Q & A
re
STICKY BLOOD SYNDROME
aka
HUGHES SYNDROME
aka
ANTI PHOSPHOLIPID
SYNDROME
aka
A P S
  • What does auto-immune mean?

    'Auto' means 'self', so autoimmune literally means that the immune system fights the body itself.
    Instead of fighting and attacking the bad tissues, such as viruses, it turns on itself amd attacks the good tissues.
    Many tissues such as thyrotoxicosis, Lupus and some types of anaemia may have an autoimmune mechanism.

  • What are Antiphospholipid Antibodies?

    Antibodies are blood proteins important in defence e.g. against infection.
    Some antibodies appear to turn against the bodys own tissues.
    Anti-phospholipid antibodies appear to alter the phospholipid skin that lines the blood vessels of organs.

  • Are there different forms of Hughes Syndrome?

    Clinically -- Yes.
    Some people have few clinical problems, while in others, the thrombosis problem is severe and recurrent.
    In yet others, the clinical problem appears confined to recurrent miscarriage with no history of further thrombosis.

  • What causes Hughes Syndrome?

    The exact cause is unknown, but it is likely to be a combination of factors, which in general contribute to the likelihood of having a thrombosis/clot.
    These include smoking, immobility (related for instance to the thrombosis seen after long air flights) and the contraceptive pill.
    There is a slight tendency for the disease to run in families and there may be a family history of clots, of miscarriages, or of other autoimmune diseases such as lupus and thyroid problems.
    A person's genetic make-up and exposure to certain trigger factors may provide the right environment in which Hughes Syndrome can develop.
    Occasionally the thrombosis occurs during an infection such as a sore throat.
    However, in the some people the thrombosis comes 'out of the blue' and from past medical histories over many years at times;
    (e.g. migraines and recurrent miscarriages help to make the diagnosis of Hughes Syndrome).

  • Can you catch Hughes Syndrome?

    No -- it is not infectious.

  • Is Hughes Syndrome a fatal disease?

    Unfortunately it can be -- often before the true diagnosis is fully established -- one of the causes of sudden collapse is a lung clot (pulmonary embolism).
    Once the diagnosis is made, adequate anticoagulation treatment should protect against a recurrence.

  • How common is Hughes Syndrome?

    Probably very common -- possibly as high as 1 in 500 of the population.
    It is suspected that people inherit a genetic component, as yet unknown, from their parents, which predisposes them to develop the Hughes Syndrome.
    They are not necessarily pre-destined to develop Hughes Syndrome, but they may be more susceptible than those without the genetic makeup.
    Relatives of Hughes Syndrome patients have an approximately 5-12% greater tendency to get the disease if family members have it.
  • If you have Lupus, does that mean you also have Hughes?

    No -- 1 in 5 patients with Lupus have 'Sticky Blood' and an increased risk of clotting.
    The reverse however, does not apply -- Most people with Hughes Syndrome do not have Lupus.
  • What is INR?

    INR (International normalized ratio) is a blood test which refers to the thickness of the blood on Warfarin treatment.
    This test compares the tendency for the patient's blood to clot against a standard blood result.
    A ratio of 1 is Normal
    A ratio of 2 is Half thick
    A ratio of 3 is Third thick -- and so on
    Patients with Hughes Syndrome and severe clotting problems such as stroke usually need an INR of around 3 to remain stable.
  • What lifestyle changes must be made to accommodate Hughes?

    Smoking and the oral contraceptive pill probably increase the risk.
    Oxygen pressure changes such as high altitudes and/or deep diving probably does increase the risk.
    At the present time, it is not known if any particular diets affect the disease directly.

  • Why is it difficult to diagnose?

    In theory, diagnosis is fairly simple.
    The blood tests are simple and cheap.
    The doctor simply needs to think of the possibility of Hughes Syndrome.
    Having said that, the features can seem so disconnected in different patients
    (e.g.memory loss, previous DVT, headaches, previous miscarriage and multiple sclerosis)
    that the diagnosis of Hughes Syndrome simply may not immediately come to mind.

  • What are the aCL and LA Tests?

    These are the two blood tests that are used to diagnose Hughes Syndrome.
    aCL stands for anti-cardiolipin
    LA stands for Lupus anti-coagulant
    *(an extremely confusing name as * it is not a  test for Lupus*)*
  • Do all Hughes patients have the same symptoms?

    No -- the diversity of symptoms is wide and can include headaches, flashing lights, giddiness, unsteady gait, and poor/cold circulation.
    Just to make life more difficult,  sometimes there are none of the above.
    The hope is that with increasing recognition of Hughes Syndrome by physicians and awaremess in patients worldwide, that more widespread blood testing for anti-phospholipid antibodies [aCL and LA] will bring many more people to the effective medical care and treatment they need.
  • Does taking Aspirin affect the test results?

    No -- the test checks for the cause not the treatment.
    However, patients on Warfarin can not have the LA test.

  • Can a cold or virus affect the test results?

    No -- not in any way known at this point in time.