Sickle Cell News
December 2014
Q&A with Dr. Kwaku Ohene-Frempong about the importance of sickle cell screening and disparities between the US and abroad
To mark the 50th anniversary of newborn screening, we did an interview with Dr. Kwaku Ohene-Frempong about screening for SCD and trait. Dr. Ohene-Frempong is the Director Emeritus of the Comprehensive Sickle Cell Center at Children’s Hospital of Philadelphia, President of the Sickle Cell Foundation of Ghana, and a NICHQ faculty member. Link http://www.nichq.org/resources/Sickle-Cell-Frempong-QA-Dec2012.html
Into Adulthood, Sickle Cell Patients Rely on ER
Patients with sickle cell disease rely more on the emergency room as they move from pediatric to adult health care, according to researchers at Washington University School of Medicine in St. Louis. Link http://www.infozine.com/news/stories/op/storiesView/sid/54200/
Sickle cell patient refuses to let disease define her
Marquita Gaines is a college student living with sickle cell disease. She was diagnosed at birth and first presented symptoms at a young age. She currently receives regular blood cell transfusions administered by registered nurses from the American Red Cross to treat and prevent complications from the disease Link http://www.cnn.com/2012/12/13/us/iyw-blood-donor-gaines/
Ask The Experts
Q: What is the best diet to give a 8 year old with sickle cell disease (Hb SS)
A: Thank you for your question. Diet in sickle cell disease is the subject of active research, but the studies are far from complete or conclusive. Researchers have not tried to demonstrate a what a "Sickle Cell Diet" should be. Here are some bits of information:
(1) Sickle hemoglobin and breakdown of sickle red blood cells place the body under high oxidant stress, so that taking anti-oxidants supplements seem like a good idea.
(2) Blood chemistries in people with sickle cell are slightly abnormal in multiple different ways. Supplementing for the deficiencies seem like a good idea.
(3) Studies have been designed to examine single-ingredient supplements such as vitamins ( folic acid, tetrahydrobiopterin, vitamin D, vitamin C, vitamin E), minerals ( zinc, magnesium), amino acids ( glutamine, arginine, citrulline), anti-oxidants, nitrite, nitrate, or fish oil. I don't remember seeing studies designed as head-to-head comparions to show what ingredient(s) are the most important.
(4) We do think that extra iron supplements are not necessary for sickle cell disease, unless somebody has lost a lot of blood or has demonstrated unusual barriers to iron absorption.
(5) Probably the only advice we can give is to eat a generally balanced diet as recommended for most American families in the general population: plenty of fruits & vegetables, balanced protein intake (meats, dairy, nuts & beans), drink plenty of fluids, eat dietary fiber, and avoid excessive amounts of sugary or fried snacks. This nutritional advice is based on dietary research on reduce the risks of diabetes, heart attacks, stroke, and some cancers.
Sincerely,
Lewis Hsu, MD
Pediatric Hematologist
Q&A with Dr. Kwaku Ohene-Frempong about the importance of sickle cell screening and disparities between the US and abroad
To mark the 50th anniversary of newborn screening, we did an interview with Dr. Kwaku Ohene-Frempong about screening for SCD and trait. Dr. Ohene-Frempong is the Director Emeritus of the Comprehensive Sickle Cell Center at Children’s Hospital of Philadelphia, President of the Sickle Cell Foundation of Ghana, and a NICHQ faculty member. Link http://www.nichq.org/resources/Sickle-Cell-Frempong-QA-Dec2012.html
Into Adulthood, Sickle Cell Patients Rely on ER
Patients with sickle cell disease rely more on the emergency room as they move from pediatric to adult health care, according to researchers at Washington University School of Medicine in St. Louis. Link http://www.infozine.com/news/stories/op/storiesView/sid/54200/
Sickle cell patient refuses to let disease define her
Marquita Gaines is a college student living with sickle cell disease. She was diagnosed at birth and first presented symptoms at a young age. She currently receives regular blood cell transfusions administered by registered nurses from the American Red Cross to treat and prevent complications from the disease Link http://www.cnn.com/2012/12/13/us/iyw-blood-donor-gaines/
Ask The Experts
Q: What is the best diet to give a 8 year old with sickle cell disease (Hb SS)
A: Thank you for your question. Diet in sickle cell disease is the subject of active research, but the studies are far from complete or conclusive. Researchers have not tried to demonstrate a what a "Sickle Cell Diet" should be. Here are some bits of information:
(1) Sickle hemoglobin and breakdown of sickle red blood cells place the body under high oxidant stress, so that taking anti-oxidants supplements seem like a good idea.
(2) Blood chemistries in people with sickle cell are slightly abnormal in multiple different ways. Supplementing for the deficiencies seem like a good idea.
(3) Studies have been designed to examine single-ingredient supplements such as vitamins ( folic acid, tetrahydrobiopterin, vitamin D, vitamin C, vitamin E), minerals ( zinc, magnesium), amino acids ( glutamine, arginine, citrulline), anti-oxidants, nitrite, nitrate, or fish oil. I don't remember seeing studies designed as head-to-head comparions to show what ingredient(s) are the most important.
(4) We do think that extra iron supplements are not necessary for sickle cell disease, unless somebody has lost a lot of blood or has demonstrated unusual barriers to iron absorption.
(5) Probably the only advice we can give is to eat a generally balanced diet as recommended for most American families in the general population: plenty of fruits & vegetables, balanced protein intake (meats, dairy, nuts & beans), drink plenty of fluids, eat dietary fiber, and avoid excessive amounts of sugary or fried snacks. This nutritional advice is based on dietary research on reduce the risks of diabetes, heart attacks, stroke, and some cancers.
Sincerely,
Lewis Hsu, MD
Pediatric Hematologist