With Nursing Perspective: Creutzfeldt Jacob Disease

Abstract

Creutzfeldt-Jakob disease (CJH); is a prion disease that occurs with symptoms of cognitive and mental state deterioration, cerebellar ataxia, myoclonic movements and vision loss. It is important to distinguish it from other diseases as it progresses rapidly and causes death. From laboratory studies, EEG is initially non-specific, and in the middle and advanced stages of the disease, periodic sharp wave discharges typically occur every 0.5-2 seconds. Its annual incidence has been reported as one in a million. The application of patients with different clinical features makes it difficult to recognize the disease. The prognosis of the disease is fast and complex, affecting the patient and family negatively in term of physically, socioeconomically and psychologically. In this disease group, where multi-faceted care needs can occur, nurses can provide a patient-centered and evidence-based care to increase the quality of life patient and their family. Another issue that concerns nurses who are in constant contact with the patient and provide care is infection control. In this article, care needs that can occur in CJH patients and nursing approaches are examined.

Keywords

• Creutzfeldt-Jakob Disease
• Patient-Centered Nursing
• Continuum of Care

Hemşirelik Bakış Açısı İle: Creutzfeldt Jacob Hastalığı

Abstract

Creutzfeldt-Jakob hastalığı (CJH); bilişsel ve ruhsal durumda bozulma, serebellar ataksi, miyoklonik hareketler ve görme kaybı bulguları ile ortaya çıkan bir prion hastalığıdır. Hızla ilerleyerek ölüme sebep olması nedeni ile diğer hastalıklardan ayırt edilmesi önemlidir. Laboratuvar incelemelerinden EEG’de başlangıçta özgül olmayan bulgular, hastalığın orta ve ileri dönemlerinde ise tipik olarak 0.5-2 saniyede bir oluşan periyodik keskin dalga deşarjları görülür. Yıllık görülme sıklığı milyonda bir olarak bildirilmiştir. Hastaların farklı klinik özelliklerle başvurması hastalığın tanınmasını güçleştirmektedir. Hastalığın prognozunun hızlı ve karmaşık olması hasta ve ailesini fiziki, sosyoekonomik ve psikolojik yönden olumsuz etkilemektedir. Çok yönlü bakım gereksinimlerinin oluşabildiği bu hastalık grubunda hemşireler, hasta merkezli ve kanıta dayalı bir bakım sunarak hasta ve ailesinin yaşam kalitesinde artış sağlayabilirler. Hastayla sürekli temas halinde bulunan ve bakım veren hemşireleri ilgilendiren bir diğer konu da enfeksiyon kontrolüdür. Bu makalede, CJH hastalarında oluşabilecek bakım gereksinimleri ve hemşirelik yaklaşımları incelenmiştir.

Keywords

• Creutzfeldt jacob hastalığı
• hemşirelik
• bakım

References

1. 1. Mackenzie G, Will R. Creutzfeldt-Jakob disease: recent developments [version 1; referees: 2 approved] F1000Research 2017; 6(F1000 Faculty Rev):2053. http://dx.doi.org/10.12688/f1000research.12681.1.
2. 2. Geschwind MD. Prion diseas. Continuum (Minneap Minn). 2015;21(6):1612–1638. http://dx.doi.org/10.1212/CON.0000000000000251.
3. 3. Eren F, Ekmekçi AH, Öztürk Ş. Sporadic creutzfeldt-jakob disease in the differential diagnosis of psychiatric disorders: Two case reports. Haseki Tip Bulteni 2018;56(3):252–255. http://dx.doi.org/10.4274/haseki.99609.
4. 4. Bishop MT, Pennington C, Heath CA. PRNP variation in UK sporadic and variant Creutzfeldt Jakob disease highlights genetic risk factors and a novel non-synonymous polymorphism. BMC Med Genet. 2009;10(1):146. DOI: 10.1186/1471-2350-10-146.
5. 5. Moda F, Gambetti P, Notari S. Prions in the urine of patients with variant Creutzfeldt-Jakob disease. N Engl J Med. 2014;371(6):530–9. DOI: 10.1056/NEJMoa1404401.
6. 6. Kurne A, Ertuğrul A, Yağcioğlu AEA, Demirci E. Creutzfeldt-Jakob Hastalığı: psikiyatrik belirtilerle başlayan bir olgu. 2005;16(1):55–59. Available from: http://www.turkpsikiyatri.com/C16S1/creutzfeldtJakob.pdf
7. 7. Kaneko K. Creutzfeldt-Jakob disease (CJD), variant CJD, and BSE. Nippon Rinsho. Japanese Journal of Clinical Medicine. 2003;61(3):9–16. Available from: https://www.who.int/zoonoses/diseases/Creutzfeldt.pdf?ua=1
8. 8. Nihat A, Mead S. Detection of Creutzfeldt-Jakob disease prions in skin: implications for healthcare. Genome Medicine. 2018;10(1):22. http://dx.doi.org/ 10.1186/s13073-018-0536-3.

9. 9. Orru CD, Yuan J, Appleby BS, Li B, Li Y, Winner D et al. Prion seeding activity and infectivity in skin samples from patients with sporadic Creutzfeldt-Jakob disease. Sci Transl Med. 2017;9(417) 77-85. http://dx.doi.org/ 10.1126/scitranslmed.aam7785.
10. 10. Vacca VM. CJD understanding creutzfeldt-jakob disease. Nursing. 2016;46(3):36–42. http://dx.doi.org/10.1097/01.NURSE.0000480598.84274.0f.
11. 11. Bailey B, Aranda S, Quinn K, Kean H. Creutzfeldt-Jakob disease: extending palliative care nursing knowledge. International Journal of Palliative Nursing. 2000; 6(3), 131–139. http://dx.doi.org/10.12968/ijpn.2000.6.3.8940
12. 12. Feifei W. A case of nursing experience of taking care of Creutzfeldt-Jakob Disease patient in China. Journal of Nursing & Care. 2016; 05(02): 2–4. http://dx.doi.org/10.4172/2167-1168.1000336
13. 13. Karasin M. Special needs populations: Perioperative care of the patientwith creutzfeldt-jakob disease. AORN Journal. 2014;100(4): 390–410. http://dx.doi.org/ 10.1016/j.aorn.2014.06.018
14. 14. Myles S, Douglas MJ, Ward HJT, Campbell H, Will RG. Variant Creutzfeldt-Jakob disease: Costs borne by families. Health and Social Care in the Community. 2002;10(2):91–98. http://dx.doi.org/10.1046/j.1365-2524.2002.00346.x
15. 15. Barnett F, McLean G. Care management of Creutzfeldt-Jakob Disease within the United Kingdom. Journal of Nursing Management 2005;13(2),111–118. http://dx.doi.org/ 10.1111/j.1365-2934.2005.00449.x
16. 16. Trevitt CR, Singh PN. Variant Creutzfeldt-Jakob disease: pathology, epidemiology, and public health implications. The American Journal of Clinical Nutrition. 2003;78(3):651–656.
17. 17. WHO manual for surveillance of human transmissible spongiform encephalopathies including variant Creutzfeldt-Jakob disease [Internet]. Chapter 6: Tissue handling and safety precautions. [Erişim tarihi: 24.11.2020]. Available from: http://whqlibdoc.who.int/publications/2003/9241545887.pdf.