Ross M. Hays

Pediatric Palliative Care Patients: A Prospective Multicenter Cohort Study

OBJECTIVE: To describe demographic and clinical characteristics and outcomes of patients who received hospital-based pediatric palliative care (PPC) consultations. DESIGN, SETTING, AND PATIENTS: Prospective observational cohort study of all patients served by 6 hospital-based PPC teams in the United States and Canada from January to March 2008. RESULTS: There were 515 new (35.7%) or established (64.3%) patients who received care from the 6 programs during the 3-month enrollment interval. Of these, 54.0% were male, and 69.5% were identified as white and 8.1% as Hispanic. Patient age ranged from less than one month (4.7%) to 19 years or older (15.5%). Of the patients, 60.4% lived with both parents, and 72.6% had siblings. The predominant primary clinical conditions were genetic/congenital (40.8%), neuromuscular (39.2%), cancer (19.8%), respiratory (12.8%), and gastrointestinal (10.7%). Most patients had chronic use of some form of medical technology, with gastrostomy tubes (48.5%) being the most common. At the time of consultation, 47.2% of the patients had cognitive impairment; 30.9% of the cohort experienced pain. Patients were receiving many medications (mean: 9.1). During the 12-month follow-up, 30.3% of the cohort died; the median time from consult to death was 107 days. Patients who died within 30 days of cohort entry were more likely to be infants and have cancer or cardiovascular conditions. CONCLUSIONS: PPC teams currently serve a diverse cohort of children and young adults with life-threatening conditions. In contrast to the reported experience of adult-oriented palliative care teams, most PPC patients are alive for more than a year after initiating PPC.

Selective dorsal rhizotomy: efficacy and safety in an investigator‐masked randomized clinical trial

The objective of this single‐center investigator‐masked randomized clinical trial was to investigate the efficacy and safety of selective dorsal rhizotomy (SDR) in children with spastic diplegia. Forty‐three children with spastic diplegia were randomly assigned on an intention‐to‐treat basis to receive SDR plus physical therapy (PT), or PT alone. Thirty‐eight children completed follow‐up through 24 months. Twenty‐one children received SDR (SDR+PT group) and 17 received PT (PT Only group). SDR was guided with electrophysiological monitoring and performed by one experienced neurosurgeon. All subjects received equivalent PT. Spasticity was quantified with an electromechanical torque measurement device (spasticity measurement system [SMS]). The Gross Motor Function Measure (GMFM) was used to document changes in functional mobility. Primary outcome measures were collected at baseline, 6, 12, and 24 months by evaluators masked to treatment.

Botulinum Toxin for Spasticity in Children With Cerebral Palsy: A Comprehensive Evaluation

BACKGROUND. Spasticity is a prevalent disabling clinical symptom for children with cerebral palsy. Treatment of spasticity with botulinum toxin in children with cerebral palsy was first reported in 1993. Botulinum toxin provides a focal, controlled muscle weakness with reduction in spasticity. Interpretation of the literature is difficult because of the paucity of reliable measures of spasticity and challenges with measuring meaningful functional changes in children with disabilities. OBJECTIVE. This study documents the effects of botulinum toxin A injections into the gastrocnemius muscles in children with spastic diplegia. Outcomes are evaluated across all 5 domains of the National Centers for Medical and Rehabilitation Research domains of medical rehabilitation. METHODS. A randomized, double-masked, placebo-controlled design was applied to 33 children with spastic diplegia with a mean age of 5.5 and Gross Motor Function Classification System Levels of I through III. Participants received either 12 U/kg botulinum toxin A or placebo saline injections to bilateral gastrocnemius muscles. Outcomes were measured at baseline and 3, 8, 12, and 24 weeks after injection. RESULTS. Significant decreases in the electromyographic representation of spasticity were documented 3 weeks after botulinum toxin A treatment. A significant decrease in viscoelastic aspects of spasticity was present at 8 weeks, and subsequent increases in dorsiflexion range were documented at 12 weeks for the botulinum toxin A group. Improvement was found in performance goals at 12 weeks and in maximum voluntary torque and gross motor function at 24 weeks for the botulinum toxin A. There were no significant differences between groups in satisfaction with performance goals, energy expenditure, Ashworth scores, or frequency of adverse effects. CONCLUSIONS. The safety profile of 12 U/kg of botulinum toxin A is excellent. Although physiologic and mechanical effects of treatment with botulinum toxin A were documented with functional improvement at 6 months, family satisfaction with outcomes were no different. Communication is needed to ensure realistic expectations of treatment.

THE ROLE OF SELECTIVE DORSAL RHIZOTOMY IN cérébral PALSY: CRITICAL EVALUATION OF A PROSPECTIVE CLINICAL SERIES

This is a prospective observational study of a consecutive series of 34 children with spastic cérébral palsy treated at a single center. 10 had spastic quadriplegia and 24 had spastic diplegia. AH were followed for at least one year. After selective dorsal rhizotomy (SDR), all children received one month of physical therapy at the center and were prescribed a program of physical therapy in their community. The children were assessed before and one year after SDR and physical therapy, using the Ashworth Scale, deep tendon reflex response, range of motion and the Gross Motor Function Measure. The results show that there is often a decrease in lower‐extremity spasticity and functional improvement after SDR with physical therapy, but that there is considerable variability in outcome. Randomized prospective clinical trials with masked objective outcome measures are needed to determine the efficacy of SDR.

Choosing and using screening criteria for palliative care consultation in the ICU: A report from the improving palliative care in the ICU (IPAL-ICU) advisory board

Objective:To review the use of screening criteria (also known as “triggers”) as a mechanism for engaging palliative care consultants to assist with care of critically ill patients and their families in the ICU. Data Sources:We searched the MEDLINE database from inception to December 2012 for all English-language articles using the terms “trigger,” “screen,” “referral,” “tool,” “triage,” “case-finding,” “assessment,” “checklist,” “proactive,” or “consultation,” together with “intensive care” or “critical care” and “palliative care,” “supportive care,” “end-of-life care,” or “ethics.” We also hand-searched reference lists and author files and relevant tools on the Center to Advance Palliative Care website. Study SelectionTwo members (a physician and a nurse with expertise in clinical research, intensive care, and palliative care) of the interdisciplinary Improving Palliative Care in the ICU Project Advisory Board presented studies and tools to the full Board, which made final selections by consensus. Data ExtractionWe critically reviewed the existing data and tools to identify screening criteria for palliative care consultation, to describe methods for selecting, implementing, and evaluating such criteria, and to consider alternative strategies for increasing access of ICU patients and families to high-quality palliative care. Data SynthesisThe Improving Palliative Care in the ICU Advisory Board used data and experience to address key questions relating to: existing screening criteria; optimal methods for selection, implementation, and evaluation of such criteria; and appropriateness of the screening approach for a particular ICU. Conclusions:Use of specific criteria to prompt proactive referral for palliative care consultation seems to help reduce utilization of ICU resources without changing mortality, while increasing involvement of palliative care specialists for critically ill patients and families in need. Existing data and resources can be used in developing such criteria, which should be tailored for a specific ICU, implemented through an organized process involving key stakeholders, and evaluated by appropriate measures. In some settings, other strategies for increasing access to palliative care may be more appropriate.

Integrating Palliative Care in the Surgical and Trauma Intensive Care Unit: A Report From the Improving Palliative Care in the Intensive Care Unit (IPAL-ICU) Project Advisory Board and the Center to Advance Palliative Care

Objective:Although successful models for palliative care delivery and quality improvement in the intensive care unit have been described, their applicability in surgical intensive care unit settings has not been fully addressed. We undertook to define specific challenges, strategies, and solutions for integration of palliative care in the surgical intensive care unit. Data Sources:We searched the MEDLINE database from inception to May 2011 for all English language articles using the term “surgical palliative care” or the terms “surgical critical care,” “surgical ICU,” “surgeon,” “trauma” or “transplant,” and “palliative care” or “end-of- life care” and hand-searched our personal files for additional articles. Based on review of these articles and the experiences of our interdisciplinary expert Advisory Board, we prepared this report. Data Extraction and Synthesis:We critically reviewed the existing literature on delivery of palliative care in the surgical intensive care unit setting focusing on challenges, strategies, models, and interventions to promote effective integration of palliative care for patients receiving surgical critical care and their families. Conclusions:Characteristics of patients with surgical disease and practices, attitudes, and interactions of different disciplines on the surgical critical care team present distinctive issues for intensive care unit palliative care integration and improvement. Physicians, nurses, and other team members in surgery, critical care and palliative care (if available) should be engaged collaboratively to identify challenges and develop strategies. “Consultative,” “integrative,” and combined models can be used to improve intensive care unit palliative care, although optimal use of trigger criteria for palliative care consultation has not yet been demonstrated. Important components of an improvement effort include attention to efficient work systems and practical tools and to attitudinal factors and “culture” in the unit and institution. Approaches that emphasize delivery of palliative care together with surgical critical care hold promise to better integrate palliative care into the surgical intensive care unit. (Crit Care Med 2012; 40:–1206)

Quantitative measurement of spasticity in children with cerebral palsy

Spasticity was quantified in nine children with spastic diplegia, using a sinusoidal displacement of the foot at frequencies from 3 to 12Hz. Ankle‐joint stiffness was separated into elastic (energystoring) and viscous (energy‐dissipating) components. ‘Path length’ was used to represent the variation in stiffness over this frequency range. Compared with 11 unaffected children, a significant difference in path lengths was demonstrated for the children with spasticity. An age‐dependent effect was demonstrated when path lengths of unaffected children were compared with those of 10 unaffected adults. A modified path‐length measure is proposed which minimizes age dependency, yet enables detection of spasticity. Passive stiffness properties of unaffected adults showed higher elastic stiffness, viscosity and friction than unaffected children. A method was developed to evade the need for temporary nerve blocks to calculate inertial properties of the foot in persons with spasticity.

Palliative care in the ICU: relief of pain, dyspnea, and thirst—A report from the IPAL-ICU Advisory Board

AbstractPurposePain, dyspnea, and thirst are three of the most prevalent, intense, and distressing symptoms of intensive care unit (ICU) patients. In this report, the interdisciplinary Advisory Board of the Improving Palliative Care in the ICU (IPAL-ICU) Project brings together expertise in both critical care and palliative care along with current information to address challenges in assessment and management.MethodsWe conducted a comprehensive review of literature focusing on intensive care and palliative care research related to palliation of pain, dyspnea, and thirst.ResultsEvidence-based methods to assess pain are the enlarged 0–10 Numeric Rating Scale (NRS) for ICU patients able to self-report and the Critical Care Pain Observation Tool or Behavior Pain Scale for patients who cannot report symptoms verbally or non-verbally. The Respiratory Distress Observation Scale is the only known behavioral scale for assessment of dyspnea, and thirst is evaluated by patient self-report using an 0–10 NRS. Opioids remain the mainstay for pain management, and all available intravenous opioids, when titrated to similar pain intensity end points, are equally effective. Dyspnea is treated (with or without invasive or noninvasive mechanical ventilation) by optimizing the underlying etiological condition, patient positioning and, sometimes, supplemental oxygen. Several oral interventions are recommended to alleviate thirst. Systematized improvement efforts addressing symptom management and assessment can be implemented in ICUs.ConclusionsRelief of symptom distress is a key component of critical care for all ICU patients, regardless of condition or prognosis. Evidence-based approaches for assessment and treatment together with well-designed work systems can help ensure comfort and related favorable outcomes for the critically ill.

Technology-dependency among patients discharged from a children's hospital: a retrospective cohort study

BackgroundAdvances in medical technology may be increasing the population of children who are technology-dependent (TD). We assessed the proportion of children discharged from a childrens hospital who are judged to be TD, and determined the most common devices and number of prescription medications at the time of discharge.MethodsChart review of 100 randomly selected patients from all services discharged from a childrens hospital during the year 2000. Data were reviewed independently by 4 investigators who classified the cases as TD if the failure or withdrawal of the technology would likely have adverse health consequences sufficient to require hospitalization. Only those cases where 3 or 4 raters agreed were classified as TD.ResultsAmong the 100 randomly sampled patients, the median age was 7 years (range: 1 day to 24 years old), 52% were male, 86% primarily spoke English, and 54% were privately insured. The median length of stay was 3 days (range: 1 to 103 days). No diagnosis accounted for more than 5% of cases. 41% were deemed to be technology dependent, with 20% dependent upon devices, 32% dependent upon medications, and 11% dependent upon both devices and medications. Devices at the time of discharge included gastrostomy and jejeunostomy tubes (10%), central venous catheters (7%), and tracheotomies (1%). The median number of prescription medications was 2 (range: 0–13), with 12% of cases having 5 or more medications. Home care services were planned for 7% of cases.ConclusionTechnology-dependency is common among children discharged from a childrens hospital.

Central ventilatory dysfunction in myelodysplasia: an independent determinant of survival

Central ventilatory dysfunction (CVD) is a significant complication of myelodysplasia with Arnold‐Chiari type II defect. The records of 616 patients with myelodysplasia were reviewed and CVD was documented in 35 cases (5·7 per cent): it was the single most common cause of death in this population. There was no significant association between CVD and level of lesion, severity of hydrocephalus, hemorrhage or infection. The effects of CVD may be variable, with some patients having spontaneous remission and others having severe, often fatal, disruption of ventilation.