Sally's story

The first sign of this condition for me was definitely the Raynaud’s. This started when I was 14, and my hands would be really blue all the time. I don’t get the typical attacks as such, but my hands just seem to be in a permanent state of spasm.

I went to the GP at the time, but he completely dismissed me. He just said that I had cold hands and I needed to stay warm! I had gone there by myself, and the whole experience just left me feeling so deflated that I thought it must be nothing after all.

Things changed when I was 18, after I moved to London to start my nurse training. I went to see the GP because I had a chest infection, but when they saw my hands, they sent me straight to A&E.

I was admitted to hospital immediately, and now it felt like there was quite a lot of urgency. At that point I was under the care of the vascular team rather than rheumatology, and I stayed in hospital from Friday until Sunday. I was given heparin infusions all weekend to thin the blood, but the moment the infusion stopped, my hands would be blue again. I had lots of blood tests that picked up some autoimmune antibodies and I also saw the rheumatologist, which is how I became known to them.

After I was discharged, I remained under the care of the rheumatologist at Charing Cross Hospital in London, where I had more tests and investigations. I was told I had Raynaud’s, but scleroderma wasn’t mentioned until about a month later. They had found calcium on my arms and said that this was probably connected to something, but they didn’t say what that was at the time.

When I was given the diagnosis, they called it CREST syndrome. Today, my official diagnosis is limited cutaneous systemic sclerosis, although some doctors have suggested it could be diffuse because I have so many abdominal issues. My care has now been transferred The Royal Free Hospital in London.

In the beginning, I had quite a few heparin infusions. Today I am prescribed immunosuppressants, and I also take losartan and nifedipine, along with hydroxychloroquine because of fasciitis in my legs.

I am given iloprost every six months via an infusion, and I take sidenifil for the Raynaud’s, plus I have had Botox in my hands.  Most recently, I have had lipotransfer, or fat transfer surgery, in my face.

The last few years have been more difficult, as I now have serious issues with my eyes. I have retinal vasculitis, and also new blood vessels growing in the eyes; and if they bleed it can cause blindness. This has caused me the most anxiety, and it is all related to scleroderma. I am now under the care of Moorfields Eye Hospital, but even they have never seen this before; they could only find two other cases documented worldwide.

For me, the skin involvement is worst on my legs. They now feel rock solid, so walking can be difficult, and I often have to sit down and take pain relief. From my knees down and also from the elbow down I get a lot of calcium, which can also be very painful. Sometimes it feels as if my legs don’t want to move at all.

Anything that requires dexterity can be tricky. I often have pain in my hands, my feet, and my legs, so I don’t do the shopping; my husband does it instead. I also can’t go down the frozen aisles in the supermarket because I have Raynaud’s in my tongue, which means I can’t talk properly. I have to wear hearing aids because it also affects my hearing.

I would say that fatigue is probably the part that has the most impact on my life. Sometimes I will be so exhausted that I have to say: ‘I’m going to bed,’ because I just can’t do any more. It also means that I have to be careful when I’m making plans. I would love to be out and about all the time, but it’s just not possible.

I have recently started working from home. I was a prostate specialist nurse, and then I went into school nursing 11 years ago. During the COVID pandemic I had a lot of health issues that were all connected to scleroderma, so ever since then I have worked from home permanently, as a community staff nurse specialising in school health.

In 2024, I underwent a lipotransfer procedure to the face, that took place at the Royal Free Hospital. I had the pre-op appointment by telephone in early December 2023, and the hospital already had a lot of my information because I was having regular blood tests. Then, one day, I got another phone call, and I was offered a cancellation appointment, to have the lipotransfer procedure in just 10 days’ time.

On the day of the surgery, I had to arrive at the hospital by 10am after fasting from midnight. It was a very long wait, and I didn’t go down for the surgery until 4.30pm.  

The operation was carried out under general anaesthetic, and I think I was in recovery by about 6.30pm. I felt very groggy for about an hour, but then I got up and dressed. I was discharged the same evening with antibiotics and pain relief, and I felt better afterwards than I thought I would.

I had a small wound on my abdomen from where the fat was harvested, and two small stitches on either side of my mouth. During the surgery, they applied the filler either side of my mouth and above my lips.

Overall, I have felt absolutely fine. The swelling has gone down a lot, and I feel so pleased with the outcome!  I feel like it has really improved my confidence, and I am very glad that I did this. It helps my physical health and especially my mouth, and there are emotional benefits as well, because it is definitely confidence-boosting. I went back to work on the Monday, although I think if I didn’t work from home, I might have delayed my return, as working from home is not very physical!

I don't know how long the effects will last, and I will be reviewed in clinic in three months’ time.

Last updated: June 2024