Sue is 62 and lives in Lincolnshire with her husband. She has scleroderma and is also living with Raynaud’s Phenomenon as well as with lupus, Sjorgrens syndrome, rheumatoid arthritis, bile salt malabsorption and cardiovascular autonomic dysfunction.
Sue was diagnosed with SSc-associated pulmonary arterial hypertension (SSc-PAH) during 2015 when she was referred to the PH team at the Royal Free Hospital in London, after she began to experience persistent breathlessness. This is her story.
"My initial diagnosis came about after I had an operation on my right leg that would not heal, and I was in a lot of pain around my ankle which I had never experienced before. I found it difficult to walk and the pain was excruciating. I was therefore sent to various consultants to see what the problem was. I saw an orthopedic consultant, the fracture clinic and various others before I finally came to see a rheumatologist during 2007. As soon as I walked into his consulting room, he said to me: "I know what you have - it is lupus." This was apparently obvious to him from the red markings on my face. This eventually led to my diagnosis of scleroderma the following year. My rheumatologist eventually referred me to The Royal Free Hospital in London, and I am still under their care; but I also have shared care with a rheumatologist at the local hospital where I live.
When I was first diagnosed, I was a little shocked; but also relieved that I finally had a diagnosis. Suddenly everything fell into place. I had a reason for feeling so tired and knew that I was not going insane! I then had further tests and investigations, and, because I had already been diagnosed with Raynaud’s many years ago when I was 16, this led to the thought process of scleroderma which was confirmed along with the Sjogren’s syndrome. I found that once a diagnosis had been made, everything became so much easier to understand. I was seen by various consultants, who all added their piece to the jigsaw that had now become my life.
I found out that I have SSc-PAH in 2015 after I started getting breathless. I saw the PH team at the Royal Free and was diagnosed after undergoing right heart catheterisation.
Living with these conditions has affected my life in many ways. One of the biggest impacts was having to retire from my job in the NHS when I was just 49. I found this quite difficult at the time. The SSc-PAH definitely slows me down, and walking is a bit of an issue as I do get out of breath. I am unable to walk our dog now but sometimes my husband will drive to the seafront, and we can have a slow, short walk which is lovely, especially on a beautiful sunny day; it lifts your spirits. Cooking, dressing, gardening and general housework are all difficult, but I try to do what I can slowly, but it may only be 15 minutes of doing something before I am in pain and tired.
By far the biggest change we had to make was having to move from a house to a bungalow. I was struggling with the stairs and with the hilly area where we lived at that time. I think it was then that I realised I could no longer do what I used to: simple things like walking, shopping and sadly, working. Life has become limited in many ways, both with large obstacles and with the small, seemingly unimportant things that have now become a challenge. However, I manage as best as I can, help is always around me and my husband cares for me, so we have "adapted" together.
I guess that apart from the big move, I have come to accept that I cannot do the things I used to, and if I do anything above my capabilities now, then I know I will pay for it for the next 24 hours at least, with the pain and breathlessness that will result. Still, I am aware that I need to rest, sleep, and recharge my batteries.
Overall, there is great understanding from all my family and friends. However, on my part, I do feel guilty and that I am a burden, although they all tell me constantly that this is NOT the case! When I am having a bad day with a lot of pain, or if my breathing is causing me grief, I know they understand that I need to rest and let my body take the time it needs to heal itself ready for the next round.
I feel that living with these conditions has definitely changed my perspective on life. Suddenly, the things you did and took for granted become difficult to do and restrictions are placed in your way, but life is short; and I do what I can do. I try not to live on the "what if's.” I enjoyed my work and I still miss it to this day, but you have to deal with today and the future, not the past. I still have my family and feel ready for what challenges and changes my conditions may bring to me in the future. I try to remember that I am still me, just a little ragged around the edges!
Most days, I stay as positive as I can. There are hard days and there are frustrating days, but every day to me is a bonus, so I would say to try and keep a smile inside and remember you are worth everything and life is very precious. If you can find alternative ways of doing things, then do not be afraid to try something different. Getting angry with yourself and the world will not change anything.
Not everyone understands the symptoms to look out for and the impact of these conditions. I was 16 when my Raynaud’s was diagnosed, but then it took years to find out that I had scleroderma. People need to be more aware of what can happen. Raynaud’s is an under-estimated illness: the pain can be unbearable. One day I would like to wake up with no pain. Once you are diagnosed it all falls into place, and then you can understand it as well. Then, when you do understand it, you can learn to live with it and to deal with it."