguish this disorder from other skeletal problems and a child with FOP will typically develop extra skeletal bones at the neck and on the shoulders, arms, chest area, legs, feet and:
Whereas FOP is so rare that it’s considered an orphan disease since the disease affects so few people its symptoms are often misdiagnosed as cancer or fibrosis, and:
Whereas currently there are approximately 250 cases of FOP in the country with 12 cases in New Jersey, and;
Whereas there is no cure or proved treatment for FOP, as a result, the median survival age is 40 with years of management, however, this delayed diagnosis and surgical interventions can worsen FOP, and:
Whereas a number of pharmaceutical companies focused on the rare disease are currently in varying stages of intervention into different therapeutic approaches for FOP, and;
Whereas raising awareness about FOP on the anniversary of the day [the] FOP gene was discovered in 2006 and about the current research being conducted into causes could encourage the medical community’s continued search for a cure and development of a treatment and prevention strategies for this rare genetic disease and other muscular-skeletal disorders involving extraskeletal bone formation;
Now, therefore be it resolved, be it proclaimed, that Mayor Carl Hokanson, Mayor of the Borough of Roselle Park in the County of Union in the State of New Jersey do hereby proclaim April 23, 2018 as Fibrodysplasia Ossificans Progressiva Awareness Day from the Borough of Roselle Park to raise public awareness of FOP and the current research being conducted into the causes and continued search for a cure and improvement treatment strategies.
Gay added after the ceremony that the proclamation will be included in a two-book portfolio of all resolutions or proclamations collected from municipalities, the governor’s office and the New Jersey State assembly as well as the State Senate. Once completed, the books will be turned over to the New Jersy Historical Society to be added to their archives and collection. Both or Roselle Park’s proclamations will be included.
Anyone seeking more information on Fibrodysplasia Ossificans Progressiva can visit the disorder’s page on the National Order of Rare Diseases website (link).