Self-management in youth with spina bifida: predictors of health and quality of life outcomes

Abstract

Purpose:

This study examined factors associated with health status and quality of life (QOL) in youth with spina bifida.

Methods:

Self-management, health complications, comorbidities, global health, and QOL were assessed. Linear and logistic regression models were used to measure associations between respondents’ characteristics, self-management scores, and outcomes.

Results:

Participants (n = 99; 18–27 years old; 87.9% myelomeningocele) were about half female (52.5%) and White (52.5%); 15.2% were Black, and 32.3% were Hispanic/Latino. An increase in self-management scores was associated with lower odds of comorbid conditions (e.g., diabetes, obesity) by about 30%. Younger age and male sex were associated with better global mental health. Independent living self-management scores were positively associated with individual health-related QOL. The association between independent living self-management and family QOL was positive when executive dysfunction was low.

Conclusion:

This study identified factors important to consider for better health and QOL in populations living with disabilities. As youth enter adult-oriented care, models of care must address declining mental health and provide gender-specific adaptive interventions to increase independent living self-management. As participants gain more independence in self-management, they appear to exhibit reduced risk of comorbid conditions and improvements in their individual health-related QOL and family QOL.

Introduction

Spina bifida (SB) is a complex congenital chronic health condition and disability involving the central nervous system, which results in secondary conditions [1,2]. For people living with SB, the degree of disability is related to the level of lesion, which may lead to paralysis, lack of sensation, and urinary and bowel incontinence [2]. Health is further compromised by multi-system involvement; the majority of individuals with SB experience genitourinary/kidney, gastrointestinal, and infectious diseases [3]. Secondary conditions result from health complications and comorbidities (e.g., urinary tract infections, pressure ulcers, metabolic dysfunction, obesity, diabetes, orthopedic conditions). As youth transition to adulthood, their risk of early mortality from preventable conditions increases [4]. Among adult clinic populations, the leading cause of death is infection (e.g., urinary sepsis, skin ulcer or wound, peritonitis, pneumonia) [4,5]. Further, adults’ limited access to medical care leads to high utilization of emergency and hospital care [3]. Rising health care utilization costs for individuals with SB in the US are estimated at over two billion annually [6], and a 15-year increased life expectancy over the past decade [7] has heightened demand for prevention of adverse outcomes through self-management for optimal health status and quality of life (QOL).

In a recent systematic review of the self-management literature in SB [8], self-management behaviors were associated with better health status, such as prevention of complications and independent living as well as less depression. This review also identified poor self-management as a correlate of higher rates of UTIs and health care utilization (emergency department visits) [8]. In SB, managing health becomes more complex when there are increasing clinical interventions for incontinence, skin breakdown, and changes in ambulation. More research is needed on prevention and management of health complications and secondary conditions through self-management and on the impact of self-management over time on the relationship between contextual factors, including family-related predictors, and later health and QOL outcomes.

Importantly, past indicators of health status have broadly been listed in the literature as frequencies of specific procedures and conditions. An alternative perspective on health status involves the use of a global measure of overall health as well as physical and mental health which will help clinicians to assess the future impact of clinical interventions and identify subgroups of individuals requiring more support and care.

Across the lifespan, people with SB rated their overall QOL and health-related QOL lower when compared to youth with and without chronic health conditions [9–13] and almost half (45.7%) of youth with SB are at-risk for poor psychosocial functioning [12]. Most of the evidence on QOL in SB is from the perspectives of families with children under 18 years of age [13]. In Murray et al. [13], health-related QOL scores improved for youth with SB aged 10–17; ratings were similar to participants with other chronic conditions in the emotional and social domains but remained significantly lower in physical and school domains. However, when compared to those without any health conditions, participants’ ratings remained lower in every domain [14]. Driscoll et al. further examined parenting-related predictors of health-related QOL in this same sample of youth with SB after omitting the physical health subscale [15]. Higher levels of SB-specific parenting stress were associated with youth ratings of their health-related QOL, and these associations were noted across child-adolescent developmental periods from 8 to 19 years of age. No other demographic or clinical factors were associated with health-related QOL [15]. Similarly, another recent study found associations between child mental health symptoms, parent health-related QOL, family functioning, and poor psychosocial QOL for children and adolescents [12].

Further, in a study of adolescents and young adults with and without SB up to 25 years old, there was a significant difference in family QOL reported by parents (i.e., about a 7-point difference on a 100-point scale); youth with and without SB did not differ on their own ratings of family QOL [16]. Moreover, the presence of SB, greater family satisfaction, lower parent stress of everyday life, and less parent depressive symptoms were significant predictors of parent-reported higher global family QOL [10].

Across the studies of children and adolescents with SB, evidence suggests associations between mental health, family-related factors, and health complications with individual and family QOL. However, the evidence is limited for psychosocial needs and outcomes of adults with spina bifida [17]. Predictors of health status and QOL, specifically as youth with spina bifida enter young adulthood (i.e., over 18 years old), have been understudied.

The current study was informed by the Individual and Family Self-Management Theory and our prior work on predictors of self-management trajectories [18–22]. Specifically, we sought to evaluate factors (including self-management) that were associated with health status and QOL of adults with SB. Self-management was assessed from the perspective of youth over 18 years of age who completed the Adolescent/Young Adult Self-Management Scale (AMIS II) interview. See Figure 1 for a model depicting the health status and QOL outcomes and how they were operationalized in this study. Three indicators of health status were used: Global health, the presence of health complications, and the prevalence of co-morbid conditions. QOL outcomes included individual health-related QOL (i.e., psychosocial QOL) and family QOL.

Figure 1.

Model for health status and quality of life outcomes.

The specific study aims were to examine associations between self-management with global health, health complications, comorbidities, individual health-related QOL, and family QOL. For global health and QOL outcomes, we controlled for predictors of self-management from prior work including contextual factors (e.g., age, gender, family income, executive function) and process factors (family functioning, family stress). Specifically, we hypothesized the following: (1) Higher self-management scores would be associated with better global health (overall health, physical health, mental health) after controlling for contextual and process factors; (2) higher self-management scores would be associated with a reduced odds of health complications and comorbidities (e.g., urinary tract infection, bowel accident, pressure injury, diabetes, obesity); and (3) higher self-management scores would be associated with better individual and family QOL (psychosocial health-related QOL, global family QOL) after controlling for contextual and process factors.

Methods

Families of youth with SB were recruited to participate in a longitudinal study examining family, psychosocial, and neurocognitive functioning from four hospitals near Chicago and through a Midwest-based Spina Bifida Association (see Papadakis and Holmbeck [23]). The primary study inclusion criteria were: (a) a SB diagnosis; (b) 8–15 years old; (c) can understand English or Spanish; (d) involvement of at least one main caregiver; and (e) lives within 300 miles of the study lab for home visit data collection. As described in prior work [19], the primary study recruited 246 families and 163 families agreed to participate; however, after initial consent, 21 families either declined to participate or could not be contacted, and two families did not meet the inclusion criteria. Participants who enrolled at Time 1 (N = 140) did not differ from those who declined on type of SB, shunt status, or the number of shunt infections [15]. Youth were 52.9% White, 13.6% Black, 27.9% Hispanic, 1.4% Asian, and 4.3% multiracial. Of those who identified as multiracial, three were White and Black; two were White and Hispanic; and one was White, Hispanic, and Asian. Slightly more females (n = 75, 53.6%) versus males (n = 65, 46.4%) participated. Over half of parents reported a family income of 50K or above (59.3%; 12 or 8.6% were missing data on income). The type of SB was 87.9% myelomeningocele, 77.9% had a shunt, and half had a lumbar lesion level (49.3%).

All participants with a minimum of one observation over the four data collection points were included in this study. The sample included 99 unique participants and 214 total observations on self-management. Subsample characteristics are presented in Table 1.

Table 1.

Sample characteristics.

	Overall (N = 99)
Age at Time 1
Mean (SD)	12.0 (2.29)
Median [Min, Max]	12.0 [8.00, 16.0]
Sex
Male	47 (47.5%)
Female	52 (52.5%)
Race/ethnicity
White	52 (52.5%)
Black	15 (15.2%)
Hispanic	32 (32.3%)
Family income
<50K	32 (32.3%)
≥50K	59 (59.6%)
Missing	8 (8.1%)
Spina bifida type
Myelomeningocele	87 (87.9%)
Not myelomeningocele	12 (12.1%)
Lesion level
Sacral	31 (31.3%)
Lumbar	48 (48.5%)
Thoracic	18 (18.2%)
Cervical	0 (0%)
Missing	2 (2.0%)
Shunt present
Yes	81 (81.8%)
No	18 (18.2%)
Intelligence quotient
Mean (SD)	84.7 (19.9)
Median [Min, Max]	84.0 [55.0, 137]
Missing	2 (2.0%)
BRIEF behavioral regulation index composite
Mean (SD)	54.1 (10.3)
Median [Min, Max]	51.7 [37.5, 85.7]
Missing	3 (3.0%)
BRIEF metacognition index composite
Mean (SD)	60.3 (11.8)
Median [Min, Max]	58.7 [40.5, 92.0]
Missing	3 (3.0%)
Family cohesion observational
Mean (SD)	3.35 (0.405)
Median [Min, Max]	3.40 [2.24, 4.19]
Missing	3 (3.0%)
Number of family stress events parent-reported (FILE)
<10	47 (47.5%)
10–20	30 (30.3%)
≥20	11 (11.1%)
Missing	11 (11.1%)
Overall self-management (baseline)
Mean (SD)	3.42 (1.24)
Median [Min, Max]	3.56 [1.00, 6.12]
overall self-management (recent)
Mean (SD)	3.80 (1.40)
Median [Min, Max]	3.91 [1.00, 6.41]
Condition self-management (baseline)
Mean (SD)	4.00 (1.46)
Median [Min, Max]	4.29 [1.00, 6.58]
Condition self-management (recent)
Mean (SD)	4.19 (1.52)
Median [Min, Max]	4.57 [1.00, 6.83]
Independent living self-management (baseline)
Mean (SD)	3.02 (1.19)
Median [Min, Max]	3.00 [1.00, 6.00]
Independent living self-management (recent)
Mean (SD)	3.53 (1.40)
Median [Min, Max]	3.45 [1.00, 6.35]

Procedures for data collection

Longitudinal home visit data collections occurred every 2 years from Time 1 through Time 5. There was a 3-year interval between Times 5 and 6. The first time point (Time 1) included children 8–15 years of age and their parents. About 25% of the sample youth reached 18 years of age at Time 3, 50% at Time 4, 75% at Time 5, and 100% at Time 6. At Time 3 and thereafter, youth over 18 years of age completed the AMIS II interview for assessment of self-management; data from Times 3–6 were used to examine self-management. The level of missing data on the AMIS II at each time point corresponds roughly to the number of individuals who were ineligible to complete the assessments at a given time point, because they were younger than 18 years old. Specifically, rates of missing data were 76.5% at Time 3, 52% at Time 4, 33.7% at Time 5, and 20.4% at Time 6. During the latter portion of Time 6, the data collection method for the interview changed from in-home to virtual sessions due to the COVID-19 pandemic. This study includes only those participants who completed the AMIS II interview in-person during the home visit data collection before the pandemic began in March 2020.

Informed consent from parents and assent from youth were obtained at Time 1 and adolescents and young adults (AYA) >18 years old were consented as adults when eligible beginning at Time 3. The longitudinal study was approved by university and hospital-based IRBs. During home visits conducted by trained undergraduate- and graduate-level research assistants, family interactions were video-recorded, and youth AMIS II interviews were audio-recorded.

Measures

Contextual and process factors

The participants’ characteristics including their demographics, condition-related factors, neuropsychological function, and family environment were study covariates. The covariates were predictors of self-management trajectories in our research and therefore were important to include in the current analyses. Complete descriptions of these measures were detailed in our prior work [19–21]; thus, they are only briefly summarized here. The Demographic Questionnaire completed by both mothers and fathers included items on the child’s date of birth, sex, race, ethnicity, and family income. Participants who identified with two or more race/ethnicity groups were recoded (e.g., an individual identifying as White and Hispanic/Latino was classified as Hispanic/Latino for the purposes of the analysis). Family income was recoded as a dichotomous variable for those earning less than or greater than 50K. A Medical Chart Review Form was used to collect condition-related data on type of SB, lesion level, and shunt status. Parents and teachers provided data on child neuropsychological functioning using the Behavior Rating Inventory of Executive Function [24]. As reported in prior work [20], composites were created from multiple informants and employed in our study as the Behavioral Regulation Index and the Metacognition Index; higher scores indicate greater executive dysfunction. The Wechsler Abbreviated Intelligence Scale was used to measure intelligence [25]. Family functioning was assessed during family interaction tasks using the Family Interaction Macro-coding System (FIMS) [26]. See Ridosh et al. [21] for a description of the observational family cohesion score. The number of family stress events were reported by parents on the Family Inventory of Life Events [27] scale.

Self-management

Adolescent/Young Adult Self-Management and Independence Scale (AMIS II).

The AMIS II interview was completed by AYA > 18 years old. The structured interview assesses the level of assistance required by the AYA to perform independent living skills and condition-related self-management. The interview includes 17 items (e.g., complication prevention, ordering medication/supplies, household skills, community living skills) scored on a 7-point Likert scale from 1 to 7 (1 = Total assistance: AYA does little or none of the activity <25% of the time; 7 = Complete independence: AYA does activity independently 100% of the time). The AMIS II total scale and subscales (condition self-management and independent living self-management) were calculated only if no more than two items were missing from the scales. An individual’s subscale or total scores were based on the mean of all items [28]. The internal consistency reliability of the AMIS II in the current study sample was high for both the total and subscale constructs (range: α = .896–.956). See Ridosh et al. [29] for more information about scoring reliability.

The AMIS scores were employed in analyses in several ways. Only two observations of self-management for each participant could be used in analyses. We selected the first (i.e., baseline) and last (i.e., recent) score of the self-management trajectory for all analyses except for the health complications and comorbidities outcomes. For those analyses, we used all available data at Time 5 and Time 6 (i.e., multiple scores for AMIS, health complications, and comorbidities).

Health status and QOL

The terms “health status,” “QOL,” and “health-related QOL” are used interchangeably in the literature; however, they represent distinct constructs [30–32]. The measurement of these constructs is further complicated by assessments of either function, perceptions, or both; few measures assess the broader conceptualization of QOL [33,34]. Health is a state of complete physical, mental, and social well-being and not simply the absence of disease [35,36]. QOL is commonly defined as “an individual’s perception of their position in life in the context of the culture and value systems in which they live and in relation to their goals, expectations, and concerns” [37, p. 551]. Health-related QOL, a domain of QOL, captures the impact of the condition on the individual’s physical, mental, and social functioning [11,14,38,39]. Given the importance of “an engaged family” in how youth and their parents define QOL [40], a concept extending the individual’s perspective of their own QOL to the family was captured by measuring global family QOL [16].

In this study, we took a comprehensive approach to the assessment of these outcomes using measures of function and perception, individual perspectives, as well as overall (i.e., global) and domain-specific scales (i.e., physical, mental, psychosocial). Specifically, global health status was assessed using a measure of overall health status with population norm data to provide ratings in comparison to population at large. The presence of health complications and co-morbidities most commonly occurring in spina bifida were assessed in addition to a PROMIS measure not previously used in SB for GI incontinence.

Global health.

The Global Health Questionnaire is a 10-item standardized measure from the Patient-Reported Outcomes Measurement Information System (PROMIS^® Scale v1.2) to broadly assess general perceptions of health [35]. Youth responded at Time 6 on a 5-point scale from (1) “Poor” to (5) “Excellent.” Three scores were employed in this study: a single item on general perception of health (i.e., Overall Health); a Global Physical Health T-score from 4 items on overall physical health, physical function, pain, and fatigue; and a Global Mental Health T-score from 4 items on QOL, mental health, satisfaction with social activities, and emotional problems. Higher scores indicate better health status [41]. The internal consistency reliability was adequate for the Global Physical Health scale α = .731 and the Global Mental Health scale α = .867.

Health complications.

Dealing with Spina Bifida Complications Questionnaire developed by the study team measures the total number of SB-related health complications that have occurred in the previous six months. At Time 5 participants reported on urinary tract infections, constipation, pressure sores, urinary accidents, bowel accidents, and kidney/bladder stones. The questionnaire assessed an additional three complications at Time 6 including shunt infections, tethered cord, and bone fractures. Youth reported the presence and frequency of each complication. The frequencies of complications were highly skewed; therefore, we employed whether the complication was present in analyses. The categorical outcomes of interest included in analyses were urinary tract infections, urinary accidents, bowel accidents, and pressure injuries.

The Gastrointestinal Bowel Incontinence (PROMIS^® Scale v1.0) is a 4-item measure of bowel incontinence issues from the previous seven days. At Time 6, youth responded on a 5-point Likert scale how frequently they had experienced an accident or soiled their underwear from (1) “No Days” to (5) “6–7 Days,” as well as how often they thought they were going to pass gas but leak stool instead from (1) “Never” to (5) “Always.” The sum total score was employed in analyses; no T-scores are available for this measure. Higher scores indicate more frequent bowel incontinence. The internal consistency reliability was adequate α = .865.

Comorbidities.

The Co-Occurring Medical Disorders Questionnaire developed by the study team measures the total number of comorbid conditions participants are diagnosed with, beyond SB. At Time 5 participants responded on the following conditions: type 2 diabetes, obesity, sleep apnea, latex allergy, cardiovascular disease (i.e., heart disease), metabolic dysfunction, osteoporosis, and chronic pain. At Time 6, type 1 diabetes was added to the questionnaire. Since there was redundancy in reporting the prevalence of diabetes, the type of diabetes variables were recoded for the presence of either type. All comorbidities were employed in analyses as categorical outcomes of interest.

Individual health-related quality of life.

The Pediatric Quality of Life Inventory (PedsQL^™, Version 4.0 Generic Core Scales Young Adult Version) [42] is a 23-item measure of the core health dimensions as delineated by the World Health Organization (Physical, Emotion, Social), as well as the role of work/school functioning. It is a multidimensional assessment based on individual’s function validated in youth over 18 years of age. At Time 6 youth responded on a 5-point Likert scale from (0) “Never a problem” to (4) “Almost always a problem.” Items were reverse-scored and linearly transformed to a 0–100 scale (0 = 100, 1 = 75, 2 = 50, 3 = 25, 4 = 0). The psychosocial summary scale score, created from the mean of 15 items that make up the emotion, social, and work/school subscales, was used in the analyses. Higher scores indicate better health-related QOL. The internal consistency reliability was adequate α = .920.

Family quality of life.

The Global Family QOL Scale (G-FQOLS) is a 3-item measure of an overall perception of family QOL in families with AYA with and without a chronic condition [16]. At Time 6 participants provided a rating on their own, family members’, and overall family QOL on a scale from (1) “Poor” to (100) “Excellent.” For the item on family members’ QOL, youth provided a rating for up to 8 family members (e.g., parents, siblings). A median of family member ratings was used as the score for that item. The global family QOL scale score created from a mean of 3 items were used in the analyses. Higher scores indicate better global family QOL. The internal consistency reliability was adequate α = .817.

Analyses

General linear models were used to estimate participants’ global physical health score as a function of their contextual factors (e.g., age, gender, family income, executive functioning), process factors (family functioning, family stress), and self-management. For models with covariates, the variable of interest was self-management and remaining variables were included if they improved model fit and parsimony as measured using Akaike’s information criterion (AIC) [43]. The same approach was used to estimate participants’ scores on global mental health, PROMIS Gastrointestinal (GI) incontinence, individual QOL, and family QOL. Regarding health complications, participants’ history of urinary tract infections, urinary accidents, bowel accidents, and pressure injuries were binary responses captured at both Times 5 and 6. For these responses, we used generalized estimating equations (or GEE models) to estimate the odds of each event as a function of participants’ AMIS score and age at the time of the response. Because participants contributed multiple responses to this analysis, each model allowed an exchangeable correlation structure with robust standard errors to account for the dependency. The same approach was used for participants’ binary diabetes, obesity, sleep apnea, latex allergy, cardiovascular disease, metabolic dysfunction/syndrome, osteoporosis, and chronic pain comorbidities. Finally, participants were asked to rate their general perception of health (i.e., overall health) at Time 6 on an ordinal scale ranging from 1 “Poor” to 5 “Excellent.” Here, we used a logistic regression model (with a cumulative logit link) to estimate the odds of better (higher) self-rated health as a function of participants’ AMIS score. All analyses were conducted using R version 4.3.2 [44] and the following packages: ggplot2 (v.3.3.3) [45], geepack (v.1.3.11) [46], and VGAM (v.1.1–9) [47].

Preliminary analyses included screening the outcome variables for outliers and skewness. Model residuals were assessed for linearity, normality, and homogeneity of variance. An attrition analysis was conducted to assess differences between those who did (n = 99) and did not (n = 41) participate in this portion of the study (see below for details on non-participants). All study variables are described as counts with proportions for nominal and ordinal variables and as a mean and standard deviation (or median with interquartile range or range) for quantitative variables.

Results

The current study sample (n = 99) ranged in age from 18 to 27 years old between Time 3 and Time 6 and about half were female (52.5%) and White (52.5%); 15.2% were Black, and 32.3% were Hispanic/Latino. The type of SB was primarily myelomeningocele (87.9%). See Table 1 for sample characteristics, including descriptive statistics for covariates (i.e., contextual and process factors).

Preliminary analyses

As reported in Ridosh et al. [19], no skewness was detected at the scale level for any time point. One outlier was detected on the psychosocial total score (i.e., a value of 8.33 on a scale from 0 to 100); however, Cook’s distance remained acceptable for all final models. Thus, data transformations were not conducted. The sample description including attrition analyses were detailed in Ridosh et al. [19] and summarized here. The following describes the 41 enrolled participants who were not included in this study. The majority of participants (n = 29, 70.7%) stopped participating in the study before they turned 18 years of age at their study visit(s). A total of 10 (24.4%) participants did not complete the AMIS interview or completed it in an online format due to COVID-19 restrictions after 1st March 2020. Finally, two (4.9%) participants identifying as Asian were excluded because the sample size was too small for the race/ethnicity analyses. For the attrition analyses, the excluded cohort was 2 years (95% CI −2.67 to −0.96; p < .001) younger than the participants who completed the AMIS interview; participants remained comparable on all other key sample characteristics (i.e., sex, race, income, lesion level; all p > .05).

Health status

On average, participants reported positive physical (M = 42.7, SD = 10.0) and mental (M = 48.2, SD = 9.95) health. Regarding the ability to carry-out every day physical activities, 48 (48.5%) respondents selected very good or excellent. About a third of participants rated their general perception of health (from single item) very good to excellent.

Surprisingly, our first hypothesis regarding the association between contextual factors, process factors, and higher self-management with better health status (i.e., physical, mental, overall health) was only partially supported. No variable was associated with global physical health and only a few contextual factors were associated with mental and overall health. Controlling for other variables, younger age (b = −1.01, CI: −1.93 to −0.09, p = .03) and male sex were associated with better global mental health (males vs. females: M_diff = 4.50, CI: 0.06–8.93, p = .047). Regarding overall health, females were less likely than males to report high levels of overall health (OR = 0.39, CI: 0.17–0.91; p = .03). Put another way, 24.4% (n = 10/41) of females versus 44.7% (n = 17/38) of males reported that their health was very good or excellent.

Health complications and comorbidities

Frequencies of health complications and comorbidities are summarized in Table 2, which lists data for both Time 5 and Time 6. Of note, at Time 6, urinary tract infections, bladder and bowel accidents occurred in 37–42% of the sample over the previous 6 months.¹ Using the PROMIS GI Incontinence measure (range: 0–20 points), participants reported moderate incontinence issues in the prior 7 days (M = 6.53, SD = 3.03). Pressure injuries were reported in 19–22% of the sample across Times 5 and 6. Less than 5% reported shunt infections, tethered cord, or bone fractures. About a third of the sample had missing data on health complications and comorbidities at Time 5 and ≤10% had missing data at Time 6.

Table 2.

Study outcomes.

	Overall (N = 99)
	Time 5	Time 6
Health complications
Urinary tract infections
No	40 (40.4%)	54 (54.5%)
Yes	27 (27.3%)	37 (37.4%)
Missing	32 (32.3%)	8 (8.1%)
Urinary accidents
No	35 (35.4%)	47 (47.5%)
Yes	31 (31.3%)	42 (42.4%)
Missing	33 (33.3%)	10 (10.1%)
Bowel accidents
No	44 (44.4%)	53 (53.5%)
Yes	23 (23.2%)	37 (37.4%)
Missing	32 (32.3%)	9 (9.1%)
PROMIS bowel incontinence
Mean (SD)		6.53 (3.03)
Median [Min, Max]		5.00 [2.00, 18.0]
Missing		8 (8.1%)
Pressure injuries
No	48 (48.5%)	69 (69.7%)
Yes	19 (19.2%)	22 (22.2%)
Missing	32 (32.3%)	8 (8.1%)
Comorbidities
Diabetes
No	27 (27.3%)	41 (41.4%)
Yes	38 (38.4%)	47 (47.5%)
Missing	34 (34.3%)	11 (11.1%)
Obesity
No	30 (30.3%)	40 (40.4%)
Yes	35 (35.4%)	47 (47.5%)
Missing	34 (34.3%)	12 (12.1%)
Sleep apnea
No	24 (24.2%)	43 (43.4%)
Yes	41 (41.4%)	45 (45.5%)
Missing	34 (34.3%)	11 (11.1%)
Latex allergy
No	36 (36.4%)	43 (43.4%)
Yes	29 (29.3%)	45 (45.5%)
Missing	34 (34.3%)	11 (11.1%)
Cardiovascular disease
No	26 (26.3%)	42 (42.4%)
Yes	39 (39.4%)	46 (46.5%)
Missing	34 (34.3%)	11 (11.1%)
Metabolic syndrome
No	26 (26.3%)	41 (41.4%)
Yes	39 (39.4%)	47 (47.5%)
Missing	34 (34.3%)	11 (11.1%)
Osteoporosis
No	25 (25.3%)	41 (41.4%)
Yes	40 (40.4%)	47 (47.5%)
Missing	34 (34.3%)	11 (11.1%)
Chronic pain
No	24 (24.2%)	40 (40.4%)
Yes	41 (41.4%)	48 (48.5%)
Missing	34 (34.3%)	11 (11.1%)
Health status
PROMIS global physical health
Mean (SD)		42.7 (10.9)
Median [Min, Max]		42.3 [19.9, 67.7]
Missing		6 (6.1%)
PROMIS global mental health
Mean (SD)		48.2 (9.95)
Median [Min, Max]		45.8 [25.1, 67.6]
Missing		6 (6.1%)
PROMIS global self-rated health
Excellent		12 (12.1%)
Very good		20 (20.2%)
Good		44 (44.4%)
Fair		16 (16.2%)
Poor		1 (1.0%)
Missing		6 (6.1%)
Quality of life
Individual psychosocial health-related quality of life
Mean (SD)		71.2 (18.4)
Median [Min, Max]		71.7 [8.33, 100]
Missing		16 (16.2%)
Global family quality of life
Mean (SD)		84.2 (14.7)
Median [Min, Max]		86.7 [37.5, 100]
Missing		15 (15.2%)

Our second hypothesis that high self-management would be associated with lower odds of health complications and comorbidities was also partially supported. In the univariate analysis, none of the self-management scores were associated with health complications reported over the previous six months. See Table 3 for factors associated with gastrointestinal incontinence. When compared to participants with a thoracic lesion level, those with a sacral lesion reported worse incontinence on the GI Incontinence scale (b = 2.92, CI: 0.31–5.53, p = .03). And controlling for all other variables in the model, every 10-point increase in executive dysfunction worsened incontinence by about 0.70 points on average (b = 0.07, CI: 0.01–0.13; p = .04). Additionally, participants identifying as White reported less severe incontinence relative to those identifying as Black (b = −2.43, CI: −4.79 to −0.08; p = .04).

Table 3.

Factors associated with PROMIS gastrointestinal incontinence.

		Univariate		Multivariate
	Valid N	$\widehat{\beta }$ (95% CI)	p	$\widehat{\beta }$ (95% CI)	p
Age	77	0.20 (−0.10 to 0.50)	.18	0.22 (−0.07 to 0.73)	.14
Sex female vs. male	77	−0.57 (−2.00 to 0.87)	.44
Race	77		.12a		.046a
White vs. Black		−2.09 (−4.50 to 0.32)	.10	−2.43 (−4.79 to −0.08)	.04
White vs. Hispanic/Latino		−0.57 (−2.60 to 1.46)	.78	−1.56 (−3.61 to 0.50)	.17
Black vs. Hispanic/Latino		1.52 (−1.22 to 4.27)	.39	1.60 (−1.96 to 3.71)	.74
Family income >50K vs. <50K	70	−0.16 (−1.92 to 1.60)	.86
Shunt no vs. yes	77	−0.36 (−2.23 to 1.51)	.70
Lesion level	75		.04a		.02a
Sacral vs. lumbar		0.35 (−1.59 to 2.29)	.90	0.60 (−1.23 to 2.42)	.71
Sacral vs. thoracic		2.52 (0.05 to 5.01)	.045	2.92 (0.31 to 5.53)	.03
Lumbar vs. thoracic		2.18 (−0.12 to 4.50)	.07	2.33 (−0.20 to 4.85)	.08
BRIEF behavioral regulation index	76	0.06 (−0.01 to 0.13)	.11
BRIEF metacognitive index	76	0.07 (0.01 to 0.13)	.02	0.07 (0.01 to 0.13)	.04
Family cohesion observational (per 1 point increase)	74	−0.88 (−2.78 to 1.03)	.36
Number of family stress events parent-reported (FILE)	70		.65a
<10 vs. 10–20 stress events		−0.19 (−2.22 to 1.84)	.97
<10 vs. ≥20 stress events		−1.18 (−4.24 to 1.88)	.63
10–20 vs. ≥20 stress events		−0.99 (−4.18 to 2.19)	.74
AMIS score (per 1 point increase)	77
Baseline total		0.29 (−0.30 to 0.87)	.33	0.30 (−0.41 to 1.00)	.80
Last total		0.22 (−0.29 to 0.74)	.39
Baseline condition		0.13 (−0.37 to 0.62)	.61
Last condition		0.08 (−0.39 to 0.56)	.73
Baseline independent living		0.38 (−0.22 to 0.98)	.21
Last independent living		0.30 (−0.21 to 0.80)	.25

Note: Valid N = The number of respondents used to compute the univariate estimate. The number of respondents used to compute the multivariate estimates = 75.

^aOverall Type III significance value.

Regarding the association between self-management and comorbidities, univariate estimates are reported in Table 4. A 1-point increase on the total self-management scale (range: 1–7) was associated with 29–36% lower odds of diabetes, obesity, metabolic dysfunction, and osteoporosis (all p < .05). These findings did not depend on IQ (all interactions p > .05) and remained consistent for both AMIS self-management subscales (i.e., condition and independent living).

Table 4.

AMIS scores associated with health complications and comorbidities.

		AMIS
		Total		Condition		Independent living
	Valid N	OR (95% CI)	p	OR (95% CI)	p	OR (95% CI)	p
Health complications
Urinary tract infections	94	1.16 (0.87–1.55)	.31	0.99 (0.76–1.27)	.92	1.14 (0.86–1.51)	.12
Urinary accidents	94	0.96 (0.75–1.23)	.75	0.95 (0.77–1.18)	.67	0.97 (0.76–1.23)	.78
Bowel accidents	94	0.91 (0.69–1.19)	.48	0.96 (0.75–1.24)	.77	0.88 (0.67–1.14)	.33
Pressure injuries	94	0.99 (0.73–1.34)	.96	1.04 (0.78–1.40)	.79	0.96 (0.71–1.28)	.77
Comorbidities
Diabetes	91	0.71 (0.54–0.94)	.02	0.70 (0.54–0.90)	.01	0.77 (0.59–1.00)	.054
Obesity	91	0.69 (0.52–0.92)	.01	0.68 (0.52–0.90)	.01	0.74 (0.57–0.97)	.03
Sleep apnea	91	0.91 (0.69–1.22)	.54	0.87 (0.67–1.13)	.29	0.96 (0.73–1.27)	.79
Latex allergy	91	1.20 (0.89–1.61)	.24	1.14 (0.87–1.49)	.34	1.20 (0.90–1.60)	.21
Cardiovascular disease	91	0.77 (0.58–1.01)	.06	0.75 (0.58–0.98)	.02	0.83 (0.64–1.09)	.18
Metabolic dysfunction	91	0.71 (0.53–0.94)	.02	0.69 (0.53–0.91)	.01	0.76 (0.58–1.00)	.047
Osteoporosis	91	0.64 (0.45–0.91)	.01	0.69 (0.53–0.90)	.01	0.78 (0.59–1.02)	.07
Chronic pain	91	0.86 (0.67–1.11)	.24	0.78 (0.61–1.00)	.049	0.94 (0.73–1.21)	.63

Note: Valid N = The number of respondents used to compute the univariate estimate.

Individual health-related QOL and family QOL

On average, youth reported that their level of individual QOL (psychosocial health-related QOL) was good (M = 71.20, SD = 18.4). Notably, there was high variability in the scores for youth with SB. As for global family QOL, youth also reported high scores (M = 84.20, SD = 14.70).

The third hypothesis was supported for individual health-related QOL and only partially supported for family QOL. See Table 5 for factors associated with Psychosocial Health-Related Quality of Life. Controlling for participants’ age, family income, and BRIEF Behavioral Regulation Index score, the most recent AMIS independent living score was associated with improved psychosocial health-related QOL (b = 4.64, CI: 0.91–8.36; p = .02). However, executive dysfunction moderated the association between family QOL and the most recent independent living self-management score (i.e., score most proximal to outcome)—even after controlling for age (interaction p = .049); no other contextual or process factor improved the model fit. Figure 2 shows the association between independent living self-management and global family quality of life stratified by level of executive dysfunction using the BRIEF Behavioral Regulation Index score. The most recent independent living self-management score was positively associated with global family QOL at low levels of executive dysfunction.

Table 5.

Factors associated with psychosocial health-related quality of life.

		Univariate		Multivariate
	Valid N	$\widehat{\beta }$ (95% CI)	p	$\widehat{\beta }$ (95% CI)	p
Age	72	−1.59 (−3.46 to 0.28)	.10	−1.75 (−3.64 to 0.13)	.07
Sex	72	−3.53 (−12.59 to 5.53)	.44
Race	72		.51a
White vs. Black		−1.39 (−16.43 to 13.60)	.97
White vs. Hispanic/Latino		5.96 (−7.51 to 19.40)	.54
Black vs. Hispanic/Latino		7.36 (−10.27 to 25.00)	.58
Family income >50K vs. <50K	66	12.05 (1.64 to 22.45)	.02	9.23 (−0.78 to 19.24)	.07
Shunt no vs. yes	72	5.40 (−5.71 to 16.51)	.34
Lesion level	70		.64a
Sacral vs. lumbar		4.33 (−7.78 to 16.40)	.67
Sacral vs. thoracic		5.42 (−12.64 to 23.50)	.75
Lumbar vs. thoracic		1.09 (−16.08 to 18.30)	.99
BRIEF behavioral regulation index	71	−0.43 (−0.92 to 0.07)	.09	−0.42 (−0.94 to 0.11)	.12
BRIEF metacognitive index	71	−0.23 (−0.61 to 0.14)	.22
Family cohesion observational (per 1 point increase)	70	1.58 (−10.16 to 13.33)	.79
Number of family stress events parent-reported (FILE)	66		.47a
<10 vs. 10–20 stress events		4.88 (−7.59 to 17.40)	.62
<10 vs. ≥20 stress events		−4.04 (−22.06 to 14.00)	.85
10–20 vs. ≥ 20 stress events		−8.92 (−27.96 to 10.10)	.50
AMIS score (per 1 point increase)	72
Baseline total		3.99 (−0.22 to 8.20)	.06
Last total		3.58 (−0.15 to 7.31)	.06
Baseline condition		3.42 (−0.24 to 7.08)	.07
Last condition		2.89 (−0.73 to 6.51)	.12
Baseline independent living		3.57 (−0.57 to 7.70)	.09
Last independent living		3.49 (0.01 to 6.97)	.049	4.64 (0.91 to 8.36)	.02

Note: Valid N = The number of respondents used to compute the univariate estimate. The number of respondents used to compute the multivariate estimates = 66.

^aOverall Type III significance value.

Figure 2.

Association between independent living self-management and global family quality of life by level of executive dysfunction.

Discussion

The current study examined associations between self-management with health status and QOL, after controlling for predictors of self-management, and findings indicated there were distinct predictors of outcomes. On average, youth with SB between 18 and 27 years of age reported good health status and psychosocial health-related QOL when compared to published norms. Overall health as reported on a single item was moderate but physical and mental health was good and very good, respectively. Surprisingly in youth’s report regarding the extent to which they carry out everyday activities (i.e., physical health), the majority of responses were between “mostly” and “completely” (scores of 4 and 5). Participants in this age group have perhaps developed adaptations for physical function that demonstrate improvements or have not begun to experience mobility or ambulation decline as reported in clinic populations with about 50% primary wheelchair users over 18 years old [48]. While no predictors of physical health were identified, a baseline of good physical health was established when compared to cut points in the general US population (42–50 good) [41].

Regarding global mental health, though rated positively, findings indicated that mental health worsens with age (1-point per year). Hence, each decade into adulthood, mental health is projected to decline, and females are at greatest risk since they are already half a standard deviation lower than males. A higher proportion of females also reported lower levels of overall health. Certainly, these findings suggest that clinicians should monitor global health status, particularly as youth enter adult-oriented care settings and suggest that interventions should target women’s mental health as a priority. Although self-management and other predictors were not associated with health status in this study, ongoing assessment of self-management behaviors are recommended to prevent health complications and comorbidities.

Our study did not detect an association between self-management and the presence of health complications including urinary tract infections (UTI), urinary accidents, bowel accidents, and pressure injuries reported in the last 6 months. This finding was contrary to expectations and may be due to measurement error; one-third of the sample had missing data at Time 5 on health complications and comorbidities. Time 5 was the first-time participants were asked to report on these data for the previous 6 months in a checklist format. The exposure to the questionnaire may have prepared youth to provide more complete data at the last data collection. Despite missing data, the prevalence of UTIs was up to 37%, which was consistent with the prevalence in a large survey of US adults with SB [49]. UTIs are a common preventable complication that lead to hospitalizations [50] and early mortality. The diagnosis and treatment of UTIs are complex and recurrence is likely. On the other hand, strategies for preventing UTIs are understudied and lack guidelines, especially for women at highest risk of infection [51].

Urinary and bowel accidents (i.e., incontinence) were much lower (37–42% at Time 6) than rates of National Patient Spina Bifida Registry (NSBPR) participants (59%). This difference may be attributed to definitions. In the current sample, no definition of urinary or bowel accidents was provided. Therefore, responses were based on their perception of accidents. Although prompted to respond based on the last 6 months, participants may have difficulty remembering whether they had instances of leakage which may have resulted in underreporting. In the context of health care appointments, registry data were collected on the frequency of urinary and bowel incontinence during the day over the last month (e.g., daily, weekly, monthly, less than monthly, and never) [52]. It may be that asking these types of questions in alternative formats is important to best capture increasing or burdensome incontinence.

The PROMIS GI Incontinence measure provided more information about incontinence issues over a recall period of 7 days. Findings indicated moderate incontinence issues; participants with lower lesion levels reported more frequent bowel incontinence issues (i.e., sacral vs. thoracic). This finding may be explained by the physiology of the spinal lesions. In lower (e.g., sacral) lesion levels, participants typically experience both constipation and incontinence [53,54]. For participants with higher lesion (e.g., thoracic) levels, neurogenic bowel patterns result in stool retention and constipation, which we did not assess in this study. However, incontinence in young adulthood is a priority for providers to address at a time for decision-making about medical procedures to manage increasing incontinence. The findings also revealed that high executive dysfunction was associated with more bowel incontinence. These findings confirmed that interventions for incontinence must be adapted for cognitive functioning. Clinicians have begun to develop practice guidelines that emphasize these adaptations; see Gandy et al. [55] for clinical recommendations.

Pressure injuries (i.e., skin breakdown) were another concerning complication in our sample, with a prevalence rate of 22%. This was consistent with reports from patients enrolled in the NSBPR cohort (20–24% in those 20–25 years old with and without myelomeningocele type). The prevalence of skin breakdown in the US survey with a broad adult age range reached an alarming rate of 43% [49]; however, in NSBPR, adults over 20 years old had a 25% prevalence of pressure ulcers in the last year [56]. These pressure ulcers are life threatening secondary conditions as noted in a study across multiple clinics, which found a startling 30% of deceased patients had a history of skin ulcer debridement, 67% of which were reported within 12 months of death [5].

The leading causes of death in SB are from infections (e.g., urinary sepsis, skin ulcer, or wound) [4,5] that could be prevented or minimized through self-management behaviors and health maintenance. See Bradko et al. [57] for a recent review on risk assessment and management of skin injury as well as resources from the Spina Bifida Association (SBA). Guidelines for integument (skin) care [58] provide specific approaches across the lifespan and the SBA has curated a set of interventions for skin integrity including education materials for health care providers and an information campaign called “Did You Look?” available on their website [59]. Indeed, maintenance of skin integrity is critical and development of effective early and ongoing prevention interventions are needed.

Study findings regarding comorbidities indicated that higher self-management scores were associated with a reduced odds of preventable conditions, such as diabetes, obesity, metabolic dysfunction, and osteoporosis. This set of findings is important because these youth already have a high prevalence of these secondary conditions at this age; almost half of the sample endorsed comorbid conditions. For obesity, the findings were consistent with the weight status of pediatric registry participants (up to 45% overall) [60]; however, diabetes prevalence (47.5%) is the most concerning, since this rate is much higher in this study than has been reported in other publications in the SB population [61]. The risk for chronic kidney disease in this population is heightened by the presence of these multiple morbidities and onset occurs 10 years earlier than in adults without SB [61]. The impact of self-management for risk reduction is promising and efforts to assess and promote engagement in health care and lifestyle behaviors, such as nutrition and physical activity participation are essential for self-management [62,63].

Youth ratings for health-related QOL were encouraging and demonstrated resilience. Findings were comparable to college age youth with and without chronic health conditions (M = 71.20, SD = 18.40; M = 67.99, SD = 11.85; M = 73.87, SD = 10.53, respectively) [42]. An 8.5-point increase was noted between scores when youth were 8–15 years old and 18–27 years. Controlling for age, family income, and executive function (Behavioral Regulation Index), the most recent rating of independent living self-management was associated with psychosocial health-related QOL. This finding draws attention to the importance of independent living behaviors (e.g., ordering medication/supplies, household tasks, community living) in this age group. Youth over 18 years of age still require moderate assistance and perform activities 50–74% of the time on their own [19].

An increase in health-related QOL by 5 points on the psychosocial health summary score for every 1-point increase in the AMIS independent living score approximates the meaningful difference score on the PedsQL reported by Varni and Limbers [42] (5.88) between young adults with and without chronic health conditions. This meaningful increase in health-related QOL for every point increase in self-management demonstrates the expected impact of enhancing self-management approaches.

As for global family QOL, youth also reported high scores although slightly lower than published data from youth 12–25 years old with and without SB (M = 87.40, SD = 15.28) [16]. Controlling for age, the association between independent living self-management and family QOL depended on the participants’ level of executive dysfunction. When executive dysfunction was low, the association between self-management and family QOL was positive and significant. When executive dysfunction was high, the association between self-management and family QOL was negative but not significant. No other factor was associated with family QOL. A possible explanation is that youth are intentionally focused on rapidly gaining the skills needed for independence transitioning to work and higher education goals. While an “engaged family” was essential to QOL [40], family roles and relationships are shifting and being redefined. In this way, the measure extended participants ratings on their perspectives of family members beyond parents; however, further analyses examining the frequencies of specific family members as youth age will be helpful in identifying sources of caregiver assistance and family support for self-management.

Limitations and future research

The present study has a number of strengths and limitations. First, this was a longitudinal study which used multiple observations of self-management. However, we selected a baseline or last rating of the self-management trajectory for inclusion in analyses. This enabled us to examine self-management to predict distal outcomes that were collected at Time 5 and/or Time 6. This also led to models which controlled for a baseline self-management total score when predicting health status and determined that the last independent living score was salient for QOL outcomes. Second, this study included contextual and process predictors of self-management from prior work in analyses. Controlling for known correlates of self-management increased the predictive validity of findings. Third, this study provided novel findings using the PROMIS global health scores for young adults living with SB. A limitation of this study was the high proportion of missing data at Time 5 on health complications and comorbidities. About a third of participants had missing data for these outcomes but this was reduced to <10% at Time 6. The reason for these items being missing may be due to the checklist format and recall period of 6 months. A shorter recall period for health complications, such as incontinence may reduce missing data in future research. Experience sampling methods, such as daily diaries or ecological momentary assessment are innovative methods being applied in SB [64,65]. Another limitation of this study is the use of a single source for outcomes. Future studies may be strengthened by including multi-source, such as the medical record or multiple informant (other family members) when collecting data on health outcomes. This study did, however, use multiple informant data for the contextual and process factors (e.g., demographic, executive function, family functioning). Finally, our interpretation of the sex differences in mental health status is tempered by the potential for social desirability response bias. The use of the PROMIS Global health measure will be useful in future studies of adults with SB to increase our understanding of subgroups that require more support and care.

Conclusions and clinical implications for rehabilitation

The present study provided evidence of a positive health status and QOL from the perspective of youth with SB; however, findings indicated that mental health declined as participants aged, and females rated their mental and overall health lower than males. Thus, it is important for models of care to evolve for integrated care including mental health assessment and accessible treatment options. Development of gender-specific interventions that combine self-management with mental health strategies are needed. Importantly, in this study, youth with a lower lesion level and more difficulty with executive function had more gastrointestinal incontinence issues highlighting the need for adaptive clinical interventions. Findings provide new evidence regarding the importance of prioritizing support for increasing independent living self-management to prevent comorbid conditions and improve individual health-related and family QOL. Ongoing assessments and psychosocial interventions for mental health are imperative in this population to monitor health status and prevent decline. Supporting families while youth gain independence in self-management, with specific adaptations for executive dysfunction, may help to preserve a high family QOL.

➤ IMPLICATIONS FOR REHABILITATION.

• Integrated models of care, including mental health assessment, are important for people living with spina bifida.

• Gender-specific interventions that combine self-management with mental health strategies are needed to prevent decline in mental health.

• Youth with a lower lesion level and more difficulty with executive function had more difficulty with gastrointestinal incontinence, highlighting the need for adaptive clinical interventions.

• Prioritizing support for increasing independent living self-management may prevent comorbid conditions and improve individual health-related and family QOL.