This Week In Sickle Cell News

Hello All!

 In April 2014, ASN' s Dr. Lewis Hsu and Dr. Bamidele Tayo visited the University of Ibadan, an ASN partner site. Dr. Hsu shared his impressions after the meeting:

ASN: I understand this was your first trip to the African continent. You visited an ASN partner institution, the University of Ibadan, Nigeria. Any first impressions about the work you see being done there?


Dr. Hsu: The energetic and skilled clinicians are making the most of limited resources. They have a strong emphasis on teaching residents and fellows.

Dr. Hsu with  Dr. Titilila Akingbola (ASN partner in Ibadan, Nigeria) and medical residents at the University of Ibadan

 
ASN: In the opening ASN discussion (see here)  you highlighted the importance of sickle cell patients and resource-poor collaborating centers gaining direct health benefits from research.  Having now visited one of the centers, are there any further comments you can make on the topic? Areas of interest?


Dr. Hsu: There are so many patients!  The amount of medical need causes me to still think that direct health benefits are important to build into research projects.  Faculty training in research appears to be underway through different channels (Fogarty, Wellcome) and nurturing some very smart people who are ready to start projects.

ASN: The purpose of the visit was to "work on issues related to randomization and treatment."   Anything interesting to report? Challenges? Breakthroughs?

Dr. Hsu: Dr. Bamidele Tayo and Dr. Titilola Akingbola have started a two-phase project and this visit is to solidify the collaboration. I had the opportunity to observe the team obtain informed consent in English and in local languages, from adult patients and from parents of pediatric patients.  I saw them draw blood from patients with poor veins, with good  skill but with technique that might create hemolysis artifacts and make those parameters suspect.

ASN: The implementation of a new born screening program, in collaboration with the Ibadan Team is one of the goals of this collaboration. What is the most challenging aspect of realizing this goal right now? Have there been any steps forward?


Dr. Hsu: Dr. Idowu Ayede built up a regional infrastructure from her neonatal sepsis prevention project. This includes community health educators for mothers, newborn data capture, a data entry team, and a computer data storage system.   We are seeking technical advice from Dr. Kwaku Ohene-Frempong and the successful newborn screening program in Kumasi, Ghana, on infrastructure like training culturally-appropriate counseling for hemoglobinopathies and capacity to test a high volume of samples for sickle hemoglobin amidst mostly fetal hemoglobin. There are probably other key ingredients to newborn screening program that we do not know yet.  Funding is needed to start the program, and then government policy and long-term funding to sustain the program permanently.

Lobby of the Pediatric Unit

Dr. Hsu continued to express optimism about the prospects of collaborations such as these and their potential to significantly improve health outcomes for sickle cell disease patients in the region.



Have a good weekend!

The ASN Team

This Week in Sickle Cell News

Hello All!

For patients with SCD, anesthetic techniques, anesthetic agents and surgical trauma pose additional risk. Perioperative care for SCD patients hence has to take into account their needs. This is the subject of a case report out of the Mustafa Kemal University Faculty of Medicine, Hatay, Turkey :
Perioperative Anaesthetic Approach in a Homozygous Sickle Cell Anaemia Patient with Frequent Pain Crises.

This week also saw the publication of important theoretical foundations for stem cell-based gene therapy, from  scientists at the Salk Institute of Biological Studies. According to a Science Daily report, the safety and reliability of existing targeted gene correction technologies were evaluated, and a new method was developed. This has important implications for attempts to model human diseases and develop potential cell replacement therapy. See the Science Daily report here. Read the research paper here.

Other findings this week:

Effects of 5‐hydroxymethyl‐2‐furfural on the volume and membrane permeability of red blood cells from patients with sickle cell disease.

Prenatal Diagnosis of Sickle Cell Disease by PCR

Clinical Significance of Assessment of Thrombospondin and Placenta Growth Factor Levels in Patients with Sickle Cell Anemia: Two Centers Egyptian Studies

β-Thalassemia hijacking ineffective erythropoietin and iron overload: Two case reports and a review of literature


Have a great weekend!


The ASN Team

This Week in Sickle Cell News

Hello All!

Health-related stigma consists of social exclusion of individuals and populations who are identified with particular health problems. It is a hidden burden of disease which often stems from misunderstandings about the etiology  and pathophysiology of disease. Health-related stigma can negatively affect  psychological and behavioral responses of both people living with the condition, and the people in their communities.  It also greatly undermines efforts to combat the disease, because it has serious implications for preventive behavior, test and care seeking behavior, quality of care and the perceptions and treatment  of people with the disease by communities, families and partners. 

As is the case for many patients of chronic diseases, sickle cell disease patients are not immune from health related stigma.  In a paper released this week, titled The Measure of Sickle Cell Stigma: Initial findings from the Improving Patient Outcomes through Respect and Trust Study Bediako et al explored the impact of stigma on sickle cell disease related outcomes in a sample of American patients. Their survey found low to medium levels of stigma, which predicated upon frequency of contact with health care systems. 

Assessing the impact of stigma on SCD related outcomes is important, particularly on the African continent which, while having the highest prevalence of the disease globally, still records low levels of awareness about the disease. There is also a lack of comprehensive treatment plans for patients. The stigma faced by sickle cell disease patients on the African continent is also significantly complicated by prevailing superstitions about the disease. Analyzing the Igbo (Nigerian) phenomenom of malevolent ogbanje:  spirit children born weak, chronically ill, and destined to die, Nigerian clinical psychologist Esther Nzewi found that 70 of the 100 children in her study believed to be malevolent ogbanje had sickle cell disease. (See: Malevolent Ogbanje: Recurrent Reincarnation or Sickle Cell Disease?) . 

A comprehensive sickle cell disease treatment strategy for African countries, hence must include not only measures to raise awareness about the disease, but also interventions to reduce stigma, as well as provide patients with support for dealing with the psychosocial stresses that health related stigma engender. As seen with the HIV/AIDS pandemic on the continent, reducing stigma is crucial to combating diseases.

Have a great weekend!

The ASN Team

This Week in Sickle Cell News

Hello All!

This week's round up of developments in sickle cell  disease research and clinical care features exciting news from the NIH's Clinical Center in Bethesda, MD where half of the adults in a stem-cell transplant trial which reversed sickle cell disease, successfully stopped anti-rejection drugs. This trial was carried out by the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) and the National Heart, Lung, and Blood Institute. This report in the Science Daily has more on the study.

This week's selection:

Development of interactive algorithm for clinical management of acute events related to sickle cell disease in emergency department

ß-Globin gene cluster haplotypes and clinical severity in sickle cell anemia patients in southern Brazil

Perioperative considerations in a sickle cell patient undergoing cardiopulmonary bypass

Assessment of Iron Status in Patient of Sickle Cell Disease and Trait and its Relationship with the Frequency of Blood Transfusion in Paediatric Patients Attending at B.S. Medical College & Hospital, Bankura, West Bengal, India 

Newborn screening for sickle cell disease–technical and legal aspects of a German pilot study with 38,220 participants 

Best Wishes!

The ASN Team