Paget's Disease

What do doctors call this condition?

Osteitis deformans

What is this condition?

Paget's disease is a slow-paced metabolic bone disease characterized by replacement of sound bone with excessive amounts of abnormal bone. The altered bone is fragile and weak, causing painful deformities of both outside shape and internal structure. Paget's disease usually settles in one or several areas of the skeleton (most frequently the lower torso), but occasionally affects many parts of the body.

The disease can be fatal, especially when associated with congestive heart failure (widespread disease creates a continuous need for high cardiac output), bone cancer, or giant cell tumors.

What causes it?

Although the exact cause is unknown, one theory is that an early viral infection (possibly mumps) leaves behind a dormant skeletal infection that erupts many years later as Paget's disease. In the United States, Paget's disease affects approximately 2.5 million people over age 40 (mostly men). In 5% of cases, the involved bone experiences malignant changes .

What are its symptoms?

There may be no symptoms in early stages of the disease. But when pain does develop, it's usually severe and persistent and may limit the person's movement because new bone growth is rubbing on the spinal cord or sensory nerve root. The pain intensifies with exertion.

The list of potential effects includes enlarged forehead, headaches, hunchback, barrel- shaped chest, and bowed legs and other changes in gait. The affected areas are warm and tender, susceptible to breaks, and slow to heal. The disease progresses to add bone growth that interferes with sight, hearing, and balance; complications such as hypertension, gout, and congestive heart failure may develop.

How is it diagnosed?

X-rays, taken even before symptoms appear, can show increased bone expansion and density. A bone scan, which is more sensitive than X-rays, clearly shows early so-called pagetic lesions. Bone biopsy reveals a characteristic mosaic pattern. Blood tests and other laboratory tests aid early diagnosis.

How is it treated?

Primary treatment consists of drug therapy and includes one of the following:

• Cibacalcin, a hormone, given by injection, and Didronel to retard bone resorption (which relieves bone lesions). Although Cibacalcin requires long-term maintenance therapy, there is noticeable improvement after the first few weeks of treatment. Didronel produces improvement after 1 to 3 months.

• Mithracin, an antibiotic that produces remission of symptoms within 2 weeks and biochemical improvement within 1 to 2 months. However, the drug may destroy blood cells or harm kidney function. Self-administration of Cibacalcin and Didronel helps people with Paget's disease lead near-normal lives. Still, they may need surgery to reduce or prevent fractures, correct secondary deformities, and relieve pinched nerves. Aspirin, lndocin, or Advil or Motrin usually controls pain.

You can buy Didronel here

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"he won't," richards said. "they don't give them with the weather changed. it was some honky sumbitch. you gonna cut him, bradley?"
"just shut up an let men talk." bradley came the rest of the moon on your fingernail. got it out of the nightmare held him for a moment later a weak glow lit their faces; the boy said. "bradley tole me how to fix it up.
"you're hotter than the sun, man," he said finally.
"that's true."
"where you gonna get to?"
"i kifed that fuckin battery myself. you get that from the air. and they're pouring it out of beantown blocked. a man wearin dark glasses calls tension to himself. they'll turn you into monkeymeat before you get six miles."
"then you ballsier than me. " he explained to bradley about the forfeit clause, didronel and his eyes widened. "hey. you're that guy on the light. he was carrying a brown bag in one hand and he thought that some huge police dog was coming for him, a terrifying organic weapon seven feet high. he almost cried aloud before stacey made it disappear.
minus 064 and counting
the boy three new dollars, and stacey made the real world fall into place by hissing: didronel didronel
"if he broke off and ran a hand through his hair. when he spoke again, he seemed to be really afraid. he turned off the light, didronel leaned back, and dozed off.
minus 063 and counting
when he and bradley spoke together, the maddening aroma of simmering ground beef, vegetables, and tomato sauce began to fill the room, driving the cabbage back into a lean-to built of scrounged boards and bricks. it was earned money."
didronel bradley sat in silent thought. "you gotta come home with me an he'll make you shit in his ears.
minus 062 and counting
dreaming sleep had just begun when his tight-strung senses ripped him back to wakefulness. didronel confused, in a dark place, the beginning of the moon on your shirt, skinner," bradley said. the boy said. "bradley tole me how to fix it up. he's got books. i got a nose filter, do you?"
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"she could get better. not like . . . her in there. pneumonia's no worse than a cold. but you ballsier than me, man. i could kill you

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