Micro- and Macrovascular Function in Children with Sickle Cell Anemia and Sickle Cell Hemoglobin C Disease

Knowing your body’s physical capabilities is a very important aspect in life. No one is exactly the same so we all fluctuate in how we perform different activates. Some things you can’t help, many people are born with health issues which take a huge toll on their physical capabilities. Hereditary defects can be passed on and you don’t get a chance to say, “No! I don’t want it.”, or just work past it. Somethings you just have to learn to live with it. Sickle cell anemia, or sickle cell disease, is just one example of such an issue. 


 
Sickle cell is an inherited mutation in the hemoglobin, which causes the proteins and red blood cells to take on a nonflexible shape. It causes a lack of oxygen and other gasses in the blood to be supplied to the body. The fact that these affected red blood cells stick to vessel walls and block blood flow induces the question if micro- and macrovascular functions can be affected in those that have this disease.

Based on the number of copies of the gene inherited different forms of sickle cell can occur. If both copies are affected the ending result is sickle cell anemia however, if only one copy is affected sickle cell hemoglobin is prominent. Sickle cell anemia typically causes death early on in life while sickle cell hemoglobin can be managed. The shape of the mutant protein allows for an odd change in shape in the red blood cells, where hemoglobin is found. The odd shape is believed to affect the flow of blood which in return affects the vascular systems ability to function. 

A French study by Berenike Mockesch and colleagues examined if the circulatory systems of children with sickle cell disease were indeed affected by their disease. It was unclear whether vascular function truly was affected before this study. In order to figure out the micro- and macrovascular function in children with sickle cell anemia and sickle cell hemoglobin C disease this study was performed. 

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Sixty-two adolescents were used for the study. Their age ranged from 10 to 16 years old. 16 were in normal health, 22 had sickle cell hemoglobin C, and 24 had sickle cell anemia- the two variations of the disease.  All of the children were monitored since their birth in Guadeloupe. Four tests were used to determine if vascular function was indeed affected by the presents of sickle cell hemoglobin C and sickle cell anemia, and if that related to their fitness level. The thermal hyperemic response was tested by the finger pad warming to about 40 degrees Celsius. This informed the researcher on how well the children could lower their temperatures when heated. Arterial stiffness measurements informed the scientists about the overall health of the muscular system. Increase stiffness is related to increased risk of heart attacks and stroke in adults. The children were also assessed on their cardiovascular activity using a questionnaire. Finally, the children were subjected to a fitness test which was evaluated by a 6-minute walking test. This test was performed with the patient walking as fast as possible, not running.

Overall, patients with sickle cell anemia had the most difficult cardiovascular health and those with sickle cell disease only showed reduced cooling capacity when compare to normal children. Yet all patients did have decreased fitness levels. It’s clear to see that children can easily be worked too hard both normal and with a sickle cell disease. Those with sickle cell diseases however are not able to recover as well as normal children. Sickle cell anemia children may have a shorter life span due to their poor cardiovascular health unlike sickle cell hemoglobin children that can manage their symptoms by altering their lifestyle. Those with a sickle cell disease should learn the activities that make them struggle and monitor their lifestyle to avoid them. Sickle cell disease is not something that will go away overtime it’s a forever disease that you must live with. Monitoring activity and increasing support will in return allow for a brighter future as an adult. 

Read about it here. 

References 

Mockesch, Berenike; Charlot, Keyne; Jumet, Stephane; Romana, Marc; Divialle-Doumdo,

Lydia; Hardy-Dessources, Marie-Dominique; Petras, Marie; Tressieres, Benoit; Tarer, Vanessa; Hue, Olivier; Etienne-Julan, Maryse; Connes, Philippe; Antoine-Jonville, Sophie. “Micro-and macrovascular funtion in children with sickle cell anaemia and sickle cell haemoglobin C disease”. Blood Cells, Molecules and Diseases, vol. 64, 2017, pp. 23-29, http://www.sciencedirect.com/science/article/pii/S1079979616302832.

National Heart, Lung, and  Blood Institue. “Normal Red Cells and Sickle Red Cells”. What Is

Sickle Cell Disease?, August 2, 2016, https://www.nhlbi.nih.gov/health/health-topics/topics/sca/.