There is no hint of exaggeration when Michelle Fincham describes her children Grace and Freddie as ‘an inspiration’.
It is exactly the right word for the siblings, who have an extremely rare skin condition - at the time it was diagnosed they were among only eight such cases in the world.
Desmoplakin deficiency is painful and potentially life-threatening, but they don’t let it stop them dancing and playing football.
The condition is a form of Epidermolysis Bullosa (EB), which effectively means their skin is so fragile that contact can lead to sore and painful blisters.
Freddie, 10, says: ‘Our skin gets very delicate when we bash it and it really hurts.
‘We then gets bits and bobs, like sores, which blow up and that makes it very hard to live with our condition.’
The youngster is a keen footballer and refuses to let his condition stop him playing in goal.
He also has to be monitored during PE at his school in Southsea.
Freddie explains: ‘If we hurt ourselves, it can become a problem but I just really want to get up and keep going.’
Grace, 11, says: ‘It is hard because, as our skin is so delicate, I could just knock my foot by accident by just tripping over myself and I will get a big graze, so we have to be quite careful with what we do.
They have never shied away from anything at all. They are very keen on getting involved with activities, even if they could have an effect on them.Peter Fincham
‘It is quite hard to live with, but to know that there are only eight people in the world that have had our condition, it makes us feel really quite special about it.’
While their form of EB is considered extremely rare, the more common strands of EB, where mild blistering occurs on contact, is estimated to affect around one in every 17,000 children born in the UK, with an estimated 5,000 children living with it.
There is currently no cure for the siblings’ condition, which is life-threatening.
‘The condition itself is quite common, but our type of EB is really rare, so for us there’s no real way of knowing what happens next’, Grace adds.
The duo were inspired back in July 2014 when the ALS Ice Bucket Challenge became a craze on social media.
People all around the world donated funds to the ALS Association and poured a bucket of ice-cold water over their heads to raise awareness for the charity that helps people with amyotrophic lateral sclerosis (ALS), otherwise known as Lou Gehrig’s Disease, which affects the muscles, leading to difficulties in speaking, swallowing and breathing.
Following the viral craze, the ALS Association announced in July 2015 that, thanks to $1million in donations received from the challenge, researchers had identified a gene that is the cause of the disease.
The challenge inspired Grace and Freddie because since they were first diagnosed with the condition, they have been receiving help from British charity Debra, which supports individuals and their families who are affected by EB.
The family are avid fundraisers and the children decided to try to start their own viral craze called the Pebble Challenge, in which they called on people to put a flat pebble in their shoes and walk or run a distance of their choice.
They encouraged people to publish their videos on YouTube and, after three months, they’ve managed to raise £1,740 for the charity.
Grace says: ‘We loved seeing the Ice Bucket Challenge videos and thought we could do a challenge.
‘I thought that we could put pebbles in our shoes because that would basically feel like having our skin condition. So we made a few videos, posted them on YouTube and had a huge reaction.’
Michelle, 36, who lives in North End, Portsmouth, says: ‘They really are an inspiration to me as they have no intentions of hiding behind their condition or letting it affect the things that they want to do or the way they live their lives.
‘They have a great quality of life and that’s down to their determined spirit, amazing lust for life and their desire to be as normal as they can be.’
She adds: ‘At times Grace can be quite indifferent about it, deciding some days at school that she won’t wear her wig and other days, she wants to. Of course they both understand how serious it is, but it is not something that we want to obsess over and we just want to let them have the life of a normal child.’
Before the diagnosis, Michelle and the siblings’ father, Pete, thought it was a form of eczema.
Michelle added: ‘There have been tough times over the years, but we have never wanted them to feel like they are victims in any way.
It’s incredibly hard to see them in pain but it really could be a lot worse.’
Grace and Freddie’s father, Peter Fincham, who lives in Southsea, says: ‘They have never shied away from anything at all. They are very keen on getting involved with activities, even if they could have an effect on them.
‘Freddie has his love of football while Grace is a keen dancer and loves dramatics. Yes, it’s a difficult illness to deal with, but they are constantly overcoming challenges and really do make me feel so proud.
‘They are from the school of ‘‘man up” and do not want to sit around and moan.’
Pete’s close friend, Dave Rodrigues, is running a series of marathons and duathlons to raise funds for Debra after being inspired by the siblings’ Pebble Challenge.
So far, the 42-year-old from Southsea has taken on nine challenges, including the Great South Run.
To donate to Grace and Freddie’s appeal, go to justgiving.com/fundraising/TheFinchamKidsDebraPebbleChallenge. To donate to Dave’s duathlon appeal, head to justgiving.com/fundraising/RodriguesDuathlon
Epidermolysis Bullosa (EB) is a condition in which any trauma or friction to the skin causes painful blisters or sores.
There are many variants of EB, each with slightly different symptoms.
So far 27 different variants have been discovered and more could be identified in the future.
In most cases, symptoms of EB are obvious from birth or soon after, but it can be diagnosed later on in childhood.
The condition is caused by faulty genes and, in most cases, can be inherited from one or both parents.
However, there are cases where neither parent has the gene but their child does.
There is currently no cure for the condition, with treatment aiming to relieve symptoms and prevent infection from occurring.
It is estimated one in every 17,000 children born in the UK will have a form of EB. For more information, go to debra.org.uk.