Frequently, when reading about a rare disease, statistics are given which show how rare the disease is; usually expressed in a number within the population that it has affected. The EU definition of a rare disease is that it affects fewer than 1 in 2000 people in a country. As the UK population is about 64 million there would need to be less than 32000 with a particular condition for it to be rare.
For the first aHUS Patients Conference in June 2011 ( click here for conference website) the number of people with aHUS in the UK was calculated at 172. So by rare disease standards, aHUS is extremely rare. The number of patients with the disease at any particular time is also described as its prevalence. The prevalent number of patients can be added to as new incidents of the disease occur. So since June 2011 the prevalence of aHUS has grown by the incidence of new aHUS cases.
An estimate for incidents of aHUS each year is very difficult to make and in a patient population so small, a range of +/- 5 make quite a significant difference.
Of the 172 prevalent UK aHUS patient population in June 2011, 134 were in England. The estimated incidence of aHUS in England is about 25 cases per year ( about 2 per month); so by the start of 2014 the prevalent number of patients with aHUS in England should have increased to around 194 (+/- 12). Indeed since 1 January there should have been another 3 incidents.
Over that time, some of the new incident patients have become known to aHUSUK and most of them have become members of aHUSUK and helped to gain a greater understanding of the disease and get treatment for all who need it. They have also also helped to raise funds for research and to make aHUS information available. This all benefits the 100, or so, likely new patient incidents in the next four years who will add to the prevalent number; as better diagnosis and eculizumab will prevent any reduction.
There is still more to be done and it would be great if all the prevalent aHUS patient population in the UK would join in with aHUSUK’s existing members to help them battle the disease.Membership is free and members can be as active as they wish.
So for Rare Disease Day this year, if you have aHUS and have not done so already, why not join together with others at aHUSUK?