A B C D E F G H I J K L M N O P R S T U V W

PAGET'S DISEASE OF BONE

What is Paget's Disease of the Bone ?

Bone consists of a meshwork of protein. On the protein strands, we find calcium salts which give the bone its strength. Bone is in a continuous state of change: new bone is laid down continuously. At the same time, existing bone is broken down. These two processes usually balance, so that the quantity of bone remains much the same. However, ageing produces a slight overall decrease in the strength of the bones. In Paget's disease this process goes wrong. The problem affects only a part of a particular bone which initially becomes soft and later thickened. When they are soft bones may bend (particularly affecting the thigh bone) and may cause pain, especially near joints, producing an arthritic-type of complaint. The thickening of the bone which occurs in some areas (such as the skull) produces deformity and may also press on certain nerves. This particularly affects the nerves of hearing so that deafness may develop.

How does Paget's Disease of the Bone occur ?

The cause of Paget's disease is not known. It appears to commence as excessive breakdown of one part of the bone. Although this may soften the bone in that area, new bone is deposited to make up for it. After a few years, the laying down of bone gets out of hand and the bone actually becomes thicker and swollen.

Why does Paget's Disease of the Bone occur ?

Some Paget's cases seem to run in families. It is usually a condition which occurs over the age of 50. Routine x-rays done on an assumed normal group of people, shows that about one in 10 have the disorder without knowing it. Only some of these affected people actually develop symptoms. There is nothing that could have been done to prevent the condition from occurring.

Treatment Involved for Paget's Disease of the Bone

Your doctor will do blood and occasionally urine tests to check the overall activity of your Paget’s disease. A bone scan (using a small amount of radioactive isotope) will accurately identify those areas of your skeleton which have been affected. If it was discovered purely accidentally while x-rays were being taken for another reason, then it may require no treatment. The likelihood of developing symptoms in such cases is probably very small indeed. If pain is present, simple painkillers may be all that is required. If the pain is continuous and interferes with work or enjoyment, various treatments are available for you and which will help to stop the abnormal removal and depositing of bone. Sometimes these drugs can be given by mouth and sometimes by injection. The most important group of drugs are called bisphosphonates. Some can be given by mouth (alendronate, risedronate, clodronate and tiludronate). The doctor will judge your response to treatment by assessing your symptoms, and by changes in the test results. Overall, the best results to date have been obtained with intravenous infusions of pamidronate. Treatment programmes require that the drug is infused daily for 1 to 2 weeks, or weekly over a period of 2 to 3 months. The drug calcitonin also benefits some people with Paget’s disease. It is usually given daily or three times a week, by injection or sometimes with a nasal spray.

During Treatment for Paget's Disease of the Bone

Response in terms of pain is different from person to person. Some people have immediate relief of pain. In others, there may be little response at all. A poor response to treatment is particularly likely to occur if the involvement by Paget's disease is near a joint. One of the drugs used in treatment (calcitonin) may produce nausea, vomiting and flushing together with tingling of the fingers. Some of these symptoms disappear as treatment continues. Some of the tablets (bisphosphonates) used for treatment of Paget's disease may also produce stomach and bowel disturbance. Although the drug pamidronate is an effective treatment, it can sometimes result in a loss of calcium from the unaffected bones of the skeleton. Taking extra calcium and vitamin D helps to prevent this. The drug tiludronate does not appear to share this problem.

After Treatment for Paget's Disease of the Bone

When a course of treatment is finished, pain may be much improved for as long as several months or even years. Further courses of treatment, are however, often necessary, and you should let the doctor know if pain returns. In any case, regular visits are suggested, so that you can be assessed using blood or urine tests. In rare cases, a growth may develop near an area of Paget's disease. Please notify the doctor of any gradually increasing swelling which is noted. Appropriate tests can then be done.

If Paget's Disease of the Bone is Left Untreated

Without treatment, both deformity and pain tend to get worse. However, in some people, the condition appears to burn out. Pain improves, but any deformity remains. There is no evidence that using any of the treatments which are currently available actually reduce the likelihood of fracture. People with Paget's disease need to be careful about placing sudden severe strain on any bone which is affected by the disorder.

Effects on Family of Paget's Disease of the Bone

Although the condition may run in families, it is not usually considered worthwhile screening other members for this condition.