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Management of Haemophilia in Developing Countries: Challenges and Options

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Abstract

There are significant challenges in managing haemophilia patients in developing countries. These challenges are (i) Lack of proper health care infrastructure and human resources suitable for haemophilia care (ii) Competing health care priorities of the government. (iii) Lack of penetrance of medical insurance in the population. (iv) Lesser visibility of the haemophilia patients in health care system (v) Low awareness across the medical profession, population and the policy makers about the condition (vi) Non availability of factor concentrates (vii) Inadequate utilization of knowledge for reducing factor concentrate use. (viii) Inadequate pain relief (ix) Challenges due to inhibitor developing (x) Viral hepatitis & (xi) Lack of research publications relevant to the country are some of the challenges faced by PWH for their management in developing country. The solutions are not easy but development of a strong patient organization with linkages with World Federation of Haemophilia is an important initial step. Following that internal and international twinning, use of internal sources, strong advocacy programme targeting government, doctors, opinion makers will solve many of the challenges in the time to come.

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References

  1. Lee CA, Berntorp EE (2010) Textbook of hemophilia, 2nd edn. Wiley Blackwell, London

    Book  Google Scholar 

  2. Ghosh K, Shetty S, Ghosh K (2008) Hemophilia: a high cost low volume disease: suitable preventive strategies for developing and developed countries. Open Hematol J 2:20–24

    Article  Google Scholar 

  3. Ghosh K (2009) Challenges of haemophilia care in India: lest we forget. Indian J Med Res 130:87–88

    CAS  PubMed  Google Scholar 

  4. Kuehn BM (2007) Global shortage of health workers, brain drain stress developing countries. JAMA 298(16):1853–1855

    Article  CAS  PubMed  Google Scholar 

  5. Ruiz-Sáez A (2012) Comprehensive care in hemophilia. Hematology 1:S141–S143. doi:10.1179/102453312X13336169156492

    Google Scholar 

  6. Dharmarajan S, Phadnis S, Gund P, Kar A (2014) Out-of-pocket and catastrophic expenditure on treatment of haemophilia by Indian families. Haemophilia 20:382–387

    Article  CAS  PubMed  Google Scholar 

  7. Khanum F, Collins P, Bowen DJ (2013) Diagnosis of haemophilia in Pakistan: current picture. J Coll Phys Surg Pak 23:450–451

    Google Scholar 

  8. Moeslichan S, Setianingsih I, Gatot D, Abdulsalam M, Munthe BG (1993) The management of hemophilia in Jakarta. Southeast Asian J Trop Med Public Health 24(Suppl 1):274

    PubMed  Google Scholar 

  9. Stonebraker JS, Brooker M, Amand RE, Farrugia A, Srivastava A (2010) A study of reported factor VIII use around the world. Haemophilia 16:33–46

    Article  CAS  PubMed  Google Scholar 

  10. Evatt B (2006) Infectious disease in the blood supply and the public health response. Semin Hematol 43(2 Suppl 3):S4–S9

    Article  PubMed  Google Scholar 

  11. Ghosh K, Joshi SH, Shetty S, Pawar A, Chipkar S, Pujari V, Madkaikar M, Pathare AV, Jijina F, Mohanty D (2000) Transfusion transmitted diseases in haemophilics from western India. Indian J Med Res 112:61–64

    CAS  PubMed  Google Scholar 

  12. Goedert JJ, Brown DL, Hoots K, Sherman KE (2004) Human immunodeficiency and hepatitis virus infections and their associated conditions and treatments among people with haemophilia. Haemophilia 10(Suppl 4):205–210

    Article  PubMed  Google Scholar 

  13. Ramia S, Klayme S, Naman R (2003) Infection with hepatitis B and C viruses and human retroviruses (HTLV-I and HIV) among high-risk Lebanese patients. Ann Trop Med Parasitol 97:187–192

    Article  CAS  PubMed  Google Scholar 

  14. Sørensen B, Benson GM, Bladen M, Classey S, Keeling DM, McLaughlin P, Yee TT, Makris M (2012) Management of muscle haematomas in patients with severe haemophilia in an evidence-poor world. Haemophilia 18:598–606

    Article  PubMed  Google Scholar 

  15. Holstein K, Klamroth R, Richards M, Carvalho M, Pérez-Garrido R, Gringeri a (2012) Pain management in patients with haemophilia: a European survey. European haemophilia therapy standardization board. Haemophilia 18:743–752

    Article  CAS  PubMed  Google Scholar 

  16. Ghosh K, Ghosh K (2008) Management of chronic synovitis in patients with hemophilia: with special reference to developing countries. Indian J Hematol Blood Transfus 24:151–154

    Article  PubMed  Google Scholar 

  17. Rodriguez-Merchan EC (2003) Radionuclide synovectomy (radiosynoviorthesis) in hemophilia: a very efficient and single procedure. Semin Thromb Hemost 29(1):97–100

    Article  PubMed  Google Scholar 

  18. Radossi P, Baggio R, Petris U, De Biasi E, Risato R, Davoli PG, Tagariello G (2003) Intra-articular rifamycin in haemophilic arthropathy. Haemophilia 9:60–63

    Article  CAS  PubMed  Google Scholar 

  19. Heijnen L, Roosendaal G, Heim M (1997) Orthotics and rehabilitation for chronic hemophilic synovitis of the ankle. An overview. Clin Orthop Relat Res 343:68–73

    Article  PubMed  Google Scholar 

  20. Kale JS, Ghosh K, Mohanty D, Pathare AV, Jijina F (2000) Use of the dual force system to correct chronic knee deformities due to severe haemophilia. Haemophilia 6:177–180

    Article  CAS  PubMed  Google Scholar 

  21. Heijnen L, Heim M, Der Maur IH (2000) World Federation of Hemophilia. Hemophilia. Manufactured shoes and orthopaedic shoes. Haemophilia 6(Suppl 1):4–6

    Article  PubMed  Google Scholar 

  22. Negrier C, Seuser A, Forsyth A, Lobet S, Llinas A, Rosas M, Heijnen L (2013) The benefits of exercise for patients with haemophilia and recommendations for safe and effective physical activity. Haemophilia 19:487–498

    Article  CAS  PubMed  Google Scholar 

  23. Ghosh K, Madkaikar M, Jijina F, Shetty S (2007) Fractures of long bones in severe haemophilia. Haemophilia 13:337–339

    Article  CAS  PubMed  Google Scholar 

  24. Lee VN, Srivastava A, Nithyananth M, Kumar P, Cherian VM, Viswabandya A, Mathews V, George B, Venkatesh K, Nair SC, Chandy M, Sundararaj GD (2007) Fracture neck of femur in haemophilia A—experience from a cohort of 11 patients from a tertiary centre in India. Haemophilia 13:391–394

    Article  CAS  PubMed  Google Scholar 

  25. Ghosh K, Nair AP, Jijina F, Madkaikar M, Shetty S, Mohanty D (2005) Intracranial haemorrhage in severe haemophilia: prevalence and outcome in a developing country. Haemophilia 11:459–462

    Article  CAS  PubMed  Google Scholar 

  26. Lowe R (2004) Trial of haemophilia treatment for intracerebral haemorrhage. Lancet Neurol 3(8):448

    Article  PubMed  Google Scholar 

  27. Ghosh K, Jijina F, Shetty S, Madkaikar M, Mohanty D (2002) First-time development of FVIII inhibitor in haemophilia patients during the postoperative period. Haemophilia 8:776–780

    Article  CAS  PubMed  Google Scholar 

  28. Ghosh K, Shankarkumar U, Shetty S, Mohanty D (2003) Chronic synovitis and HLA B27 in patients with severe haemophilia. Lancet 361(9361):933–934

    Article  CAS  PubMed  Google Scholar 

  29. Rattray B, Nugent DJ, Young G (2006) Celecoxib in the treatment of haemophilic synovitis, target joints, and pain in adults and children with haemophilia. Haemophilia 12:514–517

    Article  CAS  PubMed  Google Scholar 

  30. Ragni MV, Ojeifo O, Feng J, Yan J, Hill KA, Sommer SS, Brambilla DJ, Trucco MN (2009) HemophiliaInhibitor Study. Risk factors for inhibitor formation in haemophilia: a prevalent case-control study. Haemophilia 15:1074–1082

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  31. Aledort LM (2009) Mild and moderate factor VIII deficiency: inhibitor risks. J Thromb Haemost 7:928–929

    Article  CAS  PubMed  Google Scholar 

  32. Ghosh K, Shetty S, Kulkarni B, Nair S, Pawar A, Khare A, Baindur S, Mohanty D (2001) Development of inhibitors in patients with haemophilia from India. Haemophilia 7:273–278

    Article  CAS  PubMed  Google Scholar 

  33. Spero JA, Lewis JH, Van Theil DH, Hasiba U, Rabin BS (1978) Asymptomatic structural liver disease in hemophilia. N Engl J Med 298:1373–1378

    Article  CAS  PubMed  Google Scholar 

  34. Hasiba UW, Spero JA, Lewis JH (1977) Chronic liver dysfunction in multitransfused hemophiliacs. Transfusion 17:490–494

    Article  CAS  PubMed  Google Scholar 

  35. Ghosh K, Shetty S, Sahu D (2010) Haemophilia care in India: innovations and integrations by various chapters of Haemophilia Federation of India (HFI). Haemophilia 16:61–65

    Article  CAS  PubMed  Google Scholar 

  36. O’Mahony B, Black C (2005) Expanding hemophilia care in developing countries. Semin Thromb Hemost 31:561–568

    Article  PubMed  Google Scholar 

  37. Skinner MW (2012) WFH: closing the global gap–achieving optimal care. Haemophilia 18(Suppl 4):1–12

    Article  PubMed  Google Scholar 

  38. Öner AF, Doğan M, Kaya A, Sal E, Bektaş MS, Yesilmen O, Ayhan H, Acikgoz M (2010) New coagulant agent (ankaferd blood stopper) for open hemorrhages in hemophilia with inhibitor. Clin Appl Thromb Hemost 16:705–707

    Article  PubMed  Google Scholar 

  39. Kundu T, Shaikh A, Kutty A, Nalvade A, Kulkarni S, Kulkarni R, Ghosh K (2012) Homeopathic medicines substantially reduce the need for clotting factor concentrates in haemophilia patients: results of a blinded placebo controlled cross over trial. Homeopathy 101:38–43

    Article  PubMed  Google Scholar 

  40. Ghosh K (2004) Management of haemophilia and its complications in developing countries. Clin Lab Haematol 26(4):243–251

    Article  CAS  PubMed  Google Scholar 

  41. Ghosh K, Shetty S (2012) Bone health in persons with haemophilia: a review. Eur J Haematol 89:95–102

  42. Ghosh K, Shetty S, Jijina F, Mohanty D (2004) Role of epsilon amino caproic acid in the management of haemophilic patients with inhibitors. Haemophilia 10:58–62

    Article  CAS  PubMed  Google Scholar 

  43. Shetty S, Ghosh K, Jijina F (2006) First-trimester prenatal diagnosis in haemophilia A and B families–10 years experience from a centre in India. Prenat Diagn 26:1015–1017

    Article  PubMed  Google Scholar 

  44. Shetty S, Ghosh K (2007) Robustness of factor assays following cordocentesis in the prenatal diagnosis of haemophilia and other bleeding disorders. Haemophilia 13:172–177

    Article  CAS  PubMed  Google Scholar 

  45. Nair AP, Jijina F, Ghosh K, Madkaikar M, Shrikhande M, Nema M (2007) Osteoporosis in young haemophiliacs from western India. Am J Hematol 82:453–457

    Article  PubMed  Google Scholar 

  46. Ghosh K, Shetty S (2012) Malignancies in persons with haemophilia: 25-year data from India. Natl Med J India 25:251–252

    PubMed  Google Scholar 

  47. Shetty S, Ghosh K (2012) Cancers in patients with hemophilia: a retrospective study from the Italian Association of Hemophilia Centers: a rebuttal. J Thromb Haemost 10:1200–1201

    Article  CAS  PubMed  Google Scholar 

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Acknowledgments

Author acknowledges Haemophilia Federation of India, various haemophilia chapters in the country, Dr Devila sahu, Dr Shrimati Shetty and Dr Bipin Kulkarni of haemostasis unit in National Institute of Immunohaematology for helping us to write the article.

Conflict of interest

None declared.

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Authors and Affiliations

  1. National Institute of Immunohaematology, 13 Th Fl KEM Hospital, Parel Mumbai, 400012, India

    Kanjaksha Ghosh & Kinjalka Ghosh

  2. Department of Biochemistry, Seth GS Medical College and KEM Hospital, Parel Mumbai, 400012, India

    Kinjalka Ghosh

  3. Haemophilia Federation of India, New Delhi, India

    Kanjaksha Ghosh

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  1. Kanjaksha Ghosh
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  2. Kinjalka Ghosh
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Correspondence to Kanjaksha Ghosh.

Additional information

This paper formed a part of Dr A J Desai oration delivered in TN Medical college, Mumbai on 25th April 2015

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Ghosh, K., Ghosh, K. Management of Haemophilia in Developing Countries: Challenges and Options. Indian J Hematol Blood Transfus 32, 347–355 (2016). https://doi.org/10.1007/s12288-015-0562-x

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  • DOI: https://doi.org/10.1007/s12288-015-0562-x

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