Does the following sound any bit familiar? You are at the dinner table, enjoying a nice meal with friends and family, about to bite into that juicy steak, when someone graciously brings about the topic of mad cow disease.
Yeah, I didn’t think so
Most households converse about sports, or perhaps the latest news, but in my house, we talk about what may come and make our days a lot worse. In the spirit of spreading some love from my home to yours, let us speak about Creutzfeldt Jakob’s disease (CJD): the human variant of “mad cow” disease.
CJD is a rare disease, one that only worsens over time and is invariably fatal. It belongs to a family of human and animal diseases known as prion diseases, scientifically named transmissible spongiform encephalopathies (TSEs).
Spongiform refers to the appearance of infected brains, which become filled with tiny holes, resembling a sponge under a microscope.
Prions, on the other hand, refer to the abnormal version of ordinary prion proteins. Prions are normally harmless; however, once they fold, they change radically and harm biological processes within the body.
Image above: Normal Prion (left) compared to Diseased prion (right)
In a study conducted by the National Institute of Neurological Disorders and Stroke, or NINDS (which is the most efficient abbreviation ever), CJD affects about one in every one million people per year worldwide: in the United States alone, this totals to roughly 350 people per year.
A recent interview by ABC News introduced the world to the latest victim of this rare human form of “mad cow” disease: a thirty year old Tennessee father named Tony Gibson. Tony began experiencing symptoms of CJD about two years ago. He became so forgetful that he would get lost in his own home.
Once his condition progressed, Tony’s wife, Danielle Gibson, took him to a neurologist who diagnosed him with CJD. Doctors initially gave Tony one year to live, but he is fortunately still alive, though in poor condition.
Tony needs 24-hour care at a nursing home. His family has created a ‘GoFundMe’ site to pay for his care. They have currently raised $24,000 toward their eventual goal of $10000.
CJD is a complicated disease and every sufferer will eventually lose the battle against it. As has been popularly stated, “Knowledge is power.” When you educate yourself about a serious illness, even about one which is rare, you can gain a greater understanding of what to do to remain healthy.
When I was first introduced to mad cow disease during a dinner a few years ago, I was absolutely fascinated. One trait of the illness that both excited and frightened me was that symptoms of CJD almost always appear in later life (ages 50-70) and those symptoms are often incredibly vicious.
This means that one can have the disease and not even be aware of it. According to Medical News Today, CJD can remain dormant for up to 40 years! Even worse, once symptoms appear, the average life expectancy for those with CJD is merely 6-14 months, with most sufferers dying before a year’s time.
The precise symptoms of the disease are just as scary as their brutality. During the early stages of this illness, people may experience failing memory, behavioral changes, lack of coordination and visual disturbances.
Things only get worse from there.
Once the disease has progressed further, symptoms such as confusion, aggressive behavior, and paranoia are often salient. Final symptoms preceding death may even include blindness, coma, and weakness of extremities.
I can guess what you’re probably thinking: “Well, who says I’m going to get CJD? I literally just read that it’s super rare. Blah, blah, blah.”
Sure, I’m not denying that your chances of getting the disease are incredibly low, especially if you’re only eating meat from cows within the kosher trade circle. But there is still that .0001% chance of getting a seriously sucky disease.
Okay, to be honest, your chances are a little lower than that: 0.00000133333%.
To alleviate your extreme anxiety, I’ll tell you that CJD cannot be transmitted through the air or by normal contact, unlike other diseases. CJD can only be transmitted in one of three ways:
Sporadically. This is the most common type of CJD and accounts for the majority of cases (at least 85 percent). In sporadic CJD cases, the disease appears even though the person has no known risk factors for the illness. This doesn’t mean that there is no reason for the illness, but rather that doctors have no idea how the patient contracted the disease.
By inheritance. This variant of the disease is referred to as familial CJD. About 5 to 10 percent of cases in the U.S. are hereditary, meaning there is just nothing one can do to prevent this strain of the disease. A person’s chances of contracting CJD this way is ten times lower than that extremely minute number mentioned earlier. (Again, it was only: 0.00000133333%.)
By contamination. The transmission of the disease is possible through exposure to either brain tissue or spinal cord fluid from infected individuals. Because current sterilization methods do not destroy abnormal prions, a few people have developed CJD after being exposed to these substances during a medical procedure, such as by the result of a transplant or injections stemming from contaminated organs.
A specific case from the National Hormone and Pituitary Program (NHPP) relates that pituitary growth hormone taken from human corpses can and has caused CJD. As a result of this (and other complications), all human growth hormones used in the United States have been synthesized by recombinant DNA procedures since 1985.
On the topic of CJD by contamination, the appearance of the new variant of CJD (nv-CJD or vCJD) in multiple people in Great Britain has led to concern that “mad cow” disease may be transmitted to humans through consumption of contaminated beef as well.
Doctors are noticing that transmission through blood and plasma is causing infections, as diseased prions in CJD patients can be found in these substances. Based on this concern and as a result of other studies, the United States has enacted new protective laws.
According to these laws, people who have resided for more than three months in countries where “mad cow” is common are now restricted from donating blood. (Fun fact: this includes Journalism moderator Michael McGoldrick, who lived in England in 1989).
The United Kingdom was essentially the impetus leading to this restriction. From 1986-1998, the UK saw an epidemic of “mad cow.” By 1996 the disease appeared to have crossed over to humans, as scientists identified the first case of vCJD.
As previously mentioned, CJD is fatal and incurable: as of May 2015, it has killed 177 people in the United Kingdom. While human cases of the disease have greatly declined since 2000, the illness can theoretically remain dormant for years. Scientists have warned that as a result of this, the infected are silent carriers, and their ignorance can lead to a second wave of CJD.
After having read this article, you will hopefully have more awareness of this disease and how to prevent yourself and loved ones from contracting it. Think twice before you bite into that big juicy burger!