Individual Experiences of Paget's Disease

Emanuela’s Story

Emanuela is a 57-year-old nurse who lives in Italy. She wanted to share her experience of Paget’s disease to help others.

When I had blood tests my alkaline phosphatase (ALP)   was often high but progressive pain in my left shoulder began to restrict my movement. This led to investigations such as Magnetic Resonance Image (MRI) and x-rays. These highlighted the presence of Paget's disease in my left arm (humerus). From there a series of examinations began and I went to San Raffaele Hospital where there is expertise in Paget’s. I had an infusion of zoledronic acid. After a couple of days of fever and nausea I was fine and it practically took all my pain away, especially at night, and my ALP is now in the normal range.

The treatment gave me a better quality of life although I'm more serene than I was! I had to change jobs as I worked in the operating room. Driving for long distances is a little painful but I take rests. I take care not to strain my arm but I try to do everything I want to, just with more care and attention. For example, I try not to fall! I have a cat and also a dog, Sky, but she is very quiet. She is my happiness but sometimes she pulls too much on the leash and I have to hold her with two hands so as not to strain my Pagetic arm. I enjoy reading, cooking and mountain walks, and this year I am planning on walking the Camino de Santiago de Compostela in Spain (without Sky though) ... 100 km on foot!

The best advice I can give to anyone newly diagnosed is to find a hospital where the specialists have experience of Paget’s disease.

Emanuela

Michael's Story

“I felt that it took a long time for me to be diagnosed with Paget’s disease. I am 65 now, and although it was diagnosed only recently, looking back I think I may have had it for a number of years. I experienced pain in several areas, and Paget’s disease was suspected last year when I had an abnormal blood test and was referred to a specialist. Scans and x-rays revealed that I have Paget’s in my pelvis, left shoulder, and right arm. I was prescribed an infusion of zoledronic acid, which I had in March. The after-effects were similar to having flu, such as shivering and shaking, however, I’m so glad that I had the infusion, as the pain has improved. Should I require further treatment, I won’t hesitate to agree to it.I have to say that there isn’t anything I have had to change or give up due to Paget’s disease, and to anyone who suspects they may have Paget’s, I would advise you to go to your GP and get it assessed straight away.”

Michael

This was published in the Paget's News Magazine.

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Lynda's Story of Paget’s Symptoms and Treatment

Lynda lives in Rhode Island, United States and was diagnosed with Paget’s disease when she was fifty-five. Here she explains how Paget’s disease has affected her. 

Prior to being diagnosed with Paget’s disease, I was found to have hyperparathyroidism and osteoporosis. I also have an immune deficiency and am on medical leave from my work as an attorney.
I have Paget’s in several bones. It is in my spine, hips, legs, shoulders, and my skull. Since I have other health issues, it’s somewhat difficult to tell which of my symptoms are from Paget’s, but I have experienced bone pain and headaches. Whilst I currently don’t have a lot of pain, the bone in my skull is starting to distort so I’m concerned about potential complications, such as loss of hearing. Thankfully, I haven’t broken any bones.
I had zoledronic acid about two weeks ago and had a terrible time. I experienced vomiting, a fever and bone pain, which lasted about five days. I’m still experiencing extreme fatigue, and lack of appetite. I’m hoping it works. It will be worth it if it does. I feel better now, and I am back to daily walks and hiking, but I worry sometimes that my skull will get worse and I’ll need more treatment. I will be having a follow-up appointment with my endocrinologist.

What would you say to anyone newly diagnosed with Paget’s?
I would tell them we are fortunate. There’s treatment that can put this in remission. I would advise them to join the Paget’s Association and all the Paget’s Facebook groups.

This was first published in the Paget's News Magazine.

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Karen’s Diagnosis was a Complete Surprise
Karen lives in Queensland, Australia. She enjoys swimming, reading and photography. Before retiring, she worked in administration. Karen shares her experience below. 

For some years I’d had various symptoms, including painful swollen fingers, facial and scalp hypersensitivity, tinnitus and pain on the left side of my face, which was increasing rapidly in frequency and intensity. 
In early 2019, then aged sixty, I presented to a locum doctor for an unrelated (to Paget’s) matter. The doctor took the time to read my medical history and asked me about the unexplained pain in various sites that I’d suffered for some years. He referred me for a full body nuclear scan. This, together with blood tests, confirmed Paget’s disease. I was also diagnosed with atypical trigeminal neuralgia (facial pain), which may be a complication of Paget’s. I was prescribed anticonvulsants, which in time, controlled the attacks of pain.
I then had an infusion of zoledronate, after which I was very unwell with flu-like symptoms for a couple of days. This included sweating, fever, weakness, aches and pains all over, particularly in my lower back, and my head felt weird. A couple of months after I had the infusion, I tapered off the anticonvulsants and I have not had any hideous pain attacks since. I do have some background aches in my face and head, but these are all now manageable without any medication.
The Paget’s diagnosis was a complete and unexpected surprise for me. I knew nothing of Paget’s, but I count my blessings that a locum doctor, that I had only one consultation with, was curious enough about the unexplained symptoms noted on my medical record to send me for diagnostic testing. I should add, that I haven’t had to give up any activities because of Paget’s.  

What would you say to anyone newly diagnosed with Paget’s?
- Join the Paget’s Association! It has been an excellent source of detailed information that is easily accessed.
- Take the advice of specialists (in my case, an endocrinologist) and become proactive in managing the condition.
- Take steps to improve your bone health, your diet and general health.

Karen

Karen's story was first published in the Paget's News Magazine.

Recardo - Patient and Patron

A worldwide entertainer, business man and event’s organiser, British born Recardo Patrick first rose to fame as lead singer with the band Sweet Sensation who had a worldwide hit in 1979, with “Sad Sweet Dreamer”. He has worked alongside many other famous names including Michael Jackson, Whitney Houston, Elton John and Prince. Recardo came to be a Patron of the Paget’s Association after discovering that he had Paget’s disease. His work schedule is hectic, however, he recently took time out to talk to Diana, our Specialist Paget’s Nurse about his Paget’s disease.

Recardo was 48 when he began to experience pain radiating down his thigh. He had investigations which led to a diagnosis of Paget’s disease in his right pelvis. His endocrinologist prescribed an infusion of Zoledronate and Recardo has never looked back. Although he suffered flu–like side-effects for about 8 hours following the treatment, eventually the drug took effect and he became pain free. This was over 4 years ago and he has not had any pain or problems since. He is monitored each year and is very happy with the success of his treatment.

Recardo believes in keeping fit, training every day and partaking in extreme sports such as snowboarding. He is however, aware how disabling the complications of Paget’s disease can be in those who are severely affected, and hopes research can find a cure soon. He gives this advice to those who are diagnosed with the condition: “After the initial shock, get a good assessment, and if you need treatment don’t hesitate, Zoledronate relieved my pain and has given me long term benefit, enabling me to lead a normal life.”

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John Found the Paget's Association

"I have Paget's Disease in my pelvis and have known about it since 1991. The Consultant thought I’d had it since about 1974 and as far as I'm aware I have no family history of it. I was given a bisphosphonate infusion, which helped my pain. I didn't have any side-effects and I have not needed treatment for the last 10 yrs.

Being a member of the Paget's Association is helpful because I know I am not alone. In addition, the information they provide is good and is constantly being improved. My advice to others is no matter how bad you feel there is always someone at the Paget’s Association who can share your worries and help."

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Infusion for Paget's Disease

Maureen is a retired lab technician who enjoys cross country running and swimming.

'I have Paget’s disease in my right shin (tibia). I am careful how much stress I put on it, so I tend to do my training on soft ground.  I had an infusion in 2012. Since then, I have had no pain'.

Maureen

Paget's in My Pelvis, Back and Neck

'I have Paget’s in my pelvis, lower back and neck. In 2001, I wrote an article, “Terry’s Tale”,  in the Paget’s magazine. In the following years, I looked after my wife, often pushing her in a wheelchair. She passed away in 2013, by which time I was suffering a lot of pain with tingling, numbness and weakness in my legs. My specialist suspected pressure on nerves and therefore, prescribed Gabapentin. I also had an infusion of Zoledronate, which lowered my alkaline phosphatase from 375 to 68. I now “manage” my condition with regular back/leg massage and don’t allow myself to get too tired.'

Terence

I first started suffering pain in my right leg in around 2004. It appeared to be cyclic, where I would be pain free for 2 months, suffer right thigh pain for 3 days, then be pain free for 2 months followed by pain and so on. The pain appeared much worse at night time and affected my sleep a great deal. My GP referred me to a consultant who asked for an MRI scan of my back to see if any disc bulges were pressing on or near to any nerves. The results did show a slight bulge but instead of any invasive surgery, both the consultant and I agreed on a course of physiotherapy. Due to the cyclic pattern, it was difficult to see if the physiotherapy made any difference and in the end I just put up with the pain hoping that it would eventually subside.

Some six years later, in 2010, the frequency of the pain increased to an extent that the cyclic pattern was down to every week. In the meantime, I had been to chiropractors and physiotherapists but to no avail. My new GP referred me to another consultant who requested a further MRI scan of my back and a plain X-ray of my hips. During the follow-up appointment he said that the X-ray of the hip had just captured what appeared to be Paget’s disease of the Bone (PDB) in my right femur. He referred me to a rheumatologist who just so happened to be the original consultant I saw in 2004…

The rheumatologist sent me for a bone scan (scintigram), carried out blood tests including an alkaline phosphatase test. He also discussed my treatment plan, which was to be a Zoledronate infusion. I was told that this was one of the best treatments for PDB, which provides a good remission period.
I had my infusion in December 2010 and had flu like systems the following day. The rheumatologist also put me on Vitamin D and calcium supplements as the blood results showed these to be on the low side. My follow up appointment was to be in six months’ time. However, in January 2011, I was still in a lot of pain and re-visited the consultant. He put me on a course of steroids and took a new scan of my lumbar spine and right hip. He explained that on rare occasions Zoledronate can cause some pain. The steroids did help with the pain and I was told to continue with them until I saw him in 6 months’ time. In May 2011, however, I again had to return to the consultant earlier than expected due to an increase in pain. I had an MRI of the right femur and hip, which ruled out osteosarcoma (bone cancer). We both agreed that the original deep seated bone pain had decreased and the pain that I was getting could well be coming from the lumbar, which was suggested by the second consultant. He also told me that Zoledronate usually put Paget’s in remission for 3-5 years.

I returned again in September 2011, still complaining of pain and a new MRI still did not raise any neurological interest and again the radiologist could not see any evidence of osteosarcoma. He ruled out that the pain was coming from Paget’s and referred me to an orthopaedic consultant and a pain specialist.
I decided to see the orthopaedic consultant first (November 2011) who requested plain X-rays of my hips. These showed a loss of space in both hips. He suggested that I have physiotherapy and if that did not work, he could perform manipulation under anaesthetic (MUA), which can have some success.
I started making a pain chart, rating the pain on a daily basis from 0 to 10 where 10 would be the level that would prevent me from sleeping even when taking pain killers such as Naproxen.

When I returned to see the hip surgeon in February 2012, telling him that the physiotherapy had little effect, I showed him the chart, which he described as being very useful. We agreed that we should try MUA on the hip. This was carried out and I returned for the follow-up in April 2012 where I explained that initially the pain had subsided but had since returned. As I was coping with the pain, he discharged me with a proviso that at some stage the hip would have to be replaced.
As the pain was still there I approached the pain specialist in September 2012 and had lumbar facet joint injections, which did not provide any benefit.

I put up with the pain until December 2012, when I went back to the rheumatologist and we agreed to try a second Zoledronate infusion. This was performed in February 2013. He also said that he was convinced the pain did come from the proximal femur and if the second infusion did not work, then the only alternative would be to have a hip replacement.

After 44 years of working for the same company, I took early retirement in August 2013 and hoped that I would see an improvement in not having to drive 80 miles every day. At the end of September 2013, I wrote to the rheumatologist to refer me to the orthopaedic consultant as the pain was still bad and I was now quite depressed about the situation.

I saw the orthopaedic consultant in November 2013 and agreed to give physiotherapy one last try and if that did not work then I would go ahead with the replacement. I returned in March 2014 with that request and a further CT scan and X-ray were performed to provide information for the surgery.

My hip was replaced on 24th July 2014 and the next day the consultant told me he had found a soft tissue lump, which he had sent away together with the femoral head for analysis. I was called back and told the devastating news that it was osteosarcoma. I was referred to Stanmore hospital, a sarcoma specialist centre, where I attended and underwent 3 scans of my body to see if it had spread. I returned on the 24th September 2014 to be told that they had retested the soft tissue lump and femoral head and in actual fact the diagnosis was Primary Lymphoma of the bone (Non-Hodgkin’s Lymphoma, diffused large B cell), which is treatable.
I underwent chemotherapy and a PET scan, taken between the 2nd and 3rd cycles, revealed that the cancer was in remission. I finished my 6 cycles at the end of January 2015 and am continuing to improve from the side effects. The pain in my thigh has gone, along with all of the pain killers I was having to take.

I had my first follow-up with my hip consultant at the beginning of July, a year after it was replaced. I raised the question whether he thought that the pain I was experiencing in my thigh was due to the Lymphoma of the bone that he found during the operation but in his opinion he thought it was solely down to the Pagets. I must admit I wasn’t expecting that answer as I was convinced that the Lymphoma in the bone marrow was the culprit. I probably will never know the answer as he removed a lot of the Pagetic bone through the process to fit the prosthesis as well as of course, the femoral head.

I have spent many months researching whether there is a link between Primary Lymphoma of the Bone and Paget’s Disease of the Bone (just as there is between Paget’s and osteosarcoma) but have not found any link. Statistics show that getting Paget’s disease in the right Femur occurs in about 0.3% of adults over 55. Getting Primary Lymphoma of the bone in the right Femur is about 0.005%. That would make getting both Paget’s and Primary Lymphoma in the same bone at the same time to be 1 in 60 million.
Does that make me an extreme rarity or extremely unlucky?!!

   3D CT image showing clearly the pagetic bone and irregular build-up of bone in the right femur (on the left of the photo)

 (John's story was published in Paget's News in November 2015)

What started this disease? I have Paget’s in my lower left leg and I sometimes speculate where and how it might have begun. Many years ago, I broke my left ankle in a pit accident having caught my leg under a sleeper. I had a further bad accident when my left leg was one of the many places badly scalded by very hot steam in the Coke Works. Both occurred in 1960 when I was 25 years old. The doctors put me back together and built me up, however, it was 13 months before I worked a normal working week again. From about that time my leg did, at times, give me problems with pain and warmth. By late December 1967 there was also evidence of a slow bend in the bone and the distance between my knees was two fingers wide.

In February 1968 I was running up the stairs at work when I slipped and broke both my tibia and fibula (lower leg bones). Five days after the event it was noticed that the leg had not been properly aligned. This was corrected by cutting the plaster and then, without sedation, correcting the alignment (it hurt!). It was settled back into its plaster cast and figure 1 shows an x-ray 3 months after the event. At no time was Paget’s mentioned.
By 1974 problems with the leg had worsened, and my GP sent me to hospital for an x-ray. This was when Paget’s was diagnosed. The advice was to wear elastic stockings but this changed in 1978 when I was advised to wear normal socks.

Five years later I was hit by a discus on the leg which caused a stress fracture and doctors at the hospital I attended applied an elastic stocking. A few months later I suffered a further stress fracture when I slipped on a steep grassy bank. Attending a different hospital, I was given a knee length plaster cast until the bone healed.
For the next nine years the leg slowly deteriorated. In October 1988 the pain really kicked off. At that time I was a Principle Training Officer for South Tyneside Local Authority and whilst I was driving to work, I suddenly experienced severe pain. I discovered that this was from stress fractures which caused me both pain and warmth around the leg. My consultant, who had put me on Etidronate disodium tablets, noted that the x-ray from 1968 (Figure 1) had shown signs of Paget’s. Unfortunately, in August 1995 my consultant died and my GP and I decided to continue as best we could. By October 1997 however, the problems had gone beyond our area of expertise and I was referred to the Freeman Hospital in Newcastle-upon-Tyne. Following scans and tests and with an Alkaline Phosphatase (ALP) reading of 511, I was, put on Skeled tablets for six months, after which time my ALP reading had dropped to 85. By June 2000 it was up to 135 and a further course of Skeled brought it down further to 61.
Four years later in 2004, my ALP had crept up to 162. Pain and heat had been intermittent and co-codamol really helped with the pain. In summer 2004, I was given Actonel (Risedronate) for 2 months. Things quickly improved and I rarely experienced pain and heat. To help with my walking, I had been using two crutches since 1988. A year after taking the course of Actonel I was down to one crutch. These days I can’t walk far without my stick and occasionally need two. Since 1988 I have had a blue disabled badge but this was taken away from me recently when the rules changed. I suspect this was due to lack of knowledge regarding Paget’s on the part of those making this decision, because when I appealed and supplied some of the Paget Association’s information my badge was reinstated very quickly!

I have regular check-ups and it is now over ten years since I have needed any medication. The space however, between my knees is now two hands plus two fingers wide. The photo shows my present leg shape. Walking may be slow but I get to most places I wish to go. Due however, to the bend in my leg, I’m having, on occasions, where hills are present, to use two crutches. The main problem is standing still, when my leg gets restless and aches. Sitting and lying in bed causes no problems. I have lived with this for many years and have maintained a good work and social life. 

In August 2015, I needed more treatment. After taking a course of Risedronate Sodium tablets, my ALP reading had dropped to 65, from 155, by Jan 2016. The previous course I had was Dec 2004 and so it lasted up to August 2015. Hopefully, it will be 2026 by the time I need another - at the age of 92!"

Bill

X-ray taken in 1988

Several years after being diagnosed with arthritis in my hip, I reluctantly went to my GP because the pain had become much worse. I was given painkillers, and unable to walk very far, I took to using a walking stick...

Much later, I was referred to a consultant at the Norwich and Norfolk University Hospital and after two years of appointments, it was suggested that I should have a total hip replacement. During the long wait for my operation, however, I fell several times which resulted in my having a scan. Things changed when I was told the scan showed arthritis and Paget’s disease. “What’s Paget’s disease”, I enquired? I’d never heard of it. No one seemed to want to explain - but perhaps it was because they couldn’t!

I spent fifteen days in hospital following my hip replacement, after which I was taken home. No aftercare was offered and I felt abandoned. My son and daughter live miles away but I had to make quite a few phone calls to them. Eventually after several months, with the aid of a four-wheeled walker that my son and daughter bought me, I was able to walk down the path. Although I was still in great pain, with my walker for support, I was determined to attend a meeting in Norwich on Paget’s disease, which I had seen advertised in a local paper.

The meeting was one of the Paget’s Association’s Information Days. It was very interesting and opened my eyes to the number of people suffering silently with Paget’s disease. My story really begins after I became a member of the Paget’s Association and the Paget’s Support Network. I was put in touch with people from all over the world who also have Paget’s and who wanted to connect with others in the same position. I was amazed, as I had felt so alone. The Paget’s Association helped me connect with Rhoda, who also lives in Norfolk. Unbelievably, after a long chat, she told me she was seeing her consultant the next morning. She would ask him if he could see me.

It wasn’t long before all the appropriate arrangements had been made and I received an appointment. As soon as I saw the consultant at his desk, I knew here was somebody who would understand my pain and explain it all to me. He immediately ordered a bone scan, which revealed Paget’s in my pelvis, left tibia, coccyx and both feet and there was a hairline fracture in my pelvis! No wonder I was in a lot of pain. After assessment of my medical history, he prescribed appropriate treatment. I felt so much better - like a 25-year-old! I will always be eternally grateful to Rhoda and to the Professor for his explanation and medical help. At 90 years old, I have resigned myself to make the best of everything.

Allan’s Dancing Again

Member, Allan Kidney, Hertfordshire, explains how he was diagnosed with Paget’s disease and how he had to stop dancing.

"I am a Retired Chartered Mechanical Engineer and was aged 73 when Paget’s disease was diagnosed.  I am divorced, live on my own, and among other interests, I am a keen Ballroom, Latin American, and Argentine Tango dancer.  In the four weeks before my leg became a problem, I went to 23 dances. In September 2014 I went to my regular Friday afternoon tea dance.  I remember that, during the afternoon, I danced a ‘Ballroom Tango’ for which the music was particularly vibrant, and I remember dancing it with much vigour.

The following day, I had pain in my right leg.  I contacted the out of hours GP service and saw their doctor at the local hospital.  My leg was swollen, painful, and I had difficulty walking.  The doctor diagnosed cellulitis and prescribed steroid cream plus a course of antibiotics.

Various GP appointments followed, and at the end of October, a further prescription was given for another course of antibiotics.  At one of the appointments, the GP commented that I had ‘bandy’ legs.  If the GP had put two and two together, no mention was made to me that this might be Paget’s disease.

At my next appointment, at the beginning of November, I was referred for an x-ray of the tibia and fibula. It was from this x-ray that the GP advised me that I had Paget’s disease in my leg.  He wrote ‘Paget’s’ on a piece of paper and said that I could ‘Google’ it and find further information. He referred me to an endocrinologist.

A Little About Me. I started life in Whitehaven, Cumbria and later attended college in Tewkesbury to study nursery nursing, When I was eighteen, I married Frank. His support and loving kindness throughout my life has been invaluable.

The Journey. My symptoms of Paget’s disease started approximately 10 years ago. My legs became quite painful and then I noticed a bend in my left arm. Lumps started forming on my skull, and I was experiencing increased pain in my pelvis and hip. I had 5 bone scans over a ten year period and in 2006, following a bone density scan I was told by my doctor that I had severe osteoporosis. A course of Fosamax followed which unfortunately resulted in pain and flu like symptoms, causing me to stop taking the medication.

Following a blood test in October 2010 I was told by an emergency doctor I had first degree liver damage and was advised to stop drinking alcohol. As I had not drunk alcohol for many years, because of the medication I was on, I asked the doctor to refer me for a scan. Despite the pain in my legs, arm, pelvis, hip and skull, she refused. I felt angry and didn’t know why these symptoms were happening and felt let down by the medical profession. Why was the diagnosis taking so long? By April 2011 I had reached rock bottom. I discussed the pain with my usual GP, who explained that my alkaline phosphatase level had gradually increased. Over a ten year period the level had risen from 108 to 168, with just a slight dip whilst taking Fossamax. Suspecting I had Paget’s disease, she referred me to an endocrinologist at Lancaster Hospital and gave me literature about the Paget’s Association. In apologising it had taken so long to come to a diagnosis, she said, “I am sorry I can’t give you your life back!”

Journey’s End? The result of an isotope bone scan confirmed the presence of Paget’s disease. There followed a 5mg Zolendronic infusion at Kendal hospital, after which I felt quite poorly, with pain and flu like symptoms. It felt like a bus had run over me. After 4 - 5 months relatively pain free I started to have pain again. Seven months after the first infusion the Consultant arranged for a second. Two days after the infusion I had an increase of pain, followed 10 days later by flu like symptoms. After a few weeks these subsided and the pain in my skull decreased. It took just 6 weeks from the initial tests until the treatment. During this time I contacted and joined The Paget’s Association. I found their information pack and newsletter most informative, helping me to cope with the condition and keep me going. My GP and specialist are supportive and their truthfulness has helped allay any fears. My only regret is that it took so long to get a diagnosis and this still makes me angry. If I had been diagnosed and treated earlier it may have prevented the hearing loss I now experience.

I have up and down times as anyone does but the support of family, the Association and my GP have helped me get through and keep me going. After 57 years of marriage my husband has stayed with me through thick and thin. We are a good team.

My advice to other people with Paget’s: Get to know as much information about the condition as you can and absorb it. I truly believe that it helps take away the fear of the unknown. Involve your family, tell them about it so that they can understand and offer you moral support. Keep going and cope with the condition the best you can. Your life changes accept and adapt. Realise that most people with a long term condition can get very low, but remember you are not on your own. Some days I miss being me! If you are not in a good place mentally, it will be difficult to deal with. The support and understanding of family, friends and the Association can help you through the difficult times. My friends and relations looking puzzled ask, “What is it?” Because of the information I was given by the Association, I can explain it to them which helps them understand. Thank you to the Paget's Association for the help and information they have provided to me over the last year. Keep up the good work. (2012)

Jean has been a member of the Paget’s Association for some time and wanted to share her experience of Paget’s disease with other members. Here she discusses how Paget’s affected both her mother and herself.

I left England for South Africa in 1968, and with the long travelling time and high costs returned infrequently for family visits. On one of these occasions my mother showed me her extremely curved thigh (femur) which she proudly told me she managed to keep secret from her doctor and the district nurse. She didn’t know what had caused it, and as it didn’t bother her too much, didn’t want to know - end of discussion! I put it to the back of my mind.

I have always been pretty fit, but never took part in any sporting activities, except to do obedience training with any new dogs that we had (we normally have 3 or 4) and to take them for occasional walks. In 1994 we acquired a German Shepherd puppy and joined a new dog school, where I was delighted to see that they had various jumps and obstacles that the older dogs navigated. I was fascinated, and as soon as my dog was old enough, joined the agility class. It was during one of our practice sessions, as I was putting my dog over an A-Frame, running next to her at speed, I knocked my shin against a large wing nut that was sticking out of the side. There was amazingly no bruising, and it wasn’t broken, but I have never experienced such excruciating pain as I did for the next few weeks. As the pain subsided my leg became tremendously hot but I enjoyed the warmth during our winter and did nothing about it. About 3 months after the knock I started to get minor pains in my ankle and went to the doctor to see if I should rest my leg or run through it, as by this time I was really hooked on dog Agility & Jumping and had started entering competitions. X-Rays were followed by a visit to a specialist who diagnosed “either Paget’s disease or fibrous dysplasia” but as there was no treatment for either I was told I must just take Aspirin for the pain and go back for more X-Rays in 6 months. I could not accept this and during the next 3 months did as much research as I could in libraries and on early internet sites where I discovered The National Association for the Relief of Paget’s Disease (now mostly known as The Paget’s Association). I visited a number of doctors, alternate healers and anyone who could help me as by this time my leg had become painful and bent. I could only walk with crutches. Finally my friend’s daughter, who was working as a nurse, told me of a doctor who might be able to help me. Fortunately for me, the doctor had just read about a new bisphosphonate treatment for Paget’s disease so, in November 1985, I became one of the first patients in South Africa to have pamidronate infusions. I delightedly walked out of the hospital 2 days later, without crutches or pain, to spend the next 2 days on a camping trip!

I won’t say all was plain sailing as I had a couple of “aching leg” days over the next few months but compared to life before the infusion, it was nothing. Shortly after this I visited my homeopath who suggested that I exercise my leg to keep the muscles strong to protect the bone. This piece of advice has kept me running 2 – 3 dogs in Agility and Dog Jumping since then, training them 3 or 4 times a week and competing at club, provincial and national levels almost every weekend.

I had my second flare up of Paget’s last year (18 ½ years after the first) and was determined to treat this differently. Even without a blood test for Alkaline Phosphatase levels my X-Rays showed that the disease was in its active phase. Once again, within weeks of feeling the first pain I was on crutches, but this time, knowing a bit more about the disease, I turned this to my advantage. I hobbled around forcing my leg against the curve with each step I took while I was waiting to see the specialist for a prescription of pamidronate. Once again had no side effects. Before my second flare-up, when I lay on my side with my leg straight, the curve in the bone would put my foot about 14cm above ground – now it is only 6cm! I am delighted, as even though I will be 70 this year, I still like to wear fashionably short dresses and tight trousers and look smart in the very busy Bed and Breakfast that I run with my husband. I now wonder if it is possible to splint a bone during the active phase of Paget’s disease to straighten it?

It was years after our discussion above that my mother finally spoke to her doctor about her Paget’s disease – which after my diagnosis now had a name. She took tablets for it which didn’t agree with her, so she stopped. She is living with me now and age 97 hobbles round very slowly with a Zimmer Frame, which although she is very slim can hardly fit into because of her exceedingly curved thigh bone, and she complains about sore knees until she gets going but nothing else. I am convinced that with moderate exercise, healthy food and a positive mindset there can be a great life after Paget’s.

(2014)

Part 1 - Straightening my leg (from Paget's News May 2017)

I was diagnosed with Paget’s Disease of the right tibia in 2014, at the age of 55. I have had 3 intravenous bisphosphonate infusions at the RNOH, with a good result but I was left with the classic sabre-shaped bowing of the tibia, both forward and outwards. This was gradually damaging my knee and ankle joints, walking was painful and I needed to use a stick.

On 27^th October 2016, a Consultant surgeon at the RNOH, who specialises in Limb Reconstruction, broke my right tibia and fibula (a double osteotomy) and fitted a Taylor Spatial Frame. We make daily adjustments to the frame, to bring the knee and ankle into the correct alignment and progressively straighten the tibia. The adjustment program is almost complete, then I expect to continue to wear the frame for another 4 months whilst the bone mends and strengthens. The pain is less acute now but caring for the pin sites and walking in the frame are not easy but it will be worth it. At the end, the hope is that my leg will be straight, I will be able to walk unaided, without pain and without further damaging my knee and ankle.

Part 2 - An Interesting Journey (from Paget's News Aug 2017)

When I saw my orthopaedic surgeon at the Royal National Orthopaedic Hospital (RNOH) in October 2016, he said to me that straightening my right leg (which has Paget’s disease in the right tibia) would take about 6 months to achieve. Well, he was absolutely spot on! The Taylor Spatial Frame that did all the straightening work came off exactly 5 months post-surgery, then the plaster cast that replaced it came off a month later; almost 6 months to the day. As I write this, it’s 6 weeks since the plaster cast came off.

It’s been an interesting journey so far. In October 2016, the surgeon broke my tibia and fibula, then fitted the frame, which had bolts screwed into the tibia, as well as wires going right through the bones and out the other side of the leg! Following the surgery, I spent a week in hospital at the RNOH. We then began a programme of daily adjustments to the frame – my wife used a spanner (yes, really!) to move each of the 6 struts by 1mm per day. The adjustments were overseen and amended by the consultant, at clinic visits, every week or two, for about two months. Just before Christmas 2016, he announced that I had finally achieved a “well-aligned limb”. Then came the slow bit... We had to let time and my body work its magic and repair the leg. With the frame locked off, and no more adjustments to make, we waited patiently for new bone to grow into the gaps/breaks in my tibia. By comparison, it makes watching paint dry a positively sprint event!  

However, over the next 4 months, we watched as new bone progressively filled in the fractures. It’s strange to watch. When you look at the x-rays, it looks a lot like ‘wispy clouds’ or ‘cotton wool’ forming in the gaps between the bone; we called it ‘bone fluff’. At this stage, I attended clinics monthly and you could clearly see the progression of bone growth between successive sets of x-rays.

At 4 months, I had the lower ring of my frame removed, to help with mobilisation of the leg. At 5 months, bone regeneration/growth in my tibia was good enough to have the frame completely removed (under general anaesthetic). It was replaced with an above knee plaster cast for the final month but it was just bliss to finally be rid of the frame!

During the whole 6-month period, I walked with crutches, trying to fully weightbear through my leg, as much as comfort would allow, which has helped to stimulate bone growth. I am delighted to report that, despite Paget’s disease and 3 bisphosphonate infusions to treat it before surgery, my bone growth/regeneration has been ‘normal’, which has been hugely encouraging. Even so, there have been times when the straightening process has been a real struggle for me. I had serious discomfort and pain in the early stages, the regime of weekly sterile dressing changes to the 15 pin sites (where the frame was attached through my leg) was painstaking and just everyday living with a 2Kg frame/cage attached through your leg is complicated. It pretty much affects every aspect of your life.

All that said, it has been worth every minute, to achieve the outcome of a straight leg that I can finally walk on again. I’m not quite there yet, but nearly. I am walking with two sticks but the bone is strengthening, wasted muscles are recovering and I am driving a car again. I have regular physiotherapy to help improve strength and mobility. I look forward to a progress review in 6 weeks, to make sure that bone regeneration has continued and the fractures are fully healed. My fibula is still not fully united, but I’m working on it!

Watch this video to hear Simon explain his experience 

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Now 66 and retired, I previously worked as part-time lecturer at local colleges teaching typing and shorthand for over 20 years. I also did some secretarial work at The Paget’s Association for a few months before taking up a part time post at the local university teaching shorthand on a degree course.
I was diagnosed with Paget's disease in my vertebrae (L1) at the age of 47, after having X-rays for excruciating back pain. The pain made walking, sitting and everyday activities difficult.

I am happy with the way diagnosis was given. The results of an x-ray were given by my GP who was very good and I was referred to a specialist at Salford Royal, where I have always been given information and facts about Paget’s in a sensitive manner. Following further tests, I had bisphosphonate infusions which alleviated the pain and put the Paget’s in remission. The side effects of my first infusion were as I expected as it was explained to me that I may have flu like symptoms. The side-effects lasted an hour or two. When I needed an infusion again I didn’t experience any side-effects but I had a complication at the site of the infusion.

Over the years I had further treatments but found I was suited to Risedronate oral treatment rather than infusions. Most of my bone problems now are due to osteoarthritis. I also have type 2 diabetes, bronchiectasis and asthma.

I find being a member of the Paget’s Association helpful for information about Paget’s and to be able to contact people in a similar situation. My advice is don’t worry as most people seem to live to a good age with it. There are much worse complaints to have. Consider yourself lucky to have treatment for it!

Former, Trustee of the Paget's Association, Keith Simpson, discusses his experience of Paget's disease.

My Experience of Paget’s disease, and why I wanted to be a Trustee

For me, Paget’s came on the scene after going to the doctors with a painful leg and knees.

My golf days had become difficult and in the nineteenth (golf club bar) my friends would advise that a knee clean out was the solution. They spoke from personal experience, and after lots of nagging, I finally visited the doctors which was a rare occasion (perhaps it’s a man’s thing).

A referral to the hospital and x-rays indicated two bad arthritic hips and Paget’s in my right femur.  My consultant at the time didn’t recommend any treatment for the Paget’s disease. I did have a PSA blood test (a test that can help diagnose prostate problems, including cancer). After more visits to the hospital, I was diagnosed with aggressive prostate cancer. It was like a baseball bat hitting me between the eyes. It certainly put Paget’s on the back burner.

Of course, the leg pain did not go away and it was two years later that a consultant at my local hospital decided on Pamidronate infusions, 30mg once a week for six weeks.

My pain worsened and following a very informative phone call with the Paget’s Association’s nurse, my doctor spoke with my consultant. Urgent x-rays were carried out revealing a pathological fracture which was immediately surgically repaired with a plate and screws.

I had two weeks in hospital and six months when I could not put weight on my right leg. With no physiotherapy, there was substantial muscle loss. The fracture took approximately two years to fully heal. Detailed viewing of my earlier x-rays showed numerous insufficiency fractures (a type of stress fracture sometimes caused by normal stress upon weakened bone).

I still had two bad hips and a new hip was suggested for my left side, which was thought to be Paget’s free. After a long meeting and an anaesthetic assessment for a new left hip, it was recommended I seek the help of the Association in finding a consultant who was particularly interested in Paget’s disease. Nottingham City Hospital was recommended. This is now a Paget’s Centre of Excellence and I have never regretted the move.

After further x-rays, scans, and blood tests revealed no pagetic activity in the left hip, I was given the go ahead for its replacement. I still had pain, one bad hip and Paget’s disease. It was impossible to identify which, if not both, were giving me pain.

I have attended the Paget Association’s AGMs since 2010, and on all occasions felt them to be very informative and supportive, with presentations covering all aspects of the disease. With my pagetic history, I have been personally interested in topics relating to hip replacements, particularly with my having a repaired femur (plated screws) along with insufficiency fractures and the non-union of my fracture site. I was repeatedly informed that this would not be conducive to surgery. It was not until the Norwich Paget’s Information Day and AGM in 2016 that one of the presenters recommended a fellow surgeon, who could possibly help me. A referral from my doctor and twelve months on I have a new right hip and NO PAIN, thanks to the team at Nottingham City Hospital. It’s been a long ten years.

I am now retired and have time to give my help and support to the Paget’s Association. It is the least I can do for all the varied and continuous help the Association has given me over the years.

Summary and back to Golf

Keith struggled with Paget’s disease for several years and has been unfortunate enough to suffer complications, which led to surgery on his femur (thigh bone) to repair a fracture. Keith endured several months when he could not put weight on his right leg. He also has osteoarthritis and the effects of both this, and Paget’s disease, meant that ten years ago he had to give up playing the sport he loves - golf.

A decade on, following several operations including two hip replacements, Keith finally felt able to attempt his first round of golf.  For this significant achievement to mean even more, he decided to put his efforts to good use and raise funds for the Paget’s Association, who had supported him through his problems.

On the 20th March 2018, with the encouragement of all his supporters, Keith succeeded in playing 18 holes of golf at Sleaford Golf Club. Keith said, “It’s been ten long years but finally, I have achieved my goal of getting back to playing golf, it’s just magic.”

Share your own story by clicking here. 

Linda Feared being Confined to a Wheelchair

Member, Linda Fenlon was diagnosed in 1993, aged 45, with Paget’s disease in her skull, spine and right hip. Here she reflects on her experience.

Turning 70, last May, was a huge occasion for me. As well as a ‘big’ birthday, it marked 25 years, since I was diagnosed with Paget’s disease. I had initially expected to be in a wheelchair, long before being 70. The reason I am still standing is the excellent care, prompt diagnosis and treatment that I received from my GP and the Paget’s specialist at the Manchester Royal Infirmary (MRI).

In 1966, I went to train as a children’s nurse at Booth Hall Hospital and in my second year, as part of the training, we had to spend a week with a midwife and then a district nurse. Whilst a lady, who was confined to a wheelchair. She talked about her ‘chalky’ bones that ‘snapped’ when she turned over in bed. It turned out that she had Paget’s disease and unfortunately the treatments that I have received were not available then. The lady endured many unwelcome effects of Paget’s disease and was offered nothing but pain killers and visits from the nurses to help her. Paget’s disease isn’t common in children, so seeing this patient with the district nurse came back to me years later when I went to my GP with back and hip pain.
Following an x-ray, she told me I had Paget’s disease. I immediately visualised myself in a wheelchair as the patient that I had visited had been.

Luckily, my sensible GP arranged for me to be referred to MRI, where I saw a specialist who was knowledgeable in the disease. I realise that, even now, many people are not as lucky as I was. Many struggle to obtain a diagnosis
and to get the referral they need, to someone who understands the condition. Fortunately, I had a bone scan, treatment, and was back at work within six months, not cured from Paget’s but with it in remission.
I was treated with pamidronate and then later, when zoledronic acid became available, I was given that, and I still have this whenever the pain starts. It works for me!

Having remembered the patient with Paget’s, I vaguely knew what it was but from the GP’s surgery I went to the local library, just to check in the medical dictionary. In that dictionary was the address of the Paget’s Association. The rest is history. I have been supported by the Association for the last 26 years, attending three-monthly support group meetings and I feel lucky to have the support they provide. In addition, as the search has developed into the causes of Paget’s disease, and as I am positive for one of the genes that made me susceptible to it, over the years my family (2 sons, 5 brothers and sisters) have taken part in research, such as the Zoledronate in the Prevention of Paget’s (ZiPP) trial. Fortunately, they are all free from the genetic disposition, but I wish to thank them for their part in the research.

I wanted to share my story to show that living with Paget’s is manageable, it just may need a bit of adjustment.

In this video, Linda discusses her experience with Professor Stuart Ralston.

A worldwide entertainer, business man and event’s organiser, British born Recardo Patrick first rose to fame as lead singer with the band Sweet Sensation who had a worldwide hit in 1979, with “Sad Sweet Dreamer”. He has worked alongside many other famous names including Michael Jackson, Whitney Houston, Elton John and Prince. Recardo came to be a Patron of the Paget’s Association after discovering that he had Paget’s disease. His work schedule is hectic, however, he took time out to talk to Diana, our Specialist Paget’s Nurse about his Paget’s disease:

Recardo was 48 when he began to experience pain radiating down his thigh. He had investigations which led to a diagnosis of Paget’s disease in his right pelvis. His endocrinologist prescribed an infusion of Zoledronate and Recardo has never looked back. Although he suffered flu–like side-effects for about 8 hours following the treatment, eventually the drug took effect and he became pain free. This was over 4 years ago and he has not had any pain or problems since. He is monitored each year and is very happy with the success of his treatment.

Recardo believes in keeping fit, training every day and partaking in extreme sports such as snowboarding. He is however, aware how disabling the complications of Paget’s disease can be in those who are severely affected, and hopes research can find a cure soon. He gives this advice to those who are diagnosed with the condition: “After the initial shock, get a good assessment, and if you need treatment don’t hesitate, Zoledronate relieved my pain and has given me long term benefit, enabling me to lead a normal life.” 

John 

"My wife, Ingrid, was a Trustee of the Paget`s Association, so I thought I'd tell my story.

I was first diagnosed with Paget`s Disease in April 1991, at the age of 57. I had been suffering pain in the appendix area for some time so my GP sent me for various tests. All these failed to show the cause of the pain. I was then sent for a barium meal and an alert Radiologist spotted what he thought was Paget`s disease in my pelvic bone. When my doctor read the report he knew nothing about the disease, so had to refer to his medical books.

A subsequent full Isotope Bone Scan confirmed I had Paget`s Disease in various places including the right side of my pelvis, right upper femur, spine, right upper humerus and scapula.

On the advice of my daughter-in-law, who was a nurse in the Intensive Care Unit at Addenbrook’s Hospital, I consulted a specialist there. The consultant put me on a Pamidronate infusion. On the way home from the hospital, a 25 mile journey, I was in such pain as my whole body went into muscular spasm.  We had to turn around and go straight back to the hospital. It transpired I was allergic to something in the drug I had been given.

In view of the allergy, the consultant prescribed Risedronate which I take orally for a month, have a month’s break and then take for a further month. I do this each year. This has been a success for me in that it appears that my Paget`s has not deteriorated. Unfortunately, I am unable to have the latest Zoledronate Drug as it is my understanding that this contains something to which I am allergic.

I am able to tolerate the occasional pain but have suffered two serious problems from the disease. I had suffered with pain in my spine since my 30’s and it is now thought to have been early Paget`s, but as a result I had to give up golf and other sports. The other problem was in my upper right arm. The Paget’s caused the bone to enlarge which resulted in the muscle pulling away from the joint. I had an operation to remedy this problem and although it took over a year for the nerves and muscle to heal, it is now fully functional again. 

It was interesting to learn in 2014, when I offered my blood to be tested for research, that I have the Paget`s SQSTM1 gene mutation"