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.

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.

Predictors of cardiometabolic risk among adults with cerebral palsy.

OBJECTIVE: To examine the independent association between various anthropometric indicators and standard clinical markers of cardiometabolic health risk among adults with cerebral palsy (CP). DESIGN: Cross-sectional study. SETTING: Clinical center for CP treatment and rehabilitation. PARTICIPANTS: Adults with CP (N=43) with a mean age ± SD of 37.3±13.2 years, and Gross Motor Function Classification System (GMFCS) levels of I-V. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Adults with CP were assessed for body mass index (BMI), waist circumference (WC), hip circumference (HC), waist-to-hip ratio (WHR), waist-to-height ratio (WtHR), and serum lipid profiles. Data were analyzed with multiple regression analysis and general linear models, and are reported as means ± SDs. RESULTS: Mean BMI was 29.1±7.8kg/m(2). BMI was not associated with any measures of cardiometabolic risk. Using GMFCS categories (2 groups: GMFCS levels I-III and IV-V), BMI was significantly lower among GMFCS levels IV-V (24.2±6.2kg/m(2)) versus GMFCS levels I-III (30.1±7.6kg/m(2)). WC and WtHR were not correlated with any cardiometabolic outcomes. Conversely, measures of WHR were independently associated with various indices of risk, including total cholesterol to high-density lipoprotein (HDL) cholesterol ratio (r=.45; P<.05), HDL cholesterol (r=-.51; P<.01), and triglycerides (r=.40; P<.05), suggesting that greater WHR was indicative of elevated risk. CONCLUSIONS: It is likely that WHR represents a stronger predictor of risk, because this measure was robustly and independently associated with 3 primary clinical markers of cardiometabolic health in adults with CP.

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.

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.

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.

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.

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.

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.

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.

Functional outcomes of childhood dorsal rhizotomy in adults and adolescents with cerebral palsy.

OBJECT: In this descriptive study the authors evaluated medical outcomes, interventions, satisfaction with life, and subjective impressions about selective dorsal rhizotomy (SDR) in older adolescents and adults who had undergone the procedure as children. METHODS: A survey was administered to older adolescents (16-20 years old) and adults with CP who had undergone SDR between 1986 and 2000 at two academic centers. The patients or their caregivers participated in telephone or clinic interviews. Subjective impressions about the SDR and a history of post-SDR medical interventions were obtained. Current functional status, history and ratings of pain, educational achievement, living situation, and subjective health status were also recorded. The Diener Satisfaction with Life Scale (SWLS) was administered. RESULTS: Eighty-eight participants, mean age 25.6 ± 4.8 years (mean ± standard deviation), were interviewed at a mean of 19.6 ± 3.0 years after surgery. The distribution of current reported Gross Motor Function Classification System levels was as follows: I, 7%; II, 18%; III, 23%; IV, 36%; and V, 16%. Moreover, 56% of respondents were living with parents and 25% were living alone. Thirty-five percent were employed, and 39% were still in school. The mean overall SWLS score was 26.0 ± 7.3, indicating a high level of satisfaction with life. According to 65% of the patients, the SDR was helpful; 31% were uncertain about the procedure's efficacy. Sixty-five percent would recommend the procedure to others. Fifty-eight percent reported excellent to very good health. Forty-four percent reported pain in the past week. Fifty-one percent reported chronic back pain in general. Logistic regression analysis suggested that an increased satisfaction with life was a predictor (p = 0.01) of an affirmative response to the question about recommending the procedure to others and that better overall health showed a trend toward being such a predictor (p = 0.08). Additional interventions were frequently performed after the SDR. Seventy-four percent of participants underwent orthopedic surgery. Thirty-eight percent were currently taking oral medications for tone, and 53% had received botulinum toxin injections for spasticity treatment. Thirteen patients (15%) had an intrathecal baclofen pump placed. CONCLUSIONS: The majority of adults who had undergone SDR as children would recommend the procedure to others. Very few reported negative impressions of the procedure. Levels of satisfaction with life were generally high. Pain prevalence was similar to what has been reported in the literature for adults with cerebral palsy. Despite the SDR, further interventions, both surgical and nonsurgical, were used in the majority of patients.