Paget’s disease, also known as osteitis deformans, is an uncommon, chronic bone disorder that occurs in about 1% of the elderly population, in the US and other countries like India, China and Scandinavian nations. It is rather more common in certain geographical locations like England and Western Europe.
The disease causes the bones to grow larger and weaker than normal. After osteoporosis, it is the second type of bone disease that is fairly common in the elderly population. It may affect one or more bones, but does not spread from affected bones to the other bones in the body. It hits the elderly individuals who experience rapid isolated bone repair that cause a variety of symptoms from having softer bones to enlarged bone growth. The bones that are typically hit are those of the pelvis, spine (low back), hips, thighs, skull and arms.
Symptoms – In most cases, Paget’s disease is so mild that it does not cause any other complications. In fact, about 80% patients have no symptoms and are diagnosed with the disease after getting an x-ray performed for an unrelated reason. Most of the times, the symptoms are confused with those of arthritis.
Still the other common symptoms of the disease are:
a) Bone pain – that can occur in any bone affected by the disease or results from arthritis that develops in some patients. It often affects areas near joints, mainly the pelvic muscles and can also cause some damage to the cartilage of joints.
b) Headaches and hearing loss – can occur when the Paget’s disease affects the skull. An overgrowth of bone in the skull can cause hearing loss or headaches.
c) Pressure on nerves – can occur when the disease affects the spine. If the spine is affected, the nerve roots can become compressed. It can lead to curved spine, back pain or damage to the nerves thereby causing problems viz. tingling/numbness in the arm or the leg.
d) Hip pain – The disease often affects the pelvic muscles and the thigh bones.
e) Increased head size, bowing of limb, curvature of the spine may occur in the advanced cases of Paget’s disease.
Apart from all the above-mentioned symptoms, the most serious complication of the Paget’s disease is the bone cancer.
Causes of Paget’s disease: Bone is a living tissue that is engaged in a continual process of renewal. It continues to grow even after the individual attains a full height. During this process of renewal or remodeling the newer bones replace the old ones. When this process becomes abnormal, that is a sign of Paget’s disease. The exact cause of the disease is not known yet, but there are several genetic and environmental factors that cause it.
- Genetic factors – Up to 30% of people with the disease are more likely to have a parent, a sibling or some other family member also affected by the disease. People who inherit mutated genes from their parents are likely to have the disease too. According to a recent study, certain genes, including the SQSTM 1 (Sequestrosome 1) gene on chromosome 5 have been associated with triggering the disease.
- Viral infections – Some scientists believe Paget’s is related to a viral infection in the human bone cells that may be present for many years before the problems appear. Hereditary factors also seem to be playing a certain role in the manifestation of symptoms. The viruses linked to measles in people and distemper in dogs has been found in the people with Paget’s disease. But there have been no proof that these viruses cause the disease.
- Environmental factors – Another theory suggests that people with rural lifestyles are at an increased risk of developing Paget’s disease. Since more and more people are moving to the urban areas, there also seems to be a fall in the Paget’s disease patients.
Treatment and Remedies – Although up until now no cure for Paget’s disease has been discovered, still it is a condition that is treatable. A patient may not need any treatment if the symptoms do not manifest. However, once the disease becomes active, indicated by an elevated alkaline phosphatase level. Treatment is most effective when the disease is diagnosed early enough. One of the major goals of treatment is to relieve the bone pain and thereby preventing the disease from progressing. It does not always require treatment, but there are several options to relieve the symptoms. Some of the tips that people can easily follow are:
- Prevention of falls – Paget’s disease puts the patient at a high risk of bone thinning that leads to fractures. They should take measures to make their home fall-proof by removing slippery floor coverings, using non-skid mats in the bathtub or shower and installing handrails on stairways and grab bars in the bathroom.
- Eat well – Anyone who is at risk of the disease should include adequate levels of calcium and vitamin D in their regular diet, that helps in the absorption of calcium. Those who are being treated with bisphosphonates should especially consider this.
- Medications – Bone pain can require anti-inflammatory drugs (NSAIDS) or pain-relieving medications. Bone deformity can require supports such as heel lifts or specialized footwear. Bisphosphonates or calcitonin, are some of the medications that tend to reduce the risk of the Paget’s disease. These drugs are also used to treat certain patients with osteoporosis.
- Surgical operations – are required for the damaged joints, fractures, severely deformed bones, or when nerves are being pinched by enlarged bones.
- Exercise – Exercise is very important in maintaining bone health, avoiding weight gain and keeping joints mobile. It is very important for the Paget’s disease patients to not put too much stress on their bones so they should check with their doctors and physical therapist as to what exercises would be beneficial for them.
Some people with the Paget’s disease also feel pain in the affected bones. It can also lead to other conditions such as osteoarthritis, kidney stones and heart disease et al. The disease is very rare in individuals under 40 years of age. It is estimated that 1-2% of white adults who are over 55 years of age have Paget’s disease. This figure rises to 5-8% for those who are >80+ years old.
The disease is caused by a slowly progressive infection, that is present for many years before the symptoms appear. Genetic traits also seem to play a vital role in the instance of the disease since it affects multiple members of the family. Men are more likely than women to have the disease. The disease can lead to complications like arthritis, headaches, hearing-loss or nervous system problems, depending on which the bones are affected. If it is not treated, it can reduce the individual’s ability to perform activities of daily living.