sábado, 2 de noviembre de 2019

Treatment of Paget’s disease of the bone

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By Dr. Ananya Mandal, MDReviewed by April Cashin-Garbutt, MA (Editor)

Paget’s disease is a bone disorder that is commonly inherited genetically. This said, there is evidence that environmental factors play a role in development of the condition.

Who treats Paget’s disease?

The team of doctors and health care providers involved in treatment of Paget’s disease include:

Rheumatologists who deal with arthritis and other joint problems
Orthopaedic surgeons who deal with bone problems
Neurologists who deal with nerve problems
Endocrinologists who deal with hormones
Otolaryngologists or ENT (Ear nose and throat) surgeons

The team includes a physical therapist and a genetic and psychological counsellor. Occupational therapists may also be included in the team.

Treatment of the condition may include non-surgical therapies, periodic X-rays and so forth. (1-7)

Non-surgical or medical therapies

These therapies are targeted towards decreasing symptoms of the disease. This includes alleviation of bone pain. Medications, however, fail to reverse the effects on the bone.

For patients who have mild pain from the disease or from arthritis associated with it, nonsteroidal anti-inflammatory drugs (NSAIDs) and aspirin can be very helpful.

Regular monitoring of the disease

Regular observation or monitoring of disease progression is recommended for most patients. Periodic X-rays may be recommended for these patients.

Walking aids

Those with pelvis or leg involvement may be provided with walking aids or a cane to prevent falls and risk of fractures as well as decrease pain and improve mobility.

The decrease in pressure over the bone also leads to a lessened risk of bowing or bending of the leg bones. Braces may be prescribed to prevent dis-alignment of the bones.

Bisphosphonates for severe cases of Paget’s disease

For more severe cases medications of the class bisphosphonates are the treatment of choice. These drugs act by blocking the osteoclasts and prevent bone breakdown caused by them.

There are several types of bisphosphonates where some are taken orally or as pills and some are given by injection (intravenous form).

Alkaline phosphatase levels should be checked periodically while taking these medications as they lead to decrease in levels of serum Alkaline phosphatase.

Drugs used include Alendronate and Etidronate to be taken by mouth every day for 6 months. Tiludronate and Risedronate are also taken as pills daily for 3 months and 2 months respectively. These pills need to be taken with a large glass of water first thing in the morning and the patient is advised to remain upright for the next 30 minutes and not eat until that time has passed.

Side effects of these medicines include heartburn and a short duration of bone pain.

Injections of bisphosphonates include Pamidronate given once a month or once every few months. It is given as an infusion into the veins over a few hours.

Zoledronate is given as injection in the vein once a year over 30 minutes. Calcitonin is a hormone that is injected under the skin several times a week. This is a hormone made by the thyroid gland. This is used rarely now.

Surgical therapy for Paget’s disease

Surgical therapy is used mainly to treat the complications of Paget's disease. It is necessary if there are fractures, compression of surrounding nerves, severe arthritis or alterations in bone alignment.

Compression of vital nerves especially in the spine and skull require surgery as early as possible to prevent paralysis or other life threatening complications.

Before surgery the patient is treated with bisphosphonates that reduce the risk of excessive bleeding during surgery.

If the Paget’s disease has progressed to cancer or Paget’s sarcoma, surgery is performed to remove the entire tumor. Cancer chemotherapy and radiation therapy may also be used in these cases.

Immobilization and physical therapy

Bones affected by Paget's disease may take longer to heal than normal bones. After fractures or a surgery a longer period of immobilization and physical therapy may be necessary for healing.

Living with Paget’s disease

Usually living with Paget’s disease is not complicated and it does not affect the quality of life seriously if treated early. While this is a chronic condition, it does not spread to new bones.

Diet and Paget’s disease

People with Paget’s disease do not need a special diet. They need 1,200 mg of calcium and at least 400 IU of vitamin D every day.

After age 70 the person should take 600 IU of vitamin D each day. Those with kidney stones need to consult their physicians before taking supplements.