Nancy Olivieri

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Nancy Fern Olivieri (born 1954) is a prominent Toronto haematologist and researcher with an interest in the treatment of haemoglobinopathies. She is best known for a protracted struggle with the Hospital for Sick Children and the pharmaceutical company Apotex about the drug deferiprone. [1]

Contents

Early life

Olivieri was born to the Hamilton, Ontario, Doctor Fernando Olivieri and Victoria Olivieri. [2] Her paternal grandfather had immigrated to Hamilton from Italy in 1909. [2] Olivieri received a Bachelor of Science from the University of Toronto and an MD from McMaster University in 1978. [3] She has also pursued training in Internal Medicine and Hematology at McMaster, University of Toronto, and Harvard University. [3]

Deferiprone controversy

Starting in 1989, Olivieri was part of a group evaluating the use of a drug, deferiprone, in treating persons with the blood disorder thalassaemia. [1] Starting from 1985, this work included a clinical trial partly funded by Apotex. During the course of the trial, Olivieri became concerned about evidence that pointed to the drug being inefficacious for some patients. Olivieri informed both the research ethics board that was monitoring the study and Apotex, the drug maker. The research ethics board instructed Olivieri to inform participants about her concerns. Apotex responded by noting that Olivieri had signed a confidentiality agreement as part of the drug trial and that informing participants about her concerns, the validity of which Apotex disputed, would violate that confidentiality agreement. In 1996, Apotex threatened to vigorously pursue legal remedies against her if she disclosed her conclusions to patients. [1] Olivieri disclosed her concerns to her patients and Apotex ended the portion of the study in which she was participating. In 1998, the New England Journal of Medicine published a paper by Olivieri and seven other authors, with further study results suggesting that deferiprone led to progressive hepatic fibrosis. [4] [5]

Olivieri's scientific findings, which sparked the controversy, have been challenged on the basis of data from clinical trials conducted by Apotex. [6] [7] [8]

Deferiprone is approved for use in over 50 countries, but not in Canada. [9] It was approved in the US in 2011 under the FDA's accelerated approval program. [10]

An investigation commissioned by the Canadian Association of University Teachers (CAUT) revealed that one of Olivieri's critics, Gideon Koren, had anonymously sent disparaging letters about Olivieri to the media and colleagues. Koren initially denied responsibility, but substantial DNA evidence tied him to the letters, and he was reprimanded. [11]

Olivieri has advocated greater academic freedom and called for less control of research by pharmaceutical companies. [12] This situation was publicised extensively and was investigated by the Canadian Association of University Teachers. [11]

Awards and honours

Olivieri was awarded the 2009 AAAS Award for Scientific Freedom and Responsibility for her "indefatigable determination that patient safety and research integrity come before institutional and commercial interests and for her courage in defending these principles in the face of severe consequences." [13]

Olivieri is the 2023 recipient of the John Maddox Prize in recognition of her “determination to act with integrity…in the face of extreme pressure from the company producing it, ultimately at great personal cost” in the Deferiprone controversy. [14] The Maddox Prize is awarded by the UK-based Sense about Science charity in partnership with Nature (journal).

See also

Related Research Articles

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<span class="mw-page-title-main">Thalassemia</span> Family of inherited blood disorders

Thalassemias are inherited blood disorders that result in abnormal hemoglobin. Symptoms depend on the type of thalassemia and can vary from none to severe. Often there is mild to severe anemia as thalassemia can affect the production of red blood cells and also affect how long the red blood cells live. Symptoms of anemia include feeling tired and having pale skin. Other symptoms of thalassemia include bone problems, an enlarged spleen, yellowish skin, pulmonary hypertension, and dark urine. Slow growth may occur in children. Symptoms and presentations of thalassemia can change over time.

<span class="mw-page-title-main">Alpha-thalassemia</span> Thalassemia involving the genes HBA1and HBA2 hemoglobin genes

Alpha-thalassemia is a form of thalassemia involving the genes HBA1 and HBA2. Thalassemias are a group of inherited blood conditions which result in the impaired production of hemoglobin, the molecule that carries oxygen in the blood. Normal hemoglobin consists of two alpha chains and two beta chains; in alpha-thalassemia, there is a quantitative decrease in the amount of alpha chains, resulting in fewer normal hemoglobin molecules. Furthermore, alpha-thalassemia leads to the production of unstable beta globin molecules which cause increased red blood cell destruction. The degree of impairment is based on which clinical phenotype is present.

<span class="mw-page-title-main">Beta thalassemia</span> Thalassemia characterized by the reduced or absent synthesis of the beta globin chains of hemoglobin

Beta thalassemias are a group of inherited blood disorders. They are forms of thalassemia caused by reduced or absent synthesis of the beta chains of hemoglobin that result in variable outcomes ranging from severe anemia to clinically asymptomatic individuals. Global annual incidence is estimated at one in 100,000. Beta thalassemias occur due to malfunctions in the hemoglobin subunit beta or HBB. The severity of the disease depends on the nature of the mutation.

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Apotex Inc. is a Canadian pharmaceutical corporation. Founded in 1974 by Barry Sherman, the company is the largest producer of generic drugs in Canada, with annual sales exceeding CA$2.5 billion. By 2023, Apotex employed close to 8,000 people as Canada's largest drug manufacturer, with over 300 products selling in over 115 countries. Apotex manufactures and distributes generic medications for a range of diseases and health conditions that include cancer, diabetes, high cholesterol, glaucoma, infections and blood pressure.

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<span class="mw-page-title-main">Deferiprone</span> Iron chelator

Deferiprone, sold under the brand name Ferriprox among others, is a medication that chelates iron and is used to treat iron overload in thalassaemia major. It was first approved and indicated for use in treating thalassaemia major in 1994 and had been licensed for use in the European Union for many years while awaiting approval in Canada and in the United States. On October 14, 2011, it was approved for use in the US under the FDA's accelerated approval program.

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Treatment of the inherited blood disorder thalassemia depends upon the level of severity. For mild forms of the condition, advice and counseling are often all that are necessary. For more severe forms, treatment may consist in blood transfusion; chelation therapy to reverse iron overload, using drugs such as deferoxamine, deferiprone, or deferasirox; medication with the antioxidant indicaxanthin to prevent the breakdown of hemoglobin; or a bone marrow transplant using material from a compatible donor, or from the patient's mother. Removal of the spleen (splenectomy) could theoretically help to reduce the need for blood transfusions in people with thalassaemia major or intermedia but there is currently no reliable evidence from clinical trials about its effects. Population screening has had some success as a preventive measure.

Cooley’s Anemia Foundation (CAF) is an American 501(c)(3) nonprofit organization that funds medical research and education to benefit individuals living with the genetic blood disorder thalassemia. The major form of this disorder is also known as Cooley's anemia.

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<span class="mw-page-title-main">Transfusion-dependent anemia</span>

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References

  1. 1 2 3 Viens A, Savulescu J (2004). "Introduction to The Olivieri symposium". J Med Ethics. 30 (1): 1–7. doi:10.1136/jme.2003.006577. PMC   1757126 . PMID   14872065.
  2. 1 2 "'Uncle Red': Barton Street's kid doctor". thespec.com. August 21, 2011.
  3. 1 2 "Nancy Olivieri - Institute of Medical Science - University of Toronto". ims.utoronto.ca.
  4. ↑ Olivieri NF, Brittenham GM, McLaren CE, et al. (1998). "Long-term safety and effectiveness of iron chelation therapy with oral deferiprone in patients with thalassemia major". N. Engl. J. Med. 339 (7): 417–428. doi: 10.1056/NEJM199808133390701 . PMID   9700174. Full Text.
  5. ↑ Hadskis, Michael (2007). "The Regulation of Human Biomedical research in Canada". In Downie, Jocelyn (ed.). Canadian Health Law and Policy (textbook). et al. (Third ed.). LexisNexis. p. 304.
  6. ↑ M.A. Tanner, MRCP; R. Galanello, MD; C. Dessi, MD; G.C. Smith, MSc; M.A. Westwood, MD; A. Agus, MD; M. Roughton, MSc; R. Assomull, MRCP; S.V. Nair, MRCP; J.M. Walker, MD; D.J. Pennell, MD (2007). "A Randomized, Placebo-Controlled, Double-Blind Trial of the Effect of Combined Therapy With Deferoxamine and Deferiprone on Myocardial Iron in Thalassemia Major Using Cardiovascular Magnetic Resonance". Circulation. 115 (14): 1876–1884. doi: 10.1161/CIRCULATIONAHA.106.648790 . PMID   17372174. Full Text.
  7. ↑ Brittenham G, Nathan D, Olivieri N, Porter J, Pippard M, Vichinsky E, Weatherall D (2003). "Deferiprone and hepatic fibrosis". Blood. 101 (12): 5089–90, author reply 5090–1. doi: 10.1182/blood-2002-10-3173 . PMID   12788794. Full Text.
  8. ↑ Wanless I, Sweeney G, Dhillon A, Guido M, Piga A, Galanello R, Gamberini M, Schwartz E, Cohen A (2002). "Lack of progressive hepatic fibrosis during long-term therapy with deferiprone in subjects with transfusion-dependent beta-thalassemia". Blood. 100 (5): 1566–9. doi: 10.1182/blood-2002-01-0306 . PMID   12176871. Full Text.
  9. ↑ Savulescu J (2004). "Thalassaemia major: the murky story of deferiprone". BMJ. 328 (7436): 358–9. doi:10.1136/bmj.328.7436.358. PMC   341373 . PMID   14962851. Full Text.
  10. ↑ FDA NEWS RELEASE: FDA Approves Ferripox (deferiprone) to Treat Patients with Excess Iron in the Body, Oct. 14, 2011 https://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm275814.htm
  11. 1 2 Jon Thompson; Patricia Baird; Jocelyn Downie, Report of the Committee of Inquiry on the Case Involving Dr. Nancy Olivieri, the Hospital for Sick Children, the University of Toronto, and Apotex Inc. (PDF), www.caut.ca, retrieved 8 December 2015
  12. ↑ Olivieri N (2003). "Patients' health or company profits? The commercialisation of academic research". Sci Eng Ethics. 9 (1): 29–41. doi:10.1007/s11948-003-0017-x. PMID   12645227. S2CID   32796175.
  13. ↑ http://www.aaas.org/aboutaaas/awards/freedom/freedom2009.shtml
  14. ↑ Cressey, Daniel. "Scientists honoured for facing down lawsuits to reveal findings". Research Professional News. Retrieved 25 October 2023.

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