Doc: No effective treatment for Creutzfeldt-Jakob
Dear Dr. Roach: My 58-year-old daughter has Creutzfeldt-Jakob disease. She started to have memory problems in April 2015. I know this disease is rare, and I can’t find out much about it. Most people and even some doctors have never heard of it. We know she doesn’t have long to live, because she can’t feed or take care of herself. Needless to say, I am heartbroken. Is this something her children should worry about? I hope to learn more about this terrible disease.
Dear B.R.: I am very sorry to hear it. Of all the many diseases, physicians fear the degenerative neurological diseases the most. Creutzfeldt-Jakob disease comes in several forms; 90 percent of the time, it is sporadic Creutzfeldt-Jakob disease, caused by a genetic mutation that causes accumulation of a prion protein in the brain. This prion is infectious. In the past, improper sterilization and transplants led to transmission of the disease.
Transmission of prion protein seems to be the cause of variant Creutzfeldt-Jakob disease, of which there are about 225 reported cases in the world’s literature, mostly from the U.K., and probably related to bovine spongiform encephalopathy, so-called mad cow disease. It is believed that no cases of variant Creutzfeldt-Jakob have been acquired in the U.S.
There is a familial type of Creutzfeldt-Jakob disease, reported most commonly in Libya, Chile and Hungary. It is caused by a different gene mutation, and is distinguished molecularly. There is usually a family history.
Sporadic Creutzfeldt-Jakob disease shows rapidly progressive neurologic damage, including memory, behavior and abnormal movements. There is no effective treatment, and death usually occurs within a year of symptom onset.
More information is available at ninds.nih.gov/index.htm by typing Creutzfeldt-Jakob disease in the search box.
Dear Dr. Roach: I am trying to reduce or eliminate my use of Nexium, which I have been taking for about 10 years. I have prescription-strength 40 mg, and I have purchased over-the-counter 20-mg capsules. My intent is to take 40 mg one day and 20 mg the next and so on for a while, then go to all 20 mg every day, then take the 20 mg every other day. Depending on how I react, I will stop taking it altogether. My doctor is in support of me trying to reduce or stop taking this medication.
My question is: The drug sheets issued with the 40-mg capsule say to take the medicine and wait 60 minutes with no food. The OTC information leaflet that is included with the capsules say to take the medicine first thing in the morning, and does not indicate any need to wait before eating. Why is there a difference in these two instructions on taking Nexium? I need to know if I can just take the capsules when I get up in the morning before I eat my breakfast and not worry about waiting an hour to eat, which I find hard to do.
Dear B.G.: Tapering down the dose when trying to stop a proton pump inhibitor like esomeprazole (Nexium) is an excellent idea, as there can be worsening of symptoms if suddenly stopped. Your plan for tapering is just what I tell my patients.
Food slows and reduces the absorption of Nexium by about 50 percent. This may not be enough to make a difference in symptoms, but you should be aware of it as you are tapering off the medication.
Email questions to ToYourGoodHealth@med.cornell.edu.