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Invitation to the Table
CHRCO Hosts Conference on Pulmonary Hypertension in Sickle Cell Disease and Thalassemia

Nationally renowned investigators at Children's Hospital Research Center Oakland (CHRCO) spearheaded a major conference at the end of May to promote greater interdisciplinary research efforts in tackling the problem of pulmonary hypertension (PHT) in patients with sickle cell disease and thalassemia.

"The life expectancy of patients with these hemoglobinopathies is significantly decreased," says Frans Kuypers, PhD, a senior CHORI scientist and member of CHORI's Center for Sickle Cell Disease and Thalassemia who helped organize the conference.

A vascular disorder in which blood flow form the heart to the lungs becomes restricted, PHT has many different causes, and affects other populations than those with hemoglobinopathies. The result of PHT in hemoglobinapathy patients, however, is swift and dramatic, presenting a significant hurdle in the treatment and care of these patients.

"If the blood vessels between the heart and the lungs become narrower and narrower, the heart has to work over time, and the blood pressure goes way up," Dr. Kuypers explains. "At the first signs of PHT in sickle and thalassemia patients, there is most often a very rapid demise, resulting in death, within a year or even months."

Because PHT is associated with both the heart and lungs, both cardiologists and pulmonary specialists are involved in the care, treatment and study of PHT patients. When PHT patients also have sickle cell disease or thalassemia, hematology specialists are also on board, with all three of these specialties potentially approaching the problem from different angles and with different priorities.

"With all these specialists involved, it's critically important to get all of these folks around the same table at the same time to see if we can't find some overall approach," says Dr. Kuypers. "That's what this conference was all about."

The one-day conference attended by fellow professionals, researchers and caregivers featured clinical researchers and hematology specialists from CHRCO, including Elliot Vichinsky, MD, the chief of the division of Hematology/Oncology; Keith Quirolo, the director of pediatric sickle cell; Ward Hagar, the director of adult sickle cell; Claudia Morris, MD, in the department of emergency medicine; and Titi Singer, MD, in the department of hematology, as well as the Children's Hospital Los Angeles' pediatric cardiologist, John Wood, MD, and Boston University's pulmonary specialist and medical intensivist, Elizabeth Kling, MD. This diverse group provided a broad clinical inventory of the different approaches to understanding, researching and treating PHT.

Over the course of the conference, all these different players illuminated their perspectives and the wealth of their research, providing fertile ground for future collaborative efforts across the three different disciplines.

"Close collaboration between the three specialties is essential if you want to diagnose PHT as early as possible and understand its biomolecular mechanisms," says Dr. Kuypers. "This conference set the stage for pushing for better nationally-coordinated, NIH-funded studies to address this major problem in sickle cell and thalassemia patients."

The conference was so successful in bridging discussion across these groups in fact that the organizers found they could cancel the Q & A panel session they had planned.

"There were so many questions and answers during the talks themselves that we didn't need a special session," Dr. Kuypers says. "The audience and speakers engaged in such active dialogue that we didn't need to facilitate it ourselves."

As a result, participants left with a greater understanding of the complexity of the issue, of the need for very well-designed studies to answer the many questions surrounding PHT, and of the importance of bringing the issue to the forefront of research priorities.

"It was an excellent conference," acknowledges Dr. Kuypers. "Our goals were met."

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Monday, May 16, 2011 11:33 PM

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