 |
 |
 |
 |
||
 |
 |
 |
|
||
 |
|
||||
 |
 |
|
|||
 |
|
||||
 |
|
||||
| |
|
|
|
||
| |
|
|
|
||
| |
|
||||
| |
|
|
|||
| |
|
||||
| |
|
||||
Spastic Cerebral Palsy and Intrathecal Baclofen
As family of a primary school aged child with spastic cerebral palsy you undoubtedly have strong feelings about the various treatment recommendations and requirements that come from the world of practicing medicine. The intent of this paper is neither to tell you the ultimate cure for CP nor to make any claims about knowing more then those in the medical field; the intent is to inform you, the family, of a new option for management of spastic cerebral palsy. The term "treatment" will not be used because, although cerebral palsy is a non-progressive disability, neither does the severity of CP symptoms lessen. Thus "management," referring to keeping symptoms under control and as minimal as possible, is preferred and will be used instead.
The characteristics of cerebral palsy are as follows: Rosenbaum (2003) defines cerebral palsy as "an umbrella term covering a group of non-progressive, but often changing, motor impairment syndromes secondary to lesions or anomalies of the brain arising in the early stages of development ." Basically, he is saying that cerebral palsy refers to a group of disabilities that will not self-correct, which affect children while very young, and that disrupt the child's movement ability in connection with brain function. Cerebral refers to the brain and palsy refers to "any disorder that impairs control of body movement"(United Cerebral Palsy, 2001). The parts of the brain that may be affected are those that control verbal ability, muscle contraction or expansion, and involuntary movement. The effect on the brain frequently causes symptoms such as trouble swallowing, difficulties with fine motor tasks (such as writing or buttoning a shirt), and problems with balance. Each case of spastic cerebral palsy is quite different from any other. Individuals with spastic cerebral palsy may only have slight imbalance problems whereas others may need a wheelchair and support for eating (Cruickshank, 1955). To read about the different types of cerebral palsy see Appendix A.
Just as there are numerous types and kinds of cerebral palsy, there are also many methods physicians use for management. Depending on severity and type, management can include therapy, drug prescription, surgery, and the use of mechanical aids (National Institute of Neurological Disorders and Stroke, 2005).
Therapy can take many forms. Children with cerebral palsy may need physical, speech, occupational, or behavioral therapy. Physical therapy may include chronologically mirroring learning goals to motor skills learned by typically developing children (i.e. crawling before walking etc.), and working with the child or adult to develop skills necessary for what they will face during life such as sitting, communicating, and controlling limbs as much as possible. Speech therapy may include challenging a student who struggles to say "b" words to master a list of simple words beginning with the letter b, then raising the difficulty level. Occupational therapy may include working with the individual on learning basic needs skills such as independently using the restroom, dressing or feeding oneself. Behavioral therapy typically is paired with therapy listed above and may include related rewards such as giving the individual a balloon for mastering his/her "b" words. There are many other examples of the above types of therapy. Check with your family physician, school therapist and/or IEP team for more information.
Drug therapy is one form of management that is constantly evolving as the medical world creates and produces new types. According to the NINDS article, the three most common medications prescribed for children with spasticity are diezepam, baclofen and dantrolene. Diezepam works to relax the body and brain but may cause amnesia, drowsiness, fatigue, dizziness, behavior changes, confusion and or depression. Baclofen blocks messages that the spinal cord is sending to the brain that communicate "contract muscle." Baclofen may cause nausea/vomiting, headache, low blood pressure, constipation, hypotonia (muscle weakness) and difficulty urinating. The final most popular type of medication prescribed to individuals with spastic cerebral palsy is dantrolene. Dantrolene interferes with the process of muscle contraction by blocking calcium channels within the muscle (Kernagis, 1999, National Institute of Neurological Disorders and Stroke, 2005).
Surgery can come in a few forms depending on the need. During normal development running and jumping cause the muscles and tendons to stretch and grow in proportion with the bones. However, if a child has spastic cerebral palsy during this period of development, jumping and running are not as common. Frequently this results in children developing contractures. What happens with a contracture is the tendons and muscles do not grow as fast as the bones to which they are attached thus causing balance and postural abnormalities. This condition can be surgically remedied by lengthening the muscles but requires long recovery.
Mechanical aids include anything from basic (Velcro shoes) to complex (speech synthesizer). Essentially, anything that aids the student or adult in performing basic functions. Most recently, the addition of computer devices to the list of mechanical aid resources had helped many individuals overcome limitations. (Kernagis, 1999).
Looking at the partial list of options for management of spastic cerebral palsy listed above can be overwhelming. Now, on top of it all I am going to introduce you to one more. This last method is a hybrid of medicine and surgery and is referred to as intrathecal baclofen (in-truh-thee-kul back-low-fen) therapy (ITB). The surgery consists of implanting a miniature pump into the "intrathecal space" on the body (see Appendix C for images of where the pump is embedded) which delivers doses of the medicine baclofen at programmable intervals directly into the spinal cord.
Prior to this method the medicine baclofen was orally ingested in the form of a pill. Because baclofen must reach the spinal cord to be effective, patients were required to ingest significant amounts of the medication to compensate for the levels that eroded during transport within the body. A benefit of ITB is that only the minimum amount of medicine needs to be injected to still reap the maximum advantage.
There is much research being done and already completed regarding intrathecal baclofen therapy. A study completed by the researchers Gilmartin, Bruce, Storrs and Abbott looked at the effectiveness of intrathecal baclofen therapy on individuals with spastic cerebral palsy. With approval from the Food and Drug Adminstration, Gilmartin et al. assessed two groups of patients diagnosed with spastic cerebral palsy, one in a placebo group and the other in a control group, for over three years.
The entire study consisted of fifty-one patients: 29 male and 22 female. Among these patients, forty-four responded positively and within these patients "clinically significant relief of spasticity in the lower extremities was demonstrated by a decrease in the average Ashworth Scale score" (Gilmartin, 2000, "Chronic Baclofen Infusion" section, para.1).
As could be expected there were some negative effects resulting from this study. Some side effects include nausea, headache, dizziness, vomiting, and increased salivation. These were all present at one point or another, but they were only present in around 2% of the participating patients (Gilmartin et al., 2000). Overall the study showed a reduction in spasticity among individuals with spastic cerebral palsy when the antispasticity medication baclofen is injected directly into their central nervous system (Gilmartin et al., 2000).
A study conducted over the course of 6 years by William Campbell, Anne Ferrel, John McLaughlin and Gerald Grant (2002) on the long-term safety and efficacy of continuous intrathecal baclofen turned up some additional information regarding its value and reliability. Campbell et al. began their study with a group of 21 children and adolescents. Of these 21, 19 were diagnosed as having spastic quadriplegia (see appendix A) and the remaining two were diagnosed with spastic diplegia (also, see appendix A). All patients had been and were using wheelchairs as their main mode of transportation. Before inserting a baclofen pump into anyone the researchers had to determine whether or not their patients would indeed benefit from the medication itself. Each patient was tested and each did benefit, so a baclofen pump was inserted into each individual (see appendix C for pictures of the pump) (Campbell, 2002).
Over course of the 80 patient-years of pump operation represented, the researchers found 238 adverse events (adverse refers to "any unpleasant symptom or medical problem arising during treatment" (Campbell, 2002)). Of those 238, only 153 were considered related to the treatment. 11 device related adverse events were related directly to the pump system and all of these required surgical correction. Some non-device related adverse events included constipation, decubitus ulcers (sores caused by prolonged pressure as in sitting too long), and in two cases, acute pancreatitis (intense but very short-term inflammation of the pancreas).
Because all subjects were children and adolescents, the study relied heavily upon caregivers. Caregivers were responsible for transporting the patients to and from the clinics every one to three months as their baclofen was up for refill and for participating in tele-questionnaires regarding their perception of patient benefits. At one point, all caregivers were interviewed about their overall satisfaction of the treatment. Sixteen of 17 caregivers stated that they "would go through it again", 14 affirmed that they would have the pump replaced if/when necessary (thus perpetuating their child's outcome) and 15 reported that they would recommend this therapy to others who may need it. In the conclusion, the authors report that intrathecal baclofen "is effective in causing a major reduction in spasticity of cerebral origin in children. The system itself is highly reliable" (Campbell, 2002).
To conclude, we have looked at four typical ways to manage spastic cerebral palsy: therapy, drug prescription, surgery, and the use of mechanical aids. We have also considered two studies supporting a hybrid management method fusing medicine and surgery called intrathecal baclofen therapy. There are many resources online as well that provide descriptions of the many aspects of ITB and cerebral palsy in general (see references). In my experience as a special educator I have seen this surgery make some amazing things possible for students with spastic cerebral palsy. I would highly recommend asking professionals in your area, at your school or in your community for resources if you are interested in looking further at the potential benefits this management technique can yield.
Types of Cerebral Palsy
There are four types of cerebral palsy: spastic (70-80% of individuals with CP), athetoid (10-20%), ataxic (5%) and mixed.
· Muscles that are rigid or permanently stiff distinguishes spastic cerebral palsy. SPC is additionally catalogued according to which limbs it affects:
· Hemiplegia involves an arm and leg on the same side of the body.
· Diplegia involves primarily the legs along with a relatively minor effect on one arm.
· Triplegia involves three limbs at the same severity level.
· Quadraplegia involves all four limbs and or the entire body.
· Unhindered sluggish contorted movements typically involving all four extremities characterize Athetoid cerebral palsy.
· Tendencies of ataxic cerebral palsy include difficulties with equilibrium and balance.
· Finally, the mixed type can include any combination of the above such as an individual who had increased muscle tone yet struggles with equilibrium(National Institute of Neurological Disorders and Stroke, 2005, Rosenbaum, 2003)
Location of placement of the baclofen pump:
The pump is placed just below the skin at the waistline. It is three inches in diameter and one inch thick and connects to a small catheter through which the baclofen is released. The pump is programmed to release medication at prescribed intervals and it can be adjusted through an external programmer. When refills are needed (every two to three months,) patients must return to their doctor. There a refill is injected through the skin and into the pump through via a syringe. After five to seven years the battery life ends and the pump must be surgically removed in order to replace the battery.
Images found at: http://www.medtronic.com
Ashworth Scale:
Grade 1 No increase in tone 2 Slight increase in tone, giving a "catch" when affected part is moved in flexion or extension 3 More marked increase in tone, but affected part easily flexed 4 Considerable increase in tone, passive movement difficult 5 Affected part rigid in flexion or extension
References
Books
Cruickshank, W. (Ed.). (1955). Cerebral palsy: a developmental disability. 3rd ed.Syracuse, NY: Syracuse University Press.
Thompson, G., Rubin, I., & Bilenker, R. (1983). Comprehensive management of cerebral palsy. New York: Grune & Stratton.
Journals
Campbell, W. M., Ferrel A., McLaughlin J. F., and Grant, G. A. (2002, Oct). Long-term safety and efficacy of continuous intrathecal baclofen. Developmental Medicine and Child Neurology, 44. Retrieved Feb 21, 2005, from ProQuest database (237681501).
Disabato, J., & Ritchie, A. (2003, Jan-Mar). Intrathecal baclofen for the treatment of spasticity of cerebral origin. Journal for Specialists in Pediatric Nursing, 8. Retrieved Jan 15, 2005, from ProQuest database (334478021).
Gilmartin, R., Bruce D., Storrs B. B., and Abbott, R. (2000, Feb). Intrathecal baclofen for management of spastic cerebral palsy: multicenter trial. Journal of Child Neurology, 15. Retrieved Jan 14, 2005, from ProQuest database (49834591).
Gooch, J. L., Oberg W. A., Grams B., and Ward, L. A. (2004, Aug). Care provider assessment of intrathecal baclofen in children. Developmental Medicine and Child Neurology, 46. Retrieved Jan 14, 2005, from ProQuest database (677556191).
Hogan, S. E. (2004, fall). Energy requirements of children with cerebral palsy. Canadian Journal of Dietetic Practice and Research, 65. Retrieved Jan 14, 2005, from ProQuest database (701481291).
Rosenbaum, P. (2003, May 3). Cerebral palsy: what parents and doctors want to know. British Medical Journal, 326. Retrieved Jan 14, 2005, from ProQuest database (342590641).
Websites
Kernagis, L. (1999). Cerebral palsy - what is it?. Retrieved Feb. 17, 2005, from BRIGHT website: http://www.brightonline.org/id_5_1_CP_Overview.html.
National Institute of Neurological Disorders and Stroke. (2005, January). Cerebral Palsy: Hope Through Research. (Publication NO. 93-159). Retrieved January 14, 2005, from Office of Communications and Public Liaison: http://www.ninds.nih.gov/disorders/cerebral_palsy/detail_cerebral_palsy_pr.htm
United Cerebral Palsy, . (2001, October). Cerebral palsy - facts & figures. Retrieved Jan 17, 2005, from http://www.ucp.org/ucp_generaldoc.cfm/1/9/37/37-37/447.
Debra S. Schwulst is a student Western Washington University.
©April 2005 New Horizons for Learning
This article is in the public domain and can be freely copied and used in trainings as handouts at parent and community meetings, and in creating your school or district programs. (Please cite all sources of materials you use.)
This information is provided by:
Office of State Superintendent of Public Instruction
Special Education
P O Box 47200
Olympia, WA 98504-7200
(360) 725-6088
Fax (360)586-1631
E-mail: dgill@ospi.wednet.edu
You
are here: