BY TOBY PORTER
The Countess of Wessex has officially opened a new rare diseases centre which takes into account the problems which patients encounter in other wards.
Her Royal Highness met patients at St Thomas’ Hospital’s Rare Diseases Centre who have benefited from the project, as well as the doctors and nurses who work there.
She heard how the centre was designed specifically for patients with complex rare genetic conditions, and how it has transformed the experience they have when coming to hospital. The rare conditions treated at the Centre include:
- Epidermolysis bullosa (EB), a potentially fatal skin blistering condition which causes constant pain
- Xeroderma pigmentosum (XP), a genetic disorder affecting patients’ ability to repair the damage caused by ultraviolet (UV) light, significantly increasing their risk of skin cancer and eye disease as well as related disorders of DNA repair
- Bardet-Biedl syndrome (BBS), a genetic disorder that can cause blindness, obesity and kidney disease as well as affecting other parts of the body
- Ichthyosis, a family of rare genetic skin disorders characterised by dry, thickened, scaly skin.
The countess, who is patron of the national EB charity DEBRA, which helped to fund the new facility, was given a tour of the centre.
She was told how its features have been tailored to meet the needs of the patients.
For example, it is UV-free for XP patients, has furniture with curved edges so that it does not catch on fragile skin and includes flooring and wall colours specifically chosen based on feedback from visually-impaired patients.
The centre also contains a comfortable communal space in which patients can wait between appointments, which does not have a typical clinical environment.
The area has a forest-themed feature wall – the idea of this is to bring the outside world in, as some patients may not be able to go outside much due to their conditions.
Work is also under way on a new sensory garden located outside the centre, which will offer patients a quiet haven in which to reflect, socialise and relax when it opens later this year.
Guy’s and St Thomas’ is a national centre for patients with XP and is one of only two centres in the UK for adults with EB, and one of three for BBS patients, which means patients often travel from all over the country for their regular appointments.
After a tour around the centre, Her Royal Highness unveiled the plaque which will be displayed there.
She said: “It is a huge pleasure for me to open the Rare Diseases Centre. The testimonies we’ve heard from the patients with different rare conditions make it clear why this was so necessary.”
The Rare Diseases Centre has been funded by Guy’s and St Thomas’ Charity, the charity DEBRA, the Four Acre Trust and the Photodermatology Charitable Trust.