This Week in Sickle Cell News

Hello All!

Lining up quite nicely with last week's revelation of newly developed methods to detect Sickle Cell Disease (SCD), is the National Heart, Lung, and Blood Institute's release this week of  comprehensive, evidence-based guidelines for SCD management. These guidelines are intended to inform care and treatment of the disease over the course of the patients life time. They represent a huge step forward for management of SCD for patients in the United States. Read more here.

Clearly defined and contextually relevant guidelines for SCD treatment remains a challenge to be conquered for clinicians and other healthcare providers on the African continent. As stated by Makani et al.(2013) in a paper assessing these challenges:

"In many African countries there are few or virtually no facilities for appropriate diagnosis and management of SCD. There is limited data about frequency, clinical course, or mortality. Without this information it will be impossible to persuade African governments about the burden of this disease."

There continues to be a need for research and collaborations towards getting this infoirmation.

Other developments this week:

Predictive value of pain intensity in the clinical severity of painful crises in children and adolescents with sickle cell diseases

Population and Public Health Implications of Child Health and Reproductive Outcomes Among Carrier Couples of Sickle Cell Disorders in Madhya Pradesh, Central India

Assessment of Ventricular Function in Adults with Sickle Cell Disease: Role of Two-Dimensional Speckle-Tracking Strain

The Prevalence Rate and Neurocognitive Morbidity Associated with Obstructive Sleep Apnea in Children with Sickle Cell Disease

LIN28A Expression Reduces Sickling of Cultured Human Erythrocytes

Distribution of Sickle Cell Disease in Different Communities of Patient Visiting Out Patient Department

 Proteinuria in patients with sickle cell disease

Have a great weekend!

The ASN Team

This Week in Sickle Cell News

Hello All!

Early diagnosis of Sickle Cell Disease (SCD) remains one of the most important management strategies for the disease. Certain complications can be prevented and treated with early diagnosis. Post natal screening for  SCD and other genetic diseases is the standard for most developed  nations, which means SCD diagnosis usually comes earlier in life.  Diagnosis remains a challenge in most underdeveloped nations where the available health care infrastructure and services often do not have the capacity for such screening. The development of a quick and inexpensive tests for SCD is hence a priority. According to Science Daily, Havard Post-Doctoral fellow A.J. Kumar and his colleagues at the lab of George Whitesides, the Woodford L. and Ann A. Flowers University Professor, have developed a new test for SCD capable of providing results in just 12 minutes. The test which costs   as little as 50 cents is currently the fastest and least expensive test for SCD available.  Read more about the test and its techniques here.


Other developments this week:

Blood and marrow transplantation for sickle cell disease: Is less more?

Hydroxyurea and Growth in Young Children With Sickle Cell Disease

Phytomedicines of sickle cell crisis in Mezam Division, Cameroon: preventive and curative cares

Patient reports of health outcome for adults living with sickle cell disease: development and testing of the ASCQ-Me item banks.

The Effectiveness of Self-Management Programs on Self-Efficacy in Patients With Sickle Cell Disease

Controlled Trial of Transfusions for Silent Cerebral Infarcts in Sickle Cell Anemia

Evaluating the knowledge of sickle cell disease and hemoglobin electrophoretic pattern among people living in Sekondi-Takoradi Metropolis, Ghana

Sickle Cell Trait and Incident Ischemic Stroke in the Atherosclerosis Risk in Communities Study

Abnormalities in renal tubular phosphate handling in children with sickle cell disease

Acute Chest Syndrome

Sickle Cell Disease Management


Have a great weekend!

The ASN Team.

This Week in Sickle Cell News

Hello All!

Fetal hemoglobin (HbF) is  the primary oxygen transport protein in the human fetus. Its ability to more efficiently bind oxygen is crucial to the early stages of development. It continues to perform this role in the newborn until the sixth postnatal month, by which time is it almost completely replaced by adult hemoglobin. Certain blood disorders result in the continued production of HbF and in adults, its production can be pharmacologically reactivated. 

HbF has been found to mitigate the effects of vaso-occlusive crises in SCD patients. This is likely as a result of its high oxygen affinity. This makes it useful to the  management and treatment of SCD, especially in the prevention of vaso-occlusive crises which are triggered by low oxygenation. Research into the genes involved in HbF expression and silencing are hence key factor in the fight against SCD. In a study carried out in Northern Brazil, Cardoso et al. recently identified the alleles which primarily influence the production of high levels of HbF. Suzuki et al, also released a paper which explores the molecular mechanisms which control fetal globin gene silencing. Furthermore, according to a Science Daily report, researchers at Kings College, London have also successfully traced the global distribution of beneficial variants of genes involved in red blood cell development and HbF production in adults. These revelations have important implications for future therapies targetting SCD and other hemoglobinopathies.

In other SCD related developments:

Hematopoietic SCT for the Black African and non-Black African variants of sickle cell anemia

Duodenal perforation: an unusual complication of sickle cell anemia

Malaria Resistance and Sickle Cell Trait: A Review

Antisickling Activity and Membrane Stabilizing Effect of Anthocyanins Extracts from Adansonia digitata L. Barks on Sickle Blood Cells.

Clinical evolution of a patient with sickle-cell anemia in treatment with blood transfusion since birth

Very high levels of C-reactive protein should alert the clinician to the development of acute chest syndrome in sickle cell patients

Have a great weekend!

The ASN Team

This Week in Sickle Cell News

Hello All!

Efforts to improve the quality of life of patients living with Sickle Cell Disease often run into difficulty when faced with the question of pregnancy. As stated in this comment by Silva-Pinto et al, hematologists and obstetricians remain mostly  in the dark about outcomes for pregnant women with the disease, given that Sickle Cell disease predisposes women to a host of complications, and most of the knowledge about the disease is based on data acquired from unpregnant women. Silva-Pinto et al further carried out an analysis  of 34 pregnant women who have the disease. In addition to confirming the high prevalence of complications in pregnant Sickle Cell Disease patients, they noted some possible benefits to transfusions. Research remains crucial to improving outcomes for these and all other patients of the disease.

More from this week:

Malaria Resistance and Sickle Cell Trait

Raised Haemoglobin F (HbF) Level in Haemoglobinopathies: an Indicator of Polymorphism

Red blood cells of sickle cell disease patients exhibit abnormally high abundance of N-methyl D-aspartate receptors mediating excessive calcium uptake

Haemoglobin Patterns in Patients with Sickle Cell Haemoglobinopathies

Reduction of Intramedullary Apoptosis after Stem Cell Transplantation in Black African Variant of Pediatric Sickle Cell Anemia

Treating Pain in Sickle Cell Disease with Opioids : Clinical Advances, Ethical Pitfalls

Self-Efficacy, Transition, and Patient Outcomes in the Sickle Cell Disease Population

Serum Lipid Profile In Sickle Cell Disease Patients In Raipur District, Chhattisgarh


Sickle Cell Disease Patients With and Without Extremely High Hospital Use: Pain, Opioids, and Coping

Hemoglobin K-Woolwich (Hb KW): Its Combination with Sickle Cell Trait

Transfusional Iron Overload and Iron Chelation Therapy in Thalassemia Major and Sickle Cell Disease


Have A Great Weekend!


The ASN Team

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