‎WORLD PAGETS DISEASE DAY

‎Paget’s awareness day was established to honour the contributions of James Paget, a British surgeon and pathologist who first discovered the disease. This year’s theme focuses on ‘the pain in  paget’s disease’, not an ordinary pain caused by abnormal sensory signals.
Paget’s disease is a disorder of bone remodelling-Remodeling being a  continuous lifelong process by which bone tissue is broken down and rebuilt to keep bones strong, adapt to stress and maintain mineral balance, basically 🦴 old or damaged bone is removed ➞ new bone is formed in its place.
‎However, in paget’s  there is an increase in both bone breakdown and bone formation which results in areas of bony enlargement and deformity.

How it happens
In our bones 🦴 there  are three main cells:
– Osteoclasts- bone resorption (breaking down old/damaged bone ), release calcium to blood stream
– Osteoblasts- bone formation, mineralize bones with calcium and phosphate
– Osteocytes- regulators of mature bone cells, sense mechanical stress and signal where remodelling is needed.
In this disease there is an increase of osteoclast activity, these cells are hypersensitive to vitamin D, hyper-responsive to RANKL, a protein  which recruits and activates osteoclasts, and have antiapoptotic gene which means they are less likely to die.

The bony changes seen in this disease can be divided into 3 phases:

‎1. Initial osteolytic phase- increased bone resorption and vascularization

‎2. Mixed phase:  a blend of active bone resorption and formation where the bone formed is weaker and the collagen bundles are disorganized.

‎3. Sclerotic phase: decrease in bone resorption and increase in osteoblastic activity  (hard, dense, less vascular bones formed).
This disease can occur in one or more bones like the skull, spine, pelvis, tibia and normally presents asymptomatic in most patients, detected accidentally in blood test (high ALP marker ) and X-rays .

Symptoms include bowing of legs, enlarged skull, spinal deformities (kyphosis, scoliosis), fractures, osteoarthritis, neurological symptoms from nerve compression (hearing loss, headache, spinal cord lesions) and pain being the most common symptom. This pain is often described as deep aching and persistent, rarely bone malignancy (osteosarcoma) can occur.

Risk factors for Paget’s Disease include
‎Increased age, family history,  genetic mutation of SQSTM1 gene, male sex and ethnicity (more common in europeans and people of british decent). It is  important to get tested if your first decent family member has paget’s because the gene can be inherited.

Treatment
Biphosphates and calcitonin are the standard treatment used for paget disease however supportive treatment like pain management, proper nutrition and physical aids like physiotherapy and occupational therapy is recommended. Surgery is reserved to address complications that arise from the disease.

‎Despite all these interventions the pain does not simply disappear, patients with paget disease continue to experience pain because the primary treatment targets the underlying metabolic bone activity while residual pain often stems from secondary permanent complications like nerve compression, damage to joints, individual variation response to drugs .

International Paget’s Awareness  day celebrated 11th January every year emphasizes that Paget’s disease involves severe, often misunderstood pain beyond skeletal changes. There is a need for early diagnosis, better pain recognition, and improved patient-centered care.

‎Written by Sharon Jeremiah Anuye