Adolescence is a time of biological development, as well as significant cognitive, psychological and social change.{cs13-fn1}{cs13-fn2} It is a time of transition that prepares each young person for adult life, and involves completing the following developmental tasks:{cs13-fn2}{cs13-fn3}
- Developing a self-identity (including values, personality and sexual identity)
- Reducing physical, emotional and financial dependence on parents and caregivers
- Developing social relationships (with peers and with the opposite sex) through appropriate behaviour and communication skills
- Acquiring a profession
- Engaging in hobbies and other significant ways of spending time
In the process of completing these developmental tasks, peer relationships become increasingly important as the teenager attempts to find a sense of belonging and an identity beyond the immediate family, while the direct role of the family in shaping the person's life is significantly reduced.{cs13-fn2}{cs13-fn4} In addition, teenagers face many challenges that accompany these developmental tasks.
Being a Teenager with Spina Bifida
The challenges that all teenagers face are often intensified when the teenager has a disability such as spina bifida (see box 1). This can also include body image concerns, low self-esteem, social isolation, and stigma.{cs13-fn2}{cs13-fn4}{cs13-fn5} Moreover, parents and caregivers are confronted with new adolescence-associated aspects of living with a person with spina bifida. Due to advances in medical care and better management of the condition, the life expectancy of persons with spina bifida have greatly increased i.e. over 85% of children born with spina bifida survive into adulthood.{cs13-fn3}{cs13-fn5} Given this, it is important to address the issues persons with spina bifida and their families face in the transition from childhood to adulthood.
Box 1 | Spina Bifida
Spina bifida is a congenital condition resulting from an incomplete closure of the neural tube within the first 28 days of pregnancy, causing malformations of the spine. The abnormal development of the neural tube is most often accompanied by damage to the nerves and spinal cord. While the exact cause of spina bifida is still unknown, heredity, nutrition (notably insufficient intake of folic acid) and environmental factors are thought to play a role. Although spina bifida can be surgically treated at birth, the damage to the nervous system is permanent and there is no cure at present.{cs13-fn6}{cs13-fn7}
Spina bifida is the most common congenital condition affecting the central nervous system. According to the International Perspectives on Spinal Cord Injury (IPSCI), there are an estimated 4.5 cases of spina bifida per 10,000 live births worldwide, with some variations in country incidence rates due to different data sources used.{cs13-fn8} According to the International Federation of Spina Bifida and Hydrocephalus, neural tube defects affect approximately 320,000 newborns a year.{cs13-fn9}
Types of Spina Bifida{cs13-fn6}{cs13-fn7}
- Spina bifida occulta: a mild, symptom-free form of spina bifida, in which the spinal cord remains in the body with abnormally developed lower back bones.
- Meningocele: a moderate form of spina bifida, in which a small cerebrospinal fluid (CSF)-filled cyst (or sac) protrudes from the spinal canal and is visible on the person's back, often covered by a thin layer of skin.
- Myelomeningocele: the most common and severe form of spina bifida, in which the CSF-filled cyst also contains parts of the spinal cord and nerves that develop outside of the body. This type of spina bifida is often accompanied with hydrocephalus, an abnormal build-up of CSF in the brain causing increased pressure within the skull and an enlargement of the skull.
When the term “spina bifida” is used, it most often refers to myelomeningocele rather than the other types of spina bifida. Myelomeningocele results in a range of impairments in body functions and structures including some degree of paralysis, loss of sensation and muscle weakness in the lower extremities (hip, legs and feet), and bowel and bladder control problems. Persons with spina bifida often experience limitations in executing activities involving the lower extremities e.g. mobility and balance, often requiring assistive devices. The extent of problems a person experiences varies depending on the location and level of damage. For example, persons with damage high on the spine may have more extensive paralysis and require a wheelchair, while others with damage lower on the spine may need to only use crutches or leg braces.{cs13-fn6}{cs13-fn7}
In addition to hydrocephalus, spina bifida may also cause other abnormalities of the brain. For example, most persons with myelomeningocele have Chiari II malformations, an abnormal displacement of the lower part of the brain – much lower than normal – into the upper part of the spinal canal. This part of the brain is responsible for sensory input and controls, coordination and motor control, including maintaining balance. Symptoms include headache, upper body weakness, sensory disturbance, shooting pain, double vision and other visual disturbances, swallowing difficulties, speech problems, as well as poor coordination when walking. Persons with spina bifida have normal intelligence, but may encounter learning disabilities.{cs13-fn6}
""Navigating the critical developmental stages and successfully acquiring the necessary skills to enter adulthood may prove problematic.""
For teenagers with spina bifida navigating the critical developmental stages and successfully acquiring the necessary skills to enter adulthood may prove problematic. Indeed, beyond the physical and cognitive effects of the condition lay an array of psychosocial and participation issues as well as issues related to education that confront teenagers with spina bifida. Young people often compare themselves with other peers. Teenagers with spina bifida have reported seeing themselves different from their non-disabled peers, contributing to the difficulties they experience in forming friendships with peers without disabilities. Incontinence and being identified with the wheelchair also contributed to altered body image and social isolation.{cs13-fn2}{cs13-fn3}{cs13-fn4}{cs13-fn5}{cs13-fn10} Teenagers with spina bifida have even reported experiencing teasing by peers in a qualitative study on the transition of young adults with spina bifida.{cs13-fn5}
Participation is also often hindered by environmental barriers – inaccessible streets, public transportation, and meeting locations such as sporting facilities or places where teenagers often meet.{cs13-fn2}{cs13-fn4}{cs13-fn5}{cs13-fn10}
Implications for the Family
The transition of children with spina bifida into young adulthood has implications for the family as well. For parents with teenagers with disabilities like spina bifida, this transition period is often a time of greater stress. While teenagers without disabilities generally become more independent and leave home at a certain point, teenagers with spina bifida often need continued care from parents, and almost half of them continue to live with their parents in adulthood. Providing care to teenage children becomes more physically burdensome – assistance with physical activities such as transferring, bathing, dressing, etc. gets more difficult as the child gets physically bigger and heavier. In addition, young adults with spina bifida often continue to be financially dependent on their parents due to difficulties in entering the job market.{cs13-fn4}{cs13-fn5}{cs13-fn10}
Alongside the increased stress parents feel during this transition period, conflict between parents and the teenager with spina bifida can arise as a result of overprotectiveness or when parents provide insufficient opportunities for the teenager to gain independence.{cs13-fn4}{cs13-fn5}{cs13-fn11}
Independence is a topic that becomes more prominent during adolescence. Another is sexuality. Parents of teenagers with spina bifida are less likely to discuss issues of sexuality with their children.{cs13-fn11}
Despite the stress experienced by parents and other family members as the child with spina bifida transitions into young adulthood, families have shown great resilience in tackling the challenges they face.{cs13-fn11}
""Despite the stress experienced by parents and other family members as the child with spina bifida transitions into young adulthood, families have shown great resilience in tackling the challenges they face.""
Implications for Healthcare Provision
As with parents, health professionals who have been caring for a patient with spina bifida since a young age also have to adjust to the changing needs of that child as he or she matures into adulthood. Parents and health professionals may have difficulty giving up a trusted relationship to transition the maturing teenager's healthcare to adult providers. In addition, a level of trust between the paediatric and adult healthcare provider is also essential for smooth transition into adult healthcare.{cs13-fn3}{cs13-fn6}{cs13-fn12}{cs13-fn13}
""Ideally, the paediatric and adult healthcare provider work together during this transitional period...""
Transition of care does not only mean transfer of patient and documentation to another facility. Ideally, the paediatric and adult healthcare provider work together during this transitional period, especially considering that adult providers have been slow in establishing healthcare protocols that are adapted to the special healthcare needs of persons with spina bifida. Given the complexity of healthcare provision of spina bifida, a multidisciplinary approach, including the establishment of multidisciplinary clinics, would be ideal for addressing the range of healthcare issues related to spina bifida.{cs13-fn3}{cs13-fn6}{cs13-fn12}{cs13-fn13}
In addition, enabling the teenager with spina bifida to take over more responsibility for managing his or her care e.g. for catheterisation, is also part of this healthcare transition.{cs13-fn5}{cs13-fn12}
This case study aims to illustrate some specific challenges to independence and peer relationships face by Gabi, a 15 year-old girl living with spina bifida, and show how these challenges were addressed in a comprehensive rehabilitation programme.