Spina Bifida and Hydrocephalus

Spina Bifida and Hydrocephalus

Description #

Meet Janine.

She is 22 years old and recently moved out on her own. Janine used to walk with crutches, however, due to her ongoing weight gain, she has been using a wheelchair more often. Janine has never considered exercise or physical activity to be an option for her (she spends a lot of time doing sedentary activities such as watching movies and playing computer games), until her doctor suggested that exercise would help to improve her overall mobility. Janine sometimes struggles with following directions, however, she is friendly and loves talking with other people. Janine is nervous and a little unsure about this whole thing called exercise.

Meet Jason.

He is 37 years old and lives with his wife and young son. Jason was diagnosed with SB at birth and after being ambulatory (walking on two legs) for his first seven years, Jason has been using a manual wheelchair for about the last 30 years. Jason has some experience being physically active- he has played wheelchair basketball off and on since he was 16, however, he has never really spent any time working out in a gym. He knows his body well (for example, he’s well aware of the risk of pressure sores and overuse injuries for his shoulders) so Jason will be a great source for information in planning his exercise program.

What do I need to know? #

Spina bifida occurs when the spine and spinal cord doesn’t develop completely during foetal development (CSEP, 2002).

Can be experienced differently by different people. May result in any or all of the following:

  • Partial or total paralysis
  • Loss of sensation
  • Abnormal development of lower limbs

There are three types of spina bifida:

  • Occulta: also known as hidden, individual may experience very few or no complications,
  • Meningocele: spinal cord does not come through completely, nerve supply is typically not effected,
  • Mylomeningocele: most common type of SB, spinal cord and nerves are completely exposed and typically found in lumbar area

Often accompanied by hydrocephalus (related to fluid accumulated around the brain), which is associated with learning disabilities and orthopaedic deformities

Risk of developing pressure sores for those who are seated for prolonged periods of time (Plaum, Riemer, & Froslie, 2006)

  • This is a frequent complication for many wheelchair users
  • May not be associated with physical activity, but it’s important to be aware of the risk, especially if a participant indicates they have a pressure sore or are keeping watch on an areasporadically)


“My parents actually said, ‘I wonder, is there a gym in that building?’ so it was my dad who went up and said… ‘I think there’s a lift in that and there’s a gym and everything, why don’t you give it a go?’ So about six months ago… I went up and never looked back and it’s amazing, it changed my life dramatically you know, it’s fabulous!”

Why Exercise? Why is it important? #

Exercise is important for everyone! For people with SBH, the benefits of regular exercise include:

  • Enjoyment,
  • Development of self-concept & increase self-esteem
  • Opportunity to develop social relationships
  • Enhance mood & mental well-being
  • Improve physical functioning
  • Lessen fatigue & improve sleep quality
  • Increase strength, flexibility & range of motion

“It’s not all about you have to do this, you have to do it for your arms, it’s not, it’s for your heart, to keep it healthy because we are sitting down all the time. You have to keep moving, to keep your heart healthy really. That’s what it’s really for… I suppose I am rare in the sense that I do love what I do. But it’s not for the love of it, it’s really you’re looking after your heart.”

We want you to know #

In the words of people experiencing spina bifida on a daily basis, here are a few things they would like you to know about how they take part in fitness and exercise.

Keep an open mind

“It’s not much fun really. When you’re stuck in a chair twenty-four seven. Now [since I’ve been exercising regularly] I’m able to move myself around and I’m able to wheel the chair and stuff like that. But you know I’d like them [personal trainers] to, you know, have an open mind about what they think I should be able to do.”

Keep it fun!

“I’d love to do the gym with an active person that’s bubbly…And I know they kind of have to stay serious with their job and everything. Don’t get me wrong. But, you know, somewhere where they’d have a bit of crack [fun] as well.”

Include me in group fitness classes!

“so I went up, she had a look at me and said “right, I just need to know your movement and what you are capable of doing” and… she gave me an option of coming on my own or being involved in a class but I said… I’d be more eager to go in a class because of motivation and I haven’t looked back… they’re all able bodied people in the gym, I am the only wheelchair user so what she does, is adapts that class for me, so I am not, there’s never a point where I’m just sitting looking while they are doing something, I’m doing something as well you know which is fantastic.”

Encourage me to work hard!

“It’s just the way she adapts things and like yeah it doesn’t matter, you’re in a wheelchair but it doesn’t mean I’m gonna change… “I’m not gonna go any easier on you.” You know, which I appreciate. You know I’m not gonna go any easier on you because you are well capable she said… she just keeps pushing but not like that like in a bad way that I’d be crying or you know, good encouragement you know.”

“Encouragement is really important… like I could very easily back down and say “ah I’m not going to gym anymore” if I was having a bad day, “ah I’m not going”, but because I know that she’s up there, and… she motivates me and says “come on! Come on!” that’s what gets me through”

I want to be social too!

“Exercise and being healthy but, I think you have to have a balance… it’s social as well. So I talk to all those people now that are involved in the class and…  ‘yeah, see you next week, Are you coming?’ You know… interaction is great.”

Where do I start? Getting to know Someone with Spina Bifida & Hydrocephalus #

Given the individual nature of how people experience disability and impairment, this section will highlight the importance of the consultation phase. Many staff are nervous about their first meeting and this section will hopefully make them feel a little more comfortable and better prepared to manage this first step.

The Consultation #

TIP: To help you identify strategies that may be useful to create a supportive environment, you may wish to use the Inclusive TIMES tips below as a checklist. Ask the individual (or their support worker/ family member) which strategies they think will work best. This will help you to promote independence and encourage the individual to self-direct their support.

Use the consultation as an opportunity to get to know your new participant. In addition to gathering medical information and details about their fitness/ physical activity experience, you can focus on goal setting and learn about what they want. In addition to talking about exercise, ask the participant about how they will get to the fitness center, and what kind of support (if any) they need to be ready to exercise (for example, accessing the locker room).

You want to support the participant to be as independent as possible. Depending on the individual, this may change over time as they become more familiar with the setting and feel more confident and capable. Some participants may choose to include task-specific training as part of their goal setting (e.g., working on movements that will help them with getting out of bed or stopping quickly and changing directions in a busy environment).

When someone with SBH comes in for their consultation you may notice the following:

  • They may be overweight or obese (Higher rates of obesity are found in adult women with spina bifida) (Dosa, Foley, Eckrich, Woodall-Ruff, & Liptak, 2009)
  • They may have a number of unhealthy behaviours to work on (e.g., poor eating habits, sedentary activities, lack of regular physical activity participation)
  • They may experience symptoms of depression
  • They may have poor health-related fitness (Buffart, Van Den Berg-Emons, Van Wijlen-Hempel, Stam, & Roebroeck, 2008)
  • The may come in on their own or with a family member/ friend
  • They may seem like they are no different from anyone else!
  • They may use a wheelchair, crutches, or walk on their own
  • They may look bored or uninterested, but it’s possible they’re having a hard time following what you are saying
  • The transition age (becoming a young adult) is a critical time to encourage behaviour change (Bauman, Milligan, Lee, & Riva, 2012)

Many young people with spina bifida did not learn enough about how to live a healthy lifestyle- you can make a difference and help these young adults develop healthier habits!

Here’s the good news: Young adults with spina bifida who took part in regular physical activity identified fewer barriers to participation & reported a higher quality of life 

What are you going to do?

  • Direct your questions to the person with SBH, not their family member/ friend
  • Be patient. Listen.
  • Ask about current habits & behaviours (e.g., eating, exercising), but don’t judge. Be friendly and open. Remind the participant that it’s ok if they haven’t had good habits- you’re there to help them!
  • It’s important to build a relationship with the individual at this stage- don’t worry about saying exactly the right thing. Just be yourself. Be patient. Think about how you would like to be treated.
  • If you find the participant agreeing with everything you say, you can ask a few questions to make sure they understand everything you are talking about- some people with spina bifida have learning disabilities and may need to hear things a couple of times or in different ways in order to understand
  • It’s ok to ask about the kind of assistance they might need. For example, do they want a hand to open the door? If not, let them do it themselves. Again, be patient. If this is something they are working on then you need to give them the space to do so.

Implications for Exercise #

Many adults with SBH are pretty sedentary because they have not been encouraged to take part in exercise or physical activity. This doesn’t mean people with SBH shouldn’t exercise, there are lots of benefits, including improved movement, overall functioning, and quality of life. However, you should be aware of the following when someone with SBH starts a new exercise program (or completes a fitness assessment!).

  • They may be at risk of autonomic dysreflexia (due to inability to perceive pain for those with a spinal injury at T6 or higher) (Bauman, Milligan, Lee, & Riva, 2012)
    • This is a sudden (and potentially dangerous) increase in blood pressure
    • Other signs and symptoms include headache, flushed skin, sweating above the level of injury and changes in heart rate
    • Can be caused by complications from bowel and bladder, skin or respiratory issues
    • Ask the participant about their injury level. Have they experienced autonomic dysreflexia before? If so, they should be able to tell you what to look for.
    • Pay attention to the participant! If you notice any sudden changes in their appearance, such as flushed checks, sweating, rapid heart rate, take a break from exercise. Talk to the participant to see if they can identify why it’s happening. Suggest they manage or remove the issue before beginning exercise again. If this is not possible, medical treatment may be necessary.
  • They may experience shoulder pain (Nawoczenski, Ritter-Soronen, Wilson, Howe, Ludewig, 2006)
    • Recommend stretching and strengthening exercises that use a pulling motion
    • For example use the following link to view a video by the National Centre on Health, Physical Activity and Disability (NCHPAD): http://www.nchpad.org/Videos/PLwMObYmlSHaNyPBsDN4ATKi_Cn-dTyuK5#video-11
    • This will help to balance all of the pushing motion that is done throughout the day (for those that wheel) and can reduce pain and improve posture and function
  • They may have difficulties associated with motivation (Crytzer, Dicianno, & Fairman, 2013) (as mentioned above, due to previous barriers to exercise, low participation rates are common)
    • Use text/ email reminders to encourage return participation
  • They may not believe in themselves or feel confident in their abilities to exercise
    • To improve exercise efficacy, provide quality instruction about weight training & other exercise that you introduce
    • Adults with SCI & SB who received weight training instruction were more confident in their abilities, more likely to continue with exercise post intervention, & better able to perform activities of daily living! (Wise, 1999)
  • Their assistance needs and/or symptoms may change over time (Tornbom, Jonsson, & Sunnerhagen, 2013)
    • As you continue to work with the participant, have regular check ins to see how they are doing. Do they still the right level of support to complete their exercise program? Are there ways they could be more independent?
    • Continue to recommend regular exercise and physical activity participation to help manage the symptoms (this will be especially important as they age and symptoms become more aggravating)
  • People who use manual and power are less likely to participate in community activities and may have higher rates of depression (Dicianno, Gaines, Collins, & Lee, 2009)
    • Help them to make new social connections! Make it fun to come out to exercise in the community! Sometimes it might seem like a lot of work for wheelchair users (e.g., booking transit, planning out bathroom access, etc.), however, if you can make someone feel welcomed, they’ll want to come back
    • Introduce the participant to other people (this includes both staff & community members), encourage staff members to use participants’ first names, suggest the participant try out group fitness classes
  • Many people with hydrocephalus have a shunt (plastic tube) placed near their brain to help drain the fluid
    • Avoid trauma or any stress to the head and neck during activities
      • Be mindful of this during stretching or group fitness activities
      • Discuss the risk with participants so they are aware as well!

Don’t forget:

Every person is like every other person, like some other person, and like no other person.

Monitoring Exertion & Exercise Intensity #

It’s really important that people who are new to exercise learn how to monitor and describe how they are feeling or their response to exercise. One helpful way to do this is to introduce the Rate of Perceived Exertion (RPE) scale.  The smiley face RPE Scale or the OMNI scale (see below for examples of both) may be more suitable for participants with various disabilities than the age-predicted maximal heart rate (220 – age) (Stanish & Aucoin, 2007).

In the beginning, it is also recommended that participants measure their heart rate (using wearable technology such as a heart rate monitor may make this easier!). This information will help the trainer and participant to work together to learn about how the participant perceives their exertion during exercise and may help to inform programming strategies. For example, a trainer may encourage the participant to work towards a specific level of RPE, which can be complemented with information from the HR monitor to measure exercise intensity. Monitoring HR may also be useful in the event that participants are exercising at a level beyond the recommended intensity, which may happen if the participant is very engaged in the activity or trying to make a positive impression on the trainer.

OMNI Scale

Smiley Face Relative Perceived Exertion Scale

Medication & Exercise: What do you need to know? #

Although you will not be administering medication, it’s important that you have a brief conversation with the participant to ensure they are aware of any side effects of their medications that may impact their ability to take part in exercise and/or specific safety considerations.

Individuals with SBH may or may not be taking regular medications. Similar to the variability you will find across individuals, you will find that different people are taking different medications to manage different symptoms or secondary conditions. It is recommended that you talk about this during the consultation to see if there are any side effects that you should be aware of. You may also want to check in with them each time they come in to work out to see if there are any changes or any new information that you should know. It is also important to ensure the individual has the go ahead from their health care provider prior to beginning a new exercise program.

Inclusive TIMES: Tips & Strategies for Individuals with Spina Bifida & Hydrocephalus #

The tips and strategies found within the inclusive TIMES section will help you to create an environment that will support your participant’s participation in physical activity as independently as possible. It is important that you involve the participant whenever possible, along the way to ensure the program that you design will meet their needs physically, as well as emotionally and socially. Introducing a few simple strategies such as offering visual images of the participant’s program will help them to be more independent in completing their fitness programme. Ultimately, they will gain confidence and belief in their abilities (increasing self-efficacy) and experience self-determination (sense of control over their own destiny). This is key to establishing healthy habits that will be adopted for the long term.

Time #

  • Send a reminder (e.g., text) about appointment or exercise session
  • Be flexible with start time to accommodate for delays due to transit issues
  • Begin with shorter duration and lower intensity activities, progressing gradually to moderate intensity.
  • Schedule workouts for a time when there are typically less people in the gym (e.g., mid-morning). This will help with navigating the gym space for those using a wheelchair

Instruction #

  • Use a menu type approach with activity cards. The participant can choose from exercises you suggested to create a personalized workout
  • Provide clear, simple instructions in more than one format (e.g., verbal and visual).
  • Break complex movements into simple discrete movements
  • Introduce the RPE or WHEEL Scale and teach participant to learn their body’s response to various intensities. This will help them become aware of what intensity is appropriate for the body on any given day.
  • Use cycle or arm ergometer to measure aerobic capacity
  • Provide options for completing exercises in seated or standing position (depending on individual’s wheelchair use)
  • Have a chair available for you to use when demonstrating exercises from a seated position
  • Clear, simple verbal cues can be used to encourage proper form
  • Offer words of encouragement! Exercise and fitness might be new (and a little scary!) for the participant. Let them know you’re there to help!
  • Discover Accessible Fitness from NCPHAD is a great resource for exercises for wheelchair users (http://incfit.org/files/Discover%20Accessible%20Fitness_FINAL.pdf)

Movement #

  • Recommend upper body strength exercises that use a ‘pulling’ motion to balance all of the ‘pushing’ that is done by a wheelchair user
  • Use dynamic flexibility movements to warm up and static stretching to cool down (focus on tight areas such as pelvis, calves, and hip flexors)
  • Recommend a gentle group exercise class such as seated yoga or tai chi that might focus on both balance and flexibility
  • Introduce new movements and activities that the participant may never have considered trying! Remember, you have so much knowledge about fitness and exercise to offer!
  • Ask the participant if they have difficulty with performing any activities of daily living (for example, getting out of bed, opening a cupboard). Incorporate task-specific movements into their training programme!
  • If the participant has a shunt, be mindful of any exercise or movement that increases risk to the head and neck area. Avoid if necessary, or modify to reduce strain on the area.

Environment #

  • Choose equipment that provides as much support as possible (e.g., use a recumbent bike instead of an upright one)
  • Remove clutter and extra equipment where possible to open up the space and improve accessibility
  • Work with allied professionals to reassure the participant that their programme is safe. It’s important that the participant feels safe and supported in their fitness programme.
  • Seats for resting should be available for participants who need regular rest periods.
  • Use non-traditional equipment to assist with balance (for example, the back of a chair may offer the right amount of support- be sure the equipment is stable and can support body weight if necessary.

Support #

  • Use the consultation to discuss the desired level of support for the individual (this will be individual & will be unique to each participant). Some may require support staff/family members or use assistive technology, others may not.
  • If the participant has or is at risk of CHD or other heart conditions, work with their health professionals, especially in the beginning to ensure the participant is exercising at an appropriate & safe intensity (see relevant UFIT Explorer elements).
  • Social support is vital to promote a healthy lifestyle! Introduce the participant to others and suggest activities with partners to create a positive, fun & enjoyable atmosphere that is motivating
  • Link with local SBH groups if more specific information is needed or to connect participants to local supports or community opportunities.
  • Discuss the participant’s overall lifestyle choices (e.g., nutrition, sleep hygiene), to identify areas where they can enhance their overall health. For example, does the participant smoke? Eat healthy? Are there changes you can suggest?

References #

  • Barf, H. A., Post, M. W. M., Verhoef, M., Jennekens-Schinkel, A., Gooskens, R. H. J. M., & Prevo, A. J. H. (2009). Restrictions in social participation of young adults with spina bifida. Disability & Rehabilitation, 31, 921-927.
  • Bauman, C. A., Milligan, J. D., Lee, F. J., & Riva, J. J. (2012). Autonomic dysreflexia in spinal cord injury patients: an overview. The Journal of the Canadian Chiropractic Association, 56, 247–250.
  • Buffart, L. M., Van Den Berg-Emons, R. J. G., Van Wijlen-Hempel, M. S., Stam, H. J., & Roebroeck, M. E. (2008). Helath-related physical fitness of adolescents and young adults with myelomeningocele. European Journal of Applied Physiology, 103, 181-189.
  • Buffart, L. M., Van Den Berg-Emons, R. J., van Meeteren, J., Stam, H. J., & Roebroeck, M. E. (2009). Lifestyle, participation, and health-related quatliy of life in adolescents and young adults with myelomeningocele. Developmental Medicine & Child Neurology, 51, 886-894.
  • Crytzer, T. M., Dicianno, B. E., & Fairman, A. D. (2013). Effectiveness of an upper extremity exercise device and text message reminders to exercise in adults with spina bifida: A pilot study. Assistive Technology, 25, 181-193
  • Canadian Society for Exercise Physiology. (2002). Inclusive fitness and lifestyle services for all disabilities, Section IX, p. B9
  • Dicianno, B. E., Gaines, A., Collins, D. M., & Lee, S. (2009). Mobility, assistive technology use, and social integration among adults with spina bifida. American Journal of Physical Medicine & Rehabilitation, 88, 533-541.
  • Dosa, N. P., Foley, J. T., Eckrich, M., Woodall-Ruff, D., & Liptak, G. S. (2009). Obesity across the lifespan among persons with spina bifida. Disability & Rehabilitation, 31, 914-921.
  • Exercise is Medicine, ‘Your Prescription for Health Series’, available at: http://exerciseismedicine.org/assets/page_documents/YPH_All.pdf [accessed 6th April 2016]
  • Harvard School of Public Health, ‘Healthy Eating Plate & Healthy Eating Pyramid’, available at: https://www.hsph.harvard.edu/nutritionsource/healthy-eating-plate/ [accessed 9th May 2016]
  • Martin Ginis, K. A., Evans, M. B., Mortenson, W. B., & Noreau,, L. M. (2017). Broadening the conceptualization of ‘participation’ of persons with physical disabilities: A configurative review and recommendations. Archives of Physical Medicine and Rehabilitation, 98(2), 395-402. doi: 10.1016/j.apmr.2016.04.017.
  • Nawoczenski, D. A., Ritter-Soronen, J. Wilson, C. M., Howe, B. A. Ludewig, P. M. (2006). Clinical trial of exercise for shoulder pain in chronic spinal injury. Physical Therapy, 86, 1604-1619.
  • O’Connor, F., Casa, D., Davis, B., Pierre, P., Sallis, R. and Wilder, R., (2013), ACSM Sports Medicine: A Comprehensive Review, Lippincott Williams and Williams, China
  • Pescatello, L., Arena, R., Riebe, D., Thompson, P., eds. (2014), ACSM’s guidelines for exercise testing and prescription, 9th Edition, Baltimore: Lippincott Williams & Wilkins
  • Plaum, P. E., Riemer, G., & Froslie, K. F. (2006). Risk factors for pressure sores in adult patients with myelomeningocele: A questionnaire-based study. Cerebrospinal Fluid Research, 3, 14-18.
  • Ploemen, M. A. T., Verschuren, O., van Mechelen, C., Borst, H. E., de Leeuw, A. J., van der Hoef, M. & de Groot, J. F. (2015). Personal and environmental factors to consider when aiming to improve participation in physical activity in children with spina bifida: A qualitative study. BMC Neurology, 15, 1-11.
  • Soe, M. M., Swanson, M. E., Bolen, J. C., Thibadeau, J. K. , & Johnson, N. (2012). Health risk behaviours among young adults with spina bifida. Developmental Medicine & Child Neurology, 54, 1057-1064.
  • Specht, J., King, G., Brown, E., & Foris, C. (2002). The importance of leisure in the lives of persons with congenital physical disabilities. American Journal of Occupational Therapy, 56, 436-445.
  • Swain, D., Brawner, C., Chambliss, H., Nagelkirk, P., Paternostro Bayles, M. and Swank, A., eds. (2014), ACSM’s Resource Manual for Guidelines for Exercise Testing and Prescription, 7th Edition, Baltimore: Lippincott Williams & Wilkins
  • Tornbom, M., Jonsson, U., & Sunnerhagen, K. S. (2013). Increasing symptoms, met and unmet needs in adults with cerebral palsy or meningomyelocele: A longitudinal follow-up. Scandinavian Journal of Disability Research, 15, 249-263.
  • Wise, J. B. (1999). Effects of a curriculum to generalize self-efficacy from weight training exercises to activities of daily living in adults with spinal cord injuries or spina bifida. (Doctoral Dissertation). Retrieved from CINAHL Complete. (104143359)
  • WHO Global Action Plan 2013-2020, available at: http://apps.who.int/iris/bitstream/10665/94384/1/9789241506236_eng.pdf [accessed 12th July 2016]

 Further information/ Recommended Resources #


Copyright © 2020 by UNESCO Chair , Institute of Technology Tralee

All rights reserved. No part of this publication may be reproduced, distributed, or transmitted in any form or by any means, including photocopying, recording, or other electronic or mechanical methods, without the prior written permission of the publisher, except in the case of brief quotations embodied in critical reviews and certain other noncommercial uses permitted by copyright law. For licensing requests, write to the publisher, addressed as below:

UFIT c/o, UNESCO Chair, Institute of Technology Tralee, Tralee, Co Kerry. Ireland

or info@justdoufit.org


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