Description: Sickle Cell in Focus is a hugely successful and intensive 2.5 day annual conference. Now in its 7th year, the heart of the event is to update and discuss the current clinical and management issues of sickle cell disease and thalassaemia. This year the programme will include more debates, delegate presentations on complex case studies and more time to network. The conference attracts international and national speakers and delegates and will be of interest to consultants, medical trainees and other healthcare professionals involved in the care of patients with red blood cell disorders, as well as academics with a research interest in this field
7th Annual Sickle Cell and Thalassaemia Conference: UK 3-5 October 2013
Improving the quality of life for patients affected with Sickle Cell Disease and Thalassaemia
17. Hamideh D, Alvarez O. Sickle Cell Disease Related Mortality in the United States (1999–2009 ). [Epub ahead of print 23 APR 2013 DOI: 10.1002/pbc.24557].
Sickle cell trait (SCT) is usually benign. However, there are some conditions that may lead to SCT-related problems and put athletes with the trait at particular risk. In 2010 the National Collegiate Athletic Association (NCAA) issued a policy that required all Division I (DI) student-athletes to confirm their SCT status or sign a liability waiver to opt out of testing. Athletic trainers and team physicians play key roles in the policy implementation and we examined their perceptions and practices. Between December 2013 and March 2014 we interviewed 13 head athletic trainers and team physicians at NCAA Division I colleges and universities in North Carolina. We used an interview guide with open-ended questions covering knowledge of SCT, historical screening and education practices, current implementation, and policy benefits and challenges. Participants were knowledgeable about SCT and thought the policy was beneficial in providing SCT health information to and for student-athletes. Schools varied in provision of genetic counseling, offering the waiver, SCT tests administered, and other aspects. Challenges included: insufficient guidance from the NCAA; financial considerations; and misunderstanding of the relationships of race and ancestry to SCT risk. Athletic staff found the policy valuable, but felt it needs clarity and standardization.
Treatment for anemia depends on its cause.
Hematopoietic stem cell transplantation from HLA-matched sibling donors results in disease free survival of >90% in patients with sickle cell disease (SCD) but only about 18% have suitable donors. Unrelated cord blood transplantation (UCBT) is one way to expand donor options for patients with severe SCD but historically has been associated with high graft rejection rates (50-62%). We hypothesized that the addition of thiotepa to a previously tested reduced intensity conditioning (RIC) regimen would support engraftment after UCBT in SCD patients. Nine children (3-10 years) with cerebrovascular complications of SCD underwent 5-6/6 HLA-matched (A, B and DRB1 loci) UCBT after conditioning with hydroxyurea, alemtuzumab, fludarabine, thiotepa and melphalan. A calcineurin inhibitor and mycophenolate mofetil were used for graft-versus-host-disease (GVHD) prophylaxis. With median follow up of 2.1 (range 1-4.2) years, seven patients had sustained donor cell engraftment and are free of SCD, two had autologous recovery. Acute GVHD (grade 2-4), mild, and moderate chronic GVHD developed in three, two, and one patient respectively. Of five patients >2 years post-UCBT, four have discontinued systemic immunosuppression. Seven patients had viral infections (CMV, EBV, RSV or Adenovirus) and recovered. The 1-year overall and disease free survival rates were 100% and 78% respectively. Thus, this RIC regimen was able to achieve donor engraftment in the majority. Future efforts will focus on further reducing acute GVHD and viral infection rates.
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Sickle cell anemia, (SCA) one of the three distinct types of sickle cell disease, is the most common inherited blood disorder in the United States. The disease affects the following:
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Anemia is when the level of healthy red blood cells (RBCs) in the body becomes too low. This can lead to health problems because RBCs contain hemoglobin, which carries oxygen to the body's tissues. Anemia can cause a variety of complications, including fatigue (tiredness) and stress on the body's organs.