Diana Wilkinson
PAGET’S disease of bone can cause pain, deformity and various complications. It is often a neglected disease, but it is treatable.
As a specialist Paget’s nurse, I run the Paget’s Association’s National Nurse Helpline and am aware that many with Paget’s disease of bone do receive excellent care. It is also clear to me however, that many others suffer unnecessarily because they have not been referred to a specialist who has expertise in Paget’s for a complete assessment, and when required, treatment. Sadly, people are sometimes told that referral to a specialist is not necessary, and occasionally some are even told that there is no treatment, so they must go home and take analgesics!
I believe that our charity, The Paget’s Association, are developing excellent strategies which will influence the medical world and help to raise awareness generally.
So what is Paget’s disease of Bone?
It is a metabolic bone disorder characterised by excessive breakdown and formation of bone, resulting in weakened bones that can lead to pain, severe deformity, deafness and fractures. Typically, diagnosis occurs in those over 55 and it is sometimes discovered by chance when an x-ray or blood test is carried out for another reason. The UK has the highest prevalence of Paget’s disease in the world, affecting up to 1 in 12 older men and 1 in 20 women over the age of 80.
As I write this I have worked as a specialist nurse for the Paget’s Association for just over 3 years. In that time, the thing that has always amazed me is the sheer range of effects that Paget’s disease can have from those with no symptoms at all, to those with widespread disease and severe disability.
In very rare cases, Paget’s disease can lead to osteosarcoma (bone cancer). Indeed this rare event happened to a gentleman, who sadly died last year, at the age of 57. His story is in the Paget’s Association’s May newsletter. In putting this article together with his wife, I have to say I found it difficult to do so without a tear in my eye. As a nurse who was previously a bowel cancer screening practitioner, and for many years a palliative care nurse, I am accustomed to breaking bad news and dealing with the consequences of advanced cancer. It was, therefore, difficult to understand why this particular experience troubled me so. Perhaps it is because Paget’s-associated osteosarcoma is such a rare complication of Paget’s disease. that to come across a family so utterly devastated by Paget’s, is very sad indeed, and I wish they had found the support of our charity earlier than they did.
The good news is that over recent years Paget’s disease has generally become less common and less severe although the reason for the decline is unknown.
A blood test for a chemical called alkaline phosphatase (ALP) is usually elevated in people with Paget’s disease. Levels tend to rise with increasing bone activity, although it is important to note that a high ALP level is not always due to Paget’s. ALP is also produced by cells in the liver and so further more specific blood tests can be carried out if it is unclear which cells have caused the increase. It is important though to note, that a normal or minimally elevated level of ALP may be seen in some patients who only have a limited area of bone affected by active Paget’s. I’ve spoken to people who, with the benefit of hindsight, have looked back with their general practitioner over years of routine blood tests and found that their ALP has been steadily rising within the normal range.
Bisphosphonates are used to treat Paget’s disease: oral risedronate, intravenous pamidronate and more recently, intravenous zoledronic acid. Treatment is effective at controlling the excessive bone turnover and in many people relieves pain caused directly by active Paget’s disease. The more potent of the treatments is Zoledronic acid, and the potential for prolonged remission with this might prevent the onset of long term complications which impair quality of life. In fact, many patients with whom I’ve spoken will not need further treatment after just one infusion of Zoledronic acid. For the majority of patients with Paget’s disease, the benefits of bisphosphonate treatment outweigh any risks. The reason this is particularly true for those with Paget’s disease is that bisphosphonates are given for a short period of time, when the patient’s signs and symptoms require it.
The most common reason for those with Paget’s disease to visit their GP and for calling our Nurse Helpline, is pain. Pain in Paget’s disease may be directly related to the disease itself as a consequence of increased bone activity or it can occur from a fracture, pressure on the nerves from enlarged bone or as the result of deformity of the bones, which can put stress on joints and soft tissues. Paget’s is also associated with the development of osteoarthritis because the presence of Paget’s disease in adjacent bone may accelerate the development of osteoarthritis in a joint. It is therefore, worth considering osteoarthritis as a cause of ongoing pain when the Paget’s itself has been treated. Where Paget’s disease is concerned it is important to know which particular bone/s are affected because the symptoms and possible complications can vary depending on the bones involved. Paget’s in the skull for instance may cause deafness.
We know that genetics are involved in causing Paget’s disease but an unknown environmental element is thought to trigger development of the condition. I’m intrigued by this and other unanswered questions such as why it sometimes affects bones just on one side of the body. Let’s hope answers are found soon which will lead to a better understanding and we can take a step closer to a cure or a way to prevent Paget’s occurring in the first place.
Anyone requiring information or support should visit the Paget’s Association’s website
Diana Wilkinson is specialist Paget’s nurse at the Paget’s Association