Paget’s disease can cause arthritis, pain, deformities and more

#MiddleburyCT #PagetsDisease #UrothelialCancers

DEAR DR. ROACH: My husband has been diagnosed with Paget’s disease. I would like to know more about it; this is the first time I have ever heard of it. I would like to know the treatments and how much damage affects the bones. He is 76, had prostate cancer and underwent radiation treatment. – C.W.

ANSWER: Paget’s disease of the bone is a common disease in people of European ancestry, with a lifetime prevalence of approximately 3%, although it remains undiagnosed in many. The cause of the disease appears to be a defect in bone reabsorption by the osteoclast, a specialized cell that normally helps maintain bone health. Paget’s disease is not a cancer. It is normally diagnosed over the age of 55.

Most cases of Paget’s disease are asymptomatic; however, without treatment, some people will develop arthritis, pain, deformities and fractures. The bone deformities may lead to nerve compression and, in the case of the tiny bones in the ear, hearing loss. X-rays and a high alkaline phosphatase (ALP) blood test are suggestive of the diagnosis. A bone scan is recommended to see the extent of damage from the disease.

Fortunately, highly effective treatments are available. Bisphosphonate drugs, the same drugs that are used to treat many people with osteoporosis, are very effective. A single treatment with zoledronic acid is effective at stopping (not reversing) the progression of the disease, and fewer than 20% of people will need a second dose. Diagnosing and treating early are ideal.

I don’t think his prostate cancer or radiation treatment has anything to do with his diagnosis of Paget’s.

DEAR DR. ROACH: I’m a 76-year-old male who was recently diagnosed with transitional cell carcinoma of the ureter, but I don’t have a history of smoking or any occupational exposure to chemicals, plastics, rubber or dyes. My cancer was found through a CT scan with contrast dye after having asymptomatic hematuria. It’s isolated to the proximal ureter and a kidney. How rare is that?

I have to have the kidney and ureter removed. Can you talk about living with one kidney and the adjustments that I’ll have to make? – R.P.

ANSWER: Urothelial cancers (the new name for transitional cell cancers) come from the lining of the urinary tract and can be present in the kidneys, ureters (the tubes that carry urine from the kidneys to the bladder), bladder and urethra (the tube that carries urine outside of the body). They are not common, about one per 100,000 people per year.

About 17% of people with cancer in the ureter will also have cancer in the bladder at the same time, and almost half will develop cancer in the bladder later in life. Careful screening, such as a look inside the bladder every six months, is essential.

You are correct that smoking and certain industrial exposures are risk factors for developing these cancers, but so is the place of origin; cancer syndromes such as Lynch syndrome; other toxins such as the drug phenacetin; and traditional Chinese herbs containing aristolochic acid. For localized diseases, complete removal is the safest approach, since there may be multiple small areas affected by cancer.

Living with one kidney is not that different from living with two, as the other kidney somewhat compensates for the missing one. Some medications may need to have lower doses, but usually, this is not the case.

Dr. Roach regrets he is unable to answer individual questions, but he will incorporate them in the column whenever possible. Readers may email questions to ToYourGoodHealth@med.cornell.edu.

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