Q: My 64-year-old wife has been diagnosed with a type of dementia known as Creutzfeldt-Jakob disease. There is no cure or medicine that our doctor is aware of—any suggestions on what we can do to fight this terrible disease? If there is no medicine available, perhaps there are some herbs or over the counter vitamins that might slow this disease that you could recommend.
A: As you have most likely come to know, your wife has a rare brain disorder that has or will cause a significant deterioration in her mental and physical abilities. If her diagnosis is indeed Creutzfeldt-Jakob disease and not another form of dementia, I am sorry to say there is no proven treatment that will halt or cure the progression of this terrible disease. The best we currently can offer are targeted treatments to lessen the severity of her symptoms, as well as provide support services (homecare, others) to assist the whole family through this very difficult time.
Creutzfeldt-Jakob disease is part of a group of rare brain disorders (one person in 1 million people per year worldwide) known as transmissible spongiform encephalopathies. In other words, an infected brain becomes filled with holes and looks like a sponge under the microscope. The triggering agents are abnormal prion proteins known as proteinaceous infectious particles. But no matter what the fancy name or cause, the sad fact is they lead to a progressive brain disorder with symptoms that may include:
- Behavioral and personality changes
- Trouble with balance and coordination
- Impaired and eventual loss of memory, vision and speech
- Difficulty in thinking and in judgment
- Tingling, numbness and muscle paralysis
While each and every person may experience some, all, or additional symptoms in varying degrees and order, the end result is the same—death from complications due to conditions such as heart failure, pneumonia or even respiratory failure.
There are three main types of CJD, plus a more recently identified variant form known as bovine spongiform encephalopathy. The differences include:
- Sporadic, the most common, accounts for 80 percent to 85 percent of known cases. There is no known cause, and it may randomly develop in those between the ages of 45 and 75. Once subtle symptoms occur (mood swings, headache, sleep disturbance, others), there is a rapid deterioration and eventual death, often within six months.
- Hereditary is an inherited form causing 10 percent to 15 percent of all cases of CJD. If one parent was affected by the genetic mutation in the prion gene causing this disease, each of their children will have a 50 percent chance of inheriting this specific form of CJD. Those affected tend to show symptoms between the ages of 30 and 50. While the duration of illness is usually two to five years, there is great variability. Sometimes the person deteriorates rapidly over three months, while others have symptoms that slowly progress over several decades.
- Acquired, or iatrogenic, is extremely rare (less than 1 percent of cases). Those affected may have may have inadvertently acquired this disease after contact with an infected tissue (brain, central nervous system, cornea, others) or contaminated instrument used during a medical procedure. However, there is no evidence that CJD is contagious to others through touching or being around the affected person.
- Variant CJD, otherwise known as bovine spongiform encephalopathy or mad cow disease. While very rare, it is concerning and the number of cases are being closely watched. It seems to strike at a much earlier age, with the median age of death being 28. The initial symptoms often begin as significant changes in behavior and personality, followed by nerve pain, numbness and tingling in the limbs, and difficulty in movement. Once symptoms appear, death tends to occur within 13 to 14 months.
While I do not know what form of CJD has affected your wife (there are subtle differences in early symptoms and progression of the disease), the ultimate outcomes are the same. It is frustrating that I cannot offer better news, but I would highly encourage you to get a second opinion from a board-certified neurologist or dementia specialist. If by chance her dementia is from another cause (medication, illness, tumor, etc.) that is potentially treatable, there may be some good news ahead. If the CJD diagnosis is confirmed, there are clinical trials attempting to find successful treatment approaches. A good site to visit is the United States National Institutes of Health.
Lastly, as a caregiver you are undoubtedly under a lot of stress. A good source to provide support for you is the Family Caregiver Alliance. I am also a strong believer in palliative and hospice care. These great specialists not only help the patient, but the whole family. For further information, please go to the Web site of the National Hospice and Palliative Care Organization.
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