- Email: [email protected]
Survey of U.S. adults with Spina Bifida
Disability and Health Journal xxx (xxxx) xxx
Contents lists available at ScienceDirect
Disability and Health Journal journal homepage: www.disabilityandhealthjnl.com
Survey of U.S. adults with Spina Bifida Christopher P. Morley a, b, c, *, Sara Struwe d, Morgan A. Pratte e, Gerald H. Clayton f, Pamela E. Wilson f, Brad E. Dicianno g, Margaret K. Formica a, h, Laura A. Schad a, Judy Thibadeau d, Margaret A. Turk i, a a
Department of Public Health & Preventive Medicine, Upstate Medical University, 750 E Adams St, Syracuse, NY, USA Department of Family Medicine, Upstate Medical University, Syracuse, NY, USA c Department of Psychiatry & Behavioral Sciences, Upstate Medical University, Syracuse, NY, USA d Spina Bifida Association, 1600 Wilson Blvd, Suite 800, Arlington, VA, 22209, USA e Hospital Administration, Upstate Medical University, 750 E Adams St, Syracuse, NY, USA f Department of Physical Medicine & Rehabilitation, University of Colorado Denver, USA g Department of Physical Medicine & Rehabilitation, University of Pittsburgh School of Medicine School of Medicine, Kaufmann Medical Building, Suite 901, 3471 Fifth Avenue, Pittsburgh, PA, 15213, USA h Department of Urology, Upstate Medical University, 750 E Adams St, Syracuse, NY, USA i Department of Physical Medicine & Rehabilitation, Upstate Medical University, Syracuse, NY, USA b
a r t i c l e i n f o
a b s t r a c t
Article history: Received 16 April 2019 Received in revised form 12 July 2019 Accepted 26 July 2019
Background: Spina Bifida (SB) is one of the most common birth defects and causes of permanent disability in the United States (US), with approximately 3.5 cases per 10,000 live births. Objective: To identify complications associated with SB related to skin breakdown, pain, and urinary tract infections (UTIs), and to examine socio-demographic differences related to these complications. Methods: Exploratory cross-sectional study via online of a national US convenience sample of adults with SB. Results: We collected 1485 survey responses, of which 852 had complete, useable data. Skin breakdown in one or more locations during the past year was reported by 43.1%. After controlling for sociodemographic characteristics, only mobility variables remained significant predictors of skin breakdown (assistive device use OR ¼ 3.119, 95% CI: 1.749, 5.564; using a wheelchair OR ¼ 6.336, 95% CI: 3.442, 11.662). Pain in past seven days was reported by 46.9%. Single respondents (OR ¼ 0.621; 95% CI: 0.419, 0.921) and those with at least a Bachelor's degree (vs high school degree or less, OR ¼ 0.468; 95% CI: 0.283, 0.774) were less likely, and those using assistive devices were significantly more likely (OR ¼ 1.960; 95% CI: 1.163, 3.303), to report pain. About one-third (32.7%) reported having a UTI within the past 12 months. Notably, almost half (49.6%) of respondents did not answer this question. The presence of UTIs was not significantly related to any socio-demographic characteristics assessed. Conclusions: Adults with SB in the US live with a wide range of complications which are potentially undermonitored, with predictors of complications that require further research. © 2019 Published by Elsevier Inc.
Keywords: Spina bifida Disability Surveys Cross-sectional Epidemiology
Introduction Spina Bifida (SB) is one of the most common birth defects and
* Corresponding author. Department of Public Health & Preventive Medicine, Upstate Medical University, 750 E Adams St, WSK, 2262, Syracuse, NY, USA. E-mail addresses: [email protected] (C.P. Morley), [email protected] (S. Struwe), [email protected] (M.A. Pratte), Gerald.Clayton@ childrenscolorado.org (G.H. Clayton), [email protected] (P.E. Wilson), [email protected] (B.E. Dicianno), [email protected] (M.K. Formica), [email protected] (L.A. Schad), [email protected] (J. Thibadeau), [email protected] (M.A. Turk).
causes of permanent disability among children and adolescents in the United States, with a rate of approximately 3.5 cases per 10,000 live births.1 While strides have been made over the past couple decades in reducing the overall prevalence of SB, there has been limited research and intervention aimed at addressing healthcare across the lifespan for those with condition. A growing number of individuals with SB are transitioning to adulthood, resulting in a population with increased medical needs due to the combination of secondary conditions associated with SB and chronic conditions experienced by the general aging population.2 Existing SB surveillance efforts often focus on pediatric
https://doi.org/10.1016/j.dhjo.2019.100833 1936-6574/© 2019 Published by Elsevier Inc.
Please cite this article as: Morley CP et al., Survey of U.S. adults with Spina Bifida, Disability and Health Journal, https://doi.org/10.1016/ j.dhjo.2019.100833
2
C.P. Morley et al. / Disability and Health Journal xxx (xxxx) xxx
populations, and there is currently limited health information about adults who are aging with this condition. Assessments of costs associated with preventable conditions in adults with SB have existed for decades,3 and small studies using localized surveys or existing data have documented the presence of pain, secondary complications,4 and restrictions on social participation5 related as a result of SB.6e10 On a broader scale, in the United States (US), the National SB Patient Registry (NSBPR) was developed in 2008 through collaboration between the Spina Bifida Association and the Centers for Disease Control and Prevention to gain better understanding of patient care in Spina Bifida clinics across the United States. Analyses from the NSBPR have identified common medical procedures, bladder management methods, mobility levels, and other health issues related to SB among patients involved in the cohort.11e13 However, only a few of the clinics participating in the NSBPR provide services to patients beyond adolescence, resulting in low representation of adult experiences.14 While the NSBPR is a valuable resource for examining clinical outcomes among patients with SB, this registry does not account for individuals who receive care outside of specialty clinics or those who do not have access to care at all. Therefore, we currently lack a more nationally representative data source that can be used to identify health status issues within the general adult SB population. In order to expand upon the body of knowledge regarding the adult experience of SB, beyond what is captured in smaller studies or in the NSBPR, researchers from two universities collaborated with the Spina Bifida Association to develop and conduct a nationwide survey of adults with SB. The primary objective of this report is to describe our survey methodology, as well as to identify medical complications associated with SB related to skin breakdown, pain, and urinary tract infections (UTIs), and to examine sociodemographic differences related to these complications. We also make note of shunt presence and complications in the survey population. Methods Study design & survey development This was an exploratory cross-sectional study that implemented an online survey to gather information from a national convenience sample of adults with SB. Survey items were selected and suggested by a panel of researchers, including two physiatrists, a neuroscientist, an epidemiologist, a medical social scientist with expertise in survey design and special populations, and other public health professionals. The included items were written de novo, as well as drawn from items included in the NSBPR. The survey was comprised of 44 questions overall, and was organized into four
primary domains: 1) Demographics: informed consent to participate in the survey; date of birth; ZIP code; gender; ethnicity; race; marital status; education level; employment status; and income sources. 2) Healthcare Access: insurance; usual source of care; and healthcare facilities utilized for different medical situations including routine care, SB-related issues, and emergencies. 3) Health conditions (SB-related and general): functional motor level; ambulation status; skin breakdown; leg fractures and amputations; pain and its interference with daily activities and mood; UTIs; bladder and bowel management; shunt status, function, and infections; general medical conditions (e.g. hypertension, obesity, asthma, sleep problems, etc.); and mental health. 4) Permission to re-contact for future surveys. An outline of survey questions is provided in Table 1, and a copy of the survey is available upon request. Following survey item development, an online instrument was created using SurveyMonkey® software. The online instrument was tested among 10 adults with SB for suitability, function, question clarity, format, and length of the survey. Amendments to the survey were incorporated accordingly, following the pilot testing period. This study was reviewed and approved by the Institutional Review Board at the lead university on the study.
Sample & data collection The target population consisted of adults (18 years and older) with a diagnosis of SB. The survey was launched at the 2016 Spina Bifida Association National Conference in Bloomington, MN to an audience of approximately 700 people. They were asked to share the information with their own networks electronically and via word of mouth. The online survey link was disseminated by electronic notification to contact lists by staff from the Spina Bifida Association and partnering organizations via email and social media. Detailed explanation of the intent was included with the electronic notifications. It included information about the purpose of the survey, our intent to use the information to guide future directions of services and to assist in explaining the adult experience with SB. Dissemination strategies included: - Sending information to over 40,000 contacts in the US Spina Bifida Association database - Sending information to 130 SB Clinics in the US that were known to the SBA in 2016
Table 1 Description of survey items. Question Numbers
Content
Description
1 2e14
Consent language, with yes/no option Name, date of birth, gender, race, ethnicity, marital status, education level, employment status
15e17 18e22 23e28
Consent to participate Contact & Demographic information Income and Insurance Sources of Medical Care Medical Issues
29e32 33e37 38e39 40 41e44
Pain Bladder and Bowel Shunts Depression & Anxiety Permission to Re-contact
a
Sources of income, type and source of health insurance coverage Source of routine/primary care (if none, reason), sources of Spina Bifida-related care, sources of emergency care Ambulation status, functional level of lesion, general comorbid medical issues, skin breakdown, leg fractures, amputations Any chronic pain (last 7 days), pain interference with day-to-day activities and mood, location of pain UTIa (past 12 months), bladder & bowel emptying, bladder or bowel accidents Presence of shunt, lifetime history of shunt infection PHQ-4 Permission to re-contact, preferred methods of re-contact
UTI ¼ urinary tract infection.
Please cite this article as: Morley CP et al., Survey of U.S. adults with Spina Bifida, Disability and Health Journal, https://doi.org/10.1016/ j.dhjo.2019.100833
C.P. Morley et al. / Disability and Health Journal xxx (xxxx) xxx
- Sending out information through 17 Spina Bifida Association Chapters - Standing links on web and social media pages (SBA had over 36,000 followers on its pages in 2016) - Sending information to the United Spinal Association Although it is not possible to know how many individuals ultimately saw a link or direct invitation to participate in the survey, we estimate that approximately 50,000 individuals were reached through these dissemination efforts. Participation was voluntary and respondents were permitted to decline answering any questions that they wished to disregard. The data collection period lasted for approximately 6 months in duration, from June 2016 to December 2016. Analysis In order to verify that all respondents were at least 18 years of age, cases with missing information for date of birth were removed from the sample. Additionally, duplicate cases, in which the same individual submitted multiple responses to the survey, were identified by matching name, date of birth, and/or IP address. Upon identification of duplicate cases, the first response submitted was retained and subsequent responses were discarded. Subjects with extensive missing data were excluded from analyses. Several variables were re-categorized in preparation for the analyses described below. Education classifications were combined to create three main categories: 1) high school or less (less than a high school diploma, high school diploma or equivalent, or technical school/trade certificate); 2) some college (some college education or Associate's degree); and 3) Bachelor's degree or greater (Bachelor's degree, Master's degree, or Doctoral/Professional degree). Two primary marital status categories were determined: 1) single; and 2) partner or ever married (partner, married, separated/ divorced, or widowed). Employment status was also classified into two categories: 1) employed (employed full/part-time or work for family or own business); and 2) not employed (not employed, volunteer, or occasional worker). Three categories were utilized for insurance status: 1) no insurance; 2) public insurance (i.e. Medicaid, Medicare); and 3) private insurance. The ambulation status variable was originally comprised of four categories based on the classifications defined by Hoffer et al.,15 which include community ambulation (able to walk in the community, may require use of assistive devices or wheelchair for long trips), household ambulation (walk only indoors with assistive devices, may use wheelchair for some indoor activities) therapeutic ambulation (walk only in therapy sessions, otherwise use wheelchair), and wheelchair use (exclusively use wheelchair for all mobility). Additionally, an “unknown/unsure (describe as needed)” option was available for respondents who felt they did not belong in one of the four given categories. Upon preliminary analysis of the mobility variable, it was evident that a substantial proportion of respondents selected “unknown/unsure” as their mobility status and the majority of respondents who selected this option had provided commentary indicating that they walk without the use of any assistive devices. Therefore, a fifth category was created to represent the mobility status of these individuals, which had not been captured in the original four Hoffer categories. Furthermore, the Hoffer categories were condensed by combining community with household ambulation to create a “community þ household” category, and therapeutic ambulation with wheelchair use to create a “therapeutic þ wheelchair” category. This re-classification resulted in three primary mobility levels: 1) walk without assistive devices; 2) community þ household; and 3) therapeutic þ wheelchair. In order to characterize the sample, a descriptive analysis was
3
conducted to provide an overview of socio-demographic information and medical complications associated with SB among the study sample. Medical complications that were assessed included skin breakdown (yes/no), pain (yes/no), UTIs (yes/no), and shunt status (yes/no). Respondents that reported experiencing pain were then asked to rate how much pain interferes with their day-to-day activities and overall mood using a 10-point scale (1 ¼ no interference and 10 ¼ complete interference). Frequencies and percentages for skin breakdown and pain by location were also determined. Associations between socio-demographic characteristics and skin breakdown, pain, UTIs, and shunt status were then examined by conducting c2 and t-tests, as appropriate. Multivariable logistic and linear regression models were also used to adjust for the following variables: age (continuous), gender (male or female), ethnicity (Hispanic or non-Hispanic), race (White or non-White), marital status (single or partner/ever married), education (high school or less, some college, or Bachelor's degree or greater), employment (employed or not employed), insurance coverage (no insurance, public insurance, or private insurance), and mobility: 1) walk without assistive devices; 2) community þ household; and 3) therapeutic þ wheelchair. Descriptive statistics, c,2 and t-tests were conducted using SPSS Version 23, and regression models were tested using SAS Version 9.3. A distribution map was also created using UDS Mapper to illustrate the number of respondents per state in the United States. Results A total of 1485 survey responses were collected, of which 1445 individuals provided consent to participate in the study. A total of 373 cases were removed from the sample due to missing date of birth information and 174 cases were removed due to duplicate responses. Only 3 respondents were under the age of 18 and therefore eliminated from the sample. Finally, 43 respondents who indicated non-U.S. residence were also removed from the sample. Upon elimination of invalid responses, 852 cases remained for inclusion in the analysis sample. Respondent gender, race, ethnicity, and age are presented in Table 2. Nearly three-quarters of the sample were female (73.6%) and about one-quarter were male (25.9%), while the remaining respondents selected “other” as their gender or did not respond to the question. The vast majority of respondents (83.7%) identified as being White only, while only 4.0% identified with multiple races and 3.8% identified as being Black or African American only. About 8.6% of the sample indicated that they were of Hispanic or Latino ethnicity. Over one-third of the sample was between ages 25e34 years (35.1%), followed by 28.5% between the ages 35e44 years. Overall, the mean age of respondents was 37 years (SD ¼ 11), with an age range from 18 to 87 years. Fig. 1 illustrates the distribution of respondents by state within the United States, demonstrating a high concentration of respondents from some of the more populous states of the Northeast and Southeast regions, in addition to California and Texas. This distribution corresponds to the current United States population estimates by state, suggesting that in general, greater numbers of respondents were observed from states with greater population estimates. Table 3 presents respondent education level, marital status, employment status, and insurance status. When asked to indicate current or most recent education level, the greatest percentage of respondents reported having some college education (40.2%) followed by having a Bachelor's degree or greater (31.5%). In regard to marital status, the majority (57.9%) of respondents indicated that they were single. When asked about employment status, 51.7% reported that they were not employed. The majority of respondents
Please cite this article as: Morley CP et al., Survey of U.S. adults with Spina Bifida, Disability and Health Journal, https://doi.org/10.1016/ j.dhjo.2019.100833
4
C.P. Morley et al. / Disability and Health Journal xxx (xxxx) xxx Table 2 Respondent gender, race, ethnicity, and age. Characteristic Gender Female Male Other Missing Race White Black or African American Asian American Indian/Alaska Native Native Hawaiian/Pacific Islander Multiple Races Other Unsure Missing Ethnicity Hispanic or Latino Not Hispanic or Latino Unsure Prefer not to answer Missing Age 18e24 yrs. 25e34 yrs. 35e44 yrs. 45e54 yrs. 55e64 yrs. 65 yrs and older
N (%)
Table 3 Respondent education level, marital status, employment status, and insurance status. Characteristic
627 (73.6) 221 (25.9) 1 (0.1) 3 (0.4) 713 (83.7) 32 (3.8) 15 (1.8) 4 (0.5) 0 (0.0) 34 (4.0) 28 (3.3) 23 (2.7) 3 (0.4) 73 (8.6) 718 (84.3) 10 (1.2) 41 (4.8) 10 (1.2) 107 (12.6) 299 (35.1) 243 (28.5) 141 (16.5) 48 (5.6) 14 (1.6)
(54.9%) reported having health insurance coverage through some type of public insurance (i.e. Medicaid, Medicare) and 31.1% reported being enrolled in private health insurance. Results for both the original and condensed categories for ambulation status are provided in Table 4. After combining the original categories as previously described, 39.9% of individuals
Education level High school or less Some college Bachelor's degree or greater Missing Marital status Single Partner or ever married Other Prefer not to answer Missing Employment status Employed Not employed Missing Insurance status No insurance Public insurance (Medicaid/Medicare) Private insurance Unsure Missing
N (%) 231 (27.0) 343 (40.2) 268 (31.5) 10 (1.2) 493 (57.9) 323 (37.9) 18 (2.1) 17 (2.0) 1 (0.1) 359 (42.1) 441 (51.7) 52 (6.1) 27 (3.2) 468 (54.9) 265 (31.1) 35 (4.1) 57 (6.7)
walk in the home or community and may use assistive devices (e.g., crutches, braces), another 35.8% walk only therapeutically or primarily use a wheelchair, and 13.3% do not use assistive devices. The remaining respondents were either unsure of their mobility status or did not respond to this question on the survey. Overall, 43.1% of the sample reported experiencing skin breakdown in one or more locations during the past year (Table 5). Among those with skin breakdown, the most frequently reported location was the foot (55.3%), followed by the buttocks (39.0%) and the lower body region (27.0%), which was described as the thigh, hip, knee, shin or ankle. The presence of skin breakdown was
Fig. 1. - Respondent distribution map, United States. Note: Alaska and Hawaii not to scale.
Please cite this article as: Morley CP et al., Survey of U.S. adults with Spina Bifida, Disability and Health Journal, https://doi.org/10.1016/ j.dhjo.2019.100833
C.P. Morley et al. / Disability and Health Journal xxx (xxxx) xxx
5
Table 4 Respondent mobility level. Original Categories
N (%)
Condensed Categories
N (%)
Walk without assistive devices Community ambulation Household ambulation Therapeutic ambulation Wheelchair use Unsure Missing
113 (13.3) 272 (31.9) 68 (8.0) 13 (1.5) 292 (34.3) 20 (2.3) 74 (8.7)
Walk without assistive devices [Community þ Household]
113 (13.3) 340 (39.9)
[Therapeutic þ Wheelchair]
305 (35.8)
Unsure Missing
20 (2.3) 74 (8.7)
Table 5 Medical conditions associated with Spina Bifida. Condition Skin breakdown Yes No Unknown Missing Skin breakdown by locationa Head/neck Upper body Lower body (thigh, hip, knee, shin, ankle) Trunk (back, chest) Lower back Buttocks Genitals Foot Other Chronic pain related to Spina Bifida Yes No Unknown Missing Pain by locationa Head/neck Upper body Lower body (thigh, hip, knee, shin, ankle) Trunk (back, chest) Lower back Genitals Foot Other Pain interference (mean ± SD) Day-to-day activities Overall mood UTIb Yes No Unknown Missing Shunt Yes No Unknown Missing
N (%) 367 (43.1) 411 (48.2) 7 (0.8) 67 (7.9) 6 (1.6) 9 (2.5) 99 (27.0) 12 (3.3) 25 (6.8) 143 (39.0) 31 (8.4) 203 (55.3) 22 (6.0) 400 (46.9) 344 (40.4) 42 (4.9) 66 (7.7) 186 (46.5) 159 (39.8) 264 (66.0) 130 (32.5) 315 (78.8) 15 (3.8) 123 (30.8) 24 (6.0) 5.35 ± 2.39 5.56 ± 2.42 279 (32.7) 133 (15.6) 17 (2.0) 423 (49.6) 458 (53.7) 304 (35.7) 3 (0.4) 87 (10.2)
a Among respondents with the condition; respondents able to select more than one option; percentages do not sum to 100%. b UTI ¼ urinary tract infection.
significantly associated with employment status, in which 46.7% of respondents who are not employed reported having skin breakdown compared to only 37.6% of those who are employed (p ¼ 0.010). Ambulation status was also significantly associated with skin breakdown; those who do not use assistive devices to ambulate were least likely to experience skin breakdown at 20.4% compared to those who may walk with assistive devices at 42.6% and those who were wheelchair users at 62.0% (p < 0.001). After controlling for all socio-demographic characteristics in the multivariable logistic regression model, only the association with mobility remained significant; those who may walk with assistive devices had 3.119 (95% CI: 1.749, 5.564) times the odds of skin
breakdown compared to those who do not use assistive devices, and those who primarily use a wheelchair had 6.336 (95% CI: 3.442, 11.662) times the odds of skin breakdown compared to those who do not use assistive devices, as shown in Table 6. Table 5 presents that 46.9% of all respondents had experienced pain in the past seven days. Among those, the most common locations where pain was experienced included the lower back (78.8%) and lower body (66.0%) regions. The average pain interference rating for day-to-day activities was 5.35 (SD ¼ 2.39) and for overall mood was 5.56 (SD ¼ 2.42). The presence of pain was significantly associated with gender (female: 56.4% vs. male: 46.1%; p ¼ 0.014), marital status (single: 48.0% vs. partner/ever married: 63.2%; p < 0.001), education level (high school or less: 61.6% vs. some college: 55.2% vs. Bachelor's degree or greater: 46.7%; p ¼ 0.007), and mobility (no use of assistive devices: 51.0% vs. may walk with assistive devices: 63.5% vs. wheelchair use: 42.5%; p < 0.001). Some of these associations remained significant when controlling for other variables in a logistic regression model, as presented in Table 6. Respondents who were single were significantly less likely to report pain than those who have a partner or have ever been married (OR ¼ 0.621; 95% CI: 0.419, 0.921), those with at least a Bachelor's degree were less likely to report pain than those with a high school degree or less (OR ¼ 0.468; 95% CI: 0.283, 0.774), and those who may use assistive devices were significantly more likely to report pain than those who do not use assistive devices (OR ¼ 1.960; 95% CI: 1.163, 3.303). When comparing mean ratings of pain interference with day-to-day activities and overall mood, there were significant differences by insurance status for both day-to-day activities (no insurance: m ¼ 6.93 vs. public insurance: m ¼ 5.43 vs. private insurance: m ¼ 5.11; p ¼ 0.016) and mood (no insurance: m ¼ 7.29 vs. public insurance: m ¼ 5.49 vs. private insurance: m ¼ 5.57; p ¼ 0.022). Furthermore, marital status was related to pain interference with day-to-day activities (single: m ¼ 5.09 vs. partner/ever married: m ¼ 5.69; p ¼ 0.009), as were education level (high school or less: m ¼ 5.55 vs. some college: m ¼ 5.55 vs. Bachelor's degree or greater: m ¼ 4.80; p ¼ 0.012) and employment status (not employed: m ¼ 5.66 vs. employed: m ¼ 4.80; p < 0.001). However, these factors were not related to pain interference with mood. Table 7 presents the adjusted associations resulting from multivariable linear regression models for the two pain interference outcome measures, showing that ethnicity, race, marital status, and employment status are all statistically significantly related to pain interference with day-to-day activities in multivariable models at the p ¼ 0.05 level, while only insurance status is significantly associated with both pain interference and overall mood. About one-third (32.7%) of respondents reported that they have had a UTI within the past 12 months. Notably, almost half (49.6%) of respondents did not answer this question. The presence of UTIs was not found to be significantly related to any of the sociodemographic characteristics assessed in the analyses. Over half (53.7%) of respondents indicated having a shunt. The presence of a shunt was determined to be significantly associated with gender (female: 57.2% vs. male: 68.5%; p ¼ 0.005), age group
Please cite this article as: Morley CP et al., Survey of U.S. adults with Spina Bifida, Disability and Health Journal, https://doi.org/10.1016/ j.dhjo.2019.100833
6
C.P. Morley et al. / Disability and Health Journal xxx (xxxx) xxx
Table 6 Logistic regression odds ratios of breakdown, pain, and UTIsb by socio-demographic characteristics. Characteristic
Skin Breakdown (n ¼ 588) OR (95% CI)
Age (Continuous) 1.000 (0.983, 1.018) Female (vs. Male) 1.445 (0.971, 2.151) Hispanic or Latino (vs. Not) 1.015 (0.516, 1.996) Non-White (vs. White) 0.876 (0.525, 1.462) Single (vs. Ever Married/Partner) 0.910 (0.617, 1.344) Education (vs. High School) Some college 0.827 (0.532, 1.284) Bachelor's or greater 0.871 (0.536, 1.416) Employed (vs. Not Employed) 0.723 (0.478 1.095) Insurance (vs. Private Insurance) No insurance 1.525 (0.519, 4.482) Public insurance 0.926 (0.607, 1.411) Ambulation status (vs. walk without assistive devices) [Community þ Household]; 3.119** (1.749, 5.564) [Therapeutic þ Wheelchair] 6.336** (3.442, 11.662) a b
Pain (n ¼ 559) OR (95% CI)
UTI (n ¼ 311) OR (95% CI)
Shunt (n ¼ 572) OR (95% CI)
1.002 (0.985, 1.021) 1.193 (0.798, 1.783) 0.879 (0.452, 1.709) 0.908 (0.543, 1.518) 0.621a (0.419, 0.921)
0.991 1.648 0.818 1.222 0.942
0.944** (0.925, 0.963) 0.688 (0.437, 1.083) 1.409 (0.649, 3.058) 0.712 (0.403, 1.258) 1.517a (1.004, 2.293)
0.736 (0.467, 1.163) 0.468** (0.283, 0.774) 0.890 (0.582, 1.361)
1.069 (0.577, 1.981) 1.491 (0.716, 3.103) 0.823 (0.440, 1.540)
1.205 (0.732, 1.983) 1.488 (0.861, 2.572) 0.946 (0.593, 1.509)
0.879 (0.296, 2.605) 0.775 (0.507, 1.183)
1.624 (0.303, 8.697) 1.604 (0.874, 2.941)
0.836 (0.279, 2.506) 2.138** (1.355, 3.371)
1.960a (1.163, 3.303) 0.888 (0.513, 1.536)
1.031 (0.501, 2.120) 1.402 (0.619, 3.179)
1.829a (1.072, 3.122) 5.316** (2.921, 9.675)
(0.966, (0.924, (0.275, (0.532, (0.536,
1.017) 2.939) 2.437) 2.807) 1.654)
p 0.05; **p 0.01. UTIs ¼ urinary tract infections.
Table 7 Linear regression parameter estimates of pain interference by socio-demographic characteristics. Characteristic
Pain Interference with Daily Activities (n ¼ 328) b (p-value)
Pain Interference with Mood (n ¼ 328) b (p-value)
Age (Continuous) Female (vs. Male) Hispanic or Latino (vs. Not) Non-White (vs. White) Single (vs. Ever Married/Partner) Education (vs. High School or less) Some college Bachelor's or greater Employed (vs. Not Employed) Insurance (vs. Private Insurance) No insurance Public insurance Ambulation status (vs. walk without assistive devices) [Community þ Household]; [Therapeutic þ Wheelchair]
0.024 (0.072) 0.346 (0.248) 1.387 (0.010) 1.089 (0.007) 0.778 (0.007)
0.016 (0.255) 0.528 (0.095) 0.423 (0.456) 0.408 (0.334) 0.422 (0.161)
0.105 (0.735) 0.638 (0.079) 0.883 (0.005)
0.076 (0.817) 0.616 (0.108) 0.348 (0.295)
1.743 (0.020) 0.189 (0.542)
1.855 (0.019) 0.309 (0.343)
0.358 (0.340) 0.180 (0.666)
0.737 (0.063) 0.180 (0.683)
(18e24 yrs: 64.8%, 25e34 yrs: 70.7%, 35e44 yrs: 63.3%, 45e54 yrs: 52.3%, 55 yrs: 11.7%; p 0.001), marital status (single: 69.7% vs. partner/ever married: 44.8%; p 0.001), employment (not employed: 66.5% vs. employed: 54.7%; p ¼ 0.001), mobility (walk without assistive devices: 38.3% vs. may walk with assistive devices: 50.5% vs. wheelchair use: 79.5%; p 0.001), and insurance status (no insurance: 34.8% vs. public insurance: 68.6% vs. private insurance: 45.3%; p 0.001). Several associations remained significant after controlling for all socio-demographic characteristics in the multivariable logistic regression model; those who were older in age were less likely to report having a shunt (OR: 0.944; 95% CI: 0.925, 0.963), those who were single had 1.517 (95% CI: 1.004, 2.293) times the odds of having a shunt compared to those who have a partner or have ever been married, and those with public insurance had 2.138 (95% CI: 1.355, 3.371) times the odds of having a shunt than those with private insurance. In regards to mobility status, those who may use assistive devices to walk had 1.829 (95% CI: 1.072, 3.122) times the odds of having a shunt and those who primarily use a wheelchair had 5.316 (95% CI: 2.921, 9.675) times the odds of having a shunt compared to those who are able to walk without any assistive devices. Discussion This unique study reports and evaluates the experiences of a relatively large sample of adults with SB living in the US, including
those not necessarily receiving care in a SB clinic, compared with what exists in the literature to date. The majority of people with SB in the United States are now older than 18 years of age.16 Although significant progress has been made in conducting research in adults through the NSBPR, a limitation of doing research on patients receiving care in formalized SB clinics is that it does not sample adults who lack access to care or who receive care through other means. This study, therefore, may help fill a gap in research by sampling some adults not typically captured in other studies, and provide information about whether those studies are representative of the greater SB population. In 2016 and at the time of this survey, 27% of those enrolled in the NSBPR were 18 years and older, with ages ranging from 18 to 89 years. Roughly 53% of respondents to this survey reside in states without clinics that see adults and participate in the NSBPR. Compared with previously reported outcomes using NSBPR data, this survey captured more responses from females (74% vs. 53e57%) and white respondents (84% vs. 64%e84%), and fewer with private health insurance (31% vs. 42e54%).17 Skin breakdown in this study was associated with lower ambulation ability, and the most common location was the foot. These findings are consistent with a previous NSBPR publication which found that pressure ulcers were most commonly seen on the foot and were related to wheelchair use and higher level of lesion which is also correlated with ambulation status.13 In that study, pressure ulcers were also related to urinary incontinence, shunt
Please cite this article as: Morley CP et al., Survey of U.S. adults with Spina Bifida, Disability and Health Journal, https://doi.org/10.1016/ j.dhjo.2019.100833
C.P. Morley et al. / Disability and Health Journal xxx (xxxx) xxx
presence, above the knee orthopedic surgery, recent surgery, and male sex. It is not surprising that self-reported UTIs were not related to any of the other variables. Healthcare providers sometimes erroneously tell their patients they have a UTI and treat them on the basis of laboratory testing alone. However, individuals with SB and neurogenic bladder who self-catheterize often have chronic bacterial colonization of the bladder which can be mistaken for a UTI by providers.18 The diagnosis of UTI in patients with neurogenic bladder who self-catheterize requires the presence of symptoms in addition to laboratory evidence. Moreover, patients may also mistakenly believe they have a UTI when a different medical condition is present. The lack of response by almost 50% of participants when asked about the presence of UTIs may reflect confusion about what constitutes a true diagnosis of UTI. Since UTI, whether accurately diagnosed or not, is one of the most common reasons for hospitalization, an opportunity exists for patient and clinician education on this topic.19 The finding of a relationship between pain interference and mood symptoms with having no insurance or public insurance may suggest under treatment or underrecognition of symptoms in this population. Indeed, Dicianno et al. found that depressive symptoms are common and undertreated in adults with SB, and may bear relation to pain.20 It is possible that socioeconomic status or access to healthcare resources may affect mood and pain outcomes given the relationship of insurance to these variables. More research in this area is warranted. Approximately 50% of the patients in this study reported having a shunt for hydrocephalus. This figure is slightly lower than that reported in other studies.12 However, a lower shunt prevalence may be due to a higher proportion of older adults in our study. A survival effect may have occurred due to older and less severely affected adults who were born prior to the availability of surgical shunting for hydrocephalus having been more likely to survive. The fact that older adults in this study were less likely to have a shunt supports this finding. The relationship between shunting with lower ambulation ability is not surprising given that individuals with myelomeningocele subtype are more likely to have hydrocephalus and shunting and also tend to have reduced mobility.12 The finding that those with SB, especially those with shunts, are less likely to be in a relationship has been reported in other work.21 Several limitations to this study deserve discussion. First, several states were more fully represented in this survey than others. One possible reason for this is that survey distribution relied heavily on SBA connections to individuals, Chapters, and other organizations. Many of the states represented had an SBA Chapter or SBA has many contacts from those locations. Second, because ability to ambulate in the community was defined as “able to walk in the community, may require use of assistive devices or wheelchair for long trips,” based on the original classification by Hoffer et al.,15 some individuals who walk in the community but do so without assistive devices chose “other” as their ambulation status and indicated so in their comments. These individuals would be considered community ambulators in the original Hoffer classification because it allows for individuals who walk with or without assistive devices to be included in this category. However, because some individuals who marked “other” did not provide detail as to why they chose this category, we were not able to merge the “other” group with the “community ambulator” group. This may have resulted in a category of ambulation status that included individuals who were clinically indistinct from those in another category. Work is planned to further delineate the mobility categories. The survey instrument itself was exposed to an initial pilot test, with stakeholder-based validation only. A full validation process
7
(such as deployment in a small, known sample, with contemporaneous access to medical records for verification of responses) was not possible with the given resources and time constraints when the survey was developed. However, we believe the initial pilot test, and incorporation of end-user feedback, provided a robust developmental step in the creation of the survey. Additionally, due to the open-access nature of the survey, the data were subject to a number of validation issues and were therefore thoroughly reviewed and cleaned as needed. The open access nature of the survey, and its deployment online, may have led to several selection biases, as well as validity issues. For example, it was not possible to verify whether the respondent was truly an adult with SB. However, given the nature of questions and the length of the survey, it is unlikely many individuals completed the survey as a simple hoax. More seriously, it is possible that our methods of dissemination oversampled those with better access to the internet, those with more direct engagement with national organizations (such as the Spina Bifida Association), those with fewer health complications, and so forth. Indeed, a large portion of the initial sample was eliminated because those responses were clearly duplicates, incomplete, or were revealed to be submitted by ineligible participants. It is not possible to estimate, with any degree of certainty, how the elimination of these responses affected our findings (since many of those responses came from anonymous respondents, who did not complete the questionnaire). However, as our results are not dissimilar from smaller, previous studies,4e6,9,10,22 we believe the descriptive information presented here to be useful in informing further research. However, the large number of omitted, unusable responses (593 of 1445) should be taken into consideration when considering these findings. Finally, we did not collect particular subtypes of SB. Individuals with myelomeningocele, the most severe form of SB, commonly have multiple secondary conditions which impact patient outcomes. The current survey was also not designed to measure comparability of complications (or rates of complications) between SB and other conditions, such as spinal cord injuries. Absence of this variable limits our ability to delineate differences among subgroups of participants. However, because a majority of individuals with myelomeningocele have hydrocephalus and require shunting, shunt status may also serve as a proxy variable for this distinction.18 Nevertheless, the information presented here should be taken as descriptive information from a particular convenience sample. While it may be used as a reference point, there still exists a need for gold-standard studies to examine true population prevalence rates, both for reference within the population of adults with SB, as well as for comparison with other populations who have conditions resulting in similar complications, and with the general population. Adults with SB in the US live with a wide range of complications which are potentially under-monitored, with predictors of complications that require further research. Ideally, a combination of ongoing analysis of NSBPR real-time data, augmented by continued survey-based monitoring to capture those who may not be included as adults in the NSBPR and/or who may have intermittent data reported in the registry, should continue, in order to identify and further explain predictors of SB complications that affect the lives of those who live with SB. Conclusions Using an exploratory cross-sectional study implemented through an online survey to gather information from a national convenience sample of adults with SB, we attempted to identify medical complications associated with SB related to skin breakdown, pain, and UTIs, and to examine socio-demographic differences related to these complications.
Please cite this article as: Morley CP et al., Survey of U.S. adults with Spina Bifida, Disability and Health Journal, https://doi.org/10.1016/ j.dhjo.2019.100833
8
C.P. Morley et al. / Disability and Health Journal xxx (xxxx) xxx
This unique study was meant to provide a population-wide, general overview of the healthcare conditions of adults taking the survey, viewed from outside the clinical context. Our findings provide insight into the health status of an under represented group of people. It is our hope that this study will provide the impetus for further detailed exploration of these and other issues and include additional methods to increase participation in areas throughout the country. Given the likelihood that improved, continual care for pediatric patients with SB will lead to additional adults with SB, the results of this limited study illustrate the necessity for additional research into the healthcare status and requirements of the growing adult populations with SB. This is critical to improving the care of adults with SB. Without further exploration, the resources to assure appropriate care cannot be guaranteed.
7.
8.
9.
10.
11.
12.
Disclosures 13.
None of the authors have any real or apparent conflicts of interest with the conduct of the study or the interpretation of results described in this manuscript to disclose.
14.
15.
Funding This work was supported by the Spina Bifida Association. References 1. Parker SE, Mai CT, Canfield MA, et al. Updated national birth prevalence estimates for selected birth defects in the United States, 2004-2006. Birth Defects Res Part A Clin Mol Teratol. 2010;88(12):1008e1016. https://doi.org/10.1002/ bdra.20735. 2. Webb TS. Optimizing health care for adults with spina bifida. Dev Disabil Res Rev. 2010;16(1):76e81. https://doi.org/10.1002/ddrr.99. 3. Kinsman SL, Doehring MC. The cost of preventable conditions in adults with spina bifida. Eur J Pediatr Surg. 1996;6(Suppl 1):17e20. https://doi.org/10.1055/ s-2008-1071031. 4. Verhoef M, Barf HA, Post MWM, van Asbeck FWA, Gooskens RHJM, Prevo AJH. Secondary impairments in young adults with spina bifida. Dev Med Child Neurol. 2004;46(6):420e427. 5. Barf HA, Post MWM, Verhoef M, Jennekens-Schinkel A, Gooskens RHJM, Prevo AJH. Restrictions in social participation of young adults with spina bifida. Disabil Rehabil. 2009;31(11):921e927. https://doi.org/10.1080/0963828080 2358282. 6. Veenboer PW, Procee AI, Verheijden JMA, Bosch JLHR, van Asbeck FWA, de
16. 17.
18.
19.
20.
21.
22.
Kort LMO. Medical and psychosocial problems in middle-aged spina bifida patients: survey among members of the Dutch patients' association. Disabil Rehabil. 2014;36(7):539e545. https://doi.org/10.3109/09638288.2013.801522. Wagner R, Linroth R, Gangl C, et al. Perception of secondary conditions in adults with spina bifida and impact on daily life. Disabil Health J. 2015;8(4):492e498. https://doi.org/10.1016/j.dhjo.2015.03.012. Griffith CH, Georgesen JC, Wilson JF. Specialty choices of students who actually have choices: the influence of excellent clinical teachers. Acad Med. 2000;75(3): 278e282. http://www.ncbi.nlm.nih.gov/pubmed/10724318. Accessed September 9, 2016. Wilson R, Lewis SA, Dicianno BE. Targeted preventive care may be needed for adults with congenital spine anomalies. Pharm Manag PM R. 2011;3(8): 730e738. https://doi.org/10.1016/j.pmrj.2011.05.021. Alriksson-Schmidt A, Josenby AL, Lindquist B, Westbom L. Pain and health status in adults with myelomeningocele living in Sweden. Castillo J. J Pediatr Rehabil Med. 2018;11(4):255e264. https://doi.org/10.3233/PRM-170517. Sawin KJ, Liu T, Ward E, et al. The national spina bifida patient registry: profile of a large cohort of participants from the first 10 clinics. J Pediatr. 2015;166(2): 444e450. https://doi.org/10.1016/j.jpeds.2014.09.039. e1. Dicianno BE, Karmarkar A, Houtrow A, et al. Factors associated with mobility outcomes in a national spina bifida patient registry. Am J Phys Med Rehabil. 2015;94(12):1015e1025. https://doi.org/10.1097/PHM.0000000000000404. Kim S, Ward E, Dicianno BE, et al. Factors associated with pressure ulcers in individuals with spina bifida. Arch Phys Med Rehabil. 2015;96(8):1435e1441. https://doi.org/10.1016/j.apmr.2015.02.029. e1. Thibadeau JK, Ward EA, Soe MM, et al. Testing the feasibility of a national spina bifida patient registry. Birth Defects Res Part A Clin Mol Teratol. 2013;97(1): 36e41. https://doi.org/10.1002/bdra.23094. Hoffer MM, Feiwell E, Perry R, Perry J, Bonnett C. Functional ambulation in patients with Myelomeningocele. : JBJS. J Bone Jt Surg. 1973;8(5). http:// journals.lww.com/jbjsjournal/Abstract/1973/55010/Functional_Ambulation_ in_Patients_with.14.aspx. Liptak GS, Garver K, Dosa NP. Spina bifida grown up. J Dev Behav Pediatr. 2013;34(3):206e215. https://doi.org/10.1097/DBP.0b013e31828c5f88. Castillo J, Lupo PJ, Tu DD, Agopian AJ, Castillo H. The national spina bifida patient registry: a decade's journey. Birth Defects Res November. 2018, e1407. https://doi.org/10.1002/bdr2.1407. Dicianno BE, Sherman A, Roehmer C, Zigler CK. Co-morbidities associated with early mortality in adults with spina bifida. Am J Phys Med Rehabil. 2018;97(12): 861e865. https://doi.org/10.1097/PHM.0000000000000964. Dicianno BE, Wilson R. Hospitalizations of adults with spina bifida and congenital spinal cord anomalies. Arch Phys Med Rehabil. 2010;91(4):529e535. https://doi.org/10.1016/j.apmr.2009.11.023. Dicianno BE, Kinback N, Bellin MH, et al. Depressive symptoms in adults with spina bifida. Rehabil Psychol. 2015;60(3):246e253. https://doi.org/10.1037/ rep0000044. Murray CB, Lennon JM, Devine KA, Holmbeck GN, Klages K, Potthoff LM. The influence of social adjustment on normative and risky health behaviors in emerging adults with spina bifida. Health Psychol. 2014;33(10):1153e1163. https://doi.org/10.1037/hea0000050. Wagner R, Linroth R, Gangl C, et al. Perception of secondary conditions in adults with spina bifida and impact on daily life. Disabil Health J. 2015;8(4): 492e498. https://doi.org/10.1016/j.dhjo.2015.03.012.
Please cite this article as: Morley CP et al., Survey of U.S. adults with Spina Bifida, Disability and Health Journal, https://doi.org/10.1016/ j.dhjo.2019.100833
Contents lists available at ScienceDirect
Disability and Health Journal journal homepage: www.disabilityandhealthjnl.com
Survey of U.S. adults with Spina Bifida Christopher P. Morley a, b, c, *, Sara Struwe d, Morgan A. Pratte e, Gerald H. Clayton f, Pamela E. Wilson f, Brad E. Dicianno g, Margaret K. Formica a, h, Laura A. Schad a, Judy Thibadeau d, Margaret A. Turk i, a a
Department of Public Health & Preventive Medicine, Upstate Medical University, 750 E Adams St, Syracuse, NY, USA Department of Family Medicine, Upstate Medical University, Syracuse, NY, USA c Department of Psychiatry & Behavioral Sciences, Upstate Medical University, Syracuse, NY, USA d Spina Bifida Association, 1600 Wilson Blvd, Suite 800, Arlington, VA, 22209, USA e Hospital Administration, Upstate Medical University, 750 E Adams St, Syracuse, NY, USA f Department of Physical Medicine & Rehabilitation, University of Colorado Denver, USA g Department of Physical Medicine & Rehabilitation, University of Pittsburgh School of Medicine School of Medicine, Kaufmann Medical Building, Suite 901, 3471 Fifth Avenue, Pittsburgh, PA, 15213, USA h Department of Urology, Upstate Medical University, 750 E Adams St, Syracuse, NY, USA i Department of Physical Medicine & Rehabilitation, Upstate Medical University, Syracuse, NY, USA b
a r t i c l e i n f o
a b s t r a c t
Article history: Received 16 April 2019 Received in revised form 12 July 2019 Accepted 26 July 2019
Background: Spina Bifida (SB) is one of the most common birth defects and causes of permanent disability in the United States (US), with approximately 3.5 cases per 10,000 live births. Objective: To identify complications associated with SB related to skin breakdown, pain, and urinary tract infections (UTIs), and to examine socio-demographic differences related to these complications. Methods: Exploratory cross-sectional study via online of a national US convenience sample of adults with SB. Results: We collected 1485 survey responses, of which 852 had complete, useable data. Skin breakdown in one or more locations during the past year was reported by 43.1%. After controlling for sociodemographic characteristics, only mobility variables remained significant predictors of skin breakdown (assistive device use OR ¼ 3.119, 95% CI: 1.749, 5.564; using a wheelchair OR ¼ 6.336, 95% CI: 3.442, 11.662). Pain in past seven days was reported by 46.9%. Single respondents (OR ¼ 0.621; 95% CI: 0.419, 0.921) and those with at least a Bachelor's degree (vs high school degree or less, OR ¼ 0.468; 95% CI: 0.283, 0.774) were less likely, and those using assistive devices were significantly more likely (OR ¼ 1.960; 95% CI: 1.163, 3.303), to report pain. About one-third (32.7%) reported having a UTI within the past 12 months. Notably, almost half (49.6%) of respondents did not answer this question. The presence of UTIs was not significantly related to any socio-demographic characteristics assessed. Conclusions: Adults with SB in the US live with a wide range of complications which are potentially undermonitored, with predictors of complications that require further research. © 2019 Published by Elsevier Inc.
Keywords: Spina bifida Disability Surveys Cross-sectional Epidemiology
Introduction Spina Bifida (SB) is one of the most common birth defects and
* Corresponding author. Department of Public Health & Preventive Medicine, Upstate Medical University, 750 E Adams St, WSK, 2262, Syracuse, NY, USA. E-mail addresses: [email protected] (C.P. Morley), [email protected] (S. Struwe), [email protected] (M.A. Pratte), Gerald.Clayton@ childrenscolorado.org (G.H. Clayton), [email protected] (P.E. Wilson), [email protected] (B.E. Dicianno), [email protected] (M.K. Formica), [email protected] (L.A. Schad), [email protected] (J. Thibadeau), [email protected] (M.A. Turk).
causes of permanent disability among children and adolescents in the United States, with a rate of approximately 3.5 cases per 10,000 live births.1 While strides have been made over the past couple decades in reducing the overall prevalence of SB, there has been limited research and intervention aimed at addressing healthcare across the lifespan for those with condition. A growing number of individuals with SB are transitioning to adulthood, resulting in a population with increased medical needs due to the combination of secondary conditions associated with SB and chronic conditions experienced by the general aging population.2 Existing SB surveillance efforts often focus on pediatric
https://doi.org/10.1016/j.dhjo.2019.100833 1936-6574/© 2019 Published by Elsevier Inc.
Please cite this article as: Morley CP et al., Survey of U.S. adults with Spina Bifida, Disability and Health Journal, https://doi.org/10.1016/ j.dhjo.2019.100833
2
C.P. Morley et al. / Disability and Health Journal xxx (xxxx) xxx
populations, and there is currently limited health information about adults who are aging with this condition. Assessments of costs associated with preventable conditions in adults with SB have existed for decades,3 and small studies using localized surveys or existing data have documented the presence of pain, secondary complications,4 and restrictions on social participation5 related as a result of SB.6e10 On a broader scale, in the United States (US), the National SB Patient Registry (NSBPR) was developed in 2008 through collaboration between the Spina Bifida Association and the Centers for Disease Control and Prevention to gain better understanding of patient care in Spina Bifida clinics across the United States. Analyses from the NSBPR have identified common medical procedures, bladder management methods, mobility levels, and other health issues related to SB among patients involved in the cohort.11e13 However, only a few of the clinics participating in the NSBPR provide services to patients beyond adolescence, resulting in low representation of adult experiences.14 While the NSBPR is a valuable resource for examining clinical outcomes among patients with SB, this registry does not account for individuals who receive care outside of specialty clinics or those who do not have access to care at all. Therefore, we currently lack a more nationally representative data source that can be used to identify health status issues within the general adult SB population. In order to expand upon the body of knowledge regarding the adult experience of SB, beyond what is captured in smaller studies or in the NSBPR, researchers from two universities collaborated with the Spina Bifida Association to develop and conduct a nationwide survey of adults with SB. The primary objective of this report is to describe our survey methodology, as well as to identify medical complications associated with SB related to skin breakdown, pain, and urinary tract infections (UTIs), and to examine sociodemographic differences related to these complications. We also make note of shunt presence and complications in the survey population. Methods Study design & survey development This was an exploratory cross-sectional study that implemented an online survey to gather information from a national convenience sample of adults with SB. Survey items were selected and suggested by a panel of researchers, including two physiatrists, a neuroscientist, an epidemiologist, a medical social scientist with expertise in survey design and special populations, and other public health professionals. The included items were written de novo, as well as drawn from items included in the NSBPR. The survey was comprised of 44 questions overall, and was organized into four
primary domains: 1) Demographics: informed consent to participate in the survey; date of birth; ZIP code; gender; ethnicity; race; marital status; education level; employment status; and income sources. 2) Healthcare Access: insurance; usual source of care; and healthcare facilities utilized for different medical situations including routine care, SB-related issues, and emergencies. 3) Health conditions (SB-related and general): functional motor level; ambulation status; skin breakdown; leg fractures and amputations; pain and its interference with daily activities and mood; UTIs; bladder and bowel management; shunt status, function, and infections; general medical conditions (e.g. hypertension, obesity, asthma, sleep problems, etc.); and mental health. 4) Permission to re-contact for future surveys. An outline of survey questions is provided in Table 1, and a copy of the survey is available upon request. Following survey item development, an online instrument was created using SurveyMonkey® software. The online instrument was tested among 10 adults with SB for suitability, function, question clarity, format, and length of the survey. Amendments to the survey were incorporated accordingly, following the pilot testing period. This study was reviewed and approved by the Institutional Review Board at the lead university on the study.
Sample & data collection The target population consisted of adults (18 years and older) with a diagnosis of SB. The survey was launched at the 2016 Spina Bifida Association National Conference in Bloomington, MN to an audience of approximately 700 people. They were asked to share the information with their own networks electronically and via word of mouth. The online survey link was disseminated by electronic notification to contact lists by staff from the Spina Bifida Association and partnering organizations via email and social media. Detailed explanation of the intent was included with the electronic notifications. It included information about the purpose of the survey, our intent to use the information to guide future directions of services and to assist in explaining the adult experience with SB. Dissemination strategies included: - Sending information to over 40,000 contacts in the US Spina Bifida Association database - Sending information to 130 SB Clinics in the US that were known to the SBA in 2016
Table 1 Description of survey items. Question Numbers
Content
Description
1 2e14
Consent language, with yes/no option Name, date of birth, gender, race, ethnicity, marital status, education level, employment status
15e17 18e22 23e28
Consent to participate Contact & Demographic information Income and Insurance Sources of Medical Care Medical Issues
29e32 33e37 38e39 40 41e44
Pain Bladder and Bowel Shunts Depression & Anxiety Permission to Re-contact
a
Sources of income, type and source of health insurance coverage Source of routine/primary care (if none, reason), sources of Spina Bifida-related care, sources of emergency care Ambulation status, functional level of lesion, general comorbid medical issues, skin breakdown, leg fractures, amputations Any chronic pain (last 7 days), pain interference with day-to-day activities and mood, location of pain UTIa (past 12 months), bladder & bowel emptying, bladder or bowel accidents Presence of shunt, lifetime history of shunt infection PHQ-4 Permission to re-contact, preferred methods of re-contact
UTI ¼ urinary tract infection.
Please cite this article as: Morley CP et al., Survey of U.S. adults with Spina Bifida, Disability and Health Journal, https://doi.org/10.1016/ j.dhjo.2019.100833
C.P. Morley et al. / Disability and Health Journal xxx (xxxx) xxx
- Sending out information through 17 Spina Bifida Association Chapters - Standing links on web and social media pages (SBA had over 36,000 followers on its pages in 2016) - Sending information to the United Spinal Association Although it is not possible to know how many individuals ultimately saw a link or direct invitation to participate in the survey, we estimate that approximately 50,000 individuals were reached through these dissemination efforts. Participation was voluntary and respondents were permitted to decline answering any questions that they wished to disregard. The data collection period lasted for approximately 6 months in duration, from June 2016 to December 2016. Analysis In order to verify that all respondents were at least 18 years of age, cases with missing information for date of birth were removed from the sample. Additionally, duplicate cases, in which the same individual submitted multiple responses to the survey, were identified by matching name, date of birth, and/or IP address. Upon identification of duplicate cases, the first response submitted was retained and subsequent responses were discarded. Subjects with extensive missing data were excluded from analyses. Several variables were re-categorized in preparation for the analyses described below. Education classifications were combined to create three main categories: 1) high school or less (less than a high school diploma, high school diploma or equivalent, or technical school/trade certificate); 2) some college (some college education or Associate's degree); and 3) Bachelor's degree or greater (Bachelor's degree, Master's degree, or Doctoral/Professional degree). Two primary marital status categories were determined: 1) single; and 2) partner or ever married (partner, married, separated/ divorced, or widowed). Employment status was also classified into two categories: 1) employed (employed full/part-time or work for family or own business); and 2) not employed (not employed, volunteer, or occasional worker). Three categories were utilized for insurance status: 1) no insurance; 2) public insurance (i.e. Medicaid, Medicare); and 3) private insurance. The ambulation status variable was originally comprised of four categories based on the classifications defined by Hoffer et al.,15 which include community ambulation (able to walk in the community, may require use of assistive devices or wheelchair for long trips), household ambulation (walk only indoors with assistive devices, may use wheelchair for some indoor activities) therapeutic ambulation (walk only in therapy sessions, otherwise use wheelchair), and wheelchair use (exclusively use wheelchair for all mobility). Additionally, an “unknown/unsure (describe as needed)” option was available for respondents who felt they did not belong in one of the four given categories. Upon preliminary analysis of the mobility variable, it was evident that a substantial proportion of respondents selected “unknown/unsure” as their mobility status and the majority of respondents who selected this option had provided commentary indicating that they walk without the use of any assistive devices. Therefore, a fifth category was created to represent the mobility status of these individuals, which had not been captured in the original four Hoffer categories. Furthermore, the Hoffer categories were condensed by combining community with household ambulation to create a “community þ household” category, and therapeutic ambulation with wheelchair use to create a “therapeutic þ wheelchair” category. This re-classification resulted in three primary mobility levels: 1) walk without assistive devices; 2) community þ household; and 3) therapeutic þ wheelchair. In order to characterize the sample, a descriptive analysis was
3
conducted to provide an overview of socio-demographic information and medical complications associated with SB among the study sample. Medical complications that were assessed included skin breakdown (yes/no), pain (yes/no), UTIs (yes/no), and shunt status (yes/no). Respondents that reported experiencing pain were then asked to rate how much pain interferes with their day-to-day activities and overall mood using a 10-point scale (1 ¼ no interference and 10 ¼ complete interference). Frequencies and percentages for skin breakdown and pain by location were also determined. Associations between socio-demographic characteristics and skin breakdown, pain, UTIs, and shunt status were then examined by conducting c2 and t-tests, as appropriate. Multivariable logistic and linear regression models were also used to adjust for the following variables: age (continuous), gender (male or female), ethnicity (Hispanic or non-Hispanic), race (White or non-White), marital status (single or partner/ever married), education (high school or less, some college, or Bachelor's degree or greater), employment (employed or not employed), insurance coverage (no insurance, public insurance, or private insurance), and mobility: 1) walk without assistive devices; 2) community þ household; and 3) therapeutic þ wheelchair. Descriptive statistics, c,2 and t-tests were conducted using SPSS Version 23, and regression models were tested using SAS Version 9.3. A distribution map was also created using UDS Mapper to illustrate the number of respondents per state in the United States. Results A total of 1485 survey responses were collected, of which 1445 individuals provided consent to participate in the study. A total of 373 cases were removed from the sample due to missing date of birth information and 174 cases were removed due to duplicate responses. Only 3 respondents were under the age of 18 and therefore eliminated from the sample. Finally, 43 respondents who indicated non-U.S. residence were also removed from the sample. Upon elimination of invalid responses, 852 cases remained for inclusion in the analysis sample. Respondent gender, race, ethnicity, and age are presented in Table 2. Nearly three-quarters of the sample were female (73.6%) and about one-quarter were male (25.9%), while the remaining respondents selected “other” as their gender or did not respond to the question. The vast majority of respondents (83.7%) identified as being White only, while only 4.0% identified with multiple races and 3.8% identified as being Black or African American only. About 8.6% of the sample indicated that they were of Hispanic or Latino ethnicity. Over one-third of the sample was between ages 25e34 years (35.1%), followed by 28.5% between the ages 35e44 years. Overall, the mean age of respondents was 37 years (SD ¼ 11), with an age range from 18 to 87 years. Fig. 1 illustrates the distribution of respondents by state within the United States, demonstrating a high concentration of respondents from some of the more populous states of the Northeast and Southeast regions, in addition to California and Texas. This distribution corresponds to the current United States population estimates by state, suggesting that in general, greater numbers of respondents were observed from states with greater population estimates. Table 3 presents respondent education level, marital status, employment status, and insurance status. When asked to indicate current or most recent education level, the greatest percentage of respondents reported having some college education (40.2%) followed by having a Bachelor's degree or greater (31.5%). In regard to marital status, the majority (57.9%) of respondents indicated that they were single. When asked about employment status, 51.7% reported that they were not employed. The majority of respondents
Please cite this article as: Morley CP et al., Survey of U.S. adults with Spina Bifida, Disability and Health Journal, https://doi.org/10.1016/ j.dhjo.2019.100833
4
C.P. Morley et al. / Disability and Health Journal xxx (xxxx) xxx Table 2 Respondent gender, race, ethnicity, and age. Characteristic Gender Female Male Other Missing Race White Black or African American Asian American Indian/Alaska Native Native Hawaiian/Pacific Islander Multiple Races Other Unsure Missing Ethnicity Hispanic or Latino Not Hispanic or Latino Unsure Prefer not to answer Missing Age 18e24 yrs. 25e34 yrs. 35e44 yrs. 45e54 yrs. 55e64 yrs. 65 yrs and older
N (%)
Table 3 Respondent education level, marital status, employment status, and insurance status. Characteristic
627 (73.6) 221 (25.9) 1 (0.1) 3 (0.4) 713 (83.7) 32 (3.8) 15 (1.8) 4 (0.5) 0 (0.0) 34 (4.0) 28 (3.3) 23 (2.7) 3 (0.4) 73 (8.6) 718 (84.3) 10 (1.2) 41 (4.8) 10 (1.2) 107 (12.6) 299 (35.1) 243 (28.5) 141 (16.5) 48 (5.6) 14 (1.6)
(54.9%) reported having health insurance coverage through some type of public insurance (i.e. Medicaid, Medicare) and 31.1% reported being enrolled in private health insurance. Results for both the original and condensed categories for ambulation status are provided in Table 4. After combining the original categories as previously described, 39.9% of individuals
Education level High school or less Some college Bachelor's degree or greater Missing Marital status Single Partner or ever married Other Prefer not to answer Missing Employment status Employed Not employed Missing Insurance status No insurance Public insurance (Medicaid/Medicare) Private insurance Unsure Missing
N (%) 231 (27.0) 343 (40.2) 268 (31.5) 10 (1.2) 493 (57.9) 323 (37.9) 18 (2.1) 17 (2.0) 1 (0.1) 359 (42.1) 441 (51.7) 52 (6.1) 27 (3.2) 468 (54.9) 265 (31.1) 35 (4.1) 57 (6.7)
walk in the home or community and may use assistive devices (e.g., crutches, braces), another 35.8% walk only therapeutically or primarily use a wheelchair, and 13.3% do not use assistive devices. The remaining respondents were either unsure of their mobility status or did not respond to this question on the survey. Overall, 43.1% of the sample reported experiencing skin breakdown in one or more locations during the past year (Table 5). Among those with skin breakdown, the most frequently reported location was the foot (55.3%), followed by the buttocks (39.0%) and the lower body region (27.0%), which was described as the thigh, hip, knee, shin or ankle. The presence of skin breakdown was
Fig. 1. - Respondent distribution map, United States. Note: Alaska and Hawaii not to scale.
Please cite this article as: Morley CP et al., Survey of U.S. adults with Spina Bifida, Disability and Health Journal, https://doi.org/10.1016/ j.dhjo.2019.100833
C.P. Morley et al. / Disability and Health Journal xxx (xxxx) xxx
5
Table 4 Respondent mobility level. Original Categories
N (%)
Condensed Categories
N (%)
Walk without assistive devices Community ambulation Household ambulation Therapeutic ambulation Wheelchair use Unsure Missing
113 (13.3) 272 (31.9) 68 (8.0) 13 (1.5) 292 (34.3) 20 (2.3) 74 (8.7)
Walk without assistive devices [Community þ Household]
113 (13.3) 340 (39.9)
[Therapeutic þ Wheelchair]
305 (35.8)
Unsure Missing
20 (2.3) 74 (8.7)
Table 5 Medical conditions associated with Spina Bifida. Condition Skin breakdown Yes No Unknown Missing Skin breakdown by locationa Head/neck Upper body Lower body (thigh, hip, knee, shin, ankle) Trunk (back, chest) Lower back Buttocks Genitals Foot Other Chronic pain related to Spina Bifida Yes No Unknown Missing Pain by locationa Head/neck Upper body Lower body (thigh, hip, knee, shin, ankle) Trunk (back, chest) Lower back Genitals Foot Other Pain interference (mean ± SD) Day-to-day activities Overall mood UTIb Yes No Unknown Missing Shunt Yes No Unknown Missing
N (%) 367 (43.1) 411 (48.2) 7 (0.8) 67 (7.9) 6 (1.6) 9 (2.5) 99 (27.0) 12 (3.3) 25 (6.8) 143 (39.0) 31 (8.4) 203 (55.3) 22 (6.0) 400 (46.9) 344 (40.4) 42 (4.9) 66 (7.7) 186 (46.5) 159 (39.8) 264 (66.0) 130 (32.5) 315 (78.8) 15 (3.8) 123 (30.8) 24 (6.0) 5.35 ± 2.39 5.56 ± 2.42 279 (32.7) 133 (15.6) 17 (2.0) 423 (49.6) 458 (53.7) 304 (35.7) 3 (0.4) 87 (10.2)
a Among respondents with the condition; respondents able to select more than one option; percentages do not sum to 100%. b UTI ¼ urinary tract infection.
significantly associated with employment status, in which 46.7% of respondents who are not employed reported having skin breakdown compared to only 37.6% of those who are employed (p ¼ 0.010). Ambulation status was also significantly associated with skin breakdown; those who do not use assistive devices to ambulate were least likely to experience skin breakdown at 20.4% compared to those who may walk with assistive devices at 42.6% and those who were wheelchair users at 62.0% (p < 0.001). After controlling for all socio-demographic characteristics in the multivariable logistic regression model, only the association with mobility remained significant; those who may walk with assistive devices had 3.119 (95% CI: 1.749, 5.564) times the odds of skin
breakdown compared to those who do not use assistive devices, and those who primarily use a wheelchair had 6.336 (95% CI: 3.442, 11.662) times the odds of skin breakdown compared to those who do not use assistive devices, as shown in Table 6. Table 5 presents that 46.9% of all respondents had experienced pain in the past seven days. Among those, the most common locations where pain was experienced included the lower back (78.8%) and lower body (66.0%) regions. The average pain interference rating for day-to-day activities was 5.35 (SD ¼ 2.39) and for overall mood was 5.56 (SD ¼ 2.42). The presence of pain was significantly associated with gender (female: 56.4% vs. male: 46.1%; p ¼ 0.014), marital status (single: 48.0% vs. partner/ever married: 63.2%; p < 0.001), education level (high school or less: 61.6% vs. some college: 55.2% vs. Bachelor's degree or greater: 46.7%; p ¼ 0.007), and mobility (no use of assistive devices: 51.0% vs. may walk with assistive devices: 63.5% vs. wheelchair use: 42.5%; p < 0.001). Some of these associations remained significant when controlling for other variables in a logistic regression model, as presented in Table 6. Respondents who were single were significantly less likely to report pain than those who have a partner or have ever been married (OR ¼ 0.621; 95% CI: 0.419, 0.921), those with at least a Bachelor's degree were less likely to report pain than those with a high school degree or less (OR ¼ 0.468; 95% CI: 0.283, 0.774), and those who may use assistive devices were significantly more likely to report pain than those who do not use assistive devices (OR ¼ 1.960; 95% CI: 1.163, 3.303). When comparing mean ratings of pain interference with day-to-day activities and overall mood, there were significant differences by insurance status for both day-to-day activities (no insurance: m ¼ 6.93 vs. public insurance: m ¼ 5.43 vs. private insurance: m ¼ 5.11; p ¼ 0.016) and mood (no insurance: m ¼ 7.29 vs. public insurance: m ¼ 5.49 vs. private insurance: m ¼ 5.57; p ¼ 0.022). Furthermore, marital status was related to pain interference with day-to-day activities (single: m ¼ 5.09 vs. partner/ever married: m ¼ 5.69; p ¼ 0.009), as were education level (high school or less: m ¼ 5.55 vs. some college: m ¼ 5.55 vs. Bachelor's degree or greater: m ¼ 4.80; p ¼ 0.012) and employment status (not employed: m ¼ 5.66 vs. employed: m ¼ 4.80; p < 0.001). However, these factors were not related to pain interference with mood. Table 7 presents the adjusted associations resulting from multivariable linear regression models for the two pain interference outcome measures, showing that ethnicity, race, marital status, and employment status are all statistically significantly related to pain interference with day-to-day activities in multivariable models at the p ¼ 0.05 level, while only insurance status is significantly associated with both pain interference and overall mood. About one-third (32.7%) of respondents reported that they have had a UTI within the past 12 months. Notably, almost half (49.6%) of respondents did not answer this question. The presence of UTIs was not found to be significantly related to any of the sociodemographic characteristics assessed in the analyses. Over half (53.7%) of respondents indicated having a shunt. The presence of a shunt was determined to be significantly associated with gender (female: 57.2% vs. male: 68.5%; p ¼ 0.005), age group
Please cite this article as: Morley CP et al., Survey of U.S. adults with Spina Bifida, Disability and Health Journal, https://doi.org/10.1016/ j.dhjo.2019.100833
6
C.P. Morley et al. / Disability and Health Journal xxx (xxxx) xxx
Table 6 Logistic regression odds ratios of breakdown, pain, and UTIsb by socio-demographic characteristics. Characteristic
Skin Breakdown (n ¼ 588) OR (95% CI)
Age (Continuous) 1.000 (0.983, 1.018) Female (vs. Male) 1.445 (0.971, 2.151) Hispanic or Latino (vs. Not) 1.015 (0.516, 1.996) Non-White (vs. White) 0.876 (0.525, 1.462) Single (vs. Ever Married/Partner) 0.910 (0.617, 1.344) Education (vs. High School) Some college 0.827 (0.532, 1.284) Bachelor's or greater 0.871 (0.536, 1.416) Employed (vs. Not Employed) 0.723 (0.478 1.095) Insurance (vs. Private Insurance) No insurance 1.525 (0.519, 4.482) Public insurance 0.926 (0.607, 1.411) Ambulation status (vs. walk without assistive devices) [Community þ Household]; 3.119** (1.749, 5.564) [Therapeutic þ Wheelchair] 6.336** (3.442, 11.662) a b
Pain (n ¼ 559) OR (95% CI)
UTI (n ¼ 311) OR (95% CI)
Shunt (n ¼ 572) OR (95% CI)
1.002 (0.985, 1.021) 1.193 (0.798, 1.783) 0.879 (0.452, 1.709) 0.908 (0.543, 1.518) 0.621a (0.419, 0.921)
0.991 1.648 0.818 1.222 0.942
0.944** (0.925, 0.963) 0.688 (0.437, 1.083) 1.409 (0.649, 3.058) 0.712 (0.403, 1.258) 1.517a (1.004, 2.293)
0.736 (0.467, 1.163) 0.468** (0.283, 0.774) 0.890 (0.582, 1.361)
1.069 (0.577, 1.981) 1.491 (0.716, 3.103) 0.823 (0.440, 1.540)
1.205 (0.732, 1.983) 1.488 (0.861, 2.572) 0.946 (0.593, 1.509)
0.879 (0.296, 2.605) 0.775 (0.507, 1.183)
1.624 (0.303, 8.697) 1.604 (0.874, 2.941)
0.836 (0.279, 2.506) 2.138** (1.355, 3.371)
1.960a (1.163, 3.303) 0.888 (0.513, 1.536)
1.031 (0.501, 2.120) 1.402 (0.619, 3.179)
1.829a (1.072, 3.122) 5.316** (2.921, 9.675)
(0.966, (0.924, (0.275, (0.532, (0.536,
1.017) 2.939) 2.437) 2.807) 1.654)
p 0.05; **p 0.01. UTIs ¼ urinary tract infections.
Table 7 Linear regression parameter estimates of pain interference by socio-demographic characteristics. Characteristic
Pain Interference with Daily Activities (n ¼ 328) b (p-value)
Pain Interference with Mood (n ¼ 328) b (p-value)
Age (Continuous) Female (vs. Male) Hispanic or Latino (vs. Not) Non-White (vs. White) Single (vs. Ever Married/Partner) Education (vs. High School or less) Some college Bachelor's or greater Employed (vs. Not Employed) Insurance (vs. Private Insurance) No insurance Public insurance Ambulation status (vs. walk without assistive devices) [Community þ Household]; [Therapeutic þ Wheelchair]
0.024 (0.072) 0.346 (0.248) 1.387 (0.010) 1.089 (0.007) 0.778 (0.007)
0.016 (0.255) 0.528 (0.095) 0.423 (0.456) 0.408 (0.334) 0.422 (0.161)
0.105 (0.735) 0.638 (0.079) 0.883 (0.005)
0.076 (0.817) 0.616 (0.108) 0.348 (0.295)
1.743 (0.020) 0.189 (0.542)
1.855 (0.019) 0.309 (0.343)
0.358 (0.340) 0.180 (0.666)
0.737 (0.063) 0.180 (0.683)
(18e24 yrs: 64.8%, 25e34 yrs: 70.7%, 35e44 yrs: 63.3%, 45e54 yrs: 52.3%, 55 yrs: 11.7%; p 0.001), marital status (single: 69.7% vs. partner/ever married: 44.8%; p 0.001), employment (not employed: 66.5% vs. employed: 54.7%; p ¼ 0.001), mobility (walk without assistive devices: 38.3% vs. may walk with assistive devices: 50.5% vs. wheelchair use: 79.5%; p 0.001), and insurance status (no insurance: 34.8% vs. public insurance: 68.6% vs. private insurance: 45.3%; p 0.001). Several associations remained significant after controlling for all socio-demographic characteristics in the multivariable logistic regression model; those who were older in age were less likely to report having a shunt (OR: 0.944; 95% CI: 0.925, 0.963), those who were single had 1.517 (95% CI: 1.004, 2.293) times the odds of having a shunt compared to those who have a partner or have ever been married, and those with public insurance had 2.138 (95% CI: 1.355, 3.371) times the odds of having a shunt than those with private insurance. In regards to mobility status, those who may use assistive devices to walk had 1.829 (95% CI: 1.072, 3.122) times the odds of having a shunt and those who primarily use a wheelchair had 5.316 (95% CI: 2.921, 9.675) times the odds of having a shunt compared to those who are able to walk without any assistive devices. Discussion This unique study reports and evaluates the experiences of a relatively large sample of adults with SB living in the US, including
those not necessarily receiving care in a SB clinic, compared with what exists in the literature to date. The majority of people with SB in the United States are now older than 18 years of age.16 Although significant progress has been made in conducting research in adults through the NSBPR, a limitation of doing research on patients receiving care in formalized SB clinics is that it does not sample adults who lack access to care or who receive care through other means. This study, therefore, may help fill a gap in research by sampling some adults not typically captured in other studies, and provide information about whether those studies are representative of the greater SB population. In 2016 and at the time of this survey, 27% of those enrolled in the NSBPR were 18 years and older, with ages ranging from 18 to 89 years. Roughly 53% of respondents to this survey reside in states without clinics that see adults and participate in the NSBPR. Compared with previously reported outcomes using NSBPR data, this survey captured more responses from females (74% vs. 53e57%) and white respondents (84% vs. 64%e84%), and fewer with private health insurance (31% vs. 42e54%).17 Skin breakdown in this study was associated with lower ambulation ability, and the most common location was the foot. These findings are consistent with a previous NSBPR publication which found that pressure ulcers were most commonly seen on the foot and were related to wheelchair use and higher level of lesion which is also correlated with ambulation status.13 In that study, pressure ulcers were also related to urinary incontinence, shunt
Please cite this article as: Morley CP et al., Survey of U.S. adults with Spina Bifida, Disability and Health Journal, https://doi.org/10.1016/ j.dhjo.2019.100833
C.P. Morley et al. / Disability and Health Journal xxx (xxxx) xxx
presence, above the knee orthopedic surgery, recent surgery, and male sex. It is not surprising that self-reported UTIs were not related to any of the other variables. Healthcare providers sometimes erroneously tell their patients they have a UTI and treat them on the basis of laboratory testing alone. However, individuals with SB and neurogenic bladder who self-catheterize often have chronic bacterial colonization of the bladder which can be mistaken for a UTI by providers.18 The diagnosis of UTI in patients with neurogenic bladder who self-catheterize requires the presence of symptoms in addition to laboratory evidence. Moreover, patients may also mistakenly believe they have a UTI when a different medical condition is present. The lack of response by almost 50% of participants when asked about the presence of UTIs may reflect confusion about what constitutes a true diagnosis of UTI. Since UTI, whether accurately diagnosed or not, is one of the most common reasons for hospitalization, an opportunity exists for patient and clinician education on this topic.19 The finding of a relationship between pain interference and mood symptoms with having no insurance or public insurance may suggest under treatment or underrecognition of symptoms in this population. Indeed, Dicianno et al. found that depressive symptoms are common and undertreated in adults with SB, and may bear relation to pain.20 It is possible that socioeconomic status or access to healthcare resources may affect mood and pain outcomes given the relationship of insurance to these variables. More research in this area is warranted. Approximately 50% of the patients in this study reported having a shunt for hydrocephalus. This figure is slightly lower than that reported in other studies.12 However, a lower shunt prevalence may be due to a higher proportion of older adults in our study. A survival effect may have occurred due to older and less severely affected adults who were born prior to the availability of surgical shunting for hydrocephalus having been more likely to survive. The fact that older adults in this study were less likely to have a shunt supports this finding. The relationship between shunting with lower ambulation ability is not surprising given that individuals with myelomeningocele subtype are more likely to have hydrocephalus and shunting and also tend to have reduced mobility.12 The finding that those with SB, especially those with shunts, are less likely to be in a relationship has been reported in other work.21 Several limitations to this study deserve discussion. First, several states were more fully represented in this survey than others. One possible reason for this is that survey distribution relied heavily on SBA connections to individuals, Chapters, and other organizations. Many of the states represented had an SBA Chapter or SBA has many contacts from those locations. Second, because ability to ambulate in the community was defined as “able to walk in the community, may require use of assistive devices or wheelchair for long trips,” based on the original classification by Hoffer et al.,15 some individuals who walk in the community but do so without assistive devices chose “other” as their ambulation status and indicated so in their comments. These individuals would be considered community ambulators in the original Hoffer classification because it allows for individuals who walk with or without assistive devices to be included in this category. However, because some individuals who marked “other” did not provide detail as to why they chose this category, we were not able to merge the “other” group with the “community ambulator” group. This may have resulted in a category of ambulation status that included individuals who were clinically indistinct from those in another category. Work is planned to further delineate the mobility categories. The survey instrument itself was exposed to an initial pilot test, with stakeholder-based validation only. A full validation process
7
(such as deployment in a small, known sample, with contemporaneous access to medical records for verification of responses) was not possible with the given resources and time constraints when the survey was developed. However, we believe the initial pilot test, and incorporation of end-user feedback, provided a robust developmental step in the creation of the survey. Additionally, due to the open-access nature of the survey, the data were subject to a number of validation issues and were therefore thoroughly reviewed and cleaned as needed. The open access nature of the survey, and its deployment online, may have led to several selection biases, as well as validity issues. For example, it was not possible to verify whether the respondent was truly an adult with SB. However, given the nature of questions and the length of the survey, it is unlikely many individuals completed the survey as a simple hoax. More seriously, it is possible that our methods of dissemination oversampled those with better access to the internet, those with more direct engagement with national organizations (such as the Spina Bifida Association), those with fewer health complications, and so forth. Indeed, a large portion of the initial sample was eliminated because those responses were clearly duplicates, incomplete, or were revealed to be submitted by ineligible participants. It is not possible to estimate, with any degree of certainty, how the elimination of these responses affected our findings (since many of those responses came from anonymous respondents, who did not complete the questionnaire). However, as our results are not dissimilar from smaller, previous studies,4e6,9,10,22 we believe the descriptive information presented here to be useful in informing further research. However, the large number of omitted, unusable responses (593 of 1445) should be taken into consideration when considering these findings. Finally, we did not collect particular subtypes of SB. Individuals with myelomeningocele, the most severe form of SB, commonly have multiple secondary conditions which impact patient outcomes. The current survey was also not designed to measure comparability of complications (or rates of complications) between SB and other conditions, such as spinal cord injuries. Absence of this variable limits our ability to delineate differences among subgroups of participants. However, because a majority of individuals with myelomeningocele have hydrocephalus and require shunting, shunt status may also serve as a proxy variable for this distinction.18 Nevertheless, the information presented here should be taken as descriptive information from a particular convenience sample. While it may be used as a reference point, there still exists a need for gold-standard studies to examine true population prevalence rates, both for reference within the population of adults with SB, as well as for comparison with other populations who have conditions resulting in similar complications, and with the general population. Adults with SB in the US live with a wide range of complications which are potentially under-monitored, with predictors of complications that require further research. Ideally, a combination of ongoing analysis of NSBPR real-time data, augmented by continued survey-based monitoring to capture those who may not be included as adults in the NSBPR and/or who may have intermittent data reported in the registry, should continue, in order to identify and further explain predictors of SB complications that affect the lives of those who live with SB. Conclusions Using an exploratory cross-sectional study implemented through an online survey to gather information from a national convenience sample of adults with SB, we attempted to identify medical complications associated with SB related to skin breakdown, pain, and UTIs, and to examine socio-demographic differences related to these complications.
Please cite this article as: Morley CP et al., Survey of U.S. adults with Spina Bifida, Disability and Health Journal, https://doi.org/10.1016/ j.dhjo.2019.100833
8
C.P. Morley et al. / Disability and Health Journal xxx (xxxx) xxx
This unique study was meant to provide a population-wide, general overview of the healthcare conditions of adults taking the survey, viewed from outside the clinical context. Our findings provide insight into the health status of an under represented group of people. It is our hope that this study will provide the impetus for further detailed exploration of these and other issues and include additional methods to increase participation in areas throughout the country. Given the likelihood that improved, continual care for pediatric patients with SB will lead to additional adults with SB, the results of this limited study illustrate the necessity for additional research into the healthcare status and requirements of the growing adult populations with SB. This is critical to improving the care of adults with SB. Without further exploration, the resources to assure appropriate care cannot be guaranteed.
7.
8.
9.
10.
11.
12.
Disclosures 13.
None of the authors have any real or apparent conflicts of interest with the conduct of the study or the interpretation of results described in this manuscript to disclose.
14.
15.
Funding This work was supported by the Spina Bifida Association. References 1. Parker SE, Mai CT, Canfield MA, et al. Updated national birth prevalence estimates for selected birth defects in the United States, 2004-2006. Birth Defects Res Part A Clin Mol Teratol. 2010;88(12):1008e1016. https://doi.org/10.1002/ bdra.20735. 2. Webb TS. Optimizing health care for adults with spina bifida. Dev Disabil Res Rev. 2010;16(1):76e81. https://doi.org/10.1002/ddrr.99. 3. Kinsman SL, Doehring MC. The cost of preventable conditions in adults with spina bifida. Eur J Pediatr Surg. 1996;6(Suppl 1):17e20. https://doi.org/10.1055/ s-2008-1071031. 4. Verhoef M, Barf HA, Post MWM, van Asbeck FWA, Gooskens RHJM, Prevo AJH. Secondary impairments in young adults with spina bifida. Dev Med Child Neurol. 2004;46(6):420e427. 5. Barf HA, Post MWM, Verhoef M, Jennekens-Schinkel A, Gooskens RHJM, Prevo AJH. Restrictions in social participation of young adults with spina bifida. Disabil Rehabil. 2009;31(11):921e927. https://doi.org/10.1080/0963828080 2358282. 6. Veenboer PW, Procee AI, Verheijden JMA, Bosch JLHR, van Asbeck FWA, de
16. 17.
18.
19.
20.
21.
22.
Kort LMO. Medical and psychosocial problems in middle-aged spina bifida patients: survey among members of the Dutch patients' association. Disabil Rehabil. 2014;36(7):539e545. https://doi.org/10.3109/09638288.2013.801522. Wagner R, Linroth R, Gangl C, et al. Perception of secondary conditions in adults with spina bifida and impact on daily life. Disabil Health J. 2015;8(4):492e498. https://doi.org/10.1016/j.dhjo.2015.03.012. Griffith CH, Georgesen JC, Wilson JF. Specialty choices of students who actually have choices: the influence of excellent clinical teachers. Acad Med. 2000;75(3): 278e282. http://www.ncbi.nlm.nih.gov/pubmed/10724318. Accessed September 9, 2016. Wilson R, Lewis SA, Dicianno BE. Targeted preventive care may be needed for adults with congenital spine anomalies. Pharm Manag PM R. 2011;3(8): 730e738. https://doi.org/10.1016/j.pmrj.2011.05.021. Alriksson-Schmidt A, Josenby AL, Lindquist B, Westbom L. Pain and health status in adults with myelomeningocele living in Sweden. Castillo J. J Pediatr Rehabil Med. 2018;11(4):255e264. https://doi.org/10.3233/PRM-170517. Sawin KJ, Liu T, Ward E, et al. The national spina bifida patient registry: profile of a large cohort of participants from the first 10 clinics. J Pediatr. 2015;166(2): 444e450. https://doi.org/10.1016/j.jpeds.2014.09.039. e1. Dicianno BE, Karmarkar A, Houtrow A, et al. Factors associated with mobility outcomes in a national spina bifida patient registry. Am J Phys Med Rehabil. 2015;94(12):1015e1025. https://doi.org/10.1097/PHM.0000000000000404. Kim S, Ward E, Dicianno BE, et al. Factors associated with pressure ulcers in individuals with spina bifida. Arch Phys Med Rehabil. 2015;96(8):1435e1441. https://doi.org/10.1016/j.apmr.2015.02.029. e1. Thibadeau JK, Ward EA, Soe MM, et al. Testing the feasibility of a national spina bifida patient registry. Birth Defects Res Part A Clin Mol Teratol. 2013;97(1): 36e41. https://doi.org/10.1002/bdra.23094. Hoffer MM, Feiwell E, Perry R, Perry J, Bonnett C. Functional ambulation in patients with Myelomeningocele. : JBJS. J Bone Jt Surg. 1973;8(5). http:// journals.lww.com/jbjsjournal/Abstract/1973/55010/Functional_Ambulation_ in_Patients_with.14.aspx. Liptak GS, Garver K, Dosa NP. Spina bifida grown up. J Dev Behav Pediatr. 2013;34(3):206e215. https://doi.org/10.1097/DBP.0b013e31828c5f88. Castillo J, Lupo PJ, Tu DD, Agopian AJ, Castillo H. The national spina bifida patient registry: a decade's journey. Birth Defects Res November. 2018, e1407. https://doi.org/10.1002/bdr2.1407. Dicianno BE, Sherman A, Roehmer C, Zigler CK. Co-morbidities associated with early mortality in adults with spina bifida. Am J Phys Med Rehabil. 2018;97(12): 861e865. https://doi.org/10.1097/PHM.0000000000000964. Dicianno BE, Wilson R. Hospitalizations of adults with spina bifida and congenital spinal cord anomalies. Arch Phys Med Rehabil. 2010;91(4):529e535. https://doi.org/10.1016/j.apmr.2009.11.023. Dicianno BE, Kinback N, Bellin MH, et al. Depressive symptoms in adults with spina bifida. Rehabil Psychol. 2015;60(3):246e253. https://doi.org/10.1037/ rep0000044. Murray CB, Lennon JM, Devine KA, Holmbeck GN, Klages K, Potthoff LM. The influence of social adjustment on normative and risky health behaviors in emerging adults with spina bifida. Health Psychol. 2014;33(10):1153e1163. https://doi.org/10.1037/hea0000050. Wagner R, Linroth R, Gangl C, et al. Perception of secondary conditions in adults with spina bifida and impact on daily life. Disabil Health J. 2015;8(4): 492e498. https://doi.org/10.1016/j.dhjo.2015.03.012.
Please cite this article as: Morley CP et al., Survey of U.S. adults with Spina Bifida, Disability and Health Journal, https://doi.org/10.1016/ j.dhjo.2019.100833