PROJECT RESOURCES
Authoritative tools and resources on Sickle Cell Disease available to all
Tools and resources for each of the concerned items are included in this section. They include ARISE outputs, as well as relevant material developed in the framework of other Sickle Cell Disease-related initiatives. Select a category to access resources.
Epidemiology studies
Epidemiological studies to estimate the prevalence of SCD disease, predict future healthcare needs, map the existing healthcare provision and infrastructure.
Dormandy E. et al. How many people have sickle cell disease in the UK?
Ghafuri D.L. et al. World Health Organization’s Growth Reference Overestimates the Prevalence of Severe Malnutrition in Children with Sickle Cell Anemia in Africa
Modell B. et al. Global epidemiology of haemoglobin disorders and derived service indicators
Kountouris P. et al. IthaGenes: An Interactive Database for Haemoglobin Variations and Epidemiology
Piel F.B. et al. Global epidemiology of sickle haemoglobin in neonates: a contemporary geostatistical model-based map and population estimates
Newborn Screening and Comprehensive care
Implementing a Newborn Sickle Cell Disease screening and comprehensive care programme is a key output for ARISE.
Laboratory diagnostics and quality assurance systems
Improve the quality and capacity of laboratory diagnostic testing services allows a timely diagnosis available to all patients. This allows to prevent complications.
Cherif-Alami S. et al. Serum Immunoglobulin Levels in Children with Sickle Cell Disease: A Large Prospective Study
Sickle Cell Disease complications (e.g. pain, chronic and organ damage)
Sickle Cell Disease might lead to severe complications such as pain and damages in organs, that might be fatal if not properly addressed.
Michele Arigliani et al. Differences in lung function between children with sickle cell anaemia from West Africa and Europe.
Inusa P.D.B. et al. Sickle Cell Disease—Genetics, Pathophysiology, Clinical Presentation and Treatment
Inusa P.D.B. et al. An Educational Study Promoting the Delivery of Transcranial Doppler Ultrasound Screening in Paediatric Sickle Cell Disease: A European Multi-Centre Perspective
Inusa P.D.B. et al. Dilemma in Differentiating between Acute Osteomyelitis and Bone Infarction in Children with Sickle Cell Disease: The Role of Ultrasound
Allali S. et al. Hepatobiliary Complications in Children with Sickle Cell Disease: A Retrospective Review of Medical Records from 616 Patients
Denoix E. et al. Primary Hyperparathyroidism in Sickle Cell Disease: An Unknown Complication of the Disease in Adulthood
Veluswamy S. et al. Vaso-Occlusion in Sickle Cell Disease: Is Autonomic Dysregulation of the Microvasculature the Trigger?
Telfer P. et al. Evaluation of a Non-Parenteral Opioid Analgesia Protocol for Acute Sickle Cell Pain Episodes in Children
El-Amin N. et al. International Differences in Outpatient Pain Management: A Survey of Sickle Cell Disease
Community training and Implementation Science
Comprehensive training for all professionals and an engagement programme for community and families will be essential to establish the appropriate environment for population screening.
Inusa P.D.B. et al. Utilising the ‘Getting to Outcomes’ Framework in Community Engagement for Development and Implementation of Sickle Cell Disease Newborn Screening in Kaduna State, Nigeria
DiMartino L. D. et al. The sickle cell disease implementation consortium: Translating evidence-based guidelines into practice for sickle cell disease
Hsu LL et al. Community Health Workers as Support for Sickle Cell Care
eHealth technologies
eHealth technologies are expected to accelerate the implementation, dissemination, and sustainability of the Newborn screening programme.
Genomics and genetics of Sickle Cell Disease
Improved knowledge in genetics and genomics of Sickle Cell Disease lead to improve the diagnosis, treatment, and prevention of sickle cell disease and other chronic illnesses.
Ezekekwu C. A. et al. Sickle Cell Disease Clinical Trials and Phenotypes
Puri L. et al. GSTM1 and Liver Iron Content in Children with Sickle Cell Anemia and Iron Overload
Treatments for Sickle Cell Disease
Bone marrow transplantation is the only curative therapeutic approach for Sickle Cell Disease. Patients affected by Sickle Cell Disease can be treated with Hydroxyurea. However, the recent research progress can offer new therapeutic approaches.
Inusa P.D.B. et al. Low-dose hydroxycarbamide therapy may offer similar benefit as maximum tolerated dose for children and young adults with sickle cell disease in low-middle-income settings
Bernaudin F. et al. Why, Who, When, and How? Rationale for Considering Allogeneic Stem Cell Transplantation in Children with Sickle Cell Disease
Saraf S.L. et al. Allogeneic Hematopoietic Stem Cell Transplantation for Adults with Sickle Cell Disease
Nazon C. et al. Optimizing Hydroxyurea Treatment for Sickle Cell Disease Patients: The Pharmacokinetic Approach
Rozen L. et al. Is There Any Improvement of the Coagulation Imbalance in Sickle Cell Disease after Hematopoietic Stem Cell Transplantation?
Limerick F. et al. Choice of Donor Source and Conditioning Regimen for Hematopoietic Stem Cell Transplantation in Sickle Cell Disease
Howard J. et al. Voxelotor in adolescents and adults with sickle cell disease (HOPE): long-term follow-up results of an international, randomised, double-blind, placebo-controlled, phase 3 trial – (discover more)
Inusa P.D.B. et al. Will the changing therapeutic landscape meet the needs of patients with sickle cell disease? – (discover more)
Inusa P.D.B. et al. Global burden of transfusion in sickle cell disease
Ethics and Regulations
Ethical and regulatory issues to be considered to conduct research activities in the framework of the project.
Guidelines for Ethical Conduct of Biomedical Research Involving Human Subjects in Kenya, 2004
National Code of Health Research Ethics, 2007 (Nigeria)
Regulation (EU) 2016/679 – General Data Protection Regulation, 2016
Nigeria Data Protection Regulation, 2019
The Data Protection Act, 2019 (Kenya)
Context Analysis of the Nigerian ethical and legal framework (ARISE task 2.1)
Context Analysis of the Kenyan ethical and legal framework (ARISE task 2.1)
Other resources
For further information on ARISE project achievements, you can access the documents available on the Zenodo ARISE community
Mainous A.G. III et al. Shared Care for Adults with Sickle Cell Disease:An Analysis of Care from Eight Health Systems
Inusa P.D.B. et al. An Educational Study Promoting the Delivery of Transcranial Doppler Ultrasound Screening in Paediatric Sickle Cell Disease: A European Multi-Centre Perspective
Brousse V. et al. One-Fifth of Children with Sickle Cell Anemia Show Exercise-Induced Hemoglobin Desaturation: Rate of Perceived Exertion and Role of Blood Rheology
Inusa P.D.B. et al. Geographic Differences in Phenotype and Treatment of Children with Sickle Cell Anemia from the Multinational DOVE Study
Honsel V. et al. Comparison between Adult Patients with Sickle Cell Disease of Sub-Saharan African Origin Born in Metropolitan France and in Sub-Saharan Africa
Inusa P.D.B. et al. Paediatric to adult transition care for patients with sickle cell disease: a global perspective
The Royal College of Pathologists. The July edition of the College Bulletin