Horn Blower

NHS. For the undiagnosed Behçets Patient this can
be a disaster, jeopardising sight, digestive tract
and even life itself.What is Behçet's disease?
Behçets disease is a rare, chronic, lifelong disorder
that involves inflammation of blood vessels
throughout the body. It is a multisystem disease; it
may involve all organs and affect the central
nervous system, causing memory loss and
impaired speech, balance and movement and
symptoms of Behçet’s always tend to include
recurrent oral and genital ulcers, and eye
inflammation. The effects of the disease may
include blindness, stroke, swelling of the spinal
cord, and intestinal complications. Behçet’s tends
to come and go in a series of attacks (‘flare-ups’)
throughout life, however for those diagnosed at a
young age life expectancy can be greatly reduced.
Although Behçet’s disease is considered incurable
at present, incurable does not mean untreatable.
There are several ways in which the immune
system can be suppressed to an appropriate level
to reduce the extra inflammation, and this
suppresses the symptoms.
What is a rare disease?
A disease is defined as rare when it affects less
than 1 in 2,000 citizens.
No-one knows for sure how many people with
Behçet’s disease there are in the UK, but it is
estimated that there are about 1 in 100,000 - that
is, about 1000 people.
The rare disease patient is the orphan of health
systems, often without diagnosis, without
treatment, without research, therefore without
reason to hope.
Rare diseases are life-threatening or chronically
debilitating diseases with a low prevalence and a
high level of complexity.
Patients with very rare diseases and their families
are particularly isolated and vulnerable. The life
expectancy of rare disease patients is significantly
reduced and many have disabilities that become a
source of discrimination and reduce or destroy
educational, professional or social opportunities.
Research on rare diseases is scarce.
Children with Behçets
It may be a surprising statement but clinicians and
experts have no idea how many children have
Behçets in the UK, neither does the Society, our
best guess is 40 to 50. Children (under 18) who
have Behçets tend to suffer greatly with lack of
diagnosis and lack of treatment (there are no
licenced drugs for Behçets in the UK). The
inexperienced doctor is reluctant to treat a young
patient who exihibit Behcet symptoms and often
they can be hospitalised under observation for
weeks with little or no medication at all. The
Society would like to complete research into the
number and location of young Behçets patients
and the British Paediatric Rheumatology Group
are experienced at carrying out this type of work
and have graduates who can over a 12 to 15
month period attempt to gain the information we
need to both highlight the numbers and express
concerns over treatment. This work is estimated
to cost £15,000 and would cover the whole UK
resulting in a clear picture of numbers, location
and treatment, information invaluable to our new
centres of excellence (London Birmingham and
Liverpool) and to the patients themselves who can
look forward to more rapid diagnosis and early
treatment.
Five Patient Concerns
Following are the 5 main concerns of patients who
are Behçets sufferers and sum up the difficulties
encountered in A) getting a diagnosis and B)
dealing with the disease once it takes a hold.
There are many unusual symptoms that appear
over a period of time and maybe not appear all
together; this leads to problems with diagnosis.
The GP is the first port of call and most have never
dealt with a patient with Behcets. There is no
diagnostic test for Behçet's.
The sufferer doesn't always look ill. However the
person can have ulcers of the mouth, legs,
genitals, swollen joints, severe and continuous
headaches and many other debilitating symptoms.
They have overwhelming fatigue and feel really ill;
they find it difficult to function properly on a day
to day basis. A lot of serious complications can
occur with the illness, for example, if untreated,
blindness can occur very quickly and other serious
health problems are a distinct prospect.
Patients can very quickly feel isolated and lonely
when dealing with this illness. They feel
overwhelmed. They can lose their confidence,
their job, their income, and any relationship they
had; they can become dependent on benefits that
are difficult to obtain because of the intermittent
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