'I started feeling unusual cold sensations in my fingers, then my airways started shutting off, now I take 250 tablets a week'

A 47-year-old dad who takes takes 250 tablets a week and relies on oxygen due to a rare health condition has surpassed his life expectancy and believes he still has "plenty left" to give.

Scott Nell had always been healthy but in November 2017 began experiencing unusual cold sensations in his fingers, which turned as white as paper. He later found it difficult to breathe, finding his airways were "shutting off" if he leaned to one side.

After numerous tests and consultations with specialists, the garden designer was diagnosed with diffuse cutaneous systemic sclerosis, a form of scleroderma. This rare autoimmune disease causes the skin to harden and thicken, can lead to severe organ complications, and can be fatal.

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In March 2018, Scott underwent monthly chemotherapy treatments for a year, now reduced to once every six months, to help soften the skin and tissue around his internal organs. However, he now struggles with lung function issues and pain due to the muscle-wasting nature of the condition.

Despite being given less than two years to live in March 2022, Scott, from County Durham, has outlived this prognosis and is now keen to raise awareness of the disease. He encourages others to seek early diagnoses so they can live longer and spend more time with their loved ones – for Scott, this means his wife Charlotte, 48, his 15-year-old daughter Tiffany, and his step-sons.

"I was originally given less than two years to live and I’ve exceeded that already, I still feel like I’ve got plenty left," Scott told PA Real Life.

Scleroderma is a rare, progressive autoimmune condition caused by the body producing too much collagen resulting in the hardening of the skin, according to Scleroderma and Raynaud’s UK (SRUK), the leading charity supporting people with the disease.

Approximately 19,000 people in the UK live with the condition which can stop parts of the body from functioning properly and, in severe cases, cause physical disability and be life-threatening. It is also common to have another condition called Raynaud’s alongside scleroderma, which causes the small blood vessels in the extremities such as the hands, feet, fingers or toes to be over-sensitive to changes in temperature, cold conditions and emotional stress.

Scott said he started to experience unusual sensations in his fingers in November 2017 – which was later diagnosed as Raynaud’s. "There was something going on with my fingers and one occasion, I was working and laying a drive and it was snowing but it was so cold, I couldn’t stand it," he said.

"I took my gloves off and my fingers were white, like sheet-of-paper white. I was really quite shocked and I had to go into the car and heat them."

Scott said he then noticed fluid appearing under the skin of one of his fingers and he also felt "excessively tired". He remembered: "I woke up one morning and I could not straighten my arms and legs out, it took me a few minutes."

This alarming symptom led Scott to book an appointment with his GP, where they conducted stool and blood tests. The blood test revealed a marker known as SCL70, an antibody found in approximately 20 percent of scleroderma patients, as per NHS information.

"My wife and I googled it but we never thought (scleroderma) must be what I’ve got," he admitted. It was during a consultation with a rheumatologist that Scott discovered indentations on his fingertips, which were producing calcium deposits.

"They were pushing away out of my fingertips... the skin was so tight you couldn’t grab anything and he said I had scleroderma," Scott explained. An X-ray of his lungs showed no issues at that time, even though scleroderma can significantly affect the lungs.

After some time on medication to manage his symptoms, by March 2018, Scott began to experience severe breathing difficulties. "As soon as I leaned over to one side, my airwaves just shut off," he described.

Struggling to breathe, he tried sleeping upright until it became unbearable. Morphine provided some relief for his breathing.

Eventually, a specialist in Newcastle confirmed his diagnosis with diffuse cutaneous systemic sclerosis, a form of scleroderma. He was advised to undergo chemotherapy treatment, saying the chemicals included can help soften the skin and tissue around the internal organs. "It’s not intensive, such as for a cancer patient, and I lost very little hair," he explained.

"I had chemotherapy every month for a year to start with and they reduced that to once every six months, which is where I am now."

Alongside this, Scott now takes around 250 tablets each week, saying it is a "whole arrangement". Some of this includes morphine, which helps with his breathing and pain, paracetamol, of which he takes around eight tablets a day, and antibiotics.

"I pretty much live in a certain amount of pain," he said. "I’m on tablets to stop me from having diarrhoea, tablets to stop me from being sick, immuno-suppressing tablets.

"It’s a lot. I think some of my tablets are to combat the ill-feeling from other tablets."

Scott also needs to use oxygen tanks when he is awake to help him breathe. "It’s difficult to sleep and I sleep on the sofa, it’s very rare that I actually sleep with my wife, because there I can position myself so I’m not tossing and turning," he said. The disease has also caused Scott’s skin to tighten.

"Sclero means hardening and derma means skin – so hardening of the skin," he explained. "It can produce a lot of calcium deposits, which can sometimes cause scarring and disfigurement. I think because I was diagnosed really quickly it saved the best part of my appearance – I think actually the tightening of my skin probably took a few years off me."

Scott said his wife, his daughter, Tiffany, and his step-sons find the disease difficult to process. "My line of thought is their suffering, not mine," he added. "It’s not going to be a quick process for me, I know I’m going to suffer for a long time and the end."

Scott advised those who may have received a diagnosis "not to panic". "If you think you might have scleroderma, don’t back off if a doctor suggests it could be something else," he said. "Anyone who has a confirmed diagnosis, I would say not to panic and there are new treatments coming out all the time."

"Early diagnosis is the key – the sooner you’re on treatment, the longer you have to live, the longer you’ve got with your family."

For more information, visit the website for SRUK here: sruk.co.uk.