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Written By: Agencies | Published : June 18, 2015 5:37 PM IST
Sickle cell disease includes disorders such as sickle anaemia and thalassaemia, among others, depending upon which haemoglobin gene is abnormal in the person. @Shutterstock
According to the World Health Organization, approximately 5% of the world s population carries trait genes for hemoglobin disorders, mainly sickle-cell anemia and thalassemia. Sickle Cell Anemia is an inherited form of anemia wherein there exists a dearth in the number of healthy red blood cells in the body which play the essential role of carrying adequate amounts of oxygen to all its parts. The lack of oxygen supply caused due to sickle cell anemia makes a person vulnerable to acute bacterial infections, necrosis, and chronic pain syndromes. The damage caused by sickle cell anemia is not just restricted to the immune system of the body and associated symptoms; it hampers the reproductive health of the individual as well , said Dr. Kaberi Banerjee, IVF and Infertility specialist and the Clinical Director of Advance Fertility & Gynecological Center, New Delhi, on the occasion of World Sickle Cell Awareness Day.
Sickle cell anaemia -- here's a brief description
The red blood cells in a healthy human being are flexible and round and they move easily through the blood vessels. In a person suffering from sickle cell anemia, the red blood cells are rigid, sticky and shaped like sickles or crescent moons. These irregularly shaped cells can get stuck in small blood vessels, which can slow or block blood flow and oxygen to parts of the body.
There are various ways in which human fertility is affected by sickle cell disease. Research has been mainly focused on males as opposed to females in this regard. However, the condition affects both the sexes equally. The effect of sickle cell anemia begins with delayed growth and pubertal development. Sexual maturation is usually delayed by 1.5 to 2 years on an average; however most young adults go on to develop it in a normal fashion.
Reproductive health and SCA -- what you need tot know?
According to Dr. Kaberi Banerjee, Regular blood transfusions are necessitated in patients with Sickle Cell Anemia. Iron toxicity due to transfusion overload could cause a reduction in hormone secretion in both men and women. Zinc supplementation is found to aid the growth and increase testosterone levels in males with sickle cell disease.
Testosterone undecanoate injections and clomiphene have been found effective in increasing the libido of male patients and curing erectile dysfunction. Hydroxyurea therapy has evolved to be one of the preferred modes of treatments for these patients. It is important to note that fertility preservation is extremely important for female patients suffering from the disease for they are at risk for pregnancy-related complications as well as potential teratogenicity.
Preimplantation genetic diagnosis can go a long way in producing a genetically normal offspring. World Sickle Cell awareness Day is a good platform to raise awareness about this lesser known diseases and the various fertility management techniques that are available today for patients.
Statistics of sickle cell anemia (SCA)
About 24% of males with sickle cell disease have significantly smaller testicular volume and lower testosterone concentration. Semen analysis in men with the sickle cell disease shows abnormalities in up to 90% of patients. These may include low sperm density/count, low active motility, and abnormal morphology. In the case of females, delayed puberty and sexual maturation can be the cause of delayed pregnancy. Menarche is delayed in young girls but when it comes to the patterns of bleeding, they are as per normal standards. The only bothersome feature is that menses are associated with sickling related pains. This can be overcome to an extent by Depot medroxyprogesterone acetate (DMPA) injections or progesterone -only pills.
The stress of avoiding unwanted/unplanned pregnancies is paramount in patients affected with the disease. Thus, for the prevention of unwanted pregnancies, either barrier or depot progesterone pills are recommended. It is the onus of the medical fraternity to properly counsel patients with sickle cell anemia so that they can give way to a healthy progeny.
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Image Source: Shutterstock
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