Stories of the World

Five Years Beyond: Losses and Legacy of Creutzfeldt-Jakob Disease

Kent McLean was a bright, hard-working, gentle family man. At 61, he was a Boeing Engineer with a PhD in Physics, active in his local church, happily married with two daughters and the first grandchild on the way. He enjoyed working outside in the yard or on projects around the house. He was healthy, and on most counts, quite happy.

In late June of 2002, he began experiencing problems with his vision. At first, he thought he needed a new prescription for his glasses. When that didn’t right things, he went back to the doctor. Other symptoms began occurring: numbness in his right hand, fatigue, words dropped from his speech. He stopped driving, and more serious medical consults began.

Doctors tested fluid and tissue. An MRI turned up clean, as did tests for various cancers and Multiple Sclerosis. Everything appeared normal in the labs, but nearly every day another symptom appeared. Kent could no longer hold a fork, and cradled his right hand like a wounded paw. With a brilliant mind and an articulate sense of detail, he patiently described his symptoms as they unfolded.

A gifted piano player since childhood, he sat down at the grand piano one evening only to find he could not play a single note. He could hear the music in his head, but could not translate it through his fingers. That evening signaled something was very wrong.

As the days progressed and the doctors continued without answers, the sense of urgency grew. Over the course of a single work day, he could no longer make sense of his email. He could see the words clearly, but had no comprehension of more than one or two words strung together. At the end of that day, he said quietly, “I hope we figure this thing out. I’m no good here.” He stopped working and took a short term leave of absence.

In the beginning of August, doctors suspected Multiple Myloma, a rare and untreatable kind of cancer. The sense of desolation increased, and still there was unknowing. Another MRI, an EEG, more tests. Finally the doctors sent him home saying, “We don’t know what else to test for.”

On August 6th, doctors from the University of Washington Medical center in Seattle made a grim diagnosis: Creutzfeld-Jakob Disease, a rapidly progressing, degenerative, neurological disorder, affecting one in a million. There is no cure or treatment. It is always fatal. The time estimate was one month to a year. Kent was dead three weeks later.

The end weeks were a tortuous unraveling: loss of speech and muscle coordination, a constant agitation of movement, muscle spasms that knocked over lamps and threatened pictures on the wall, a violent refusal of medications or injections, and eventually of all food and liquid. He was ready to end the suffering, though the agony continued for another six days, even without any nourishment or water. He passed away at a hospice facility on August 29, 2002, just weeks after showing symptoms that anything was amiss.

Little is known about Creutzfeldt-Jakob Disease, a quickly degenerative brain disorder that falls under classification as a Prion disease among others such as Gerstmann-Straussler-Scheinker disease, Fatal familial insomnia, and Kuru in humans, and bovine spongiform encephalopathy, or BSE, commonly referred to as Mad Cow Disease in cattle, Scrapie in sheep and goats, and Chronic Wasting Disease in elk and deer.

In Washington State, CJD cases are on the rise. Since 1997 there have been 44 documented cases, in a state with less than six million people. More have gone undiagnosed, but conveniently Washington State remains a non-reportable state, thereby not requiring doctors or health officials to report more accurate numbers or researchers to examine trends in geography, or other risk factors. The tragedy of under-reporting, or common misdiagnoses of Alzheimer’s or Parkinson’s, is that it greatly underscores the need for public awareness, broader safeguards, money for research and treatment, and support to the number of families suffering alone, with little information and littler hope.

There are four strains of Creutzfeldt-Jakob Disease. The most widespread strain, the one from which Kent McLean and countless others around the world have died, is called Sporadic CJD. This strain makes up about 85% of all CJD cases and has no clear, identifiable cause.

Another form of CJD, Variant Creutzfeldt - Jakob disease, comes from contaminated beef, and swept across the UK and other parts of Europe in the 1980’s and 1990s. From that outbreak came increased surveillance and mandatory reporting of the illness, a vast increase in cattle testing before slaughter, and the outlawing of the most dangerous farming practices such as using blood, recycled animal tissue, spinal cord, and other matter in the feeding of live animals. It takes less than a gram of infected feed to contaminate an animal. If that same animal is used in countless ways, and those humans and animals become infected, the cycle continues its sick spiral.

In the United States, none of these measures have been taken. Reporting of CJD cases is not required, animal feed can still contain animal byproducts, and cow blood is even mixed with milk for supplements to calves to increase growth and weight. Animals are slaughtered with a device that causes such trauma to the head that brain and bits of spinal cord are found throughout the meat. Prime cuts may be no safer than ground beef. Animal byproducts are used in sauces, flavorings, makeup, and a thousand other household items. With current practices, there is no way to trace contamination, and no accountability.

The U.S. government, in fact, has recently scaled back testing for mad-cow disease by 90%. Just three years after the scare in Washington State where an infected cow was found and caused dozens of nations to boycott American beef, the government has decided the cost is too high, and the risk for human consumption too low. Rather than adopt safe practices to protect the food supply, and to protect American beef interests abroad, it has chosen to look the other way.

Creutzfeldt - Jakob disease can also be inherited, with 5-15% of CJD cases developing from inherited mutations of the prion protein gene. Inherited forms include Gerstmann-Straussler-Scheinker syndrome and fatal familial insomnia.

Additionally, CJD can be contracted in iatrogenic cases through medical procedures such as corneal transplants, implantation of inadequately sterilized electrodes in the brain, and injections of contaminated pituitary growth hormone. Sterilization practices such as cooking, washing, and boiling don’t kill infectious prions that may be on surgical instruments, so other precautions must be taken. CJD has also been proven to be transmitted through infected blood.

While some diseases, notably certain cancers or other illnesses that affect celebrities, are held up to the public as the ones needing the most funding and awareness, lesser known illnesses, especially those on the increase without known cause or treatment, generate just as much suffering for families and patients enduring the long road without answers.

If Kent McLean had not gone into Physics, he would have studied medicine. He was fascinated with the human body and called it the most magnificent machine. May science intersect with service to raise awareness and answers about this disease so that others may not experience such suffering, and so that Kent’s life, and those of other CJD victims, can continue to bring remembrance and hope.