Creutzfeldt Jakob Disease: Causes, Symptoms and Treatment

Creutzfeldt Jakob Disease: Causes, Symptoms and Treatment One of the diseases that has severely impacted the lives of people these days is Creutzfeldt-Jakob disease (CJD) which is a condition that is fatal and rare which has impact on the brain of human being. CJD causes damage to the brain that gets worsened with the passage of time. The patients diagnosed with the disease die within one year as people become immobile (Solassol et al., 2006). CJD is usually caused by an abnormal protein known as prion. Before prion is understood, proteins function for body must be clarified. For every human being, proteins are important for life and are present in body of all living things. Every part of humans body has good composition of protein such as muscles, hair, fingernails, bones, skin, blood, body organs and eyes. After water, protein is the second most important constituent of body (Belay et al., 2003). According to LiveScience Staff (2012), protein is mainly used for building, maintenance and repairing of body tissues; it is present in various forms which perform many jobs in human body and the structure of each protein determine its function. The building blocks of proteins are long strings of amino acids which get folded and curled into complex three dimensional shapes which allow proteins to perform their job. Dr. Stanley B. Prusiner from University of California i.e. San Francisco purified an agent which was infectious made of unique kind of protein and referred to it as prion in 1982; Nobel Prize in Physiology or Medicine was awarded to him in 1997 for discovery of Creutzfeldt-Jakob Disease (CJD) that was mainly caused by prions. Prions get accumulated in brain at much higher levels that cause permanent damage to the nerve cells which cause various neurological symptoms. These infectious particles are different in behavior from conventional bacteria and viruses which cannot be destroyed by exposing to heat and radiation. Even antiviral and antibacterial medicines have no effects on prions; hence, presently, there is lack of valuable treatment for CJD (Belay et al., 2003). Understanding Creutzfeldt-Jakob Disease (CJD) Basically, CJD is a very rare form of deadly form of dementia which can spread fastly in the body from brain. Prions are mostly found in brain and are harmless; when they are not given proper shapes, they may have effects that can be devastating which can even attack brain, kill its cells and create holes or gaps in tissue of brain (Ironside, 2009). Prion diseases have been found in both animals and humans; such diseases were in news of mid 1980s along with Bovine Spongiform Encephalopathy (BSE) epidemic which was referred to as Mad Cow disease, it is a cattle disease. In humans, it is known as CJD. The disease is found in about one to two people among million people worldwide. Types of CJD There are basically two types of CJD i.e. Classic CJD and Variant CJD (vCJD). The three types of classic CJD are Sporadic CJD, Familial or genetic CJD and Iatrogenic or Acquired CJD. Sporadic CJD More than 90% of people diagnosed with CJD are suffering from this type of CJD and it is mainly found in people aged in between 45 to 75. There are no specific symptoms for this CJD (Shah et al., 2009); however, some early symptoms such as depression may be present but it can quickly progress into confusion and problems of memory which is commonly observed in dementia, followed by loss of balance and coordination along with progressive blindness (Piouti et al., 2012). Familial or genetic CJD Familial or genetic CJD is one of the rare genetic condition in which the persons genes are inherited from one parent who causes the protein to get mutated into a prion in later stages of life that trigger the symptoms of CJD. In other words, it appears in families that have abnormal gene. About 7% of cases have this type of CJD. In order to diagnose it, blood test is done; person having this abnormal gene has 50% chance of transmitting it to children. The other rare form of this CJD are Fatal Familial Insomnia (FFI) and Gerstmann-Strà ¤ussler-Scheinker (GSS); both of these are rarely found in people (Brand et al., 2006). It occurs in people who have age of 50 years. Iatrogenic or Acquired CJD This type of CJD is rarely found in people as it is found in those people who get CJD while accidental transmission that takes place during a medical procedure like corneal grafts, instruments in neurosurgery, human pituitary hormone therapy or human dura-mater grafts (Mayo Clinic Staff, 2010). Variant CJD People having average age of 28 years are found to be suffering from Variant CJD. It is usually developed from eating of beef that was infected with BSE. It can even occur with blood transfusion in which variant CJD is transmitted from one person to other. Once the symptoms are identified, the disease spreads quickly in the body (Tattum et al., 2010). Signs and Symptoms of CJD In many of the cases, classic CJD looks similar to various other dementias; the disease comes on fastly and then declines the ability of thinking as symptoms start appearing. Some of the most commonly found symptoms are that the person may have swings in mood, problems related to memory, interest absence and not behaving like oneself, having difficulty while walking such as keeping balance, dementia rapid progressions such as loss of memory and other thinking capabilities, problems in vision such as blindness, stiffening of limbs, twitching or jerks in muscles, feeling of clumsiness, speaking problem such as slurred speech, difficulty in swallowing and akinetic mutism in which person can move eyes and appear to be alert but can neither move voluntarily or speak (Espinosa, Bensalem-Owen, Fee, 2010; Sikorska et al., 2004). However, people who are in CJDs later stages face problems such as losing awareness as it can be seen in various neurological examinations. While the disease in the early stages, CJD patients are scared and it can be a distressing situation for them; it is usually associated with hallucinations that are visual which means seeing things that are not present there which might develop uncomfortable feeling in them (Andrews, 2012). People who are suffering from sporadic CJD usually live than twelve months after the appearance of signs and symptoms; the maximum life is two years. Before death, some people fall into condition of coma; the main reasons of death are heart failure, pneumonia or respiratory failure such as breathing problem (Rossetti Dunand, 2007). Among the most common symptoms found in Variant CJD people are changes in behavior, depression, withdrawal from social gatherings, difficulty in walking, dementia that is progressive, unable to do movement or speak and pain and odd kind of sensation in limbs or face (LiveScience Staff, 2012). Causes of CJD According to studies of Ironside (2009) and Tattum et al. (2010), CJD is caused by slow virus or other small organisms. The agent that causes this disease has several characteristics which are different from viruses and bacterias. The organisms are difficult to kill as they dont have genetic information on them in the form Nucleic acids such as DNA or RNA and has long period of incubation before symptoms are made visible. However, it has been found that it is mostly caused by prion proteins that can occur in both normal and infectious form. The normal forms have same shapes as that of amino acids but infectious ones differ in their shapes from normal proteins. Once they appear, abnormal ones start to get aggregated which start affecting the brain. Only 5-10% cases are inherited ones that arise either from mutation or changes in gene which are responsible for controlling the formation of normal prion proteins. Diagnosis of CJD CJD is difficult to diagnose especially when it is in its initial stages; there is no specific test available for diagnosing in living person. Only way to make sure that person had CJD is by examining the tissue of brain after his death. However, there are some other tests and procedures can be used for diagnosis of the disease such as medical history examination as it will help doctors in learning the persons symptoms and signs when they get started as CJD gets spread quickly; Magnetic Resonance Imaging (MRI) in which brains picture is taken to identify the difference between various types of CJD; Computerized Tomography (CT) scan in which brains picture will be taken to diagnose the disease; Puncture of Lumbar which means taking fluid form the spine of a person by using syringe and a needle, it is done to identify the infections of brain; blood tests to identify if there is any chance of genetic CJD; Electroencephalogram (ECG) in which electrical activity of brain is measured and b rain autopsy in which tissue of brain is examined after persons death (Puoti et al., 2012; Shah et al., 2009). Treatment for CJD Up till now, there has been no success in developing right treatment for CJD. The researchers have done numerous tests with many drugs such as steroids, antiviral agents, antibiotics, amantadine, acyclovir and many more but they have not been able to find perfect cure for this disease (Puoti et al., 2012). Some studies are still in progression to develop appropriate treatment for patients but none of them have been successful in benefiting the human beings. The treatment for CJD that is being followed by various doctors is aimed at alleviation of symptoms and trying to make the individuals as comfortable as the experts can do so that they have the will power of fighting against the disease. Some of the opiate drugs can relieve pain when people suffer from it but the drugs such as Sodium Valproate and Clonzaepam definitely help in relieving of myoclonus. When the disease is in later stages, position of person is changed frequently so that he gets comfortable and bedsores can be prevented. For draining urine, a catheter can be used as it helps in controlling the function of bladder and artificial feeding can also be used. Precautions for CJD Although there are chances that CJD gets transmitted by being careless during medical procedures but still people need to take care of various things that can help them in remain protected from this disease. CJD is not a contagious disease that can be transmitted either by social or sexual contact or via air or feeding, touching or even taking care of person suffering from CJD at home. some of the basic precautionary measures that need to be followed are washing hands either before eating or drinking, covering wounds or cuts with bandages that are waterproof, protecting face and hands from being exposes to blood or fluids of body of person who is affected by this disease and taking special care in blood transfusions so that CJD infected person does not give his blood. In order to avoid variant CJD, beef from selected countries should be eaten; countries that have high risk of TSE are more vulnerable to risk. The only options available for avoiding this type of CJD are either to eat beef from countries that emphasize on strict regulations on its quality and avoid eating parts of cattle that have high risks such as spinal cord, intestines, brain and eyes. Conclusion Creutzfeldt Jakob disease (CJD) is among the fastest growing disease that is being faced by many people worldwide. It has become important for researchers to identify right course of treatment for this disease so that patients can get full recovery. In order to make sure that people who have genetic CJD are given appropriate treatment before symptoms start to become visible, it is important that people get their blood tests done at regular intervals. When any of the signs and symptoms is observed in people, it is advised that they contact a doctor immediately who can ensure that right tests are done for identification of this disease. Still, the researchers have to do extensive work for identifying the main causes of CJD along with developing a remedial solution. Among all the available options, the best one is to contact any well-known doctors who are specialist in neurology who will take the patient in right treatment method. Hence, everyone needs to make sure that they have complete information about this disease so that they can face it with determination and strong will power. Palliative Care for Cancer Patients | Literature Review Palliative Care for Cancer Patients | Literature Review Gretha Cabral Literature Review Cancer has become a global concern in the recent decades and various researches have raised the need for palliative care for cancer patients besides the treatment that they receive from their respective hospitals. Researches show that palliative care prolongs the life of patients more as compared on relying the treatments alone. According to research conducted by Julia Medew, patients who are suffering from lung cancer could enjoy longer, quality life and low depression where they bestowed palliative care immediately after they are diagnosed with lung cancer, as opposed to starting at the late stages of the disease. According to this study, Medew aimed that; the findings would change the views of both the patients and the doctors on the quality of palliative care (Medew, 2011). The care aims at improving the quality of life of the for patients with progressive, serious illnesses through paying high attention to the control of their symptoms and pain, recognizing their spiritual and p sychological needs and providing the necessary support to the patients and their families. Medew`s study divided 151 patients with lung cancer into two groups in order to clearly outline the quality of palliative care (Medew, 2011). The first group of lung cancer patients was to solely rely on the standard treatment that the patients would receive from the health facilities while the second group was to receive the same treatment that would be accompanied by immediate referral to palliative care within a time range of twelve weeks after diagnose. Consequently, the findings showed that, the average survival period of patients in the second group was around three months longer and those patients reported living a high quality life and far much better moods as compared to the first group. Moreover, this American study was seconded by Ian Haines, who was a Melbourne cancer specialist who recommended that, palliative care should be administered by doctors to cancer patients. This suggestion was made because most health professionals, cancer patients and many other individuals realized that palliative care can serve as the only default measure of managing cancer when all other measures have been exhausted. He also suggested that, if early referral of cancer patients to professional palliative care can be an expensive measure, the governments should subsidize by increasing the allocation of more funds to the ministry of health since its value outweighs the costs that may be associated with it (Medew, 2011). Professor Haines also gave another case study that recently followed 333 cancer patients up to their death. The study also divided the patients into groups where one involved patients who were hospitalized to receive cancer treatments while the other group was treated and discharged in order to receive additional palliative care at their respective homes. The study findings showed that, patients in the first group died within a short time interval than those in the second group. In addition, those patients in the first group also experienced emotional and physical distress than those patients in the second group who died while receiving palliative care at their homes. The study concluded that, faster occurrence of deaths in patients who are admitted in health facilities was highly contributed by high extents of grief and more post-traumatic stress that arose from the family members and friends of those patients (Medew, 2011). His study also established that early referral of patients to palliative care helps caregivers and patients to understand their treatment options better especially at the end of their life. Furthermore, this early referral helps to reduce debilitating expensive treatments such as chemotherapy and excessive utilization of limited medical resources. In addition, most cancer patients are not used to palliative care in most countries and this unfamiliarity calls for the need for providing information to patients, their family members and friends sensitizing to them the quality of palliative care. According to research conducted by Ms. Etheredge, one of the officials of hospice palliative care society, she found that, palliative care frightens most individuals including cancer patients since most people are used to the tradition of patients being admitted to hospitals for treatment. Therefore, discharging cancer patients after treating them and then requiring them to seek palliative care at their respective homes elevate stress to patients since the latter concludes that they are being sent to meet their deaths at home. She suggested that, the governments should construct a cancer hospice in a strategic point since according to her, the environment where palliative care is provided matters most (Furse, 1999). She further suggested that, any other hospice to be constructed near to water and bus route since these are the two primary necessities that assist the caregivers and the families. Moreover, she also seconded the decision of the health minister of Australia in launching the national palliative care awareness week that would inform people about the equity and access of this care (Brigid, 2011). During the launching of this palliative care awareness campaign, the health minister also outlined the palliative strategic plan to inform the services that palliative care offers. In addition, brochures printed in Greek, Chinese, Vietnamese, Italian and polishes were distributed to different people during that week outlining palliative care services. This distribution of brochures was to enhance the reach of palliative care information to more people especially those who both don’t speak English and are not aware of the services provided by palliative care. In addition, according to Mark Colvin, most of the cancer patients in Australia are not aware of the existence, quality and the services that are provided by palliative care centers because most doctors do not sensitize and refer these patients to palliative services. According to study that surveyed a sample of one thousand doctors all over the country and within the duration of one year, more than eight hundred doctors do not refer cancer patients to palliative care. Merrilyn stone, for example, was not referred to palliative care when she was initially diagnosed with breast cancer in 1990 (Barrett, 2004). This lack of referent made her to still firm her believe that palliative care is intended for the dying people and this made it harder for her to understand the value of palliative care when her condition came back eleven years later. Moreover, according to Afaf Girgis, this lack of referring patients to palliative care during the early stages of cancer development enhances the perception of the community and the patients that palliative care is intended for individuals who are at the death`s door and this is much untrue. Girgis further outlines this lack of referring cancer patients to palliative care predisposes the patient to more and severe consequences when the disease progresses to its advanced stages. In the long run, the particular patients live less additional days, as opposed to when they would have been early referred to palliative care services (Barrett, 2004). However, according to Rebecca Barrett, the study found that, very few health professionals who used to refer their patients to palliative care. In addition, in case these health professionals happen to refer cancer patients to palliative care, the latter was mostly intended to control pain but not for emotional or psychological support or legal and financial support. Consecutively, according to Dr. Sundquist, most health professionals concentrate more on curing the cancer patients and most are unaware of the quality of palliative care. He further challenges this doctors` works and suggests that, the latter needs to understand more is needed to cancer patients besides treatment services (Barrett, 2004). Moreover, cancer patients need additional services that include practical and emotional support that involves palliative care. Moreover, in Australia, Whyalla hospital developed project of constructing Whyalla cancer treatment Centre that would cancer services almost at par with the same treatment services that were offered in Adelaide. Initially, many cancer patients were forced to travel long distances in order to access cancer services at Adelaide hospital (Bruce, 2012). The construction of Whyalla cancer Centre aimed at reducing the transportation burden of cancer patients to long distances. According to whale news, the cancer center would offer a wide range of palliative services to the cancer patients especially those who were recently diagnosed with cancer. According to the chairman of his cancer project Mr. champion, the cancer Centre would offer services such as creating space for training activities and research, counseling of cancer patients, chemotherapy, outpatient care services among others. Additionally, the Whyalla cancer Centre would operate in cooperation with the already established Adelai de health facility in order to share health professionals and other technical equipments. This cooperation statement was echoed by Mr. Champion who said â€Å"the specialists will determine if the complexity of the cancer requires the patient to receive treatment in Adelaide or if they can remain in Whyalla.† (Bruce, 2012) However, the recent researches show that most patients suffering from cancer would prefer to meet their deaths at their homes rather than at the health facilities. These researches recommend that health professionals should follow the wishes of cancer patients on where the latter wants to receive palliative care. In connection to this, Eurobodalla health services have provided ambulance protocols that would facilitate doctors to respond to the emergency calls from the homes of cancer patients (Oconnor, 2014). These services would enable cancer patients to receive palliative care at their homes as they wish. References Barrett, R. (2004). Cancer patients missing out on palliative care; palliative support. Sydney, Australian Broadcasting Corporation. ABC Premium News Retrieved from http://search.proquest.com/docview/458087832? Brigid, O. (2011). Palliative care boosted. Melbourne, News Digital Media. Vic.Sunday Herald Sun Retrieved from http://search.proquest.com/docview/867896592? Bruce, K. (2012). Good news for cancer patients. Whyalla, S. Aust, Fairfax Media Publications Pty Limited. Whyalla News Retrieved from http://search.proquest.com/docview/1081480966? Furse, L. (1999). Strategy for better palliative care. Canberra, A.C.T, Fairfax Media Publications Pty Limited. Retrieved from http://search.proquest.com/docview/1012751431? Medew, J. (2011). Early palliative care could prolong life. Canberra, A.C.T, Fairfax Media Publications Pty Limited. Retrieved from http://search.proquest.com/docview/1020447587? Medew, J. (2011). Palliative care extends length and quality of life. Sydney, N.S.W., Fairfax Digital. Sydney Morning Herald Retrieved from http://search.proquest.com/docview/854392582? Oconnor, K. (2014). Treatment available closer to home for palliative patients. Batemans Bay, N.S.W, Fairfax Media Publications Pty Limited. Bay Post Retrieved from http://search.proquest.com/docview/1516592167? Gretha Cabral s3483440