Ms. Yuen, a genetic counselor, was a member of one of the first concentrated efforts to study large families with VHL and find the VHL gene. From those beginnings in 1978 the VHL gesne was first mapped in 1993 and the gene for the Hawaii family was identified in 1996. She is pictured here with her husband Ted in Indonesia.

I last talked to Juliet Hsia (pronounced "Shah") at her Waikiki apartment in mid-May 2000. She had just begun her courageous five-month battle with pancreatic cancer. She was as vivacious, warm, and outgoing as ever when we spoke that day. As usual, it was wonderful to talk to her. However I was filled with grief and apprehension knowing her advanced cancer diagnosis. Juliet died peacefully at home in the evening on 16 September 2000, surrounded by her loving family and friends. Her death ended our friendship of almost 23 years, but her inspiration, influence, and memory will be with me for the rest of my days.

J M. Lamiell, Honolulu, 1996

I first met Juliet and Ted Hsia in January 1978. I was working at the Army hospital in Honolulu, Hawaii and had recently encountered my first von Hippel-Lindau (VHL) patients. I wanted to continue to see these VHL patients, but it could only be done at an Army hospital in the context of research since many in the extended Hawaiian VHL family were not eligible for military health care. As an internal medicine resident, I knew very little about genetics and VHL. Therefore my supervisor referred me to Dr. Ted Hsia, who was a University of Hawaii professor of genetics, to investigate the possibility of a collaborative VHL research study. Ted ran the clinical genetic service at Kapiolani Medical Center in Honolulu with his wife Juliet, who was a genetic counselor.

I instantly liked Juliet and Ted, and I have rarely experienced such immediate and profound friendship. They listened intently as I described my VHL encounters. Ted was exceptionally knowledgeable, and he had a seemingly endless supply of VHL research ideas. Juliet was warm, receptive, and intense. It was obvious that Juliet was primarily concerned with the people who might be VHL research subjects. At times, she was outspoken in her patient advocacy. Juliet did everything she could to support Ted, in their marriage as well as their professional lives. Juliet and Ted made a perfect team. They quickly and willingly became my mentors, and I am profoundly grateful for their guidance. Meeting them certainly changed my life, and it is fair to say, also changed the lives of many affected by VHL.

For more than two years, I met frequently with Juliet and Ted at Kapiolani Medical Center to discuss the Hawaiian VHL family members I had seen, which totaled about 200 by the time the study was finished. I always looked forward to seeing Juliet and Ted. Juliet and Ted began to see VHL family members as well, and we had several meetings with as many family members as possible. Juliet always provided what Ted has called "the human touch" to our technical clinical endeavors, and she constructed the large pedigree chart for this family. She was an essential and valuable team member.

I left Hawaii in July 1980 and did not see Juliet until 1996. We did talk frequently since our VHL research continued, and Ted and I took some time to write a paper about the clinical features of VHL that was not published until 1989. Juliet fully supported Ted’s early involvement with the VHL Family Alliance, created in 1992 by Joyce Graff, Peggy Graham, and Susan Warnick. Juliet and Ted attended the VHL Meeting organized by Dr. Hartmut Neumann in Freiberg, Germany in 1994. Juliet and Ted were primarily responsible for the 1996 Honolulu VHL Meeting.

The Hawaiian VHL family was the largest ever reported. The full support and participation of this family led to the first linkage study, which was unsuccessful, because of the technology limitations in 1980.

After Dr. James Gusella at Harvard University located the Huntington disease gene on chromosome 4 in 1984, Ted enticed him to look for the gene change causing VHL, which we felt was a single gene based on our studies. Dr. Gusella gave the project to his post-doctoral fellow, Dr. Bernt Seizinger, who worked indefatigably on this project. Dr. Seizinger, worked closely with Juliet, Ted, and me, accepting blood from the Hawaiian VHL family and convincing many scientists to collaborate by donating samples from several other families, especially the large one in Newfoundland.

After four years, the VHL gene was located on the short arm of chromosome 3. Perhaps the reports on the Hawaiian family spurred the work by the NIH, the Cambridge group in England under Dr. Maher, and many others, to race to identify the VHL gene and study its properties, as well as the many other studies and advances that were reported in the late 1980’s and 1990’s. None of this may have happened without the full cooperation of the Hawaiian VHL family, due in no small part to the psychosocial rapport Juliet established with this family.

Juliet Yuen was born and raised in Kuala Lumpur, Malaysia. Ted Hsia, who was born in Shanghai, attended medical school at Oxford University, England, followed by resident training in London, and post-doctoral training in genetics at Yale University. Juliet and Ted met in London, and they married in 1956.

Ted, who was a medical geneticist at the Yale University School of Medicine in Connecticut, moved to Hawaii with Juliet and their children in 1977. Juliet stepped into a void to become Ted’s office administrator and genetic counselor, ably based on her past expertise and on-the-job-training. She worked with genetic counselors nationally and internationally, to assist people with birth defects, genetic disorders, or who have high risk of inherited conditions like VHL. Genetic counselors explain, in simple terms, the complicated diagnoses made by medical geneticists, as well as treatment options.

Juliet Hsia, known professionally by her maiden name Yuen, played a major role in establishing genetic counseling in Hawaii after 1977. She worked closely with Ted at Kapiolani Medical Center and the University of Hawaii until her retirement in 1996. For many years, Juliet was the only genetic counselor in the state of Hawaii. Juliet was instrumental in helping to provide testing for inherited anemias common in Hawaii’s Chinese, Filipino and Laotian populations. She was heavily involved with counseling members of the extended Hawaiian VHL family. Juliet was a past officer of the National Society of Genetic Counselors, she published papers in national and international journals (some cited below), and she lectured in several countries. Juliet was well known for her strong patient advocacy. Juliet was an excellent teacher. One of her former University of Hawaii students, Janet Brumblay, noted that, "She was one of the best teachers I ever had. She could take something very scientific and make it easy for anyone to understand. She also just had a way of making everyone part of her own family."

Juliet had a deep Christian faith. She was active at Calvary-by-the-Sea Lutheran Church in Hawaii, serving as a past president of the Council of Deacons. She championed the role of women and minorities as a national and international delegate to the Evangelical Lutheran Church of America.

Juliet and Ted raised five sons: Martin, Calvin, Franklin, Duncan, and Gordon; and there are seven grandchildren.

The Juliet and Ted Hsia Foundation was recently formed with the intent of supporting many worthy causes for generations to come, just as Juliet and Ted did during their many years together. Those interested can log into the website at http://www.hsiafoundation.org, make email contact at Info@HsiaFoundation.org, or call (808) 943-1058 for more information. The Website contains many pictures of Juliet, her family, and friends.

Juliet was an inspiration for all that knew her. We will miss her.

References

Yuen J, Jewell R, Lamiell MJ, Hsia YE: Impact of a late-onset autosomal dominant precancerous disease on the knowledge and attitudes of a large kindred. Birth Defects Orig Artic Ser. 1984;20(6):135-46.Go RC, Lamiell JM, Hsia YE, Yuen JW, Paik Y: Segregation and linkage analyses of von Hippel Lindau disease among 220 descendants from one kindred. Am J Hum Genet. 1984;36(1):131-42.Yuen J: Asian Americans. Birth Defects Orig Artic Ser. 1987;23(6):164-70.Hsia YE, Yuen J, Hunt JA, Rattamanasay P, Hall J, Takaesu N, Titus EA, Fujita J, Ford CA: The different types of alpha-thalassemia: practical and genetic aspects. Hemoglobin. 1988;12(5-6):465-84.Yuen J, Hsia YE, Hall J: Thalassemia heterozygotes in Hawaii: ethnic attitudes toward screening and prenatal diagnosis. Hemoglobin. 1988;12(5-6):801-16.Seizinger BR, Rouleau GA, Ozelius LJ, Lane AH, Farmer GE, Lamiell JM, Haines J, Yuen JW, Collins D, Majoor-Krakauer D, et al: Von Hippel-Lindau disease maps to the region of chromosome 3 associated with renal cell carcinoma. Nature. 1988;332(6161):268-9.Ireland JH, Luo HY, Chui DH, Chu B, Yuen J, Hsia YE: Detection of the (—SEA) double alpha-globin gene deletion by a simple immunologic assay for embryonic zeta-globin chains. Am J Hematol. 1993;44(1):22-8.Hsia YE, Miyakawa F, Baltazar J, Ching NS, Yuen J, Westwood B, Beutler E: Frequency of glucose-6-phosphate dehydrogenase (G6PD) mutations in Chinese, Filipinos, and Laotians from Hawaii. Hum Genet. 1993;92(5):470-6.

Johnson JL, Yuen J, Nishimoto P, Johnson RC, Johnson RL: Family-centered care: thriving in Hawaii under Part H. Clin Commun Disord. 1994;4(4):254-65.

As printed in the VHL Family Forum 8:4, December 2000. For permission to reprint, please contact VHL Family Alliance, editor@vhl.org. Further information is available from the VHL Family Alliance, info@vhl.org. Further information is available from the VHL Family Alliance, info@vhl.org.

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