Paget’s Disease

Paget’s disease is a chronic condition of the bones. The natural process for your bones is to break down and regrow from time to time. In Paget’s disease, the normal process is broken. The excess breakdown of bones and continued regrowth make the bones regrow too fast. Thus, the bones are softer and bigger than usual. This can result in recurrent fractures and deformities in the bones. The bones in your pelvis, spine, skull, and legs are usually most affected.

1. Bone cells regenerate like your skin cells where the old bone is removed, and new bone replaces it. This process is known as bone remodelling.
2. Osteoclasts (cells that absorb the old bone) and osteoblasts (cells that make the new bone) are involved in this process. 
3. In Paget’s disease, the osteoclast cells start malfunctioning and absorb the bone faster. 
4. The osteoblasts then try to make up for this fast absorption by quickly producing new bone. However, the new bones are larger and weaker than usual.
5. The exact reason for developing Paget’s disease is not known. 
6. Environmental factors and other genetic factors have been linked to the cause of this disease.

People with Paget’s disease are primarily asymptomatic. Symptoms are mostly like those of arthritis. The symptoms of Paget’s disease include:

1. Pain in your bones
2. Hip pain 
3. Damage to the cartilage in your joints
4. Pressure on your nerves
5. Bowing of your limbs
6. Curvature of your spine

1. Patient history: Your physician will conduct the diagnosis for Paget’s disease by asking you questions about your condition. All your symptoms of pain, discomfort and tingling numbness of your arms and legs need to be mentioned to your doctor. Symptoms of bony deformities, recurrent fractures, and other signs like bowing of limbs will help give your doctor a complete picture.
2. Clinical examination: A physical examination helps rule out other causes of bony pains. With the help of a clinical examination, your physician can identify any structural abnormality in your skeletal shape or bony deformities. 
3. Investigations: 
   1. X-rays: The bony deformities and abnormalities are found on x-rays. You can see the areas of bone breakdown, enlargement of the bones, malformations, and any structural anomalies on the x-ray.
   2. Blood tests: Your alkaline phosphatase levels in the blood are elevated in Paget’s disease.
   3. Bone scans: Bone scans are advised to identify the affected bones in your body due to Paget’s disease. A radioactive substance is first injected into your body, then the material travels to the affected parts of your bones. These spots then light up on the scan, showing the affected bones.

You are more likely to develop Paget’s disease due to the following risk factors:

1. Genetics: This disease is a hereditary disorder that tends to run in families. In about 25–40% of the cases, you may have a relative struggling with the same disease.
2. Age: Paget’s disease usually occurs in people above the age of 40 years.
3. Environmental factors: While certain environmental factors may play a role in the prevalence of Paget’s disease, the exact mechanism has not been proven yet.

Since most people with Paget’s disease are asymptomatic, you may not require treatment. If your disease is active and causing symptoms and complications at high-risk sites like the skull or the spine, you may need the following treatment.

1. Medications: Drugs that are used to manage osteoporosis are prescribed for Paget’s disease. Bisphosphonates are one such group of medicines that are either given intravenously or orally to manage your condition. If you cannot tolerate bisphosphonates, you may be prescribed calcitonin, a naturally occurring hormone in your body. This hormone helps in calcium regulation and bone metabolism in your body. 
2. Surgery: In certain rare cases, surgical intervention may be required to:
   1. Help your fractures heal 
   2. Replace the damaged joints
   3. Realign the deformed bones
   4. Reduce the pressure on your nerves
3. Lifestyle changes: To reduce the risk of developing complications, you can try the following lifestyle changes:
   1. Be careful: Avoid falls and be careful of your surroundings. Since Paget’s disease can result in bone fractures and deformities, you need to avoid falling. Use a cane or a walker for support while walking
   2. Fall-proof your surroundings: Remove the slippery floor coverings, tuck away the cords and wires, use non-skid mats in the shower, and install handrails on your stairways and in the bathroom to prevent falls.
   3. Exercise daily: Regular exercise helps maintain your joint mobility and improves bone strength. Consult your doctor regarding the exercise best suited to your condition. The type, intensity, and duration of exercise that suits you must be discussed with your doctor. 
   4. Eat healthy food: Your diet must contain calcium and vitamin D to help in bone growth and calcium absorption. Include vitamin and calcium supplements if required after consulting your doctor.

When Paget’s disease is not treated in time, it may lead to the following complications:

1. Arthritis
2. Heart disease
3. Neurological problems like tingling or numbness, weakness, or pain in your arms and legs
4. Hearing loss
5. Vision loss
6. Loose teeth
7. Kidney stones
8. Fractures and deformities
9. Paget’s sarcoma – a form of cancer that rarely occurs in about 1% of people with Paget’s disease