It is quite a mouth full: dominant cystoid macula dystrophy or DCMD. This serious, hereditary eye disease only occurs in Brabant. And then again in a limited region. Ninety people in the Oss, Den Bosch and Veghel triangle have it. Family members also have this disease with the Bekkers family, including 20-year-old Jara. She is facing an uncertain future.
Because the sight of Jara and her family members will get worse and worse, that is a fact. How bad? Unknown. When? Unknown. And there is no medicine. Wim Bekkers also has the disease. “I still have a good view and can’t complain. But my daughters only see around twenty percent.” Wim is worried. He has recently suffered from ‘dancing lines’ in his sight. “My cousin has that too and looks double. It limits you enormously in you.”
DCMD is a genetic disease. “It’s in the seventh chromosome,” says Wim. “A deviation causes moisture blades in the back of the eyeball. If they break, scar tissue arises. That tissue takes your view away. At the end of the ride you will have about 10 to 20 percent visibility.” At the moment it is still unknown whether the family members can also be completely blind.
“It’s really a Brabant thing.”
According to the Bekkers family, the doctors have been able to find a ‘patient zero’ via family tree research. “This person comes from the eighteenth century,” says Wim. “All family lines that have developed from this person can have DCMD.” The chance of this is fifty percent. It is unclear why one brother will get it and the other sister is not at all. “Until your twentieth year of life it goes very fast,” says Wim. “After that, the disease stabilizes, but after the age of 50 it comes on again.”
In total there are only 90 patients who have the disease and they all come from East Brabant. “It’s really a Brabant thing,” says Wim. “Outside of this region, no patient with the disease is known. That is why there is also less priority when it comes to research.”
The family will therefore organize a benefit event in the Lambertuskerk in Veghel on 12 April to raise money for more research into the disease. Jara Bekkers has established her hope for that. She still sees reasonable, but has to make a lot of effort to find a balloon in the air, for example. “And my friends always wave extra hard when I get it. I always have to concentrate well.” Sometimes Jara gets a big headache.
“I still want to do things in the short term.”
Jara thinks extra about her future and all the things she could still do. “You really want to know what the future has in store, but you don’t know that. You know that the view will deteriorate, that it will happen once, but they cannot say when and how. I will think otherwise about the future. I would still like to see the Northern Lights. I still want to do things in the short term.” Whether she wants children? Jara is not thinking about that yet. “I’m too young for that.” It can now be prevented from the disease via IVF. So that gives peace. ”