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SpEd

VIEWS: 6 PAGES: 7

									SpEd
Video Transcript
What is Spina Bifida

(Music)

Male Speaker: What is spina bifida.

Female Speaker: Spina bifida or Myelomeningocele is a defect of the neural tube. The neural tube is
the embryonic structure that develops into the brain and spinal cord. This defect in development
occurs during the first weeks of pregnancy and is present in approximately one - to - two babies per
one thousand born in the U.S.

In the most common form of spina bifida, a portion of the undeveloped spinal cord herniates through
the baby's back forming a myelomeningocele. Bone and muscle cannot grow around the incompletely
developed cord and the vertebrae in that area do not close. This creates a gap through which the
spinal cord herniates.

Some of these myelomeningocele sacks are covered with skin. In others, tissues and nerves are
exposed.

Male: The spina bifida or myelomeningocele as it is called, which is the opening in the back with the
exposed neural elements that's where the word myelo comes in, and meningocele means sack. This
lesion can occur anywhere in the spine, from the top to the bottom. When the spine closes,
embryonogically it closes very early in development. It closes from the center towards the ends, it
knits up. And when it fails to close, it may fail to close at the bottom end that would be a lumbar or
sacral myelomeningocele. If it fails to close at the top end, that actually would be something at the
base of the skull or in the back of the brain cavity called an anencephaly or ancephalocele.

The worse lesions we see are the ones that occur early in embryongolic development. And those
would be the thoracic or thoracial lumbar myelomeningocele which occur mid to low thoracic spine.
These lesions vary in size, and severity. The children that have just simply a sack in the back with no
nerve tissue in it have no neurological deficit, no paralysis but when there is a very significant defect
in the spine and actually the spinal cord is malformed and not properly closed, then the child will have
paralysis of the legs, the bows in bladder control, possibly sexual function from the level below.

Male Speaker: What causes spina bifida?

Female Speaker: We do not know the exact cause or causes of spina bifida. It may be due to a
combination of environmental and genetic factors.

Male: If there's a history of neural tube defects in a family, a mother may choose to come in and
have an Alpha Feto Protein Test form as a screening measure. Increased alpha feto protein may
indicate a neural tube defect.

Female Speaker: Current research focuses on the possible role of diet as one of the important
factors.

Male Speaker: Is it inherited?

Female Speaker: Spina bifida seems to run in families. But it is not possible to predict which families
will have a child with this problem. Spina bifida does not occur in a simple hereditary pattern.

If a couple has a child with spina bifida there chances of having another child with the same problem
will be about 1 in 50. There is a 1 or two per hundred chance of brothers or sisters having a child with
spina bifida.

Male Speaker: What is the treatment for spina bifida?

Male: The treatment for spina bifida is very complex. It starts usually with neurosurgical care.
When a child is born with an open spine, the neurosurgeons are called and a decision is made to repair
this wound. Because it is an open surgical wound caring a risk to the child of meningitis or infection in
the spinal fluid.

The initial correction is done very soon after birth. Usually within hours and certainly within a day or
two at the most. And it involves a team of surgeons at this institution composed of neurosurgeons and
plastic surgeons closing the open wound in the back, attempting to get tissues and skin coverage over
the open spinal cord. This is a mechanical procedure to close a deficit and does not necessarily
correct the neurological abnormality that the child is born with.

Male Speaker: Why is Hydrocephalus often a problem with spina bifida?

Male: The children that have spina bifida many times have a situation or condition called
hydrocephalus. And hydro means water and cephalous is brain. So that's the medical term for water
within the brain.

In the child with myelomeningocele approximately 70% of the child will have this associated
hydrocephalus. In which the fluid spaces will be very large, there's difficulty with circulation of the
spinal fluid and subsequent re-absorption. As the fluid space is enlarged, the head enlarges the
places where the bones come together, the sutures of the skull begin to split, and the child can
actually become very ill from increased pressure in the head.

Female Speaker: A specific problem causing hydrocephalus is the Arnold-Chiari Malformation. Which
is a herniation of some of the brain into the cervical spine.

Male: This is a side view of what's called a magnetic resonance scan to visualize the malformation
that we call the Arnold-Chiari Malformation.

Just for point of reference, these are the tubercle bodies’ tubes or the bones of the spine in the
cervical area. This is the spinal cord, the gray shadow here that has spinal fluid around it, the black
shadow on both sides.

Normally the part of the brain that we use for balance called the cerebellum stops in this area, at the
base of the skull. But in this child with the malformation called Arnold-Chiari, this finger of
cerebellum called a cerebral tonsil, actually extends down through the hole at the base of the skull,
impaling or impinging upon the spinal cord in this area.

Female Speaker: In an infant, these signs may include, difficulty breathing, noisy breathing, a weak
cry or even death. The Arnold-Chiari Malformation in the older child may cause slowly developing
stiffness of the arms.

MRI scans help to visualize the skull and neck areas and will provide critical information in the decision
to treat conservatively or with surgical intervention.

Surgery is often required for hydrocephalus. One end of special shunt tubing is placed in the baby's
head and the other end inserted into the abdominal area so that the fluid will drain. If this were not
done, the baby might have developed marked brain damage or even die.

Male: In the child with hydrocephalus, it’s important for us to establish some type of drainage. And
obviously drainage to the outside to a bag would not be permanent and would be fought with the risk
of infection. So the simple solution is something called a ventricle peritoneal shunt. And what that
means is that the fluid space in the brain, the ventricle is merely plumbed with some soft stylistic
tubing running underneath the skin to the free space of the abdomen or the peritoneal space. Usually
these shunts stay in the children for their life.

Female Speaker: The shunt may need to be replaced if there is infection, if the child out grows the
length of the tubing or if the shunt blocks up and stops functioning. Such a malfunction can be
detected by a CT scan. As a parent, you can watch for early signs of shunt malfunction. Signs to
watch for include, irritability, vomiting, enlargement of head with a possible bulging forehead, seizures
and rarely noisy breathing. In older children look for signs such as headaches, personality changes and
declining school performance.

Male Speaker: After these operations are done, what can we expect our baby to be able to do?

Female Speaker: The surgical operations cannot correct an abnormal spinal cord. All the spinal nerves
below the spina bifida defect are usually affected. Thus those nerves cannot transmit messages from
the brain to the muscles. This results in muscle weakness or paralysis of the muscles affected.

The nerves also cannot transmit messages to the brain from the skin. Resulting in lack of sensation to
pain. This lack of communication between brain and skin also results in a lack of sensation to heat and
cold. The areas of the body affected depend on the level at which the spina bifida occurred.

Male Speaker: What can be done for these problems?

Female Speaker: Usually a medical and surgical team work together to treat the problems that result
from spina bifida. Therapists then work with the patient to plan muscle exercises and other
therapies.

The lack of sensation can also result in infection from small unfelt cuts and burns. Lack of sensation
also makes the child prone to the development of pressure sores.
The team will advise parents in appropriate skin care to avoid such problems.

Male Speaker: Will my baby have normal intelligence and be able to go to school?

Female Speaker: You baby may have normal intelligence. About 70% of children with spina bifida have
a normal IQ. The remainder may show some degree of retardation.

The medical community now recognizes that children with spina bifida may have poor eye to hand
coordination. This decreased eye to hand coordination can be associated with poor fine motor control
and may be associated with a learning disability.

At the present time there are educational methods available for such specific learning problems.
Additional educational tools are currently being developed to meet the needs of the person with spina
bifida.

Male Speaker: Will my baby's vision and hearing be normal? Will they have seizures?

Female Speaker: Hearing is usually normal in children with spina bifida. However, vision may be
affected by a condition called strabismus. Strabismus has a tendency for a child to be cross eyed
and often can be corrected by an ophthalmologist.

Female: Strabismus means that the two eyes are looking at an object; they're not looking in the same
direction. They can either be pointing towards each other or one eye can be pointing out. And they
may alternate between the two.

One of the ways we can fix it, we can start out by trying patching therapy. If the vision is unequal it’s
important that the child has equal vision in both eyes. And if necessary it may even involve surgery to
correct the problem.

Female Speaker: Some children experience seizures as a result of brain damage. Seizures can often
be controlled with medication.

Male Speaker: Will my child be able to walk?

Female Speaker: This depends on the level of the spina bifida defect as well as the presence of other
abnormalities.

The physical therapist will implement a program of stretching and strengthening exercises designed to
improve the muscle tone. Orthopedic surgeons and physical medicine specialists can perform
procedures which can help your child sit, stand and move around more easily.

Since each child's condition is different, the treatment for your child will be individualized to fit his
or her needs.

Male: Generally if the neurological level is such that the child has strong quadriceps muscles, which
are the muscles that extend the knee, that child will usually be able to walk with short braces. If the
quadriceps muscles are not strong, then generally longer braces or a wheelchair will be necessary for
mobility.

If there are associated fixed deformities of the lower extremities, these often need physical therapy
bracing or surgery to correct these to help the child with walking or standing.

Male Speaker: What other orthopedic deformities might be present?

Male: In the lower extremity one of the most common foot deformities is a club foot deformity. The
child is often born with; this usually takes surgery to correct. There are other foot deformities that
can develop later on because of muscle and balance that may require therapy, bracing or surgery to
correct. There can be contractures of the knees or dislocation of the hips that often occur in spina
bifida. The hips often do not need to be put back in the socket for the child to be mobile and to walk.
Occasionally hip surgery is necessary if they are fixed deformities.

Female Speaker: Exercises, braces, splints or surgical procedures may be helpful for treatment or
prevention.
Male Speaker: What is scoliosis?

Female Speaker: Scoliosis is a sideways bending of the spine. It may be present at birth or develop
later in childhood. When trunk muscles are weak they fail to support the back adequately and cause
scoliosis.

Male: Scoliosis is a curvature of the spine towards the side and also a rotation of the spine in the
transverse or axial plane. So it’s a complex defamatory.
We think of it as a 6 degrees of a freedom of motion kind of defamatory.

Female Speaker: Another cause of scoliosis is tethering or binding of the spinal cord to other tissue
at the site of original repair of the back. This may cause stretching of the cord which weakens the
spinal muscles and causes more curving or bending of the spine.

Male Speaker: Can scoliosis be treated?

Female Speaker: Yes. Treatment may include bracing and or surgery. Tethering of the cord can
happen at any age but if detected surgery can be done to free the cord and stop the progression of
scoliosis.

Male: Well, de-tethering is hopeful and it’s been a very popular procedure in the last few years. But
it’s not a panacea, it’s not without risks, its not 100% successful. And there are even some
complications such as worsening of the conditions for which it’s done.

In general, it will tend to help spasticity and help urinary bladder functions and some times slow the
progression of scoliosis.

Female Speaker: Spinal surgery may also be required to correct Kyphosis more commonly known as
hunchback syndrome. Kyphosis occurs in 8-15% of patients with spina bifida.

Male Speaker: Why do children with spina bifida often have urinary tract infections?

Female Speaker: The reason for urinary tract infections in children with spina bifida stems back to
nerve damage. This nerve interruption prevents children from feeling when their bladder is full.
Another problem faced by children with spina bifida is the inability to urinate because they cannot
relax the sphincter to let the urine flow out. There may even be a combination of leaking urine and
incomplete bladder emptying.

Male: Well the important thing early in life is the follow them closely. Not all children develop
problems with significant infection difficulties with their bladder. But if they do, it’s important to
keep an eye on them and follow their bladders for the changes in pressure that can occur.

Female Speaker: If the bladder is not completely emptied, the urine may back up or accumulate in the
ureters and the kidneys. This backing up of urine is called Reflux. Reflux can cause damage to the
kidneys.

Male: Reflux as we mentioned a little while ago is important because the bladder in a normal child
tends to have a nice ob-ways shape here and not very irregular. This is a child with spina bifida who's
fairly young. She has a bladder that's fairly normal in a parents. Problems develop later as the
infection difficulties become more of a problem where there may be pressures that get higher in the
bladder and we can develop problems with more elongated bladders as we see here which can lead to
reflux of urine up into the kidneys.

With a systogram, which is what this picture is, where you put a catheter in the bladder and fill it
with some contrast. We should not see contrast running up into the ureters and the collecting
systems like we see here. In this case there's dilatation trotuicoity with the collecting system which
is abnormal and this is what is called as vesicle uritary reflux and can be a problem with infection and
the high pressures and cause kidney damage later in life.

So, the main thing is to watch these children very closely so we can hope to prevent this from
occurring by using medications to help lower the pressures in the bladder and putting them on a
technique of emptying the bladder with a catheterization schedule which helps keeps these pressures
down and also helps decrease the potential for severe infections.

Female Speaker: As your child matures, he or she will go through developmental phases just as any
child. However children with spina bifida tend to mature sexually about two years earlier than their
peers. Thus, they will be curious and concerned about their sexuality at an earlier age.

Some males may experience a problem in achieving an erection due to the nerve damage from spina
bifida. However, many males with spina bifida can function sexually and are fertile. Women with spina
bifida are able to have intercourse they usually are able to achieve orgasm as well as to bear children.

Male Speaker: Will my child have normal bowel control?

Female Speaker: As with the bladder, nerve damage may case the child with spina bifida to be
unaware of a full rectum.

Male: The great majority of children with spina bifida do not have normal bowel control. There is the
rare child who through biofeedback can get normal control. Most children with spina bifida will not
be able to sense when they're going to have a bowel movement and will not be able to prevent
themselves from having a bowel movement because they don't have control.

Female Speaker: A number of techniques are used to help manage bowel function and constipation.
These may include special diets, specific elimination schedules and possible medication.

Male: The majority of children, that involves putting them on fiber and sometimes other laxatives to
get stools to move through there. The other things that are commonly used are suppositories and
enemas which can allow kids to time their stools. So to get control over when they're going to have
those stools so they don't leak and have accidents at school or the social embarrassment of that. But
can put a suppository in or in some instances an enema and have that bowel movement before school or
have it after school or whatever time is going to work best in their schedule and doesn't make their
schedule work around their bowel movements.

Male Speaker: Will they have to be on a special diet?

Female: A child with spina bifida may have some specific nutritional needs that perhaps would best be
addressed with a help of a registered dietician. The common problems that we see in children with
spina bifida are obesity or overweight and constipation. What we would look for if the child has these
problems, if that their diet contained lots of food that are high in fiber in order to help with the
constipation and low calorie food choices. The foods that are high in fiber also tend to be low in
calories so we tend to have the same foods will help them both with those problems.
Is there a link between vitamins and spina bifida?

Male: It has been well shown that in Britain, Ireland where the incidence is much higher that the
administration of vitamins, especially B Complex and now with Folic Acid as a B vitamin, that mothers
of children who have had spina bifida in the family already or relatives with spina bifida, the incidence
that these mothers would take vitamins is much lower for having further spina bifida children. We
for the past several years have been giving spina bifida mothers, relatives with spina bifida in the
families, um multivitamins with folic acid to anyone who is contemplating a pregnancy. In recent
weeks, it’s come out in the news and the medical literature that probably that all mothers
contemplating a pregnancy and of course since we don't know when pregnancies are going to occur that
all fertile women should probably take a multivitamin with folic acid.

Female Speaker: First and foremost remember that your baby is a baby. In spite of his medical
needs he has the same non-medical needs as any other baby and will surely need cuddling, feeding,
diapering, bathing, soothing and most of all loving.

But also remember, he will not always be a baby. He will grow up and have a future. This future will
include both medical and non-medical concerns. It will include attending school, going through
adolescence, with its ups and downs, reaching adulthood and increased responsibility.

(Music)

(End of video)

								
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