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Osteitis deformans
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This disease is characterized by periods of excessive bone destruction, followed by excessive bone formation. Paget's disease slowly causes its damage over the years, resulting in bones that are dense, enlarged, deformed, and fragile.
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- Can have little or no symptoms
- Bone pain -- may affect any bone.
- Headache and hearing loss if the bones of the skull and ear are involved
- Skull may enlarge
- Frequent fractures
- Hip pain, thigh pain
- Back pain
- Deformed spine -- destruction of the vertebral bones and cartilage (rubbery material between bones) of the backbone or spine (kyphosis).
- Bowed legs -- refers to the shape the legs take after the tibia and femur bones are affected
- Collapse of the vertebral bones of spine may put pressure on the nerve roots and cause tingling electric sensations in fingers and toes, numbness, and muscle weakness.
- When bones enlarge, they can put pressure on the blood vessels and nerves of the brain, spine, and eyes.
- Problems with vision
- Teeth may become loose.
- Heart Failure --
as Paget's advances, the heart has to work harder to pump blood to the enlarged bones. Symptoms of Heart Failure often occur if there is other underlying heart disease present. These include shortness of breath, fatigue, and swollen ankles.
- Kidney Stones --
can cause Blood in the Urine and severe mid back pain.
- Sudden bone pain in Paget's patient may be due to sarcoma (type of Bone Cancer) that is sometimes seen in Paget's disease.
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- The cause is unknown.
- Genetics -- abnormalities of genes (proteins that make DNA) on chromosome number 18 is being researched as a cause.
- Infection -- virus involvement is being researched.
- Possibly a Slow Virus infection in those with the abnormal gene for Paget's disease
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- Your general physician may request help from variety of specialists such as an endocrinologist, rheumatologist, orthopedic surgeon, neurologist, or an otolaryngologist.
- Full medical history -- symptoms (pain, fractures, etc.), illnesses, surgeries, medications, habits, allergies, family history
- Physical signs of symptoms:
- Deformed spine or bone
- Increased temperature over the affected bone can sometimes be felt.
- Scalp may show enlargement over the forehead and temples.
- The head circumference may be measured and if > 55 cm, it is enlarged.
- The legs and thighs have a bowed appearance.
- The doctor may hear a whooshing sound (bruit) over the enlarged bone. This is the sound of excess blood traveling through the bone.
- Eyes -- vision assessed by using special charts.
- Changes called angioid streaks, optic atrophy, or retinitis pigmentosa are seen in the back of the eyes of some patients by using a device called an ophthalmoscope.
- Hearing is tested and if abnormal, the patient is sent for a full hearing test by an audiologist.
- The penis may have an abnormal shape (Peyronie's Disease).
- Urine sample may be tested for presence of blood if Kidney Stones are
suspected. Also, for pyridinoline cross links, usually high in Paget's.
- Blood samples are analyzed for kidney and liver, and a Complete blood count (white and red blood cells) is done.
- Blood levels of a chemical called Alkaline phosphatase are often increased.
- Blood Calcium levels are rarely increased.
- Blood is sent for BGP (osteocalcin) -- a chemical released by the cells (osteoclasts) that break down the bones. BPG levels are often high in Paget's.
- X-Rays -- fractures, bone enlargement, and areas of bone destruction
- CAT scan and MRI provide detailed views of the bones.
- Bone scans -- done by injecting radioactive dye into the blood followed by X-Rays.
- Bone biopsy --
sample or a piece of the bone is surgically removed and examined. Biopsy is only needed if all other tests have failed in diagnosing Paget's.
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- Both sexes affected equally.
- Family history -- in a first-degree relative (parent, offspring, siblings) increases the risk by 10-40%.
- Usually after age 50, but may occur earlier
- Caucasian
- Rare in African descent
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- Devices -- splint, hearing aid, walkers, canes, and wheelchairs may all be helpful.
- Physical and occupational therapists can teach coping techniques and provide tips on devices and exercises that may improve the quality of life.
- Good diet, exercise, and weight control is a must.
- Surgery -- fracture repair, joint replacement, repair of deformed bone, and relieving pressure of nerves and other structures.
- Medications:
- Patients may need Calcium supplements and vitamin D and sunshine (consult with doctor).
- Bisphosphonates -- these stop bone destruction and include Didronel, Fosmax, Skelid, Actonel, Aredia, and other drugs taken for 2-6 months at a time. The bisphosphonates are used carefully in those with kidney disease.
- Calcitonin injections such as Miacalcin given 3 times a week by the patient using a small needle under the skin for 1.5 - 3 years
- Aspirin, Celebrex, Motrin, Tylenol, and Codeine can help with pain.
- The above drugs can be used in combination.
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Contact your doctor for treatment. Do not lose hope because there is a lot you can do to keep Paget's under control until a cure is found. You can get more information by calling the Paget's Foundation in the United States (212- 509-5335).
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