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Celebrating 60 Years of Caring

Celebrating 60 Years of Caring. NHF Nursing Luncheon November 14, 2008 Jim Munn, RN, MS. Hemophilia: Early Observations - Second Century.

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Celebrating 60 Years of Caring

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  1. Celebrating 60 Years of Caring NHF Nursing Luncheon November 14, 2008 Jim Munn, RN, MS

  2. Hemophilia: Early Observations- Second Century For it was taught: If she circumcised her first child and he died [as a result of bleeding from the operation] and a second one died [similarly], she must not circumcise her third child. R. Judah, the Patriarch, redactor of the Mishnah Rosner F. Medicine in the Bible and the Talmud, revised ed, New York: Yeshiva University Press, Ktav Publishing House, 1995.

  3. Early Observations Tenth Century Islamic Surgeon Abul Qasim Al-Zahravi (Albucasis) 936-1013 CE • Considered greatest surgeon of Middle Ages • Referred to a condition interpreted by scholars as hemophilia in his encyclopedic work on medicine and surgery, al-Tasrif Al-Tasrif Uman ‘Ajiza An Al-Taalif.

  4. Early Observations Twelfth Century Physician and Talmudist Maimonides states in the Mishneh Torah1 “If a woman had her first son circumcised and he died as a result of the circumcision, which enfeebled his strength, and she similarly had her second [son] circumcised and he died as a result of the circumcision - whether [the latter child] was from her first husband or her second husband - the third son may not be circumcised at the proper time [on the eighth day of life]…”2 1. Rosner F. “Moses Maimonides.” Ann Intern Med. 1965;372:1135-1204. 2. Mishneh Torah, Hilkhot Milah.1:18.

  5. Early Observations 1803: John Conrad Otto • Provided first accurate account of hemophilia in the modern medical literature • His investigation of “bleeders” was published in a New York journal under the title, “An Account of an Hemorrhagic Disposition Existing in Certain Families” • Traced to woman who settled in Plymouth, New Hampshire in 1720 Otto JC. The Medical Repository.1803;Vol VI (No 1):1-4.

  6. Early Observations 1820:Nasse of Bonn • Germanphysician 1778-1851 • Formulated observations on inheritance of hemophilia • “Nasse’s Law” • Transmitted by unaffected females to their sons Nasse CF. Arch Med Erfahr. 1(1820):385.

  7. Early Observations • 1828: The word “hemophilia” first appears in a description of inherited bleeding disorders by Physician Frederick Hopff at the University of Zurich • 1840: First recorded case of hemophilia treatment by transfusion written by Samuel Lane in The Lancet 1 • 1893: First documentation of abnormal prolongation of coagulation in capillary tube in hemophilics2 1. Farr AD. J Royal Soc Med.April 1981;74(4):301-305. 2. Wright AE. Br Med J.1893;2:223-225.

  8. Early Observations: Other Highlights of the Early 1900s • 1911: Publication of “monumental review of pedigrees of families with bleeding disorders”1 • 1920-1930: Hemophilia treatments published; plasma for transfusions introduced • 1926: Erik vonWillebrand describes bleeding disorder affecting both sexes • 1937: IV administration of redissolved plasma precipitate shown to shorten blood clotting time2 • 1937: First permanent blood bank established in US 1. Ingram GIC. J Clin Pathol.1976;6:3. 2. Brinkhous KM. A Short History of Hemophilia. Handbook of Hemophilia. New York: American Elsevier, 1975.

  9. 1940s Early to Mid-1940s: Medical progress accompanies war • Charles Drew sets standard for collecting, storing and transporting plasma under “battlefield conditions” • Edwin Cohn, Harvard biochemist, first to use fractionation process to produce albumin in 19441 • Albumin usefulness spurs research in blood component therapy by “clotters” • 1946-1947: Hemophilia defect in plasma component2 1. Latham A Jr. Vox Sang. 1986;51(3):249-52. 2. Brinkhous KM. Proc Soc Exper Biol and Med. Oct. 1947;66:117-120.

  10. 1940s 1948: A golden decade for coagulation research begins • Hemophilia Foundation established 6/15/48 • Hemophilic dog colony established: Chapel Hill, North Carolina ©UNC Center for Thrombosis and Hemostasis Smith N. A History of the National Hemophilia Foundation. New York: National Hemophilia Foundation, 1984.

  11. 1950s • First recommendation for home infusion • 1959: First hemophilia treatment center in Rochester, NY Photos of Mary Gooley (above) courtesy of the Mary M. Gooley Hemophilia Center, Rochester, NY Smith N. A History of the National Hemophilia Foundation. New York: National Hemophilia Foundation, 1984.

  12. 1950s Biggs R, et al. Br Med J.Dec. 27, 1952;1378. With permission from BMJ Publishing Group.

  13. 1950s 1952: Evolution of the definition of hemophilia: • A blood clotting disorder affecting males with two possible major protein deficiencies: • FVIII - Hemophilia A • FIX - Hemophilia B • Thromboplastin generation test for measuring FVIII developed • Coagulation cascade discovered Langdell RD, et al. J Lab Clin Med. 1953;41:637-647.

  14. 1950s 1958: First use of prophylaxis in Hemophilia A conducted in Sweden by Prof. Inga Marie Nilsson

  15. 1960s • 1960s:NIH grants create research climate • 1961: Hyland Laboratories begins work on FVIII concentrate • 1966: Peanut flour touted as treatment for hemophilia – Nature 1 1. Nature. February 13, 1969. Vol. 185, No. 4711, pp. 469-470.

  16. 1960s 1965: Discovery of Cryoprecipitate Judith Graham Pool, MD File photo courtesy of HANDI, NHF. Pool JG, Shannon AE. N Engl J Med.1965:273:1443-1447.

  17. 1960s • 1966: Hyland announces commercial availability of FVIII concentrates • 1969:FIX concentrate licensed1 1. Hoag MS, et al. N Eng J Med.1969;280(11):581-6.

  18. 1960s Continuous infusion • Predictable and sustained level over time • Cost savings due to decreased • Laboratory evaluations • Clearance over time • Ideal replacement for surgical interventions and specific bleeding episodes in hemophilia McMillan CW, et al. Br J Haematol. 1970;18:659-667. Hathaway WE, et al. Am J Hematol.1984;17:85-88. Shulman NR, et al, Ann Intern Med. 1967;67:856.

  19. 1960s 1969: First hemophilia camp established

  20. 1970s • 1970s:Home infusion therapy: a common treatment practice • 1971: von Willebrand’s factor identified¹ • 1974: First successful immune tolerance² • 1975: Federally funded comprehensive hemophilia treatment centers initiated • 1977: Mannucci discovers DDAVP increases FVIII and vWF levels3 • Zimmerman, TS, et al. J Clin Invest.1971;50:244-254. • Brackmann HH, Lancet 1977; 2 (8044): 933. • Mannucci PM. Lancet. 1977;1:869-872.

  21. Regional Hemophilia Treatment Center Network

  22. 1970s Revisiting use of Prophylaxis UK study – 9 boys with Hem A <1% • Randomized, blinded, crossover study • Had target joints present • Received 25% correction with FVIII or placebo once a week, followed by week of rest • Showed 15% reduction in bleeding episodes in 100 day period (from 13.6 episodes to 11.6) • 66% decrease in bleeds in first three days of the week Aronstam. 1976. Brit J Haematol. 33:81. .

  23. 1970s Transmission of blood-borne diseases: • Hepatitis B: HBV • Hepatitis non-A, non-B: Later called “C” • HIV • Mannucci PM, et al. J Clin Pathol.1975;28(8):620-624. • Schramm W, et al. Blut.1989;59(4):390-392.

  24. Hepatitis B Virus: HBV • Lipid-enveloped DNA virus • Replicates within liver cells • Transmitted by exchange of bodily fluids • 90% recover with immunity; 10% develop chronic HBV of which 20-30% progress to cirrhosis • Virion sensitive to heat and solvent/detergent • 1981: Hep B vaccine plasma derived • 1987: Hep B vaccine recombinant licensed Electron micrograph of Hepatitis B Virions courtesy of the CDC

  25. The Price of Independence Non-A, non-B (Hepatitis C)recognized as transmissible via plasma-derived products • 1986: Commercial screening for non-A, non-B hepatitis surrogate markers • 1989: Hepatitis C isolated • 1990: Hepatitis C Ab testing of donated blood begins

  26. Hepatitis C Virus: HCV • Lipid-enveloped RNA virus • Replicates within infected liver cells • Transmitted by the exchange of bodily fluids • 85% or more with acute HCV infection progress to chronic hepatitis* • Virion sensitive to heat and solvent/detergent • No vaccine available * Seeff LB. Am J Med.Dec. 1999;107[6B]:10S-15S.

  27. 1980s MMWR July 16, 1982/31(27):365-367.

  28. The Price of Independence • 1983: Suspicion that HIV threatened the worldwide blood supply • April 1, 1983: Hemofil-T, first heat-treated FVIII concentrate in the U.S. • 1984:Montagnier1 and Gallo2 discover HTLV-3 (HIV) • 1984:Efficacy of heat treatment for viral inactivation demonstrated • 1984:Recall of blood products initiated • 1985: ELISA test used to detect HIV antibodies among blood donors • 1985: Safety net: • Donor deferral • Viral inactivation methods • HIV antibody testing 1. Barre-Sinoussi F, et al. Science 1983; 220(4599):868-71. 2. Gallo RC, et al. Science 1984; 4;224(4648):500-3.

  29. The Heroes We Care For Photo courtesy of Mary Lou Cygan, R.N., P.N.P.

  30. 1980s

  31. 1990s • 1991 – Karolinska University – Stockholm, Sweden Dr. Petrini and colleagues describe prevention of joint disease in boys with hemophilia under age two. Lusher, J. 2008. HemAware. 13: 8.

  32. 1990s Soucie JM, et al. Blood.2000;96(2):437-442.

  33. 1990s Mortality decreased 40% in patients using a comprehensive hemophilia treatment center (HTC) “The finding that HTCs have a significant effect on reducing mortality in patients with hemophilia supports the effectiveness of such centers in providing specialized preventative care.” Soucie JM, et al. Blood. 2000;96(2):437-442.

  34. 1990s Prophylaxis recommendation • Primary • Early institution ~ 1-2 years with the aim of maintaining trough > 1% • Longitudinal assessment of joint status essential • Secondary • Appropriate for intervention to prevent joint and other disease associated morbidities MASAC Recommendation #35. March 11, 1994.

  35. 2000s • Gene therapy trials begun • Joint Outcome Study published1 • National Pain Survey • Willetabs and Wilbrintin advertised as treatment for vWD Manco-Johnson, et al. NEJM. 2007. Vol. 357:(6) 535-544.

  36. Hemophilia A: Evolution of Therapy

  37. Hemophilia B: Evolution of Therapy

  38. The Future • Longer acting concentrates • Recombinant therapy for vWD • Alternate route therapies • Gene transplantation • Elimination of transfusion-associated infections • Understand and overcome inhibitor development • Quality of life issues • Elimination of joint morbidity • Optimizing the individual’s social and academic performance

  39. The Future… …the promise of achieving your potential

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