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World Sickle Cell Day 2018: Causes, Symptoms, Treatment of the painful inherited blood disorder

World Sickle Cell Day 2018: Causes, Symptoms, Treatment of the painful inherited blood disorder: A healthy person has spherical red blood cells, but some can suffer from the World Sickle Cell Day which is regarded as rigid, sticky and crescent-shaped, sickle-like red blood cells (RBCs).

This one is inherited blood disorder which can be acquired from either or both the parents. In this condition, The irregular shaped RBCs, get stuck in the small blood vessels and slow down or block the blood flow and oxygen to the several parts of the body.

This one is also regarded as a painful disease and while there is no cure yet for sickle cell anaemia, certain treatments can help relieve the pain and also avoid ailments associated with this disorder anyway.

the United Nations General Assembly in 2008, established, the World Sickle Cell Day which was by to increase awareness about this genetic disorder that can be cured and managed, if diagnosed will be caught early.

What are the symptoms?

A senior consultant, Dr. Amita Mahajan, of the Paediatric Oncology and Haematology, Indraprastha Apollo Hospitals shares and note down the symptoms that a person suffering from SCD might show.

* Patients can develop low hemoglobin, requiring transfusions.

* They can also develop periodic episodes of pain, called crises starting from the first year of life, which can be very disabling.

* Such patients are also prone to developing repeated pneumonia, painful swelling of hands and feet, sudden enlargement of the spleen (an organ that fights infection), stroke, vision problems, delayed growth and other complications of the bone.

What are the causes?

This one is an inherited disorder and even if one of the parents has it, the child is prone to the disease and may have one normal haemoglobin gene and other one defective form of the gene. Thus, their bodies will produce both normal and sickle cell haemoglobin. though, if both the parents have an affected gene, then the child can also develop and prone to the disease anyway.

What are the precautions one can take?

* Dehydration and lack of oxygen must be kept away from.

* Patients must remain hydrated at all times and should take extra care if they are traveling to higher altitudes.

* Patients affected by the disease need to follow up with haematologists on a regular and daily basis.

What is the treatment available?

* Patients with this condition can have additional folic acid and protection from pneumonia which is the form of a vaccine and antibiotic prophylaxis.

* Hydroxyurea can considerably benefit patients suffering from this disease in reducing or eradicate the needed for blood transfusion, reduction in painful episodes and a decrease in the risk of stroke, these are some other complications associated with the disease.

* Allogeneic Bone Marrow Transplant, is The only curative treatment of the disease which also has a very high success rate. If there is Human Leukocyte Antigen (HLA) matched donor in the family, then that donor will be preferred the most. Though, if there is no matched family donor, a potential donor can be searched from the Bone Marrow Donor Registries. This is regarded as the Matched Unrelated Donor transplant.

* Currently, newborn screening programs have been started in certain parts of India; so that affected babies can be identified early and proper treatment can begin to give them. If there is an affected child in a family, this is quite possible to check for this condition in the next pregnancy so that the family has the option of terminating the pregnancy anyway.