Skin-related issues have a significant impact on health, activities of daily living, and quality of life among people with spina bifida. Data presented by select clinics that participate in the National Spina Bifida Patient Registry reported that 26% of individuals had a history of pressure injuries with 19% having had one in the past year. The spina bifida community lack direct guidelines on prevention of these and other skin related issues. The Integument (skin) Guidelines focus on prevention, not treatment, of existing problems.
Using a consensus building methodology, the guidelines were written by experts in spina bifida and wound care.
The guidelines include age-grouped, evidence-based guidelines written in the context of an understanding of the whole person. They are presented in table format according to the age of the person with spina bifida.
These guidelines present a standardized approach to prevention of skin-related issues in spina bifida. Discovering what results in successful minimization of skin-related issues with testing of technology or prevention strategies is the next step in protecting this vulnerable population.
Data presented by select clinics that participate in the National Spina Bifida Patient Registry (NSBPR) indicate that 26% of individuals had a history of pressure injuries and 19% reported having had one in the past year . Complications related to wounds were reported as the second most common primary diagnosis in spina bifida clinics . The literature on this topic indicates that the cost to care for an individual patient with a pressure injury ranges from $20,900 to $151,700 per pressure injury . A multi-clinic study from the NSBPR identified seven factors associated with pressure injuries that included the level of lesion, wheelchair use, urinary incontinence, shunt presence, above the knee orthopedic surgery, recent surgery and male gender .
|Age group (from guidelines)||Clinical questions|
|1–2 years 11 months||
|3–5 years 11 months||
|6–12 years 11 months||
|13–17 years 11 months and 18 years||
When comparing spina bifida patient admissions over a five-year period, with or without pressure injury, it was found that the average cost of hospitalization increased by 10% . At the same time, the estimated average length of stay increased by 24% in the presence of pressure injuries among hospitalized patients with spina bifida, compared with their peers without these injuries . These hospitalization statistics further emphasize the importance of attention to skin integrity in a coordinated care plan within the spina bifida population.
Although skin issues are not confined to pressure injuries, pressure injury prevention programs have shown as much as a 67% reduction in incidence with a substantial reduction in the cost of care . With that goal in mind, the information campaign to improve skin care awareness and wound prevention, “Did You Look?” is being evaluated as a prevention program . Elements from this campaign are included in these guidelines.
These Integument (skin) Guidelines focus on prevention of skin issues and not treatment of existing problems. Though spina bifida specific evidence is limited, practices related to wound prevention in other populations can be applied to the spina bifida population. These guidelines are built on that evidence as well as clinical experiences of health care professionals in the spina bifida community.
1.1Guidelines goals and outcomes
The desired clinical outcomes are clinically based on achieving the best skin health with consideration of the function of the skin along with quality of life that can be affected by loss of skin integrity. The goals of the Integument Guidelines are both practical and aspirational. Practical in the delineation of specific steps to minimize disruptions in skin integrity, and aspirational in our goal to dramatically reduce the incidence of pressure injury and thus have a significantly positive outcome on the health and financial impact on individuals with spina bifida.
Outcomes of the guidelines are written as levels of prevention. For the Integument Guidelines, the following three outcomes were identified.
Primary (intervene before an adverse health effect occurs)
• Maximize healthy skin, minimize disruptions in skin integrity.
Secondary (screen and identify problems in the earliest stages)
• Increase awareness of skin issues, risks, self-assessment, and prevention measures.
Tertiary (attempt to improve quality of life and reduce the symptoms)
• Improve health outcomes with minimal skin integrity issues across the lifespan.
The methodology for developing these guidelines has been published by Dicianno et al. . The process included one hundred spina bifida experts from around the world who used a consensus building methodology.
A set of clinical questions was the foundation of the Integument Guidelines. These clinical questions were used to focus attention on the outcomes or goals of the guidelines. Table 1 shows clinical questions specific to five sequential age groups. It was these questions that informed the research and writing of the guidelines.
|0–11 months||1.||Discuss insensate skin with parents and caregivers.|||
|2.||Discuss the risk factors that may contribute to impaired skin integrity.||[3, 7, 9–15]|
|3.||Teach parents and caregivers to inspect the skin (especially weight bearing or insensate areas) for changes in color, texture, and temperature.||[4, 7, 9–15]|
|4.||Discuss the need to check water temperature and encourage the use of a bath water thermometer.||[7, 9–12]|
|5.||Suggest that parents and caregivers check for hot surfaces that have been exposed to the sun such as car seats and playground equipment.||[7, 9–11]|
|6.||Recommend the use of barrier creams to protect the skin from damage as a result of bowel and bladder incontinence.||[7, 11, 12, 16]|
|1–2 years,11 months||1.||Teach parents and caregivers to inspect the skin (especially weight bearing or insensate areas) for changes in color, texture, and temperature.||[3, 4, 7, 9–16]|
|2.||Recommend the use of barrier creams to protect the skin from damage as a result of bowel and bladder incontinence.||[7, 11, 12, 16]|
|3.||Discuss the need to check water temperature and encourage the use of a bath water thermometer.||[7, 9–12]|
|4.||Suggest that parents and caregivers check for hot surfaces that have been exposed to the sun such as car seats and playground equipment.||[7, 15,16]|
|5.||Teach parents and caregivers how to inspect for well-fitting orthoses.||[3, 7, 16]|
|6.||Teach parents and caregivers that the child should wear protective clothing and footwear over insensate areas.||[7, 15, 16]|
|7.||Suggest that parents and caregivers seek treatment if the child’s skin is compromised.||[7, 16]|
|3–5 years,11 months||1.||Teach parents and caregivers to inspect the skin daily (especially weight bearing or insensate areas) for changes in color, texture, and temperature.||[3, 4, 7, 9–16]|
|2.||Encourage the child’s involvement in skin inspection.||[7, 10–16]|
|3.||Teach child to develop awareness of insensate areas.||[7, 10–16]|
|4.||Review with parents and caregivers the consequences of heat, moisture, or pressure to insensate areas.||[7, 10–16]|
|5.||Recommend the use of barrier creams to protect the skin from damage as a result of bowel and bladder incontinence.||[7, 10–16]|
|6.||Discuss the need to check water temperature and encourage the use of a bath water thermometer.||[7, 10–17]|
|7.||Suggest that parents and caregivers check for hot surfaces that have been exposed to the sun such as car seats and playground equipment.||[7, 10–17]|
|8.||Teach parents and caregivers how to inspect for well-fitting orthoses.||[7, 9–12]|
|9.||Teach parents and caregivers that the child should wear protective clothing and footwear (including water shoes in a pool or on pool deck) over insensate areas.||[7, 15, 16]|
|10.||Suggest that parents and caregivers seek treatment if the child’s skin is compromised.||[7, 16]|
|6–12 years,11 months||1.||Teach parents and caregivers to inspect the skin daily (especially weight bearing or insensate areas) for changes in color, texture, and temperature.||[3, 4, 7, 9–16]|
|2.||Encourage the child’s involvement in skin inspection.||[7, 9–16]|
|3.||Teach child to develop awareness of insensate areas.||[7, 10–16]|
|4.||Review with parents and caregivers the consequences of heat, moisture, or pressure to insensate areas.||[7, 10–16]|
|5.||Teach parents and caregivers how to look for well-fitting orthoses and other equipment that may cause injury to skin.||[7, 15, 16]|
|6.||Teach parents and caregivers that the child should wear protective clothing and footwear over insensate areas.||[7, 10, 16]|
|7.||Discuss the need to check water temperature and encourage the use of a bath water thermometer.||[7, 10–16]|
|9.||Promote adequate hydration and proper nutrition for healthy skin.||[7, 14, 18, 19], Nutrition, Metabolic Syndrome, and Obesity Guidelines|
|10.||Encourage parents, caregivers, and the child to keep skin clean and dry.||[7, 9–14]|
|11.||Suggest wearing seamless socks that are clean and dry.||[7, 12, 14]|
|12.||Suggest the use of antiperspirant on areas with perspiration, such as the feet and intertriginous areas.||[7, 16]|
|13.||Encourage parents and caregivers seek treatment if the child’s skin is compromised.||[7, 12]|
|14.||Advise parents and caregivers to engage non-ambulatory children in pressure relieving activities every 15 minutes.||[7, 10–11, 16–17]|
Table 2, continued
|13–17 years, 11 months and||1.||Inspect skin daily. Explore the teen perceptions of self-efficacy for skin checks and barriers to skin checks. Develop plans to increase self-efficacy, if needed.||[3, 4, 7, 10–16]|
|18 years||2.||Suggest children and adults who use wheelchairs to use a pressure-relieving cushion and check it daily.||[7, 12–16, 19]|
|3.||Identify and discuss risk factors that specifically increase the risk of pressure injuries in children and adults with spina bifida, such as using a wheelchair, having had surgery above the knee, shunts, a higher level of lesion, recent surgery, bladder incontinence, and being of the male gender.||[3, 7, 9–12, 14–16]|
|4.||Review with the caregiver, child, or adult the consequences of heat, moisture, or pressure to insensate areas.||[7, 9–17]|
|5.||Teach parents, caregivers, child, adult how to inspect for well-fitting orthoses.||[7, 9–17]|
|6.||Discuss the need to check water temperature and encourage the use of a bath water thermometer.||[7, 9–17]|
|7.||Suggest that children and adults check for hot surfaces that have been exposed to the sun such as car seats.||[7, 10–16]|
|8.||Promote adequate hydration and proper nutrition for healthy skin.||[7, 14, 18–19], Nutrition, Metabolic Syndrome, and Obesity Guidelines|
|9.||Encourage parents, caregivers, children, and adults to keep skin clean and dry.||[7, 9–14]|
|10.||Suggest wearing seamless socks that are clean and dry.||[7, 12, 14]|
|11.||Suggest the use of antiperspirant on areas with perspiration, such as the feet and intertriginous areas.||[7, 16]|
|12.||Seek treatment if the skin is compromised.||[7, 12]|
|13.||Advise children and adults who are non-ambulatory and use a wheelchair to engage in pressure-relieving activities every 15 minutes.||[7, 10–11, 16–17]|
|14.||Teach safe transfer skills to non-ambulatory patients.||[7, 14]|
|15.||Seek treatment immediately for any pressure injury. Refer to wound clinic for any pressure injury at stage three or greater.||[1, 5, 7, 12]|
The guidelines are presented in Table 2. The first column are the guidelines for skin care. The literature supporting the guidelines is listed in the column on the right. Each age group is represented with consideration to the potential variations in each individual’s functional abilities. New research which supports the guidelines has been added to this table. Note that some of the guidelines are cross-referenced to other guideline chapters.
The guidelines for integument care provide a rational and comprehensive approach to the prevention of skin integrity issues in the spina bifida population. Given the large number of people with spina bifida who present with a pressure injury, prevention is a priority . These guidelines provide a rational and effective approach to preventing or minimizing integument issues.
We know from previous work what factors are associated with an increased risk of pressure injury . So what more can be done? There has been interval work since the release of the guidelines that has strived to improve outcomes in skin integrity. Studies focusing on evidence-based wellness programs, mobile health, applications for smart phone applications, and other novel technologies have shown improved health regarding preventable secondary conditions in spina bifida  as well as short term positive changes in self-management and independence . In one such system, a smartphone application allowed the uploading photos of pressure injuries through a secure system . Studies utilizing common technology can greatly enhance evaluation, diagnosis, and initiation of care.
This is further reinforced by a review article looking at the potentially positive effect of compensatory feedback technologies . Although this article addressed spinal cord injury patients, there is considerable overlap in the literature on wheelchair users between spina bifida and spinal cord injury. With varied feedback technologies included, the potential positive effect of compensatory feedback and technology to guide behaviors that reduce the risk of pressure injury is significant .
The wound care literature supports a standardized approach that is important for pressure injury prevention which includes risk assessment, evidence-based guidelines, prevention strategies, and care specific to vulnerable populations. The “Did You Look” bundle of risk assessment, skin assessment, and education is one such approach and is currently being tested through select National Spina Bifida Patient Registry clinics .
In summary, these guidelines provide levels of prevention strategies customized to specific age groups that promote skin integrity across the lifespan of individuals with spina bifida.
This edition of the Journal of Pediatric Rehabilitation Medicine includes manuscripts based on the most recent “Guidelines For the Care of People with Spina Bifida,” developed by the Spina Bifida Association. Thank you to the Spina Bifida Association for allowing the guidelines to be published in this forum and making them Open Access.
The Spina Bifida Association has already embarked on a systematic process for reviewing and updating the guidelines. Future guidelines updates will be made available as they are completed.
• Timothy J. Brei, MD, Spina Bifida Association Medical Director; Developmental Pediatrician, Professor, Seattle Children’s Hospital
• Sara Struwe, MPA, Spina Bifida Association President & Chief Executive Officer
• Patricia Beierwaltes, DNP, CPNP, Guideline Steering Committee Co-Chair; Associate Professor, Nursing, Minnesota State University, Mankato
• Brad E. Dicianno, MD, Guideline Steering Committee Co-Chair; Associate Medical Director and Chair of Spina Bifida Association’s Professional Advisory Council; Associate Professor, Department of Physical Medicine and Rehabilitation, University of Pittsburgh School of Medicine
• Nienke Dosa MD, MPH, Guideline Steering Committee Co-Chair; Upstate Foundation Professor of Child Health Policy; SUNY Upstate Medical University
• Lisa Raman, RN, MScANP, MEd, former Spina Bifida Association Director, Patient and Clinical Services
• Jerome B. Chelliah, MD, MPH, Johns Hopkins Bloomberg School of Public Health
• Julie Bolen, PhD, MPH, Lead Health Scientist, Rare Disorders Health Outcomes Team, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention
• Adrienne Herron, PhD Behavioral Scientist, Intervention Research Team, National Center for HIV/AIDS, Viral Hepatitis, STD, and TB Prevention, Centers for Disease Control and Prevention
• Judy Thibadeau, RN, MN, Spina Bifida Association Director, Research and Services; former Health Scientist, National Spina Bifida Program, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention
The development of these Guidelines was supported in part by Cooperative Agreement UO1DD001077, funded by the Centers for Disease Control and Prevention. Its contents are solely the responsibility of the authors and do not necessarily represent the official view of the Centers for Disease Control and Prevention or the Department of Health and Human Services.
Conflict of interest
There are no conflicts represented by the authors.
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