Symptoms Of Pagets Disease
The symptoms of Pagets Disease can be overlooked or confused with arthritis.
Pagets disease (also known as osteitis deformans) is a chronic disorder that typically results in enlarged and deformed bones and osteoporosis.
Bone is living tissue that is continually renewed or remodeled... a process which removes old bone and replaces it with new bone. Paget's disease (of bone) disrupts this process by breaking old bone down faster than new bone can be built. Over time, the body responds by generating new bone at a faster than normal rate which results in softer and weaker bones that can lead to bone pain, deformities, fractures and osteoporosis. Symptoms of Paget's disease usually occur in people over 40 years old and appear in men more commonly than women.
Scientists haven't identified a cause of Pagets disease of bone, though they have discovered several genes that appear to be linked to the disorder. Some scientists believe the disorder is related to a viral infection in the bone cells that may be present for many years before symptoms of Pagets disease appear. Hereditary factors seem to influence whether you're susceptible to the disease.
After the age of 40, siblings and children of someone with Pagets disease may wish to take an alkaline phospatase blood test every two or three years. If the alkaline phosphatase level is above normal, other tests such as a bone-specific alkaline phosphatase test; bone scan; or x-ray can be performed.
SYMPTOMS OF PAGETS DISEASE
Many patients do not know they have Pagets disease because they have a mild case without symptoms or they confuse the symptoms with those of arthritis or other disorders. Following is a list of some of the symptoms of Pagets Disease:
- Bone pain is the most common symptom and can occur in any bone affected by the disease. It often localizes to areas adjacent to the joints.
- Neurological problems- such as hearing loss, headache and rarely, vision loss
- Pressure on a nerve can also cause numbness, tingling, weakness, hearing loss and double vision.
- Hip pain may occur when Pagets disease affects the pelvis or thighbone.
- Teeth may spread due to pressure on the anterior teeth.
- Increased head size, bowing of limb, or curvature of the spice may occur in advanced cases.
In most cases, the symptoms of Pagets disease increase slowly. The disease can be managed effectively in nearly all people and is rarely fatal. Possible complications include:
- Osteoarthritis. This degenerative joint disease is a common long-term complication of Pagets disease.
- Heart failure. Unusually extensive Pagets disease may force your heart to work harder to pump blood to the affected areas of your body. In people with pre-existing heart disease, this increased workload can lead to heart failure.
- Sarcoma. A rare complication is a bone cancer known as sarcoma, also called osteosarcoma or osteogenic sarcoma, which may develop in bones affected by Pagets disease. This complication occurs in less than 1 percent of people with symptoms of Pagets disease and usually doesn't develop until many years after the onset of Pagets.
The goal of treatment for Pagets disease is to relieve bone pain and prevent the progression of the disease. The bisphosphonate class of drugs is used most often to treat the condition but should be taken only under a doctor’s advice. Worsening of kidney function, which may progress to kidney failure, has been reported with drugs of the bisphosphonate class.
In Canada and the US, the drug most commonly used to treat Pagets bone disease is Didronel® which is produced by Proctor and Gamble (Didrocal® if supplemented with calcium carbonate)- also known as Etidronate in its generic form.
In 2007, Proctor and Gamble recommended the following treatment regime for Pagets disease:
- Initial Treatment Regimen
- 5 to 10 mg/kg/day-not to exceed 6 months, or
- 11 to 20 mg/kg/day- not to exceed 3 months
Doses above 10 mg/kg/day should be reserved for when:
- lower doses are ineffective, or
- there is an overriding need to suppress rapid bone turnover (especially when irreversible neurologic damage is possible) or reduce elevated cardiac output.
Doses in excess of 20 mg/kg/day are not recommended.
Retreatment should be initiated only after:
- a Didronel-free period of at least 90 days, and
- there is biochemical, symptomatic or other evidence of active disease process.
Doctors are advised to monitor patients every 3 to 6 months, although some patients may go drug free for extended periods. Retreatment regimens are the same as for initial treatment. For most patients the original dose will be adequate for retreatment. If not, consideration should be given to increasing the dose within the recommended guidelines.
The response to therapy with Didronel is often slow but will continue for months after therapy is discontinued, so dosage should not be increased prematurely. In many patients, the disease will be suppressed for at least 1 year following cessation of therapy. The upper limit of this period has not been determined.
Method of Treatment
Didronel is taken by mouth on an empty stomach two hours before meals. To maximize absorption, patients should avoid taking the following items within two hours of taking the medication:
- Food- especially food high in calcium, such as milk or milk products.
- Vitamins with mineral supplements or antacids which are high in calcium, iron, magnesium, or aluminum.
If stomach upset occurs, it may be relieved somewhat by taking divided doses during the day instead of one daily dose.
ZOLEDRONIC ACID (ACLASTA/RECLAST)
Both Health Canada and the USFDA have approved Zoledronic Acid (Aclasta/Reclast) for the treatment of the symptoms of Pagets disease of bone. Zoledronic acid is provided through a 5 mg infusion once a year given intravenously over no less than 15 minutes. (When used to prevent osteoporosis in postmenopausal women, a 5 mg infusion is given once every 2 years.) It is also recommended that patients with Pagets disease receive 1500 mg elemental calcium and 800 IU vitamin D daily- particularly during the 2 weeks after dosing.
Health Canada warns that the use of Aclasta is not recommended in patients with severe impairment in kidney function.
DIET AND EXERCISE
People with Pagets disease (and especially those taking bisphosphonates) should ensure that they receive the recommended levels of bone building minerals and vitamins and have sufficient exercise. Reliance on medication alone is discouraged.
For less than 10 cents a day, Puritan's Pride Bone Care will provide the calcium, vitamin D, magnesium and vitamin K needed to build healthy bones.