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Disability and Health Journal
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Research Paper
Perception of secondary conditions in adults with spina bifida and impact on daily life Robert Wagner, M.D.a,*, Ronna Linroth, O.T., Ph.D.a, Candice Gangl, O.T.D., O.T.R/L.a, Nancy Mitchell, M.A., O.T.R/L.a, Michelle Hall, C.P.O., F.A.A.O.P.a, Rhonda Cady, Ph.D., R.N.a, and Matthew Christenson, B.S.b a
Gillette Children’s Specialty Healthcare, St. Paul, MN, USA b Professional Data Analysts, Inc., Minneapolis, MN, USA
Abstract Background: Spina bifida is a congenital defect of the neural tube resulting in motor and sensory disruption. Persons with spina bifida can also experience executive function impairments. Secondary conditions are physical, medical, cognitive, emotional, or psychosocial consequences to which persons with disabilities are more susceptible. Our experience suggested clinicians underappreciate the presence and impact of secondary conditions in adults with spina bifida because they do not specifically ask for this information. Objective: Describe the presence and impact of secondary conditions on daily life, as perceived by adults with spina bifida. Methods: A clinic-based sample was recruited from the active patient population of an adult specialty center for spina bifida-related care. All subjects were verbally administered a survey developed through literature review and clinical experience of the researchers. The survey measured the presence and perceived impact of secondary conditions. Recruitment and survey data collection occurred over a 6-month period to maximize age representation. Survey data were stratified by age, gender and lesion level for analysis. Results: Seventy-two respondents completed the survey. Pain was commonly reported, along with pressure ulcers, bowel & bladder concerns, depression, sleep disturbance, and limited social and community participation. No significant relationships were found between the presence or perceived impact of secondary conditions and age, gender or level of lesion. Conclusions: Secondary conditions in spina bifida are present by early adulthood. Identifying these conditions during clinical encounters requires specific rather than general questions. Future study should evaluate earlier initiation of preventative measures by pediatric providers. Ó 2015 Elsevier Inc. All rights reserved. Keywords: Spina bifida; Secondary conditions; Adults; Impact of disability; Patient perceptions
Spina bifida is a fault (dysraphism) in the development of the spine and spinal cord resulting in a gap or lesion in the spine.1,2 Classification is based on the level of the lesion in the spine and the presence or absence of hydrocephalus.3 In general, the higher the spinal lesion, the greater the neurologic and orthopedic impairment.3 This congenital neurodevelopmental disorder is a common disabling birth defect in the United States, and surveillance data aggregated from the National Birth Defect Prevention Network4 estimated a prevalence of 3.5 cases per 10,000 This manuscript has not been published and will not be submitted elsewhere for publication while being considered by the journal Disability Health Journal. Preliminary information from this study was shared at the second World Congress on Spina Bifida in 2012. The authors have no conflicts of interest to disclose. * Corresponding author. 435 Phalen Boulevard, St. Paul, MN 55130, USA. Tel.: þ1 651 634 1937; fax: þ1 651 265 7356. E-mail address: [email protected] (R. Wagner). 1936-6574/$ - see front matter Ó 2015 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.dhjo.2015.03.012
live U.S. births.5 Overall survival has improved and in the United States over 75% of persons with spina bifida live into early adulthood.6 With the growing number of adults with spina bifida, research into long-term health needs is warranted. The purpose of our study was to describe how adults with spina bifida perceive the presence and impact of their secondary conditions on daily life, and to use this information to better screen for secondary conditions during clinical encounters. Secondary conditions are broadly defined as, ‘‘those physical, medical, cognitive, emotional, or psychosocial consequences to which persons with disabilities are more susceptible by virtue of an underlying condition, including adverse outcomes in health, wellness, participation, and quality of life.’’7(p186) Adults with spina bifida are at risk for secondary conditions such as bowel and bladder problems, pain, fatigue, injury, depression, obesity and pressure sores or ulcers because of motor and sensory disruption at
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the level of the spinal cord lesion. The impact of secondary conditions has only recently appeared in disability literature. Secondary data analysis of the Nationwide Inpatient Sample from the 2004e05 Healthcare Cost Utilization Project9 found one-third of hospitalizations for adults with spina bifida attributed to potentially preventable secondary conditions.10 Another retrospective review identified problems associated with aging and spina bifida that included pressure ulcers and resulting amputation, scoliosis, urinary incontinence, social isolation, and limited employment.11 While evidence exists that secondary conditions have negative clinical implications, missing from literature is a description of the perceived impact of secondary conditions on a person’s daily life.3,12 Persons with spina bifida are also subject to less visible executive function impairments.8,13 Executive dysfunction is a complex phenomenon not limited to spina bifida and more easily characterized than defined. ‘‘Executive functions are those involved in complex cognitions, such as solving novel problems, modifying behavior in the light of new information, generating strategies, or sequencing complex actions.’’14(p50) Failure to attend to the impact of executive dysfunction when interviewing a person with spina bifida will limit a clinician’s ability to recognize and attend to early manifestations of problems. The International Classification of Functioning, Disability, and Health (ICF) is a World Health Organization (WHO) framework for measuring health and disability at both individual and population levels.15 Since anyone can be at risk for a change in health status that results in some manner of disability, disability is seen as a universal human experience and not something affecting only a minority of the population. The WHO ICF framework serves as a conceptual guide and justification for the current study, which emphasizes the perceived impact of secondary conditions as distinct from their clinical severity. The objective of this descriptive study was to explore two related questions: 1) how common are secondary conditions in our patients with spina bifida? and; 2) how do secondary conditions impact people’s lives? Survey data collected from consenting adults attending a specialty spina bifida clinic was analyzed to test for a relationship between age, gender and level of lesion on the perceived presence and impact of secondary conditions. The study was designed with attention to executive function challenges to obtain the most accurate information possible without clinical confirmation.
Methods Setting and respondents The study was conducted in the outpatient clinic of Gillette Lifetime Specialty Healthcare (GLSH). The research protocol was approved by the governing Institutional Review Board (IRB) and procedures followed were
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in accordance with the ethical standards of the IRB and with the Helsinki Declaration of 1975 as revised in 2000. GLSH was established in 2001 to address the specialty health needs of adolescents and adults with childhood-onset disabilities e including spina bifida e in an adult-oriented, integrated, multi-disciplinary, rehabilitation-focused environment.16 Our service is distinct from the main pediatric services provided by the parent organization, Gillette Children’s Specialty Healthcare.17 GLSH is one of nine programs serving the longitudinal needs of adults with spina bifida.18 Clients seen at GLSH come from the surrounding metropolitan area, Greater Minnesota, Iowa and Wisconsin and range from formal physician referrals to self-referrals based on ‘word of mouth’ within the disability community. Clients with spina bifida vary in degree of impairment. All our clients are adults and while survival to adulthood has improved, an association between level of spinal lesion and survival has been established.6,19 An information packet was sent to all GLSH patients, age 18 or older, with a primary diagnosis of spina bifida (n 5 192). The packet included a letter describing the study, and copies of consent and survey forms, to allow participants and when applicable, legal guardians, sufficient time to read these materials and formulate questions prior to recruitment. Recruitment was conducted inperson when 97 of the 192 adults with spina bifida presented for clinic appointments during a subsequent six month period. Each potential subject was approached for participation interest and screened for recruitment eligibility, which was the ability to understand and answer questions independently. This criterion was based on clinical judgment and patient/caregiver input. No formal assessment tool was used. Of the 97 persons approached for recruitment, five did not meet screening criteria, 20 declined participation and 72 provided written informed consent. Data collection The study used a quantitative survey to collect information about secondary conditions experienced by adults, age 18 and older, with spina bifida. The spina bifida secondary conditions (SBSC) survey tool is specific to adults with this condition. Development of the SBSC incorporated emerging adult milestone domains defined by Zukerman, Devine and Holmbeck,20 review of the current literature, and the clinical experience of our multi-disciplinary study team. We know executive function14 challenges occur in persons with spina bifida.13 During a universal screening program for foot health in our adult spina bifida clinic, we found foot lesions even when patients answered ‘‘no problems’’ to the question, ‘‘Do you have any problems?’’ Our foot screening experience suggested that general questions do not elicit accurate information and we paid particular attention to how we worded the SBSC survey questions, using simple and concrete language to minimize misunderstanding. Face validity of the SBSC was
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established by the Gillette Lifetime Consumer Advisory Group, a nine member group consisting of current Gillette Lifetime consumers with Cerebral Palsy, Spina Bifida and other disabling conditions. This group of consumer experts reviewed the SBSC and discussed whether the survey items addressed the range of secondary conditions experienced by adults like themselves. Minor changes were suggested but details of this review were lost. All suggested changes were incorporated into the final SBSC but establishment of content validity is not possible. No additional survey psychometrics or cognitive testing was conducted. The SBSC survey measured respondent perception of the presence of secondary conditions and the impact of these conditions on their lives. No clinical measurements or assessments were collected, no data was abstracted from the subject’s medical record, and no interventions were provided. The survey was verbally administered, in-person or by telephone, to all respondents over a six month period, by two of the authors (R.L., C.G). Both were trained using mock survey administration sessions with volunteers (n 5 2) matching respondent eligibility criteria. Feedback regarding consistency in administering the interview script was solicited from the volunteers, but data to support formal inter-rater reliability testing was not collected during these sessions. Periodic meetings between the surveyors during the six month data collection period ensured consistency in administering the interview script. Informed consent, survey administration, and collection of demographic information took approximately 1 h per participant. A $10 gift card was provided at the end of the survey session. Domains of the SBSC include mobility, activities of daily living, emerging adult milestones and secondary musculoskeletal, skin, gastrointestinal, urological, pain and mental health conditions (Appendix A). Respondents are asked to reflect on the past 12 months when answering survey questions. Respondents characterize mobility using defined options: transferring, wheelchair use, and ambulation. Difficulty performing activities of daily living (ADL) and the perceived impact of the difficulty is assessed for the seven domains listed in Table 2. This differs from standard ADL measurements, which focus on clinician assessment of functional status.21 Self-perception of emerging adulthood milestones20 is assessed by asking respondents’ whether having spina bifida affects their ability to reach these milestones (Table 3). The perceived presence of musculoskeletal, skin, gastrointestinal, urological, pain and mental health secondary conditions are assessed by asking if the respondent experienced the condition. Respondents answering ‘yes’ are asked to rate the impact of the condition on daily life as not at all, somewhat, or greatly (Table 4). This approach differs from standard pain measurements, which focus on pain intensity using numerical or visual scales.21 Comments from respondents that could not be quantified on the survey form are captured for later use.
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Table 1 Respondent demographics
Demographic category Gender Female Male Age 18e24 years 25e44 45e68 O 68 Level of lesion Thoracic Lumbar Sacral Missing/unknown Hydrocephalus Yes No/missing/unknown Highest level of education High school College degree Currently working Yes No Living arrangement Independently, with or without assistance In group home or with family or friends a
Respondent sample (n 5 72)
Clinic population (n 5 185)a
n
%
n
47 25
65% 35%
98 87
53% 47%
23 37 12 0
32% 51% 17%
43 108 30 4
23% 59% 16% 2%
5 57 5 5
7% 79% 7% 7%
47 25
65% 35%
54 18
75% 25%
41 31
57% 43%
23
32%
49
68%
%
p-value 0.074
0.316
Data from 2013 clinic visits.
Data analysis Age and gender of the survey respondent sample is compared to the clinic spina bifida population. The perceived presence of general (n 5 5) and specific secondary (n 5 15) conditions (Table 4) is binary coded and counted and referred to as perceived condition count. The perceived impact of the same general and specific secondary conditions is binary coded and counted for responses of ‘‘somewhat’’ or ‘‘greatly’’ affecting daily life and referred to as perceived impact count (Table 4). The secondary condition pain is binary coded and counted by pain site (n 5 7) and referred to as perceived pain site count. Perceived condition count, perceived impact count and perceived pain site count are treated as separate continuous dependent variables. Independent categorical variables include gender, age category (18e24, 25e44, 45e68) and level of myelomeningocele lesion (thoracic, lumbar, and sacral). The null hypothesis for each dependent variable states no difference between categorical groups. Records with missing data are excluded from analysis. Descriptive and inferential analysis used SPSSÒ software (IBM Corp., Armonk, NY). Chi-square tests for similarity across independent variables, by age and gender, by age and level of lesion, and by gender and level of lesion. Analysis of variance
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Table 2 Respondent perception of ADL performance and impact of perceived difficulty on daily life Perceived difficulty as Activity of daily Perceived difficulty somewhat or greatly living (ADL) performing the ADL impacting daily life Dressing Cooking Bathing Transferring Walking Stair climb/descent Propelling wheelchair
21 25 18 23 52 50 11
(29%) (35%) (25%) (32%) (73%) (69%) (15%)
16 20 14 17 35 35 8
(76%) (80%) (78%) (74%) (67%) (70%) (72%)
(ANOVA) tests for differences between age category and perceived condition count, level of lesion and perceived condition count, age category and perceived impact count, and level of lesion and perceived impact count. Tukey HSD pairwise comparisons are made if overall ANOVA is statistically significant. Differences between gender and perceived condition count, and gender and perceived impact count are analyzed using the Student t-test. Perceived pain site count is tested against independent variable groups (age, level of lesion, gender) using similar AN OVA and t-test analysis. In addition, chi-square tests for similarities across independent variable group and individual pain site. A p-value less than 0.05 is considered statistically significant and rejects the null hypothesis.
Results All 72 consented individuals completed the SBSC survey, with 95% completing in-person and the remainder completing by telephone. Of the 72 respondents, 52 have been GLSH clinic patients for two or more years. Gender and age data reported by survey respondents (not drawn from the medical record) appear in Table 1, along with gender and age data extracted from the electronic medical record for the population of persons with spina bifida Table 3 Respondent perception of the impact of spina bifida on adult milestones Adult milestones Sometimes or always feel this way Having spina bifida affects my:
n
%
Ability to participate in the community Ability to go to college Choices in having children Plans for the future Personal relationships (dating, marriage, close friendships) Ability to work (employment) Independence Options of where I can live Transportation options (car, bus)
38
53%
18 39 40 40
25% 54% 56% 56%
37 35 36 37
51% 49% 50% 51%
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Table 4 Respondent perception of presence and impact of secondary conditions within past 12 months Perceived presence Perceived condition of condition as somewhat or General and specific greatly impacting secondary conditions n % daily life Musculoskeletal and skin Burn Pressure sore/skin breakdown Lower limb edema Fracture or broken bone Muscle or tendon injury Scoliosis Limited range of Motion Gastrointestional Constipation Stool incontinence Urological Urinary tract infection Urinary incontinence Mitrofanoff Mental health Depression Sleep problems Neurosurgical Shunt malfunction or infection Pain Wrist paina Neck pain Shoulder pain Back pain Knee pain Hip pain Other area of painb
72
59
62
50
6
65
100 9% 54% 50% 10% 21% 51% 50% 82% 65% 54% 86% 58% 36% 28% 69% 53% 61% 8% 8% 90% 32% 51% 54% 71% 32% 38% 24%
29% 69% 50% 86% 87% 46% 60% 83% 90% 79% 81% 74% 87% 98% n/a
52% 70% 79% 78% 70% 63% 94%
Chi-square analysis of pain site and age; p-value ! 0.05 considered statistically significant. b Chi-square analysis of pain site and gender; p-value ! 0.05 considered statistically significant. a
receiving care at GLSH. While the age distribution of survey respondents skews somewhat younger than the clinic’s overall spina bifida population, the mean and median age of respondents (33 and 31 years) is similar to the clinic population (34 and 32 years) and chi-square indicates no significant difference between respondent and clinic population age distributions (Table 1). The gender distribution of survey respondents skews female and when compared to the clinic population, this difference approaches statistical significance (Table 1). Chi-square of independent variables indicates similarity across groups with no significant difference when compared by age and gender ( p 5 0.524), age and level of lesion ( p 5 0.213) and gender and level of lesion ( p 5 0.969). Additional demographic data as reported by respondents is listed in Table 1. Over 80% of respondents have a lesion level of lumbar or lower. Five subjects did not know their lesion level and are subsequently excluded from all analysis using this variable. The majority of respondents reported
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living at home, not married or dating and a limited number of friends. Nearly half reported having a personal care attendant and for these persons, 39% reported qualifying for 24 h care. Over half the respondents did not drink alcohol and 85% did not smoke. Respondent’s perceived difficulty performing ADLs and the perceived impact of this difficulty on daily life are shown in Table 2. The majority of respondents (86%) perceived difficulty with at least one ADL and half perceived difficulty with three or more ADLs. Difficulty walking and climbing stairs is perceived as difficult by the majority of respondents while dressing, cooking, bathing, transferring and propelling a wheelchair is perceived as difficult by less than a third of respondents. When an ADL is perceived as difficult, the majority of respondents felt the difficulty somewhat or always impacted daily life. Self-perceptions of the effects of spina bifida on adulthood milestones and life choices are shown in Table 3. With the exception college attendance, 50% or more respondents perceived spina bifida as negatively affecting their ability to achieve these milestones. Perceived presence and impact of secondary conditions Data on the perceived presence and impact of general (italic) and specific (non-italic) secondary conditions appear in Table 4. All respondents reported the presence of least one secondary condition, and 97% reported the condition impacted daily life. The median number of perceived specific secondary is six and the median number of these secondary conditions having a perceived impact on daily life is five. Neurogenic bowel, predisposing to constipation and fecal incontinence and common in spina bifida, is reported by the majority of study respondents. Over half of respondents reported at least one UTI in the previous year. All respondents reported the presence of musculoskeletal and skin secondary conditions and 90% of respondents reported the presence of pain. Over 50% of respondents replied ‘‘yes’’ to the question ‘‘Do you have problems with depression?’’ ANOVA and t-test analysis fails to reject the null hypotheses which state that perceived presence and perceived impact of secondary conditions does not change with age, gender or level of lesion. No significant differences are found between age categories and the dependent variables perceived condition count, perceived impact count and perceived pain site count, and gender and the same dependent variables. A nearly significant relationship is found between level of lesion and perceived condition count, but Tukey HSD pairwise comparisons yield non-significant p-values (thoracic vs. lumbar p 5 0.117; thoracic vs. sacral p 5 0.932; lumbar vs. sacral p 5 0.283). Only chi-square analysis between independent variable group and individual pain site rejects the null hypothesis, with. respondents in the middle age group (25e44) reporting significantly more back pain than other age groups ( p 5 0.045), and females reporting significantly more ‘other pain’ than males ( p 5 0.020).
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Discussion This study explored the perceived presence and impact of secondary conditions on adults with spina bifida using patient-reported survey data. We hypothesized that secondary conditions are more prevalent in older than younger individuals based on increasing vulnerability with age. While secondary conditions are common in our study population, the perceived presence or impact did not increase significantly with age or vary significantly by gender or level of lesion. These findings suggest that young adults with spina bifida enter the adult care system with secondary conditions already present. Earlier initiation of secondary condition preventative measures by pediatric providers is warranted and future study of the impact of preventative interventions is needed. Our findings also suggest a competing explanation. Prior study of factors influencing medical research participation found Caucasian women under the age of 65 more likely to participate in research.22 Although the age distribution of our respondent sample matches previous prospective study of adults with spina bifida,11 the age and gender distribution of our respondent sample also matches medical research participation patterns,22 and could influence our findings. In addition, all respondents were recruited from a multi-disciplinary spina bifida specialty clinic and over 70% were clinic patients for two or more years. The majority of our younger respondent sample (18e68 years) benefited from advancements in pre-natal and post-natal screening and care.6,8 Over 80% have a lesion level of lumbar or lower and are the population most likely to survive to adulthood.6,19 Our findings could reflect the overall influence of these factors on the health of our respondents. To fully understand the relationship between age, gender, level of lesion and secondary conditions, prospective longitudinal outcomes research is needed. To our knowledge, this is the first report of perceived secondary conditions, from a sample that includes only adults with spina bifida. Our study design differs from other secondary conditions research, which used clinical data, historical medical record or claims data.11,23 Although our study relied on patient-reported data, the perceived presence and impact of specific secondary conditions match previous reports from the literature. Respondent report of stool incontinence (54%) match findings from a longitudinal study6 and report of urinary tract infection (58%) align with findings from retrospective review of clinical records.23 Musculoskeletal, gastrointestinal and urological secondary conditions are reported by more than twothirds of respondents as somewhat or greatly impacting their daily lives. These findings mirror retrospective reviews of national datasets and hospital records on the incidence of secondary conditions and hospitalization frequency10,11,24 and the association between urinary tract infections, pressure sores and health care usage.25 The specificity of our findings to objective clinical studies could
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be explained by our attention to executive dysfunction, and asking specific rather than general survey questions. It is common in clinical practice to ask a patient, ‘‘Do you have pain?’’ If the answer is ‘‘yes’’, we ask the location of the pain and the severity on a scale of 1e10. In our study, questions about pain are asked in the context of specific joints or body regions. Almost all respondents reported pain in at least one site and the majority perceived the pain as impacting their daily lives. These findings differ from prospective clinical assessment studies, where 28% of subjects report any type of pain,23 and 16% of subjects report back pain and 10.6% report neck pain.26 While the prospective study did not specify how the question of pain was asked, our findings suggest that drawing attention to a specific area of the body before asking about pain and the impact of pain on daily life may be a more sensitive approach to eliciting reports of pain in individuals with spina bifida. An association between disability and depression has been established27 and identifying mental health secondary conditions is a priority for persons with spina bifida. While our finding of perceived problems with depression approximates those from a study of young adults with spina bifida who completed the self-report Hopkins Symptom Checklist,28 it is far greater than the 5% reported in a prospective clinical assessment study.23 The high percentage of positive responses in our study compared to the clinical assessment study suggests that perceived depression may be more prevalent in the spina bifida population and warrants more focused investigation. Adolescents and young adults strive to achieve milestones of reduced parental dependency and increased selfmanagement activities.29 The majority of our respondents self-reported limited work and social opportunities. Surveyors observed in many respondents e and in themselves e a sense of sadness when they asked about the number of friends. Comments from respondents indicated that those with computer access or a cell phone had greater connectedness and social participation than those without these resources. These findings are consistent with established research on achieving adult milestones.20 An examination of transition issues of young adults with spina bifida labeled ‘‘struggling for independence, limited social interactions and stigma and building inner strength’’ 29 (p869) as key themes, while a national survey found the presence of secondary conditions decreased the likelihood of employment for adults with disability.30 Respondents selfreported varying levels of participation in activities of daily living based on the availability of assistance and personal preference relative to how they prioritize expenditure of effort, time, and energy consumption. Early identification and corresponding interventions that reduce the impact of secondary conditions could facilitate achievement of adult milestones in persons with spina bifida. Future studies should address this issue.
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Limitations We acknowledge inherent limitations in our study design that limit the ability to generalize results to the large population of adults with spina bifida. A survey instrument was developed specifically for this study because no validated instrument was available. While content validity was assessed by our Consumer Advisory Group, the inability to report these findings limits validity of the SBSC. Other properties that could influence results but were not quantified (e.g. cognitive ability, socioeconomic status) are unknown. The secondary conditions surveyed by the SBSC are specific to spina bifida and do not include secondary conditions related to aging or lifestyle, such as obesity, diabetes, hypertension and asthma. Including these conditions could influence the results of this study. Sampling is limited by availability of respondents presenting during scheduled clinic appointments at GLSH multi-disciplinary spina bifida clinic and meeting screening criteria. The majority of respondents are female and less than 44 years of age. Over 80% of respondents have a lesion level of lumbar or lower, representing persons with spina bifida most likely to survive to adulthood.6,19 The younger, female and ‘healthier’ distribution differs from prior reports of adults with spina bifida.11,23 A population recruited from other clinic settings could yield different results.
Conclusion The primary intent of this study is to describe and better understand persons with spina bifida served in our clinic. The results of basic inferential analyses are presented to provide preliminary evidence of the need for further study. Our study found secondary conditions of spina bifida are present by early adulthood, and are perceived as impacting daily life. Pain, musculoskeletal conditions, constipation, incontinence, and skin lesions are particularly prevalent. With the exception of pain, we found secondary conditions do not significantly increase with age. This finding indicates the importance of preventative measures before the transition to adult care, and the potential impact of multidisciplinary adult spina bifida care. Future study is needed to evaluate the effectiveness of initiating secondary condition preventative interventions by pediatric providers, and the National Spina Bifida Registry31 is collecting data to support longitudinal health outcomes measurement of adult-focused multi-disciplinary spina bifida clinics. Findings from this study were translated into our clinical practice. We found that asking specific rather than general survey questions generated responses matching objective clinical assessment studies. This knowledge was translated by our clinicians, who began using specific and concrete questions to assess the presence and impact of secondary conditions during clinical encounters. We found a high
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incidence and perceived impact of neurogenic bowel and bladder secondary conditions. This knowledge was used to develop an evidence-based approach to identify and care for persons with constipation, and fecal and urinary incontinence. Eliciting an individual’s perception of health and disability allows clinicians to more effectively direct care, and empowers adults with spina bifida to better manage their own health and health care.
10.
11. 12. 13.
Acknowledgments
14.
We would like to express our appreciation to study respondents who allowed us a glimpse into their lives and to providers and staff at Gillette Lifetime Specialty Healthcare who accepted with professional grace the disruption that we brought to clinic flow. Becky Nelson, LICSW, at Gillette Lifetime Specialty Healthcare was instrumental in the design of the study and the survey. We appreciate the technical assistance of Gillette Children’s Specialty Healthcare Research Administration Department.
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17. 18.
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Supplementary data Supplementary data related to this article can be found at http://dx.doi.org/10.1016/j.dhjo.2015.03.012.
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2014. Accessed 14.11.14, http://www.hcup-us.ahrq.gov/nisoverview. jsp; 2014. Dicianno BE, Wilson R. Hospitalizations of adults with spina bifida and congenital spinal cord anomalies. Arch Phys Med Rehabil. 2010;91(4):529e535. Roach JW, Short BF, Saltzman HM. Adult consequences of spina bifida: a cohort study. Clin Orthop Relat Res. 2011;469(5):1246e1252. Webb TS. Optimizing health care for adults with spina bifida. Dev Disabil Res Rev. 2010;16(1):76e81. Heffelfinger AK, Koop JI, Fastenau PS, et al. The relationship of neuropsychological functioning to adaptation outcome in adolescents with spina bifida. J Int Neuropsychol Soc. 2008;14(5):793e804. Elliott R. Executive functions and their disorders: Imaging in clinical neuroscience. Br Med Bull. 2003;65(1):49e59. World Health Organization. Towards a Common Language for Functioning, Disability and Health (ICF) [online]. Available, http://www. who.int/entity/classifications/icf/training/; 2002. Gillette Lifetime Specialty Healthcare for Older Teens and Adults. Gillette Children’s Specialty Healthcare. Accessed 2/5, http://www. gillettechildrens.org/conditions-and-care/gillette-lifetime-specialty-heal thcare-for-older-teens-and-adults/#patientsandconditionswetreat; 2014. Why Gillette? Gillette Children’s Specialty Healthcare. Accessed 2/5, http://www.gillettechildrens.org/why-gillette/; 2014. Piatt JH. Treatment of myelomeningocele: a review of outcomes and continuing neurosurgical considerations among adults. J Neurosurg Pediatr. 2010;6(6):515e525. Oakeshott P, Hunt GM, Poulton A, Reid F. Expectation of life and unexpected death in open spina bifida: a 40-year complete, non-selective, longitudinal cohort study. Dev Med Child Neurol. 2010;52(8):749e753. Zukerman JM, Devine KA, Holmbeck GN. Adolescent predictors of emerging adulthood milestones in youth with spina bifida. J Pediatr Psychol. 2011;36(3):265e276. McDowell I. Measuring Health: A Guide to Rating Scales and Questionnaires. 3rd ed. Oxford; New York: Oxford University Press; 2006. Shavers VL, Lynch CF, Burmeister LF. Racial differences in factors that influence the willingness to participate in medical research studies. Ann Epidemiol. 2002;12(4):248e256. Werhagen L, Gabrielsson H, Westgren N, Borg K. Medical complication in adults with spina bifida. Clin Neurol Neurosurg. 2013;115(8): 1226e1229. Kinsman SL, Doehring MC. The cost of preventable conditions in adults with spina bifida. Eur J Pediatr Surg. 1996;6(S 1):17e20. Mahmood D, Dicianno B, Bellin M. Self-management, preventable conditions and assessment of care among young adults with myelomeningocele. Child Care Health Dev. 2011;37(6):861e865. Verhoef M, Barf HA, Post MW, van Asbeck FW, Gooskens RH, Prevo AJ. Secondary impairments in young adults with spina bifida. Dev Med Child Neurol. 2004;46(6):420e427. Mitra M, Wilber N, Allen D, Walker DK. Prevalence and correlates of depression as a secondary condition among adults with disabilities. Am J Orthop. 2005;75(1):76e85. Bellin MH, Dicianno BE, Osteen P, et al. Family satisfaction, pain, and quality-of-life in emerging adults with spina bifida: a longitudinal analysis. Am J Phys Med Rehabil. 2013;92(8):641e655. Ridosh M, Braun P, Roux G, Bellin M, Sawin K. Transition in young adults with spina bifida: a qualitative study. Child Care Health Dev. 2011;37(6):866e874. Ipsen C. Health, secondary conditions, and employment outcomes for adults with disabilities. J Disabil Policy Stud. 2006;17(2):77e87. About National Spina Bifida Patient Registry. NCBDDD j CDC, http:// www.cdc.gov/ncbddd/spinabifida/nsbprregistry.html; 2015. Accessed 19.02.15.
Disability and Health Journal
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Research Paper
Perception of secondary conditions in adults with spina bifida and impact on daily life Robert Wagner, M.D.a,*, Ronna Linroth, O.T., Ph.D.a, Candice Gangl, O.T.D., O.T.R/L.a, Nancy Mitchell, M.A., O.T.R/L.a, Michelle Hall, C.P.O., F.A.A.O.P.a, Rhonda Cady, Ph.D., R.N.a, and Matthew Christenson, B.S.b a
Gillette Children’s Specialty Healthcare, St. Paul, MN, USA b Professional Data Analysts, Inc., Minneapolis, MN, USA
Abstract Background: Spina bifida is a congenital defect of the neural tube resulting in motor and sensory disruption. Persons with spina bifida can also experience executive function impairments. Secondary conditions are physical, medical, cognitive, emotional, or psychosocial consequences to which persons with disabilities are more susceptible. Our experience suggested clinicians underappreciate the presence and impact of secondary conditions in adults with spina bifida because they do not specifically ask for this information. Objective: Describe the presence and impact of secondary conditions on daily life, as perceived by adults with spina bifida. Methods: A clinic-based sample was recruited from the active patient population of an adult specialty center for spina bifida-related care. All subjects were verbally administered a survey developed through literature review and clinical experience of the researchers. The survey measured the presence and perceived impact of secondary conditions. Recruitment and survey data collection occurred over a 6-month period to maximize age representation. Survey data were stratified by age, gender and lesion level for analysis. Results: Seventy-two respondents completed the survey. Pain was commonly reported, along with pressure ulcers, bowel & bladder concerns, depression, sleep disturbance, and limited social and community participation. No significant relationships were found between the presence or perceived impact of secondary conditions and age, gender or level of lesion. Conclusions: Secondary conditions in spina bifida are present by early adulthood. Identifying these conditions during clinical encounters requires specific rather than general questions. Future study should evaluate earlier initiation of preventative measures by pediatric providers. Ó 2015 Elsevier Inc. All rights reserved. Keywords: Spina bifida; Secondary conditions; Adults; Impact of disability; Patient perceptions
Spina bifida is a fault (dysraphism) in the development of the spine and spinal cord resulting in a gap or lesion in the spine.1,2 Classification is based on the level of the lesion in the spine and the presence or absence of hydrocephalus.3 In general, the higher the spinal lesion, the greater the neurologic and orthopedic impairment.3 This congenital neurodevelopmental disorder is a common disabling birth defect in the United States, and surveillance data aggregated from the National Birth Defect Prevention Network4 estimated a prevalence of 3.5 cases per 10,000 This manuscript has not been published and will not be submitted elsewhere for publication while being considered by the journal Disability Health Journal. Preliminary information from this study was shared at the second World Congress on Spina Bifida in 2012. The authors have no conflicts of interest to disclose. * Corresponding author. 435 Phalen Boulevard, St. Paul, MN 55130, USA. Tel.: þ1 651 634 1937; fax: þ1 651 265 7356. E-mail address: [email protected] (R. Wagner). 1936-6574/$ - see front matter Ó 2015 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.dhjo.2015.03.012
live U.S. births.5 Overall survival has improved and in the United States over 75% of persons with spina bifida live into early adulthood.6 With the growing number of adults with spina bifida, research into long-term health needs is warranted. The purpose of our study was to describe how adults with spina bifida perceive the presence and impact of their secondary conditions on daily life, and to use this information to better screen for secondary conditions during clinical encounters. Secondary conditions are broadly defined as, ‘‘those physical, medical, cognitive, emotional, or psychosocial consequences to which persons with disabilities are more susceptible by virtue of an underlying condition, including adverse outcomes in health, wellness, participation, and quality of life.’’7(p186) Adults with spina bifida are at risk for secondary conditions such as bowel and bladder problems, pain, fatigue, injury, depression, obesity and pressure sores or ulcers because of motor and sensory disruption at
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the level of the spinal cord lesion. The impact of secondary conditions has only recently appeared in disability literature. Secondary data analysis of the Nationwide Inpatient Sample from the 2004e05 Healthcare Cost Utilization Project9 found one-third of hospitalizations for adults with spina bifida attributed to potentially preventable secondary conditions.10 Another retrospective review identified problems associated with aging and spina bifida that included pressure ulcers and resulting amputation, scoliosis, urinary incontinence, social isolation, and limited employment.11 While evidence exists that secondary conditions have negative clinical implications, missing from literature is a description of the perceived impact of secondary conditions on a person’s daily life.3,12 Persons with spina bifida are also subject to less visible executive function impairments.8,13 Executive dysfunction is a complex phenomenon not limited to spina bifida and more easily characterized than defined. ‘‘Executive functions are those involved in complex cognitions, such as solving novel problems, modifying behavior in the light of new information, generating strategies, or sequencing complex actions.’’14(p50) Failure to attend to the impact of executive dysfunction when interviewing a person with spina bifida will limit a clinician’s ability to recognize and attend to early manifestations of problems. The International Classification of Functioning, Disability, and Health (ICF) is a World Health Organization (WHO) framework for measuring health and disability at both individual and population levels.15 Since anyone can be at risk for a change in health status that results in some manner of disability, disability is seen as a universal human experience and not something affecting only a minority of the population. The WHO ICF framework serves as a conceptual guide and justification for the current study, which emphasizes the perceived impact of secondary conditions as distinct from their clinical severity. The objective of this descriptive study was to explore two related questions: 1) how common are secondary conditions in our patients with spina bifida? and; 2) how do secondary conditions impact people’s lives? Survey data collected from consenting adults attending a specialty spina bifida clinic was analyzed to test for a relationship between age, gender and level of lesion on the perceived presence and impact of secondary conditions. The study was designed with attention to executive function challenges to obtain the most accurate information possible without clinical confirmation.
Methods Setting and respondents The study was conducted in the outpatient clinic of Gillette Lifetime Specialty Healthcare (GLSH). The research protocol was approved by the governing Institutional Review Board (IRB) and procedures followed were
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in accordance with the ethical standards of the IRB and with the Helsinki Declaration of 1975 as revised in 2000. GLSH was established in 2001 to address the specialty health needs of adolescents and adults with childhood-onset disabilities e including spina bifida e in an adult-oriented, integrated, multi-disciplinary, rehabilitation-focused environment.16 Our service is distinct from the main pediatric services provided by the parent organization, Gillette Children’s Specialty Healthcare.17 GLSH is one of nine programs serving the longitudinal needs of adults with spina bifida.18 Clients seen at GLSH come from the surrounding metropolitan area, Greater Minnesota, Iowa and Wisconsin and range from formal physician referrals to self-referrals based on ‘word of mouth’ within the disability community. Clients with spina bifida vary in degree of impairment. All our clients are adults and while survival to adulthood has improved, an association between level of spinal lesion and survival has been established.6,19 An information packet was sent to all GLSH patients, age 18 or older, with a primary diagnosis of spina bifida (n 5 192). The packet included a letter describing the study, and copies of consent and survey forms, to allow participants and when applicable, legal guardians, sufficient time to read these materials and formulate questions prior to recruitment. Recruitment was conducted inperson when 97 of the 192 adults with spina bifida presented for clinic appointments during a subsequent six month period. Each potential subject was approached for participation interest and screened for recruitment eligibility, which was the ability to understand and answer questions independently. This criterion was based on clinical judgment and patient/caregiver input. No formal assessment tool was used. Of the 97 persons approached for recruitment, five did not meet screening criteria, 20 declined participation and 72 provided written informed consent. Data collection The study used a quantitative survey to collect information about secondary conditions experienced by adults, age 18 and older, with spina bifida. The spina bifida secondary conditions (SBSC) survey tool is specific to adults with this condition. Development of the SBSC incorporated emerging adult milestone domains defined by Zukerman, Devine and Holmbeck,20 review of the current literature, and the clinical experience of our multi-disciplinary study team. We know executive function14 challenges occur in persons with spina bifida.13 During a universal screening program for foot health in our adult spina bifida clinic, we found foot lesions even when patients answered ‘‘no problems’’ to the question, ‘‘Do you have any problems?’’ Our foot screening experience suggested that general questions do not elicit accurate information and we paid particular attention to how we worded the SBSC survey questions, using simple and concrete language to minimize misunderstanding. Face validity of the SBSC was
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established by the Gillette Lifetime Consumer Advisory Group, a nine member group consisting of current Gillette Lifetime consumers with Cerebral Palsy, Spina Bifida and other disabling conditions. This group of consumer experts reviewed the SBSC and discussed whether the survey items addressed the range of secondary conditions experienced by adults like themselves. Minor changes were suggested but details of this review were lost. All suggested changes were incorporated into the final SBSC but establishment of content validity is not possible. No additional survey psychometrics or cognitive testing was conducted. The SBSC survey measured respondent perception of the presence of secondary conditions and the impact of these conditions on their lives. No clinical measurements or assessments were collected, no data was abstracted from the subject’s medical record, and no interventions were provided. The survey was verbally administered, in-person or by telephone, to all respondents over a six month period, by two of the authors (R.L., C.G). Both were trained using mock survey administration sessions with volunteers (n 5 2) matching respondent eligibility criteria. Feedback regarding consistency in administering the interview script was solicited from the volunteers, but data to support formal inter-rater reliability testing was not collected during these sessions. Periodic meetings between the surveyors during the six month data collection period ensured consistency in administering the interview script. Informed consent, survey administration, and collection of demographic information took approximately 1 h per participant. A $10 gift card was provided at the end of the survey session. Domains of the SBSC include mobility, activities of daily living, emerging adult milestones and secondary musculoskeletal, skin, gastrointestinal, urological, pain and mental health conditions (Appendix A). Respondents are asked to reflect on the past 12 months when answering survey questions. Respondents characterize mobility using defined options: transferring, wheelchair use, and ambulation. Difficulty performing activities of daily living (ADL) and the perceived impact of the difficulty is assessed for the seven domains listed in Table 2. This differs from standard ADL measurements, which focus on clinician assessment of functional status.21 Self-perception of emerging adulthood milestones20 is assessed by asking respondents’ whether having spina bifida affects their ability to reach these milestones (Table 3). The perceived presence of musculoskeletal, skin, gastrointestinal, urological, pain and mental health secondary conditions are assessed by asking if the respondent experienced the condition. Respondents answering ‘yes’ are asked to rate the impact of the condition on daily life as not at all, somewhat, or greatly (Table 4). This approach differs from standard pain measurements, which focus on pain intensity using numerical or visual scales.21 Comments from respondents that could not be quantified on the survey form are captured for later use.
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Table 1 Respondent demographics
Demographic category Gender Female Male Age 18e24 years 25e44 45e68 O 68 Level of lesion Thoracic Lumbar Sacral Missing/unknown Hydrocephalus Yes No/missing/unknown Highest level of education High school College degree Currently working Yes No Living arrangement Independently, with or without assistance In group home or with family or friends a
Respondent sample (n 5 72)
Clinic population (n 5 185)a
n
%
n
47 25
65% 35%
98 87
53% 47%
23 37 12 0
32% 51% 17%
43 108 30 4
23% 59% 16% 2%
5 57 5 5
7% 79% 7% 7%
47 25
65% 35%
54 18
75% 25%
41 31
57% 43%
23
32%
49
68%
%
p-value 0.074
0.316
Data from 2013 clinic visits.
Data analysis Age and gender of the survey respondent sample is compared to the clinic spina bifida population. The perceived presence of general (n 5 5) and specific secondary (n 5 15) conditions (Table 4) is binary coded and counted and referred to as perceived condition count. The perceived impact of the same general and specific secondary conditions is binary coded and counted for responses of ‘‘somewhat’’ or ‘‘greatly’’ affecting daily life and referred to as perceived impact count (Table 4). The secondary condition pain is binary coded and counted by pain site (n 5 7) and referred to as perceived pain site count. Perceived condition count, perceived impact count and perceived pain site count are treated as separate continuous dependent variables. Independent categorical variables include gender, age category (18e24, 25e44, 45e68) and level of myelomeningocele lesion (thoracic, lumbar, and sacral). The null hypothesis for each dependent variable states no difference between categorical groups. Records with missing data are excluded from analysis. Descriptive and inferential analysis used SPSSÒ software (IBM Corp., Armonk, NY). Chi-square tests for similarity across independent variables, by age and gender, by age and level of lesion, and by gender and level of lesion. Analysis of variance
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Table 2 Respondent perception of ADL performance and impact of perceived difficulty on daily life Perceived difficulty as Activity of daily Perceived difficulty somewhat or greatly living (ADL) performing the ADL impacting daily life Dressing Cooking Bathing Transferring Walking Stair climb/descent Propelling wheelchair
21 25 18 23 52 50 11
(29%) (35%) (25%) (32%) (73%) (69%) (15%)
16 20 14 17 35 35 8
(76%) (80%) (78%) (74%) (67%) (70%) (72%)
(ANOVA) tests for differences between age category and perceived condition count, level of lesion and perceived condition count, age category and perceived impact count, and level of lesion and perceived impact count. Tukey HSD pairwise comparisons are made if overall ANOVA is statistically significant. Differences between gender and perceived condition count, and gender and perceived impact count are analyzed using the Student t-test. Perceived pain site count is tested against independent variable groups (age, level of lesion, gender) using similar AN OVA and t-test analysis. In addition, chi-square tests for similarities across independent variable group and individual pain site. A p-value less than 0.05 is considered statistically significant and rejects the null hypothesis.
Results All 72 consented individuals completed the SBSC survey, with 95% completing in-person and the remainder completing by telephone. Of the 72 respondents, 52 have been GLSH clinic patients for two or more years. Gender and age data reported by survey respondents (not drawn from the medical record) appear in Table 1, along with gender and age data extracted from the electronic medical record for the population of persons with spina bifida Table 3 Respondent perception of the impact of spina bifida on adult milestones Adult milestones Sometimes or always feel this way Having spina bifida affects my:
n
%
Ability to participate in the community Ability to go to college Choices in having children Plans for the future Personal relationships (dating, marriage, close friendships) Ability to work (employment) Independence Options of where I can live Transportation options (car, bus)
38
53%
18 39 40 40
25% 54% 56% 56%
37 35 36 37
51% 49% 50% 51%
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Table 4 Respondent perception of presence and impact of secondary conditions within past 12 months Perceived presence Perceived condition of condition as somewhat or General and specific greatly impacting secondary conditions n % daily life Musculoskeletal and skin Burn Pressure sore/skin breakdown Lower limb edema Fracture or broken bone Muscle or tendon injury Scoliosis Limited range of Motion Gastrointestional Constipation Stool incontinence Urological Urinary tract infection Urinary incontinence Mitrofanoff Mental health Depression Sleep problems Neurosurgical Shunt malfunction or infection Pain Wrist paina Neck pain Shoulder pain Back pain Knee pain Hip pain Other area of painb
72
59
62
50
6
65
100 9% 54% 50% 10% 21% 51% 50% 82% 65% 54% 86% 58% 36% 28% 69% 53% 61% 8% 8% 90% 32% 51% 54% 71% 32% 38% 24%
29% 69% 50% 86% 87% 46% 60% 83% 90% 79% 81% 74% 87% 98% n/a
52% 70% 79% 78% 70% 63% 94%
Chi-square analysis of pain site and age; p-value ! 0.05 considered statistically significant. b Chi-square analysis of pain site and gender; p-value ! 0.05 considered statistically significant. a
receiving care at GLSH. While the age distribution of survey respondents skews somewhat younger than the clinic’s overall spina bifida population, the mean and median age of respondents (33 and 31 years) is similar to the clinic population (34 and 32 years) and chi-square indicates no significant difference between respondent and clinic population age distributions (Table 1). The gender distribution of survey respondents skews female and when compared to the clinic population, this difference approaches statistical significance (Table 1). Chi-square of independent variables indicates similarity across groups with no significant difference when compared by age and gender ( p 5 0.524), age and level of lesion ( p 5 0.213) and gender and level of lesion ( p 5 0.969). Additional demographic data as reported by respondents is listed in Table 1. Over 80% of respondents have a lesion level of lumbar or lower. Five subjects did not know their lesion level and are subsequently excluded from all analysis using this variable. The majority of respondents reported
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living at home, not married or dating and a limited number of friends. Nearly half reported having a personal care attendant and for these persons, 39% reported qualifying for 24 h care. Over half the respondents did not drink alcohol and 85% did not smoke. Respondent’s perceived difficulty performing ADLs and the perceived impact of this difficulty on daily life are shown in Table 2. The majority of respondents (86%) perceived difficulty with at least one ADL and half perceived difficulty with three or more ADLs. Difficulty walking and climbing stairs is perceived as difficult by the majority of respondents while dressing, cooking, bathing, transferring and propelling a wheelchair is perceived as difficult by less than a third of respondents. When an ADL is perceived as difficult, the majority of respondents felt the difficulty somewhat or always impacted daily life. Self-perceptions of the effects of spina bifida on adulthood milestones and life choices are shown in Table 3. With the exception college attendance, 50% or more respondents perceived spina bifida as negatively affecting their ability to achieve these milestones. Perceived presence and impact of secondary conditions Data on the perceived presence and impact of general (italic) and specific (non-italic) secondary conditions appear in Table 4. All respondents reported the presence of least one secondary condition, and 97% reported the condition impacted daily life. The median number of perceived specific secondary is six and the median number of these secondary conditions having a perceived impact on daily life is five. Neurogenic bowel, predisposing to constipation and fecal incontinence and common in spina bifida, is reported by the majority of study respondents. Over half of respondents reported at least one UTI in the previous year. All respondents reported the presence of musculoskeletal and skin secondary conditions and 90% of respondents reported the presence of pain. Over 50% of respondents replied ‘‘yes’’ to the question ‘‘Do you have problems with depression?’’ ANOVA and t-test analysis fails to reject the null hypotheses which state that perceived presence and perceived impact of secondary conditions does not change with age, gender or level of lesion. No significant differences are found between age categories and the dependent variables perceived condition count, perceived impact count and perceived pain site count, and gender and the same dependent variables. A nearly significant relationship is found between level of lesion and perceived condition count, but Tukey HSD pairwise comparisons yield non-significant p-values (thoracic vs. lumbar p 5 0.117; thoracic vs. sacral p 5 0.932; lumbar vs. sacral p 5 0.283). Only chi-square analysis between independent variable group and individual pain site rejects the null hypothesis, with. respondents in the middle age group (25e44) reporting significantly more back pain than other age groups ( p 5 0.045), and females reporting significantly more ‘other pain’ than males ( p 5 0.020).
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Discussion This study explored the perceived presence and impact of secondary conditions on adults with spina bifida using patient-reported survey data. We hypothesized that secondary conditions are more prevalent in older than younger individuals based on increasing vulnerability with age. While secondary conditions are common in our study population, the perceived presence or impact did not increase significantly with age or vary significantly by gender or level of lesion. These findings suggest that young adults with spina bifida enter the adult care system with secondary conditions already present. Earlier initiation of secondary condition preventative measures by pediatric providers is warranted and future study of the impact of preventative interventions is needed. Our findings also suggest a competing explanation. Prior study of factors influencing medical research participation found Caucasian women under the age of 65 more likely to participate in research.22 Although the age distribution of our respondent sample matches previous prospective study of adults with spina bifida,11 the age and gender distribution of our respondent sample also matches medical research participation patterns,22 and could influence our findings. In addition, all respondents were recruited from a multi-disciplinary spina bifida specialty clinic and over 70% were clinic patients for two or more years. The majority of our younger respondent sample (18e68 years) benefited from advancements in pre-natal and post-natal screening and care.6,8 Over 80% have a lesion level of lumbar or lower and are the population most likely to survive to adulthood.6,19 Our findings could reflect the overall influence of these factors on the health of our respondents. To fully understand the relationship between age, gender, level of lesion and secondary conditions, prospective longitudinal outcomes research is needed. To our knowledge, this is the first report of perceived secondary conditions, from a sample that includes only adults with spina bifida. Our study design differs from other secondary conditions research, which used clinical data, historical medical record or claims data.11,23 Although our study relied on patient-reported data, the perceived presence and impact of specific secondary conditions match previous reports from the literature. Respondent report of stool incontinence (54%) match findings from a longitudinal study6 and report of urinary tract infection (58%) align with findings from retrospective review of clinical records.23 Musculoskeletal, gastrointestinal and urological secondary conditions are reported by more than twothirds of respondents as somewhat or greatly impacting their daily lives. These findings mirror retrospective reviews of national datasets and hospital records on the incidence of secondary conditions and hospitalization frequency10,11,24 and the association between urinary tract infections, pressure sores and health care usage.25 The specificity of our findings to objective clinical studies could
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be explained by our attention to executive dysfunction, and asking specific rather than general survey questions. It is common in clinical practice to ask a patient, ‘‘Do you have pain?’’ If the answer is ‘‘yes’’, we ask the location of the pain and the severity on a scale of 1e10. In our study, questions about pain are asked in the context of specific joints or body regions. Almost all respondents reported pain in at least one site and the majority perceived the pain as impacting their daily lives. These findings differ from prospective clinical assessment studies, where 28% of subjects report any type of pain,23 and 16% of subjects report back pain and 10.6% report neck pain.26 While the prospective study did not specify how the question of pain was asked, our findings suggest that drawing attention to a specific area of the body before asking about pain and the impact of pain on daily life may be a more sensitive approach to eliciting reports of pain in individuals with spina bifida. An association between disability and depression has been established27 and identifying mental health secondary conditions is a priority for persons with spina bifida. While our finding of perceived problems with depression approximates those from a study of young adults with spina bifida who completed the self-report Hopkins Symptom Checklist,28 it is far greater than the 5% reported in a prospective clinical assessment study.23 The high percentage of positive responses in our study compared to the clinical assessment study suggests that perceived depression may be more prevalent in the spina bifida population and warrants more focused investigation. Adolescents and young adults strive to achieve milestones of reduced parental dependency and increased selfmanagement activities.29 The majority of our respondents self-reported limited work and social opportunities. Surveyors observed in many respondents e and in themselves e a sense of sadness when they asked about the number of friends. Comments from respondents indicated that those with computer access or a cell phone had greater connectedness and social participation than those without these resources. These findings are consistent with established research on achieving adult milestones.20 An examination of transition issues of young adults with spina bifida labeled ‘‘struggling for independence, limited social interactions and stigma and building inner strength’’ 29 (p869) as key themes, while a national survey found the presence of secondary conditions decreased the likelihood of employment for adults with disability.30 Respondents selfreported varying levels of participation in activities of daily living based on the availability of assistance and personal preference relative to how they prioritize expenditure of effort, time, and energy consumption. Early identification and corresponding interventions that reduce the impact of secondary conditions could facilitate achievement of adult milestones in persons with spina bifida. Future studies should address this issue.
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Limitations We acknowledge inherent limitations in our study design that limit the ability to generalize results to the large population of adults with spina bifida. A survey instrument was developed specifically for this study because no validated instrument was available. While content validity was assessed by our Consumer Advisory Group, the inability to report these findings limits validity of the SBSC. Other properties that could influence results but were not quantified (e.g. cognitive ability, socioeconomic status) are unknown. The secondary conditions surveyed by the SBSC are specific to spina bifida and do not include secondary conditions related to aging or lifestyle, such as obesity, diabetes, hypertension and asthma. Including these conditions could influence the results of this study. Sampling is limited by availability of respondents presenting during scheduled clinic appointments at GLSH multi-disciplinary spina bifida clinic and meeting screening criteria. The majority of respondents are female and less than 44 years of age. Over 80% of respondents have a lesion level of lumbar or lower, representing persons with spina bifida most likely to survive to adulthood.6,19 The younger, female and ‘healthier’ distribution differs from prior reports of adults with spina bifida.11,23 A population recruited from other clinic settings could yield different results.
Conclusion The primary intent of this study is to describe and better understand persons with spina bifida served in our clinic. The results of basic inferential analyses are presented to provide preliminary evidence of the need for further study. Our study found secondary conditions of spina bifida are present by early adulthood, and are perceived as impacting daily life. Pain, musculoskeletal conditions, constipation, incontinence, and skin lesions are particularly prevalent. With the exception of pain, we found secondary conditions do not significantly increase with age. This finding indicates the importance of preventative measures before the transition to adult care, and the potential impact of multidisciplinary adult spina bifida care. Future study is needed to evaluate the effectiveness of initiating secondary condition preventative interventions by pediatric providers, and the National Spina Bifida Registry31 is collecting data to support longitudinal health outcomes measurement of adult-focused multi-disciplinary spina bifida clinics. Findings from this study were translated into our clinical practice. We found that asking specific rather than general survey questions generated responses matching objective clinical assessment studies. This knowledge was translated by our clinicians, who began using specific and concrete questions to assess the presence and impact of secondary conditions during clinical encounters. We found a high
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incidence and perceived impact of neurogenic bowel and bladder secondary conditions. This knowledge was used to develop an evidence-based approach to identify and care for persons with constipation, and fecal and urinary incontinence. Eliciting an individual’s perception of health and disability allows clinicians to more effectively direct care, and empowers adults with spina bifida to better manage their own health and health care.
10.
11. 12. 13.
Acknowledgments
14.
We would like to express our appreciation to study respondents who allowed us a glimpse into their lives and to providers and staff at Gillette Lifetime Specialty Healthcare who accepted with professional grace the disruption that we brought to clinic flow. Becky Nelson, LICSW, at Gillette Lifetime Specialty Healthcare was instrumental in the design of the study and the survey. We appreciate the technical assistance of Gillette Children’s Specialty Healthcare Research Administration Department.
15.
16.
17. 18.
19.
Supplementary data Supplementary data related to this article can be found at http://dx.doi.org/10.1016/j.dhjo.2015.03.012.
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