Pulmonary function studies in Kuwaiti children with sickle cell disease and elevated Hb F

Conflicting ventilatory defects have been reported in children with sickle cell disease (SCD). In Kuwait, the disease is relatively mild with a low incidence of acute chest syndrome and other complications, presumably due to the Arab-Indian haplotype chromosomal background and elevated Hb F levels. There have been no previous studies of pulmonary function in patients with this haplotype. Pulmonary function test (PFT) was carried out on 28 steady state children with SCD (21 homozygous sickle cell (SS), seven S beta(o) thal) and two group of controls: 17 age- and sex-matched healthy children and 10 children with HbH disease. The charts of the SCD patients were reviewed for frequency of acute chest syndrome and vaso-occlusive crisis. The mean values of forced vital capacity (FVC) (83.2 +/- 11.9 vs. 91.2 +/- 11.7) and vital capacity (VC) (81.5 +/- 11.8 vs. 90.5 +/- 10.9) were significantly lower in the SS patients compared with healthy controls (p < 0.05). Similarly, these values were significantly lower than in those of the HbH group (p < 0.001 for VC and p < 0.01 for FVC). The mean forced expiratory volume in 1 s (FEV1) was lower in SS patients (86.4 +/- 11.5) compared with healthy controls (94.2 +/- 14.2), but the difference was not significant (p = 0.07). Also, the FEV1 was significantly lower in SS patients than in the HbH group (p < 0.001). There was no significant difference in the PFT parameters between SS patients with acute chest syndrome and those without. Although patients with frequent vaso-occlusive crisis had lower PFT parameters, the differences were not significant in comparison to those with infrequent crisis. This study revealed an early restrictive and obstructive pulmonary function pattern in steady state children with SCD. The finding also indicates that the changes of PFT parameters in SS patients could not be attributed to anaemia per se as patients with HbH who also have chronic anaemia did not show similar changes. This observation underscores the early occurrence of pulmonary involvement, even in patients with an otherwise relatively mild SCD.     

Barriers to preventive health care for young children

PURPOSE: To review the literature on barriers to availability, access, and utilization of preventive health care for young children three to five years of age and their families and to discuss the role of nurse practitioners (NPs) in future research, education, and practice in this area. DATA SOURCES: A comprehensive literature search was conducted of online material and CINAHL and Medline (CD-ROM 1990 to present). In addition, experts in this area were asked to recommend extra reading materials. Additional references in textbooks and articles were examined. CONCLUSIONS: The literature review supports that there are major barriers to be addressed in the areas of availability, access, and utilization of preventive health care services for young children and their families. Major concerns include mandatory system for preventative health care, lack of health insurance coverage, cultural issues, and parental effects. IMPLICATIONS FOR PRACTICE: Health professionals in the community will need to work together to reevaluate current preventive health care practices for young children. Alternative methods for approaching and providing preventive health care services may become increasingly important if these services for young children are to be provided at current or increased levels.     

Barriers to evidence-based practice in primary care

Background.  Evidence-based practice is one of the most important underlying principles in modern health care. In the United Kingdom, successive governments have highlighted the fact that a quality health service is built upon the use of best evidence. Health professionals are becoming more accountable within clinical governance structures for the care they provide. The need to use robust research findings effectively is a critical component of their role. However, studies show that a number of barriers prevent the effective use of best available evidence.
Aim.  This study aimed to identify barriers to evidence-based practice in primary care.
Method.  A specially designed questionnaire was used to gather respondents' perceptions of the barriers to evidence-based practice. Data were collected in 2000/2001.
Findings.  Findings show that general practitioners (GPs) ranked barriers differently to community nurses. GPs believed that the most significant barriers to using evidence in practice were: the limited relevance of research to practice, keeping up with all the current changes in primary care, and the ability to search for evidence-based information. In contrast, the most significant barriers to the identified by community nurses were poor computer facilities, poor patient compliance and difficulties in influencing changes within primary care. This suggests that these two groups may require different strategies for barrier removal.
Conclusions.  Identifying barriers is just the first step to addressing issues surrounding the use of evidence-based practice. Extra resources will be needed if these barriers are to be tackled. However, if the resultant change improves the health and wellbeing of people and communities, then the extra costs would be offset by more efficient use of services.     

Developing nursing care guidelines for children with Hodgkin''s disease

Oncology patients are generally treated on therapeutic research protocols that detail medical treatment. Nursing care is not clearly defined in these protocols; therefore, the need to develop a set of guidelines specifically for nursing care was identified. To further enhance the specialized care that our pediatric oncology patients receive, we developed nursing care guidelines to accompany specific protocols. One of our most highly accruing protocols is designed to treat patients diagnosed with Hodgkin's disease. In an effort to increase understanding of this clinical trial, decrease potential for errors, and improve overall quality of patient care, nursing care guidelines were created. To develop the nursing care guidelines, nurses created a seven-step process: (1) studied the therapeutic protocol, established ongoing interactions with the principal investigator (brainstorming), reviewed benchmarking, (2) created the first draft of the guidelines, compared the formatting of this particular set of guidelines with those previously developed at this setting (drafting), (3) field tested guidelines, (4) revised the guidelines and subjected them to additional field testing, (5) examined the guidelines for implications related to teaching tools, (6) implemented the guidelines through in-services, and (7) developed an evaluation plan with pre- and post-tests that indicated improved disease and treatment knowledge among participating nurses. Potential contributions from implementing nursing care guidelines that parallel therapeutic protocols include more accurate and complete research data collection and a more defined role for nurses in the protocol development process. The guidelines also offer a useful, detailed resource to deliver complex protocol-directed care.     

Cerebral oximetry in patients with sickle cell disease

BACKGROUND: There is limited information concerning the brain's oxygen supply and demand in patients with sickle cell disease. DESIGN: We measured near-infrared spectroscopy of brain oxygenation in 27 patients with sickle cell disease regardless of vaso-occlusive crisis, 14 normal healthy controls, and five anaemic patients without sickle cell disease. We also measured pre- and post-transfusion cerebral oximetry in 14 additional sickle cell disease patients who were on transfusion programmes. RESULTS: The mean cerebral oxygen saturation in the combined steady-state and vaso-occlusive crisis population was found to be significantly lower than that in the controls and in anaemic patients without sickle cell disease (47.7% vs. 61.3%, 59.8%, P < 0.0001). Cerebral oxygen saturation failed to correlate with the haemoglobin concentration (r = 0.51, P > 0.5). However, cerebral oxygen saturation increased from 40.4% to 49.6% (P = 0.01) and correlated significantly with the haemoglobin level (r = 0.553, P = 0.003) in 14 subjects studied before and after transfusions. In seven subjects who received simple transfusions, cerebral oxygen saturation correlated strongly and positively with the haemoglobin level (r = 0.811, P = 0.001) and with percent normal haemoglobin (r = 0.786, P = 0.002), and negatively with abnormal sickle haemoglobin (r = -0.775, P = 0.003). None of these correlations was found to be statistically significant in the seven subjects given exchange transfusions. Cerebral oxygen saturation measured in the sickle cell disease subjects after transfusions was still significantly lower than in the anaemic subjects without sickle cell disease and in the normal controls (49.6% vs. 59.8% and 61.3%, P = 0.001). CONCLUSIONS: We found that patients with sickle cell disease have subnormal values of cerebral oxygen saturation. Red cell transfusions significantly increased the brain oxygenation in these patients. Cerebral oximetry may be a useful, noninvasive method for assessing the effect of circulating normal red cells in sickle cell patients after transfusions.     

Use of a dual lumen port for automated red cell exchange in adults with sickle cell disease

Red cell exchange (RCE) is a common procedure in adults with sickle cell disease (SCD). Implantable dual lumen Vortex (DLV) ports can be used for RCE in patients with poor peripheral venous access. We performed a retrospective cohort study of RCE procedures performed in adults with SCD. The main objective of the study was to compare the inlet speed, duration of procedures and rate of complications performed through DLV ports to those performed through temporary central venous and peripheral catheters. Twenty‐nine adults with SCD underwent a total of 318 RCE procedures. Twenty adults had DLV ports placed and 218 procedures were performed using DLV ports. Mean length of follow‐up after DLV port placement was 397 ± 263 days. Six DLV ports were removed due to infection and 1 for malfunction after a mean of 171 ± 120 days. Compared to temporary central venous and peripheral catheters, DLV port procedures had a greater rate of procedural complications, a longer duration, and a lower inlet speed (all P < 0.01). When accounting for the maximum allowable inlet speed to avoid citrate toxicity, 40% of DLV port procedures were greater than 10% below maximum speed, compared to 7 and 14% of procedures performed through temporary central venous and peripheral catheters (P < 0.0001). In conclusion, DLV ports can be used for RCE in adults with SCD, albeit with more procedural complications and longer duration. The smaller internal diameter and longer catheter of DLV ports compared to temporary central venous catheters likely accounts for the differences noted. J. Clin. Apheresis 30:353–358, 2015. © 2015 Wiley Periodicals, Inc.     

Blood bank issues associated with red cell exchanges in sickle cell disease

Sickle cell disease (SCD) patients are prone to develop complications that include stroke, acute chest syndrome, and other crises. Some of these complications require chronic transfusion therapy or red cell exchange (RCE), either for therapeutic or prophylactic reasons. Due to a discrepancy of red cell antigens between African Americans and Caucasians (majority blood donors), the incidence of alloantibody formation is very high, which makes it difficult to find compatible red cell units, especially for urgent RCE. Some of the above conditions require immediate oxygen delivery to the tissues. Thus, SCD patients undergoing RCE should receive red blood cells with special attributes that include matching for Rh and Kell blood group antigens; RBCs should be fresh in order to provide (1) immediate oxygen delivery and (2) longer surviving cells to reduce the interval between RCE. Also, these units should be pre-storage leukoreduced to prevent febrile non-hemolytic reactions and screened for sickle cell traits to avoid transfusing red cells containing HbS. This requires a concerted effort between the apheresis unit, the local blood bank, and the central blood supplier.     

A pseudoaneurysm within a subperiosteal collection in a patient with sickle cell disease

Sickle cell disease involves long bones in the form of infection or subperiosteal collections. Rare pseudoaneurysm/aneurysm formation is also known to occur in the intracranial and visceral territories. We report a small subperiosteal pseudoaneurysm that developed within a subperiosteal abscess in the tibia in a patient with sickle cell disease. This case adds to the known spectrum of musculoskeletal abnormalities resulting from this condition. © 2009 Wiley Periodicals, Inc. J Clin Ultrasound, 2010     

Communication skills and cultural awareness courses for healthcare professionals who care for patients with sickle cell disease

AIMS: This paper reports a project evaluating the efficacy and impact of a pilot communication skills and cultural awareness course for healthcare professionals who care for patients with sickle cell disease. BACKGROUND: Poor communication between patients with sickle cell disease and healthcare professionals causes suspicion and mistrust. Many patients feel that they are negatively labelled by the healthcare system and are sceptical of opening themselves to an unsympathetic system. They may therefore appear hostile and aggressive when interacting with healthcare professionals, which in turn leads to distortions and misunderstandings between both groups. The use of good communication skills by healthcare professionals is therefore vital for good healthcare practice. METHODS: Forty-seven healthcare professionals took part in a series of three pilot courses each lasting 3 days. Healthcare professionals were taught a repertoire of communication skills and cultural awareness strategies to use in challenging situations that arise in their care of sickle cell patients. Expert facilitators used a variety of teaching techniques, such as professionally-made videos, role-play, and group exercises. Participants' confidence in dealing with challenging situations was assessed at baseline, immediately after the intervention, and at 3- and 6-month postintervention. FINDINGS: A repeated measures anova revealed a statistically significant increase in confidence from pre- to postcourse scores. Confidence scores further increased from immediately postcourse and 3 months postcourse follow-up. These were then maintained at 6 months postcourse. CONCLUSION: The overall findings of this local study demonstrated that this type of communication skills and cultural awareness training had a positive and enduring impact on professionals' perceived ability and confidence in communicating with patients with sickle cell disease. Participants attributed this to the learner-centred approach of the course that provided them with the opportunity to transfer and apply the taught skills in their daily practice. This type of training might be helpful in reducing mistrust and increasing empathetic responses in healthcare professional.     

Prothrombotic aspects of sickle cell disease

Sickle cell disease (SCD) is a hematologic disorder caused by a well‐characterized point mutation in the β‐globin gene. Abnormal polymerization of hemoglobin tetramers results in the formation of sickle red blood cells that leads to vascular occlusions, hemolytic anemia, vascular inflammation and cumulative, multiple organ damage. Ongoing activation of coagulation is another hallmark of SCD. Recent studies strongly suggested that hypercoagulation in SCD is not just a secondary event but contributes directly to the disease pathophysiology. In this article we summarize mechanisms leading to the activation of coagulation, review data indicating direct contribution of coagulation to the pathology of SCD and, we discuss the anticoagulation as a possible treatment strategy to attenuate the disease progression