Mad " Human" Disease Final Draft

Emily Martin
eamartin@usieagles.org
English 201.501
Final Draft
1728 words

Mad “Human” Disease

On July 10, 1999, Francis Will passed away from a debilitating disease known as variant Creutzfeldt – Jakobs Disease. He was a veteran, grandfather, father, and devoted husband to his wife of nearly 50 years. He first showed signs of becoming ill a mere 18 months earlier. From the time the symptoms started until the day he passed away, his family and friends saw a connection to another disease commonly found in cattle. This progressive neurological illness is known as Mad Cow Disease. His passing left his family and doctors with more questions than answers. They wondered when did he get the disease, what could have been done to stop it, and how do they protect themselves from it. However, government officials and Centers for Disease Control and Prevention were less than willing to give them any real answers or admit that he actually had it. Through this research hopefully the family will be able to have some answers to their questions.

Mad Cow Disease is also known by the more medical term BSE, Bovine Spongiform Encephalopathy. BSE is a negative, progressive, neurological illness found in cattle. It results from an infection from a transmittable retarded protein known as a prion. This prion doesn’t start out harmful, but as a proactive protein. It is still unclear how the protein changes into the deadly prion, which attacks the central nervous system and connective organs in cattle (BSE 1).

Mad Cow Disease, or Bovine Spongiform Encephalopathy, was first discovered in the United Kingdom in 1986, although it is thought to have origins in the 1970’s (BSE 1). From 1986 to mid 2006 nearly all the cases of Mad Cow Disease were reported in the United Kingdom. In 2005, however, Portugal reported the highest number of cows infected with Mad Cow Disease per million over the age of 24 months (Variant 2). For the first time in history from 2001 to mid 2006, four countries outside of the United Kingdom reported diagnosed cases of Mad Cow Disease in their cattle herds (Variant 1). These countries included Canada, Israel, Japan, and the United States.

It is thought to have started from farmers feeding their cattle herds a scrapie-infected sheep meat and bone meal mix, and then cannibalizing younger cattle. Scrapie, much like Bovine Spongiform Encephalopathy, is a mutated prion disease, is a neurologically degrading disease commonly found in sheep. It causes sheep to loose voluntary and involuntary organ function (BSE 1). These sheep often stop eating and therefore become to skinny to slaughter and sell for profit. In turn, they become feed for other animals. Cattle farmers often feed this infected meat and bone mix to there cows because it was originally thought that the disease could not be passed from one species to another species. However, the farmers were wrong and cows began to start showing some of the same crazed animal symptoms as the sheep herds shortly after being fed the meat and bone meal. The timeline in which the cattle herds started showing signs after eating the sheep feed varies from a few months to a little more than a year.

Not learning from their original mistakes, and not wanting to waste the meat from their now sick cows, cattle farmers would slaughter them and feed them to the younger calves hoping they would thicken up and make up for the lost profits. Again, the farmers were wrong and saw large numbers of cows and calves becoming ill and even giving birth to sick calves. Cattle farmers needing to make ends meet continued to sell the infected meat to processing plants and distribute it into the public meat market (BSE 3).

Cattle that become infected often experience a sense of confusion, in the sense that they forget how to walk or eat. These cows are often seen walking in circles for long periods of time. More symptoms often witnessed by cattle farmers are a sagging head, staggering foot steps, wide eyes, lack of or loss of appetite, poor skin quality and irritability. After Mad Cow Disease had been discovered in Canada and in the Untied States in 2004, the Food and Drug Administration and Canadian Food Inspection Agency issued nation wide feed bans. These bans included cattle tissue capable of transmitting Mad Cow Disease through any form of animal product, such as the brain, spinal cord, lungs, eyes, and other nervous system organ. The animal products included in the ban were animal feeds, commercial pet foods, and fertilizers used to strengthen pet medication and dietary supplements (Variant 2). However, even with all the new bans on feed, the progression of the disease in humans hasn’t stopped. For many people, they were already infected months, if not years before the ban was even a thought.

From early 1996, there have been an increasing number of patients experiencing some of the same symptoms of the cattle diagnosed with Bovine Spongiform Encephalopathy. Doctors called this human disease variant Creutzfeldt – Jakobs Disease, seeing as a Creutzfeldt – Jakobs Disease already exists but had nothing to do with cattle or prions (Variant 1). Doctors recorded that both diseases, Mad Cow Disease and Creutzfeldt – Jakobs Disease, are caused by a rare protein malfunction resulting in a central nervous system attacking prion. Both victims, cattle and humans, which are infected by the disease, see long incubation periods, often 18 months to three and a half years, before the fatal brain disease becomes visible (CJD 1). The occurrence of the disease in humans is rare but it can be detected in anyone, anywhere. It is one disease that does not pick its victim from a race, ethnic background, or economic status. The diagnosed cases of variant Creutzfeldt – Jacobs Disease are found in the areas where the cattle have been diagnosed with Bovine Spongiform Encephalopathy.

The Centers for Disease Control and Prevention state that from 1995 to 2006 only 195 cases of variant Creutzfeldt – Jakobs disease had been diagnosed world wide. An overwhelming number of them, 162 cases, were discovered in the United Kingdom. 20 cases were discovered in France. 4 cases were discovered in Ireland and 2 cases were discovered in the United States. Canada, Italy, Japan, the Netherlands, Portugal, Saudi Arabia and Spain all experienced 1 case (Variant 1). Each of these countries with 1 case found that the infected patients lived in or traveled to the United Kingdom during the 1993 outbreak. Out of all of these patients only 3 of them did not get the disease from cattle. They received the disease through a blood transfusion (">Boen 2). The evidence of a botched blood transfusion urged the United States to set stricter guidelines in 1999, outlining a geography-based donor referral list to reduce the risk of transmitting to disease across the United States (Variant 2).

Even though the Food and Drug Administration and Centers for Disease Control and Prevention doesn’t believe the disease is common, the United States government seems to be doing more to stop the spread of the disease in its own country (">Boen 1). The Centers for Disease Control and Prevention says that there have only been three cases of variant Creutzfeldt – Jakobs Disease discovered in the United States (">Boen 2). However, they are doing very little to protect its citizens when visiting other countries. The CDC recommends not eating the meat in the United Kingdom if one should visit there. They suggest that if a tourist has to eat the meat, they should stick to solid pieces of meat such as steak, but never eat beef brains, burgers, sausages, or meat patties (Variant 3).

Even though health standards are getting stronger and stronger there is still no way to stop the spread of the disease. As of August 2006 there was still no known treatment to stop the progression of the prion disease (Variant 4). The only way a true diagnosis of the disease can be acquired is by an autopsy (">Epidemiology 1). By that time it is too late to treat the patient or try to find a cure.

As for Francis Will and his family, they experienced a number of hardships trying to prove he actually suffered and passed away from variant Creutzfeldt – Jakobs Disease, the human form of Mad Cow Disease. His family, along with a number of people in the advanced medical community from Washington University in St. Louis, School of Medicine, and the Medical Examiner in Evansville, Indiana, believe the number of people passing away from variant Creutzfeldt – Jakobs Disease is much greater than reported by the Centers for Disease Control and Prevention, and they are working hard to open the eyes of the public and make them aware of the real dangers. Many in the medical community feel that the Centers for Disease control and Prevention, Food and Drug Administration, and other government agencies are keeping the realities of the disease as quiet as possible as to not upset the economic balance is the meat industry. However, there is no proof that the CDC or FDA are falsifying evidence, so no one can accuse them of doing anything wrong. Doctors and scientist alike just feel that they are seeing and trying to treat a larger number of infected patients than is being reported to them by the CDC. For now, the only question for Francis Will’s family and the other victim’s families left to wonder is, “when are we going to see an end to this horrible disease?”


Works Cited

Boen, Jennifer L. “’Mad Cow’ variant not now a risk: only 3 vCJD cases in the US were confirmed; all had link to UK beef.” Knight Ridder Tribune Business News (16 June 2007): 3 pg. 2 Aug 2007 http://proquest.umi.com/pqdweb?index=34&did=1289500941&SrchMode=1&sid=8&Fmt=3&VInst=PROD&VType=PQD&RQT=309&VName=PQD&TS=1187381321&clientId=4130>

“BSE (Bovine Spongiform Encephalopathy, or Mad Cow Disease).” Centers for Disease Control and Prevention (18 May 2007): 3 pg. 2 Aug. 2007 http://www.cdc.gov/ncidod/dvrd/bse/index.htm.

“CJD (Creutzfeldt – Jakob Disease, Classic).” Centers for Disease Control and Prevention (13 April 2007): 2 pg. 2 Aug 2007 http://www.cdc.gov/ncidod/dvrd/cjd/

“Creutzfeldt – Jakob Disease Epidemiology; New Creutzfeldt – Jakob disease epidemiology research reported from National Institute of Public Health, Department of Epidemiology.” Medical Letter on the CDC & FDA (Aug. 2007): 2 pg. 2 Aug. 2007 http://proquest.umi.com/pqdweb?index=0&did=1313343791&SrchMode=1&sid=10&Fmt=3&VInst=PROD&VType=PQD&RQT=309&VName=PQD&TS=1187382064&clientId=4130>

“Creutzfeldt – Jakob Disease (Variant) and Bovine Spongiform Encephalopathy (Prion Diseases).” Centers for Disease Control and Prevention (10 Mar 2007): 4 pg. 2 Aug. 2007 http://www.cdc.gov/print.do?url