<p>The magnitude of the problem of sickle cell disease (SCD) in India is very large and diverse. For people living with SCD there are several unmet health care needs making it pertinent to focus on recognizing the important public health burden of SCD in the country. In a bid to achieve this goal, the Indian College of Hematology (ICH) of the Indian Society of Hematology and Blood Transfusion (ISHBT) in collaboration with Indian Council of Medical Research (ICMR) has formulated a Guideline on Management and Control of Sickle Cell Disease in India. This was achieved by a national taskforce comprising top experts from the field who held multiple sessions to deliberate on different aspects and form consensus on preparing a comprehensive document on SCD care in India. The taskforce has presented its recommendations on various aspects of SCD in India, all of which have been evaluated based on the best evidence. Where the evidence was weak, the recommendations have been based on consensus among the experts. This review which is adapted from the comprehensive guideline document gives a concise treatise on various aspects of sickle cell disease in India along with specific recommendations. The section on screening and diagnosis provides an evidence-based background to scientifically select the most appropriate and feasible test option(s) in various Indian practice settings. The other aspects covered in this review are management of SCD in stable condition, iron overload and chelation therapy, vaso-occlusive crisis, management of other complications of SCD, blood transfusion in SCD, haematopoietic stem cell transplant and gene therapy in SCD, immunization and antibiotic prophylaxis in SCD and monitoring of patients with SCD. It also gives guidance on special circumstances such as pregnancy and surgery in SCD. It concludes with a note on prevention and control of SCD in India with an outline of a defined roadmap to make this possible.</p>

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Indian College of Hematology (ICH)& Indian Council of Medical Research (ICMR) Joint Consensus Document on Management and Control of Sickle Cell Disease in India

  • Rabindra Kumar Jena,
  • Manoranjan Mahapatra,
  • Tuphan Kanti Dolai,
  • Rakhee Kar,
  • Maitreyee Bhattacharyya,
  • Vikram Mathews,
  • Bontha V. Babu,
  • Tulika Seth,
  • Dipty Jain,
  • Seema Bhatwadekar,
  • Jina Bhattacharyya,
  • Prashant Sharma,
  • Priyanka Samal,
  • Haraprasad Pati,
  • Sunil Kumar Agarwalla,
  • Somanath Mukherjee,
  • Gopal K. Ray,
  • Damodar Das,
  • S. N. Agragami,
  • Neha Singh,
  • Felicitas Roelofsen,
  • Dinabandhu Sahoo,
  • Moupali Ghosh,
  • Biswa Prakash Patri

摘要

The magnitude of the problem of sickle cell disease (SCD) in India is very large and diverse. For people living with SCD there are several unmet health care needs making it pertinent to focus on recognizing the important public health burden of SCD in the country. In a bid to achieve this goal, the Indian College of Hematology (ICH) of the Indian Society of Hematology and Blood Transfusion (ISHBT) in collaboration with Indian Council of Medical Research (ICMR) has formulated a Guideline on Management and Control of Sickle Cell Disease in India. This was achieved by a national taskforce comprising top experts from the field who held multiple sessions to deliberate on different aspects and form consensus on preparing a comprehensive document on SCD care in India. The taskforce has presented its recommendations on various aspects of SCD in India, all of which have been evaluated based on the best evidence. Where the evidence was weak, the recommendations have been based on consensus among the experts. This review which is adapted from the comprehensive guideline document gives a concise treatise on various aspects of sickle cell disease in India along with specific recommendations. The section on screening and diagnosis provides an evidence-based background to scientifically select the most appropriate and feasible test option(s) in various Indian practice settings. The other aspects covered in this review are management of SCD in stable condition, iron overload and chelation therapy, vaso-occlusive crisis, management of other complications of SCD, blood transfusion in SCD, haematopoietic stem cell transplant and gene therapy in SCD, immunization and antibiotic prophylaxis in SCD and monitoring of patients with SCD. It also gives guidance on special circumstances such as pregnancy and surgery in SCD. It concludes with a note on prevention and control of SCD in India with an outline of a defined roadmap to make this possible.