Myelomeningocele (Spina Bifida) Fact Sheet

Definition: NICHCY, 2011 Spina Bifida means cleft spine, which is an incomplete closure in the spinal column. In general, the three types of spina bifida (from mild to severe) are: 1. Spina Bifida Occulta: There is an opening in one or more of the vertebrae (bones) of the spinal column without apparent damage to the spinal cord. 2. Meningocele: The meninges, or protective covering around the spinal cord, have pushed out through the opening in the vertebrae in a sac called the meningocele. However, the spinal cord remains intact. This form can be repaired with little or no damage to the nerve pathways. 3. Myelomeningocele: This is the most severe form of spina bifida, in which a portion of the spinal cord itself protrudes through the back. In some cases, sacs are covered with skin; in others, tissue and nerves are exposed. Generally, people use the terms spina bifida and myelomeningocele interchangeably. This is the definition provided by the Nationaly Dissemination Center for Children with Disabilities Criteria for Diagnosis: Spina bifida is often diagnosed before birth. There are some cases (in instances where spina bifida is mild, such as spina bifida occulta) where symptoms are unnoticed until after the child is born. Prenatal Diagnosis During the second trimester, if there is an unusually high amount of alphafetoprotein in the mothers bloodstream, this indicates to doctors that the fetus has a neural tube defect. (This is determined through a maternal serum alpha fetoprotein and ultra sound.) A discovery of high amounts of alpha-fetoprotein will call for additional testing. Teston include: ultrasound or amniocentesis to help determine if spina bifida is present. Amniocentesis cannot determine how severe the spina bifida is, but it can determine for certain whether it is present. Postnatal Diagnosis Mild cases can be detected postnatally through X-ray examinations. A magnetic resonance imaging (MRI) or a computed tomography (CT) scan can reveal a clearer view of the spine and vertebrae. These exams can reveal if spina bifida occulta is present. Characteristics of the most severe form of spina bifida, Myelomeningocele Paralysis or muscle weakness below the area of the spine where the incomplete closure or cleft occurs. Loss of sensation below the cleft. Loss of bowel and bladder control. Complications Most people with spina bifida are noted as having a level of normal intelligence. Complications of SB range from inconsequential physical problems to severe physical and mental disabilities. These factors are dependent upon the person and the specific form of SB that is present.

-Hydrocephalus: fluid buildup can cause build up in the brain, and about 70%-90% of children diagnosed with Myelomeningocele (the most severe form of SB) have hydrocephalus. Its controlled through shunting. (NICHCY Disability Fact Sheet, 2011.) -Shunting: a surgical procedure that relieves the buildup of fluid in the brain. Brain damage is likely to occur if a drain (shunt) is not implanted (blindness and seizures are common also if this procedure is not done.) (Spina Bifida Association, 2008.) -Allergies: studies have found that 73% of children with SB are allergic to latex. Symptoms: watery eyes, hives, wheezing, rash, and life-threatening breathing problems. (Spina Bifida Association, 2008.) Help for Babies with SB SB is considered relatively common. However, there is now surgery to protect most children with SB from developing hydrocephalus. This operation can be performed in the first two days after birth, so most children with SB live full lives. Although these new surgeries help children to live full lives, they will need medical examinations as a supportive method to determine how SB may affect their other key developmental processes. The early intervention system is present in every state, so parents can get help their children need at a young age. -Early Intervention: system of services meant to help infants with disabilities until their 3rd birthday. This is one of the mandated laws of the IDEA. If a child with SB is categorized as needing an IFSP, they will be set up with this to fit their needs. -IFSP: Individualized Family Service Plan. The IFSP will make clear the needs of the family and the chld. This is provided through a sliding-fee (contingent upon their income.) Help for School-Aged Children Besides early intervention, IDEA requires all children with disabilities receive the necessary assistance in school, free of charge, if it is necessary. Then, an IEP will be developed based upon the needs of child. Educational Approaches/Implications Because children with Myelomeningocele, the most severe case of SB, will need medical operations throughout their school years, they should have accommodations made so that they can do well in school. A substantial number of children with Myelomeningocele need to learn how to manage their bowel and bladder functions. Some even need catheterization (the insertion of a tube to allow the passage of urine.) Clean catheterization is vital for children with severe SB. This can be incorporated in a bladder management program. Lots of children learn to do this on their own early on. In a few instances, children who have hydrocephalus have a few conditions that need accommodations for learning: Difficulty paying attention Difficulty expressing and/or understanding language Difficulty grasping reading or math. Most students receive the care they need during early intervention so this is not very common.

Successful inclusion for students with SB requires the school to, sometimes, change equipment and even the curriculum. In Section 504 of the Rehabilitation Act of 1973, there is a requirement for accessible facilities for those who need this. Some people with SB may need this. These accommodations include Ramps Adding elevators Offering courses on the ground level Children who have Myelomeningocele will need to learn mobility skills and may need assistance such as: Crutches Braces Wheel chairs Prevention Because there is no known single cause for spina bifida in children, prevention methods are based on studies and implications. The main source for prevention starts with mothers-to-be. If the diet of the future mother is enriched with folic acid, the chances of spina bifida are reduced. Foods with folic acid include: Whole grains Beans Leafy vegetables Fruits Folic acid supplements are now provided for future mothers.

DART Project Student Case Study: JOANNA

Name of Student: Joanna Degree Program: BA Honours in Business Studies and Human Resources Year/ Level of Study: Year 1 / Full-time Type of Institution: Campus based (Urban) Disability: Spina Bifida & Hydrocephalus (Wheelchair-user) Case study methodology: Interview with student and students support worker Learning & Teaching Contexts: Various (including lectures, class discussions, group work) Specific Issues/ Key Terms Library access; disclosure; assessment; lecturer support; trust This case study focuses on the experience of Joanna, a first year full-time student in the Department of Business Studies at an urban campus-based university. It was developed on the basis of an extensive interview with Joanna and her support worker. As well as detailing Joannas experiences, the Case Study incorporates perspectives provided by Joannas support worker.

NOTE: Joanna has consented to her name being used in the Case Study. To safeguard the anonymity of her support worker she will be referred to as SW throughout this Case Study.

INTRODUCTION Joanna is a full-time student who is just completing the first year of a BA in Business Studies and Human Resources. At the start of the next academic year she intends to change her degree to a BA Honours in Human Resources (HR) because of a particular interest she has developed in the subject area following study and visits to several HR departments. Joanna was born with Spina Bifida and Hydrocephalus. The Spina Bifida has left Joanna paralysed from the waist down and a wheelchair user; the hydrocephalus (water on the brain) means Joanna has a shunt in the right side of her brain and a valve in her heart. If pressure builds up causing headaches, Joanna is able to press the valve, relieving the pressure. Because of the Hydrocephalus, Joanna has difficulty with maths, including working with figures, spatial awareness and drawing graphs. Joanna lives at home with her parents and travels to campus for her lectures by taxi. Whilst on campus she has a support worker with her at all times to help her get to classes and make use of library facilities.

PREVIOUS EDUCATION Joanna attended a specialist nursery until the age of 3 and since then has attended mainstream schools always with the help of a support worker. During her time at infant and junior school, Joanna didnt encounter any difficulties with other students or teachers, apart from one teacher at infant school who Joanna described as coping with her disability by assuming she was stupid and ignoring her. Similarly at high school, Joanna did not encounter any difficulties with students or teachers, although she and her parents had to fight for two years to get Joanna accepted into the mainstream school of their choice. At high school Joanna studied GCSEs followed by a GNVQ (General National Vocational Qualification) and VCE (Vocational Certificate of Education) in Business Studies. Joanna feels that the two year fight to get her into a mainstream secondary school typifies the way in which her disability has affected her education. She and her parents have always had to be one step ahead, continually planning to ensure schools and courses will be accessible far in advance of final decisions being made.

TRANSITION TO HIGHER EDUCATION

Following successful completion of the GNVQ and VCA in Business Studies, Joanna decided to continue studying this subject area to degree level because it was something she had developed an interest in. Before choosing this degree programme Joanna consulted with school teachers and also contacted different universities to speak with business studies lecturers. Joanna feels her subject area choice was not influenced by her disability. However, Joanna narrowed down her final choice based on disability related factors. Firstly, Joanna wanted to be able to continue living at home whilst studying; she feels more comfortable at home and dislikes change and says that this could be related to her disability. Secondly, Joannas choice was influenced by the prevalence of maths based modules within the degree programme due to the Hydrocephalus Joanna has difficulties with maths and so opted for a university where the business studies degree programme involved less maths than similar courses elsewhere. THE STUDENTS EXPERIENCE Learning and Teaching Experience Joanna has encountered several barriers in learning situations since starting at university. Certain modules (such as economics, IT and data analysis) that have involved numerical work have as anticipated been very difficult for her. Joanna also has to be constantly aware of access to different learning environments such as classrooms, lecture theatres and libraries. Access issues include lifts to classrooms situated above the ground floor, the height of desks, width of doors etc..

Lectures Joanna has occasionally encountered the access issues previously described. During lectures, Joanna prefers to sit at the front because it makes her feel more involved in the lesson, while being closer to the door ensures an easier exit in case of fire. Generally, Joanna feels it is important that lecturers are not only aware of students disabilities, but the specific impact the disabilities can have on individuals. This could be achieved if students and lecturers could meet in private at the start of term so that the student can explain to the lecturer about their disability and how it will impact on their studies. This would lead to greater understanding for the lecturer and negate the likelihood of unforeseen problems cropping up later in the year. For Joanna, being able to hold this conversation in private, rather than in front of her fellow students, is the key. Group Work

In group work, Joanna has faced several difficulties. On a practical level, she has to make sure that group work away from the main classroom is planned for a location accessible to her something she occasionally has to remind her fellow group members about. In addition, Joanna does not feel comfortable about disclosing her Hydrocephalus to other students and so if calculations are involved she asks another group member to carry out that piece of work. She prefers to say I dont like numbers, can somebody else do that than explain her disability and ask someone to help her with the number work. Joanna says she would only mention her disability if she had to. Class Discussions Joanna tends to keep quiet during class discussions as she doesnt like talking in front of large groups of people preferring discussions on a one to one basis. Although Joanna feels this is mainly down to her personality she admits it could also have something to do with her disability. Accessing online and paper-based resources Using the recommended reading lists for modules, Joanna searches for books in the library using IT facilities. Once she has located a book, if unable to reach the shelf, Joana will ask her support worker or one of the library staff to get the book for her. However, because of the height of Joannas wheelchair she is not able to browse the upper shelves and pick up alternative books that arent suggested by tutors potentially missing out on other beneficial reading. When carrying out research Joanna prefers to use the Internet because this enables her to do all the searching and locating of information independently. Work Placements To date, Joanna hasnt had to undertake any work placements for her degree. If such a situation arises in future Joanna says she would have to check out the accessibility of potential organisations in advance. For example, making sure toilets and lifts are accessible and emergency evacuation procedures are in place. In terms of disclosing her disability, Joanna wouldnt disclose her Hydrocephalus and difficulty with numerical work straight away. She would only disclose if a trusting relationship has been established with a colleague/ manager, and only then, if a problem arises. Assessment / Examinations Joanna has encountered some difficulties with examinations mainly due to the Hydrocephalus affecting her ability to carry out numerical work and also her memory. Joanna receives extra time for examinations, which she rarely uses. She does not use any enabling technology within an exam setting. Joanna prefers coursework as this gives her the time to plan, step by step the work that needs to be done and also sort out any problems as and when they arise. Joanna uses a laptop with voice recognition software that allows her to dictate her assignments and then have them read back to her. The voice recognition software was allocated to Joanna through a Needs Assessment carried

out by the University Disability Services prior to the start of Joannas degree the funding for the software came from the Disabled Students Allowance. Once completed, a member of Joannas family helps her with proof reading her coursework as Joanna finds proof reading difficult. In terms of meeting deadlines, Joanna does not have any real problems because work assignments are given out well in advance of the due date. If work was due back in at short notice this would be more problematic for Joanna, as it wouldnt allow her time to plan.

Impact on Learning and Academic Progress Throughout her time in education, Joanna and her parents have adopted a strategy of staying one step ahead. This, combined with thorough planning, has meant that being a wheelchair user has not impacted significantly on Joannas learning and academic progress. The only exception that Joanna cites is not being able to browse books on higher shelves in the library and so potentially missing out on wider reading. Arguably, the Hydrocephalus (which is unseen and often not disclosed by Joanna) has had a more significant impact. Particular modules involving numerical work cause Joanna the most difficulties. On one such occasion, unable to communicate effectively with her lecturer, Joanna had to proactively approach a different lecturer to get the help and support she needed. The new lecturer met with Joanna on a one to one basis and went through the processes stage by stage, allowing Joanna to ask questions and gain an understanding of the subject. Being able to meet on a one to one basis was hugely beneficial for Joanna in terms of understanding. However, the fact that this help was not forthcoming from the initial lecturer, and that Joanna had to proactively seek a solution herself, meant Joanna experienced unnecessary difficulty prior to completing this particular module. Other practices adopted by the university that Joanna has found useful include lecturers giving out bulleted handouts to accompany modules, instead of dictating all information. The handouts enable all students to take brief notes at the same time as listening and so this practice is beneficial to disabled and non disabled students alike. In addition Joanna receives module guides and reading lists at the start of each module, showing her week by week, topics to be covered. This enables Joanna to plan ahead and let her support worker know times and dates when she will need assistance and when she will need to get books from the library in advance of lessons. Joanna has found the ongoing support from the Universitys Disability Support Office very helpful. However she feels that internal communication between faculties within the university could have been better. On one occasion the lifts, which Joanna relies on, broke down. Rather than Joanna being informed, she found herself having to let everyone know her support worker, the disability office, security etc.. Improved internal communications could have saved her a lot of time and unnecessary difficulties.

Joanna is very independent and likes to do things herself. Although this has helped Joanna succeed, she is also aware that sometimes she may be missing out on support that could be beneficial (e.g. making use of the subject librarian). SUPPORT WORKERS PERSPECTIVE Joanna has a support worker (SW). SWs role involves helping Joanna throughout her academic day. This involves such things as helping her to get from place to place or finding relevant books in the library. SW has developed a close working relationship with Joanna and believes Joannas strong independent streak has developed from the way she has always had to fight to be accepted into mainstream education. With respect to learning and teaching issues, SW echoes many of the points made by Joanna. In addition to these she identifies some further difficulties Joanna experiences. For instance, as well as not being able to browse books in the library, Joanna initially avoided the library completely because the difficulty she experienced in getting through the turnstile made her feel very conspicuous. SW also feels that Joanna would have benefited from more support from academic staff. SW and Joanna are not aware that Joanna has a personal tutor which means Joanna has had to make difficult decisions about choosing modules involving numerical work without the support and advice of her academic tutors. As a result Joanna has tended to avoid such modules, arguably at a detriment to her education. In addition, the lack of a personal tutor means Joanna has had to convey all information regarding her disability to a number of individual departments, which Joanna and the SW have found very frustrating. SW also thinks Joanna needs more understanding from lecturers. For instance, in class discussions Joanna remains silent because she doesnt like to stand out. With better understanding, lecturers would try harder to draw Joanna in. In terms of assessment, SW believes more joined-up thinking between course leaders is needed. Joannas experience of exams in relation to this has been variable. Sometimes, the preparation is excellent, Joanna is brought into the room a few minutes beforehand with clear access to a strategically allocated desk and with consultation as to whether she is comfortable. While at other times, there has been no preparation at all, initially Joanna is left at the back of the queue outside and then has great difficulty accessing narrow aisles and getting passed settled students to find a vacant place. Attention is drawn to Joanna and the SW when they have to ask for a more strategically appropriate place. Students have to move and the problem is solved but unnecessary and unwelcome attention is drawn to Joanna and the SW when the examination itself is stressful enough for the both of them. SW and Joanna agree that having a dedicated centre, where disabled students can take their exams, may work. However, this would require arrangements to be discussed with disabled students in advance. Finally, SW believes Joanna has missed out on the social side of Higher Education because of her disability. For instance the Student Union bar is up a flight of stairs which means Joanna can not meet people there for a drink and a chat and get to know other students better. Also, if

outside seminars and meetings are arranged, the venues are often inaccessible for wheelchair users. So in these respects, Joanna is prevented from enjoying the wider HE experience.

CONCLUSIONS / RECOMMENDATIONS A number of good practice points emerge from this case study. Tutors with students who experience mobility difficulties might wish to consider the following: Do not assume that all students with mobility difficulties have the same needs. Do not assume that if a students disability is obvious (e.g. they use a wheelchair) that he / she may not be disabled in other ways. He / she may have another unseen disability that impacts on their study Make time to talk to disabled students in private to find out how the disability affects them, and what sort of support they may need When speaking to prospective disabled students, make sure they are also in touch with the university disability office. By contacting the disability office early, Joanna was able to ensure her study support needs were met right from the beginning If arranging an out of hours seminar, make sure it is held in an accessible venue Be aware that disabled students may have more difficulty in forming social networks. Try to ensure students take part in group work / class discussions so that they get to know their fellow students. Talk with other lecturers or course leaders: it may be that a uniform strategy can be put in place for a student, which both serves to meet their needs, as well as helping individual academics with the responsibility for teaching. A simple process, along the lines of emailing actions required by academics involved in teaching a particular disabled student prior to the term, on key issues, could provide useful guidance. In terms of more specific learning and teaching issues, academic tutors might consider the following actions: Hand out module guides at the start of each module. Detailing weekly activities will help disabled students to plan ahead e.g. gets books in advance / arrange support etc. Hand out notes to accompany lectures. This means students will be able to concentrate on the subject in hand rather than desperately trying to keep up Be aware that some disabled students are lacking in confidence and do not like to draw attention to themselves. Try to ensure they are always drawn into class discussions When planning group work / meetings with students, consider accessibility / location of class rooms / offices If asking students to carry out research in the library, be aware that some students with mobility difficulties may not be able to browse the shelves. Therefore make sure that students are made aware of resources available to help them e.g. subject librarian, electronic library catalogue Be aware that it can take disabled students longer to do coursework than non-disabled students. Give students assignments well in advance of the final deadline to allow them sufficient time to plan and carry out the work needed

 When it comes to exam time, make sure disabled students are involved with discussions about exam facilities and arrangements. Ensure all course tutors are aware of the disabled students exam requirements so that the provision of support can be consistent from module exam to module exam. A certain amount of sensitivity may be needed when doing group work. Leaving students to split up into their own groups may leave certain disabled students, lacking social confidence, in a vulnerable position. Overcoming this issue is admittedly not easy, and there are no straightforward answers, but it is something which you may wish to pre-empt in classroom and group work situations. You may choose, for example, to put students into groups yourself using a method that does not single out the disabled student (e.g. go around the class allocating students numbers from one to four to create four groups etc. The method splits up cliques, while offering no special treatment to the disabled student, nor does it bring any particular attention to the disabled student. This is in contrast to splitting groups by their location in the classroom, which maintains cliques, or allowing students to choose their own groups.)

In the final analysis, this case study highlights the need for tutors to form trusting working relationships with disabled students. Even though some of Joannas difficulties were caused by her non disclosure of hydrocephalus, a supportive tutor would have enabled her to make better decisions about overcoming the difficulties in a proactive, supportive environment. There would also have been benefits for the tutor in terms of increased understanding and awareness of issues faced by students with mobility difficulties, which potentially could benefit future disabled students. Case Study found: here More Case studies DART can be found: here

References: Centers for Disease Control and Prevention. (2010). Data and statistics. Available online at: http://www.cdc.gov/ncbddd/spinabifida/data.html Coakley, R. M.; Holmbeck, G. N., & Bryant, F. B. (2006). "Constructing a prospective model of psychosocial adaptation in young adolescents with spina bifida: An application of optimal data analysis". Journal of Pediatric Psychology 31 (10): 10841099. Devine, K. A.; Gayes, L., Purnell, J., & Holmbeck, G. N. (in press). "Close friendships of children and adolescents with spina bifida: Reciprocity and social adjustment". Journal of Pediatric Psychology. Holmbeck, G. N.; DeLucia, C., Essner, B., Kelly, L., Zebracki, K., Friedman, D., & Jandasek, B. (2010). "Trajectories of psychosocial adjustment in adolescents with spina bifida: A 6year, four-wave longitudinal follow-up". Journal of Consulting and Clinical Psychology 78 (4): 511525. Holmes LB (1988). "Does taking vitamins at the time of conception prevent neural tube defects?". JAMA 260 (21): 3181. Iwamoto J, Abe H, Tsukimura Y, Wakano K (2004). "Relationship between radiographic abnormalities of lumbar spine and incidence of low back pain in high school and college football players: a prospective study". The American journal of sports medicine 32 (3): 7816. Kinsman SL, Johnston MV. Congenital anomalies of the central nervous system. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 592. March of Dimes. (2009, August). Spina bifida. Available online at: www.marchofdimes.com/ birthdefects_spinabifida.html NICHCY 1825 Connecticut Avenue N.W. Washington, DC 2009 Available online: here. Retrieved 12 March 2012. Rowley, L. (2007). Welcoming babies with spina bifida: A message of hope & support for new & expectant parents. Available online at the Children and Adults with Spina Bifida and Hydrocephalus website: http://www.waisman.wisc.edu/~rowley/sb-kids/wbwsb.html "Spina Bifida Occulta". SBA. Retrieved 10 March 2012. Steinberg EL, Luger E, Arbel R, Menachem A, Dekel S (2003). "A comparative roentgenographic analysis of the lumbar spine in male army recruits with and without lower back pain". Clinical radiology 58 (12): 9859

By: Ruth Merceron EDS 543