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How the disease is treated
 Due
to the increased tendency to clot, the main aim of treatment
is to 'thin' the blood, so that this tendency is reduced.
This is usually achieved for most patients, by providing anticoagulant
(literally anti-clotting) therapy based on the extent of the
clotting.
Main
types of treatment
There are currently
three main medications – aspirin, heparin or warfarin.
For many thousands of patients, one “junior” aspirin
a day (approximately 75-80 mg, or one quarter of a regular aspirin)
is all that is required. Its main action is to make the platelets
of the blood less "sticky" and is sufficient to hold
off clotting. It is the main medication used in pregnancy in
Hughes Syndrome patients with previous miscarriage.
For those who have
actually had a thrombosis, the choice is between heparin and
warfarin. Heparin is a very useful anticoagulant but unfortunately
is only available as an injection, and cannot be given long
term because of an increased risk of osteoporosis. It is,
however, used in some Hughes Syndrome pregnancies, as warfarin
is potentially toxic to the developing fetus.
For most people with thrombosis, warfarin is the treatment
of choice. Used for many decades warfarin is the 'gold standard'
anticoagulant tablet and, provided that the anticoagulation
dose is monitored carefully to test that the patients blood
is thinning to the correct degree, it is an extremely safe
drug. Those taking warfarin are monitored with a standard
clotting test called the INR (International Normalized Ratio)
- a procedure carried out either in GP's or hospital anticoagulant
clinics, or, increasingly, by the patient themselves with
self-testing
INR machines.
For other symptoms,
most patients are treated on a case-by-case basis, or ailment-by-ailment
basis. Plaquenil is an effective treatment option for general
symptoms such as rash, fatigue and pain.
| Notes
on the main three treatments |
| Aspirin |
- Low dose
aspirin (‘junior aspirin’, ‘baby
aspirin’) – at a dose of between 75mg
to 100mg a day is known to be an effective anti-clotting
agent, making the blood platelets less sticky.
- Safe.
Side effects are rare (indigestion; allergy especially
in asthmatics)
- Not sufficient
treatment of those with major thrombosis or stroke
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| Heparin |
- Newer
“low molecular weight” heparin (such as
‘Clexane’ or ‘Fragmin’) has
replaced older preparations
- Only available
as injection
- Useful
as a ‘first line’ (immediate action) treatment
in thrombosis, prior to warfarin use
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| Warfarin (Coumadin) |
- Despite
being dismissed as ‘rat poison’ by some
journalists, warfarin is an extremely safe and effective
medicine. It has a good long track record having been
around since 1950s
- Side effects
are rare – the only vital issue is to keep the
dosage correct
- The thinning
of the blood achieved by warfarin is measured on an
scale called ‘INR’ (International Normalized
Ratio): normal’ is “1”; half thick
blood is “2” and one third blood is “3”
(think of it as ‘full cream’, half cream’,
‘skimmed milk’)
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For most patients who have suffered a thrombosis, an INR of
2 is used. However, many patients with Hughes Syndrome –
especially those with headaches require more intense blood
thinning so an INR of 3 or more is maintained. For more information
about living with anticoagulants, please visit Anticoagulation
Europe.
Other drugs
- Clopidogrel
(PLAVIX) – This drug has a mechanism similar to aspirin
in making platelets less ‘sticky’. Although
expensive, there are real reasons for looking at Plavix.
Firstly, Plavix does not have the irritant effects on the
stomach which can occur with aspirin (fortunately rare with
the low dose). Neither is it a problem in asthmatics, who
may prove allergic to aspirin. Thirdly, huge studies in
cardiology, where aspirin and Plavix have been compared
in the prevention of heart attacks, have discovered some
evidence that, under certain circumstances, Plavix may have
the edge over aspirin.
- Intravenous
Immunoglobulin (IVIG) – This consists of an intravenous
preparation of protein (globulin) pooled from a large number
of donors. It is extremely expensive, and its action is
short-lived. Some years ago, it was found to help in some
cases of thrombocytopenia (low platelets) and has since
been used in a variety of autoimmune diseases with mixed
success. Despite its apparent limitations, it has a good
safety record and is being tested in some patients with
Hughes Syndrome, particularly those with low platelet counts.
Immunosuppressives
– These drugs (most commonly azathioprine and methotrexate)
are widely used in autoimmune diseases such as lupus and
rheumatoid arthritis. They have proved disappointing in
patients with primary APS. So also has plasma exchange –
an attractive idea for removing antibodies, but with little
convincing published success – as yet.
Antimalarials – hydroxychloroquine (Plaquenil) is
an extremely useful drug in lupus and Sjögrens Syndrome.
It is particularly effective in helping skin rashes, fatigue,
and aches and pains. One of the additional actions of Plaquenil
is as a (mild) anti-clotting agent – rather like junior
aspirin. Thus, in lupus patients with Hughes Syndrome it
might well have extra, hidden, benefits.
How you can help yourself
In theory, increasing the amount of essential fatty acids (EFAs)
in your diet, particularly omega-3 EFAs found in oily fish,
should help reduce the risk of thrombosis, but there are no
clinical trials to suggest this is the case.
It is worth noting that fish oils also contain large amounts
of vitamin A, which may be harmful in pregnancy.
No other 'alternative' treatments have been shown to help.
 Patients
can also reduce their risk of thrombosis from causes other
than the Hughes Syndrome (APS) by making lifestyle changes.
They should stop smoking, make sure they are a healthy weight,
and take regular exercise.
The oral
contraceptive pill increases the risk as does oxygen pressure
changes such as high altitude flying or diving.
You should be aware of the signs and symptoms
of Hughes Syndrome and report any of these to your doctor
as soon as they occur.
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