Pediatric Palliative Care Research Articles

Capacity Planning in Paediatric Palliative and Hospice Care: An Italian Case

Although the growing demand for healthcare services has enhanced the occurrence of inefficiencies in care provision, healthcare systems should ensure safe, timely and equitable access to care. In particular, the long waiting lists, the lack of beds, and the shortage of healthcare workforce have highlighted the necessity to effectively manage and strategically plan the available resources. Capacity Planning (CP) contributes to determine what, when, where and how much product or service to deliver, with the goal of analysing and optimizing the use and allocation of human and material resources (e.g., physical spaces, equipment, beds, workforce) based on the demand. CP demonstrates to be extremely relevant for hospital units to schedule appointments, diagnostics, surgeries and admissions. Complex contexts and broader healthcare networks could benefit even more from this approach, helping to coordinate multiple care providers and aligning the available resources with the demand pace. The current research presents the implementation of CP in the context of Paediatric Palliative and Hospice Care (PPHC), delivered in an Italian facility. PPHC provides long-term, interdisciplinary and coordinated care between territorial, ambulatory and hospice services. It encompasses follow-up activities, parental training, end-of-life and respite care. In the Italian case analysed, the shortage of beds affects the quality of care delivered to these patients, in terms of accessibility to the service, effectiveness and timeliness of care. Hence, a CP analysis has been performed to pursue a twofold objective: a) to understand if the current capacity of the facility meets the demand for care; b) to improve productivity through an optimized allocation of resources, particularly the available beds and the healthcare workforce. The expected results involve a more effective use of resources, an increased bed occupancy rate and a reduction in rescheduled admissions due to inadequate planning. Optimizing capacity in this specific setting can serve as a pilot project for other similar contexts, aiming to improve patients’ access to healthcare services, their quality of life and supporting the workforce involved in PPHC. This research offers new insights from both the academic and managerial point of view, providing evidence about the implementation of CP in complex healthcare settings.

Paediatric palliative care in Aotearoa New Zealand-current state and future direction.

This paper seeks to explore the current state of paediatric palliative care in Aotearoa New Zealand. The low priority afforded to paediatric palliative care for more than two decades has had a significant impact on service provision, education and research within this specialty. As a result, provision of specialist paediatric palliative care to children with serious illness and their whānau (family, including extended family) is inequitable and vastly inadequate. This paper considers the consequences of having limited access to specialist palliative care for children and whānau, and outlines what is required for both service development and current priorities for research.

Goals of Surgical Interventions in Youths Receiving Palliative Care

Most youths receiving palliative care undergo many surgical interventions over their lifetimes. The intended purposes of interventions in the context of goals of care are not commonly articulated. To describe the goals and purposes of surgical intervention in youths receiving palliative care and propose a framework discussing intervention using goal-oriented language. This retrospective cohort analysis was conducted among a subset of patients enrolled between April 2017 and March 2021 in a prospective multicenter cohort study of youths receiving palliative care (the Pediatric Palliative Care Research Network's Shared Data and Research [SHARE] Study). Patients younger than 30 years receiving palliative care services were eligible for inclusion in SHARE, and all enrolled at Boston Children's Hospital/Dana Farber Cancer Institute, a SHARE site, were included in this study. Goals and purposes of all surgical interventions from the time of diagnosis through the present were abstracted from patient records. A goal and purpose framework was generated using a hybrid deductive-inductive approach based on established goals-of-care frameworks and the clinical context of surgical interventions. Data were analyzed in September 2023. Primary outcomes included goals and purposes of surgical interventions performed in the study population. Among 197 youths receiving palliative care (mean [SD] age at palliative care start, 8.01 [7.53] years; 108 male [54.8%]; 6 Asian [3.0%], 12 Black [6.1%], 129 White [65.5%], and 16 with >1 race [8.1%]; 27 Hispanic [13.7%] and 142 not Hispanic [72.1%]), almost all individuals (189 youths [95.9%]) underwent at least 1 surgical intervention (mean [SD] 17.5 [16.3] interventions; median [IQR] 13 [5-22] interventions). Of 3331 surgical interventions, there were 878 interventions (26.5%) conducted with the goal of life extension, 1229 interventions (37.1%) conducted for life enhancement, and 79 interventions (2.4%) conducted for both goals; the remaining 1130 interventions (34.1%) held neither goal. Most interventions were performed with the purpose of diagnosis (1092 interventions [32.9%]) or cure and repair (1055 interventions [31.8%]), with fewer performed for the purpose of placing or maintaining assistive technology (696 interventions [21.0%]) or for supportive (434 interventions [13.1%]) or temporizing (39 interventions [1.2%]) purposes. Patients with cardiovascular disease and cancers constituted approximately half (592 patients [56.1%]) of those undergoing curative or repair interventions, whereas youths with neurologic or genetic conditions constituted approximately half (244 patients [56.2%]) of those undergoing supportive interventions. In this cohort study, nearly all youths underwent surgical intervention, and the purposes of intervention differed by serious illness type. These findings suggest that conversations centered on a proposed framework concerning goals and purposes of surgical intervention may facilitate goal-concordant, high-quality care for youths with serious illness.

The role and perception of the caregiver in a specialized pediatric palliative care center in medicine preparation and administration: a survey study

BackgroundIn 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.MethodsBetween 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.ResultsA 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.ConclusionsMany 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.

Evaluation of infectious agents in the pediatric palliative care unit

Aim: The use of broad-spectrum antibiotics, changes in patient profile, prolonged life expectancy, increased use of medical devices and technologies such as catheters, intravenous fluids, gastrostomy and tracheostomy have led to changes in the number and types of microorganisms isolated from infections and cultures. There are no data on infections seen in pediatric palliative care. The aim of this study was to determine the frequency of infections and the pathogens produced in culture, to identify the sites of growth and to review what should be done. Materials and Methods: This was planned as a cross-sectional, retrospective study between 11/18/2018 and 09/01/2023. Sociodemographic data, reason for hospitalization, number of hospitalizations, primary diagnoses, comorbid conditions, medical devices and technology used, time and place of culture, number of cultures, microorganisms grown and factors associated with culture growth were examined. Statistical analysis was performed with the SPSS 18.0 program. p

Pediatric Palliative Care Simulation Improves Resident Learning Outcomes: an 11-Year Review

Context: Many general pediatrics residents lack sufficient opportunities to conduct difficult conversations with families, particularly about end-of-life care. Simulation learning is an effective means of practicing professional skills. A pediatric palliative care (PPC) physician is uniquely suited to mentor residents and fellows learning to lead difficult conversations through simulation. Co-facilitation of the simulated difficult conversation by a bereaved parent or family member enhances the learning experience. Objectives: To report 11-years’ experience simulating difficult conversations with bereaved parent-actors. Methods: PPC physicians developed two simulations to teach difficult conversations to clinical learners at a midwestern quaternary pediatric medical center. Bereaved parents and hospital chaplains co-facilitated the simulation. The first portrayed the death of an infant following emergency resuscitation, and the second, a goals-of-care conversation with the parent of a child with a degenerative condition. A de-novo evaluation rubric was prepared using the six Accreditation Council for Graduate Medical Education (ACGME) Core Competencies to evaluate the participant's performance in the simulation. Results: For the first simulated scenario (N=194 residents; N=16 fellows), residents improved significantly on 16/21 ACGME-based criteria between encounters; for the second (N=118 residents; N=14 fellows), residents improved significantly on 10/21 criteria. Fellows’ performance did not improve significantly in either scenario, but they presented with high baseline scores. Conclusions: Simulations with bereaved parent actors improved general pediatrics residents’ performance and comfort during difficult conversations and are transportable to diverse settings.

Pediatric Palliative Care and the Anesthesiologist

Pediatric Palliative Care and the Anesthesiologist

Clownselling: an in-depth narrative review on the therapeutic influence of clowning therapy in paediatric and adult cancer treatment and palliative care

Cancer and its treatments impose substantial physical and psychological burdens on patients, underscoring the need for innovative and holistic interventions. This narrative review explores the evolving role of clownselling in paediatric and adult cancer care, tracing its historical progression from entertainment to a therapeutic modality. Recognizing the potential of clowning therapy to address emotional, psychological, and existential challenges, the review systematically assesses its impact on treatment adherence, quality of life, and contributions in palliative care settings. By elucidating the therapeutic process, principles, and goals, it provides a comprehensive understanding of the foundations of clowning therapy. Given its positive influence on well-being, the integration of clowning therapy into interdisciplinary care teams and institutional policies is advocated. Future directions include diversified research, technology integration, standardized protocols and awareness campaigns, ensuring the sustained evolution of clowning therapy in fostering holistic well-being within the intricate tapestry of cancer care.

Perceptions of physicians caring for pediatric patients with cancer in Europe: insights into the use of palliative care, its timing, and barriers to early integration.

Integrating pediatric palliative care (PPC) into pediatric oncology standard care is essential. Therefore, it is important to assess physicians' knowledge and perceptions of PPC to optimize its practice. To evaluate the knowledge, comfort levels, and perspectives of physicians regarding the timing and perceived barriers to integrating PPC into pediatric cancer care across Europe. The Assessing Doctors' Attitudes on Palliative Treatment (ADAPT) survey, originally developed for other global regions, was culturally and contextually adapted for Europe. The survey was distributed via the European Society of Paediatric Oncology (SIOPE) membership listserv. Any physicians caring for children with cancer across Eastern, Southern, Central, and Northern Europe were invited to complete the survey. A total of 198 physicians from 29 European countries completed the ADAPT survey. Physicians demonstrated relative agreement with the World Health Organization's guidance; median alignment was 83.4% (range 59.9%-94.1%). Although most respondents felt comfortable addressing physical (84.4%) and emotional (63.4%) needs, they felt less comfortable addressing spiritual needs (41.9%) and providing grief and bereavement support (48.5%). There were significant regional differences, such as physicians in Eastern and Southern Europe reporting a lack of PPC specialists, opioids, and home-based care, while those in Northern and Central Europe did not. Physicians caring for children with cancer throughout Europe have a good understanding of PPC. However, misconceptions about PPC persist, requiring educational and capacity-building efforts. Additionally, the regional differences in perceived barriers must be addressed to ensure equitable access to PPC for all European children with cancer.

Bereaved parents' perceptions of their cancer-ill child's last month with or without palliative care -a nationwide study.

Cancer is still the leading cause of non-accidental death in childhood, although the majority of children diagnosed in high-income countries survive their illness. In accordance with international standards, equal and early access to palliative care should be available to children and adults. Yet communication and prognostic disclosure may influence the timing of involvement in palliative care. Purpose: To investigate whether parents perceived that their child received palliative care and to what extent that contrasted parents' perceptions of their child's care and symptoms in the last month of life. A nationwide population-based parental questionnaire study in Sweden, one to five years after their child's death (n=226). Descriptive statistics were used. A majority of parents (70%) reported that they were aware that their child received palliative care and they were informed about the incurable disease (57%) within 3 months before the child died. The most common diagnosis among children receiving palliative care was a brain tumor (45%) with a disease related death (90%) and the care was often received at home (44%). Based on the reports of parents who felt that their child did not receive palliative care, 45% were informed within days or hours about the child's incurable disease, 45% of these children were diagnosed with leukemia, 60% died at the intensive care unit, and 49% died of treatment-related complications. It was most common for families who lived in urban areas (28%) to report their child received palliative care, in comparison to families living in sparsely populated areas (11%). A significant proportion of parents whose child received palliative care (96%) stated that the healthcare professionals were competent in caring for their child, for those who reported no palliative care it was slightly lower (74%). In both groups many children were affected by multiple symptoms the last month of life. The study findings highlight the role of understanding parental perceptions of pediatric palliative oncology care, the role of initiating palliative care early, the need of access to national equitable PC and professional competence across the lifespan, regardless of diagnosis and place of residence.

Palliative Care Referral Patterns and Implications for Standardization in Cardiac ICU.

Evidence suggests that pediatric palliative care involvement (PPC) is beneficial to medically complex patients. Historically, PPC involvement has been overlooked or delayed and varies by institution but PPC awareness has increased in cardiovascular ICUs (CVICU) and so we investigated frequency and timeliness of PPC referral for patients dying in ICU. Retrospective study of pediatric cardiac patients experiencing death in ICU to review PPC presence and timing of initial PPC, most recent PPC, and interventions, therapies, CPR, and presence of do-not-resuscitate DNR discussion. Fifty-four patients died during a 5-year period aged 11d-17y (54% male). PPC involvement occurred in 40/54 (74%). Of those patients without PPC, the Center to Advance Palliative Care (CAPC) guidelines would have supported PPC in 11/14 (79%). DNR discussion was more likely in PPC patients (63% vs 14%; p = 0.0011), though often only on DOD. Comparing prior to DOD, PPC patients were still more likely to have DNR discussion (55% vs 0%; p = 0.0003). PPC patients were no less likely to have CPR on DOD (28% vs 43%, p = 0.29). PPC occurred frequently in patients experiencing death in CVICU. However, frequently the initial PPC occurred within a week or day of death. Patients without PPC would often qualify under published guidelines. Standardization, timing, and patient identification for PPC will expand efficacy in CVICU.

Association of inpatient and outpatient pediatric palliative care with healthcare utilization and end-of-life outcomes in pediatric oncology.

Pediatric palliative care (PPC) is associated with improved end-of-life (EOL) outcomes. Inpatient and outpatient PPC have unique roles during the disease course. Yet, it is unknown whether the location of PPC receipt (inpatient vs. outpatient) is associated with healthcare utilization and EOL outcomes for pediatric and adolescent and young adult oncology patients. A retrospective single-institution chart review of pediatric patients (age 0-28) with cancer who died between January 2015 and December 2022 was performed to compare EOL outcomes and healthcare utilization metrics among inpatient PPC, any outpatient PPC, and non-PPC recipients. Demographics and clinical factors were analyzed by PPC receipt location. Among 450 patients, 292 (64.9%) received PPC (inpatient only 35%, any outpatient 65%). Patients who died without receiving PPC dropped from 69% to 22% following development of an outpatient PPC clinic (p<.001). In the last 6months, 1month, and last week of life, inpatient PPC recipients spent more days admitted to the hospital and intensive care unit (all p<.001), and had more intensive medical interventions performed (p<.01). Outpatient PPC recipients were less likely to receive intravenous (IV) chemotherapy (p<.01) or intubation (p=.05), and more likely to receive hospice, die at home, and have an outpatient do-not-resuscitate order (all p<.001). PPC receipt substantially increased after the creation of an outpatient PPC clinic, suggesting that outpatient PPC is critical in the provision of PPC to children with cancer. Outpatient PPC was associated with fewer hospital days, IV chemotherapy, and intubation at EOL, while increasing hospice enrollment and home death.

Non-invasive respiratory support in children and young adults with complex medical conditions in pediatric palliative care

ObjectiveDyspnoea and sleep-disordered breathing (SDB) are common in children with life-limiting conditions but studies on treatment with non-invasive ventilation (NIV) or continuous positive airway pressure (CPAP) are scarce. The aim...

The purpose of an individual care plan in pediatric palliative care according to healthcare professionals: a qualitative study

BackgroundTo ensure high-quality pediatric palliative care (PPC) and enable healthcare professionals (HCPs) to provide person-centered care for the individual child with a life-limiting or life-threatening illness and their family, the Individual Care Plan (ICP) for PPC was developed. However, in practice, the ICP for PPC is not used for all children in need of PPC. Insight into why, for which problem and for which children HCPs wish to use an ICP in PPC is therefore necessary to assure that the ICP can be fully implemented in practice.MethodsTwo semi-structured focus group interviews were held with 15 experienced healthcare professionals in PPC. Data were thematically analyzed.ResultsParticipants wish to use an ICP to collaboratively establish a plan with parents for the current and future care and treatment of the child. By doing so, they hope to achieve coordination and continuity of care, to achieve shared decision-making, and to support parents and other healthcare professionals in the care of the child. Participants think the ICP could also be used in the care for children with complex chronic conditions.ConclusionThe purposes for which healthcare professionals wish to use an ICP in PPC are broader than the purpose for which the ICP for PPC was developed. Future research should focus on whether the use of an ICP in PPC practice can achieve these purposes.

Roles of pediatric surgeons in palliative pediatric oncology.

Pediatric surgeons engaged in oncology will inevitably treat patients receiving palliative care, but their role in this context is poorly described. This article identifies some of the challenges and opportunities of surgical involvement in pediatric oncology palliative care, underscoring how the surgeon's expertise can be exploited to significantly benefit children with cancer. Specific examples of skills (procedural, communication, and coordination) that surgeons can provide to the multidisciplinary palliative care teams are described and the importance of collaboration is highlighted.

Everyday Ethics or Deference to Expertise: Experiences of Pediatric Palliative Care Teams with Ethics Consultancy.

Background: Little is known about the extent to which pediatric palliative care (PPC) clinicians are engaged in ethics consults or how they perceive interactions with ethics consultants. Objective: Describe the extent to which PPC team members serve in pediatric health care ethics (PHCE) consultancy roles and to describe their experiences interacting with pediatric ethic consultant services. Design: Online survey distributed to members of the American Academy of Pediatrics and American Academy of Hospice and Palliative Care pediatric and ethics section and special interest groups in the United States. Results: Eighty-six responses were obtained (response rate 45%) from PPC teams in 70 different children's hospitals located in 34 states. Almost all (97%) reported a functional ethics consult service such that PPC is not expected to meet the ethics need of the institution. A person involved on the PPC team also performed ethics consults in half (49%) of the settings, predominantly the PPC physician. Most respondents who perceive PPC teams engage in ethics-relevant work as part of their everyday PPC work. Formal ethics training was lacking among PPC members involved in ethics consults with few ethics degrees (15%), certifications (6%), or fellowships (2%). Discord (67%), conflict (49%), limitations to treatment (48%), and distress (41%) were cited as the most frequent reasons for which PPC teams consult ethics. PPC respondents identified role clarity, coordinated engagement, timely presence, and open communication as strong PHCE consultant practices. Conclusions: PPC team members performing ethics consults may benefit from additional ethics education and training. Further research is warranted to delineate scope of ethics consultancy relevant to PPC and role clarity.

Perceived impact of pediatric palliative care: caregiver's perspective.

Caregivers of children with complex chronic diseases (CCD) or life-limiting conditions (LLC) experience heightened strain. Understanding their concerns is essential for effective support, particularly in pediatric palliative care (PPC) where compassionate support is crucial. Amid substantial growth in PPC services in Portugal, assessing the effectiveness of care in addressing these concerns and adapting to the evolving needs of parents is imperative. The aim of this study was to perceive the impact of PPC from the caregivers' perspective. Participants consisted of children with CDD or LLC and their caregivers followed by the PPC team. Institutionalized children were excluded. Our mixed-methods study involved analyzing sociodemographic characteristics and assessing caregiver and child well-being using quantitative data and explored caregiver concerns and the impact of PPC through qualitative data obtained from semi-structured interviews. We included 43 children and caregivers. Caregivers (90.6% mothers; mean age 40) often experience fatigue (90.7%) although they rarely feel exhausted (67.4%). Sleep disturbances (58.1%) are the children's most frequent perceived symptom. Seven categories emerge from caregivers' concerns. Among the five categories arising from caregivers' perceived impact of PPC integration, 95.3% prioritize enhanced contact with healthcare professionals. Increased support to facilitate caregiver rest and early referral emerges as primary suggestions for improving PPC. The PPC appears to be effective in alleviating parents' primary concerns, highlighting the crucial importance of early referral. Implementing strategies to provide respite for caregivers and closer monitoring of these children becomes a priority to improve the quality of life for this inherently intertwined dyad, the child-family unit.

A Nationwide Study to Evaluate Accessibility to Specialized Pediatric Palliative Care in Italy-Patients, Needs, and Critical Issues: The PalliPed Study.

Background: PalliPed is the first Italian nationwide project aimed at describing the characteristics of patients accessing specialized pediatric palliative care (PPC) and their families, in the main care settings (hospice, home care, and hospital). The project's secondary aim is to assess the extent and quality of regional PPC networks/facilities and the number of dedicated resources. In this article, we present the results of the first part of the project. Methods: All Italian PPC centers/facilities were invited to participate in the project. Children and young adults in the care of the specialized PPC networks/structures as of 24 October, 2022, including prenatal care, were involved. Children's eligibility for specialized PPC was assessed according to the Assessment Form for Complex Clinical Needs in Pediatrics (ACCAPED Scale) and after a multidisciplinary assessment by the healthcare team. Data were collected through an online survey. Results: A total of 867 patients were described. The lack of adequate specialized PPC service emerged, according to the available estimate of specialized PPC needs, as well as the need for improved referral to PPC by pediatricians or territorial services, particularly for infants and oncological patients. More family support measures also seem necessary, particularly for the mothers. Healthcare providers' communication skills should be improved to ensure greater involvement of patients and families in care decisions. Conclusions: This analysis represents the first step toward defining a constantly updated database for the census and monitoring of specialized PPC activities at the national level. This research model can be extended to other realities in different countries, allowing comparison of different care models.

Implementation and Evaluation of a Pediatric Palliative Care Curriculum for Adult-Track HPM Fellows

IntroductionHospice and Palliative Medicine (HPM) fellowship training provides education on caring for patients from early childhood through adulthood. Yet, there are few guidelines about how to teach these key components, and more specifically the components of Pediatric Palliative Care (PPC), to fellows who have a variety of backgrounds prior to entering HPM fellowship. MethodsWe created a curriculum in PPC that could be used with adult-track HPM fellows. We developed an outline for material to cover during each of the three weeks of PPC clinical rotations. We also created a longitudinal lecture series encompassing several foundational topics and concepts in PPC. We conducted “Just-in-Time” evaluation of the clinical rotation curriculum utilizing a critical reflection model as well as focus groups with fellows and faculty members to further explore the knowledge, attitudes, and perceptions of the entire PPC curriculum. Transcripts were coded Nvivo and template analysis was used to understand code distribution between and within focus groups. ResultsEight overarching codes emerged from the focus groups: “Staffing Issues,” “Learning Structure,” “Useful Material,” “Topics Attending Contingent,” “Time Management,” “Teachable Moment,” “Not Useful Material,” and ”Adherence to Teaching Materials.” ConclusionThe study revealed some limitations in the current curriculum design, such as its lack of consistency. However, it also highlighted several strengths, including the importance of flexibility and personal responsibility for learning. The results provide valuable insights into the experiences among fellows and educators at a single institution with a PPC Curriculum for adult-track HPM fellows.

Intrathecal baclofen therapy as treatment for spasticity and dystonia: Review of cases in a pediatric palliative care unit

IntroductionPatients managed in the Pediatric Palliative Care Integral Unit (PPCIU) have serious neurological conditions that involve significant damage at central nervous system level. The movement disorder is a very common clinical problem and for the patients where an adequate control of muscle tone is not achieved with usual techniques or drugs, intrathecal baclofen therapy (IBT) should be considered. Materials and methodsDescriptive retrospective study based on the review of clinical records of patients who received IBT being followed by the PPCIU of Madrid Autonomous Region in the timeframe between September 2012 and February 2021. ResultsIBT was implanted in 8 patients affected by infantile cerebral palsy (ICP) with a Gross Motor Function Scale (GMFCS) IV–V, 3 patients was a Pantothenate kinase deficit-associated neurodegeneration (PKAN), 2 had Acquired Brain Damage, and the remaining 3 had, respectively, 2 glutaric aciduria type I (GA-1), and poly-malformative syndrome. In all patients we observed a period of clinical stability after IBT, we call this period “honeymoon”. Two patients died while in the honeymoon period, at 24.9 and 19.6 months from implantation of the pump; the median of duration of the honeymoon period in the remaining 14 was 14.4 months (IQ: 8.3–25.8). ConclusionsIBT was not only used in patients with non-progressive diseases, but also in the group of patients with neurodegenerative or progressive diseases. In all of them, after implantation of the device, we have objectified a period of clinical stability and a better control of muscle tone disorders.