CJD: A Rare, Fatal Illness Affecting Senior Citizens - Essay Sample

Introduction

Creutzfeldt-Jakob disease (CJD) is a fatal braid illness which causes deterioration of cells and organs leading to loss of function. Though rare (with a mere 0.001% occurrence), the disease is deadly and most of the affected individuals usually perish within one year (Stonebrook, 2007). It affects mostly the senior citizens from 60years onwards. The disease comes in three categories. The first and most prevalent is sporadic CJD, which catches a person even when no known risk factors exist for the disease. The second category is hereditary CJD, which occurs along the genetic lineage of a family. The last and least common is acquired CJD, which catches some individuals after exposure to certain medical regimes and procedures.

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Origin and Reservoirs

CJD is caused by abnormal prions, a group of harmless proteins which become infectious when misfolded or when they misshapen (Stonebrook, 2007). Another name for prion diseases like CJD is transmissible spongiform encephalopathies (TSEs). They are spongiform because once infected, brains grow holes to the point of being extensively spongy. Although prion proteins form all over the body, the transformation from normal to prion proteins occurs mostly in the nervous system (Sirakov, 2019). For acquired CJD, the abnormal protein comes from outside sources such as contaminated meat. The foreign prion attaches to and changes the conformation of the body's normal proteins and spreads to the brain where they poke holes.

For hereditary CJD, there might have been a mutation in the prion gene in the person's gametes and these mutations may spread to his offspring to cause the disease. In sporadic CJD, the blame lies with the protein synthesis systems, which are said to malfunction as a person ages; with the possibility of misfolding proteins. Prions clump together and attack the normal proteins in the nervous system to cause damage and nerve cell loss (Sirakov, 2019), thus CJD's degenerative nature.

Transmission and Points of Entry

CJD is a low-risk disease and does not transmit through physical or sexual contact and is also not air-borne. As illustrated above, it develops either sporadically (for no apparent clear causes), by genetic transmission, or by contamination. More than 70% of CJD cases are spontaneous, 15% are hereditary, while less than 5% of them are acquired by contamination (Stonebrook, 2007).

Infection by contamination may happen during a medical operation like organ transplant or brain surgery done with instruments that are contaminated with prions- standard sterilization methods may not completely eliminate prions. So, it can get transmitted by interaction with spinal cord fluid or brain tissue from infected individuals. CJD acquired by the latter methods is called iatrogenic CJD (Carrico, 2005). One may also get CJD by consuming meat from animals suffering from mad cow disease.

Onset and Symptoms

The symptoms of CJD only begin to manifest once brain damage starts and this may take up to 40 years to show (Carrico, 2005). At this point, the health condition of the patient begins to drastically deteriorate. The distinctive signs include a fast development of myoclonus and dementia. It involves changes in patient behaviour and moods, personality, impaired judgement, and memory loss. As CJD progresses, coordination problems worsen with increasing clonic spasms of muscles and muscle groups. The patient's vision begins to fail and may degenerate to total blindness as their speech becomes blurred too. The patient's systems continue to fail up to a point when they cannot speak or move anymore, and they slip into a coma.

Diagnosis and Treatment

The hallmark of CJD is its rapid progression of symptoms; it happens in days or weeks, with death occurring within a year. As of today, there exists no single text procedure to conclusively single out CJD, but some tests may give hints. The first test involves measuring the patient's brain activity using an Electroencephalogram (EEG); the second one detects changes in brain activity using Brain magnetic resonance imaging (MRI), the third one checks for prions in spinal fluid using spinal taps (lumbar puncture), and lastly, protein misfolding cyclic amplification (PMCA) can help to detect misfolded proteins in the nervous tissue (Stonebrook, 2007).

CJD currently has no known vaccine, cure, or preventive drugs. The rarity of CJD and the robust progression of symptoms have complicated most research trials. Present remedies involve enabling families to support the patients better and managing the symptoms. For instance, opiate drugs can help relieve pain while antiseizure drugs and muscle relaxants such as sodium valproate clonazepam can help in managing muscle stiffness and spasms (Carrico, 2005). Furthermore, artificial feeding and intravenous fluids can be infused to manage the symptoms during the later stages of Creutzfeldt-Jakob disease.

Prevention of CJD

The science community has come up with measures to avoid spreading the disease. To prevent acquired CJD, hospitals exclusively use synthetic human growth hormone to avoid transfusing abnormal prions. Also, if a patient who has undergone brain or spinal tissue surgery gets suspected to have CJD, the apparatus used must all get destroyed. For lumbar punctures, the kits must be used only once. In the USA, people with CJD risk factors are not eligible to donate blood (Stonebrook, 2007). Further, governments have put in place strict regulations of cattle import from countries with high mad cow disease prevalence, restrictions on the sale of animal feed, guidelines for handling sick animals, and resections on the animal's parts that can be processed for consumption.

Conclusion

Creutzfeldt-Jakob disease is a fatal ailment and researchers are still working to develop viable cures and prevention drugs. For those who are already affected, the family members can seek medical assistance for the patient as well as get counselling services to help cope with the patient's withdrawal patterns and other CJD symptoms.

References

Carrico, R. (2005). Creutzfeldt-Jakob disease: A comprehensive guide for healthcare personnel. Louisville, Ken: Chicago Spectrum Press.

Sirakov, I. N. (2019). Prions: Some physiological and pathophysiological aspects.

Stonebrook, M. J. (2007). Creutzfeldt-Jakob disease: New research. New York: Nova Biomedical Books.