Can be Cured of Sickle Cell Disease and Thalassemia After Sibling Cord Blood Transplantation: Results from ViaCell and Children's Hospital & Research Center Oakland
ViaCell, Inc. (Nasdaq: VIAC) and Children's Hospital & Research Center Oakland reported results today that children with Sickle Cell Disease and Thalassemia can be cured with umbilical cord blood from a compatible sibling. At the Sickle Cell Disease Association of America and National Institutes of Health (NIH) 35th Annual Convention, Dr. Mark Walters, Director of the Blood and Marrow transplant program at Children's Hospital & Research Center Oakland presented research data demonstrating that cord blood from a relative can be an effective source of stem cells for transplantation in children affected with Sickle Cell Disease and Thalassemia and may have advantages over bone marrow transplantation.
"Patients with Sickle Cell and Thalassemia often lead debilitating lives," said Dr. Walters. "Through continued research and transplant success, sibling umbilical cord blood has proven to be effective in curing children of these blood disorders. I expect the use of umbilical cord blood will continue to increase and as we gain more experience using cord blood stem cells in transplant medicine, I believe it could outpace the use of bone marrow in transplant medicine."
The data presented at the Sickle Cell Disease Association of America and NIH meeting showed outcomes from children treated under The Sibling Connection Program, a directed sibling transplant program implemented by ViaCord and Children's Hospital Oakland Research Institute (CHORI), the research arm of Children's Hospital & Research Center Oakland. This program has resulted in cord blood treatments in more than 100 children to date. Of the children treated under the Sibling Connection Program, 17 were transplanted for Sickle Cell Disease and 23 were transplanted for Thalassemia. The median age of patients treated for Sickle Cell Disease was 8 years and 5 years for patients treated for Thalassemia.
Transplantation of sibling umbilical cord blood has demonstrated clinical advantages over bone marrow transplantation in young children. In particular, the risk of graft-versus-host (GvHD) disease, a common side-effect and the leading cause of death in transplant medicine, is reduced. Of the children treated, six patients with Sickle Cell Disease had acute GvHD. No patients treated for Sickle Cell Disease had chronic GvHD. In addition, no acute or chronic GvHD was observed in patients transplanted for Thalassemia.
The median time to neutrophil recovery (ANC greater than 500 cells per microliter) and platelet recovery (greater than 20,000 per microliter) in patients treated for Sickle Cell Disease was 18 days and 36 days, respectively. 82% of the patients treated for Sickle Cell Disease survive and are disease-free. The median time to neutrophil recovery (ANC greater than 500 cells per microliter) and platelet recovery (greater than 20,000 per microliter) in patients treated for Thalassemia was 25 days and 47 days, respectively. 96% of the patients treated for Thalassemia survive and 91% are disease-free.
In 2006, ViaCell and CHORI combined their efforts in the area of directed transplants for sibling donor umbilical cord blood to form the Sibling Connection Program. To date, over 100 children have been treated by cord blood from units collected and processed through this program. This includes transplants through cord blood collected, preserved and stored with ViaCord and transplants using cord blood stored through CHORI's Sibling Donor Cord Blood Program. The Sibling Connection Program provides ViaCord's comprehensive cord blood collection, processing and five years of storage at no cost to families who have a child diagnosed with a condition that can be treated with cord blood stem cell transplant and meet the other requirements of the program.
http://www.genengnews.com/news/bnitem.aspx?name=23356278Abstract from the Sickle Cell Centers Meeting:
SIBLING DONOR CORD BLOOD TRANSPLANTATION FOR HEMOGLOBINOPATHIES
Mark C Walters, MD, Lynn Quirolo, RN, Sandie Edwards, Joanna Lee, PhD, Shanda Robertson, Kate Falcon, RN, Robert Briddell, Keith C Quirolo, MD and Bert Lubin, MD. Hematology/Oncology, Children's Hosp & Research Center at Oakland, Oakland, CA, United States, 94609 and Viacell, Inc, Cambridge, MA, United States, 02142.
The Sibling Donor Cord Blood Program was initiated in 1998 as a resource to collect, characterize, and release for transplantation cord blood units (CBU) from families affected by malignant and non-malignant disorders. As of April 2007, 2265 CBUs have been collected among referrals from all 50 US States. The categories of participation include malignant disorders (n=1128 or 50%), sickle cell disease (SCD) (n=669 or 30%), thalassemia (n=133 or 6%), and other hereditary or rare hematological conditions (n=335 or 14%).
To date, 100 children have been treated by cord blood transplantation (CBT) from CBUs in the Sibling Connection Program, 72 using the CBU as the sole source of hematopoietic cells. There was a very high rate of CBU utilization, particularly among thalassemia families where 23 of 133 (17%) of CBUs collected have been released for CBT. CBT recipients with thalassemia major (N=23) had a median age of 5.2 (range, 2.4 – 15) years and received CB grafts with a median total nucleated (TNC) and CD34+ cell dose of 8.3 x 107/kg and 1.7 x 105/kg recipient weight, respectively. CBT recipients with SCD (N=17) had a median age of 8.4 (range, 2 – 14.4) years and received CB grafts with a median TNC and CD34+ cell dose of 4.1 x 107/kg and 1.1 x 105/kg recipient weight, respectively. Hematopoietic cells from cord blood and marrow collections from the same sibling donor were combined in 29% and 35% of SCD and thalassemia recipients, respectively. The median time to ANC >500/mm3 and platelet >20,000/mm3 was 18 and 36 days, respectively among SCD recipients and was 25 and 47 days, respectively among thalassemia recipients. Among all 40 patients with hemoglobinopathies, 36 (90%) survive, and 35 (88%) survive disease-free. Graft rejection with disease recurrence occurred in 1 patient (2.5%). The rates of survival and event-free survival were somewhat better among children with thalassemia compared to those with SCD (96% and 91%, respectively, compared to 82% and 82%, respectively), which might reflect the younger age of thalassemia children at CBT. Of interest, there was no acute or chronic graft-versus-host disease (GVHD) after CBT for thalassemia, and 6 children with SCD developed grade I-III acute, but none had chronic GVHD after CBT.
These results confirm that CBT like bone marrow transplantation is curative therapy for children with clinically significant hemoglobinopathies. The ability to combine cord blood collections with a marrow harvest from the sibling donor effectively reduced the incidence of graft rejection. Transplantation of sibling CBUs in lieu of bone marrow may be particularly advantageous in very young children where cell dose and GVHD, in particular, have an important bearing upon outcome.
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Utah Jazz: Son's disease to delay Boozer Camp is 2nd to sickle cell treatment
if(requestedWidth > 0){ document.getElementById('articleViewerGroup').style.width = requestedWidth + "px"; document.getElementById('articleViewerGroup').style.margin = "0px 0px 10px 10px"; } By his own estimation, Carlos Boozer has been "working his tail off" in the offseason so he will be in the best shape possible when he joins the Jazz in training for the upcoming season.
But his reunion with his teammates in training camp has been delayed while he cares for his year-old son, Carmani, who recently underwent a bone marrow transplant to treat his sickle cell disease and remains in a Miami hospital.
The Jazz have media day Monday, after which they'll travel to Boise, Idaho, for training camp that runs through Oct. 6. The Jazz have given Boozer permission to report late. It's unknown when he'll join the team.
"As soon as my son is home from the hospital and stable, I will join my teammates," Boozer said in a statement. "Your thoughts and prayers are appreciated by me and my family during this time. We have been in contact with the Jazz organization, including Mr. [Larry] Miller, Kevin O'Connor, and Coach [Jerry] Sloan, and all of my teammates, and everyone has been incredibly supportive of my family during these days. We want everyone to know how grateful we are for that." Doctors used stem cells in the procedure from the umbilical cord of Boozer's wife, CeCe, who gave birth to twins in the summer. The procedure is one of the many new treatments researchers are studying, Edwards said.
"It's an exciting time because there are a lot of new treatments, but it's also depressing, because we don't have the funding for them," she said. "There is one genetic defect that causes this, and if we put some energy into it, this could be the first genetically cured disease."
http://origin.sltrib.com/sports/ci_7023819--------------------------------------------------------------------------------
Consumer Taskforce on Newborn Screening Established
WASHINGTON, Sept. 17 (AScribe Newswire) -- Genetic Alliance announced today the establishment of the Consumer Taskforce on Newborn Screening (CTF-NBS). The ten-member group includes parents who have experienced a range of NBS outcomes - carrier identification, false positive screening, and typical/normal screening - as well as those who have a child with a condition for which there is no medical treatment at this time, and those whose child did not have access to screening for the condition s/he has. Thus, the CTF-NBS includes parents who are experienced in various aspects of the NBS system in addition to those who are just starting to navigate these issues. The CTF-NBS will ensure the integration of consumer perspectives in the planning and implementation of the Consumer Focused Newborn Screening projects, two cooperative agreements awarded by the Genetic Services Branch of Health Resources Services Administration/HHS.
Several advocacy organizations are partnering with Genetic Alliance in the CTF-NBS: Cares Foundation, Children's Sickle Cell Foundation, Citizens for Quality Sickle Cell Care Foundation, Hunter's Hope Foundation, and Save Babies through Screening Foundation. Adds Micki Gartzke, Director of Education and Awareness for Hunter's Hope Foundation, "This Taskforce brings consumers of NBS together in a novel and innovative way. Through our discussions, the taskforce will ensure that the consumer perspective is central to the models produced from these projects."
By bringing together a broad spectrum of viewpoints, we create a forum for the assessment of different ideas about newborn screening using the question "what is at stake?" This focus enables multiple interests to be boiled down to their core fundamental issues, leading to the empowerment of consumers from different interest groups. This process will help both veteran and new NBS stakeholders to understand the issues and policies around NBS. Victoria Odesina, Co-Founder and Board member of Citizens for Quality Sickle Cell Care, remarked, "I hope that through this project, the sickle cell 'community' will have a better understanding of and involvement in NBS issues for health promotion."
Through the Consumer Focused Newborn Screening projects, the CTF-NBS will be instrumental in identifying and empowering other consumers in the NBS community. For example, by preparing consumers to attend the Secretary's Advisory Committee on Heritable Disorders and Genetic Diseases in Newborns and Children (ACHDGDNC) meetings, the CTF-NBS will be encouraging others to participate in education and policy initiatives for NBS. This will not only increase the education of these consumers but also the greater knowledge of the stakeholder communities that they represent. "The CTF-NBS will disseminate knowledge and understanding about NBS issues to the communities and stakeholders that we represent," notes Jill Levy-Fisch, President of Save Babies Through Screening Foundation.
Natasha Bonhomme, Program Coordinator for the Consumer Focused Newborn Screening projects, will manage the CTF-NBS. CONTACT: Natasha Bonhomme, nbonhomme@geneticalliance.org, 202-966-5557, x. 211
http://geneticalliance.org/ --------------------------------------------------------------------------------
A new Sickle Cell PSA running in Connecticut
See the video on Youtube at
http://www.youtube.com/watch?v=Ttt9h81H_sc--------------------------------------------------------------------------------
MDs Launch Network to Treat Sickle Cell By ANDALE GROSS – Sep 26, 2007
KANSAS CITY, Mo. (AP) — Ernestine Diamond says her intensely painful sickle cell crises started when she was a baby, but her doctors back then knew nothing about the disease. It wasn't until she had complications during the birth of one of her own children about 20 years later that she learned she had sickle cell anemia — an inherited disorder in which defective hemoglobin causes red blood cells to take on a sickle or crescent shape, hindering blood flow and depriving organs and tissues of oxygen.
Now, at age 80, Diamond is proof that sickle cell doesn't have to be a death sentence and that patients can live well into adulthood. But she and others on the disease's front line say the health care community is lagging when it comes to providing adult sickle cell patients with consistent care. "You face problems being an adult sickle cell patient," said Diamond of Kansas City, Mo. "You really have to fight to get support."
A network of health care professionals and researchers from Missouri, Kansas and more than 30 other states is working to do something doctors and other advocates have been trying to do for years: improve the sickle cell landscape so it better addresses the needs of adult patients. The group — called the Sickle Cell Adult Provider Network — is developing what its members say will be the first-ever comprehensive set of best practices for doctors and nurses to follow when treating adult sickle cell patients.
Sickle cell specialist Dr. Kathryn Hassell of Denver, who formed the network, said the first phase of the practices will be available in the next few months."Everybody who works with sickle cell recognizes it's time to start paying attention to the adults," said Hassell, director of the Colorado Sickle Cell Treatment and Research Center. "We're so good at raising the children and keeping them healthier into adulthood. It's time to do that for the adults."The practices will cover such issues as how to better manage pain for adults who have sickle cell pain crises, and whether regular blood transfusions can improve conditions of adult patients like they do children.
"I have younger patients who get blood exchanges once a month, and I don't think they should be denied that once they're adults just because there's no research," said Elizabeth Nelson, a University of Missouri Health Care nurse clinician and member of the Sickle Cell Adult Provider Network.Besides feeling intense pain throughout the body for days or weeks, sickle cell patients also can suffer breathing problems and be more at risk of having a stroke or developing an infection or organ failure.Patients generally are given pain medicine and intravenous fluids during a sickle cell pain crisis. If complications arise, they might be given a blood transfusion.
The number of people in the U.S. who have sickle cell has risen about 42 percent in recent years, said representatives of both the Sickle Cell Adult Provider Network and Sickle Cell Disease Association of America. It's now around 100,000, they said, with more adults with the disease living longer.Sickle cell mainly affects blacks, but also a growing number of Hispanics."In general people are living longer, but that shift is even more dramatic in sickle cell because of the improvements in treatment," Hassell said. "Now, we fully expect people with sickle cell disease to live into their 40s and 50s and even into their 60s and 70s." For years, sickle cell was seen as a young person's disease. "People honestly didn't think people with sickle cell would live to their 40th birthday," said Joseph Telfair, public health research professor at the University of North Carolina at Greensboro. Better treatment for infections and pain crises has led to patients living much longer, Telfair said.
"Now, you have really healthy persons with sickle cell disease who are anywhere between 18 and over who are now showing up (at hospitals and doctor's offices), and people don't know how to treat them," he said. Dr. Zahida Yasin, who's also a member of the Sickle Cell Adult Provider Network, said there needs to be more treatment centers for adults who have sickle cell."We are at the same crossroads with adults with sickle cell disease that we were with the children 30 or 40 years ago," said Yasin, an associate hematology/oncology professor at the University of Cincinnati.
Truman Medical Center in Kansas City, Mo., has an adult sickle cell center. Within the last year, the sickle cell center was redesigned to update the examination rooms and other areas. "We are trying to provide a very calm, relaxing, therapeutic environment, because that's so important to sickle cell patients," said Dr. Angela Garner, the center's medical director. Diamond is among the center's 130 patients. She said the center helps patients who have received consistent treatment in their younger years continue to get the proper care as adults.
Patient Sherry Webb, 41, also of Kansas City, Mo., said she likes how the center designs treatment plans that fit each patient's specific needs.
"We all have our different needs, and what might work for me might not work for the next person," said Webb, whose pain crises are usually sparked by stress.
Diamond and Webb said it's comforting to know that the center's staff of three keeps regular, daily hours and has sickle cell expertise. But despite bright spots like the center at Truman, they said the struggle continues for adult sickle cell patients. Things are coming up for us," Diamond said. "But they're slow in coming."
Sickle Cell Adult Provider Network: SCAPN, a network of health care professionals from more than 30 states, is developing a set of guides for health care workers to follow when treating adult sickle cell patients, the Associated Press reports. The guides will address pain management and the benefits of blood transfusions. The disease mostly affects blacks, but a number of Hispanics are being diagnosed with the disease
http://www.uchsc.edu/scapn/--------------------------------------------------------------------------------
In a Lifetime of Sickle Cell, the Evolution of a Disease
Most sickle cell anemia patients do not live long enough to span generations of doctors. But Gladys Jacobs was around when I was a medical resident in the 1980s, and she is around now. Her career — as a patient and an activist — demonstrates how the understanding of sickle cell disease has changed.
Gladys’s condition was initially misdiagnosed, as was all too common for sickle cell cases in the early ’60s. It was not until then, as the historian Keith Wailoo writes in “Dying in the City of the Blues” (University of North Carolina Press, 2001), that the disease “found its way into the public consciousness.”
When Gladys went to doctors complaining of joint aches, which were the common painful crises characteristic of sickle cell disease, she was met with skepticism. Doctors, she recalls, called her a faker.
http://www.nytimes.com/2007/10/09/health/09essa.html?_r=1&ref=health&oref=slogin--------------------------------------------------------------------------------
RUTGERS HISTORIAN KEITH WAILOO ELECTED TO INSTITUTE OF MEDICINE
New Brunswick, N.J. – Keith Wailoo, Martin Luther King Professor of History at Rutgers University, has been elected to the Institute of Medicine, one of four learned academies that advise the government on scientific matters. Wailoo, a historian of health and medicine, has helped shape new understandings of disease, politics and culture in America. He is the seventh Rutgers professor and the second historian from Rutgers to be elected to the institute.
Wailoo is a member of Rutgers’ Institute for Health, Health Care Policy and Aging Research and is founding director of the Center for Race and Ethnicity at Rutgers. Wailoo’s award-winning books, such as Dying in the City of the Blues: Sickle Cell Anemia and the Politics of Race and Health (University of North Carolina Press, 2001), have earned accolades for “elucidating questions of racial justice and inequality, and promoting human understanding.” Other major works on such topics as the impact of genetic medicine and new technology in American society include The Troubled Dream of Genetic Medicine: Ethnicity and Innovation in Tay-Sachs, Cystic Fibrosis, Sickle Cell Disease (Johns Hopkins University Press, 2006); and Drawing Blood: Technology and Disease Identity in Twentieth-Century America (Johns Hopkins University Press, 1997).
Wailoo is one of 65 new members of the Institute of Medicine (IOM). Like the other three national learned academies – the National Academy of Sciences, the National Research
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Could I Be a Better Patient? By Jennifer Huget Special to The Washington Post Tuesday, September 25, 2007; Page HE06
You think you've got this being-a-patient thing down pat: You put on your paper gown (opening in the back), flip through a dog-eared People magazine, have your blood pressure taken, see the doctor for five minutes, answer his or her questions, pay your co-pay and get back to work.
But is there anything you can do to get more out of that doctor visit?
A lot, according to three experts: Richard Frankel, a geriatrics professor and senior research scientist at the Regenstrief Institute of the Indiana University School of Medicine; Carolyn Clancy, director of the federal Agency for Healthcare Research and Quality (AHRQ); and Richard Kellerman, president of the American Academy of Family Physicians (AAFP). And you'll help your doc do a better job, too.
1.Keep Lots of Lists, and Carry Them Around: Take time to write down your medical history, including any diagnoses and key lab tests you've had, any hospitalizations or surgeries, all the medications you take -- including any herbal remedies -- and your allergies and immunizations, Kellerman advises. And carry the list with you. "You never know when you go out of town whether you're going to end up in an emergency room somewhere," he notes.
2.Bag Your Meds: Before seeing your doctor, put all your medicine bottles -- again, including any herbals or over-the-counter drugs you use -- in a bag and take them along. Your doctor can sort through them with you, looking for potentially dangerous interactions, getting rid of drugs that have expired and simply confirming that "the blue pill that I'm talking about is the same blue pill the patient is thinking of," Kellerman says.
3.Make Another List -- of Questions: "Write down what you want to talk to the doctor about," Kellerman says, "and prioritize." Frankel suggests letting your doctor know from the get-go if you have more than one concern and indicating which one is your top priority. Clancy suggests checking the AHRQ Web site for its question-builder feature (click "Questions Are the Answer" under "Consumers & Patients").
4.And Don't Be Shy About Asking Them: Asking questions "sounds easy," Clancy says, "but people don't do it very often," citing a study showing that the average patient asks only 1.4 questions per office visit -- including inquiries about parking. "If the doctor has a stethoscope in his ears, it's not the best time," Kellerman says. "Otherwise, it's open season."
5.Put It on Record: "If you have made a list of questions or concerns, ask politely to make that list a part of your permanent record," advises Frankel. "That makes it more or less a legal document, leaving no question as to whether you and the doctor talked about that stuff or not. It's a parallel process to the doctor's making written notations," he notes.
6.Take Notes: Write down the things you and your doctor discuss, making sure you understand what the doctor said. Don't hesitate to ask the doctor to repeat something, Frankel says, or even to write it down for you. "We know that there are a lot of medical errors that result from the lack of checking patients' comprehension," Frankel adds. If the doctor doesn't check to make sure you've understood, you should.
7.Bring a Friend: A trusted friend or relative can serve as an extra pair of ears in the doctor's office, Kellerman notes. "Particularly when a patient is older or if you expect something complicated, or bad news, it's helpful to have a spouse, son or daughter -- someone you trust with confidential information" -- join you. "Oftentimes they come up with questions you don't think of in these stressful times," Kellerman adds.
8.Be Wary of Online Health Info: Not all health information on the Internet is reliable, Kellerman cautions. "Talk to your doctor about which Web sites they feel comfortable with. Some have their own Web sites with links to other sites they've checked themselves," he says. Start with the AAFP site.
9.Track Down Test Results: "If you have a test, procedure or operation, be sure you know what happens" during that procedure, Clancy says. "I've had patients who have had hysterectomies but don't know whether their ovaries were removed." That information has important implications for a woman's health, she says. Don't count on your doctor to give you the details, she cautions, as sometimes such communication slips through the cracks; be prepared to ask.
10.Keep a Symptom Diary: "If you've been having symptoms on and off, and that's part of why you've made the appointment, it helps to have some kind of diary or record" of when those symptoms occurred, Clancy says. And don't worry about looking like a hypochondriac, she says; the doctor will appreciate the data and can help you sift the worrisome symptoms from the less important ones.
11.Offer Feedback: "It is our responsibility as citizens to provide feedback" to our doctors, "even if it's a difficult situation," Frankel says. "Be prepared to say, 'Gee, this isn't what I expected; it hasn't gone well,' " if need be, he suggests. "If you have trouble buying an airline ticket, you don't hesitate a nanosecond before saying, 'Can I speak to your supervisor?' But that doesn't often happen in a medical situation." For the most part, Frankel says, "physicians are very open to feedback."
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Articles in the Medical Literature
Dyson, SM; Atkin, K; Culley, LA and Dyson, SE (2007) The educational experiences of young people with sickle cell disorder: a commentary on existing literature. Disability and Society 22 (6): 581-594.
http://www.informaworld.com/openurl?genre=article&issn=0968-7599&volume=22&issue=6&spage=581 Lee SP, Ataga KI, Zayed M, Manganello JM, Orringer EP, Phillips DR, Parise LV. Phase I study of eptifibatide in patients with sickle cell anaemia. Br J Haematol. 2007 Oct 3;
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=17916103&ordinalpos=1&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum Lavelle D, Chin J, Vaitkus K, Redkar S, Phiasivongsa P, Tang C, Will R, Hankewych M, Roxas B, Singh M, Saunthararajah Y, Desimone J. Oral decitabine reactivates expression of the methylated gamma-globin gene in Papio anubis. Am J Hematol. 2007 Nov;82(11):981-5.
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=17696208&ordinalpos=1&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum van Beers EJ, van Tuijn CF, Nieuwkerk PT, Friederich PW, Vranken JH, Biemond BJ. Patient-controlled analgesia versus continuous infusion of morphine during vaso-occlusive crisis in sickle cell disease, a randomized controlled trial. Am J Hematol. 2007 Nov;82(11):955-60.
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=17617790&ordinalpos=2&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum Wu CJ, Gladwin M, Tisdale J, Hsieh M, Law T, Biernacki M, Rogers S, Wang X, Walters M, Zahrieh D, Antin JH, Ritz J, Krishnamurti L. Mixed haematopoietic chimerism for sickle cell disease prevents intravascular haemolysis. Br J Haematol. 2007 Nov;139(3):504-7. No abstract available.
McLeod BC. Evidence based therapeutic apheresis in autoimmune and other hemolytic anemias. Curr Opin Hematol. 2007 Nov;14(6):647-54.
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=17898570&ordinalpos=4&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum Klings ES. Pulmonary hypertension of sickle cell disease: More than just another lung disease. Am J Hematol. 2007 Oct 9;
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=17924550&ordinalpos=8&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum H Adewoye A, C Chen T, Ma Q, McMahon L, Mathieu J, Malabanan A, H Steinberg M, F Holick M. Sickle cell bone disease: Response to vitamin D and calcium. Am J Hematol. 2007 Oct 9;
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=17924548&ordinalpos=9&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum Delea TE, Hagiwara M, Thomas SK, Baladi JF, Phatak PD, Coates TD. Outcomes, utilization, and costs among thalassemia and sickle cell disease patients receiving deferoxamine therapy in the United States. Am J Hematol. 2007 Oct 9;
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=17924547&ordinalpos=10&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum Deane C, Goss D, O'driscoll S, Melllor S, Pohl K, Dick MC, Height SE, Rees DC. Transcranial Doppler Scanning and the Assessment of Stroke Risk in Children with HbSC disease. Arch Dis Child. 2007 Oct 9;
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=17925326&ordinalpos=11&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum Hagar RW, Michlitsch JG, Gardner J, Vichinsky EP, Morris CR. Clinical differences between children and adults with pulmonary hypertension and sickle cell disease. Br J Haematol. 2007 Oct 3;
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=17916102&ordinalpos=15&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum Marlin L, Connes P, Antoine-Jonville S, Tripette J, Montout-Hedreville M, Sanouiller A, Etienne-Julan M, Hue O. Cardiorespiratory responses during three repeated incremental exercise tests in sickle cell trait carriers. Eur J Appl Physiol. 2007 Oct 2;
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=17909842&ordinalpos=17&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum Quinn CT, Lee NJ, Shull EP, Ahmad N, Rogers ZR, Buchanan GR. Prediction of adverse outcomes in children with sickle cell anemia: a study of the Dallas Newborn Cohort. Blood. 2007 Oct 1;
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=17909076&ordinalpos=21&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum Bernaudin F, Socie G, Kuentz M, Chevret S, Duval M, Bertrand Y, Vannier JP, Yakouben K, Thuret I, Bordigoni P, Fischer A, Lutz P, Stephan JL, Dhedin N, Plouvier E, Margueritte G, Bories D, Verlhac S, Esperou H, Coic L, Vernant JP, Gluckman E; SFGM-TC. Long-term results of related myeloablative stem-cell transplantation to cure sickle cell disease. Blood. 2007 Oct 1;110(7):2749-56. Epub 2007 Jul 2.
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=17606762&ordinalpos=23&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum Sebastiani P, Nolan VG, Baldwin CT, Abad-Grau MM, Wang L, Adewoye AH, McMahon LC, Farrer LA, Taylor JG 4th, Kato GJ, Gladwin MT, Steinberg MH. A network model to predict the risk of death in sickle cell disease. Blood. 2007 Oct 1;110(7):2727-35. Epub 2007 Jun 28.
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=17600133&ordinalpos=24&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum Vasavda N, Ulug P, Kondaveeti S, Ramasamy K, Sugai T, Cheung G, Rees DC, Awogbade M, Bannister S, Cunningham J, Menzel S, Thein SL. Circulating DNA: a potential marker of sickle cell crisis. Br J Haematol. 2007 Oct;139(2):331-6.
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=17897311&ordinalpos=26&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum Vekilov PG. Sickle-cell haemoglobin polymerization: is it the primary pathogenic event of sickle-cell anaemia? Br J Haematol. 2007 Oct;139(2):173-84.
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=17897293&ordinalpos=27&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum --------------------------------------------------------------------------------
Ask the Experts
Question: Sickle cell patients have a tendency to have stunted growth. What causes this?
Answer:
There are probably multiple answers:
1) high energy to produce new red blood cells in order to replace broken red blood cells
2) high energy needs to pump blood around the body faster, to compensate for the anemia
3) inefficient use of calories because of inflammatory signals (high C-reactive protein, low leptins) from tissue damage
4) inefficient use of calories because of damaged kidney or liver (buildup of urea and acid, possibly iron overload)
5) abnormal hormonal response and delayed puberty (lack of hormones)
6) shorter spine because of bone infarcts damaging the vertebra
The major solution is to reduce sickling of red blood cells - this can be done with hydroxyurea, chronic blood transfusion, or bone marrow transplant. People with sickle cell generally gain some weight after these anti-sickling treatments.
A minor solution is to greatly improve the diet, probably increase the amount of protein by a lot.... the research on nutritional therapy for sickle cell disease is still being done, and I cannot make a specific recommendation to you of how much increase and exactly what to increase. High protein diets should only be started with the approval of your doctor, especially if there is kidney damage that might make it hard to excrete the breakdown products of the high protein diet.
Vitamins, antioxidants, and other nutritional supplements are also being researched as treatment to help people with sickle cell disease. There are very few results from this research, but some companies are trying to market nutritional supplements based on unproven ideas. Beware of companies that make pseudo-scientific claims and charge high prices for nutritional supplements that skirt the Food and Drug Administration's tight rules for proving medical treatments to be safe and effective.
Sincerely,
-Lewis Hsu, MD, PhD
Pediatric Hematologist
For more frequently asked questions please see:
http://www.scinfo.org/faq.htm --------------------------------------------------------------------------------
Featured Web Links
Explore Stem Cells at
http://www.explorestemcells.org/index.phpWilliam E. Proudford Sickle Cell Fund, Inc.
http://www.wepsicklecell.org/index.html also see
http://www.btimes.com/News/article/article.asp?NewsID=82486&sID=4International Association of Sickle Cell Nurses and Physician Assistants (IASCNAPA), the only association of nurses, physician assistants, social workers, and other health care professionals caring for individuals with sickle cell disease. The association was established in 1990, and at present, we have over 300 members worldwide.
http://www.iascnapa.org/--------------------------------------------------------------------------------
Conferences and Activities of Interest to the Sickle Cell Community
October 20 - Atlanta Eighth Annual Sickle Cell Education Day sponsored by Children's Healthcare of Atlanta
Sickle Cell Education Day is an event for everyone who has ever been affected by sickle cell disease: children, parents, relatives, friends of the family, teachers and healthcare workers. Those attending the event will have access to information about current therapies, forums to share stories and ask questions, and tips for living with sickle cell disease. In addition to the adult general session, there will be programs for school-age children (ages 6 to 12) as well as adolescents (age 13 and older).
Saturday, Oct. 20, 2007 Loudermilk Center 40 Courtland St. Atlanta, GA 30303
RSVP Though not required, preregistration is greatly appreciated. Call 404-785-0873 by Friday, Oct. 12, 2007, to let us know if you will be attending. Preregistered attendees will receive additional raffle tickets the day of the event
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Columbus Ohio October 13 - You are invited to a fabulous day of thrills, laughter, cheers and football at the Sickle Cell Movie Premier Party!!!!
Watch the new Tyler Perry Movie, “Why Did I Get Married”, stay after for a Tailgate Party and view the OSU Buckeyes on the movie screen as they roll over Kent State for only $25.00 per person!!! The festivities begin Saturday, October 13, 2007 at 9:00 am at the Arena Grand Theater located at 175 West Nationwide Boulevard , Columbus , Ohio 43215 . Tickets on sale now! Seating is limited, so call 614-228-0157 to reserve your space!
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October 1-5, 2007 London, England - Annual Sickle Cell and Thalassemia Course at Governor’s Hall, ST Thomas ’ Hospital, London Diagnosis, Prevention and Survival Outcomes, Laboratory Diagnosis -Pitfalls Screening and Re-Testing: HPLC, DNA Analysis, Mass Spectrometry (MSMS) Techniques Etc Establishing Clinical Networks of Care, New Insights In Iron Chelation , Stem Cell Transplantation, Pre-implantation Genetic Diagnosis. Neuro-Psychological Issues, Treatment Standards- Paediatric / Adults, Patient management in the community -Nursing Perspectives. Stroke in Sickle Cell Disease: TCD Session Exam Case Scenarios-MRCPath, MRCPCH, MRCP. CME applied Contacts: Cynthia.Ugochukwu@gstt.nhs.uk 0+44 () 2071884486 (Cynthia), 0+44 (0)2071887774 (Sec to Baba Inusa)
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October 19-21, 2007 Maison de la Chimie, Paris, France ESH, Eurocord, Netcord, EBMT , UT MD Anderson Cancer Center Biology and Clinical Applications of Cord Blood Cells ESH, Centre Hayem, Hopital Saint-Louis, 1, avenue Claude-Vellefaux, 75475 Paris Cedex 10, France.
Telephone: +33-1 42 06 65 40 / Fax: +33-1 42 06 05 87
Emails: caroline.lamy@univ-paris-diderot.fr or marie-france.simon@univ-paris-diderot.fr
Website:
http://www.esh.org--------------------------------------------------------------------------------
Save the Date- May 2, 2008 - New York - NY Sickle cell Symposium SPONSORED BY NEW YORK METHODIST HOSPITAL SICKLE CELL/THALASSEMIA PROGRAM
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For pictures and updates see the news page at
http://www.scinfo.org/news.htm If you would like to obtain or unsubscribe to this monthly e-mail newsletter about the latest sickle cell news or read past archives go to:
http://listserv.emory.edu/archives/sicklecell.html --
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