Sara W. Day

High risk of recurrent stroke after discontinuance of five to twelve years of transfusion therapy in patients with sickle cell disease

Although long-term transfusion therapy is at least 90% effective in preventing recurrent strokes after an initial cerebrovascular accident in patients with sickle cell disease, it is unknown how long transfusion therapy should be continued. To address this question, we prospectively discontinued transfusions in 10 patients with sickle cell disease whose median duration of transfusion therapy after an initial stroke was 9 1/2 years (range 5 to 12 years). Before the transfusions were discontinued, patients were examined by cerebral angiography, magnetic resonance imaging of the head, neuropsychologic testing, electroencephalography, and a complete neurologic examination. Within 12 months after transfusion therapy was stopped, 5 of 10 patients had had an ischemic event. Three events caused relatively mild deficits in the same areas as those originally affected. Two were associated with massive intracranial hemorrhage, including one on the contralateral side of original involvement. An additional patient died suddenly of unknown causes. Of the four remaining patients, three declined to resume transfusion and are relatively well at greater than or equal to 18 months after therapy was stopped. The studies performed before transfusions were stopped were not predictive of recurrent stroke. The risk of recurrent cerebrovascular accident in this group was significantly greater than the estimated risk of 10% in patients who are receiving long-term transfusion therapy (p = 0.002). This adverse outcome suggests that patients with sickle cell disease who have had a stroke must receive long-term transfusion indefinitely or a suitable therapeutic alternative must be devised.

Parental health beliefs and compliance with prophylactic penicillin administration in children with sickle cell disease.

Purpose Prophylactic penicillin is effective in preventing severe invasive pneumococcal infection in children with sickle cell disease. In some families, compliance has been problematic. The aims of this study were to monitor compliance and to assess the efficacy of the Health Belief Model (HBM) in predicting compliance. Methods Fifty mothers of children with sickle cell disease, ages 6 to 60 months, participated in the study. On enrollment, mothers completed surveys assessing their health beliefs regarding sickle cell disease and infections. Compliance was assessed through self-reporting by the mothers and through review of local pharmacy records of penicillin refills. Results Sixty percent of the mothers reported that they were highly compliant with obtaining the prescribed 14-day refills. Pharmacy records indicated that only 12% actually adhered to this schedule. The self-reports were significantly related to compliance ratings; mothers who admitted less than optimal compliance averaged 42 days between refills, compared with 19 days for mothers who reported good compliance. Varying perceptions identified through the HBM accounted for approximately 30% of the variance in compliance rates. The perceived burdens of picking up the refills and remembering to administer the medication were the most significant factors. Conclusions Educational efforts alone are not sufficient to ensure compliance with penicillin prophylaxis. Routinely monitoring compliance through pharmacy records, reviewing parental beliefs about sickle cell disease and infections, and exploring barriers to treatment will promote dialogue about the importance of strict compliance with this relatively simple yet life-saving prophylaxis.

Therapy preference and decision-making among patients with severe sickle cell anemia and their families&

Patients with severe sickle cell anemia (SCA) may benefit from therapeutic intervention with hydroxyurea (HU), chronic red cell transfusion (CT), or stem cell transplantation (SCT). Determination of best treatment is complicated by the tradeoff between each treatments risks and benefits and the lack of data comparing them to determine efficacy. We explored factors that influenced making decisions regarding interventions and examined the relations between treatment preference and health‐related quality of life (HRQOL).

Penicillin- and cephalosporin-resistant strains of Streptococcus pneumoniae causing sepsis and meningitis in children with sickle cell disease☆☆☆★

OBJECTIVE We investigated the possibility that antimicrobial-resistant pneumococci were causing invasive disease in children with sickle-cell disease (SCD). STUDY DESIGN Records of all children with SCD observed at the Mid-South Sickle Cell Center (MSSCC) at LeBonheur Childrens Medical Center were reviewed from January 1990 to June 1994. Children with SCD and pneumococcal sepsis were identified. The Streptococcus pneumoniae isolates from these children were examined for serotype and antimicrobial susceptibilities. Two additional children not observed in the MSSCC had pneumococcal sepsis caused by penicillin-resistant isolates and were also included. RESULTS Antimicrobial susceptibility testing of the six penicillin-resistant isolates revealed that four were resistant to trimethoprim-sulfamethoxazole, two to erythromycin, and one to clindamycin. The two isolates that were resistant to ceftriaxone also were multiply resistant. From the MSSCC, 26 children had pneumococcal sepsis during the 4 1/2-year period studied. Five of these children (19%) died. Four (15%), including one who died, were infected with penicillin-resistant strains. CONCLUSION Pneumococcal sepsis, meningitis, and infections of other foci in children with SCD may be caused by S. pneumoniae that is resistant to one or more antimicrobial agents, including penicillin. The addition of vancomycin to the antibiotics currently used for initial management should be considered in areas where the antibiotic resistance of S. pneumoniae is prevalent.

The Cognitive and Academic Impact Of Sickle Cell Disease

Sickle cell disease (SCD) affects over 30,000 students in the United States. Central nervous system complications are widespread among students with SCD and include stroke, silent cerebral infarction, and cognitive impairment. The effects of these complications may lead to academic failure, limited career options, and for some, total disability. Despite studies describing the significant academic and cognitive impact of sickle cell disease, reports describing interventions are limited. There is a lack of awareness among educators of the academic risks associated with sickle cell disease and a lack of appropriate resource allocation. The school nurse, as community health advocate, will be called upon to bridge the gap among healthcare providers, parents, students, and educators. This article provides a review of both recent and landmark studies describing the cognitive and academic impact of sickle cell disease and discusses the role of the school nurse as an advocate, liaison, and educator.

A sustainable model for pediatric oncology nursing education in low-income countries

Effectiveness of a nurse educator in the pediatric oncology unit in Guatemala was assessed by measuring completion of an education course, chemotherapy and central line competency, continuing education, and cost. All newly hired nurses completed the education course. Of the nurses employed, 86% participated in the chemotherapy course, and 93% achieved competency; 57% participated in the central line course, and 79% achieved competency. The nurses completed a mean of 26 hours continuing education yearly. The annual direct cost of the educator (

Recurrent Streptococcus pneumoniae sepsis in children with sickle cell disease

244/nurse) was markedly less than other models. This is an effective and sustainable means to educate nurses in low‐income countries. Pediatr Blood Cancer 2012; 58: 163–166.

Outpatient therapy with ceftriaxone and oral cefixime for selected febrile children with sickle cell disease

Streptococcus pneumoniae sepsis is the most common invasive infection among patients with sickle cell disease. The risk of a recurrent episode of sepsis and subsequent death in those patients who have had a previous septic event is much higher. Patients with sickle disease who have had pneumococcal sepsis should continue penicillin prophylaxis indefinitely and should not be candidates for out-patient management of febrile episodes.

Baseline standards for paediatric oncology nursing care in low to middle income countries: position statement of the SIOP PODC Nursing Working Group

Purpose: Children with sickle cell disease are at increased risk for bacterial sepsis and, when febrile, are usually hospitalized for intravenous antibiotic therapy pending results of blood cultures. In this study, we prospectively identified a group of febrile patients with sickle cell disease who were at low risk for sepsis and treated them with outpatient therapy. Patients and Methods: Children identified as low risk for sepsis were treated with an initial dose of intravenous ceftriaxone, followed by outpatient therapy with oral cefixime, and were monitored for 14 days after the initial visit. Compliance was assessed by phone calls to parents and by analysis of urine samples. Results: In 107 eligible febrile episodes (80 patients) over a 21-month period, no patient developed sepsis. One child developed bacteremia 3 days after completing the course of cefixime, and one had splenic sequestration on the fourth study day. Both patients did well. Side effects of cefixime were modest, and overall compliance was excellent (∼95%), although urine samples were returned by only 56% of parents. Conclusion: We conclude that outpatient therapy is safe and effective in febrile patients with sickle cell disease who meet the criteria for a low risk of sepsis.

Use of joint commission international standards to evaluate and improve pediatric oncology nursing care in Guatemala

www.thelancet.com/oncology Vol 15 June 2014 681 Antoine Brouquet, Bernard Nordlinger* Department of Surgical Oncology and Digestive Surgery, Hôpital Bicêtre, Assistance Publique–Hôpitaux de Paris, Le KremlinBicêtre, Université Paris-Sud INSERM 986, France (AB); and Department of General Surgery and Surgical Oncology, Hôpital Ambroise Paré, Assistance Publique–Hôpitaux de Paris, 92100 Boulogne-Billancourt, France (BN) bernard.nordlinger@apr.aphp.fr