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1.
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Cochrane Database Syst Rev. 2015 May 8;5:CD001916. [Epub ahead of print]
Abstract
BACKGROUND:
Sickle cell disease comprises a group of genetic blood disorders. It occurs when the sickle haemoglobin gene is inherited from both parents. The effects of the condition are: varying degrees of anaemia which, if severe, can reduce mobility; a tendency for small blood capillaries to become blocked causing pain in muscle and bone commonly known as 'crises'; damage to major organs such as the spleen, liver, kidneys, and lungs; and increased vulnerability to severe infections. There are both medical and non-medical complications, and treatment is usually symptomatic and palliative in nature. Psychological interventions for individuals with sickle cell disease might complement current medical treatment, and studies of their efficacy have yielded encouraging results. This is an update of a previously published Cochrane Review.
OBJECTIVES:
To examine the evidence that psychological interventions improve the ability of people with sickle cell disease to cope with their condition.
SEARCH METHODS:
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, which comprises references identified from comprehensive electronic database searches and the Internet, handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search of the Group's Haemoglobinopathies Trials Register: 17 February 2015.
SELECTION CRITERIA:
All randomised or quasi-randomised controlled trials comparing psychological interventions with no (psychological) intervention in people with sickle cell disease.
DATA COLLECTION AND ANALYSIS:
Both authors independently extracted data and assessed the risk of bias of the included studies.
MAIN RESULTS:
Twelve studies were identified in the searches and seven of these were eligible for inclusion in the review. Five studies, involving 260 participants, provided data for analysis. One study showed that cognitive behaviour therapy significantly reduced the affective component of pain (feelings about pain), mean difference -0.99 (95% confidence interval -1.62 to -0.36), but not the sensory component (pain intensity), mean difference 0.00 (95% confidence interval -9.39 to 9.39). One study of family psycho-education was not associated with a reduction in depression. Another study evaluating cognitive behavioural therapy had inconclusive results for the assessment of coping strategies, and showed no difference between groups assessed on health service utilisation. In addition, family home-based cognitive behavioural therapy did not show any difference compared to disease education. One study of patient education on health beliefs showed a significant improvement in attitudes towards health workers, mean difference -4.39 (95% CI -6.45 to -2.33) and medication, mean difference -1.74 (95% CI -2.98 to -0.50). Nonetheless, these results may not apply across all ages, severity of sickle cell disease, types of pain (acute or chronic), or setting.
AUTHORS' CONCLUSIONS:
Evidence for the efficacy of psychological therapies in sickle cell disease is currently limited. This systematic review has clearly identified the need for well-designed, adequately-powered, multicentre randomised controlled trials assessing the effectiveness of specific interventions in sickle cell disease.
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PMID: 25966336 [PubMed - as supplied by publisher]
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2.
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Am J Hematol. 2015 May 11. doi: 10.1002/ajh.24051. [Epub ahead of print]
Abstract
Red blood cell (RBC) alloimmunization is a significant clinical complication of sickle cell disease (SCD). It can lead to difficulty with cross-matching for future transfusions and may sometimes trigger life-threatening delayed hemolytic transfusion reactions. We conducted a retrospective study to explore the association of clinical complications and age of red blood cells (RBC) with alloimmunization in patients with SCD followed at a single institution from 2005-2011. One hundred and sixty six patients with a total of 488 RBC transfusions were evaluated. Nineteen patients (11%) developed new alloantibodies following blood transfusions during the period of review. The median age of RBC units was 20 days (interquartile range: 14-27 days). RBC antibody formation was significantly associated with the age of RBC units (p = 0.002), with a hazard ratio of 3.5 (95% CI: 1.71-7.11) for a RBC unit that was 7 days old and 9.8 (95% CI: 2.66-35.97) for a unit that was 35 days old, 28 days after the blood transfusion. No association was observed between RBC alloimmunization and acute vaso-occlusive complications. Although increased echocardiography-derived tricuspid regurgitant jet velocity (TRV) was associated with the presence of RBC alloantibodies (p = 0.02), TRV was not significantly associated with alloimmunization when adjusted for patient age and number of transfused RBC units. Our study suggests that RBC antibody formation is significantly associated with older age of RBCs at the time of transfusion. Prospective studies in patients with SCD are required to confirm this finding. This article is protected by copyright. All rights reserved.
© 2015 Wiley Periodicals, Inc.
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PMID: 25963831 [PubMed - as supplied by publisher]
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3.
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Br J Haematol. 2015 May 5. doi: 10.1111/bjh.13477. [Epub ahead of print]
Abstract
Sickle cell disease induces specific brain alterations that involve both the macrocirculation and the microcirculation. The main overt neurovascular complications in children are infarctive stroke, transient ischaemic attack and cerebral haemorrhage. Silent cerebral infarction, cognitive dysfunction and recurrent headache are also common. Cerebrovascular disease selectively affects children with the HbSS or HbS-β0genotypes (i.e. sickle cell anaemia). The incidence of stroke peaks between 2 and 5 years of age (1·02/100 patient-years) and increases with the severity of the anaemia. Most strokes can be prevented by annual transcranial Doppler screening from 2 to 16 years of age and providing chronic blood transfusion when this investigation shows elevated blood-flow velocities. The role for hydroxycarbamide in children with abnormal transcranial Doppler findings is under investigation. After a stroke, chronic blood transfusion is very strongly recommended, unless haematopoietic stem cell transplantation can be performed. Routine magnetic resonance imaging shows that more than one-third of children have silent cerebral infarction, which is associated with cognitive impairments. Screening for silent infarcts seems legitimate, since their presence may lead to supportive treatments. The role for more aggressive interventions such as hydroxycarbamide or chronic blood transfusion is debated.
© 2015 John Wiley & Sons Ltd.
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PMID: 25944412 [PubMed - as supplied by publisher]
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4.
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Lancet Haematol. 2014 Dec 1;1(3):e95-e103.
Minniti CP1, Gorbach AM1, Xu D1, Hon YY1, Delaney KM1, Seidel M1, Malik N1, Peters-Lawrence M1, Cantilena C1, Nichols JS1, Mendelsohn L1, Conrey A1, Grimes G1, Kato GJ1.
Abstract
BACKGROUND:
Well-tolerated and effective treatments are needed for chronic leg ulcers in sickle cell anaemia. Topical sodium nitrite, a known nitric oxide donor, enhances blood flow in ulcers and has known bacteriostatic effects. We aimed to assess the safety, tolerability, and pharmacokinetics of topical sodium nitrite in patients with sickle cell disease and chronic leg ulcers.
METHODS:
We enrolled adult patients from an ambulatory clinic at the National Institutes of Health (Bethesda, MD, USA) with sickle cell anaemia with leg ulcers (with a surface area of 2.5-100 cm2) persisting for at least 4 weeks into a safety and tolerability phase 1 dose-escalation trial of topical sodium nitrite. Increasing concentrations of sodium nitrite cream were applied twice weekly for 4 weeks to one ulcer per patient at five dose levels (0.5%, 1%, 1.5%, 1.8%, and 2%). The primary endpoints were safety and tolerability, with secondary endpoints of pharmacokinetics, blood flow, and wound healing. Pain relief was analysed post hoc. Endpoints were analysed over time for the whole study population and according to dose level. This study is registered with ClinicalTrials.gov, number NCT01316796.
FINDINGS:
Between April 4, 2011, and March 19, 2013, we enrolled 18 adult patients with sickle cell anaemia and leg ulcers into our trial. We assigned three patients into each cohort, and each cohort was treated with a different concentration of sodium nitrite cream (cohort 1: 0.5%, cohort 2: 1.0%, cohort 3: 1.5%, and cohort 4: 2.0%). Patients were not enrolled into the next cohort dose until we were able to establish that no dose-limiting toxicities were observed. An additional six patients were enrolled to cohort 3a: 1.8%, after two patients in cohort 4 had asymptomatic drops in diastolic blood pressure. No grade 3-4 adverse events were observed, and there were no serious adverse events or dose-limiting side-effects. Pharmacokinetic analysis showed that systemic absorption of sodium nitrite was very low. Application of topical sodium nitrite was associated with a significant increase in peri-wound cutaneous blood flow measured by laser speckle contrast imaging (p=0.0002), corroborated by increased peri-wound skin temperature by infrared thermography (p=0.0119). We recorded a dose-dependent decrease in leg ulcer size (p=0.0012) and pain (p<0.0001). Ulcers healed completely in three patients who received the highest concentrations of topical sodium nitrite (the 1.8% and 2% cream). In our post-hoc analysis of pain, brief pain inventory scores improved in pain severity (p=0.0048) and pain interference (p=0.0013).
INTERPRETATION:
Our results indicate that topical sodium nitrite 2% cream is suitable for additional clinical trials in adults with sickle cell anaemia to promote healing of leg ulcers.
FUNDING:
National Heart, Lung and Blood Institute Division of Intramural Research (National Institutes of Health).
PMCID: PMC4415859 Free PMC Article
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PMID: 25938131 [PubMed]
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5.
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Pediatr Blood Cancer. 2015 Apr 30. doi: 10.1002/pbc.25563. [Epub ahead of print]
Author information:
1Department of Community Health and Psychiatry, University of the West Indies, Mona, Kingston, Jamaica.
Abstract
We undertook a cost effectiveness analysis (CEA) of hydroxyurea (HU) in preventing stroke recurrence and/or death. We followed 43 children with sickle cell disease from 2000 to 2009 after having a first clinical stroke, of whom 10 opted for HU therapy. HU use led to decreased stroke recurrence and death without significantly increasing the annual cost of care per patient (J$83,250 vs. J$76,901, P = 0.491). The incremental cost effectiveness ratio (ICER) for prevention of stroke recurrence amounted to J$169,238 (US$1,900), while that for death prevention equalled J$635,843 (US$7,140). HU may be recommended when safe and affordable transfusion therapy is not feasible. Pediatr Blood Cancer © 2015 Wiley Periodicals, Inc.
© 2015 Wiley Periodicals, Inc.
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PMID: 25929458 [PubMed - as supplied by publisher]
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6.
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J Pediatr. 2015 May;166(5):1226-32. doi: 10.1016/j.jpeds.2015.01.054.
Abstract
OBJECTIVE:
To assess the rates and types of complications associated with deep sedation in children with sickle cell disease (SCD) and to explore potential risk factors.
STUDY DESIGN:
This was a retrospective cohort study of children with SCD and a comparison group of children without SCD who underwent magnetic resonance imaging with deep sedation. The rates of general and SCD-associated sedation complications were calculated, and potential associated clinical and laboratory variables were assessed.
RESULTS:
A total of 162 sedation records in 94 subjects with SCD and 324 sedation records in 321 subjects without SCD were assessed (mean age, 4.3 years in both groups). Pentobarbital, fentanyl, and midazolam were used in the majority of sedation episodes without routine presedation transfusion. Sedation-related complication rates did not differ significantly between the SCD and comparison groups. Within 1 month after the sedation procedure, 17 children (10%) experienced a vaso-occlusive pain episode (VOE), and 2 children (1.2%) developed acute chest syndrome. Preprocedure and postprocedure rates of these complications did not differ significantly. Subjects who developed VOE after sedation had a significantly higher VOE rate before sedation, but no other significant clinical or laboratory risk factors were identified.
CONCLUSION:
Deep sedation in young children with SCD using a standard protocol is safe, with a sedation-related complication rate comparable to that of the general pediatric population. The observed rate of VOE, although not significantly higher than expected, warrants further investigation.
Copyright © 2015 Elsevier Inc. All rights reserved.
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PMID: 25919732 [PubMed - in process]
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7.
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J Adv Nurs. 2015 Apr 27. doi: 10.1111/jan.12678. [Epub ahead of print]
Abstract
AIMS:
To examine the relationship between pain and satisfaction in patients with sickle cell disease.
BACKGROUND:
Frequency and severity of unrelieved sickle cell pain are positively associated with mortality. Yet, information is scarce on whether sickle cell patients are satisfied with their pain level.
DESIGN:
A cross-sectional, correlational analysis of baseline data from a randomized clinical trial.
METHODS:
A randomized sample of adult outpatients was recruited between February 2007-March 2011. Patients completed the PAINReportIt® , containing measures of pain, satisfaction and socio-demographics. We analysed data using Kendall's rank correlations, analysis of variance, Tukey-Kramer post hoc tests, Fisher's tests and proportional odds logistic regression.
RESULTS:
There were statistically significant correlations between pain outcomes and satisfaction with pain level, but average pain intensity more strongly discriminated groups based on satisfaction with pain level. Among pain variables bivariately associated with patient satisfaction with pain level, only pain expectation maintained its significant relationship with satisfaction with pain level when average pain intensity was controlled. A smaller percentage of our sickle cell patients reported moderate to severe pain intensity (28%) or high composite pain index (39%), while reporting being satisfied with pain their level than reported in earlier studies using different measures and populations (70-94%).
CONCLUSION:
Satisfaction with pain level was an unambiguous measure of patient satisfaction and a promising indicator of pain that did not show the paradoxical relationship between satisfaction and pain seen with past measures.
© 2015 John Wiley & Sons Ltd.
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PMID: 25916256 [PubMed - as supplied by publisher]
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8.
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Transfusion. 2015 Apr 23. doi: 10.1111/trf.13134. [Epub ahead of print]
Author information:
1Department of Physics.
Abstract
BACKGROUND:
Sickle cell disease (SCD) is characterized by hemoglobin polymerization upon deoxygenation. Polymerization causes the sickle cells to become rigid and misshapen (sickling). Red blood cell (RBC) dehydration greatly increases polymerization. Cycles of sickling and unsickling cause an influx of calcium that leads to loss of potassium via the calcium-activated Gardos channel, which dehydrates the cells leading to increased polymerization. In this study the effects of nitric oxide (NO) and its congeners on RBC deformability were examined, focusing on sickle RBCs (sRBCs).
STUDY DESIGN AND METHODS:
RBCs from patients with SCD and from nonpatients were exposed to various compounds that release NO or its congeners. Intracellular calcium was increased using a calcium ionophore or cycling of oxygen tension for sRBCs. Deformability was measured by laser-assisted osmotic gradient ektacytometry.
RESULTS:
Consistent with a previous report, sodium nitroprusside (SNP) was found to protect against calcium-induced loss of deformability in normal RBCs, but (contrary to some previous reports) no effect of any NO donors was observed when calcium influx was not induced. Importantly, in studies of deoxygenation-induced dehydration of sRBCs, SNP resulted in substantial improvements in deformability (p = 0.036) and hydration (p = 0.024). Sodium nitrite showed similar trends. SNP was shown to have no effect on calcium influx, but reduced potassium efflux.
CONCLUSION:
These data suggest that SNP and perhaps certain nitrogen oxides (like nitrite) inhibit the Gardos channel and may be able to protect sickle cells from dehydration and thereby improve outcome in the disease.
© 2015 AABB.
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PMID: 25912054 [PubMed - as supplied by publisher]
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9.
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J Emerg Med. 2015 Apr 21. pii: S0736-4679(15)00053-0. doi: 10.1016/j.jemermed.2014.12.080. [Epub ahead of print]
Abstract
BACKGROUND:
Emergency Department Reliance (EDR: total emergency department [ED] visits/total ambulatory [outpatient + ED] visits) differentiates acute episodic ED users from those who may not have adequate access to outpatient care.
OBJECTIVE:
This study's aim was to investigate age-related patterns of EDR and associated health-care costs in pediatric patients with sickle cell disease (SCD) and those transitioning from pediatric to adult care.
METHODS:
State Medicaid data were used for this study. Patients with two or more SCD diagnoses and one or more blood transfusion were included. Quarterly rates of ED visits, EDR, SCD complications associated with ED visits, and ED visits resulting in hospitalization were evaluated. Risk factors associated with high EDR and the association between high EDR and health-care costs were explored through regression analyses.
RESULTS:
A total of 3208 patients were included. The most common SCD complications associated with ED visits were pain, infection, and pneumonia. Beginning at the age of 15 years, EDR rose from 0.17 to 0.29 visits per quarter at age 22 years, and remained high throughout adulthood. Regression analyses indicated that patients were most likely to have high EDR during the post-transition period and when experiencing an SCD complication. Patients with high EDR incurred statistically significantly higher inpatient and ED costs, resulting in significantly higher total health-care costs.
CONCLUSIONS:
Compared to children, patients transitioning to adulthood relied more on the ED for their care. In addition, patients with high EDR incurred more days in the hospital and significantly higher health-care costs, highlighting the need to improve transition-related support, including better access to primary care and increased engagement with patients with SCD.
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Am J Hematol. 2015 May;90(5):376-80. doi: 10.1002/ajh.23961. Epub 2015 Feb 25.
Abstract
Most adults with sickle cell disease (SCD) receive care for their acute painful episodes in an emergency department (ED) setting. The purpose of this article is to describe the impact of opening a dedicated treatment center for adults with SCD [Sickle Cell Infusion Clinic (SCIC)] on patient outcomes and on hospital discharges for SCD. Descriptive data including demographics, time to first dose of narcotic, and pain scores were collected on patients presenting to the SCIC and ED. Maryland hospital discharge data were obtained from the Maryland Health Services Cost Review Commission. Analyses were conducted using T tests, χ(2) tests, and simple generalized estimating equation regression models accounting for the clustered nature of observations, as appropriate. There were 3,874 visits to the SCIC by 361 unique patients; 85% of those visits resulted in the patient being sent home. During the same time period, there were 3,408 visits to the ED by 558 unique patients with SCD. The overall admission rate from the ED for these patients was 35.9% but decreased significantly over the time period with a rate of 20% in December 2011. There was a significant decrease in readmissions over time for the entire Baltimore Metro area with the likelihood of readmission decreasing by 7% over time. The SCIC model provides adults with SCD access to high quality care that decreases the need for hospital admission. Further research needs to be done to evaluate the cost effectiveness of this model. Am. J. Hematol. 90:376-380, 2015. © 2015 Wiley Periodicals, Inc.
© 2015 Wiley Periodicals, Inc.
PMCID: PMC4409504 [Available on 2016-05-01]
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PMID: 25639822 [PubMed - in process]
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11.
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Blood. 2015 Mar 31. pii: blood-2014-07-589283. [Epub ahead of print]
Camus SM1, De Moraes JA1, Bonnin P2, Abbyad P3, Le Jeune S4, Lionnet F5, Loufrani L6, Grimaud L6, Lambry JC3, Charue D1, Kiger L7, Renard JM1, Larroque C8, Le Clésiau H8, Tedgui A1, Bruneval P1, Barja-Fidalgo C9, Alexandrou A3, Tharaux PL1, Boulanger CM1, Blanc-Brude OP10.
Abstract
Intravascular hemolysis describes the relocalization of heme and hemoglobin from erythrocytes to plasma. We investigated the concept that erythrocyte membrane microparticles (MP) concentrate cell-free heme in human hemolytic diseases, and that heme-laden MP have a physiopathological impact. Up to one third of cell-free heme in plasma from 47 patients with sickle cell disease (SCD) was sequestered in circulating MP. Erythrocyte vesiculation in vitro produced MP loaded with heme. In silico analysis predicted that externalized phosphatidylserine in MP may associate with and help retain heme at the cell surface. Immunohistology identified hemoglobin-laden MP adherent to capillary endothelium in kidney biopsies from hyperalbuminuric SCD patients. In addition, heme-laden erythrocyte MP adhered and transferred heme to cultured endothelial cells, inducing oxidative stress and apoptosis. In transgenic SAD mice, infusion of heme-laden MP triggered rapid vaso-occlusions in kidneys, and compromised microvascular dilation ex vivo. These vascular effects were largely blocked by heme-scavenging hemopexin and by the phosphatidylserine antagonist annexin-a5, in vitro and in vivo. Adversely remodeled MP carrying heme may thus be a source of oxidant stress for the endothelium, linking hemolysis to vascular injury. This pathway might provide new targets for the therapeutic preservation of vascular function in SCD.
Copyright © 2015 American Society of Hematology.
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PMID: 25827830 [PubMed - as supplied by publisher]
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12.
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Am J Hematol. 2015 May;90(5):381-5. doi: 10.1002/ajh.23956. Epub 2015 Apr 1.
Manwani D1, Chen G, Carullo V, Serban S, Olowokure O, Jang J, Huggins M, Cohen HW, Billett H, Atweh GF, Frenette PS, Shi PA.
Abstract
Intravenous immunoglobulin (IVIG) decreases neutrophil adhesion to endothelium and red blood cell-neutrophil interactions in sickle cell mice undergoing vaso-occlusion. In this Phase I clinical trial of sickle cell anemia (SCA) patients admitted with pain crisis, we evaluated the status of adhesion molecules on neutrophils in control and IVIG-treated subjects pre- and post-infusion up to 800 mg/kg, the same dose used in murine studies. Mac-1 function significantly decreased from baseline in the low-dose IVIG (200-400 mg/kg) cohorts. IVIG-related adverse events may have occurred in the high-dose (600-800 mg/kg) cohorts. There were no significant increases in neutrophil and leukocyte counts, suggesting that IVIG may more selectively inhibit Mac-1 function as opposed to neutrophil adhesion. This study provides the first in-human validation of pre-clinical murine studies that IVIG can decrease Mac-1 function. Am. J. Hematol. 90:381-385, 2015. © 2015 Wiley Periodicals, Inc.
© 2015 Wiley Periodicals, Inc.
PMCID: PMC4409477 [Available on 2016-05-01]
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PMID: 25616042 [PubMed - in process]
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13.
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Am J Med. 2015 May;128(5):541-4. doi: 10.1016/j.amjmed.2014.11.020. Epub 2014 Dec 9.
Abstract
BACKGROUND:
The American Pain Society recommends that individuals experiencing sickle cell crisis receive parenteral pain medication within 30 minutes of assessment. We examined factors affecting achievement of this standard at the Johns Hopkins Sickle Cell Infusion Center.
METHODS:
Baseline patient care time intervals and data on variables affecting the ability to achieve the American Pain Society goal were measured. Time to first parenteral opiate administration was modeled using simple and multivariable linear regression.
RESULTS:
Mean time from initial assessment to first dose was initially 41 minutes. Increased nurse to patient ratio decreased time to first dose.
CONCLUSIONS:
Of the factors associated with improved times to first dose, only nurse to patient ratio is amenable to process change, suggesting it as a potential target for future interventions.
Copyright © 2015 Elsevier Inc. All rights reserved.
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PMID: 25498167 [PubMed - in process]
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J Pediatr Health Care. 2015 Mar 12. pii: S0891-5245(15)00056-5. doi: 10.1016/j.pedhc.2015.01.012. [Epub ahead of print]
The Daily Experiences of Adolescents in Lebanon With Sickle Cell Disease.
Abstract
OBJECTIVES: Despite the psychosocial and physical consequences associated with sickle cell disease (SCD), the daily lived experience of adolescents diagnosed with this disease is a phenomenon rarely described. The objective of this study was to explore the daily lived experience of adolescents with SCD living in Lebanon.
METHOD: Twelve adolescents with SCD between the ages of 12 and 17 years were interviewed with use of a semi-structured interview during a routine follow-up visit after they were assessed as being pain free. Interviews were transcribed verbatim, and thematic analysis was conducted.
RESULTS: Adolescents with SCD experience a layered burden consisting of physical, emotional, and sympathetic pain that affects much of their daily personal and social lives. Nevertheless, they seem to claim normalcy and to downplay their pain and suffering in order to limit their caregivers' distress.
CONCLUSION: These findings can be used to assist health care providers in designing culturally sensitive interventions specifically designed for adolescents with SCD and their families to enable them to better cope with their illness.
J Pediatr Health Care. 2015 Jan-Feb;29(1):54-60. doi: 10.1016/j.pedhc.2014.06.007. Epub 2014 Aug 10.
Educational intervention to improve the health outcomes of children with sickle cell disease.
Abstract
INTRODUCTION: Although sickle cell disease (SCD) is the most common single gene disorder worldwide, caregivers of children do not have adequate knowledge about the illness and its management. The purpose of this study was to assess the efficacy of education along with tailored written materials in changing the behaviors of caregivers to help them provide better care for children with SCD.
METHODS: A preintervention and postintervention quasi-experimental design was used. A convenience sample of 43 caregivers of 57 children were asked to complete a questionnaire related to their knowledge of SCD before and after educational sessions. The educational sessions (the intervention) were provided to caregivers at the Children's Cancer Center in Lebanon by one registered nurse, one certified pediatric nurse practitioner, and one pediatric hematologist. Emergency department (ED) visits and hospitalizations were compared 2 months before and 2 months after the intervention.
RESULTS: A statistically significant increase was found in the knowledge of caregivers about the cause, symptoms, and management of the disease. A statistically significant decrease occurred in the number of hospitalizations before and after the intervention but not in the number of visits to the ED. Multiple regression analysis found that none of the background variables were related to knowledge, ED visits, or hospitalizations.
CLINICAL IMPLICATIONS: Education and written materials written in a simple language that is understood by 5th-graders were beneficial in improving the knowledge of caregivers and in decreasing the number of hospitalizations of children with SCD.
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