Raising Awareness for Non-Western Sickle Cell Disease in the Netherlands: The Sickle Cell Fund’s Efforts

In the Netherlands, approximately 2,000 people suffer from the hereditary blood disorder sickle cell disease. This is comparable to cystic fibrosis, a disease that affects about 1,500 people in our country. Worldwide, this ratio is quite different with about 9 million people with sickle cell disease versus 70,000 patients with cystic fibrosis. Yet cystic fibrosis is more well known. Sickle cell disease mainly occurs in people of color, and that is probably the reason rode that the disease receives less attention. The Sickle Cell Fund wants to change that.

On World Sickle Cell Day, June 19, the Sickle Cell Fund, together with the Amsterdam theater DeLaMar, is organizing an event for patients, their family and friends, doctors, researchers and other interested parties. Here researchers have their say, there are performances and the documentary ‘My blood affects’ shown. Howard Komproe hosts as well ambassadors Jörgen Raymann and Miffy are among others present.

Too painful not to know
In sickle cell disease, the red blood cell has a crescent (sickle) shape instead of a donut. Sickle cells have a shorter life span causing severe anemia. The cells also clog the blood vessels because they hook into each other and get stuck. This results in damage to all organs. People with sickle cell disease are fatigued throughout their lives and experience extreme pain on a regular basis. Often hospitalizations are necessary to treat the pain.

For the time being, the disease cannot be cured. People with sickle cell disease die on average 30 years earlier in the Netherlands than healthy people. The disease is hereditary. If both parents are carriers, each child has a 25% chance of having sickle cell disease. In the Netherlands, carrier status occurs in one in seven people from Suriname, the Caribbean, Central Africa, Morocco, Turkey and Syria. Of the 300,000 children born with sickle cell disease worldwide, many do not live past the age of 5 without proper care.

Research
It is still extremely difficult to find funding for sickle cell disease research. That is why the Sickle Cell Fund was established five years ago. The aim of the foundation is to finance scientific research into better treatment, care and ultimately: a cure. In recent years, the Sickle Cell Fund has awarded a total of eleven grants totaling €750,000 to research projects.

During the event in DeLaMar on World Sickle Cell Day, the researchers of eight studies will explain their methodology and findings. These include studies into the impact of cognitive functions on the disease picture and course, the effect of distraction in the form of Virtual Reality or music on patients’ perception of pain, and the possible cure through stem cell transplants. Because it is difficult to find suitable blood donors for this, the Sickle Cell Fund has invited the blood supply organization Sanquin to be present to draw attention to this problem.

My blood affects
In the documentary My blood affects by Maxime Miedema and Eden Goedhuys, people who suffer from sickle cell disease and their relatives have their say. The documentary highlights themes that are of great influence to patients and family members. These are, for example, parenthood, death, the hope of a possible cure and the need for more awareness about illness.

Dr. Marjon Cnossen, pediatrician-haematologist at Erasmus UMC and chairman of the Sickle Cell Fund says in the film: “I think that as a society we pay less attention to diseases that specifically belong to people of color. That has to change. There must be more equality in care and more equality in research into better treatment and cure. We have to work on that together”.