Sickle cell disease: the H.U.B is campaigning for rare blood donations and against received ideas

On the occasion of World Sickle Cell Day on 19 June, the Erasmus Hospital and the HUDERF, national and European reference centres for sickle cell disease within the Brussels University Hospital (H.U.B), are focusing on the disease. Among care staff as well as the general public, received ideas regarding this disease, its symptoms and its complications fuel a series of prejudices resulting in social exclusion as well as difficulty in stimulating the blood donations needed to transfuse these patients. In Belgium almost 90% of sickle cell disease patients require regular blood transfusions.

Sickle cell disease is a genetic disease of the blood that affects 1 child for every 2,329 births. Particularly frequent among populations of Caribbean, African and Mediterranean origin, it is the commonest genetic disease in Belgium. It is characterised by a deformation of the red blood cells that results in severe anaemia, a local obstruction in the blood circulation and a reduction in the oxygen supply to several vital organs. A child with sickle cell disease has episodes of extreme pain, periods of acute anaemia and is much more prone to serious infections. Complications of this rare multisystemic disease can affect the brain, heart, lungs, kidneys, bones and eyes. These become established over time, increase in frequency in adulthood and have a major impact on comorbidities and mortality. Since January 2023 sickle cell disease has been one of the diseases detected by the Guthrie test in the Wallonia-Brussels Region, this making it possible to identify and treat patients at an early stage of the disease within a reference centre. Hydroxyurea, chronic transfusion and bone marrow transplants are at present the only treatments available in Belgium that modify the symptoms of the disease to reduce complications and improve the quality of life of patients. The sole curative option is to transplant stem cells (bone marrow) but its applications are limited.

This rare and complex disease requires treatment at specialised centres such as the HUDERF and Erasmus Hospital, both recognised as reference centres for rare diseases of the red blood cell at national and European level since 2017. More than a third of patients on the sickle cell disease register in Belgium are being followed up by the H.U.B for the treatment by multidisciplinary teams of the various aspects of this disease. Our haematologists are central to the clinical biological follow-up, treatment and prevention of complications. The referring nurses, essential links in ensuring awareness and an effective response to a patient's needs, coordinate the care and the professionals who provide it. Our biologists play a major role in screening and boosting awareness of the risk of transmission to children. Finally, our psychologists, dietitians and social workers pay special attention to pain management, emotional support, well-being and diet. For children and adolescents, supporting the child in his or her psychological, social, school and family reality is a central element of care in encouraging the development of autonomy despite the illness as well as adherence to treatment and knowledge of the disease. It is essential to help the child to acquire the awareness and know-how to continue to look after themselves throughout their life, with the solid support of medical teams in an adult environment.

Most sickle cell patients will need a blood transfusion at some point. Some of them will need regular transfusions throughout their life. Hence the supply problems. The transfused blood must match as closely as possible that of the sickle cell patients who have blood groups that can be rare in Belgium. At present we regularly face shortages and that is particularly true for the blood groups found predominantly among non-Caucasian populations. Treating sickle cell patents is a daily battle and it is essential to continue research into developing new treatments. A number of clinical trials involving medicines are proposed for both adults and children and especially in the field of cell therapy, with gene therapy a promising avenue for curing rare diseases of the red blood cell.

To assist sickle cell patients, a campaign for rare blood donations focusing on sickle cell disease is being launched by our teams within the hospital and on social networks. To give blood, please go to the Erasmus Hospital Blood Donation Centre or donate via any Red Cross blood donation center.

Save the date! Festival Drépanocyt’OSE from 17 to 19 June at Tour & Taxis

During the weekend of 17 June 2023, associations active in the field of combating sickle cell disease are organising a festival to increase public awareness of the disease. Experts from the Erasmus Hospital and the Children's Hospital will also be there to represent professionals from the H.U.B.

• Saturday 18 June from 1 pm to 5 pm at our stand at Tour et Taxis
• Monday 19 June, World Sickle Cell Day, during the conference at Tour & Taxis
• For more information: https://www.festival-drepanocytose.com