Treatment of spina bifida

•October 16, 2008 • Leave a Comment

Treatment for spina bifida depends on the severity of the condition.

  • Most people with spina bifida occulta require no treatment at all.
  • Children with meningocele typically require surgical removal of the cyst and survive with little, if any, disability.
  • Children with myelomeningocele, however, require complex and often lifelong treatment and assistance. Almost all of them survive with appropriate treatment starting soon after birth. Their quality of life depends at least partially on the speed, efficiency, and comprehensiveness with which that treatment is provided.

A child born with myelomeningocele requires specialty care.

  • The child should be transferred immediately to a center where newborn surgery can be performed.
  • Treatment with antibiotics is started as soon as the myelomeningocele is recognized; this prevents infection of the spinal cord, which can be fatal.
  • The operation involves closing the opening in the spinal cord and covering the cord with muscles and skin taken from either side of the back. The most common complications are tethered spinal cord and hydrocephalus, which can have very severe consequences.

Each person with severe spina bifida requires intensive and complex care by a trained and coordinated team.

  • The care team includes one or more pediatricians, neurologists, neurosurgeons, orthopedic surgeons, physical medicine specialists, endocrinologists, urologists, physical therapists, orthotics specialists, occupational therapists, psychologists, nurses, dietitians, social workers, and other professionals.
  • If at all possible, the individual with spina bifida should receive care at a specialized multidisciplinary spina bifida clinic where all necessary services, which are extensive, can be provided in a coordinated and convenient manner.

There is no cure for spina bifida. The goal of treatment for spina bifida is to allow the individual to achieve the highest possible level of function and independence. Treatment should address any disability, physical, emotional, or educational, that interferes with that person’s potential

 

The focus of treatment is developing strength, mobility, and independence. Many of these children will walk. For others, accessibility is the goal.

  • Parents should work with a physical therapist to learn how to exercise the baby’s legs to maximize strength and movement. They should begin these exercises as soon after the first surgery as possible. This not only readies the child for walking, but also prevents osteoporosis due to disuse.
  • Children with spina bifida should be provided with prolonged physical therapy, physical education, or adaptive training while in school.
  • Many children can become mobile by wearing a brace or using crutches or an orthotic. These devices allow the child to function at the best possible level by helping with balance, posture, and control. 
  • Additional operations may be necessary to correct problems interfering with walking and other functions.
  • Despite this assistance, some children with spina bifida will never be able to walk independently. These children will use a wheelchair for the rest of their lives.

 

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hydrocephalus and ventriculoperitoneal shunt

•October 16, 2008 • Leave a Comment

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Hydrocephalus is an excessive volume of CSF within the cranium – importance of this is that most babies born with spina bifida have hydrocephalus. If hydrocephalus is left untreated and continues to progress it will lead to compression and subsequent damage to developing brain.

A ventriculoperitoneal shunt is a device which is inserted to drain excess CSF from ventricles into peritoneal cavity preventing pressure on the brain

There are several different types of shunts that can be used to drain the fluid to different parts of the body, but the ventriculoperitoneal shunt, which drains the fluid into the peritoneal cavity is the most common shunt used. It involves the placement of a ventricular catheter (a tube made of silastic), into the cerebral ventricles to bypass the flow obstruction/malfunctioning arachnoidal granulations and drain the excess fluid into the peritoneal cavity, from where it can be resorbed.

Alternative shunts include those that drain to the right atrium (ventriculo-atrial shunt), pleural cavity (ventriculo-pleural shunt), and gallbladder. A shunt system can also be placed in the lumbar space of the spine and have the CSF redirected to the peritoneal cavity (LP Shunt).

Prognosis of SB

•October 16, 2008 • Leave a Comment

The prognosis for people with SB varies, but depends mainly on the severity of the condition (and the associated complications). Prognosis is obviously better for patients with occulta (the mildest form of SB), and worse for patients with myelomeningocele.

The children with poor prognosis ar those who are completely paralysed, have serious infection of the spinal cord, have hyrocephalus or other birth defects.

Level of the lesion also affects prognosis, for obvious reasons, Long-term survival is dependent on adherence to appropriate bowel and bladder regimens and careful management of urinary complications to prevent renal failure.

With proper care most children with SB live well into adulthood, also improved surgical procedures have improved prognosis – e.g. (experimental, i think) prenatal fetal surgery which involves sewing up/closing the shunt/opening in the fetus spinal cord has been associated with a decreased risk for Chiari II (whereby the brain stem and cerebellum protude in the spinal canal) – hence less/no hydrocephalus.

According to http://www.patient.co.uk/showdoc/40025296/ :

In a UK-based survey of Spina Bifida patients born between 1963 and 1971 and surveyed 20 years later:

  • 50% had died (largely the most severely disabled)
  • 84% of survivors required CSF shunting
  • 70% had an IQ>80
  • 37% lived independently in the community
  • 39% drove a car
  • 30% could walk >50 m
  • 26% in open employment
  • One-third needed daily care
  • A minority of patients had severe dependency/disability – need for respiratory support, blindness and dialysis-dependence
  • Those who had a need for CSF-shunt revision had worse outcomes in terms of attainment and independence

Psychosocial – Rural

•October 16, 2008 • Leave a Comment

Although Bright has a small hospital, they do not have any specific paediatricians, let alone spina bifida specialists.  Bright is situated over an hour away from the two nearest regional centres (Albury-Wodonga (110kms), or Wangaratta (80kms)) or 3 ½ hours from Melbourne, of which a substantial amount of time is spent on poor quality country roads.

 

With a population of only 2200 residents, it is unlikely there would many cases of spina bifida in the history of the town.  Therefore it is reasonable to assume that it is a medical problem poorly understood by the majority, especially the school age children Janet would have to mix with.  Although Bright College (prep to year 12) is fitted with good quality disabled facilities, the psychological support offered to Janet would be limited.

 

In considering this information, what follows is a quick summary of issues Janet might be facing:

          Inability to access immediate, good quality emergency health services

          Long drive times to access regular check-ups with specialists: this could put a strain on the family budget with petrol costs, and her mum requiring to take time off work

          Extra time driving on country roads, which is a significant risk factor for car accidents, especially in the winter months (icy roads/snow)

          Prejudice from the community

          Limited access to school programs for students with disabilities (because there wouldn’t be the numbers to secure funding)

 

However, the nature of Bright’s small town mentality means that Janet’s family would be quite likely to receive extra support from community members – an unofficial system which doesn’t operate in the city.

teenage girls with spina bifida

•October 15, 2008 • Leave a Comment

Spina Bifida and being a 13 year old teenage girl

 

Effects of Spina Bifida on menstration

Precocioius puberty – people with spina bifida often experience precoscious puberty, the exact reason why being unknown.  The average age of menarche in the U.S. is 12.5 years old.  For girls with spina bifida the average age is 1 to 1.5 years earlier.

 

Social Effects

People with spina bifida may need a wheelchair or braces and might have trouble urinating and deficating.  A combination of these three would be hard for a 13 year old girl.  Having sleep overs, attending high school and playing school sport, socialising with friends, dancing to Miley Cyrus and meeting boys could possibly be hampered.  However in today’s world these conditions can be managed and schools have become a lot more accessible to people in wheelchairs

 

Sex

There is no reason why people with spina bifida can’t have sex.  However non latex condoms should be used as roughly three quarters of people with spina bifida have an allergy to latex.  Also some people with spina bifida might not be able to experience orgasms easily or as euphoric as people without spina bifida. 

 

Josh

 

Sensory and mobility complications of L5 lesion

•October 15, 2008 • Leave a Comment

By Ludo

Paralysis and loss of sensation
In the case of myelomeningocele the spinal cord protrudes from the spine throughout pregnancy and is closed by surgery shortly after birth. As a result the spinal cord is almost invariably damaged and defective, the nerves being disorganised and broken. As a result messages from the brain (controlling and initiating movement) and those from the body to the brain (giving the sensations of touch, pain and position) are impaired. However, paralysis, muscle weakness and loss of sensation occur only at the level of the lesion and downward. Due to lack of skin sensation and decreased mobility, skin tissue breakdown may occur resulting in pressure sores. In most cases the head, upper trunk and arms are not affected. The level of impairment varies considerably depending on the amount of damage and the location of the lesion.

Movement & gait

The extent of the lesion provides a good estimate of the extent of lower limb functioning.
Of course, every individual is different, and each person with SBH is affected slightly differently.
Because nerves are affected at and below the point at which malformation occurs, the higher up the spine it occurs, the greater the paralysis (immobility) of the lower limbs will be.
Most people with SBH will need leg braces or more help to walk. Braces can help to support and protect weak muscles or joints.
Sometimes people will use braces or crutches for short distances, and use a wheelchair when they are more likely to get tired, or to leave their hands free.
Most individuals will need one or more orthopaedic (bone or joint or soft tissue) operations over the course of their life to assist in maximising function.
The level of paralysis should not change as the person with SBH gets older. A range of problems, however, commonly occur throughout life. These include:

scoliosis (spinal curving due to muscle imbalances) which requires surgery.

tethered cord (the scar tissue where the lesion has been repaired “sticks”, not allowing the spinal cord to move) which causes a range of problems and also requires surgery.

T12 & above With braces, can only walk short distances; with walker or crutches, slightly longer. Will mostly use a wheelchair, even in childhood.
L1 – L3 Leg braces with a waistband; will use crutches. Wheelchair for distances
L4 Will usually need braces, perhaps above the knee; crutches or cane; wheelchairs when older
L5 – S Short leg braces; may need crutches or cane.

Causes and Incidence of Spina Bifida

•October 14, 2008 • Leave a Comment

Spina bifida is the most common neural tube defect. Its incidence varies with population and races, but is estimated at 1-2 in 1000 live births.

There are four types of spina bifida:   occulta, closed neural tube defects, meningocele, and myelomeningocele.

Occulta is the mildest and most common form in which one or more vertebrae are malformed.  The name “occulta,” which means “hidden,” indicates that the malformation, or opening in the spine, is covered by a layer of skin.  This form of spina bifida rarely causes disability or symptoms.

Closed neural tube defects make up the second type of spina bifida.  This form consists of a diverse group of spinal defects in which the spinal cord is marked by a malformation of fat, bone, or membranes.  In some patients there are few or no symptoms; in others the malformation causes incomplete paralysis with urinary and bowel dysfunction.

In the third type, meningocele, the meninges protrude from the spinal opening, and the malformation may or may not be covered by a layer of skin.   Some patients with meningocele may have few or no symptoms while others may experience symptoms similar to closed neural tube defects.

Myelomeningocele, the fourth form, is the most severe and occurs when the spinal cord is exposed through the opening in the spine, resulting in partial or complete paralysis of the parts of the body below the spinal opening.    The paralysis may be so severe that the affected individual is unable to walk and may have urinary and bowel dysfunction.

Spina Bifida Causes

Both genetic factors (heredity) and environmental factors, such as nutrition and exposure to harmful substances, probably contribute to spina bifida. Spina bifida does seem to run in families, although with mixed patterns of inheritance. Having a child with spina bifida increases the chance that another child will also have spina bifida by 8 times. In about 95% of cases of spina bifida, however, there is no family history of neural tube defects.

Research has suggested that many cases of spina bifida can be prevented by adequate intake of folic acid (folate) before and during early pregnancy. However, people with spina bifida appear to have abnormal metabolism of folic acid. This suggests that the underlying problem in spina bifida may be an inborn defect in folic acid metabolism rather than a simple deficiency in this nutrient. However. it is recommended that all women of childbearing age take folic acid supplements to prevent the occurence of spina bifida.

Sources

http://www.emedicinehealth.com/spina_bifida/page2_em.htm#Spina%20Bifida%20Causes

http://www.ninds.nih.gov/disorders/spina_bifida/detail_spina_bifida.htm

By Rachel