• SOURCE: Journal of the American Medical Association

Slated Version: Two Therapies To Treat Sickle Cell Disease Are Underutilized

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349796

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INTRO: Sickle cell disease is a life-threatening genetic disorder affecting almost a hundred thousand Americans. The disease is associated with severe pain and several chronic complications. During the last five years, an expert panel reviewed existing medical studies and trials on sickle cell disease. The report highlights two therapies that are effective but underutilized when it comes to treatment. More from Catherine Dolf in this week’s JAMA Report. B-ROLL Isaiah laying in chair, pic of Isaiah in Taekwando outfit ISAIAH BELL IS 12. HE LOVES FOOTBALL AND TAEKWANDO. SOT/FULL Isaiah Bell - Sickle Cell Patient Super@:04 Runs:04 “I get to kick people in taekwondo…that’s just awesome.” B-ROLL Pic of Isaac with diploma, with friends ISAAC OKAH IS 18. HE’S HEADED TO COLLEGE AND WANTS TO BE AN ACTOR. SOT/FULL Isaac Okah, Jr. - Sickle Cell Patient Super@:13 Runs:04 “There are so many things about acting that just intrigues me.” B-ROLL Split screen of Isaiah sitting with mom and Isacc walking with his mom JUST TWO OF THE THOUSANDS LIVING WITH SICKLE CELL DISEASE IN THE UNITED STATES. SOT/FULL George R. Buchanan, M.D., - UT Southwestern Medical Center Super@ :20 Runs:08 “Pain is the major manifestation of people with sickle cell disease. The pain is unpredictable, it’s recurrent and it can be extremely severe.” B-ROLL Graphic: Sickle Cells, pain points in the body THE PAIN IS CAUSED BY SICKLE SHAPED RED BLOODS CELLS THAT BLOCK THE SMALL BLOOD VESSELS IN THE BODY. THIS CAUSES AN ARRAY OF SERIOUS HEALTH PROBLEMS. SOT/FULL George R. Buchanan, M.D., - UT Southwestern Medical Center Super@ :35 Runs:14 (Video covering middle of bite: Dr. Buchanan talking with Isaiah and his mom) “Many doctors are unfamiliar with how to treat these events. Having those physicians knowledgeable about sickle cell disease and familiar with the guidelines that are being recommended here will allow them to give better care to these patients.” B-ROLL Dr. Buchanan walking, sitting at microscope GXF FULL JAMA COVER DR. GEORGE BUCHANAN FROM THE UNIVERSITY OF TEXAS SOUTHWESTERN MEDICAL CENTER AND A PANEL OF EXPERTS SPENT 5 YEARS REVIEWING MORE THAN 30 YEARS OF MEDICAL STUDIES AND TRIALS ON SICKLE CELL DISEASE. THIS SPECIAL COMMUNICATION APPEARS IN JAMA, JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION. SOT/FULL George R. Buchanan, M.D., - UT Southwestern Medical Center Super@ 1:04 Runs:13 (Video covering 2nd half of bite: cu of Dr. Buchanan’s looking in microscope “There are improved treatments. Hydroxyurea is greatly effective in reducing pain and other complications and prolonging patients’ lives. Unfortunately most people aren’t taking that medicine yet and it’s a big problem.” B-ROLL Isaac and mom talking, blood being tested, pic of Isaac’s eyes swollen shut ISAAC IS TAKING THIS F-D-A APPROVED MEDICATION WHICH DECREASES THE RIGIDITY AND INFLEXIBILITY OF HIS RED BLOOD CELLS. IT IS ALSO PREVENTING EPISODES LIKE THIS. SOT/FULL Voice of: Isaac Okah, Jr. - Sickle Cell Patient Super@ 1:25 Runs:03 (Video covering bite: pic of eyes swollen shut) “My eyes got swollen shut. I couldn’t see at all.” SOT/FULL Gift Okah - Isaac’s Mother Super@ 1:34 Runs:08 (Video covering 1st part of bite: Isaac and mom walking) “For almost 5 years now we have not had any pain crisis because he stayed on that medication.” SOT/FULL George R. Buchanan, M.D., - UT Southwestern Medical Center Super@1:41 Runs:10 (Video covering 1st part of bite: blood transfusion bag) “Blood transfusions are an extremely important treatment for people with sickle cell disease either given acutely as a single time or on a monthly basis, long-term.” SOT/FULL Natasha Jones - Isaiah’s Mother Super@1:46 Runs:06 “The blood transfusions just kind of helped him stay on top of his game.” Isaiah Bell - Sickle Cell Patient Super@ 1:52 Runs:03 “It’s not too big of a problem for me.” B-ROLL Mircroscope behind graphics: Panel Recommendations Treating Severe Pain with Intravenous Opiods Using Penicillin To Treat Children Up to Age Five Isiah and Dr. Buchanan, Isaac talking with mom OTHER RECOMMENDATIONS INCLUDE, TREATING SEVERE PAIN WITH INTRAVENOUS OPIODS AND USING DAILY PENICILLIN TO PREVENT INFECTION IN CHILDREN UP TO AGE FIVE WHO HAVE THE DISEASE. ISAIAH, ISAAC AND THEIR FAMILIES CONTINUE TO PARTICIPATE IN RESEARCH AND REMAIN HOPEFUL. SOT/FULL Isaac Okah, Jr. - Sickle Cell Patient Super@2:08 Runs:07 “Who knows maybe they’ll come across a breakthrough and find a cure for sickle cell, I mean you never know you just gotta keep hoping for it.” B-ROLL Isaac on camera CATHERINE DOLF, THE JAMA REPORT. TAG: THE EXPERT PANEL ALSO SAYS THERE ARE GAPS IN THE RESEARCH AND ALSO IN THE NUMBER OF BLOOD SPECIALISTS AND SICKLE CELL EXPERTS THAT CAN PROVIDE SPECIALIZED CARE TO PATIENTS WITH SICKLE CELL DISEASE. Please see the complete study for additional information, including other authors, author contributions and affiliations, financial disclosures, funding and support, etc. TO CONTACT: Dr. George Buchanan call: Lori Soderbergh at (214) 648-2876 doi.10.1001/jama.2014.jsc.140004 ADDITIONAL SOUNDBITES: George R. Buchanan, M.D., - UT Southwestern Medical Center QUOTE 1 Runs:13 “Every person with sickle cell disease needs to have access to a provider, to a health care professional who can see them on an ongoing basis, understand them, get to know them and be available to offer care when needed.” QUOTE 2 Runs:10 “Someday I’d like to think that people with sickle cell disease can be offered curative treatment not just to control their condition truly but to cure it. I’m very hopeful about the future.”
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