Better quality of life with Sickle Cell Disease

Danae Beskers & Rebekka Lennings | 03-06-2021

Sickle Cell Disease (SCD) is a condition that is most common in certain parts of Africa, sometimes even with a prevalence of 45%, however also in the Netherlands about 800 people suffer from the disease. SCD can have serious consequences and targeted treatment is not always available. RR Mechatronics, an experienced developer of diagnostic and analytical lab equipment, therefore intends to develop the instrument ‘Shearox’ supported by a European subsidy.

Sickle cell disease: a hereditary condition with serious consequences
More than 300,000 babies are born each year with a severe form of SCD1, most of which, in Africa, will die before the age of five. In Europe, with much better care available, most SCD patients die between the ages of 40 and 60. The sickle cell variant of blood originated more or less simultaneously with human evolution some 70-150 thousand years ago and has spread from central Africa and into Saudi Arabia or central India because it provided an evolutionary advantage in protecting against malaria. People with SCD have an inherited DNA defect that causes their red blood cells to take the shape of a sickle ("C" shape) instead of the usual round shape. This makes the cells less able to transport oxygen from the lungs to the tissues and organs of the patient. In addition, their abnormal shape allows the cells to clump together more easily which leads to the obstruction of blood vessels, and they are more quickly broken down in the spleen, causing chronic anaemia. People who survive the difficult first years of life can suffer from a wide range of health complaints, such as fatigue, severe pain attacks, strokes and eventually organ damage.

Towards a personalized treatment
Currently, there are some treatments available to make life easier for people with SCD. Although every patient has the exact same genetic mutation, every patient is very different. A correct dose of available medication is therefore very important. If the dose is too high, the patient may experience serious side effects, but if the dose is too low, the patient is at risk of the aforementioned pain attacks and organ damage. RR Mechatronics wants to help to give each unique patient a correct dose, by making it possible to quickly and objectively measure the effect of therapies. In collaboration with UMC Utrecht and the Danish hospital Rigshospitalet, RR Mechatrowants to develop Shearox: an instrument that measures the oxygen affinity of red blood cells with a higher accuracy. This allows a personal and optimized treatment dose to be determined based on the effect the treatment has on the patient. By adjusting the dose quickly and accurately to the individual patient, the quality of life of patients with SCD can be greatly improved. In addition to SCD, measuring the oxygen affinity of red blood cells has great potential for the study of chronic fatigue after a COVID-19 infection (long COVID).

RR Mechatronics is supported in this interesting and important development by the European innovation program Eurostars-2. Innofunding has supported RR Mechatronics in writing the subsidy application and obtaining the subsidy in order to further stimulate the implementation of their technological development.