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Abstract Background In pediatric palliative care, the main caregiver is primarily responsible for managing pharmaceutical therapies. Few data are available regarding the influence of this burden on quality of life in terms of time, concerns as well as a considerable risk of administration errors and adverse effects. This study aims to investigate how caregivers prepared and administrated medication, including errors and associated expectations, to identify improvement interventions. Methods Between October 2022 and March 2023, a descriptive single-center survey study was carried out in the tertiary care pediatric palliative center of the Padova University Hospital. Participants were the caregivers of the patients followed by our center up to 23 years old, receiving at least one drug daily and who cannot self-administer their therapy. The questionnaire consisted of 18 multiple-choice and semi-closed questions, grouped into 4 main topics: therapy preparation, therapy administration, administration errors and therapy assessment. Results A total of 100 caregivers responded to the survey. Mothers represented the main caregiver (91%). The prevalence of polypharmacy was 67% across the patients. 52% of caregivers handled prescriptions at least three times per day and for 32% it took to prepare them more than 5 min each time. Only 59% reported to have been trained for preparing and administrating drugs. 14% reported having made at least a drug administration error due to the tiredness or the complexity of therapeutic regimens in the preceding three months. Nearly one caregiver out of three felt their child was using too many drugs. 73% positively welcomed the possibility of having clinical pharmacist-led counseling. Conclusions Many caregivers of pediatric palliative care patients frequently have trouble planning, preparing and delivering pharmacological therapy to their children. Attempting to simplify medication regimens, choosing formulations that are simpler to administer and measure, investing in improved caregiver training, talking about therapies with carers, and involving clinical pharmacists to clarify their doubts could be all potential strategies to improve this condition and reduce their burden.

Abstract 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.

Background Despite evidence showing that the intranasal and sublingual routes are safe and effective in providing analgesia, no data are available about their day-to-day use in the emergency department (ED). The aim of this study was to assess the frequency of the use of the intranasal and sublingual routes, and the clinical characteristics of the patients receiving analgesia through these routes.Methods A multicentre study was performed in the EDs participating in the Pain in Paediatric Emergency Room research group. It included a survey and a retrospective data collection in which the medical records of all patients who received analgesia from 1 April 2022 to 31 May 2022 were collected.Results 48 centres (91%) answered the survey. The intranasal and sublingual routes were used in 25 centres (52%). 13 centres (27%) used both routes, 9 centres (19%) used only the sublingual and 3 centres (6%) used only the intranasal route.12 centres (48%) participated in the retrospective study. Data about 3409 patients, median age 9 years (IQR 5–12), were collected. Among them, 337 patients (9.6%) received sublingual analgesia, and 87 patients (2.5%) received intranasal analgesia. The intranasal route was employed for injuries in 79 (90.8%) cases, and fentanyl was the drug delivered in 85 (97.7%) cases. The sublingual route was used mainly for injuries (57.3%), but also for abdominal pain (15.4%), musculoskeletal pain (14.5%) and headache (10.7%). Paracetamol, ketorolac and tramadol were administered through this route.Conclusions The use of the intranasal and sublingual routes for analgesia in the paediatric ED is still limited.

Abstract Background Accurate estimation of the specialized pediatric palliative care (PPC) burden and the definition of the extent and quality of PPC service in Italy represent urgent needs to enable the proper allocation of PPC resources and the definition of prevention and educational plans. The PalliPed project aimed to provide the first comprehensive assessment of the characteristics of Italian patients requiring PPC, the quality and extent of regional PPC networks/facilities, and the number of dedicated resources. In this paper, we present the results of the second part of the project, regarding the implementation and quality of PPC services in Italy. Methods The PalliPed study had an observational cross-sectional design. All Italian specialized PPC centers/facilities were invited to participate in the project and complete a survey on the characteristics of PPC centers/facilities in different care settings, reporting data as of 24 October 2022. Data were collected online. Results 19 PPC specialized centers/facilities from 12 Italian regions and two autonomous provinces responded to the survey. Among them, 11 are regional referral centers. Seven Italian regions out of 20 reported no PPC centers/facilities, mainly in central-southern Italy. Less than half (45%) of the regional referral centers cover the entire regional territory, and three offer 24/7 service. Ten centers have a dedicated team. Half of the eight non-referral centers offer 24/7 service and have a dedicated team. A total of 1,092 patients were reported by 18 centers as of 24 October 2022. Over the years, an increasing number of patients has been reported, rising from 1,202 (2019) to 1,544 (2021). The dedicated staff is inadequate, and most healthcare providers are not recognized at an institutional level. A shortage of ‘young’ staff and a lack of specific training was reported, particularly among nurses (77% had no training in PPC). Conclusions The results obtained show how training, information, and research interventions are still necessary for the reorganization of the available resources and definition of proper strategies to respond dynamically to the new emerging needs of these populations. At the same time, our study represents a first step in defining a national registry of PPC models, useful for monitoring evolutions, and critical issues and planning any new or corrective strategy.

The literature widely supports the benefits of early integration of palliative care into pediatric oncological care; however, many barriers to its successful integration remain. Integrating palliative care as early as possible in the oncology pathway is critical, but other criteria are relevant to positive results. This paper aims to contribute to the early/late referral dualism in pediatric palliative care (PPC) and highlight the importance of a collaborative approach between oncologists and palliative care teams. This study investigates the impact of early versus late referral to PPC, intersecting it with the synergy work between services and the related outcomes. The four pediatric cancer cases were selected based on clinical (e.g., disease duration, multiple treatments, and pain management), management (e.g., involvement of multiple services and multiple home–hospital transitions), and relevance of multidisciplinary team (e.g., difficult clinical decisions and ethical discussions) criteria. A mixed-methods approach was employed, combining qualitative case analysis using clinical diaries, literature review, and practice guidelines development. Critical clinical information, time course, clinician–family communication, and patient involvement were analyzed. The outcomes show how simultaneous care creates continuous discussion and dialogue between professionals. The results indicate the importance of better communication and care coordination to improve patient and family satisfaction, highlighting the uniqueness of the pediatric field and the relationship with children and families. Through the discussion of clinical cases and a literature review, we provide practical guidance for clinicians working in oncology and PPC. These findings underscore the crucial need for a multidisciplinary approach in pediatric oncology, advocating policy changes to support early PPC integration and translate it into complementarity best operating practices. In conclusion, besides assessing the timeliness of referral to the PPC service, the synergy, harmony, and choral work of the professionals involved are equally valuable for a quality-of-life-oriented care plan.

Background: Children with medical complexity (CMC) often require multiple medications, leading to polypharmacy, which seems to be linked to adverse effects, administration errors, and increased caregiver burden. This study aimed to describe the prevalence of polypharmacy, medication burden, off-label drug use, and associated costs. Methods: Conducted at the Pediatric Palliative Care Center of Padua, Italy, from August to October 2021, this cross-sectional observational study included patients up to 23 years old with at least one prescribed drug. Data were collected from medical records and caregiver interviews. Drug costs were collected from the Italian Medicine Agency. Descriptive statistical analysis was performed. For comparisons among categorical variables, the Chi-square test was used, and for those among continuous variables, the ANOVA test was used. Results: This study analyzed treatment regimens of 169 patients with a median age of 12.5 years (0.3–23). Polypharmacy was present in 52.7% of patients, and medication burden was observed in 44.4%, both varying significantly by primary diagnosis (p < 0.001). The median daily cost per patient was EUR 2.2 (IQR 0.9–7.1), with significant variation among subgroups. Only 34.6% of prescriptions were off-label. Conclusions: polypharmacy and medication burden are frequent among our CMC population, with some differences according to primary diagnosis.

Pediatric palliative care (PPC) is defined as “the active care of the child’s body, quality of life, mind and spirit, also giving support to the family”. PPC should be established once a diagnosis of life-limiting or life-threatening disease is reached and should continue as long as necessary. Therefore, pediatric palliative care (PPC) can continue for years, also given the improved care approaches for children with life-limiting or life-threatening diseases. Over time, the child may grow to become a young adult, and when this happens, the transition to adult healthcare services must be undertaken. This article discusses possible interventions, fostering an efficient transition from pediatric to adult palliative care. A narrative review presents issues, experiences, and existing programs. A “Perspectives” section presents opinions and proposals by the authors. The transition process is not limited to a change from pediatric to adult services. Rather, it includes the entire process of the development of the child and requires interdisciplinary management with proper planning and collaboration among professionals of pediatric and adult teams.

Abstract 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.

Abstract Background Medical cannabis may be a useful tool for managing treatment-resistant epilepsy and chronic pain, which affect many patients in pediatric palliative care (PPC); however, little evidence is available in this setting. Case presentation We aimed to describe a clinical experience in a setting where high-level evidence may not be obtained. We report our clinical experience in a pediatric palliative care department in Italy. Caregivers reported changes in intensity and frequency of pain and epilepsy events. Six patients received a titrated plant extract of cannabis sativa for 1 year. Only mild and transient adverse events occurred: drowsiness, euphoria, restlessness and tachycardia; the resolution was either spontaneous or obtained by modifying the administration schedule. Treatment was never discontinued. No overdoses occurred. All patients experienced seizures during the pre-treatment observation period, and obtained a reduction in seizure frequency, although with variable extent while receiving cannabis. In addition, a benefit on pain was observed, based on the caregiver’s evaluation, and a reduction of analgesic use. Conclusion Our experience suggests that a titrated plant extract preparation of medical cannabis may be useful to control treatment-resistant pain and epilepsy in PPC patients.

The proper assessment of needs and outcomes in pediatric palliative care (PPC) is imperativeto ensure the best possible service to patients and families. However, given the multidimensionalnature of PPC, the low number of patients in this setting, the heterogeneity of diseases, the presenceof cognitive impairment in many patients, and the physiological development of children, outcomescan be complex and difficult to measure. Consequently, in this context, the use of standardizedand validated tools to assess the needs of children and families, to assess symptom severity, andto estimate the quality of PPC service represent a current need. Even if efforts have been made tostandardize approaches and tools for palliative care in adults, to our knowledge, a similar comprehensiveassessment of PPC has not yet been conducted to date. This narrative review provides anoverview and discusses the evaluation of tools currently applied in PPC, with an educational intentfor healthcare providers. We found that several instruments are available to assess different dimensionsof PPC. We proposed a classification into eligibility tools, patient and family needs assessmenttools, and care assessment tools. At present, two main eligibility tools exist, the PaPaS Scale and theACCAPED Scale questionnaire. Most of the tools for patient and family needs assessment have notbeen specifically validated in the PPC setting, and many may be more readily applied in researchsettings rather than in daily practice. Similar considerations can be made for tools assessing QoL,while tools assessing PPC service quality seem to be easily applied. Efforts to develop new specifictools and validate existing ones are undoubtedly advocated. However, in the patient’s best interest,PPC healthcare providers should start using available tools, regardless of their validation status.

Background: One of the main challenges for paediatric palliative care (PPC) is the management of concomitant, different and severe symptoms that frequently affect the quality of life of PPC patients and are often refractory to commonly used pharmacological treatments. Consequently, many efforts are still needed to find the best therapeutic options to handle these refractory conditions. Since the first synthesis of ketamine in the 1960s, its pharmacokinetic and pharmacodynamic properties have been largely investigated and its potential wide range of clinical applications has become clear. However, this molecule still receives poor attention in some areas, including in children and PPC. This narrative review analyses the use of ketamine in children and the potential extension of its applications in PPC in order to provide new options for treatment in the PPC setting. Methods: Scientific papers published before October 2020 on MEDLINE, EMBASE and the Cochrane Library were considered. The cited references of the selected papers and the authors’ personal collections of literature were reviewed. The terms “palliative care”, “ketamine”, “neuropathic pain”, “procedural pain”, “status epilepticus”, “refractory pain” and “child”, adding “age: birth–18 years” on a further filter were used for the search. Discussion: The use of ketamine in PPC should be more widely considered due to its overall favourable safety profile and its efficacy, which are supported by an increasing number of studies, although in settings different from PPC and of mixed quality. Ketamine should be proposed according to a case-by-case evaluation and the specific diagnosis and the dosage and route of administration should be tailored to the specific needs of patients. Furthermore, there is evidence to suggest that ketamine is safe and efficacious in acute pain. These findings can prompt further research on the use of ketamine for the treatment of acute pain in PPC. Conclusion: Ketamine could be a suitable option after the failure of conventional drugs in the treatment of different refractory conditions in PPC.

Abstract Aim Children and adolescents affected by somatization and somatic symptom disorder commonly refer to emergency services. Due to the absence of specific guidelines for the emergency setting and to a possible lack of knowledge, these patients are at risk of being unrecognized and mismanaged. This study aims at proposing a clinical practice to approach and manage these patients and their families in the emergency setting. Methods This manuscript derived from the work of a research group of italian pediatric emergency physicians and anesthesiologists, with an expertise in pain management, members of the PIPER group. The research group reviewed the literature about psychosomatic pain and somatic symptom disorder and developed a clinical practice specific for the pediatric emergency setting. Results The manuscript provides information about the main clinical features shared by patients with psychosomatic pain and about current diagnostic criteria and appropriate management in the emergency setting. Furthermore, it highlights the possible pitfalls in which the emergency physician may run into dealing with these patients. Conclusion This clinical practice should be seen as a starting point toward a better understanding of patients with psychosomatic pain and a standardization of care in the pediatric emergency setting.

Abstract Background We report the clinical outcomes observed in our patients with SMA type 1 or 2 receiving nusinersen, and we comment on the ethical implications of this treatment, in line with our results and those reported by Audic et al. in their analysis published in the Orphanet Journal of Rare Diseases. Methods We analyzed records of all children with a genetically diagnosed SMA and clinically confirmed diagnosis of SMA Type 1 or 2 to whom nusinersen was offered. Follow-up lasted 30 months. Results Among the 17 children with SMA type 1, 6 interrupted treatment with nusinersen due to adverse events or lack of efficacy. Of the remaining 11 patients, 9 are responding to therapy, though multidisciplinary complex care is still required. All those children started nusinersen at a very early age. Eighteen patients with SMA type 2 received nusinersen; five required treatment interruption. The other 13 patients are still on nusinersen therapy, and 6 are responders. Among the seven non-responders, only two met the inclusion criteria of the pivotal trial. Conclusions Our analysis further supports the findings reported in the study by Audic et al. We believe that a wider use of nusinersen in clinical practice would require a comprehensive assessment of its actual benefits weighed against the discomfort caused to patients, as well as the identification of the patients who may obtain the best benefits from this treatment.

BackgroundPediatric palliative care (PPC) is defined as the prevention and relief from suffering of families and children with life-limiting (LLDs) or life-threatening diseases (LTDs). These patients often experience pain, with morphine being the most widely used drug to treat it. Few studies investigated the role of methadone in PPC patients, although it is considered among the most effective and underutilized drugs in PPC.ObjectivesOur aim was to evaluate the efficacy, safety, and dosage of methadone in PPC.MethodsBetween August and October 2021 PubMed, Scopus and the Cochrane Library were searched for studies on the use of methadone in children with LLDs and LTDs. Articles were included if they met the following criteria: published in the last 10 years, English language, patients aged 0–23 years; children enrolled in a PPC center or receiving declared support from a PPC service; reporting of specific data on methadone in interventional trials, observational studies, or case series on >10 patients. The reporting of the article was guided by the PRISMA guidelines, and a critical appraisal of the included studies was performed using the JBI-tool.ResultsAfter duplicates removal and full-text assessment, four studies were included and another one was added after checking the references of the retrieved papers. All were retrospective, and the literature is concordant in documenting the lack of evidence. A total of 116 children received methadone in PPC. From our review emerges the poor quality of data collection: in only one study pain was assessed with standardized scales. All studies documented the effectiveness of methadone in treating complex pain, either nociceptive or neuropathic. No serious adverse events were reported, with no cases of cardiac arrhythmias.ConclusionOur results suggest that methadone could represent a suitable strategy for treating pain in PPC. However, the evidence base is insufficient, and further research is warranted.

Infants, children and adolescents with life-limiting and life-threatening disease need long-term care that may change according to disease’s natural history. With the primary goal of quality of life, the psychologist of pediatric palliative care (PPC) network deals with a large variety of issues. Little consideration has been given to the variety of intervention areas of psychology in PPC that concern the whole life span of the patient and family. The PPC network is composed by a multidisciplinary team of palliative care specialists that intervenes at home, in the hospital and in every place where the patient is living. The network coordinates different public health services to respond to clinical, psychosocial and spiritual needs. In these scenarios, the psychological need is not a single event but a moment inserted in the complexity of the child’s needs. This retrospective monocentric project consists of an analysis of characteristics of psychological interventions in our PPC service. The time frame taken into consideration is 2019–2020, analyzing the clinical records of 186 patients of Pediatric Palliative Care and Pain Service of Veneto Region (Italy). The areas that emerged in the analysis show how the intervention of the psychologist in PPC does not concern only end-of-life, but a series of topics that are significant for the family to guarantee psycho-social wellbeing oriented toward the best quality of life. In conclusion, these different topics highlight the complexity of the child and family experience. This variety must be taken into consideration, the psychologist must increase holistic support with a dedicated skills curriculum.

Nusinersen is the first oligonucleotide-based drug that is approved for the treatment of spinal muscular atrophy. In January 2020, the WHO declared COVID-19 a pandemic and nusinersen-provider centers had to postpone planned infusions for some children along with other related interventions. Considering the important contribution that the intrathecal infusions and other support activities could have on the quality of life of spinal muscular atrophy patients and their families, this emergency could have a relevant impact on the course of the pathology. The present work aims to assess the clinical and social issues that arise for spinal muscular atrophy children in care at the referral pediatric palliative care Centre of Padua (Veneto) from a delay in nusinersen infusions, resulting from the contingent COVID-19 restrictions. This evaluation has been carried out in both the short and long term after the first lockdown period and can be considered as a “proxy” of a situation of a possible delay in administration or management of infusions, due to other different causes.

Abstract Background Pediatric palliative care (PPC) addresses the physical and psychological needs of children suffering from life-limiting diseases. To define prevention and educational plans and to properly allocate resources, a precise estimation of the PPC burden is required. Objectives To estimate the current number of children requiring PPC in Italy, useful to assist policy-makers and healthcare bodies in the organization and allocation of PPC resources. Methods Literature data, The Global Atlas of Palliative Care at the End of Life and Italian national databases have been consulted. Results According to our estimation, at present, a total of 20,540–32,864 children in Italy require PPC (34–54 children/100,000 inhabitants) of whom 18 children/100,000 inhabitants require specialized PPC. Conclusions The present work is a fundamental tool to be used by the institutions, the local networks of PPC and the health programmers when formulating organizational models and care plans consistent with the actual need for PPC.

Abstract In the pediatric setting, management of pain in the emergency department – and even in common care – is a challenging exercise, due to the complexity of the pediatric patient, poor specific training of many physicians, and scant resources. A joint effort of several Italian societies involved in pediatrics or in pain management has led to the definition of the PIPER group and the COPPER project. By applying a modified Delphi method, the COPPER project resulted in the definition of 10 fundamental statements. These may represent the basis for improving the correct management of children pain in the emergency department.

Abstract Background The definition of the eligibility criteria of newborn, infant, child, or adolescent patients for palliative care (PC) is complicated by the fact that these patients generally present with very specific case histories that make it inadvisable to directly adopt existing PC protocols devised for adult patients. Thus, the goal of this paper is to define a standard set of criteria for establishing pediatric palliative care (PPC) eligibility. Methods The method adopted was that of the consensus conference. According to the guidelines issued by the Higher Institute of Health, the Board of the Italian Society for Palliative Care (i.e. steering committee) appointed a multidisciplinary group of eight health care professionals (i.e. doctors, nurses and psychologists) who worked from May 2014 to February 2016 to reach a consensus over PPC eligibility. This panel of relevant experts redacted a report summarizing all available scientific information concerning PPC, which was then submitted to the attention of a multidisciplinary jury composed of specialists and non-specialists of the field. The document thus produced was subsequently reviewed by an extended team of experts. Results The consensus conference drafted a final document determining the guidelines for PPC eligibility of newborns, infants, children, and adolescents suffering from either oncological or non-oncological diseases. Conclusions This report provides health care providers with practical guidelines on how to define the eligibility of pediatric patients for PPC. Given the current situation in Italy, these guidelines will be instrumental in assisting the implementation of adequate generalist and specialist PPC services as well as in helping policymakers draft and implement national legislation pertaining to PPC.

Purpose of review: The aim of this study is to assess the most significant Perinatal Palliative Care (PnPC) development projects in the literature and summarize the shared key principles.Recent findings: PnPC is a new concept in neonatal intensive care approach. Advancements in perinatal diagnostics and medical technology have changed the landscape of the perinatal world. The threshold of viability continues to decrease, and diagnostic information is available earlier in pregnancy and more rapidly at the bedside; overall outcomes continue to improve. This rapid technological improvement brings ethical debates on the quality of life of patients with life-limiting and life-threatening conditions and the need to involve the family in the decision-making process, according to their wishes and cultural beliefs. Although the Perinatal Hospice concept was developed in the 1980s in the US, the first recommendations on how to develop a PnPC pathway were published in the early 2000s. We considered the most relevant position statements or guidelines on PnPC published in the last two decades. Some of them were more pertinent to pediatrics but still useful for the fundamental concepts and PnPC project's development.Summary: Health care providers and institutions are encouraged to develop PnPC programs, which have the goal of maximizing the quality of life of infants with non-curable conditions. These may generally include the following: a formal prenatal consultation; development of a coordinated birth plan between obstetrician, newborn care, and family; access to other neonatal and pediatric specialties, as needed; comfort palliative care during the prenatal, birth, and postnatal periods; and psychosocial and spiritual support for families, siblings, and staff.

Abstract Background Paediatric palliative care (PPC) aim to ensure the control of symptoms and the best possible quality of life for patients whose underlying disease, characterized by an unstoppable evolution and negative prognosis, no longer responds to specific treatments. The scientific evidence in this context are very deficient and, in order to obtain welfare objectives consistent with the situation, in the overwhelming majority of cases the prescription of drugs is off-label for indication of use and/or for age and/or for way of administration and/or formulation. The Agenzia Italiana del Farmaco - AIFA and the Italian Society of Palliative Care (Società Italiana di Cure Palliative - SICP), under a dedicated working group, wrote a document that collects the scientific evidence available to support the off-label use of medicines more frequently used in PPC. The goal is to certify the consolidated off-label use of these drugs and propose their use under the Law 648/96, in the absence of data from its pivotal clinical trials. Aim of the commentary is to report the conditions for this important work and to present the 10 drugs, usually used off-label in PPC and in pain therapy, now included in Law 648/96. Conclusion This work is deemed essential to resolve, at least in part, the lack of availability of medicines researched and approved.

Mucopolysaccharidoses (MPSs) are a group of rare, genetic lysosomal storage disorders. They are caused by deficiencies of the lysosomal enzymes involved in the degradation of glycosaminoglycans (GAGs). Pain is a common feature in mucopolysaccharidoses. However, the pathophysiology of pain in this group of diseases is still unclear and genesis of pain is multifactorial. Currently, poor data about pain management in these patients are available. Here, we present our clinical experience in complex pain management in three children with MPS.

We describe a case of a paediatric patient affected by mandibular fibrous dysplasia (FD) with severe and chronic pain who was successfully treated with zoledronic acid (ZOL): a third-generation bisphosphonate. Further research is needed to assess its safety and efficacy as a treatment option for FD in the paediatric population.

We report our clinical experience on the effect of Scrambler Therapy (ST) for a child with acute mixed pain refractory to pharmacological treatment. ST, recently proposed as an alternative treatment for chronic neuropathic pain in adults, is a noninvasive approach to relieve pain, by changing pain perception at brain level. It is safe and has no side effects. Further research is needed to assess its efficacy for acute pain and for paediatric population.

There are numerous difficulties encountered in the diffusion of palliative care (PC) in developed countries. A correct and widespread awareness of PC among the general public represents an important factor for its enhanced diffusion and use. The aim of this study is to verify the level of awareness of PC among Italians and their perception of the needs of patients with incurable illness. A random sample of 1897 adult subjects, aged 18 to 74 years, representative of the Italian population, was interviewed after stratification by gender, age (6 strata), education (3 levels), geographic area of residency (4 areas) and town of residency (4 dimensions). 59.4% of those interviewed had heard of PC, but only 23.5% of them believed that they had an adequate or precise idea of what PC is; 27% of them did not know or had a mistaken idea about the nature of PC. The most accepted perception was that PC alleviates pain and improves quality-of-life. The principal concerns attributed to incurable patients were: fear of suffering and of death, and the principal needs were perceived as: relief from pain and physical suffering. The primary needs of the family were identified in: medical and nursing care at home, followed by: care provided by volunteers and psychological support. The most appropriate care-setting for these patients was indicated as their home, possibly with the support of professional carers. In the case of children, the main concern indicated was that of being "separated" from family, friends, home and toys. Only 45.2% of interviewees thought that they knew a person who had experienced PC. In conclusion, the awareness of the Italian population of PC is scarce and often incorrect. In order to achieve a greater diffusion and better use of PC in our country, this awareness needs to be improved by appropriate intervention.

Procedural pain is an important aspect of care in pediatrics, and particularly in pediatric oncology where children often consider this to be the most painful experience during their illness. Best recommended practice to control procedural pain includes both sedative-analgesic administration and non-pharmacological treatments, practiced in an adequate and pleasant setting by skilled staff. A nationwide survey has been conducted among the Italian Centers of Pediatric Hematology-Oncology to register operators’ awareness on procedural pain, state of the art procedural pain management, operators’ opinions about pain control in their center, and possible barriers impeding sedation-analgesia administration. Based on indications in the literature, we discuss the results of the survey to highlight critical issues and suggest future directions for improvement. Future objectives will be to overcome differences depending on size, improve operators’ beliefs about the complexity of pain experience, and promote a global approach to procedural pain.