scholarly journals Impact of sickle cell disease on patients’ daily lives, symptoms reported, and disease management strategies: Results from the international Sickle Cell World Assessment Survey ( SWAY )

2020 ◽  
Author(s):  
Ifeyinwa (Ify) Osunkwo ◽  
Biree Andemariam ◽  
Baba P.D. Inusa ◽  
Fuad El Rassi ◽  
Beverley Francis‐Gibson ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Disease Management ◽  
Sickle Cell ◽  
Management Strategies ◽  
Cell Disease ◽  
Daily Lives ◽  
Assessment Survey

scholarly journals Feasibility of an mHealth self-management intervention for children and adolescents with sickle cell disease and their families

2021 ◽  
Author(s):  
Shannon Phillips ◽  
Julie Kanter ◽  
Martina Mueller ◽  
Amy Gulledge ◽  
Kenneth Ruggiero ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Rating Scale ◽  
Management Strategies ◽  
Self Management ◽  
Cell Disease ◽  
Vessel Occlusion ◽  
Feasibility Assessment ◽  
Management Intervention ◽  
Scale Scores

Abstract Sickle cell disease (SCD) is an inherited hemoglobinopathy that leads to blood vessel occlusion and multiorgan complications, including pain, that may be experienced daily. Symptom management often begins at home, and tools are needed to support self-management strategies that can be implemented by children with SCD and families. The purpose of this study was to assess the feasibility of the mHealth self-management intervention (application) Voice Crisis Alert V2 for children with SCD and families. Feasibility assessment was guided by the Reach, Efficacy, Adoption, Implementation, and Maintenance framework. Data were collected with 60 dyads (children with SCD/caregivers) at four time points. Self-management data were collected via application use, and postintervention interviews were conducted. Analyses included descriptive statistics and constant comparison with directed content analysis. Recruitment was completed in 28 weeks, with 82% retention at end-of-intervention. Mobile Application Rating Scale scores and interview data indicated high satisfaction. From baseline to mid-intervention, 94% of dyads used the application (75% of total use); 45% used the application from mid-intervention to the end-of-intervention. Dyads made 2,384 actions in the application; the most commonly used features were recording health history and recording and tracking symptoms. Few reported issues with the application; most issues occurred early in the study and were corrected. After the intervention period was completed, 37% continued to use the application. Feasibility was confirmed by meeting recruitment and retention goals, high adoption of the application, and high reported satisfaction with the application. Challenges with sustained use were encountered, and areas for improvement were identified.

scholarly journals Oxidative stress in sickle cell disease; pathophysiology and potential implications for disease management

2011 ◽  
Vol 86 (6) ◽  
pp. 484-489 ◽  
Author(s):  
Erfan Nur ◽  
Bart J. Biemond ◽  
Hans-Martin Otten ◽  
Dees P. Brandjes ◽  
John-John B. Schnog ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Sickle Cell Disease ◽  
Disease Management ◽  
Sickle Cell ◽  
Cell Disease

scholarly journals How I treat and manage strokes in sickle cell disease

Blood ◽  
2015 ◽  
Vol 125 (22) ◽  
pp. 3401-3410 ◽  
Author(s):  
Adetola A. Kassim ◽  
Najibah A. Galadanci ◽  
Sumit Pruthi ◽  
Michael R. DeBaun
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Exchange Transfusion ◽  
Management Strategies ◽  
Cell Disease ◽  
Transfusion Therapy ◽  
Hematopoietic Stem ◽  
Neuro Imaging

Abstract Neurologic complications are a major cause of morbidity and mortality in sickle cell disease (SCD). In children with sickle cell anemia, routine use of transcranial Doppler screening, coupled with regular blood transfusion therapy, has decreased the prevalence of overt stroke from ∼11% to 1%. Limited evidence is available to guide acute and chronic management of individuals with SCD and strokes. Current management strategies are based primarily on single arm clinical trials and observational studies, coupled with principles of neurology and hematology. Initial management of a focal neurologic deficit includes evaluation by a multidisciplinary team (a hematologist, neurologist, neuroradiologist, and transfusion medicine specialist); prompt neuro-imaging and an initial blood transfusion (simple followed immediately by an exchange transfusion or only exchange transfusion) is recommended if the hemoglobin is >4 gm/dL and <10 gm/dL. Standard therapy for secondary prevention of strokes and silent cerebral infarcts includes regular blood transfusion therapy and in selected cases, hematopoietic stem cell transplantation. A critical component of the medical care following an infarct is cognitive and physical rehabilitation. We will discuss our strategy of acute and long-term management of strokes in SCD.

scholarly journals Current and novel therapies for the prevention of vaso-occlusive crisis in sickle cell disease

2020 ◽  
Vol 11 ◽  
pp. 204062072095500
Author(s):  
Ifeyinwa Osunkwo ◽  
Deepa Manwani ◽  
Julie Kanter
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Severe Pain ◽  
Management Strategies ◽  
Organ Damage ◽  
Care Utilization ◽  
Cell Disease ◽  
High Resource ◽  
Emerging Treatments

Individuals with sickle cell disease (SCD) are living further into adulthood in high-resource countries. However, despite increased quantity of life, recurrent, acute painful episodes cause significant morbidity for affected individuals. These SCD-related painful episodes, also referred to as vaso-occlusive crises (VOCs), have multifactorial causes, and they often occur as a result of multicellular aggregation and vascular adherence of red blood cells, neutrophils, and platelets, leading to recurrent and unpredictable occlusion of the microcirculation. In addition to severe pain, long-term complications of vaso-occlusion may include damage to muscle and/or bone, in addition to vital organs such as the liver, spleen, kidneys, and brain. Severe pain associated with VOCs also has a substantial detrimental impact on quality of life for individuals with SCD, and is associated with increased health care utilization, financial hardship, and impairments in education and vocation attainment. Previous treatments have targeted primarily SCD symptom management, or were broad nontargeted therapies, and include oral or parenteral hydration, analgesics (including opioids), nonsteroidal anti-inflammatory agents, and various other types of nonpharmacologic pain management strategies to treat the pain associated with VOC. With increased understanding of the pathophysiology of VOCs, there are several new potential therapies that specifically target the pathologic process of vaso-occlusion. These new therapies may reduce cell adhesion and inflammation, leading to decreased incidence of VOCs and prevention of end-organ damage. In this review, we consider the benefits and limitations of current treatments to reduce the occurrence of VOCs in individuals with SCD and the potential impact of emerging treatments on future disease management.

scholarly journals Current modalities of sickle cell disease management

Blood Science ◽  
2020 ◽  
Vol 2 (4) ◽  
pp. 109-116
Author(s):  
Adekunle Sanyaolu ◽  
Ejoke Agiri ◽  
Carl Bertram ◽  
Latasha Brookes ◽  
Jesy Choudhury ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Disease Management ◽  
Sickle Cell ◽  
Cell Disease

scholarly journals Current sickle cell disease management practices in Nigeria

2013 ◽  
Vol 6 (1) ◽  
pp. 23-28 ◽  
Author(s):  
N. Galadanci ◽  
B. J. Wudil ◽  
T. M. Balogun ◽  
G. O. Ogunrinde ◽  
A. Akinsulie ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Disease Management ◽  
Sickle Cell ◽  
Management Practices ◽  
Cell Disease

scholarly journals An unusual case of sarcoidosis in an adult patient with sickle cell disease: Management with methotrexate and low dose of steroid

2013 ◽  
Vol 88 (3) ◽  
pp. 243-243 ◽  
Author(s):  
Letizia Delmonte ◽  
Alberto Zamo ◽  
Maurizio Cantini ◽  
Lucia de Franceschi
Keyword(s):  
Sickle Cell Disease ◽  
Disease Management ◽  
Adult Patient ◽  
Sickle Cell ◽  
Low Dose ◽  
Unusual Case ◽  
Cell Disease

scholarly journals Self management strategies for people with sickle cell disease in Ghana

2021 ◽  
Author(s):  
◽  
Andrews Adjei Druye
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Health Professionals ◽  
Best Practice ◽  
Management Strategies ◽  
Preventive Health ◽  
Self Management ◽  
Sequential Data ◽  
Cell Disease ◽  
Management Actions

<p>Sickle cell disease (SCD) is the commonest inherited haemoglobinopathy worldwide. Although it is a chronic disease, there is presently no international best practice for self-management of SCD. This exploratory sequential mix-methods study was designed to describe the self-management strategies utilised and their effectiveness for persons with SCD in Ghana. Self-management was conceptualised as actions that concerned preventive health, self-monitoring, self-diagnosing, self-treatment and self-evaluation. A four-stage sequential data collection and analysis process was used. Methods included website review, key informant interviews and surveys. The website review extracted from publically available websites the advice provided to people with SCD regarding keeping well, minimising the development of complications and managing complications. Health professional (n=9) interviews concerned self-management advice and the experiences patients shared of their self-management. Patients with SCD (n=9) and parents of children with SCD (n=10) interviews captured their experiences of self-management; and 112 patients (≥ 16 years) and 201 parents of children (≤15 years) were surveyed about their self-management strategies. Analysis used descriptive and inferential statistics and content and thematic techniques. Patients and parents reported that despite receiving limited clinical advice on self-management they undertook at least one self-management action daily. Most patients reported using multiple strategies; preventive health and maintenance actions were the commonest. Whilst most actions were directed at pain, patients and parents also outlined strategies for managing problems such as fever, leg ulcers, anaemia, priapism, abdominal swelling, jaundice, and hip pain. Most self-management actions were clinically safe, however several unsafe actions identified could pose health risks to patients. Overall, parents tended to be more frequent users of self-management actions for their children than the adults were for themselves. Apart from being an adult or child, self-management was not significantly influenced by key vulnerabilities and contextual factors. Although, many patients and parents had tried traditional practices, most only utilised recommendations from health professionals. Most patients and parents had rated the quality their or their child’s quality of life as good. There is a need for the development of an international, evidenced-informed approach to self-management of SCD. The Ministry of Health, health professionals and the Sickle Cell Association of Ghana could work collectively on this to ensure a culturally appropriate and economically viable approach is developed and implemented.</p>

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