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4th January 2021

Standing strong with Paget’s disease

Simon Leigh had nearly recovered from ankle surgery when he started experiencing intense pain and swelling on the same leg. X-rays and scans led his doctors to an unexpected diagnosis and a little-used medical procedure.

“Investigative X-rays showed abnormal bone growth in my right tibia and the doctors initially thought it might be bone cancer,” recalls Simon, a retired army engineer. “Needless to say, I was more than a bit upset.”

Upon further investigation using magnetic resonance imaging (MRI) and computed tomography (CT) technologies, doctors were able to look at more detailed images of his leg tissues and bones. “The CT scan was the clincher – that’s when they were able to diagnose me with Paget’s disease,” he tells This Is MedTech. “I count myself extremely fortunate for my quick diagnosis.”

According to the Paget’s Association in the UK, Paget’s disease is the second most common metabolic bone disease after osteoporosis and is most often diagnosed in people over 50. It’s characterised by abnormalities in a person’s bone renewal and repair process; the affected bone may be enlarged and misshapen as a result.

Although Simon had been struggling to walk because of the increasing pain, he noticed for the first time that his lower leg had developed a very pronounced curvature.

“I had a bone scan to see if there was any disease activity elsewhere in my body. The more active the disease, the more brightly the image glows,” he explains. “My tibia was lit up like a Christmas tree, but thankfully it wasn’t anywhere else.” Nevertheless, at 55 years old, such a severe case was rare.

Nearly two years later, after intense drug treatments involving intravenous bisphosphonate infusions, Simon’s disease was in remission and he was ready for surgery. In a rarely used procedure for Paget’s patients, the surgeon had to break the tibia and fibula and fit an external device called an Ilizarov frame, which would straighten the two bones while realigning his hip, knee and ankle.

“The frame had bolts screwed into the tibia, as well as wires going right through the bones and out the other side of the leg,” says Simon. “Using a spanner – yes, a spanner! – we could move the bone in any direction by adjusting the bolts from the outside.” It took six weeks of precise daily adjustments to get his bones aligned. Then came a long wait.

“Over the next four months, we watched as new bone progressively filled in the fractures. It’s strange to watch,” says Simon, noting that he had regular X-rays to monitor progress. “It looks like wispy clouds forming in the gaps between the bone.”

Despite having serious discomfort and pain in the early stages, the procedure was successful and Simon feels it was well worth the six-month journey. He later had the same surgery on his left leg because it was shorter than his right leg after the first operation.

Now he’s walking without any assistance and is passionate about sharing his story with other Paget’s patients to give them hope about what’s possible with modern medtech.

“It’s amazing what they can do with such a clever piece of equipment. I couldn’t have been rehabilitated without that technology.”

Paget’s Disease Awareness Day takes place on 11 January.