Adults with cerebral palsy and functional decline: A cross-sectional analysis of patient-reported outcomes from a novel North American registry.

BACKGROUND: Adults with cerebral palsy (CP) have unique healthcare needs and risks, including high risk of functional decline. Understanding functional decline is an area of priority for CP research. OBJECTIVE: Describe factors associated with patient-reported changes in function among adults with CP living in the community. METHODS: Cross-sectional analysis of adult patient-reported outcomes collected by the CP Research Network (CPRN) Community Registry. RESULTS: Participants included 263 respondents (76% female (n = 200); mean age 42 years (SD 14); 95% White (n = 249); 92% non-Hispanic (n = 241)). Many reported functional changes, most commonly a decline in gross motor function since childhood (n = 158, 60%). Prevalence of gross motor decline varied significantly by Gross Motor Function Classification System (GMFCS) level (p < 0.001), but neither hand function decline (p = 0.196) nor communication decline (p = 0.994) differed by GMFCS. All types of decline increased with increasing age, with statistically significant differences between age groups (p < 0.001 gross motor; p = 0.003 hand function; p = 0.004 communication). Those with spastic CP (n = 178) most commonly reported gross motor functional decline (n = 108/178, 60.7%). However, the prevalence of gross motor decline did not significantly differ between those with spastic CP and those without spastic CP (p = 0.789). CONCLUSIONS: Many adults in the CPRN Community Registry reported functional decline, most commonly in gross motor function. Functional decline across domains increased with age. Further research into risk stratification and preventive and rehabilitative measures is needed to address functional decline across the lifespan.

Comorbidity Clusters Among Adults With Cerebral Palsy: A Latent Class Analysis.

INTRODUCTION: Adults with cerebral palsy are at risk for early multimorbidity onset, but little is known about the composition of multimorbidity profiles or how these profiles present across adulthood. The objective of this study was to identify multimorbidity profiles and association with mortality among adults with cerebral palsy. METHODS: This retrospective cohort study used a random 20% fee-for-service Medicare database from January 1, 2008 to December 31, 2019 from adults aged ≥18 years with cerebral palsy. Latent class analyses using 4-class models were conducted within each age cohort (young adults aged 18-39 years, middle adulthood aged 40-64 years, and older adults aged ≥65 years) to determine patterns of 30 comorbidities defined using the International Classification of Diseases, Ninth Revision codes, identified from January 1, 2008 to December 31, 2010, and their association with mortality through December 31, 2019 (up to 11 years of follow-up); statistical analysis was performed in 2023. RESULTS: Three classes were relatively consistent in the composition of comorbidities across young (n=7,020), middle (n=13,554), and older (n=4,193) cohorts: (1) low morbidity (low proportion of all comorbidities) representing 50.1% (young), 41.4% (middle), and 30.9% (older) of the cohorts; (2) neurologic multimorbidity (e.g., epilepsy, intellectual disabilities) representing 26.0% (young), 26.6% (middle), and 14.7% (older) of the cohorts; and complex multimorbidity (e.g., cardiorespiratory, nutritional, musculoskeletal, neurologic) representing 26.0% (young), 26.6% (middle), and 14.7% (older) of the cohorts. The fourth class varied by young (mental health disorders), middle (hypertension), and older (hypertension and osteoarthritis) age cohorts. Compared with the low morbidity class, other classes had an increased mortality rate for each age cohort (hazard ratio range=1.34-5.58, all p<0.001). CONCLUSIONS: Findings provide insight into varied multimorbidity profiles and associations with mortality across the life course for adults with cerebral palsy.

Adults with cerebral palsy and chronic pain experience: A cross-sectional analysis of patient-reported outcomes from a novel North American registry.

BACKGROUND: Chronic pain is common among adults with cerebral palsy (CP) and an area of priority for research and treatment. OBJECTIVE: Describe the pain experience and its functional and quality of life impact among adults with CP with chronic pain in the community. METHODS: Cross-sectional analysis of adult patient-reported outcomes collected by the Cerebral Palsy Research Network Community Registry. RESULTS: Among all participants in the Community Registry, n = 205 reported having chronic pain, and 73 % of those (n = 149) completed the Chronic Pain Survey Bundle (75 % female; mean age 43 years (SD 14 years); 94 % White; 91 % non-Hispanic). Back and weight-bearing joints of lower extremities were most frequently reported as painful. There were no differences in average pain severity scores between varying GMFCS levels (H = 6.25, p = 0.18) and age groups (H = 3.20, p = 0.36). Several nonpharmacologic interventions were most frequently reported as beneficial. Participants with moderate to severe average pain scores (5-10) had higher levels of pain interference (p < 0.01) and depression (p < 0.01), and lower levels of satisfaction with social roles (p < 0.01) and lower extremity function (p < 0.01). Pain interference was significantly positively correlated with depression, and negatively correlated with upper and lower extremity function and satisfaction with social roles. CONCLUSIONS: Chronic pain is experienced by adults with CP of varying ages and functional levels and is associated with several adverse quality of life and functional outcomes. Improved understanding of chronic pain in this population will facilitate the development and study of treatment interventions optimizing health, function, participation, and quality of life.

Perspective: A multi-trait integrative approach to understanding the structural basis of bone fragility for pediatric conditions associated with abnormal bone development.

Bone development is a highly orchestrated process that establishes the structural basis of bone strength during growth and functionality across the lifespan. This developmental process is generally robust in establishing mechanical function, being adaptable to many genetic and environmental factors. However, not all factors can be fully accommodated, leading to abnormal bone development and lower bone strength. This can give rise to early-onset bone fragility that negatively impacts bone strength across the lifespan. Current guidelines for assessing bone strength include measuring bone mineral density, but this does not capture the structural details responsible for whole bone strength in abnormally developing bones that would be needed to inform clinicians on how and when to treat to improve bone strength. The clinical consequence of not operationalizing how altered bone development informs decision making includes under-detection and missed opportunities for early intervention, as well as a false positive diagnosis of fragility with possible resultant clinical actions that may actually harm the growing skeleton. In this Perspective, we emphasize the need for a multi-trait, integrative approach to better understand the structural basis of bone growth for pediatric conditions with abnormal bone development. We provide evidence to showcase how this approach might reveal multiple, unique ways in which bone fragility develops across and within an array of pediatric conditions that are associated with abnormal bone development. This Perspective advocates for the development of new translational research aimed at informing better ways to optimize bone growth, prevent fragility fractures, and monitor and treat bone fragility based on the child's skeletal needs.

The age-related association between serum creatinine and cardiorespiratory morbidity and mortality and fractures among adults with cerebral palsy.

PURPOSE: Serum creatinine may be an objective biomarker of salient health issues in adults with cerebral palsy (CP). The objective was to assess the age-related association between serum creatinine with 3-year risk of cardiorespiratory morbidity/mortality and fracture among adults with CP. PATIENTS AND METHODS: This retrospective cohort study used medical records between Jan. 1, 2012 and Oct. 2, 2022 from adults ≥18 years old with CP. The association between baseline serum creatinine with the 3-year risk of all-cause mortality, respiratory/cardiovascular morbidity/mortality, and fracture was assessed by age and sex using logistic regression. The discriminative ability of serum creatinine alone and in conjunction with other variables was assessed. RESULTS: Over the 3-year follow-up, 8.3% of 1368 adults with CP had all-cause mortality, 25.6% had respiratory morbidity/mortality, 12.4% had cardiovascular morbidity/mortality, and 8.9% sustained a fracture. The association between serum creatinine with outcomes was dependent on age. For younger adults, lower creatinine had a higher odds ratio (OR) for all-cause mortality, respiratory morbidity/mortality, and fracture. For 51-60 year olds, higher creatinine had a higher OR for cardiovascular morbidity/mortality. Serum creatinine alone had modest prediction of outcomes, and generally improved prediction when added to models that included sex and co-occurring intellectual disabilities and epilepsy (c-statistic range, 0.54-0.84). CONCLUSIONS: Lower serum creatinine may reflect frailty while higher levels may reflect kidney dysfunction, helping to explain the differential associations by age. Serum creatinine may be a useful biomarker as part of risk prediction models for these salient health issues for adults with CP.

Population- and individual-level trajectories of opioid prescription patterns among adults with cerebral palsy: a retrospective cohort study.

BACKGROUND: There is little epidemiologic evidence on opioid prescription among adults with cerebral palsy (CP). AIM: To describe the population- and individual-level opioid prescription patterns for adults with versus without CP. METHOD: This retrospective cohort study used commercial claims (Optum's de-identified Clinformatics® Data Mart Database) from the USA from 01/01/2011-12/31/2017 from adults ≥ 18 years old with CP and matched adults without CP. For the population-level analysis, monthly estimates of opioid exposure were described for adults ≥ 18 years old with CP and matched adults without CP. For the individual-level analysis, group based trajectory modelling (GBTM) was used to identify groups of similar individual-level monthly opioid exposure patterns for adults with CP and matched adults without CP for 1-year starting from their first opioid exposure month. RESULTS: For the population-level, adults with (n = 13,929) versus without (n = 278,538) CP had a higher prevalence of opioid exposure (~ 12%, ~ 8%) and days supplied (median, ~ 23, ~17) monthly over 7 years. For the individual-level, there were 6 trajectory groups for CP (n = 2099) and 5 for non-CP (n = 10,361). Notably, 14% of CP (comprising 4 distinct trajectory groups) and 8% (comprising 3 distinct groups) of non-CP had variably high monthly opioid volume for extended periods; exposure was higher for CP. The remaining had low/absent opioid exposure trajectories; for CP (non-CP), 55.7% (63.3%) had nearly absent exposure and 30.4% (28.9%) had consistently low exposure to opioids. CONCLUSION: Adults with versus without CP were more likely to be exposed to opioids and for a longer duration, which may alter the risk-benefit balance of opioids.

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.

Underlying Causes of Death among Adults with Cerebral Palsy.

BACKGROUND: Adults with cerebral palsy (CP) represent a growing population whose healthcare needs are poorly understood. The purpose of this study was to examine trends in the underlying causes of death (UCOD) among adults with CP in the United States. METHODS: A national cohort was created from the Centers for Disease Control and Prevention Wide-ranging Online Data for Epidemiologic Research (WONDER) database from 1999 to 2019. The UCOD was determined using the International Statistical Classification of Diseases and Related Health Problems, Tenth Revision (ICD-10 code G80x, Infantile CP) based on death certificate adjudication. Crude and age-adjusted mortality rates (AAMRs), as well as 95% confidence intervals (CIs) were calculated for adults with CP. RESULTS: There were 25,138 deaths where CP was listed as the UCOD between 1999-2019. There was a steady increase in the UCOD attributable to CP in both crude mortality rates and AAMRs, with the highest rates occurring in 2019. The highest co-occurring secondary causes of death were other diseases of the nervous system (e.g., epilepsy), diseases of the respiratory system (e.g., pneumonia), symptoms, signs, and abnormal clinical and laboratory findings, not elsewhere classified (e.g., dysphagia), and diseases of the circulatory system (e.g., cardiovascular disease). CONCLUSIONS: Listing the UCOD as CP should be accompanied by other mechanisms leading to mortality in this population.

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.

Five-year risk of fracture and subsequent fractures among adults with cerebral palsy.

Background: Epidemiologic evidence documenting the incidence of fracture and subsequent fractures among adults with cerebral palsy (CP) is lacking, which could inform fracture prevention efforts. The objective was to characterize the 5-year rate of initial and subsequent fragility fractures among adults with CP. Methods: This retrospective cohort study used Medicare claims from 01/01/2008-12/31/2019 from adults ≥18 years old with CP (n = 44,239) and elderly ≥65 years old without CP (n = 2,176,463) as a comparison. The incidence rate (IR), IR ratio (IRR), and site distribution were estimated for the initial and subsequent fragility fractures over 5-years by sex and age. Results: The IR of fragility fracture at any site over the 5-year follow-up was similar for 18-30-year-old men with CP (IR = 5.2; 95%CI = 4.4-5.9) and 30-34-year-old women with CP (IR = 6.3; 95%CI = 5.3-7.2) compared to the same sex youngest-old (65-74 years old) without CP (IRR = 1.09 and 0.94, respectively, both P > 0.05), and increased with older age for those with CP. The number of fragility fractures and IR of subsequent fragility fractures was similar for young men and middle-aged women with CP compared to elderly without CP, and increased with older age for those with CP. The proportion of fragility fracture at the tibia/fibula decreased while the vertebral column and multiple simultaneous sites (most involved hip/lower extremities) increased with older age. Conclusion: Young and middle-aged adults with CP had similar-to-worse initial and subsequent fragility fracture profiles compared to the general elderly population- a well characterized group for bone fragility. Findings emphasize the need for fracture prevention efforts at younger ages for CP, possibly by ~5 decades younger.

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.

Preventive Care for Adults With Cerebral Palsy and Other Neurodevelopmental Disabilities: Are We Missing the Point?

Preventive care techniques are cornerstones of primary care for people with neurodevelopmental disabilities such as cerebral palsy (CP). However, well-established methods evaluating health constructs may not be applied in the same way for adults with CP, as compared to the general population, due to differences in anatomy/physiology, leading to missed opportunities for interventions, medication modifications, and other primary/secondary prevention goals. One barrier to care prevention comes from misinterpretation of values to capture health constructs, even when measurements are accurate. In this Perspective, we emphasize the need for differential interpretation of values from commonly used clinical measures that assess for well-known medical issues among adults with CP obesity risk, bone health, and kidney health. We provide technical, but simple, evidence to showcase why the underlying assumptions of how some measures relate to the health construct being assessed may not be appropriate for adults with CP, which may apply to other neurodevelopmental conditions across the lifespan.

Excess healthcare spending associated with fractures among adults with cerebral palsy.

BACKGROUND: Fractures represent a triple threat to adults with cerebral palsy (CP): common, accumulate early in adulthood, and are consequential to health. An economic evaluation of fractures in CP is needed to highlight priorities for allocating resources to clinical and public health programs aimed at preventing fractures and their disease sequela. OBJECTIVE: To identify short-term healthcare costs associated with fractures among adults with CP. METHODS: A retrospective cohort study was performed using Optum's de-identified Clinformatics® Data Mart Database from 01/01/2011-12/31/2017. The primary cohort included adults ≥ 18 years old with CP with an incident fracture (CP+Fx), and cost estimates were compared with: CP without fractures (CPw/oFx) and without CP+Fx (w/oCP+Fx). A difference-in-difference (DiD) analysis compared the change in pharmacy and medical costs between cohorts from the one-year baseline period through the one-year post-index period in three-month quarters. RESULTS: CP+Fx (n = 855) had higher mean costs in the baseline and follow-up periods compared with CPw/oFx (n = 5667) and w/oCP+Fx (n = 588,042). The first post-index quarter DiD estimate suggests that CP+Fx accumulated an excess $6462 (95%CI = $3810-$9021) compared with w/oCP+Fx and $17,197 (95%CI = $14,418-$19,833) compared with CPw/oFx. The CP+Fx cohort had higher DiD estimates in the other follow-up quarters, but they were not statistically significant compared with CPw/oFx. When stratified by fracture site, vertebral column fractures for CP+Fx vs. w/oCP+Fx accumulated an excess $25,226 (95%CI = $12,639-$37,417). CONCLUSIONS: Fractures, especially of the vertebral column, were associated with high healthcare costs among adults with CP. Studies are needed to identify cost-effective opportunities to utilize available resources to prevent fractures and their costly sequela for CP.

A core outcome set for multimorbidity risk in individuals with cerebral palsy.

AIM: To: (1) investigate the importance of outcome measurement instruments (OMIs) within a core outcome set (COS) for multimorbidity (at least two chronic health conditions) risk in individuals with cerebral palsy (CP); (2) investigate the feasibility of OMIs within the COS in international clinical research settings in adolescents and adults with CP; and (3) describe the associations between the COS data and Gross Motor Function Classification System (GMFCS) levels. METHOD: Eighty-three individuals with CP completed a survey on health outcomes: physical behaviour, nutrition, sleep, endurance, body composition, blood pressure, blood lipids, and glucose. A cross-sectional study assessed the feasibility of the COS in 67 adolescents and adults with CP (mean age 30y, SD 15y 1mo, min-max: 14-68y, 52.2% male) at four centres. Prevalence of multimorbidity risk and associations with GMFCS levels are described. RESULTS: Most participants rated physical behaviour, nutrition, sleep, and endurance as very important. Body composition, blood pressure, nutrition, and sleep were highly feasible since data were collected in 88% or more participants who consented to having the assessments. Physical behaviour, cardiorespiratory endurance, and blood draws were collected in less than 60% of participants. Total time sedentary (ρ=0.53, p<0.01) and endurance (ρ=-0.46, p<0.01) were significantly associated with GMFCS level. INTERPRETATION: The COS identified that most participants had poor sleep quality and endurance, did not have healthy diets, and showed increased sedentary behaviour. Individuals with CP valued these outcomes as most important, suggesting a need to assess these modifiable behaviours in this population. Objective measures of physical behaviour and cardiorespiratory endurance in the COS required additional personnel, time, and participant burden. We recommend that healthcare providers should perform a simpler first screen using questionnaire-based assessments and then focus the use of the remainder of the COS if required for the patient.

UM DESFECHO CENTRAL DEFINIDO PARA RISCO DE MULTIMORBIDADE EM INDIVÍDUOS COM PARALISIA CEREBRAL: OBJETIVO: (1) Investigar a importância dos instrumentos de medição de desfechos (IMD) dentro de um conjunto de desfechos principais (CDS) para risco de multimorbidade (pelo menos duas condições crônicas de saúde) em indivíduos com paralisia cerebral (PC); (2) Investigar a viabilidade de IMD dentro do CDS em cenários internacionais de pesquisa clínica em adolescentes e adultos com PC; e (3) Descrever as associações entre os dados do CDS e os níveis do Sistema de Classificação da Função Motora Grossa (GMFCS). MÉTODO: Oitenta e três indivíduos com PC completaram uma pesquisa sobre desfechos em saúde: comportamento físico, nutrição, sono, resistência, composição corporal, pressão arterial, lipídios no sangue e glicose. Um estudo transversal avaliou a viabilidade do COS em 67 adolescentes e adultos com PC (idade média de 30 anos, desvio padrão de 15 anos e 1 mês, min-max: 14-68 anos, 52,2% do sexo masculino) em quatro centros. São descritas a prevalência do risco de multimorbidade e as associações com os níveis de GMFCS. RESULTADOS: A maioria dos participantes classificou o comportamento físico, nutrição, sono e resistência como muito importantes. Composição corporal, pressão arterial, nutrição e sono foram altamente viáveis, uma vez que os dados foram coletados em 88% ou mais dos participantes que consentiram em realizar as avaliações. Comportamento físico, resistência cardiorrespiratória e coleta de sangue foram coletados em menos de 60% dos participantes. O tempo total de sedentarismo (ρ = 0,53, p < 0,01) e resistência (ρ = −0,46, p < 0,01) foram significativamente associados ao nível de GMFCS. INTERPRETAÇÃO: O CDS identificou que a maioria dos participantes tinha má qualidade e resistência do sono, não tinha dietas saudáveis e apresentava um comportamento sedentário aumentado. Indivíduos com PC valorizaram esses desfechos como mais importantes, sugerindo a necessidade de avaliar esses comportamentos modificáveis nessa população. Medidas objetivas de comportamento físico e resistência cardiorrespiratória no CDS exigiram pessoal adicional, tempo e sobrecarga do participante. Recomendamos que os profissionais de saúde realizem uma primeira triagem mais simples usando avaliações baseadas em questionários e, em seguida, concentrem o uso do restante do CDS, se necessário para o paciente.

Clinical bone health among adults with cerebral palsy: moving beyond assessing bone mineral density alone.

AIM: To understand associations among bone mineral density (BMD), bone mineral content (BMC), and bone area, and their association with fractures in adults with cerebral palsy (CP). METHOD: This retrospective cohort study included 78 adults with CP with a hip dual energy X-ray absorptiometry (DXA) from 1st December 2012 to 3rd May 2021 performed at the University of Michigan. Data-driven logistic regression techniques identified which, if any, DXA-derived bone traits (e.g. age/sex/ethnicity-based z-scores) were associated with fracture risk by sex and severity of CP. BMC-area associations were examined to study the structural mechanisms of fragility. RESULTS: Femoral neck area was associated with lower age-adjusted odds ratios (ORs) of fracture history (OR 0.72; 95% confidence interval [CI] 0.49-1.06; p=0.098), while higher BMD was associated with higher odds of incident fracture (OR 3.08; 95% CI 1.14-8.33; p=0.027). Females with fracture had lower area than females without fracture but similar BMC, whereas males with fracture had larger area and higher BMC than males without fracture. The paradoxical BMD-fracture association may be due to artificially elevated BMD from BMC-area associations that differed between females and males (sex interaction, p˂0.05): males had higher BMC at lower area values and lower BMC at higher area values compared to females. INTERPRETATION: BMD alone may not be adequate to evaluate bone strength for adults with CP. Further research into associations (or integration) between BMC and area is needed.

The paradoxical relationship between severity of cerebral palsy and renal function in middle-aged adults: better renal function or inappropriate clinical assessment?

PURPOSE: To determine the association between severity of cerebral palsy with serum creatinine (sCr) and sCr-based equations to estimate glomerular filtration rate (eGFR), a marker of renal function. METHODS: A clinic-based sample of 30-64 year-olds with cerebral palsy was examined and stratified by motor impairment: gross motor function classification system (GMFCS) I/II (n = 79), GMFCS III (n = 78), and GMFCS IV/V (n = 137). sCr, which is influenced by muscle mass, was obtained and sCr-based eGFR was calculated using the Modification of Diet in Renal Disease (MDRD) and Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equations. RESULTS: sCr was lower with increasing GMFCS. The opposite pattern was observed for eGFR: GMFCS IV/V had significantly higher eGFR derived from MDRD compared to other GMFCS groups; GMFCS III had significantly higher eGFR compared to GMFCS I/II. The pattern was similar for CKD-EPI derived eGFR. CONCLUSIONS: According to widely used clinical assessment methods for renal function, higher severity of cerebral palsy among adults is associated with better renal function, which is incongruent with their other biological systems. This paradoxical relationship is likely driven by lower muscle rather than true renal function, and thus, sCr-based eGFR may overestimate renal function, especially for GMFCS IV/V. Further prospective studies are needed.Implications for rehabilitationCommon methods of clinical assessment may over-estimate renal function for adults with cerebral palsy (CP), potentially giving a false positive for normal renal health due to their reliance on muscle mass.This study of a clinic-based sample of middle-aged adults with CP highlights the paradoxical relationship between severity of CP and renal function, which is likely driven by methodological limitations in the presence of low muscle mass rather than actual better renal function.It is recommended that clinicians have a high suspicion of abnormal renal function and the need for a nephrology consultation, especially with changes in creatinine levels, even within the normal range.Rehabilitation for adults with CP must have a strong focus on muscle and kidney health, especially for patients with more severe forms of CP.

Shared Physiologic Pathways Among Comorbidities for Adults With Cerebral Palsy.

Objective: Aging with cerebral palsy is accompanied by a declining health and function status across neurological and non-neurological systems. There is a need to understand the shared pathophysiology among comorbidities for adults with cerebral palsy, to inform clinical assessment and guidelines for interventions to improve healthful aging. To begin defining multimorbidity, this study identified the most common comorbidity combinations and their association with mortality among a representative sample of adults with cerebral palsy. Methods: Data from 2016 to 2018 were used from a random 20% sample from the fee-for-service Medicare database. Adults ≥18 years with cerebral palsy and 25 neurological and non-neurological comorbidities were obtained from 2016. Principal component (PC) analysis identified the most common comorbidity combinations, defined as individual PCs. Cox regression estimated the hazard ratio (HR) of 2-year mortality including all PCs and demographics in a single model. To facilitate comparisons, PC scores were transformed into quintiles (reference: lowest quintile). Results: Among the 16,728 adults with cerebral palsy, the most common comorbidity combinations (PCs) in order were: cardiorespiratory diseases, dysphagia, and fluid/electrolyte disorders; metabolic disorders (e.g., diabetes, renal disease, hypertension); neurologic-related disorders (e.g., dementia, cerebrovascular disease); gastrointestinal issues; and orthopedic-related disorders. During the 2-year follow-up, 1,486 (8.9%) died. In the adjusted model, most PCs were associated with an elevated mortality rate, especially the first PC (5th quintile HR = 3.91; 95%CI = 3.29-4.65). Discussion: This study identified the most common comorbidity combinations for adults with cerebral palsy, many of them were deadly, which may inform on the underlying pathophysiology or shared characteristics of multimorbidity for this population.

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.