Unravelling the role of mind-body therapies in paediatric palliative care: A narrative review.

AIM: Given the multifaceted nature of paediatric palliative care (PPC), integrative medicine and mind-body therapies, in particular, are increasingly used in this setting. However, to our knowledge, their use in PPC has never been specifically reviewed. Therefore, this work aims to provide a comprehensive overview of the application and possible beneficial effects of mind-body therapies in the PPC setting. METHODS: Papers about the use of meditation and mindfulness, biofeedback, hypnosis, yoga, tai chi and qi gong, imagery, creative outlets, and art therapy in PPC were identified by browsing PubMed and CINHAL. RESULTS: We found that the harmless nature of mind-body therapies has been extensively described, and available studies consistently show some benefits on the anxiety, stress, and quality of life of parents/caregivers and healthcare providers. However, well-grounded and robust evidence does not fully support the efficacy of mind-body therapies, especially in patients. CONCLUSION: Considering the peculiarity of the PPC setting, we suggest that mind-body therapies should be further considered and properly investigated through larger controlled studies and according to the different populations of PPC children, as well as for parents, caregivers, and healthcare professionals.

A 15-year experience in pediatric palliative care: a retrospective hospital-based study.

BACKGROUND: The current Italian scenario of pediatric palliative care (PPC) services is characterized by inadequate coverage of the territory. Therefore, it is important to improve the referral of patients to the most appropriate setting (community care, general PPC, or specialized PPC) and to improve the delivery of PPC care. METHODS: Aiming at obtaining information about the referrals to the Padua Pediatric Hospice that could help estimate the investments needed to improve the provision of care, a retrospective analysis has been carried out. The rate of proper referral and discharge, the number of patients followed at the hospice, the mortality rate, and the length of follow-up were analyzed, and, when possible, data were stratified by oncological and non-oncological diseases. RESULTS: The analysis showed that of the 870 patients referred to the Padua Pediatric Hospice between 2008 and 2022, 76% were affected by non-oncological conditions. 82% of patients referred were taken in charge and most of the remaining patients have been inappropriately referred. The analysis showed a growing number of total referrals, which increased by 195% from 2008 to 2022. An increase in proper referrals and referrals of non-oncological patients was observed alongside a decrease in oncological patient referrals and a trend toward a decrease in discharge rates. A decreased mortality was observed in patients with non-oncological conditions, with only 6% of deceased patients in 2022. Moreover, a longer survival with a median follow-up length of 43 months was observed among patients with non-oncological conditions who were followed up at the Padua Pediatric Hospice for more than 12 months. Conversely, the short survival rate observed for oncological patients suggests that those patients should have been referred to PPC earlier to benefit from palliative care for longer periods. CONCLUSIONS: Considering these data, it is expected that the number of patients needing PPC services will steadily increase in the next years. Hence, there is a need to invest resources to provide the best care delivery model encompassing specific pathways for the transition into adulthood, the establishment of networks within all the Italian regions, and an efficient referral to the more suitable setting of care.

Severe trichothiodystrophy and cardiac malformation in a newborn carrying a novel GTF2H5 homozygous truncating variant.

We report a newborn patient with trichothiodystrophy-3 (TTD3) caused by a novel homozygous variant in the GTF2H5 gene. His severe phenotype included congenital ichthyosis, complex posterior cranial fossa anomaly, life-threatening infections, bilateral cryptorchidism, and, notably, a complex cardiac malformation, which is unprecedented in TTD3 patients.

A pilot study on sports activities in pediatric palliative care: just do it.

BACKGROUND: There has been a growing interest in studying the value of physical exercise in children with disabilities or chronic health conditions because of evidence of improvement in quality of life, social acceptance, and physical functioning. However, only scant evidence exists for routine sports activities in children requiring pediatric palliative care (PPC), and in most cases, such evidence has been collected in oncological patients. The Pediatric Hospice of Padua is the referral center for PPC in the Veneto region (northern Italy). Starting from the experience of this PPC center, this pilot study aims to describe the personal experience of children and young people who practice physical activity and their caregivers' perspectives, focusing particularly on the emotional and social impact of exercise and sports practice. METHODS: Patients involved in at least one regular and structured sports activity were included in the pilot analysis. Two different ICF-CY (International Classification of Functioning, Disability and Health-Children and Youth Version) scales ("Body Function" and "Activity and Participation") were filled out to assess the children's global functional competence. Two online ad hoc questionnaires were created and administered to children, when able to respond, and caregivers. RESULTS: A total of 9% of patients reported being involved in a sports activity. All children who played sports did not have indications of cognitive retardation. The most practiced sport was swimming. The use of standardized methods such as ICF-CY shown that severe motor impairments do not limit sports accessibility. Questionnaires result suggest that sports are a positive experience for both children needing PPC and their parents. Children encourage other children to do sports and can find the positive side even in difficulties. CONCLUSION: Since PPC is encouraged as early as the diagnosis of incurable pathologies, the inclusion of sports activities in the context of a PPC plan should be considered from the perspective of improving quality of life.

End-of-life care for children with complex congenital heart disease: Parents' and medical care givers' perceptions.

AIM: In complex congenital heart diseases (CHD), patients may remain affected by significant morbidity and mortality after surgery. We analysed the end-of-life (EoL) care in children with severe CHD who died in our institution and investigated perspectives of parents and health-care professionals (HCPs). METHODS: Medical records of all children (age < 18 years old) affected by a severe CHD who died in a tertiary cardiac care centre were reviewed. Subsequently, a cross-sectional questionnaire-based study of parents and HCPs of children involved in the study was designed. RESULTS: In total, 30 children died (median age: 45 days; range: 15 days to 3.4 years). Of them, 97% (31/32) died in an intensive care unit setting and were intubated and sedated at EoL. A total of 77% (23/30) died without parents being present at bedside. Eighteen families and 10 HCPs were interviewed. For 61% of the parents (11/18) and 70% of the clinicians (7/10), the goal of therapy at the EoL was 'to lessen your child's suffering as much as possible'. Overall, 44% of parents (8/18) and 50% of HCPs recognised that their child had no chance of survival 'a few days before the child died'. CONCLUSIONS: We believe that these data suggest an unconscious reluctance to change goals of care in EoL, shifting from intensive care to comfort and quality of life.

Pain management in children has significantly improved in the Italian emergency departments.

AIM: Pain in children is often poorly assessed and treated in Italian emergency departments (EDs) as found in a survey conducted among the centres of the "Pain in Pediatric Emergency Room (PIPER)" Study Group in 2010. Our aim was to evaluate the changes in pain management in Italian EDs in the last years. METHOD: A structured questionnaire about pain assessment, protocols, use of local anaesthetics before venipuncture, opioids and adjuvants was mailed to 46 Italian EDs between November 2017 and April 2018. RESULTS: Pain was always assessed at triage in 34 centres (73.9%). Algometric scales were used in over 90% of EDs. Triage protocols were available in 37 centres (80.4%). Local anaesthetics before venipuncture were always used in six centres (13.0%). Protocols included opioids and adjuvants in 73.0% and 47.2%, respectively. Triage pain assessment was always done in 91.0% of the centres joining the PIPER Study Group up to 2015 and 56.5% in ones that joined the Group after 2015 (P = .017). Local anaesthetics before venipuncture were given in 39.1% of the centres joining until up to 2015 and 13.0% in ones that joined the Group after 2015 (P = .003). CONCLUSION: Paediatric pain management has significantly improved in Italian EDs in the last 8 years. The centres joining the PIPER Study Group for longer time have shown better results for the indicators considered in the survey.

Use of ketamine by paediatricians in Italian paediatric emergency departments: a missed opportunity?

Procedural sedation and analgesia with ketamine are part of daily practice for children undergoing painful procedures in the paediatric emergency department (ED) of North America. A massive number of studies demonstrate ketamine's safety and efficacy in the hands of trained ED paediatricians, with few severe adverse events (SAEs) recorded. Since there are no data on ketamine's usage in Italian paediatric EDs, we created a survey to examine procedural sedation with ketamine in the EDs of the Italian PIPER (Pain in Paediatric Emergency Room) group, which includes 36 paediatric EDs providing 1.4 million paediatric visits each year. Results were reviewed using frequencies to describe responses. Thirty-two out of 36 centres replied to the questionnaire. In 6 (19%) out of 32 centres, ketamine is not used at all in the paediatric ED. In 6 centres (23%) of 26 which use ketamine, this drug is autonomously administered by the emergency paediatrician, whereas in 20 (77%) of them it is exclusively managed by the anaesthesiologist on call.Conclusion: ketamine is autonomously administered only by a small percentage of Italian emergency paediatricians. There is an increasing need for implementation of procedural sedation training and use of ketamine in the everyday practice outside the operating room in paediatric EDs. What is Known: • Ketamine is safely and efficaciously administered for children's procedural sedation and analgesia by trained emergency paediatricians in the everyday practice outside the operating room in North America. • In the Italian setting, there are no data at all concerning ketamine's usage by the emergency paediatricians for procedural sedation and pain control. What is New: • In this study emerged that ketamine is poorly administered by Italian emergency paediatricians for procedural sedation and analgesia outside the operating room. • A great deal of educational effort should be made to widen ketamine based procedural sedation availability in Italian emergency departments by spreading specific training tracks and guidelines.

Needle-related pain and distress management during needle-related procedures in children with and without intellectual disability.

Children with intellectual disability frequently undergo needle-related procedures for diagnosis or treatment. Nevertheless, only a few studies deal with pain and distress management during the procedure in this population of children. This study aimed to investigate the number of anxiety and pain management techniques performed during needle procedure in children with intellectual disability (cases) compared to a population of children without intellectual disability (controls). This multicenter cohort study was performed from July 2016 to January 2018 in the pediatric ward of four urban hospitals in Italy. Eligible subjects were children with and without intellectual disability, from 4 to 17 years old, who needed venipuncture or intravenous cannulation for diagnosis or treatment. Use of topical anesthesia, distraction techniques, and physical or verbal comfort during procedures were recorded. Pain and anxiety scores were also recorded. Forty-seven cases and 94 controls were recruited. Three pain- and anxiety-relieving techniques were performed during the procedure in 12 (25%) cases and in 10 controls (11%); two techniques were performed in 23 (50%) cases and in 26 (28%) controls; 12 (25%) cases and 52 (55%) controls received only one.Conclusion: In this series, children with intellectual disability received significantly more relieving techniques, but experienced more pain and anxiety when compared to children without intellectual disability. What is Known: • Children with intellectual disability experience more episodes of pain than cognitively healthy ones, and almost 10% of these episodes are due to medical procedures. What is New: • Children with intellectual disability despite receiving more relieving techniques during a needle-related procedure experienced more pain and anxiety when compared to healthy children.

Symptom management and psychological support for families are the cornerstones of end-of-life care for children with spinal muscular atrophy type 1.

AIM: This study described end-of-life care for children affected by spinal muscular atrophy type 1 (SMA1), which is characterised by progressive muscle weakness and develops in the first six months of life. METHODS: We retrospectively analysed 17 children (13 boys) who attended the University of Padua's paediatric palliative care centre in Italy from March 2000 to March 2015. All the children received supportive care without proactive respiratory intervention to prolong survival. RESULTS: The median age at admission was 3.57 months, and the median age at death was 6.80 months. The most frequent symptoms were dyspnoea and pain. In the last 72 hours of life, 15/17 children required more intense doses of morphine and, or, benzodiazepines for intractable dyspnoea and pain, but deep palliative sedation was not needed. Airway suction to manage secretions and nasogastric tubes was required in all cases. The place of death was previously planned by the parents in all cases - home, hospital or hospice - and 15/17 deaths occurred in that place. We also interviewed 16 of the 17 parents after their child died. CONCLUSION: Our study found that symptom management and psychological support for families were the cornerstones of end-of-life care for children with SMA1.

Multicentre emergency department study found that paracetamol and ibuprofen were inappropriately used in 83% and 63% of paediatric cases.

AIM: The Pain Practice in Italian Paediatric Emergency Departments assessed how appropriately analgesic drugs were being used by Italian clinicians, based on national paediatric pain guidelines. METHODS: This was a retrospective study that involved 17 Italian members of the Pain In Pediatric Emergency Rooms group. It comprised patients up to the age of 14 years who came to hospital emergency departments with pain and were treated with paracetamol, ibuprofen or opioids, such as codeine, tramadol and morphine. RESULTS: We studied 1471 patients who were given 1593 doses of analgesics. The median time to administration of analgesia was 25 minutes. Opioids were used in 13.5% of the children, and usage increased with age and with more severe clinical conditions, such as trauma: 1.6% of children under two years, 5.9% aged 3-10 and 8.0% aged 11-14. Inappropriate doses of paracetamol, ibuprofen and opioids were used in 83%, 63% and 33% of cases, respectively. The patient's age was a critical determinant of the correct analgesic dosage; for every one-year increase in the patient's age, the probability of appropriate prescriptions rose 14.8%. CONCLUSION: The appropriate use of paracetamol and ibuprofen for paediatric pain in Italian emergency departments was very poor, but improved with age.

Pain in Mucopolysaccharidoses: Analysis of the Problem and Possible Treatments.

Mucopolysaccharidosis (MPS) are a group of lysosomal storage disorders that are caused by the deficiency of enzymes involving in the catabolism of glycosaminoglycan (GAGs). GAGs incompletely degraded accumulate in many sites, damaging tissues and cells, leading to a variety of clinical manifestations. Many of these manifestations are painful, but few data are available in the literature concerning the prevalence, etiology, and pathogenesis of pain in children with MPS. This review, through the analysis of the data available the in literature, underscores the relevant prevalence of pain in MPSs' children, provides the instruments to discern the etiopathogenesis of the disease and of pain, illustrates the available molecules for the management of pain and the possible advantages of non-pharmacological pain therapy in MPSs' patients.

Acute pain management: acetaminophen and ibuprofen are often under-dosed.

Most children with pain are managed by either acetaminophen or ibuprofen. However, no study has so far investigated if children are prescribed adequate doses of acetaminophen or ibuprofen in emergency department. Aim of this retrospective study was to investigate the prevalence of under-dosage of these drugs in children presenting with pain in emergency department. Children initially prescribed with acetaminophen or ibuprofen for pain management were included. The χ 2 automatic interaction detection method was used considering the percentage variation from the minimum of the appropriate dose as dependent variable while prescribed drug, age, gender, body weight, type of hospital (pediatric or general), and availability of internal guidelines on pediatric pain management in the emergency department as independent variables. Data on 1471 children managed for pain were available. Under-dosage was prescribed in 893 subjects (61%), of whom 577 were prescribed acetaminophen and 316 ibuprofen. The use of acetaminophen suppositories, body weight <12 kg or >40 kg, and the use of oral ibuprofen identified clusters of children associated with under-dosage prescription. CONCLUSION: Prescription of acetaminophen and ibuprofen was frequently under-dosed. The use of suppositories, lower and higher body weight, and the use of ibuprofen were associated with under-dosage. Under-dosing may reflect prescription of anti-pyretic doses. CLINICAL TRIAL REGISTRATION: Agenzia Italiana del Farmaco-Observational Study Register (RSO). Registration code: PIERRE/1 What is Known: • Pain is frequent in children presented to emergency department. • International recommendations on pain management are often not implemented. What is New: • Acetaminophen and ibuprofen were frequently underdosed in children prescribed for pain in the Italian emergency departments. • Under-dosage may be related to the habit of using acetaminophen and ibuprofen in the recommended range for fever treatment.

First-time success with needle procedures was higher with a warm lidocaine and tetracaine patch than an eutectic mixture of lidocaine and prilocaine cream.

AIM: More than 50% of children report apian during venepuncture or intravenous cannulation and using local anaesthetics before needle procedures can lead to different success rates. This study examined how many needle procedures were successful at the first attempt when children received either a warm lidocaine and tetracaine patch or an eutectic mixture of lidocaine and prilocaine (EMLA) cream. METHODS: We conducted this multicentre randomised controlled trial at three tertiary-level children's hospitals in Italy in 2015. Children aged three to 10 years were enrolled in an emergency department, paediatric day hospital and paediatric ward and randomly allocated to receive a warm lidocaine and tetracaine patch or EMLA cream. The primary outcome was the success rate at the first attempt. RESULTS: The analysis included 172 children who received a warm lidocaine and tetracaine patch and 167 who received an EMLA cream. The needle procedure was successful at the first attempt in 158 children (92.4%) who received the warm patch and in 142 children (85.0%) who received the cream (p = 0.03). The pain scores were similar in both groups. CONCLUSION: This study showed that the first-time needle procedure success was 7.4% higher in children receiving a warm lidocaine and tetracaine patch than EMLA cream.

Nationwide study of headache pain in Italy shows that pain assessment is still inadequate in paediatric emergency care.

AIM: Italian national guidelines on pain management were published in 2010, but there is little information on how effective pain management is in paediatric emergency care, with other countries reporting poor levels. Using headache as an indicator, we described pain assessment in Italian emergency departments and identified predictors of algometric scale use. METHODS: All Italian paediatric and maternal and child hospitals participated, plus four general hospitals. Data on all children aged 4-14 years admitted during a one-month period with headache as their chief complaint were abstracted from clinical records. Multivariable analyses identified predictors of algometric assessment, taking into account the cluster study design. RESULTS: We studied 470 admissions. During triage, pain was assessed using a standardised scale (41.5%), informally (15.5%) or was not recorded (42.9%). Only 32.1% of the children received analgesia in the emergency department. The odds ratios for predictors of algometric assessment were non-Italian nationality (3.6), prehospital medication (1.8), admission to a research hospital (7.3) and a more favourable nurses-to-admissions ratio of 10.8 for the highest versus lowest tertile. CONCLUSION: Despite national guidelines, paediatric pain assessment in Italian emergency care was suboptimal. Hospital variables appeared to be stronger predictors of adequate assessment than patient characteristics.