Mad "Human" Disease

Emily Martin
eamartin@usieagles.org
English 201.501
Rough Draft
1371 words

Mad “Human” Disease

On July 10, 1999, Francis will past away from a debilitating disease known as variant Creutzfeldt – Jakobs Disease. He first showed signs of becoming ill a mere 18 months earlier. From the time the symptoms started until the day he past 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 form it. However, government 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. It is still unclear how the protein changes into the deadly prion, which attacks the central nervous system in cattle (BSE 1).

Mad Cow Disease 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 (Centers 2). For the first time in history from 2001 to mid 2006, 4 countries outside of the United Kingdom reported diagnosed cases of Mad Cow Disease (Centers 1). These countries included Canada, Israel, Japan, and the United States.

It is thought the have started from farmers feeding their cattle herds a scrapie-infected sheep meat and bone mix then cannibalizing younger cattle. Scrapies, a mutated prion disease, is a neurologically degrading disease commonly found in sheep that 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. In turn they become food 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 species to 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. Not learning from their original mistakes, and not wanting to waste the meat from there 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 ill calves, but 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 to all animal products, such as the brain, spinal cord, lungs, eyes, and other nervous system organ. The animal products included in the ban were animal feeds, pet foods, and fertilizers used to strengthen pet medication and dietary supplements (Centers 2). However, with all the new bans on feed hasn’t stopped the progression of the disease in humans. For many people, they were already infected for 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 (Centers 1). Doctors recorded that both disease, 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 3 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.

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 (Centers 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 (Centers 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 (Centers 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 (Centers 4). The only way a true diagnosis of the disease can be acquired is by an autopsy (Medical 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. 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. The only question for Francis Will’s family and the other victim’s families left to wonder is, “why isn’t the United States government, Food and Drug Administration, and the Centers for Disease Control admitting to the public, the overwhelming realities of the disease being discovered by the medical community at large?”

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

“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

“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