CBC News recently interviewed Amanda, Bradley, and Brad Bright, a close-knit black family living in Nova Scotia. The Brights explain that working their way through initial diagnosis and treatment brought this family even closer.
Camurati-Engelmann disease causes an increase in bone density, mostly in the legs, skull, and arms. It is a skeletal disorder causing muscle weakness, pain, and difficulty walking.
The senior member of the family (Brad) was the first to be aware that he walked with a noticeable “swagger.” One of his high school teachers brought it to his attention but he did not think it was a problem.
However, several years later he discovered he had Camurati-Engelmann disease the same time his daughter Amanda received her diagnosis.
The Brights brought Amanda to the doctor’s when they found that she could not qualify for one of the school’s entry exams that required certain physical activities. The doctors discovered significant hardening of the bones in her legs.
After a thorough examination of the Bright family, Brad, Bradley, and Amanda were diagnosed with Camurati-Engelmann Disease. Only Trina, their mother, was disease-free.
On Being Black in Halifax
Bradley, the third member of the family, is now a thirty-three year-old animator and filmmaker. Bradley produced four short films that are streaming on CBC Gem.
Bradley initially set out to tell the world how it feels Being Black in Halifax, the name of his four part film series. But he realized that adding a film entitled Normal about the rare disease engulfing his family can raise awareness. It gives others with the disease an opportunity to share their own experience with Bradley. At least that is what he hopes will happen.
Amanda The Protector
Bradley explains that Amanda was very protective of him during their many visits to the IWK Children’s Hospital. As it turned out, she was also chosen by the doctors for the bulk of diagnostic testing.
Bradley told CBC News that he really appreciates his older sister for the way she automatically assumed the role of protector. Amanda, now thirty-six-years old and a teacher, explained that she gladly took on that role when they were young and at times still takes on various responsibilities regarding their disorder.
Challenges of the Very Rare Disease
The Bright family is aware that there are people with rare diseases that have access to support groups, but this is not the case with the Brights. Currently, there are no forums or support groups for Camurati-Engelmann Disease.
Bradley told CBC that there has not been one day where he has been without pain. Bradley said that he must simply cope with the pain and added that each day requires more effort.
The Canadian Organization for Rare Disorders (CORD)
CBC spoke with Durhane Wong-Rieger, who is the president of CORD, about the challenges accompanying rare diseases. She offered that many patients may not be diagnosed during the early stages of the disease. Another critical issue is the lack of treatment or access to specialists.
Wong-Rieger added that dealing with a rare disease and not being able to find others with the same disease creates a feeling of isolation. She pointed out that the Bright family has created their own personal support group among the three family members.
Wong-Rieger said that although a disorder may be rare, it is usually associated with other disorders. Therefore, patients may be able to connect with other patients having similar conditions.
There are approximately 6,000 or 7,000 rare diseases. That would indicate that there are about three million Canadians who have a rare disease.
Amanda offered her hopes and thoughts about Bradley’s film. She said that the film may highlight similar diseases, bringing awareness to those disorders also.
