A Survey of Pneumococcal Prophylaxis Practices among Sickle Cell Providers

Research Article

J Pediatr & Child Health Care. 2016; 1(1): 1005.

A Survey of Pneumococcal Prophylaxis Practices among Sickle Cell Providers

Clay ELJ1,2, Hsu G³, Jordan L4, Xu H5 and Pace BS1*

1Department of Pediatrics, Augusta University, Augusta, GA, USA

2Department of Emergency Medicine, Augusta University, Augusta, GA, USA

3Department of Neonatology, Hospital of Ancona, Italy

4University of Miami, Miami, FL, USA

5Department of Biostatistics and Epidemiology, Augusta University, Augusta, GA, USA Co-first authors: Clay ELJ and Hsu G

*Corresponding author: Pace BS, Department of Pediatrics, Augusta University, Augusta, GA, USA

Received: June 07, 2016; Accepted: June 19, 2016; Published: June 21, 2016


Aim: To identify the most common antibiotics used for pneumococcal prophylaxis among pediatric hematologists caring for individuals with Sickle Cell Disease (SCD).

Methods: A questionnaire was distributed to pediatric hematologists attending the 8th Annual Foundation for Sickle Cell Disease Research and Educational Symposium in Miami, FL. Information was collected related to provider characteristics, practice site setting, and antibiotics prescribed for pneumococcal prophylaxis in children with SCD. The data was analyzed for timing of initiation and cessation of antibiotic prophylaxis, antibiotic regimen of choice, and practice setting among participants.

Results: Sixty-two practitioners of mixed clinical characteristics (geographic location, monthly patient volumes, and academic versus private practice setting) who care for children with SCD completed the survey. The majority of hematologists practiced in the Midwest or Eastern United States. Of the total participants, 90.3% (56 hematologists) prescribed antibiotic prophylaxis to their patients while 9.7% did not. Of the practitioners who prescribed antibiotics, 80.6% utilized penicillin as the primary treatment for pneumococcal prophylaxis, while 9.7% used amoxicillin and the remaining 11.3% of hematologists used a variety of other antibiotics. The vast majority of infants started antibiotic prophylaxis by 3 months of age and discontinued treatment by 5 years. Furthermore, the impact of practice setting suggest a tendency of private practice hematologists to used amoxicillin as the first-line drug for pneumococcal prophylaxis compared to physicians in academic settings.

Conclusion: This study demonstrates variations in drug treatment practices for pneumococcal prophylaxis among hematologists caring for children with SCD.

Keywords: Sickle Cell Disease; Penicillin; Amoxicillin; Streptococcus pneumoniae; Pneumococcal Prophylaxis


SCD: Sickle Cell Disease; HbS: Hemoglobin S; Streptococcus pneumoniae: S.pneumoniae; US: United States; PROPS-I: Prophylaxis Penicillin Study-I; PCN: Penicillin; Amox: Amoxicillin; yrs: years


Sickle Cell Disease (SCD) is a group of inherited blood disorders produced by mutations in the β-globin gene located on chromosome 11. Various missense mutations involving the 6th codon of the β-globin gene result in abnormal globin chain variants with Hemoglobin S (HbS) being the most common. Individuals affected with homozygous βS-globin mutations (HbSS) or heterozygous for the sickle mutation and β-thalassemia (HbSβ-thalassemia) have a more severe disease phenotype [1,2]. They are at higher risk for developing complications compared to individuals with milder forms of SCD such as HbSC [3,4] and HbSβ+-thalassemia [5].

The hallmark of SCD is HbS polymerization, red blood cell sickling, vaso-occlusion and tissue ischemia. The most common manifestation of vaso-occlusion is severe reoccurring painful episodes. Additionally, patients with SCD have nutritional deficiencies, splenic dysfunction, and increased susceptibility to infection placing them at risk for multi-organ damage and failure [2,6,7]. Since several organ systems are affected by SCD, a multidisciplinary approach to patient care is recommended to produce the greatest impact on morbidity and long-term survival. Recent guidelines for comprehensive care were published in 2014 by the National Heart Lung and Blood Institute “Evidence-Based Management of Sickle Cell Disease” [8]. Advances in treatment options and standardization of health supervision and surveillance practices, have improved the average lifespan with the majority of persons with SCD surviving well into adulthood [9]. To promote further progress in health care delivery, it is imperative that hematologists and general care providers understand the complex nature of SCD and deliver the current standards of care to this population thus decreasing complications and improving quality of life.

Young children with SCD are at high risk for significant morbidity and mortality [1] related to sepsis in the first 3 years of life if not treated aggressively [2]. Sickle cell patients have altered immune responses putting them at risk for invasive infections due to Streptococcus pneumoniae (S.pneumoniae) and other encapsulated bacteria. The common loss of spleen function in the first year of life, deficiencies in complement and immunoglobulin production as well as delays in the clearance of invasive pathogens contribute to increased risk of invasive infections [10-14]. The landmark Prophylaxis Penicillin Study-I (PROPS-I) and Cooperative Study of Sickle Cell Disease [15,16] established the role of early diagnosis to identify babies with SCD and the routine use of prophylactic penicillin in the standard of care for infants with SCD. It was demonstrated an 84% reduction in the incidence of infection and no deaths from pneumococcal septicemia in children treated with penicillin [15]. A follow up study PROPS-II [17] demonstrated no significant difference in the percent of positive cultures for S.pneumoniae in those patients given penicillin prophylaxis after 5 years of age compared with the placebo group. These data suggest no additional benefit of continuing penicillin treatment after 5 years of age.

In the most recent guidelines “Evidence-Based Management of Sickle Cell Disease” the following recommendations were made for standards of care practices

1) Oral penicillin prophylaxis for children with sickle cell anemia (HbSS and HbSβ-thalassemia) until 5 years of age, 2) Vaccination against S.pneumoniae and other encapsulated organisms, and 3) Proper education regarding increased surveillance and early intervention in the event of fever or signs of infection [8]. Based on findings in the PROPS-II study [17], recommendations were given to discontinue penicillin prophylaxis at 5 years of age for children with an intact spleen or no history of invasive S.pneumoniae disease. Recommendations to consider withholding penicillin prophylaxis in patients with HbSC and HbSβ+-thalassemia were also included. Despite the established guidelines and clinical evidence for effectiveness, our survey data suggest up to 9.7% of infants do not receive preventative treatment against infection. Our study illustrates variations in the first-line antibiotic prescribed for S.pneumoniae prophylaxis among hematologists caring for patients with SCD. The implications of these practices and the potential effects of long-term antibiotic prophylaxis will be discussed.

Material and Methods

Survey design - A survey was completed by attendees of the 8th Annual Foundation for Sickle Cell Disease Research and Educational Symposium in 2014. The survey was composed of 10 closed-end questions about the routine care rendered to children with SCD in their practice. The data were collect using Survey Monkey Copyright© 1999-2015. The questions were designed to collect information regarding the provider’s practice location, practice setting, patient volume, and routine practice for S.pneumoniae prophylaxis (Pneumococcal Prophylaxis Practices among Sickle Cell Providers; www.surveymonkey.com). A total of 62 pediatric hematologists caring for patients with SCD of mixed clinical backgrounds responded to the survey.

Statistical analysis

After collection of the raw data, the Fisher’s exact test was conducted to determine correlations between prophylaxis regimen preferences and provider practice settings. The data were summarized as the Mean ± Standard Deviation and p-value <0.05 was considered a statistically significant difference.


Characteristics of survey participants

The purpose of the survey was to identify variations in antibiotic prescribing practices for S.pneumoniae prophylaxis among hematologists caring for children with SCD. The survey was completed by attendees of the 8th Annual Foundation for Sickle Cell Disease Symposium, which is one of the largest gatherings of physicians caring for people with SCD. Out of 501 registered attendees, 62 pediatric hematologists completed the survey. The opportunity for attendees to complete the questionnaire was announced and a link was provided during the meeting. In addition for a limited time after the meeting, registered attendees could complete the survey online. The answers were recorded via Survey Monkey with an average completion time of 5 minutes per participant. The raw data from the surveys were compiled for statistical analysis.

The demographics of pediatric hematologists participating in the survey from several geographic locations in the United States (US) and internationally are shown in (Figure 1). The majority of the participants were from the Midwest and Eastern US regions. The characteristics of the clinical practice settings are summarized in Table 1. The majority of hematologists practiced within the continental US (91.9%) and 8.1% were from international practices. Analysis of the entire cohort revealed a wide variation of monthly patient volumes ranging from <50 patients for 43.5% to providers caring for >150 patients with SCD. These data suggest we captured a reasonable representation of hematologists with significant SCD patient loads in different practice settings.