Attitudes toward Management of Sickle Cell Disease and Its Complications: A National Survey of Academic Family Physicians

Objective. Sickle cell disease (SCD) is a disease that requires a significant degree of medical intervention, and family physicians are one potential provider of care for patients who do not have access to specialists. The extent to which family physicians are comfortable with the treatment of and concerned about potential complications of SCD among their patients is unclear. Our purpose was to examine family physician’s attitudes toward SCD management.Methods. Data was collected as part of the Council of Academic Family Medicine Educational Research Alliance (CERA) survey in the United States and Canada that targeted family physicians who were members of CERA-affiliated organizations. We examined attitudes regarding management of SCD.Results. Overall, 20.4% of respondents felt comfortable with treatment of SCD. There were significant differences in comfort level for treatment of SCD patients depending on whether or not physicians had patients who had SCD, as well as physicians who had more than 10% African American patients. Physicians also felt that clinical decision support (CDS) tools would be useful for treatment (69.4%) and avoiding complications (72.6%) in managing SCD patients.Conclusions. Family physicians are generally uncomfortable with managing SCD patients and recognize the utility of CDS tools in managing patients.

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Hematopoietic stem cell transplant referral patterns for children with sickle cell disease vary among pediatric hematologist/oncologists’ practice focus: A Sickle Cell Transplant Advocacy and Research Alliance (STAR) study

Pediatric Blood & Cancer ◽

10.1002/pbc.28861 ◽

2021 ◽

Author(s):

Emily Riehm Meier ◽

Allistair A. Abraham ◽

Alex Ngwube ◽

Isaac A. Janson ◽

Gregory M.T. Guilcher ◽

...

Keyword(s):

Stem Cell ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Hematopoietic Stem Cell Transplant ◽

Stem Cell Transplant ◽

Cell Transplant ◽

Cell Disease ◽

Referral Patterns ◽

Hematopoietic Stem ◽

Research Alliance

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Red cell storage age policy for patients with sickle cell disease: A survey of transfusion service directors in the United States

Transfusion and Apheresis Science ◽

10.1016/j.transci.2015.12.001 ◽

2016 ◽

Vol 54 (1) ◽

pp. 158-162 ◽

Cited By ~ 2

Author(s):

Matthew S. Karafin ◽

Arun K. Singavi ◽

Mehraboon S. Irani ◽

Kathleen E. Puca ◽

Lisa Baumann Kreuziger ◽

...

Keyword(s):

United States ◽

Sickle Cell Disease ◽

Sickle Cell ◽

The United States ◽

Red Cell ◽

Cell Disease ◽

Cell Storage

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Children with sickle cell disease migrating to the United States from sub-Saharan Africa

Pediatric Blood & Cancer ◽

10.1002/pbc.27000 ◽

2018 ◽

Vol 65 (6) ◽

pp. e27000 ◽

Cited By ~ 5

Author(s):

Courtney D. Thornburg ◽

Russell E. Ware

Keyword(s):

United States ◽

Sickle Cell Disease ◽

Sickle Cell ◽

The United States ◽

Sub Saharan Africa ◽

Cell Disease ◽

Sub Saharan

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Antimicrobial Resistance and Sickle Cell Protein Analysis in Invasive Non-Typhoidal Salmonella Isolated From Outpatient and Hospitalized Children Below 16 Years in Informal Settlements in Nairobi Kenya

10.21203/rs.3.rs-107187/v1 ◽

2020 ◽

Author(s):

Susan Kavai ◽

Cecilia Mbae ◽

Celestine Wanjiku ◽

Ronald Ngetich ◽

Zillah Wakio ◽

...

Keyword(s):

Sickle Cell Disease ◽

Salmonella Typhimurium ◽

Sickle Cell ◽

Informal Settlements ◽

The United States ◽

Sub Saharan Africa ◽

Cell Protein ◽

Cell Disease ◽

Resistance Phenotype ◽

Stool Samples

Abstract Background: Invasive Non-typhoidal Salmonella (iNTS) disease continues to be a major public health problem, especially in sub Saharan Africa where incidence rates are 227 cases [range 152-341] per 100,000 population. Populations at risk of iNTS include adults with HIV infection, malnourished children, those with recent malaria or sickle-cell anaemia (SCA). Individuals with SCA are at an increased risk of invasive bacterial infections with the proportion of deaths from infection reported to be as high as 38% in the United States and 29% in Jamaica. In Kenya, iNTS disease is particularly a major challenge in poor informal settlements with infants and young children less than 5 years of age being the most affected; mortality rates can be 20-25% unless prompt treatment is administered. Methods Our study was conducted in 3 outpatient sites and 1 inpatient site, the outpatient sites were all located within Mukuru informal settlement, a densely populated slum, 15km East of Nairobi City. Blood and stool samples from children with fever alone and with fever and diarrhea were collected for processing for presence of iNTS using basic microbiology procedures. Dry blood spots were also taken and processed for sickle cell protein markers using High performance liquid chromatography (HPLC). Results A total of 22,246 blood and stool samples were collected from children < 16 years of age with fever/with or without diarrhea, for a period of 6 years and subjected to microbiological culture and detection of bacterial pathogens. Out of these 741 (3.3%) tested positive for Salmonella species. A total of 338/741(41%) NTS were isolated across all the sites; these consisted of 158/741(21%) Salmonella Enteritidis and 180/741 (24%) Salmonella Typhimurium. The most common resistance phenotype was ampicillin, cotrimoxazole and chloramphenicol (35.03%). We had 12/338 (3.6%) isolates (11 of them being Salmonella Typhimurium) that were ESBL producers conferring resistance to 3rd generation cephalosporins (Amp C β-lactamases) while only 0.3% were resistant to ciprofloxacin. A total of 118 (35.03%) isolates were MDR. Out of 2684 dry blood samples subjected to HPLC for investigation of sickle cell disease traits, 1820/2684 (67%) had normal hemoglobin (Hb AA/ Hb AF); (162/2684 (6%) tested positive for Sickle Cell Traits (Hb AS/Hb AFS); while 4/2684 (0.2%) tested positive for Sickle cell disease (Hb FS). Conclusion The high MDR resistance phenotype in iNTS isolates and emerging resistance to third generation cephalosporins is of great concern in management of iNTS in our settings. Sickle cell disease was not a major factor among children with iNTS disease and no significant association with iNTS was observed.

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Newborn Screening for Sickle Cell Disease and Other Hemoglobinopathies

PEDIATRICS ◽

10.1542/peds.83.5.813 ◽

1989 ◽

Vol 83 (5) ◽

pp. 813-814

Author(s):

DORIS WETHERS ◽

HOWARD PEARSON ◽

MARILYN GASTON

Keyword(s):

Sickle Cell Disease ◽

Early Diagnosis ◽

Sickle Cell ◽

Sickle Cell Anemia ◽

Case Fatality ◽

The United States ◽

Early Mortality ◽

Cell Disease ◽

Newborn Period ◽

The Past

Hemoglobinopathies represent one of the major health problems in the United States and constitute the most common genetic disorders in some populations. Sickle cell disease (SS, SC, S-β-thalassemia) alone affects about one in 400 American black newborns, as well as persons of African, Mediterranean, Asian, Caribbean, Middle Eastern, and South and Central American origins. For the past 20 years, the medical profession has known that children with sickle cell anemia have an increased susceptibility to severe bacterial infection, particularly due to Streptococcus pneumoniae. The risk of major infection and death posed by this organism is greatest in the first 3 years of life and can occur as early as 3 months of age. In fact, this infection may be the first clinical manifestation of disease. The infection can be fulminant, progressing from the onset of fever to death in a matter of hours, and the case fatality rate is reported as high as 30%. In addition, acute splenic sequestration, another acute catastrophic event, contributes to early mortality in children with sickle cell anemia and may occur as early as 5 months of age. It has been proposed that early diagnosis to identify infants with major sickle hemoglobinopathies, who have a high risk of early mortality and morbidity, is essential to institute appropriate ongoing care and effective measures of prophylaxis and intervention. Early diagnosis of hemoglobinopathies should be in the newborn period. Even though the technology to screen infants in the newborn period has been available for the past 15 to 20 years, screening has not received widespread acceptance.

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Psychological Predictors of Pain in Children and Adolescents With Sickle Cell Disease: A Scoping Review

Journal of Pediatric Oncology Nursing ◽

10.1177/1043454218819457 ◽

2018 ◽

Vol 36 (2) ◽

pp. 150-159

Author(s):

Clare Donohoe ◽

Ellen Lavoie Smith

Keyword(s):

Sickle Cell Disease ◽

Children And Adolescents ◽

Sickle Cell ◽

Middle Eastern ◽

The United States ◽

Health Science ◽

Psychological Characteristics ◽

Cell Disease ◽

Pain Frequency ◽

Psychological Risk

Objective: Sickle cell disease (SCD) is a common red blood cell disorder that disrupts the lives of many African Americans and those of Middle Eastern heritage within the United States due to frequent pain. There is limited research quantifying biopsychosocial factors, specifically psychological characteristics, that influence pain in children and adolescents with SCD. The aim of this literature review was to identify psychological characteristics that are predictive or associated with pain in children and adolescents with SCD. Method: This review was conducted using PRISMA guidelines. Four databases, PubMed, CINAHL, PsycINFO, and Scopus, were searched using specific terms to address the aim of the review (SCD, pain, pediatrics and adolescents, and psychological characteristics). Results: The review identified a lack of consensus regarding the definitions and measurement of pain frequency and intensity. A variety of psychological characteristics were associated with pain including coping strategies, anxiety, depressive symptoms, catastrophizing, and stress. Overall, the study designs restricted the ability to fully identify psychological characteristics that predict pain. Conclusions: Health science researchers must strive for a deeper understanding about the presentation of SCD pain and psychological risk factors associated with increased pain to provide targeted screening and treatment.

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