September is National Sickle Cell Awareness Month
These medical terms are commonly used in association with Sickle Cell Disease. Knowing and understanding these terms will aid you in having an informed conversation with your physician or allied healthcare professional. This will also help parents and other caregivers to better advocate for their loved one living with SCD.
Metabolic: Relating to the chemical process that happens within living organisms to sustain life.
Metabolic disorders are genetic. A protein error or enzyme deficiency in the DNA causes an abnormality that adversely affects proper metabolic function. In Sickle cell disease, two proteins in the hemoglobin molecule switch place. The overall effect of the change is qualitative and quantitative.
In SCD the quantitative deficiency causes the bone marrow to not produce the required amount of blood for healthy metabolic function. Instead of producing 7 to 9 liters (12.3 to 15.85 pints) of blood, a person with sickle cell, produces between 3.41 to 4.55 liters (6 to 8 pints) of blood. Hence, the definition of anemia.
Qualitatively, the red blood cell do not remain round and pliable. Under certain conditions like dehydration and low oxygen, the cells sickle, become stiff and sticky. They clump together and block the flow of blood through capillaries and small veins. Remember vaso-occlusion.
Lyse: the disruption of a blood cell wall that results in its destruction or dissolution of the cell.
Hemolysis: The consistent busting of red blood cells that causes a drop in one’s blood count. SCD is an intrinsic hemolytic anemia. Red blood cells are continually destroyed faster than the bone marrow can replace them.
Aplastic Anemia: Due to some toxic agent; and infection or radiation from an x-ray can cause the bone marrow temporarily stops producing red blood cells. A precipitous drop in blood volume happens. Aplastic anemia is a life threatening complication. It is treated with blood transfusions, folate vitamin, sometimes antibiotics and/or other medication to stimulate the bone marrow back into production.
In SCD the pavovirus B-19 is the predominant cause for aplastic crisis.
Reticulocyte: New immature blood cells without a nucleus that is produced by the bone marrow.
Sickle Syndromes: A combination of symptoms specific to sickle cell disease that can present in any combination. Sickle syndrome is different for each individual living with the disease. Examples of sickle syndrome are:
· Pain episodes — crisis.
· Frequent infections — usually urinary tract infections (UTIs) or pneumococcal.
· Swelling in the area of pain — in children swelling of the extremities is known as Hand and Foot Syndrome.
· Delayed growth.
· Yellowing of the eyes.
· Fever.
· Paleness or greyish tinge to the skin.
· Symptoms of a stroke.
One or a combination of any of these symptoms in children or adults living with sickle cell disease requires immediate medical attention.
Sickle cell disease is defined as a chronic metabolic disorder that is painful and can be terminal without proper diagnosis, treatment and management.
For all of its difficulty, children and adults living with sickle cell can live healthy, productive and long lives.
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The Sickle Cell Thalassemia Patients Network is a Brooklyn-based charity serving individuals living with inherited blood disorders, and their families. Follow SCTPN on Twitter & IG @SCTPN FB @SCDThalPatientsNetwork Like Page @SCTPNWalk