Cerebral Palsy is a term that is used to describe a loss or impairment of motor function due to brain damage. This damage can happen before, during, or after birth, and the severity of the loss or impairment depends on the location in the brain. With Cerebral Palsy, a persons ability to control their own movements, balance, posture, and /or coordination is affected.
There
are three main classes of Cerebral Palsy; spastic, which is the
inability to control voluntary movement, athetoid which is the inability
to control involuntary movements and have purposeless movement, and
combination which is a mixture of both spastic and athetoid. Within the
classes, there are four descriptions of the class; monoplegia is where
the CP affects one arm, or one leg, diplegia is where both arms or both
legs are affected, hemiplegia is where half the body is affected, and
quadriplegia is where both arms and both legs are affected. Many clients
that have Cerebral Palsy also have a related disorder such as seizure
disorder, developmental delay, and vision, hearing, and speech
abnormalities.
Spina
Bifida is another diagnosis that is prominent at Matheny. Spina Bifida
is an abnormal formation of the neural tube during development in utero.
According to Merriam-Webster's Medical Dictionary (2013), the neural
tube is “the hollow longitudinal dorsal tube that is formed by infolding
and subsequent fusion of the opposite ectodermal folds in the
vertebrate embryo and gives rise to the brain and spinal cord”.
Essentially, the neural tube is like a garden hose, which can be
pinched. Anything below the pinch of the neural tube does not work
correctly and only a trickle of information from the brain reaches that
area. Fortunately, with current technology, doctors are able to go
inside the uterus and fix the abnormal formation. This has drastically
lowered the number of cases in the United States to 0.7 out of 1,000
live births.
There
are three types of spina bifida; occulta, meningocele, and
myelomeningocele. Occulta usually only affects one vertebrae near the
base of the vertebral column, where the neural tube does not close
correctly. Therefore, the plates of the vertebral arch can not fuse
together correctly which causes the “pouch”. If this happens low enough
down the vertebral column, the person will likely be asymptomatic. In
its more severe form, more than one vertebra fuse together. Again, the
individual may be asymptomatic for years, however they will likely
realize they have loss of some sensations in their lower extremities.
Meningocele
is the least common type of spina bifida. This is where the spinal cord
is fully intact and some of the vertebrates have spilt and their
meninges (cushions) are coming out through the openings. This type of
spina bifida can be detected in utero and utero surgery can take place.
The individual may suffer from bladder and/or bowel issues, some mental
problems, and may have difficulty using their lower extremities.
The
last type of spina bifida is called myelomeningocele and is the most
severe and most common form of spina bifida. Myelomeningocele “is when
the protective membrane of the spinal cord and the spinal cord itself
protrudes through the hole in the vertebral column” (Spina bifida
information sheet, 2012). When the spinal cord is completely exposed,
the risk of infection (meningitis) is very high, so directly after
birth, surgery is performed to correct the defect. People that are born
with myelomeningocele spina bifida usually have permanent physical
disabilities, and may suffer from developmental delays. Another side
effects of myelomeningocele is that the child may be born with Chiari II
malformation, “when the brainstem and cerebellum drop down into the
neck and spinal column” (Spina bifida information sheet, 2012). When
chiari II malformation is present, the spinal cord is being compacted
which will cause all biological functions to be abnormal such as;
breathing, balance, swallowing, and many other symptoms.
Lesch-Nyhan
Disease is an extremely rare X-linked disorder and a severe lack or
absence of the enzyme hypoxanthine-phosphoribosyltransferase (HPRT). The
lack of or absence of HPRT causes a very high production of uric acid
and renal dysfunction due to the build up of crystals in the kidneys.
Patients with LND also exhibit an uncontrollable desire to self
mutilate, and also suffer from neurological abnormalities that resemble
cerebral palsy. Since LND is a X-linked chromosome, it is found mostly
in males, and females are carriers. There have been less than a handful
of women with LND documented, but researchers believe this is due to an
extremely rare mutation during development.
LND
is considered a phenotype, “the external expression of a set of genes.”
(Anderson, 2013). LND is a phenotype behavior because patients have
these behaviors because of the missing enzyme HPRT, and those that don’t
have the enzyme HPRT show the behavior. At the age of two, patients
with LND will start to exhibit the self mutilating symptoms. This is in
the form of finger, and lip biting, throwing themselves, hitting their
arms, and heads against the wall. A way that is also self mutilating, is
the act of hurting those around them that they care most about. By
pushing those individuals away, they are making themselves isolated.
Even
though LND patients have this compulsion to self mutilate, they feel
pain the same as any healthy individual. They do not want to hurt
themselves, and worry about hurting themselves, or others around them. A
way to prevent the self mutilation, is to have these clients in safety
restraints. These restraints help to put the patients mind at ease, and
not worry about hurting themselves. Some other treatments to help these
patients deal with their everyday lives are; stress reduction, avoiding
disciplines and punishments, keeping the patient busy with different
activities, teeth extraction, and ignoring the behavior. When you ignore
the behavior, this is the LND behavior and not every day typical
behavior. These patients may sound scary, and just plain mean, however
Lowell T. Anderson stated it perfectly on her website saying, “It may be
more accurate to think of the patients as doing the opposite of what
they actually intend.” (Anderson, 2013). LND patients are very social,
humorous, cognitively aware of their surroundings, and tend to the needs
of others in their lives.
We
sometimes label patients by their diagnosis, and can not see past their
disabilities. Regardless of their diagnosis, or disabilities, patients
are people first, with personalities and some of the biggest hearts that
I have ever seen. Matheny is a place where children and adults can grow
and have the fullest life that can be offered.
Reference List
Anderson, L. (2013). In Lesch-nyhan disease support group. Retrieved from http://lndnet.ning.com
About cerebral palsy. (2013). Retrived from http://cerebralpalsy.org
Matheny, (2005). Overview of disabilities.
Matheny, (n.d.). Cerebral palsy
Matheny, (n.d.). Spina bifida
Matheny, (n.d.). Neuropsychology of spina bifida
Matheny, (n.d.). Lesch-nyhan disease
McDonald, Eugene, (1987). Treating cerebral palsy, ProEd, Inc.1-2
Neural tube. (n.d). In Merriam-Webster’s online dictionary (11th ed.). Retrieved from http://www.merriam-webster.com/dictionary/neural%20tube
Reck, J. (2001). Basic guidelines of caregivers on use of adaptive equipment and transfer skills, Lesch-nyhan disease. 1-5.
Retrieved from http://www.matheny.org
Spina bifida information sheet. (2012). Retrived from http://www.spinabifida.net/complications.html
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