The Nature of the Creutzfeldt-Jakob Disease

The Nature of the Creutzfeldt-Jakob Disease
Dementia is traditionally considered to be one of the possible results of aging. Its effects are heartbreaking and tremendously exhausting for the patient as well as their family and friends. There are many factors to consider. What would be the best treatment plan for my loved one? Who would care for them? These are all very difficult decisions that impact the patient and family for years to come. But what if you only had a few months to make these decisions? What if all your loved one had was a few very short months before they were gone? In Creutzfeldt-Jakob Disease (cjd), the situation is just that. With no known cause or cure, cjd takes the patient and family completely by surprise. Time is precious when you see new changes arising day after day in areas such as movement, speech and cognition. How could you make them as comfortable as posssible? What could have caused these sudden changes? What treatment options do you have in such a short period of time? Most importantly will my loved one be able to express their needs to me?
Creutzfeldt-Jakob is considered to be a transmissible dementia or transmissible spongiform encephalopathy, which means that the spread of the disease is similar to that of viruses. Further studies have shown that instead of a virus, Creutzfeldt-Jakob Disease or cjd, is considered to be caused by a proteinaceous infection agent or ?prion? (Asher et al, 2000) that attacks nerve cells causing vacuolization, the formation of holes in the cell, and eventually cell death. To date there are no effective screening procedures or medications for early diagnosis or treatment (Henderson, 2000). Like other dementias, true diagnosis can only be made during autopsy.

cjd can be diagnosed in any age group but primarily within the 50-70 year old population (Asher et al, 2000). The prevalence of the disease is estimated as effecting 0.5 to 1.5 per million people (Asher at el, 2000). In the earliest stages there is a noticable change in sleeping, eating, and cognition. Signs of depression and confusion are also present in the early stages. Many patients may seek psychiatric help in this early stage while they are aware. Like Alzheimer?s, memory loss is an initial symptom. As the disease progresses, problems in motor movement such as tremor and extrapyramidal symptoms such as aphasia, and disarthria are noted (Ferrand & Bloom, 1997). The patient deteriorates quickly after the neurological symptoms are discovered, and life expectancy ranges from 9 months to a year. Near the end of a patient?s life, a condition known as akinetic mutism occurs. Akinetic mutism (Pocchiari, 1998) is a motivation disorder where patients are aware of their surroundings but lack the initiative to move, or speak. Akinetic mutism is often found in the last stages of all dementias.

cjd can be contracted a number of ways. In all findings of cjd, the brain appears spongelike with diffuse lessions in cortical and subcortical areas. A current concern in society today is the discovery of the cjd link to bovine spongiform encephalopathy (bse) most commonly known as mad cow disease. Human consumption of bse infected beef results in a variation of the cjd encephalopathy. This new variant cjd (nvCJD) is the cause of an epidemic scare in the United Kingdom. This nvCJD has been known to infect people as young as 16 years old (Reingold, 1996). There is also fear that the infected beef could make its way into the United States. Corneal transplants, dura mater grafts, growth and gonadotropic hormone treatments derived from infected cadavers have also resulted in a form of cjd known as iatrogenic cjd. Hospitals are taking extra precautions for the sterilization of all surgical equipment in fear of a disastrous outbreak. For now, there is no cure, or successful screening measures. Several institutions and support groups such as cjd Foundation Inc., are working to find new medications and procedures for testing and treatment.

Communication is just as affected in cjd as it is in other forms of dementia. There have been cases recorded with Broca?s aphasia as the primary language problem, and also problems associated with motor movements of speech such as apraxia. In cjd cases displaying Broca?s aphasia ?word finding difficulties progressed to one-word conversational speech, severe comprehension problems and inability to repeat, read, or write? ( Ferrand & Bloom, 1997). Motor speech difficulties arising in the course of the illness will also effect the speech of the client. The speech language pathologist may find themselves asking questions about what course of therapy to take, if any. The disease is such a progressive one. Would language intervention be beneficial to the client considering the estimated life expectancy of a cjd patient? The challenge lies in preserving the language the patient has for as long as possible.

The slp must take into consideration many key factors. First of all, the patient?s best interest. Secondly, time constraints are to be considered. There is not enough time for elaborate therapy sessions. The slp must act fast to devise strategies that work for the patient and the family. The main goal is to keep this patient in touch with their family for as long as the illness will allow. Thirdly, the speech pathologist must be aware that as the illness progresses, the strategies for communication will change. Educating the family as the illness progresses will make the transitions between language strategies easier. To meet this demand, the pathologist must assess the patient?s abilities in intervals to determine the best means for communication. That communication can take whatever form works for the family. Writing, singing, nods of the head or taps with the hand may prove effective as the patient progresses in the illness. Lastly, the speech patholigist can ease some of the family?s stress by providing information about support and community groups who are dealing with similar situations. These groups can educate and counsel the family about ways to make their loved one more comfortable and perhaps ease the pain of the loss.

Creutzfeldt-Jakob Disease is a very real illness that cannot be placed on the sidelines. The bottom line is cjd needs to be studied. There is no set way to treat a progressive disease like cjd until there is more research. We know several things about cjd such as how it is spread, the symptoms, and some forms of treatment, but there is much more to be learned. The important factor during the course of cjd is the patient. We as Speech Pathologists must ask ourselves a few questions. Was the best interests of the patient met for as long as they could have been? Was the family aware of any and all support and community groups available to them during this difficult time? If we as SLP?s can answer these questions with a yes, then we have done our jobs indeed. To those who involoved with cjd, thank you. Because of the researchers, therapists, and families of cjd patients, we have the knowledge we have today. This knowledge will continue to grow, providing tests, screening measures and maybe some day a cure. To live with the hope that one day another person will not have to endure what sorrows you have can be a great motivation.

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