Joana Duran

Aims: The aims of this study were: to (1) identify barriers to care in children with sickle cell disease; (2) examine the quality of primary care services received by these children and (3) examine the relationship between barriers to care and quality of primary care services in children with sickle cell disease.Background: Effective management in children with sickle cell disease requires early access to a comprehensive range of preventive screenings, urgent care treatments for vaso‐occlusive pain crisis and ongoing prophylactic treatments.Design: A cross‐sectional survey of parents of children with sickle cell disease was conducted between April–September 2011.Methods: Parents of children with sickle cell disease completed the Barriers to Care Questionnaire and Parent's Perceptions of Primary Care.Results: Parents of children with sickle cell disease (n = 38) reported health system barriers such as inability to contact doctors or clinics, extended wait times and inconvenient clinic hours. Some barriers were reported more frequently among children with concurrent sickle cell disease and asthma, compared with those children without a concurrent asthma condition. Parents who reported more barriers were least likely to perceive their care as accessible, comprehensive and coordinated.Conclusions: Minimizing healthcare barriers may improve the quality of primary care services received by children with sickle cell disease and consequently prevent complications associated with sickle cell disease.Implications for nursing practice: Nurses and other care providers need to identify healthcare barriers, so that access, coordination, comprehensiveness and overall quality of primary care services may be improved in children with sickle cell disease.

Transfusion-related reactions cause unwanted interruptions in blood-product administration and potential complications for patients. The most common reactions are febrile nonhemolytic transfusion reactions (FNHTRs) and allergic transfusion reactions (ATRs). The presence of leukocytes in blood products has been associated with these reactions, and efficacy of leukoreduction in minimizing FNHTRs and ATRs has recently been investigated. In addition, premedication with acetaminophen and diphenhydramine is the most widely used practice in minimizing FNHTRs and ATRs, yet the benefit of this is not supported by research. The aim of this systematic literature review was to evaluate the potential benefits of both of these interventions in minimizing FNHTRs and ATRs and provide recommendations for practice. We found moderate quality evidence with strong recommendations for the practice of leukoreduction in minimizing FNHTRs but not ATRs. We did not find evidence to support the use of premedications in minimizing transfusion-related reactions, and we question the need for this practice in settings where leukoreduction is used.

Purpose: The purpose of this study was to examine (a) symptoms, (b) pain characteristics (intensity, location, quality), (c) pain medications and nonpharmacological strategies used for pain, (d) thoughts and feelings, and (e) healthcare visits. We also examined the relationship between pain and sleep.Data sources: Pain and symptoms were entered on an electronic e‐Diary using a smartphone and were remotely monitored by an advanced practice registered nurse (APRN). Sixty‐seven children and adolescents (10–17 years) reported mild to severe pain at home that did not require healthcare visits. Symptoms reported were (a) general symptoms such as tiredness/fatigue (34.7%), headache (20.8%), yellowing of the eyes (28.4%); (b) respiratory symptoms such as sniffling (32.9%), coughing (19.1%), changes in breathing (10.0%); and (c) musculoskeletal symptoms such as stiffness in joints (15.8%). A significant negative correlation was found between pain and sleep (r = −.387, p = .024). Factors that predict pain included previous history of sickle cell disease (SCD) related events, symptoms, and negative thoughts.Conclusion: Pain and multiple symptoms entered on a web‐based e‐Diary were remotely monitored by an APRN and prompted communications, further evaluation, and recommendations.Implications for practice: Remote monitoring using wireless technology may facilitate timely management of pain and symptoms and minimize negative consequences in SCD.

We examined the usability of smartphones for accessing a web-based e-Diary for self-monitoring symptoms in children and adolescents with sickle cell disease (SCD). One group of participants (n=10; mean age, 13.1±2.4 y; 5 M; 5 F) responded to questions using precompleted paper-based measures. A second group (n=21; mean age, 13.4±2.4 y; 10 M; 11 F) responded based on pain and symptoms they experienced over the previous 12 hours. The e-Diary was completed with at least 80% accuracy when compared to paper-based measures. Symptoms experienced over the previous 12 hours included feeling tired (33.3%), headache (28.6%), coughing (23.8%), lack of energy/fatigue (19.0%), yellowing of the eyes (19.0%), pallor (19.0%), irritability (19.0%), stiffness in joints (19.0%), general weakness (14.3%), and pain (14.3%), rating on average as 2.0±1.7 (on 0 to 10 scale). Overall, sleep was good (8.1±1.4 on the 0 to 10 scale). In conclusion, children with SCD were able to use smartphones to access a web-based e-Diary for reporting pain and symptoms. Smartphones may improve self-reporting of symptoms and communication between patients and their health care providers, who may consequently be able to improve pain and symptom management in children and adolescents with SCD in a timely manner.