Acute chest syndrome in children with sickle cell disease: Data from a national AIEOP cohort identify priority areas of intervention in a hub-and-spoke system

: Acute chest syndrome (ACS) is a frequent cause of hospitalization in sickle cell disease (SCD). Despite advances in acute care, many settings still lack knowledge about ACS best practices. After the AIEOP Guidelines were published in 2012, suggesting standardized management in Italy, a retrospective study was performed to assess the diagnostic and therapeutic pathways of ACS in children. From 2013 to 2018, 208 ACS episodes were presented by 122/583 kids in 11 centres. 73 were male, mean age 10.9 years, 85% African, 92% HbSS or Sβ°. In our hub-and-spoke system, a good adherence to Guidelines was documented, but discrepancies between reference centres and general hospitals were noted. Improvement is needed for timely transfer to reference centres, use of incentive spirometry, oxygen therapy and pain management

Organizing national responses for rare blood disorders: the Italian experience with sickle cell disease in childhood

Background Sickle cell disease (SCD) is the most frequent hemoglobinopathy worldwide but remains a rare blood disorder in most western countries. Recommendations for standard of care have been produced in the United States, the United Kingdom and France, where this disease is relatively frequent because of earlier immigration from Africa. These recommendations have changed the clinical course of SCD but can be difficult to apply in other contexts. The Italian Association of Pediatric Hematology Oncology (AIEOP) decided to develop a common national response to the rising number of SCD patients in Italy with the following objectives: 1) to create a national working group focused on pediatric SCD, and 2) to develop tailored guidelines for the management of SCD that could be accessed and practiced by those involved in the care of children with SCD in Italy. Methods Guidelines, adapted to the Italian social context and health system, were developed by 22 pediatric hematologists representing 54 AIEOP centers across Italy. The group met five times for a total of 128 hours in 22 months; documents and opinions were circulated via web. Results Recommendations regarding the prevention and treatment of the most relevant complications of SCD in childhood adapted to the Italian context and health system were produced. For each topic, a pathway of diagnosis and care is detailed, and a selection of health management issues crucial to Italy or different from other countries is described (i.e., use of alternatives for infection prophylaxis because of the lack of oral penicillin in Italy). Conclusions Creating a network of physicians involved in the day-to-day care of children with SCD is feasible in a country where it remains rare. Providing hematologists, primary and secondary care physicians, and caregivers across the country with web-based guidelines for the management of SCD tailored to the Italian context is the first step in building a sustainable response to a rare but emerging childhood blood disorder and in implementing the World Health Organization\u2019s suggestion \u201cto design (and) implement \u2026 comprehensive national integrated programs for the prevention and management of SCD"

Cognitive elaboration of pain in sickle-cell disease: A comparative evaluation in adults and children

The aim of this study was to analyze developmental issues in the experience of pain in patients with Sickle cell disease (SCD); a hereditary chronic hemolytic anemia characterized by pain crises. The perception of pain and its impact on psychological development varies with age, the supporting role of the family, the person's cognitions, and prior painful experiences. Methods. We compared 19 affected children to 21 adult patients, in terms of differences in perception, tolerability, affective reaction, pain projection and social adjustment consequent to the disease. Standardized measures were used to assess developmental differences. Results. The subjective perception of pain was less dramatic in children than in adults, and the perception of pain intensity was stronger in adults than in children. More than 80% of adults, but only 1/3 of children chose the most desperate images when attempting to depict pain. The quality of life, probably influenced by previous anguishing pain experiences, resulted good only in seven out of 21 adults, but in fifteen out of 19 children, and no child considered it poor. Conclusions. This difference suggests that the longer the duration of the disease, the more negative the evaluation of the experience in sickle-cell patients. The small sample size limits the generalization of our findings. More developmentally oriented studies with appropriate diagnostic instruments are warranted

The family pediatrician and the shared management of children with hemato-oncological diseases|Il pediatra di famiglia e la gestione condivisa tra ospedale e territorio del bambino emato-oncologico

Children with a hemato-oncological disease and their family must face a long course of treatment, burdened by frequent and sometimes long inpatient admissions to the Paediatric Hemato-oncology Center. The involvement of the family paediatrician in the care program, with the knowledge of the most common problems and side effects related to the disease and its treatment, can strengthen the patient-physician relationship and encourage, when possible, the management of certain complications at home, reducing the burden of recurrent hospitalizations and so contributing to the improvement of the quality of life. The article addresses the most common complications, providing indications and practical advices for their better management by the family paediatrician. © 2018, Associazione Culturale Pediatri. All rights reserved