HUMAN VACCINES & IMMUNOTHERAPEUTICS 2016, VOL. 12, NO. 3, 801–805 http://dx.doi.org/10.1080/21645515.2015.1086046
SHORT REPORT
Vaccination coverage of children with rare genetic diseases and attitudes of their parents toward vaccines Susanna Esposito, Marta Cerutti, Donatella Milani, Francesca Menni, and Nicola Principi Pediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Universita degli Studi di Milano, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
ABSTRACT
ARTICLE HISTORY
Despite the fact that the achievement of appropriate immunization coverage for routine vaccines is a priority for health authorities worldwide, vaccination delays or missed opportunities for immunization are common in children with chronic diseases. The main aim of this cross-sectional study was to evaluate immunization coverage and the timeliness of vaccination in children suffering from 3 different rare genetic diseases: Rubinstein-Taybi syndrome (RSTS), Sotos syndrome (SS), and Beckwith-Wiedemann syndrome (BWS). A total of 57 children with genetic diseases (15 with RSTS, 14 children with SS, and 28 with BWS) and 57 healthy controls with similar characteristics were enrolled. The coverage of all the recommended vaccines in children with genetic syndromes was significantly lower than that observed in healthy controls (p < 0.05 for all the comparisons). However, when vaccinated, all of the patients, independent of the genetic syndrome from which they suffer, were administered the primary series and the booster doses at a similar time to healthy controls. In comparison with parents of healthy controls, parents of children with genetic diseases were found to more frequently have negative attitudes toward vaccination (p < 0.05 for all the comparisons), mainly for fear of the emergence of adverse events or deterioration of the underlying disease. This study shows that vaccination coverage is poor in pediatric patients with RSTS, BWS, and SS and significantly lower than that observed in healthy children. These results highlight the need for educational programs specifically aimed at both parents and pediatricians to increase immunization coverage in children with these rare genetic diseases.
Received 10 June 2015 Revised 2 August 2015 Accepted 18 August 2015 KEYWORDS
Beckwith-Wiedemann syndrome; Rubinstein-Taybi syndrome; Sotos syndrome; vaccines; vaccination coverage
Introduction
Materials and methods
Despite the fact that the achievement of appropriate immunization coverage for routine vaccines is a priority for health authorities worldwide, vaccination delays or missed opportunities for immunization are common in children with chronic diseases.1-3 Falsely perceived contraindications, fear of reactivation of the underlying disease, and the different opinions of multiple specialists involved in the care of these children are the most common reasons that justify the refusal of vaccine administration by parents.4 Urgent interventions are needed to address this problem. However, because adherence to the immunization schedule varies according to the underlying disease,5 information on the existing coverage and delays for each chronic medical condition has to be collected before planning corrective methods to promote timely vaccination. Poor data are presently available regarding children with genetic disorders. Among them, only those with Down syndrome have been evaluated, showing relatively good coverage for both routine and recommended immunizations.5 The main aim of this study was to evaluate immunization coverage and timeliness of vaccination in children suffering from different rare genetic diseases and the attitudes of their parents toward vaccination.
This cross-sectional study, which was carried out between November 1, 2014, and April 30, 2015 and approved by the Ethics Committee of the Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico (Milan, Italy), involved all of the children with Rubinstein-Taybi syndrome (RSTS), Sotos syndrome (SS), and Beckwith-Wiedemann syndrome (BWS) regularly followed up in the Pediatric Highly Intensive Care Unit of the University of Milan’s Department of Pathophysiology and Transplantation. Written informed consent of a parent or legal guardian was required. The three considered genetic disorders are rare diseases with a prevalence of 1:125,000 for RSTS, 1:40,000 for SS, and 1:13,700 for BWS.6-8 RSTS and SS are associated with psychomotor delay and intellectual disability, whereas psychomotor and intellectual development is normal in BWS. All three conditions are associated with an increased risk of cancer. RSTS is also characterized by postnatal growth retardation in height and weight, microcephaly, broad thumbs, big toes, and dysmorphic facial features;6 SS presents with excessive physical growth during the first few years of life, macrocrania with a slightly protrusive forehead and pointed chin, large hands and feet, hypertelorism and down-slanting eyes;7 BWS causes
CONTACT Susanna Esposito © 2016 Taylor & Francis
[email protected]
802
S. ESPOSITO ET AL.
large body size, external ear abnormalities and low-set ears, large eyes and tongue, and mild microcephaly.8 The diagnosis of each syndrome was based on clinical findings and genetic testing showing gene mutations specific to each of them.6-8 As controls, a group of age- and sex-matched healthy children were enrolled. Healthy controls were randomly selected among those who attended the outpatient clinic of the same Department for minor surgical problems, who did not have any history of chronic underlying disease, and who lived in the same Regions of the patients. The vaccination status of both patients and controls was established by consulting the official vaccination chart, which was issued by the Vaccination Service of the regions in which the children lived. Data regarding diphtheria (D), tetanus (T), pertussis (P), polio (IPV), hepatitis B (HB), Haemophilus influenzae type b (Hib), pneumococcal conjugate (PC), meningococcus C (MC), and measles, mumps and rubella (MMR) vaccines were collected. All of the children were considered fully vaccinated when they had received the doses of each vaccine at the suggested time, according to the recommendations of the most recent edition of the National Immunization Plan.9 Moreover, the parents were administered a questionnaire (which was created and revised after pilot testing) soliciting information regarding their opinion on vaccination and on who mainly influenced their decisions on vaccination. Specific questions about influenza vaccination, which is recommended by the National Immunization Plan for these genetic diseases,9 were included. Different genetic diseases were compared each with the others and then each of them was compared with healthy controls. A contingency table analysis using the chi-square test or Fisher’s exact test, as appropriate, compared the differences between the groups. The ordered categorical data were compared using a CochranArmitage trend test. All tests were 2-tailed, and a p value
SHORT REPORT
Vaccination coverage of children with rare genetic diseases and attitudes of their parents toward vaccines Susanna Esposito, Marta Cerutti, Donatella Milani, Francesca Menni, and Nicola Principi Pediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Universita degli Studi di Milano, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
ABSTRACT
ARTICLE HISTORY
Despite the fact that the achievement of appropriate immunization coverage for routine vaccines is a priority for health authorities worldwide, vaccination delays or missed opportunities for immunization are common in children with chronic diseases. The main aim of this cross-sectional study was to evaluate immunization coverage and the timeliness of vaccination in children suffering from 3 different rare genetic diseases: Rubinstein-Taybi syndrome (RSTS), Sotos syndrome (SS), and Beckwith-Wiedemann syndrome (BWS). A total of 57 children with genetic diseases (15 with RSTS, 14 children with SS, and 28 with BWS) and 57 healthy controls with similar characteristics were enrolled. The coverage of all the recommended vaccines in children with genetic syndromes was significantly lower than that observed in healthy controls (p < 0.05 for all the comparisons). However, when vaccinated, all of the patients, independent of the genetic syndrome from which they suffer, were administered the primary series and the booster doses at a similar time to healthy controls. In comparison with parents of healthy controls, parents of children with genetic diseases were found to more frequently have negative attitudes toward vaccination (p < 0.05 for all the comparisons), mainly for fear of the emergence of adverse events or deterioration of the underlying disease. This study shows that vaccination coverage is poor in pediatric patients with RSTS, BWS, and SS and significantly lower than that observed in healthy children. These results highlight the need for educational programs specifically aimed at both parents and pediatricians to increase immunization coverage in children with these rare genetic diseases.
Received 10 June 2015 Revised 2 August 2015 Accepted 18 August 2015 KEYWORDS
Beckwith-Wiedemann syndrome; Rubinstein-Taybi syndrome; Sotos syndrome; vaccines; vaccination coverage
Introduction
Materials and methods
Despite the fact that the achievement of appropriate immunization coverage for routine vaccines is a priority for health authorities worldwide, vaccination delays or missed opportunities for immunization are common in children with chronic diseases.1-3 Falsely perceived contraindications, fear of reactivation of the underlying disease, and the different opinions of multiple specialists involved in the care of these children are the most common reasons that justify the refusal of vaccine administration by parents.4 Urgent interventions are needed to address this problem. However, because adherence to the immunization schedule varies according to the underlying disease,5 information on the existing coverage and delays for each chronic medical condition has to be collected before planning corrective methods to promote timely vaccination. Poor data are presently available regarding children with genetic disorders. Among them, only those with Down syndrome have been evaluated, showing relatively good coverage for both routine and recommended immunizations.5 The main aim of this study was to evaluate immunization coverage and timeliness of vaccination in children suffering from different rare genetic diseases and the attitudes of their parents toward vaccination.
This cross-sectional study, which was carried out between November 1, 2014, and April 30, 2015 and approved by the Ethics Committee of the Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico (Milan, Italy), involved all of the children with Rubinstein-Taybi syndrome (RSTS), Sotos syndrome (SS), and Beckwith-Wiedemann syndrome (BWS) regularly followed up in the Pediatric Highly Intensive Care Unit of the University of Milan’s Department of Pathophysiology and Transplantation. Written informed consent of a parent or legal guardian was required. The three considered genetic disorders are rare diseases with a prevalence of 1:125,000 for RSTS, 1:40,000 for SS, and 1:13,700 for BWS.6-8 RSTS and SS are associated with psychomotor delay and intellectual disability, whereas psychomotor and intellectual development is normal in BWS. All three conditions are associated with an increased risk of cancer. RSTS is also characterized by postnatal growth retardation in height and weight, microcephaly, broad thumbs, big toes, and dysmorphic facial features;6 SS presents with excessive physical growth during the first few years of life, macrocrania with a slightly protrusive forehead and pointed chin, large hands and feet, hypertelorism and down-slanting eyes;7 BWS causes
CONTACT Susanna Esposito © 2016 Taylor & Francis
[email protected]
802
S. ESPOSITO ET AL.
large body size, external ear abnormalities and low-set ears, large eyes and tongue, and mild microcephaly.8 The diagnosis of each syndrome was based on clinical findings and genetic testing showing gene mutations specific to each of them.6-8 As controls, a group of age- and sex-matched healthy children were enrolled. Healthy controls were randomly selected among those who attended the outpatient clinic of the same Department for minor surgical problems, who did not have any history of chronic underlying disease, and who lived in the same Regions of the patients. The vaccination status of both patients and controls was established by consulting the official vaccination chart, which was issued by the Vaccination Service of the regions in which the children lived. Data regarding diphtheria (D), tetanus (T), pertussis (P), polio (IPV), hepatitis B (HB), Haemophilus influenzae type b (Hib), pneumococcal conjugate (PC), meningococcus C (MC), and measles, mumps and rubella (MMR) vaccines were collected. All of the children were considered fully vaccinated when they had received the doses of each vaccine at the suggested time, according to the recommendations of the most recent edition of the National Immunization Plan.9 Moreover, the parents were administered a questionnaire (which was created and revised after pilot testing) soliciting information regarding their opinion on vaccination and on who mainly influenced their decisions on vaccination. Specific questions about influenza vaccination, which is recommended by the National Immunization Plan for these genetic diseases,9 were included. Different genetic diseases were compared each with the others and then each of them was compared with healthy controls. A contingency table analysis using the chi-square test or Fisher’s exact test, as appropriate, compared the differences between the groups. The ordered categorical data were compared using a CochranArmitage trend test. All tests were 2-tailed, and a p value
