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Erik Adolf von Willebrand: 1870-1949
This modest man discovered the most common inherited bleeding disorder while studying the genetic traits of a family in the Åland Islands in Finland.
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Erik Adolf von Willebrand was a Finnish internist whose keen interest in blood and its coagulation properties led him to uncover a new form of hemophilia.
Von Willebrand was born Feb. 1, 1870, in Vaasa, a seaport city located in western Finland.
He studied medicine at the University of Helsinki. During his summer breaks, he worked as a physician at a spa in Maarianhamina, the capital of the Åland islands, where he first learned about Ålandic hemorrhagic disease.
Von Willebrand received his medical degree from the University of Helsinki in 1896. He was awarded a doctorate from the same institution for his doctoral thesis, "Blood changes after venesection," which detailed his investigation on how blood changes after a significant blood loss. This work sparked an intense interest in blood and its coagulation properties.
Early years as a physician
From 1897 to 1900, von Willebrand worked as an intern and assistant physician at the Deaconess Institute of Helsinki. Between 1901 and 1903, he offered lectures in anatomy and was a member of the physiology department at the University of Helsinki. In 1908, von Willebrand became a lecturer in internal medicine. During this time, he focused on blood changes during muscular exercise, metabolism and obesity, as well as carbon dioxide and water exchange through the human skin.
He worked at the university until his retirement in 1935. He was also the physician-in-chief of the Deaconess Institute from 1922 to 1931.
Von Willebrand wrote many articles about obesity, gout and diabetes mellitus. He detailed a technique for evaluating ketone bodies in urine in 1912. He wrote about managing diabetes with diet, and he was a pioneer in insulin use. In 1922, von Willebrand wrote about using insulin to treat diabetic coma. He was the author of several hematology articles as well.
Curiosity led to a discovery
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Von Willebrand remains most famous, however, for his description of a familial bleeding disorder he encountered among the inhabitants of the Åland Islands. In 1925, he examined a 5-year-old girl with a history of bleeding who had been brought to Helsinki for treatment.
The little girl was the ninth of 12 children. Four of her siblings bled to death at an early age. Both of her parents came from families with bleeding disorders.
Von Willebrand was curious to know more, so he traveled to the Åland Islands to study the disease in depth. He mapped the family pedigree and found that 23 of the 66 family members had bleeding problems. Von Willebrand concluded that this was a previously unknown type of hemophilia. Initially, he called the disease "hereditary pseudo-hemophilia" because of the prolonged bleeding time. As he studied the disease more, he came to believe that platelets were involved, so he renamed it "constitution-al thrombopathy."
He noted his findings about the family in a 1926 report.
Dedicated to nature, education
Von Willebrand had interests outside of medicine, as well. He had a keen interest in nature and birds in particular. He established an aviary reserve on an island near his villa so that the native birds would be protected during their nesting season. His nephew, Otto Lindberg, a physician specializing in geriatrics, has maintained that reserve in his honor.
He was also an avid gardener. Von Willebrand worked often in the lush garden he planted around his villa, and he encouraged his grandchildren to work there for two hours every day. He generously shared his knowledge of flora and animals that surrounded the villa with his grandchildren, who summered there each year.
Von Willebrand was dedicated to his own continuing medical education. To remain up to date, he read scientific journals every morning for several hours. After World War I, though, the journals were published in English, which he could not read. Von Willebrand spent many hours in his study teaching himself English.
He has been described as a modest man who did not boast of his achievements.
Von Willebrand died Dec. 12, 1949, in Perna, Finland.
In 1994, the Åland Islands issued a postal stamp to honor von Willebrand's work.
Eponym
Von Willebrand's disease is the most common inherited bleeding disorder. It is characterized by a deficiency in the clotting protein called von Willebrand's Factor; the most common symptom is prolonged bleeding time. The clotting protein Factor VIII may also be involved. There are three major types.
Type 1 is the mildest and most common form of the disease. In patients with this type, von Willebrand's Factor is low; Factor VIII levels may also be low.
Type 2 is a mild-to-moderate form in which the von Willebrand's Factor is defective. Type 2 is subdivided into types 2A, 2B and 2C.
Type 3 is the most severe form. In patients with this type, von Willebrand's Factor is absent, and Factor VIII is low.
The disease is generally mild and often does not require treatment. If treatment is necessary, options include medications to increase the von Willebrand's Factor level, medications to prevent clot breakdown and injections of clotting factor concentrates. – by Colleen Owens