On the 19th of June we celebrate World Sickle Cell Awareness Day. This day we raise the public awareness about the sickle cell disease, sickle cell patients and treatment methods for the disease.
Sickle cell disease (SCD) is an inherited genetic abnormality of hemoglobin (the oxygen-carrying protein) in red blood cells.
Sickle Cell Disease
The signs and symptoms of SCD are caused by the sickling of red blood cells (RBC). When red blood cells sickle, become crescent, they break down prematurely which often results in anemia. Anemia causes shortness of breath, fatigue and delayed growth and development in children. The rapid breakdown of red blood cells can also cause yellowing of the eyes and skin, which are signs of jaundice.
Sickle cell patients experience a lot of pain. This is due to the sickled RBC getting stuck in the blood vessels and the damage they inflict on the organs. Additional deprivation of oxygen-rich blood and concomitant high blood pressure contribute to this damage of the lungs, kidneys, spleen, and brain and can lead to heart failure.
The Lorrca Oxygenscan
For sickle cell patients, treatment options today include managing pain, infections and reducing the frequency of vaso-occlusive crises. But because the disease manifests itself differently with each person, each patient requires a personal individual treatment plan.
Until now there is no method available to determine the severity of the disease or to measure and monitor the effect of a treatment for the individual SCD patient. With our development of the Lorrca Oxygenscan, we believe to have succeeded in doing so. The Lorrca Oxygenscan measures the ‘Point of Sickling’ (PoS) in RBC samples from SCD patients. This unique measurement directly reflects the status of the samples’ susceptibility to oxygen depletion. The PoS is typical for each patients’ RBC sample and can’t be determined by any other technology. It is a functional phenotypic assay to assess SCD.
Substantial patient measurements have been published and presented by our collaborators at different congresses last year. They have shown that the Lorrca Oxygenscan can fully characterize RBC deformability under a range of oxygen concentrations. Key measurements like the PoS were different between genotypes, and changed significantly with standard of care SCD treatments (Rab et al; 2019). Therefore, this might act as a useful biomarker of clinical severity and treatment response, being essential in monitoring existing and novel SCD treatments.
We are aware and motivated to act.
The Lorrca Team of RR Mechatronics