Curse of the Fragile Bone Disease
Source:The Herald Online
Roselyne Sachiti Deputy Features Editor
The Matchazi family in Bulawayo’s Pelandaba suburb sticks out like a sore thumb, thanks to a rare health condition that has crippled them. The four suffer from osteogenesis imperfecta or brittle bone disease. In the medical field osteogenesis
imperfecta causes a defect in the production of a protein called collagen. Callogen is an important protein that helps support the body — the scaffold on which the body is built. Osteogenesis imperfecta is due to a genetic mutation in the gene that directs the body to produce callogen.
“It can either be passed on from a parent who had the disease, or it can occur as a spontaneous mutation in a child. Most cases of osteogenesis imperfecta, he reveals, that are passed from parents due to dominant mutation (comes from one parent who has the condition) although a small number of cases are due to recessive mutations (comes from a gene from both parents, neither having the disease),” Jonathan
Cluett (About com) MD said.
Four males of the family, Jack (54), Luke (51), Nqobile (21) and Andile (15), and have lived with the disease since birth and are now accustomed to it.
The disease has brought problems for the family as Andile has been denied his right to education. His poor parents cannot afford school fees and he has not been benefiting from Basic Education Assistance Module for a while.
Despite failing to go to school Andile finds solace playing for the BCD wheelchair basketball team. He has a silver medal to show for his efforts.
His uncle Luke explains how the condition affects them.
“Our hip bones are too weak and cannot support our upper body to the extent that they fracture even when one coughs hard. I have had the condition since I was a young boy and life has been difficult,” said Luke in an interview with The Herald at their home.
Both Luke and Jack have lost their front teeth. Their legs are somewhat bent, stature is small, so is that of Andile and Nqobile. Luke uses homemade crutches to carry the weight of his upper body.
His brother Jack has a homemade wheelchair which aides his lower body, but he often uses crutches. Nqobile also uses homemade crutches while Andile can still walk without their aid though one of his legs faces sideways. These features are what distinguish this family from the entire Pelandaba.
“When I am sitting and cough or have a simple fall, my hip bones fracture. Long back, my doctors told me that the disease would disappear after 18 years but it is still haunting me, my brother and two nephews Andile and Nqobile,” he said.
He added that the disease has only affected the male members of his family.
“We were seven in our family but the disease only affected three males and spared the females. Our brother who had the same condition died years back. When we initially went to the doctors they said we had polio. We grew up in a rural area and were immunised against polio,” he said.
While doctors told them their condition was polio, they were surprised when his brother’s male children who had been immunised also had the disease raising questions about the initial diagnosis.
The family is living in dire poverty and sometimes spends the day without a single meal. When they finally eat, dinner will comprise of a very tiny portion of sadza and green leafy vegetables despite their condition requiring them to eat a lot of high protein based foods. They make sure they pray each time they eat the little food they get, so that God provides a meal for the next day.
“Because of our condition, we are not formally employed. We used to work for Associated Light Engineering, which was liquidated some years back,” Luke revealed.
He added that they now earn a living from welding window panes, chairs and stoves among others, a job they cannot do while standing.
“Because of the fragile hip bones, we weld while seated on our chairs. Standing up while doing the work means more fractures. No one is willing to employ us in this condition,” he said.
To them, each piece of metal they find abandoned is like gold as it has potential to put food on the table. Mounds of scrap metal on their premises tell a story of how desperate they are to put a single dollar in their pockets.
“We sell the scrap metal but sometimes use it when welding. The money we get is never enough to cater for our needs. We are sinking in debt,” he said.
Jack said he cannot afford to pay the US$140 per term, required at his son’s school. They have to “toss a coin” and choose between education and food. Food has always won, and Andile has been out of school for two months.
The family’s monthly electricity bill is usually US$30, but it has ballooned to US$3 000. Rentals depend on the amount of water they use each month but as of last week, their unpaid bill to Bulawayo City Council was pegged at US$2 000.
They appealed to the power utility and local authority to write off the bills and also exempt them from paying as they will never get the money to pay. Thinking of where they will get the money stresses them.
“We have not paid the bills in three years. We are fortunate that our local authority has been lenient with people living with disabilities and not cut us off. We plead with them to adopt a policy where they exempt us just in case some new people come to council,” he pleaded.
Jack said they do not take any medication for their disease, but do so whenever they are given a prescription by a doctor. He pointed out that they rarely visit doctors even when their bones are severely fractured as they cannot afford to. He does not know if any of their ancestors had the condition, all he remembers is that the disease started with him.
“I am now used to the excruciating pain, have had countless fractures on my hips. I avoid fractures by not exposing my body to conditions that strain my legs. My brother and sons also help me to weld,” he added.
According to Jack, the four have difficulties boarding public transport and always fear that something may happen to them. He has welded a wheelchair for easy movement. Jack added that his Nqobile is a sport enthusiast and also plays wheelchair basketball. He also has several medals to show for his talent.
Jack said his children also have no problems playing with other kids for as long as it is not rough.
“Government should do something to improve our livelihoods. We do not receive any money from them and hope it will start giving us disability grants like in neighbouring countries like South Africa,” he added.
He also revealed that getting customers has been difficult as there is a lot of competition.
Many companies in the area, he said, are now manufacturing window panes and are taking some of their clients as they have better equipment. He added that as such they do not sell much. Sometimes they sell the window panes for US$20 each if they are lucky. Some of their customers just pay them US$5 for their labour and supply all materials.
He revealed that he has tried to speak to the head at his son’s school to have Andile admitted for classes while he looks for money, but without success. The family has not received any assistance from the Department of Social Welfare.
He disclosed that their father died while trying to find the root cause of their condition.
They were still young, he recalls, and they would spend three months in hospital as their legs were always mysteriously breaking.
“Our father died a worried man as he could not find the root cause of our condition,” he added.
The Department of Social welfare has been of little or no help when it comes to the payment of school fees and medical assistance.
Jack’s wife Pattie Ndlovu said she has also made several visits to their offices asking for money to take Nqobile for a scan as a fractured bone of his hip is almost protruding from his flesh.
“They refused to assist me saying they wanted to see the patient first. It is difficult to carry him there since I use public transport. Kombis are 3 rand per person and the money is out of my reach,” Ndlovu said.
She said it requires a lot of patience for one to take care of people in such a condition especially when living in poverty.
Life is quite difficult for the family when one fractures a hip and there is no money for hospital fees.
“I have devised a way to treat them use a homemade traction. The treatment is usually six weeks long and the patient will have to lie on the bed for that period. I tie them to the bed and can tell when the leg has healed,” said Ndlovu who married her husband when he was already disabled but could walk.
Bedtime is a nightmare for the four male family members as they cannot sleep on the floor because of the hip problems, she pointed out.
“They do not have comfortable mattresses so I use form to cushion their fragile hips. I try by every means to ensure that they are all comfortable especially those who would have fractured a hip,” she added.
Nqobile repairs cellphones and playstations to supplement their family’s earnings, but the money is never enough. The competition is also too high and he gets US$4 when he repairs a phone.
“My clients are my treasures. I try to make them happy so that they do not desert me and go to other places. This is why my charges are not too exorbitant,” he said as he tucked away some cellphones in an old wardrobe in his bedroom.
Jonathan Cluett (About com) MD says osteogenesis imperfecta causes a defect in the production of a protein called collagen.
Callogen, he says, is an important protein that helps support the body- the scaffold on which the body is built.
He adds that osteogenesis imperfecta is due to a genetic mutation in the gene that directs the body to produce callogen.
“It can either be passed on from a parent who had the disease, or it can occur as a spontaneous mutation in a child. Most cases of osteogenesis imperfecta, he reveals, that are passed from parents due to dominant mutation ( comes from one parent who has the condition) although a small number of cases are due to recessive mutations ( comes from a gene from both parents, neither having the disease),” he said.
He added that typical problems seen in patients who have the disease include bone fragility, short stature, scoliosis, tooth defects, hearing deficits, bluish sclera, and loose ligaments.
He also said that currently, there is no known treatment for the condition and the primary treatment should be avoiding injuries and maintaining healthy bones.
“Ensuring that children with osteogenesis imperfecta eat a healthy diet, get regular exercise, and maintain a healthy weight is fundermentally important,” he adds.
This has been the life story of the Matchazi’s, one that no one wishes to be in as it takes a lot of courage, patience and strength to endure, so they say.
As Zimbabwe joins the rest of the world to celebrate the Day of the African Child on June 16, whose theme is “The rights of children with disabilities: the duty to protect, respect and fulfill”, children like Andile who are living with disabilities should not be forgotten, as they are crying out for help.