What is Paget’s Disease?
Paget’s disease of the bone, or osteitis deformans, is a chronic skeletal disorder which may result in enlarged and deformed bones in one or more regions of the skeleton. Excessive bone breakdown and formation can result in bone that is dense but fragile.
What causes Paget’s disease?
The cause of Paget’s is unknown. Recent studies, however, have suggested that the disease may be known as a “slow virus”, a virus that is dormant for many years before symptoms appear. This theory is based on the fact that bone cells in Paget’s disease have structures that make them look like cells in a virus disorder.
Which groups of people are usually affected by Paget’s disease, and how common is the disease?
Paget’s disease is common in people of Western European descent. Highest prevalence rates are found in Western Europe, The United States, Canada, Australia and New Zealand. Paget’s disease is rare in people under 40 but occurs in 1 to 3 percent of population over 65.
What are the symptoms?
Pain (including bone pain, pain in the joints and back pain) is the most common symptom. Headaches and hearing loss are common symptoms when Paget’s disease affects the skull. Deformities which may consist of an increase in head size, bowing of the limbs and curvature of the spine are less common symptoms.
The disease may first be noticed in the bones of the leg, the pelvis, the spine, or the skull. When the skull is affected, the head slowly enlarges and the patient may have difficulty finding a hat which fits. Affected leg bones tend to buckle under the weight of the body and become bowed. When the spinal column is involved it is not unusual for a person to shrink in height as the disease progresses. A painful disability of the hip joint can ensue when the pelvis is involved. Although bone pain and deformity are the most typical symptoms, other complications such as deafness, susceptibility to fractures, and neurological problems may occur.
Do most people who have Paget’s disease know that they have it?
Many people who have a mild form of Paget’s disease are unaware that they have it. Often those who have symptomatic Paget’s disease are unaware that they have it because an accurate diagnosis has not been made and their symptoms are attributed to arthritis.
How is Paget’s disease usually discovered?
Paget’s disease is often diagnosed by chance when a person has a routine X-ray or laboratory test for another reason. X-ray changes are usually characteristic of Paget’s disease. Serum alkaline phosphatase, a component of most laboratory blood tests, is usually elevated, sometimes dramatically, when Paget’s disease is present.
How can the diagnosis of Paget’s disease be established?
The diagnosis of Paget’s disease is most often determined by X-rays, and the accompanying blood tests. Very rarely a bone biopsy is needed.
What does it mean when the alkaline phosphatase level is higher than the normal range?
The increase in serum alkaline phosphatase gives an indication of the activity of the changes that are going on in the affected bones. Having an increased level of alkaline phosphatase in the blood is not harmful in itself. It is not like having high levels of sugar or cholesterol in the blood, for example.
An alkaline phosphatase test is only a helper. The doctor, therefore, measures blood alkaline phosphatase level as part of the evaluation to get an overall impression. The test may be repeated from time to time to see how the patient is doing. A high alkaline phosphatase level by itself is not necessarily a reason for treatment.
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Do the symptoms of Paget’s disease continually worsen?
Not usually, but a slow progression of symptoms may occur. The outlook for patients with mild to moderate Paget’s disease is good, since in such cases the disease progresses very slowly, if at all. Even in cases of severe or widespread disease, early recognition and treatment can bring about a positive response in most cases. Even though Paget’s disease is a chronic condition, through the proper use of medications, the disease may be controlled.
What is the prognosis for patients with Paget’s disease?
In the great majority of patients the prognosis is good, particularly if the treatment is given to patients before major changes have occurred in important bones such as the skull, the vertebrae and the bones of the lower extremity.
Is Paget’s disease a fatal disease?
Paget’s disease is rarely fatal. Osteogenic sarcoma, a form of bone cancer, an extremely rare complication of Paget’s disease, occurs in less than 1 percent of all patients.
Which bones may be affected by Paget’s disease?
Virtually every bone in the body is susceptible to Paget’s disease including the tiny bones of the middle ear. Paget’s disease most frequently occurs in the spine, skull, pelvis and bones of the lower extremity (thighs and lower legs).
Most patients have only one affected bone, while others may have two, three or more affected bones.
If Paget’s disease is present in one bone, does this mean that the disease will usually spread to other bones?
It is extremely rare for Paget’s disease to develop in adjacent or distant bones after the initial diagnosis of the disease is made
TREATMENT
Which types of physicians are specialists Paget’s disease?
Endocrinologists (physicians who specialize in hormonal and metabolism disorders) and rheumatologists (physicians who specialize in joint and muscle disorders) are most often expert in caring for Paget’s disease.
Also, orthopaedic surgeons, neurosurgeons, and otolaryngologists (physicians who specialize in ear, nose, and throat disorders) are the surgical specialists most likely to be called upon to evaluate and treat patients with Paget’s disease.
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Can Paget’s disease be cured?
At present there is no cure for Paget’s disease; however, treatment is available not only to relieve the pain and discomfort of the disease but to suppress the disease and therefore retard progression.
How is Paget’s disease treated?
Four different perception drugs are currently available. Synthetic salmon calcitonin (Calcimar) and synthetic human calcitonin (Cibacalcin) are hormones taken by self injection. Disodium etidronate (Didronel) is administered orally. Plicamycin (Mithracin) is an intravenous cancer drug which is occasionally used for difficult cases.
Particular care should be taken by patients receiving Didronel to take the medication correctly. Didronel should be taken once daily on an empty stomach with water. All tablets are taken at the same time. No food or antacid should be taken within two hours after taking Didronel. Moreover, a given treatment course of Didronel should never exceed six months.
Not everyone diagnosed as having Paget’s disease of bone requires treatment with these medications a physician experienced in treating the disease should determine which, if any, treatment to use and should supervise its administration.
New therapies are being developed which will be helpful in treating Paget’s disease. Some of these therapies will be available during the 1990’s.
When is surgery recommended for Paget’s disease?
There are generally three situations when surgery may be recommended. The first is when fractures occur in Pagetic bone. Surgical fixation of Pagetic fractures does not speed healing of bones, but may allow the fracture to heal in better position and thereby abolish the necessity for long-term immobilization which can lead to the development of osteoporosis. The second situation is when the patient develops severe degenerative arthritis. The hips and knees are the most commonly involved joints. If medication and physical therapy are no longer helpful, and if the disability is severe, surgery may be considered as an option. Total joint replacement of the hips and knee is most often reserved for the most severe case of arthritis where other methods are no longer effective. The third situation involves bone deformity, especially of the tibia. The surgical cutting of the bone (osteotomy) may be part of a procedure to realign painful weight bearing joints, especially the knee. Osteotomy of the tibia has proven to be a relatively safe and effective procedure. As with total joint replacement, osteotomy is rarely “necessary” but is an “option” to be considered. Surgery is rarely necessary, but can offer a welcome opportunity for relief of pain and return of function when nonsurgical treatments prove less effective. Pre-treatment with calcitonin or diphosphonates has helped tremendously in decreasing intraoperative bleeding and complications. If you are having surgery and your physician has not discussed pre-treatment with you, be sure to ask about this.
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When is a neurosurgeon needed?
Overgrowth of the skull, although rarely, lead to partial collapse of the skull onto the spine. This may be associated with injury to the brain. In such rare cases neurosurgeons may be needed to carry out surgery to correct the disturbances in brain function. Similarly, a bone in the mid-spine area may overgrow and, rarely compress the spinal cord, requiring surgical attention. More commonly, overgrown bone of the lower spine can irritate nerve roots and cause back pain in the back or legs; this condition may also require surgical attention.
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PAGET’S DISEASE AND OTHER MEDICAL CONDITIONS
What is the relationship between arthritis and Paget’s disease?
Paget’s disease can cause arthritis by changing bone around the joint.
1. Long bones such as in the thigh or leg may become bowed. This distorts the normal dynamics and pressures within the adjacent joints
2. Pagetic bone may become enlarged. If enlargement is uneven, the joint surface has uneven pressures.
3. If the enlargement of Paget’s bone is even, it changes the sizes of the joint surface and again causes abnormal pressures to these surfaces.
4. Bone involved with Paget’s disease is often soft and may bend. The hip joints may push the soft pelvis inward causing the person to stand bent forward with the hips not fully straight.
The pain of osteoarthritis is a very common cause of pain in Paget’s disease of the bone. Back pain which is a very common reason for people with Paget’s disease to consult a physician, is most often related to osteoarthritis or other causes and is seldom due to the Paget’s disease alone. Osteoarthritis of the back may be caused by Paget’s disease.
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Why do some Paget’s disease sufferers experience more pain at night or first thing in the morning?
Pain which arises directly from Paget’s disease is usually relatively mild and constant. More severe pain usually means that arthritis is present. At the end of an active day, the joints may be more painful. Also the pain and stiffness may be worse in the morning in some arthritic patients.
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Why do some Paget’s disease sufferers experience loss of hearing? Can anything be done to remedy this condition?
If Paget’s disease affects the temporal bone, that is, the bone surrounding the inner ear, severe and progressive loss of hearing may occur. This may involve both sides or one side predominantly. If the loss of hearing is progressive and due to Paget’s disease, treating the underlying Paget’s disease with available anti-pagetic therapies, may slow the progression of hearing loss.
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How does Paget’s disease affect the teeth?
Though Paget’s disease usually does not affect the facial bones, in severe Paget’s disease the bones around the teeth may be affected and cause loosing of the teeth, disturbance of the chewing mechanism and increased susceptibility to infection. In such cases, careful and thorough dental care is needed.
What are the possible connections between Paget’s disease and heart disease?
In patients with extensive Paget’s disease, the heart has to work harder to pump extra blood to affected bones. This could result in heart failure and even heart attacks in people who already have heart disease such as arteriosclerosis
What are the possible connections between Paget’s disease and diabetes?
There is no known relationship between Paget’s disease and diabetes. The medications for each condition do not interfere with the other disorder. However if a diabetic patient is taking insulin injections. Insulin and calcitonin should not be mixed in the same syringe.
What are the possible connections between Paget’s disease and kidney problems?
There is no known relationship between Paget’s disease and kidney problems except for the suggestion that kidney stones may be somewhat more common in patients with Paget’s disease. The reason for this may be that some patients with Paget’s disease have too much uric acid or calcium in their urine. Treatment of Paget’s disease may reduce urinary calcium but will not reduce uric acid.
HEREDITY AND PAGET’S DISEASE
Is Paget’s disease hereditary?
The answer to this question is not completely known, and this area is the subject of active research. The information currently available strongly suggests that Paget’s disease may be inherited but this is not yet proven.
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If someone in your family had or has Paget’s disease, what should you do, as you grow older, to determine if you have Paget’s disease?
It is suggested that after the age of 40 siblings and children of someone with Paget’s disease may wish to have an alkaline phosphatase blood test check every two or three years. If the blood result is elevated, appropriate tests to look for evidence of Paget’s disease in the skeleton (such as X-rays or bone scans) can then be performed. If Paget’s disease is found in this way, very early on, treatment can be started early in the hope of preventing possible problems from developing at a later time. For this reason, screening the 40 year old siblings and children of families in which Paget’s disease exists may be advisable
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MISCELLANEOUS
Is there a relationship between diet and Paget’s disease? Specifically, is there a relationship between calcium and/or vitamin D and Paget’s disease?
There is no specific relationship between diet and Paget’s disease. In general, people should receive the nutritionally proper amount of calcium (1000-1500 mg daily) and adequate sunshine or 400units of Vitamin D daily to maintain a healthy skeleton. Life-long low intake of calcium and Vitamin D can produce generalised bone disease and worsen Paget’s disease.
Is exercise recommended for Paget’s disease sufferers?
Exercise is very important in maintaining skeletal health and is thus highly recommended for patients with Paget’s disease. Exercise is also helpful in avoiding weight gain and in maintaining the mobility of the joints. Before beginning any exercise program it is wise to discuss the program with your physician.
*Source: www.dircsa.org.au/docs/Pagets.htm
