Development and pilot test of a pregnancy decision making tool for women with physical disabilities.

OBJECTIVE: Design and pilot test a new decision making tool for women with physical disabilities (impairment of physical function due to chronic conditions) considering pregnancy. DATA SOURCES AND STUDY SETTING: Quantitative surveys and qualitative interviews were collected from participants living in the community. STUDY DESIGN: Clinical guidelines and survey and focus group data about pregnancy informational and decisional needs guided content development. The tool was pilot tested in a 12-week trial with participants with physical disabilities considering or actively planning a pregnancy. Feasibility outcomes were acceptability, implementation, and demand (collected at end of the trial); preliminary efficacy focused on decisional conflict and readiness (baseline, 6 weeks, and end of trial). DATA COLLECTION: Survey data were collected using an online form. One-on-one interviews were conducted to learn more about experience using the tool. PRINCIPAL FINDINGS: Thirty eight participants with mild, moderate, or severe physical disabilities participated. Feasibility outcomes indicated that the tool provided participants with information, guiding questions, and helped them to consider multiple aspects of the decision about pregnancy. Most participants responded positively to the new decision making tool, finding it easy to use and the information balanced. Feedback highlighted opportunity for improvement, such as more specific information, peer stories, and the limitations of a paper format. There was significant linear effect of time, with increased decisional certainty and readiness, values clarity, and decisional support (partial η2 [90% CI] = 0.310 [0.08, 0.46], 0.435 [0.19, 0.60], 0.134 [0, 0.29], 0.178 [0.01, 0.35], respectively). Decisional certainty and readiness had high observed power (96.7% and 99.3%, respectively) with lower observed power for clarity and support (60.6% and 75.1%, respectively). CONCLUSIONS: The new tool shows promise for supporting women with physical disabilities in navigating pregnancy decision making. Future development of complementary strategies to support health care providers will help improve shared decision making and patient-centered care.

Post-fracture rehabilitation pathways and association with mortality among adults with cerebral palsy.

OBJECTIVE: Rehabilitation may mitigate the high mortality rates and health declines post-fracture for adults with cerebral palsy, but this is understudied. The objectives were to characterize the post-fracture rehabilitation pathways and identify their association with 1-year survival among adults with cerebral palsy. METHODS: A retrospective cohort study of adults with cerebral palsy with a fragility fracture with continuous health plan enrollment ≥1-year prior to and ≥1 day after their fracture date was performed using a random 20% Medicare fee-for-service dataset. Participants were categorized as a home discharge or inpatient rehabilitation admission post-fracture. For the home discharge cohort, weekly exposure to outpatient physical/occupational therapy (PT/OT) was examined up to 6-month post-fracture. Cox regression examined the association between time-varying PT/OTuse within 6-month post-fracture and mortality from 30 days to 1-year post-fracture before and after adjusting for confounders (e.g. medical complexity). RESULTS: Of 3598 adults with cerebral palsy with an incident fragility fracture, 74% were discharged home without inpatient rehabilitation; they were younger, but more medically complex compared to the 26% admitted to inpatient rehabilitation. Among the home discharge cohort (n = 2662), 43.1% initiated PT/OTwithin 6-month post-fracture, and cumulative PT/OTexposure post-fracture was associated with improved survival; for example, per 3 weeks of PT/OTexposure, the adjusted mortality rate was 40% lower (95% confidence interval (CI) = 0.41-0.89). CONCLUSIONS: Most adults with cerebral palsy with a fragility fracture were discharged home rather than to inpatient rehabilitation, and only 43.1% of that group initiated outpatient PT/OTwithin 6 months post-fracture. Receiving outpatient PT/OTwas associated with improved 1-year survival.

Post-Fracture Inpatient and Outpatient Physical/Occupational Therapy and Its Association with Survival among Adults with Cerebral Palsy.

Physical and/or occupational therapy (PT/OT) may improve post-fracture health and survival among adults with cerebral palsy (CP), but this has not been studied in the inpatient setting. The objective was to quantify the association between acute inpatient and outpatient PT/OT use with 1-year mortality among adults with CP. This was a retrospective cohort study of adults with CP with an incident fragility fracture admitted to an acute care or rehabilitation facility using a random 20% Medicare fee-for-service dataset. Acute care/rehabilitation PT/OT was measured as the average PT/OT cost/day for the length of stay (LOS). Weekly exposure to outpatient PT/OT was examined up to 6 months post-fracture. Cox regression examined the adjusted association between the interaction of acute care/rehabilitation average PT/OT cost/day and LOS with 1-year mortality. A separate Cox model added time-varying outpatient PT/OT. Of 649 adults with CP, average PT/OT cost/day was associated with lower mortality rate for LOS < 17 days (HR range = 0.78−0.93), and increased mortality rate for LOS > 27 days (HR ≥ 1.08) (all, p < 0.05). After acute care/rehabilitation, 44.5% initiated outpatient PT/OT, which was associated with lower mortality rate (HR = 0.52; 95% CI = 0.27−1.01). Post-fracture inpatient and outpatient PT/OT were associated with improved 1-year survival among adults with CP admitted to acute care/rehabilitation facilities.

Musculoskeletal Morbidity Among Adults Living With Spina Bifida and Cerebral Palsy.

Background: Individuals living with cerebral palsy (CP) or spina bifida (SB) are at heightened risk for chronic health conditions that may develop or be influenced by the impairment and/or the process of aging. Objectives: The objective of this study was to compare the incidence of and adjusted hazards for musculoskeletal (MSK) morbidities among adults living with and without CP or SB. Methods: A retrospective, longitudinal cohort study was conducted among adults living with (n = 15,302) CP or SB and without (n = 1,935,480) CP or SB. Incidence estimates of common MSK morbidities were compared at 4 years of enrollment. Survival models were used to quantify unadjusted and adjusted hazard ratios for incident MSK morbidities. The analyses were performed in 2019 to 2020. Results: Adults living with CP or SB had a higher 4-year incidence of any MSK morbidity (55.3% vs. 39.0%) as compared to adults without CP or SB, and differences were to a clinically meaningful extent. Fully adjusted survival models demonstrated that adults with CP or SB had a greater hazard for all MSK disorders; this ranged from hazard ratio (HR) 1.40 (95% CI, 1.33 to 1.48) for myalgia to HR 3.23 (95% CI, 3.09 to 3.38) for sarcopenia and weakness. Conclusion: Adults with CP or SB have a significantly higher incidence of and risk for common MSK morbidities as compared to adults without CP or SB. Efforts are needed to facilitate the development of improved clinical screening algorithms and early interventions to reduce risk of MSK disease onset/progression in these higher risk populations.

Opioid prescription patterns among adults with cerebral palsy and spina bifida.

Background: Pain is the most common symptom of cerebral palsy and spina bifida (CP/SB). The objective of this study was to compare the opioid prescription patterns for differing pain types and overlapping pain among adults living with and without CP/SB. Methods: Privately-insured beneficiaries were included if they had CP/SB (n = 22,647). Adults without CP/SB were also included as controls (n = 931,528). Oral morphine equivalents (OMEs) were calculated. A multivariable logistic regression was used to analyze the association between CP/SB and OMEs, across the three pain categories: (1) no pain, (2) isolated pain, and (3) pain multimorbidity. Results: Adults living with CP/SB had a higher OME prescription pattern per year than adults without CP or SB (8,981.0 ± 5,183.0 vs. 4,549.1 ± 2,988.0), and for no pain (4,010.8 ± 828.1 vs. 1,623.53 ± 47.5), isolated pain (7,179.9 ± 378.8 vs. 3,531.0 ± 131.0), and pain multimorbidity (15,752.4 ± 1,395.5 vs. 8,492.9 ± 398.0) (all p < 0.001), and differences were to a clinically meaningful extent. Adjusted odds ratios (OR) for prescribed OMEs were higher for adults with CP/SB vs. control and (1) no pain (OR: 1.51; 95%CI: 1.46, 1.56), (2) isolated pain (OR: 1.48; 95%CI: 1.44, 1.52), and (3) pain multimorbidity (OR: 1.79; 95%CI: 1.72, 1.86). Conclusions: Adults with CP/SB obtain significantly higher prescription of OMEs than adults without CP/SB.

Physical and occupational therapy utilization and associated factors among adults with cerebral palsy: Longitudinal modelling to capture distinct utilization groups.

BACKGROUND: Adults with cerebral palsy (CP) experience functional declines. Clinical rehabilitation may preserve function for this population. OBJECTIVE: To identify longitudinal physical/occupational therapy use and associated factors among adults with CP, to inform health promotion strategies. METHODS: A retrospective cohort study including adults ≥ 18 years of age with CP was performed using a random 20% Medicare fee-for-service dataset. Participants with continuous medicare enrolment from 01/01/2016-12/31/2018 were included: 2016 was the one-year baseline period; 2017-2018 was the two-year follow-up. Therapy included an indication of physical, occupational, or other forms of therapy. Two-year therapy use patterns were identified using group-based trajectory modeling. Multivariable multinomial logistic regression models identified associations between baseline characteristics with trajectory groups. RESULTS: Of 17,441, 7231 (41.5%) adults with CP had therapy use across the three-year period, and six longitudinal therapy trajectories were identified: the majority (42.5%) were low-consistent users, 13.4% moderate-consistent users, 4.4% high-consistent users, and the remaining variable users. Associations between baseline characteristics (e.g., age, sex, comorbidities) with trajectory groups varied. For example, using the low-consistent users as the reference, Black versus White were 49% less likely, Northeast versus South residency were 7.52-fold more likely, and co-occurring neurologic conditions versus CP only were up to 118% more likely to be high-consistent users (all, P < 0.05). Bone fragility and some chronic comorbidities were associated with moderate consistent users. CONCLUSIONS: The majority of adults with CP were not using physical/occupational therapy. Of those that did, there were unique longitudinal trajectories which associated differently with demographics and comorbidities.

Navigating the Pathway to Care in Adults With Cerebral Palsy.

As individuals with cerebral palsy (CP) age, they face unique challenges which complicate their ability to access and receive appropriate health care. These problems exist at the level of the health care system, the clinician, and the individual. At the system level, there is an inadequate number of professionals who are informed of and interested in the care of adults with CP. Pediatric clinicians prefer treating children, and adult caregivers are not knowledgeable about and may feel less competent about CP. Pediatric care does not translate well to the adult population, and information about best practices for adults is just starting to develop. Differences in the physiologic development of individuals with CP render well-established clinical protocols for risk screening of chronic diseases less effective. Moreover, lack of supportive resources decreases a caregiver's sense of self-efficacy in treating this population. The patient's ability to navigate these barriers is complicated by the high prevalence of comorbid cognitive impairment and mental health issues including anxiety, depression, and other psychiatric disorders; a bidirectional relationship between challenges in navigating care/needs and comorbid mental health conditions appears likely. Many patients have additional barriers related to social determinants of health, such as access to transportation, accessible health care facilities, and other personal and environmental factors that may impede health maintenance and wellness. Increasing and disseminating knowledge, harnessing the power of new technologies such as telemedicine, and addressing mental health issues are some of the methods that are available to help adults with CP navigate this road.

Pain phenotypes among adults living with cerebral palsy and spina bifida.

ABSTRACT: Chronic pain is the most commonly reported physical symptomology of cerebral palsy (CP) and spina bifida (SB) throughout the lifespan, and yet, pain is perhaps the least understood comorbidity in these populations. The objective of this study was to compare the prevalence and types of pain diagnosed among adults living with and without CP or SB. In this retrospective cohort study, we analyzed data from a nationwide commercial insurance claims database. Beneficiaries were included if they had an International Classification of Diseases, Ninth revision, Clinical Modification diagnosis code for CP or SB (n = 22,648). Adults without CP or SB were also included as controls (n = 931,623). Pain phenotypes (nociceptive, nociplastic, and neuropathic pain) and pain multimorbidity (≥2 conditions) were compared. We found that adults living with CP or SB had a higher prevalence of any pain disorders (55.9% vs 35.2%), nociceptive pain (44.0% vs 26.7%), nociplastic pain (26.1% vs 11.9%), neuropathic pain (9.6% vs 5.6%), and pain multimorbidity (21.1% vs 8.4%), as compared to adults without CP or SB, and differences were to a clinically meaningful extent. Adjusted odds ratios of nociceptive pain (odds ratio [OR]: 2.20; 95% confidence interval [CI]: 2.15-2.24), nociplastic pain (OR: 2.47; 95% CI: 2.41-2.53), neuropathic pain (OR: 2.71; 95% CI: 2.54-2.89), and other pain (OR: 3.92; 95% CI: 3.67-4.19) were significantly higher for adults living with CP or SB. In conclusion, adults with CP or SB have a significantly higher prevalence and odds of common peripheral, central, and neuropathic pain disorders and pain multimorbidity, as compared to adults without CP or SB.

Timecourse of Morbidity Onset Among Adults Living With Cerebral Palsy.

INTRODUCTION: Despite the greater risk of an array of morbidities, little is known about when morbidities occur for adults with cerebral palsy. The objective of this study is to determine the timecourse of morbidity risk/development for adults with cerebral palsy and the effect by patient-level factors. METHODS: Cross-sectional data from 2016 were used from a random 20% sample from the fee-for-service Medicare database. Diagnosis codes identified adults aged ≥18 years with cerebral palsy and 16 clinically relevant morbidities. Qualitative and quantitative approaches identified the age where each morbidity became exceedingly prevalent. The effect of the timecourse by sex, race, and co-occurring intellectual disabilities and epilepsy was examined. Data were sequestered and analyzed in 2020. RESULTS: Among 16,818 adults with cerebral palsy, the prevalence of most morbidities was already high among those aged 18-30 years, and all morbidities increased with age except liver disease and anxiety. Hypertension and diabetes exhibited a positive linear trend with age. Of the morbidities that did not exhibit a linear trend, the qualitative and quantitative approaches were consistent considering that the cardiorespiratory diseases, osteoarthritis, renal disease, and dementia became exceedingly more prevalent at age >50 years, whereas the threshold was >60 years for depression, cancer, and metastatic cancer. There were interactions with sex, race, and co-occurring intellectual disabilities and epilepsy for some of the morbidities. CONCLUSIONS: Morbidity prevalence is already elevated early in adulthood among individuals living with cerebral palsy, with an abrupt increase by age 50 years. Preventive efforts should be adopted early in the lifespan and not later than age 50 years for adults with cerebral palsy.

Development of a new pregnancy informational and decisional needs survey for women with physical disabilities.

BACKGROUND: While the literature on pregnancy and disability is growing, generating important knowledge of barriers to care and health risks, there is limited literature on pregnancy decision-making and informational needs. Such knowledge is critical for the development of interventions to mitigate the challenges women with disabilities and health care providers face in making this important decision. OBJECTIVE: /Hypothesis: Develop a survey of pregnancy decisional and informational needs of women with physical disabilities. METHODS: We developed a conceptual framework that guided item writing, defined the characteristics of the target population, engaged stakeholders with disabilities to collaborate with investigators, and tested the interpretability and relevance of items, and preferred mode of responding (online vs. telephone). A total of 123 women participated in cognitive interviews (N = 13), mode testing (N = 10), or survey data collection (N = 114). RESULTS: Instructions, three screening questions, and 156 items were tested in one round of 17 cognitive interviews; 25 items were deleted, 94 were revised, and 37 items were retained without changes. The final version of the survey included six sub-sections: the experience of making a decision; information about pregnancy and disability; things affecting a decision; knowing what is important; support for making a decision; and working with health care providers. CONCLUSIONS: The overall performance of the survey supported its content validity and utility as a useful way to gather information about the pregnancy decision-making experience and informational needs of women with physical disabilities.

Polypharmacy is a risk factor for mortality, severe chronic kidney disease, and liver disease among privately insured adults with cerebral palsy.

BACKGROUND: Adults with cerebral palsy (CP) have an increased risk for polypharmacy, premature mortality, and early development of several morbidities, including conditions associated with excess medication exposure, such as chronic kidney disease (CKD) and liver disease. To date, very little is known about the consequence of polypharmacy for adults with CP. OBJECTIVE: To determine if polypharmacy is associated with an increased risk for mortality, severe CKD, and liver disease among adults with CP, before and after adjusting for comorbid neurodevelopmental disabilities (NDDs) and multimorbidity. METHODS: This is an exploratory treatment effectiveness study. Data from the Optum Clinformatics Data Mart were used for this retrospective cohort study. Adults aged 18 years or older with a diagnosis of CP and without severe CKD (stages IV+) and liver disease were identified from the calendar year 2013 and were subsequently followed from January 1, 2014, to death, severe CKD, liver disease, loss to follow-up, or end of study period (December 31, 2017). Diagnosis codes were used to identify NDDs (intellectual disabilities, epilepsy, autism spectrum disorders, spina bifida) and 24 relevant morbidities at baseline (i.e., calendar year 2013). Polypharmacy was defined as ≥ 5 medications and hyperpolypharmacy was defined as ≥ 10 medications at baseline. Cox regression models were developed to examine the association (as HR and 95% CI) between polypharmacy and hyperpolypharmacy with mortality, severe CKD, and liver disease separately, before and after adjusting for covariates (demographics, NDDs, multimorbidity). Exploratory analyses examined the mediating effect of incident severe CKD or liver disease on the association between the exposure (polypharmacy or hyperpolypharmacy) on outcomes. RESULTS: Of the 9,238 adults with CP, 58.5% had polypharmacy and 29.5% had hyperpolypharmacy. The fully adjusted HR for mortality was 2.14 (95% CI = 1.59-2.89) for polypharmacy and 1.65 (95% CI = 1.31-2.09) for hyperpolypharmacy. The fully adjusted HR for severe CKD was 1.66 (95% CI = 1.17-2.36) for polypharmacy and 1.67 (95% CI = 1.27-2.19) for hyperpolypharmacy. The fully adjusted HR for liver disease was 1.57 (95% CI = 1.27-1.94) for polypharmacy and 1.72 (95% CI = 1.42-2.08) for hyperpolypharmacy. Incident liver disease mediated 5.37% (polypharmacy) and 7.54% (hyperpolypharmacy) of the association between the exposure with incident severe CKD for nonelderly (aged < 65 years), while incident severe CKD mediated 7.05% (polypharmacy) and 6.64% (hyperpolypharmacy) of the association between the exposure with incident liver disease for elderly (aged ≥ 65 years). CONCLUSIONS: Polypharmacy and hyperpolypharmacy are robust risk factors for risk of mortality, severe CKD, and liver disease among privately insured adults with CP. While incidence of severe CKD and liver disease had negligible effects on the association between polypharmacy with mortality, there is evidence that they mediate a considerable portion of one another and require further examination. DISCLOSURES: During the work for this study, Whitney was supported by the University of Michigan Office of Health Equity and Inclusion Diversity Fund and American Academy for Cerebral Palsy and Developmental Medicine. The funding sources had no role in the design or conduct of the study; collection, management, analysis, or interpretation of the data; preparation, review, or approval of the manuscript; or the decision to submit the manuscript for publication. The authors have no conflicts of interest to report.

Pain Phenotypes in Adults Living With Cerebral Palsy.

BACKGROUND AND OBJECTIVES: To identify pain phenotypes among adults living with cerebral palsy (CP) and compare phenotypes of pain intensity, anxiety and depressive symptoms, and self-reported perceived stress. METHODS: Seventy-one adults with CP presented to the University of Michigan (mean age = 39.3 ± 16.2; 43 women, 28 men). The median of 6 on the American College of Rheumatology fibromyalgia survey was used to classify patients for nociplastic pain centralization. The painDETECT Score was used to classify patients for neuropathic pain. These measures were then used to cross-classify each patient into 1 of 4 possible pain categories: neuropathic, nociplastic, mixed neuropathic/noclipastic, or nociceptive pain (-neuropathic/-nociplastic pain). RESULTS: Twenty-eight adults with CP (39.4%) were classified as nociceptive, 24 (33.8%) as nociplastic, 8 (11.3%) as neuropathic, and 11 (15.5%) as mixed neuropathic/nociplastic. Subgroups differed significantly on average scores on the Brief Pain Inventory pain intensity scale, the Perceived Stress Scale, and on the Patient-Reported Outcomes Measurement Information System measures of anxiety and depression; the nociceptive pain subgroup reported lower pain and emotional distress compared with the other groups. DISCUSSION: Findings suggest that type of pain is variable among adults with CP and may arise through multiple mechanisms.

Psychological morbidity among adults with cerebral palsy and spina bifida.

BACKGROUND: Very little is known about the risk of developing psychological morbidities among adults living with cerebral palsy (CP) or spina bifida (SB). The objective of this study was to compare the incidence of and adjusted hazards for psychological morbidities among adults with and without CP or SB. METHODS: Privately insured beneficiaries were included if they had an International Classification of Diseases, Ninth revision, Clinical Modification diagnostic code for CP or SB (n = 15 302). Adults without CP or SB were also included (n = 1 935 480). Incidence estimates of common psychological morbidities were compared at 4-years of enrollment. Survival models were used to quantify unadjusted and adjusted hazard ratios for incident psychological morbidities. RESULTS: Adults living with CP or SB had a higher 4-year incidence of any psychological morbidity (38.8% v. 24.2%) as compared to adults without CP or SB, and differences were to a clinically meaningful extent. Fully adjusted survival models demonstrated that adults with CP or SB had a greater hazard for any psychological morbidity [hazard ratio (HR): 1.60; 95% CI 1.55-1.65], and all but one psychological disorder (alcohol-related disorders), and ranged from HR: 1.32 (1.23, 1.42) for substance disorders, to HR: 4.12 (3.24, 5.25) for impulse control disorders. CONCLUSIONS: Adults with CP or SB have a significantly higher incidence of and risk for common psychological morbidities, as compared to adults without CP or SB. Efforts are needed to facilitate the development of improved clinical screening algorithms and early interventions to reduce the risk of disease onset/progression in these higher-risk populations.

Polypharmacy Among Privately Insured Adults with Cerebral Palsy: A Retrospective Cohort Study.

BACKGROUND: Adults with cerebral palsy (CP) have increased risk for developing various secondary chronic diseases, especially when they have other neurodevelopmental disabilities (NDDs). Multiple medications are likely prescribed to manage the greater morbidity-related burden for adults with CP; however, because health care delivery and care coordination is suboptimal for this population, adults with CP may have an increased risk for polypharmacy. To date, very little is known about the prescribing practices and extent of polypharmacy for adults with CP. OBJECTIVE: To determine the prevalence and adjusted odds of polypharmacy among adults with CP only and those with CP+NDDs, compared with adults without CP. METHODS: Data from 2017 Optum Clinformatics Data Mart, a U.S. private administrative database, was used for this retrospective cohort study. Diagnosis codes were used to identify adults (aged ≥ 18 years) with CP, NDDs (e.g., intellectual disabilities, epilepsy, and autism spectrum disorders), and 24 relevant morbidities. Polypharmacy was examined as 0-4 versus ≥ 5, 0-9 versus ≥ 10, and 0-14 versus ≥ 15 medications. Logistic regression estimated the OR and 95% CI of polypharmacy before and after adjusting for age, sex, region of residence, and multimorbidity (as 0, 1, 2, 3, 4-5, and ≥ 6 morbidities). Exploratory analyses were conducted to compare polypharmacy among young (18-40 years) and middle-aged (41-64 years) adults with CP only and CP + NDDs with elderly (≥ 65 years) adults without CP. RESULTS: Adults with CP only (n = 5,603) and CP + NDDs (n = 2,474) had higher unadjusted prevalence and adjusted OR for each polypharmacy definition compared with adults without CP (n = 9.0 million; e.g., ≥ 5 medications: adjusted OR for CP only = 1.38, 95% CI = 1.30-1.47; CP + NDDs: OR = 2.42, 95% CI = 2.20-2.67). Adults with CP+NDDs had higher unadjusted prevalence and adjusted OR of each polypharmacy definition compared with CP only. Compared with elderly without CP, the unadjusted prevalence of polypharmacy was lower for young adults with CP only (e.g., ≥ 5 medications: 60.2%, 43.8%), similar for young adults with CP+NDDs (e.g., ≥ 15 medications: 10.9%, 12.5%), and elevated for middle-aged CP only and CP + NDDs (e.g., ≥ 10 medications: 28.7%, 34.3%, 41.7%). CONCLUSIONS: Privately insured adults with CP only and CP + NDDs have an elevated prevalence of polypharmacy compared with adults without CP, even after accounting for multimorbidity. Importantly, adults aged 18-40 years with CP have a similar (CP + NDDs) prevalence of polypharmacy compared with the general geriatric population, with the prevalence increasing further for CP by middle age. DISCLOSURES: Whitney was supported by the University of Michigan Office of Health Equity and Inclusion Diversity Fund and the American Academy of Cerebral Palsy and Developmental Medicine. These funding sources had no role in the design or conduct of the study; collection, management, analysis, or interpretation of the data; preparation, review, or approval of the manuscript; or the decision to submit the manuscript for publication. The other authors have no conflicts of interest to disclose.

Cardiometabolic Morbidity in Adults With Cerebral Palsy and Spina Bifida.

PURPOSE: The purpose of this study was to compare the incidence of, and adjusted hazards for, cardiometabolic morbidities among adults with and without cerebral palsy or spina bifida. METHODS: Privately insured beneficiaries were included if they had an International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) diagnostic code for cerebral palsy or spina bifida (n = 15,302). Adults without cerebral palsy or spina bifida were also included (n = 1,935,480). Incidence estimates of common cardiometabolic morbidities were compared at 4 years of enrollment. Survival models were used to quantify unadjusted and adjusted hazard ratios (HRs) for incident cardiometabolic morbidities. RESULTS: Adults living with cerebral palsy or spina bifida had a higher 4-year incidence of any cardiometabolic morbidity (41.5% vs 30.6%) as compared to adults without cerebral palsy or spina bifida, and differences were to a clinically meaningful extent. Fully adjusted survival models demonstrated that adults with cerebral palsy or spina bifida had a greater hazard for any cardiometabolic morbidity (HR: 1.52; 95% confidence interval [CI]: 1.47, 1.57), and all but 1 cardiometabolic disorder (nonalcoholic fatty liver disease) and ranged from HR: 1.20 (1.15, 1.25) for hypercholesterolemia to HR: 1.86 (1.74, 1.98) for heart failure. CONCLUSIONS: Adults with cerebral palsy or spina bifida have a significantly higher incidence of, and risk for, common cardiometabolic morbidities, as compared to adults without cerebral palsy or spina bifida. Efforts are needed to facilitate the development of improved clinical screening algorithms and early interventions to reduce risk of cardiometabolic disease onset and progression in these higher-risk populations.

Effect of pain on mood affective disorders in adults with cerebral palsy.

AIM: To determine if pain is associated with 12-month incidence of mood affective disorders (MAD) in adults with cerebral palsy (CP). METHOD: Data from Optum Clinformatics® Data Mart (2013-2016) were used for this retrospective cohort study. Diagnostic codes were used to identify adults (≥18y) with CP, incident cases of MAD, and covariates (other neurodevelopmental conditions, sleep disorders, arthritis). Pain (any type, location) was identified between 1st October 2014 and 30th September 2015. The pain group was divided into new or consistent pain if they had a history of pain (i.e. consistent) in the 12 months before their first pain claim date. Crude incidence rates of MAD (expressed per 100 person-years) were estimated. Cox regression was used to estimate hazard ratio (95% confidence interval [CI]) of MAD after adjusting for covariates. RESULTS: Adults that had new pain (n=859; incidence rate=15.5) or consistent pain (n=1303; incidence rate=17.9) had greater crude incidence rate of MAD compared to adults without pain (n=3726; incidence rate=5.9). The elevated rate of MAD remained after adjusting for covariates, for new pain (hazard ratio=2.4; 95% CI=1.9-3.0) and consistent pain (hazard ratio=2.1; 95% CI=1.7-2.7). INTERPRETATION: Pain is associated with greater incidence of MAD in adults with CP. This association remained after accounting for potential confounding factors. WHAT THIS PAPER ADDS: What this paper adds Pain was associated with higher 12-month incidence of mood affective disorders (MAD). The 12-month MAD incidence was similar between new and consistent pain groups. The MAD incidence remained higher adjusting for neurodevelopmental comorbidities, sleep disorders, and arthritis.

Self-report of pain in young people and adults with spastic cerebral palsy: interrater reliability of the revised Face, Legs, Activity, Cry, and Consolability (r-FLACC) scale ratings.

AIM: People with cerebral palsy (CP) are often unable to express pain owing to cognitive or speech impairments. Reports that rely on observation can be inaccurate, because behaviours such as grimacing, common in people with spastic CP, resemble pain expressions. We examined preliminary validity and reliability of the revised Face, Legs, Activity, Cry, and Consolability (r-FLACC) scale in people with spastic CP. METHOD: Forty-eight young people and adults (35 females, 13 males; mean [SD] age 29y 2mo [13y]) were video-recorded during a standard examination, rating their pain (0-10) afterwards. Two raters completed the r-FLACC using the video recordings. Interrater reliability was assessed with an unconditional cross-classified random-effects model and item response theory approach; Pearson correlations measured agreement between raters and participants. RESULTS: Mean (SD) participant (n=48) pain scores were 2.48 (2.5) and mean (SD) r-FLACC scores were 1.46 (1.68). There was moderate agreement between raters (intraclass coefficient 0.41 and 0.57 respectively) but low agreement between participants and raters (r=0.26). There were no significant effects for raters (lay observers, nurses, physicians, and inexperienced raters). INTERPRETATION: Results provide mixed support for the interrater reliability of the r-FLACC in people with spastic CP. WHAT THIS PAPER ADDS: The revised Face, Legs, Activity, Cry, and Consolability (r-FLACC) scale can be reliably used by experts and lay raters for people with spastic cerebral palsy (CP). Support is mixed for interrater reliability of the r-FLACC scale used with people with spastic CP.

Greater Adipose Tissue Distribution and Diminished Spinal Musculoskeletal Density in Adults With Cerebral Palsy.

OBJECTIVES: To examine differences in adipose tissue distribution, lumbar vertebral bone mineral density (BMD), and muscle attenuation in adults with and without cerebral palsy (CP), and to determine the associations between morphologic characteristics. DESIGN: Cross-sectional, retrospective analyses of archived computed tomography scans. SETTING: Clinical treatment and rehabilitation center. PARTICIPANTS: Adults (N=352) with CP (age, 38.8±14.4y; body mass, 61.3±17.1kg; Gross Motor Function Classification System levels, I-V) and a matched cohort of neurotypical adults. Of the 41 adults with CP included in the study, 10 were not matchable because of low body masses. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Computed tomography scans were assessed for visceral adipose tissue (VAT) and subcutaneous adipose tissue (SAT) areas, psoas major area and attenuation in Hounsfield units (Hu), and cortical and trabecular BMDs. RESULTS: Adults with CP had lower cortical (ß=-63.41 Hu, P<.001) and trabecular (ß=-42.24 Hu, P<.001) BMDs and psoas major areas (ß=-374.51mm(2), P<.001) and attenuation (ß=-9.21 Hu, P<.001) after controlling for age, sex, and body mass. Adults with CP had greater VAT (ß=3914.81mm(2), P<.001) and SAT (ß=4615.68mm(2), P<.001). Muscle attenuation was significantly correlated with trabecular (r=.51, P=.002) and cortical (r=.46, P<.01) BMD, whereas VAT was negatively associated with cortical BMD (ß=-.037 Hu/cm(2), r(2)=.13, P=.03). CONCLUSIONS: Adults with CP had lower BMDs, smaller psoas major area, greater intermuscular adipose tissue, and greater trunk adiposity than neurotypical adults. VAT and cortical BMD were inversely associated.

Agreement Between Actual Height and Estimated Height Using Segmental Limb Lengths for Individuals with Cerebral Palsy.

OBJECTIVE: The purpose of this study was to determine the agreement between actual height or segmental length and estimated height from segmental measures among individuals with cerebral palsy. DESIGN: A convenience sample of 137 children and young adults with cerebral palsy (age 2-25 yrs) were recruited from a tertiary care center. Height, body mass, recumbent length, knee height, tibia length, and ulna length were measured. Estimated height was calculated using several common prediction equations. Agreement between measured and estimated height was determined using the Bland-Altman method. RESULTS: Limits of agreement were wide for all equations, usually in the range of ±10 cm. Repeatability of the individual measures was high, with a coefficient of variation of 1%-2% for all measures. The equation using knee height demonstrated a nonuniform difference in which height estimation worsened as overall height increased. CONCLUSIONS: Accurate measurement of height is important but very difficult in individuals with cerebral palsy. Segmental measures are highly repeatable and thus may be used on their own to monitor growth. However, when an accurate measure of height is needed to monitor nutritional status (i.e., for body mass index calculation), caution is warranted because there is only fair-to-poor agreement between actual height and estimated height.

Abdominal obesity is an independent predictor of serum 25-hydroxyvitamin D deficiency in adults with cerebral palsy.

BACKGROUND: Individuals with cerebral palsy (CP) are at risk for nutritional insufficiency. The purpose of the study was to examine the vitamin D status of adults with CP, and to evaluate the association between vitamin D and functional level, age, race, and anthropometric indicators of adiposity. METHODS: Serum vitamin D levels, BMI, waist circumference (WC), and functional level (measured by Gross Motor Function Classification System (GMFCS)) were examined in 112 adults with CP. Vitamin D status was assessed by serum 25-hydroxyvitamin D level (25(OH)D). The influence of motor impairment and adiposity on 25(OH)D were assessed using general linear modeling and logistic regression, with age, sex, race, and season as covariates. RESULTS: Mean vitamin D was 28.1 ± 16.0 ng/ml. Only 45% of subjects had optimal levels of 25(OH)D, 21% were insufficient and 34% were deficient. Overweight or obesity was prevalent (52%), as was abdominal obesity in men (23.5% at 102 cm cutoff) and women (31.1% at 88 cm cutoff). There was a robust association between the indicator of visceral adiposity (WC) and 25(OH)D level (p <0.001), even after controlling for age, sex, race, season, and GMFCS. According to sex-specific WC cutoffs, the odds of being deficient in vitamin D increase by a factor of 3.5 (95% CI 1.12-11.0) for abdominal obesity. GMFCS was not associated with 25(OH)D. CONCLUSIONS: Adults with CP are at risk for low vitamin D levels and overweight/obesity. Waist circumference is a strong independent predictor for low vitamin D levels.