We’ve all had a little taste of isolation this past year – but the meaning of ‘lockdown’ while affecting our lives in every way, is temporary. Yet this experience has allowed us to wrap our heads just for a moment, around the isolation of someone living with a rare disease, particularly one like Fibrodysplasia Ossificans Progressiva (FOP).
FOP is one of the rarest genetic conditions the world has ever seen. In a lockdown of devastating proportions, FOP slowly turns muscle, ligaments and tissue into bone, resulting in a second skeleton, which ultimately makes simple tasks like walking, sitting, reaching, and movement difficult.
Relief of diagnosis and proper care after living in isolation
Enter the Tin Soldiers, a non-government organisation (NGO) that works to identify people who are living in isolation with FOP, and connecting them to care. It is a mission of great importance because while someone with FOP might not know the name of the disease, they live with the isolation, the stigma and the pain of coping with a rarity that often leaves them feeling they are the only ones in the world to have it, with little hope of support. We can only imagine how terrifying it must be when you have no way of understanding the extreme things that are happening to your body.
It is a condition that affects every demographic, in every part of the globe. There are around 900 known cases of FOP globally, and statistics indicate there may be around 7,000 undiagnosed or unconnected people living with FOP. That’s a lot of people living a very isolated and challenging life. In Africa alone, there should be about 1,200 people diagnosed with FOP. We only know of 35.
“Our mission is to find the missing,” says Odette Schwegler who heads up the global NGO. Despite looking for a needle in a haystack, somehow this team has beaten all odds and in the first five months of this year alone, has identified eight – mostly children – with rare bone diseases. Five of these are FOP patients including:
A multi-generational family in a small rural farming community in the Free State – the first father and son to be identified in Africa.In Kwa Zulu Natal, a 9-year-old boy was diagnosed at Albert Luthuli Hospital by a doctor familiar with the Tin Soldiers education program aimed at medical doctors.
A child from Mafikeng was diagnosed after travelling all the way to Chris Hani Baragwanath hospital and consulting a doctor within the Tin Soldiers network.In Kenya, a doctor within the Tin Soldiers African Clinicians Collective who already had one child with FOP in her care, has diagnosed a second in that country.
“Raise-a-leg” and help us find the missing
The Tin Soldiers Global Patient Search Program has great things in store, beginning with a “Raise-a-leg” challenge for social media platforms, to be launched all over the world at the end of June in the hope of spreading awareness about FOP and ultimately finding those with FOP that are missing. Just like you can’t lick your elbow or sneeze with your eyes open – this leg raise is an impossible manoeuvre to perform. It celebrates our own collective freedom as able-bodied South Africans to move naturally – but when locked into this position, it renders one leg absolutely immobile.
“We are looking forward to having fun, gaining followers and friends, and raising funds,” says Schwegler, a filmmaker who received multiple international awards at top film festivals for her “Tin Soldiers” documentary in 2020 – a labour of love that has helped spread awareness about the disease across the globe. www.tinsoldiers.org/raise-a-leg-for-fop-challenge/
The term “Tin Soldiers” comes from a loving comment by an FOP mom who was describing to her son what was happening to him. She told him that he is the bravest of the brave, living in a body locked and stiff, with a mind as sharp as a sword. She compared him to a tin soldier.
Schwegler has also recently produced a public service announcement advert to invite the public to help in the search. “This ad was filmed to celebrate three children and a young woman, who despite their condition are normal and beautiful in every sense of the word. We’ve also recently returned from a “home visit” with global local FOP expert, Dr Christiaan Scott to meet new found FOP family members in person,” explains Schwegler. “There is nothing like the human element of this touching story to galvanise our nation and continent to keep up the search,” says Schwegler.
The search for rare is the road to diagnosis
“What we are trying to do, is identify every person who is currently undiagnosed, giving them access to a caring community, resources, support (global and local), and appropriate medical care,” says Schwegler. In their search, the Tin Soldiers team realised they would potentially come across other rare diseases along the way, and this year have identified three youngsters with very rare bone diseases who are now connected to pathways to care.
In Johannesburg, a little boy was found to have Progressive Osseous Heteroplasia (POH). “The Tin Soldiers team did in four days, what we’ve been trying to do for four years,” said the boy’s mother who at last knows the name of the disease that has turned their world upside down. In their Tin Soldier Global Patient Search, the boy and two other two teenage patients were found, one with Multiple Osteochondromas (MO); and the other with Juvenile Idiopathic Arthritis (JIA). Their isolation has now ended; and there is no doubt that these lives have been irrevocably changed because of people on the search. Rare diseases effect more people than HIV and cancer combined.
In Nairobi, a Kenyan child has now also been diagnosed by one of the doctors in the Tin Soldiers Clinicians Collective network. There is a growing realisation that having a medical and FOP community support group within Africa is incredibly important to both doctors and patients. It is essential to raise awareness among the medical fraternity, because they are the ones that can make a world of difference if they are aware of what to look for.
From nurses, to pharmacists and medical doctors these are the first line of defence. Over the past year, the Tin Soldiers team has driven the development and roll-out of a Global CME Master Series on FOP with the education of approximately 500 healthcare professionals across six continents; growing the African FOP Clinicians Collective from 1 to 10 doctors in South Africa, Kenya, Ethiopia, Nigeria and Namibia.
Based on how far they’ve come this year, The Tin Soldiers Global Patient Search team knows that this search is not impossible. “It’s all starting to come together turning the unknown into an exceptionally special community of known.”