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

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Presentation on theme: "Celebrating 60 Years of Caring NHF Nursing Luncheon November 14, 2008 Jim Munn, RN, MS."— Presentation transcript:

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

2 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, Hemophilia: Early Observations - Second Century Hemophilia: Early Observations - Second Century

3 Early Observations Tenth Century Islamic Surgeon Abul Qasim Al-Zahravi (Albucasis) 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 Early Observations “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: Mishneh Torah, Hilkhot Milah. 1:18. Twelfth Century Physician and Talmudist Maimonides states in the Mishneh Torah 1

5 Early Observations 1803: John Conrad Otto Provided first accurate account of hemophilia in the modern medical literature 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” 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 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 –German physician –Formulated observations on inheritance of hemophilia –“Nasse’s Law” Transmitted by unaffected females to their sons 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 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 : First recorded case of hemophilia treatment by transfusion written by Samuel Lane in The Lancet : First documentation of abnormal prolongation of coagulation in capillary tube in hemophilics : First documentation of abnormal prolongation of coagulation in capillary tube in hemophilics 2 1. Farr AD. J Royal Soc Med. April 1981;74(4): Wright AE. Br Med J. 1893;2:

8 Early Observations: Other Highlights of the Early 1900s 1911: Publication of “monumental review of pedigrees of families with bleeding disorders” : Publication of “monumental review of pedigrees of families with bleeding disorders” : Hemophilia treatments published; plasma for transfusions introduced : Hemophilia treatments published; plasma for transfusions introduced 1926: Erik vonWillebrand describes bleeding disorder affecting both sexes 1926: Erik vonWillebrand describes bleeding disorder affecting both sexes 1937: IV administration of redissolved plasma precipitate shown to shorten blood clotting time : IV administration of redissolved plasma precipitate shown to shorten blood clotting time : First permanent blood bank established in US 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 –Albumin usefulness spurs research in blood component therapy by “clotters” – : Hemophilia defect in plasma component 2 1. Latham A Jr. Vox Sang. 1986;51(3): Brinkhous KM. Proc Soc Exper Biol and Med. Oct. 1947;66:

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

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

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 FVIII - Hemophilia A FIX - Hemophilia B FIX - Hemophilia B –Thromboplastin generation test for measuring FVIII developed –Coagulation cascade discovered Langdell RD, et al. J Lab Clin Med. 1953;41:

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

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:

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

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

19 1969: First hemophilia camp established 1960s

20 1970s 1970s: Home infusion therapy: a common 1970s: Home infusion therapy: a common treatment practice treatment practice 1971: von Willebrand’s factor identified¹ 1971: von Willebrand’s factor identified¹ 1974: First successful immune tolerance² 1974: First successful immune tolerance² 1975: Federally funded comprehensive hemophilia treatment centers initiated 1975: Federally funded comprehensive hemophilia treatment centers initiated 1977: Mannucci discovers DDAVP increases FVIII and vWF levels : Mannucci discovers DDAVP increases FVIII and vWF levels 3 1.Zimmerman, TS, et al. J Clin Invest.1971;50: Brackmann HH, Lancet 1977; 2 (8044): Mannucci PM. Lancet. 1977;1:

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 Brit J Haematol. 33:81. Aronstam 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): Schramm W, et al. Blut. 1989;59(4):

24 Hepatitis B Virus: HBV Lipid-enveloped DNA virus Lipid-enveloped DNA virus Replicates within liver cells Replicates within liver cells Transmitted by exchange of Transmitted by exchange of bodily fluids bodily fluids 90% recover with immunity; 10% develop chronic HBV of which 20-30% progress to cirrhosis 90% recover with immunity; 10% develop chronic HBV of which 20-30% progress to cirrhosis Virion sensitive to heat and solvent/detergent Virion sensitive to heat and solvent/detergent 1981: Hep B vaccine plasma derived 1981: Hep B vaccine plasma derived 1987: Hep B vaccine recombinant licensed 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 Lipid-enveloped RNA virus Replicates within infected liver cells Replicates within infected liver cells Transmitted by the exchange of bodily fluids Transmitted by the exchange of bodily fluids 85% or more with acute HCV infection progress to chronic hepatitis * 85% or more with acute HCV infection progress to chronic hepatitis * Virion sensitive to heat and solvent/detergent Virion sensitive to heat and solvent/detergent No vaccine available No vaccine available * Seeff LB. Am J Med. Dec. 1999;107[6B]:10S-15S.

27 1980s MMWR July 16, 1982/31(27):

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

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 HemAware. 13: 8.

32 1990s Soucie JM, et al. Blood. 2000;96(2): Characteristics HTC (%) Non-HTC (%) P Severity Mild Mild <.001 Moderate Moderate Severe Severe Inhibitors <.001 Liver disease HIV infection <.001 AIDS

33 1990s Soucie JM, et al. Blood. 2000;96(2): 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 reducing mortality in patients with hemophilia supports the effectiveness of such centers in providing specialized the effectiveness of such centers in providing specialized preventative care.” preventative care.”

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

35 2000s Gene therapy trials begun Gene therapy trials begun Joint Outcome Study published 1 Joint Outcome Study published 1 National Pain Survey National Pain Survey Willetabs and Wilbrintin advertised as treatment for vWD Willetabs and Wilbrintin advertised as treatment for vWD Manco-Johnson, et al. NEJM Vol. 357:(6)

36 Hemophilia A: Evolution of Therapy YearTherapy 1950 Plasma (1-FVIII U/ml) 1966 Cryoprecipitate (5-FVIII U/ml) 1975 Lyophilized concentrates (30-FVIII U/ml) 1983 Heat treatment of lyophilized concentrates for viral attenuation 1985 Introduction of a solvent detergent for viral inactivation 1988 Monoclonal antibody-purified FVIII concentrates with heat or solvent detergent treatment 1992 Recombinant DNA products: 1 st generation 2000s 2 nd & 3 rd generation recombinant products

37 Hemophilia B: Evolution of Therapy YearTherapy 1950 Plasma (1-FIX U/ml) 1975 Lyophilized prothrombin complex concentrates or PCC (30-FIX U/ml) 1985 Heat treatment of lyophilized concentrates for viral attenuation Development of a solvent detergent for viral inactivation 1992 Chromatographic/monoclonal antibody-purified FIX concentrate with ultrafiltration and thiocyanate treatment 1997 Recombinant DNA product

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

39 …the promise of achieving your potential The Future…


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