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Sickle Cell Disease

सिकलसेल आजार · सिकल सेल रोग

सिकलसेल आजार: सिकलसेल हा अनुवंशिक रक्तविकार आहे. वारंवार अंगदुखीचे झटके, रक्तक्षय, कावीळ ही लक्षणे आहेत. हायड्रॉक्सियुरिया औषध व नियमित तपासणीने रुग्ण सामान्य आयुष्य जगू शकतो. लग्नापूर्वी तपासणी करा.

Sickle cell disease is an inherited disorder in which red blood cells become sickle-shaped, block small blood vessels and break down early. It is common in many districts of Maharashtra, including Vidarbha and parts of Marathwada.

Symptoms

  • Repeated episodes of severe pain in bones, chest or abdomen ("crisis")
  • Anaemia, jaundice, enlarged spleen in children
  • Frequent infections, swelling of hands and feet in infants
  • Stroke, chest problems or priapism in severe cases

Treatment and care

  • Hydroxyurea – reduces pain crises and hospital admissions; suitable for most patients including children
  • Folic acid, vaccinations and penicillin prophylaxis in young children
  • Plenty of fluids, avoiding extreme cold and dehydration
  • Transfusion when needed; bone marrow transplant consultation for selected patients

Prevention

A simple blood test (HPLC) before marriage or in early pregnancy identifies carriers. If both partners are carriers, prenatal testing is available.