Antiphospholipid (Hughes) Syndrome

Antiphospholipid syndrome, also known as Hughes Syndrome, is an autoimmune disorder which causes an increased risk of blood clots. People with this condition are at an increased risk of developing:shutterstock_53224042_height-400.jpg

  • Deep vein thrombosis
  • Arterial thrombosis
  • Blood clots in the brain
  • Pregnant women have an increased risk of miscarriage or stillbirth

What causes antiphospholipid syndrome? 

With antiphospholipid syndrome the immune system attacks healthy tissue; abnormal antibodies are produced which target proteins attached to fat molecules, making the blood more likely to clot.

Diagnosing antiphospholipid syndrome

Blood tests are used to diagnose antiphospholipid syndrome; these tests look for antibodies responsible antiphospholipid syndrome.

How antiphospholipid syndrome is treated

Antiphospholipid syndrome cannot be cured; however, it can be managed successfully. Blood thinning injections, such as fragmin can be self-administered and aspirin can also be taken to help thin the blood. These medications can also improve a pregnant woman’s chance of having a successful preEIS05000.jpggnancy.

Pregnancy 

Antiphospholipid syndrome can cause recurrent miscarriage or stillbirth, as well as other pregnancy complications. At least 15% of recurrent miscarriages occur as a result of antiphospholipid syndrome, and with prompt treatment, the pregnancy success rate has risen from 20% before 1990 to over 80% today.

Pregnant ladies are usually treated daily with low dose (75mg) aspirin, and if a previous loss has occurred in the second or third trimesters they may also be given fragmin injections.

Pregnancies can be affected in a number of ways:

Early pregnancy loss

Most miscarriages occur during the first 13 weeks; antiphospholipid antibodies can cause early miscarriages by preventing the embryo from embedding properly in the womb. Early miscarriages are common, and there are many possible causes. Therefore, women will not be tested for antiphospholipid antibodies until they have three consecutive early miscarriages.

Late pregnancy loss

In most pregnancies foetal death in the second and third trimesters is rare; however, it is strongly associated with antiphospholipid syndrome and therefore women with a late pregnancy loss should be tested for antiphospholipid antibodies. Women with antiphospholipid syndrome can develop clots in the placenta or around the cord which reduces the baby’s oxygen supply.

Pre-eclampsia 

Pre-eclampsia is twice as likely to occur in women with antiphospholipid syndrome.

Intrauterine growth restriction 

Intrauterine growth restriction (IUGR) are babies with a very low birthweight and they usually weigh less than 90% of babies at the same gestational age. With antiphospholipid syndrome the reduced blood flow to the placenta can cause the baby to be small for dates.

Research

What is being done to treat Antiphospholipid (Hughes) Syndrome? And what research is in place to find things to help someone with this condition? I recently came across ‘The London Bridge Hospital’ website, they are a private hospital which specialises in treating Lupus and Hughes syndrome as well as conducting clinical research.

From The London Bridge Hospital website ‘In 1983, our group described a condition in which there was an increased tendency to blood clotting. Such clots can occur at any time, and can affect veins or arteries anywhere in the body. During pregnancy, clotting of the placenta results in a tendency to miscarriage – some women suffering a dozen or more miscarriages before correct diagnosis and treatment. Features include leg and arm clots (DVTs), headache and migraine (leading in some to stroke), memory loss, chest pain, cold circulation. We initially reported the condition in a group of lupus patients but soon recognised that it could occur in individuals without any evidence of lupus’

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Clinical Studies for Antiphospholipid (Hughes) Syndrome Research

Units throughout the world are researching Antiphospholipid Syndrome; The unit at St Thomas’ obtains some funding from the Hughes Syndrome Foundation and publishes up to 40 research papers each yeaaps3r; and The London Bridge hospital is now carrying out studies with groups in America, Argentina, Brazil, France, Italy, Japan, Portugal and Spain. The International Antiphospholipid Syndrome meeting (Dr Hughes first started this in 1985) is now in its 12th meeting, and it attracts up to 1000 doctors and researchers. The discovery of Antiphospholipid Syndrome in obstetrics has meant that the odds of carry a baby to term has increased dramatically; before the discovery patients only had a 20% chance of a successful pregnancy but treatment patients now have an 80% chance.

General treatments

The aim of treatment is to ‘thin’ the bloody so that the tendency to clot is reduced, this is achieved with anticoagulant medication. There are currently three main anticoagulant medications (aspirin, heparin or warfarin) and for most patients one low dose aspirin (75mg) a day is enough to stop the blood from clotting. Heparin (such as Fragmin) is a very effective anticoagulant and is offered in injection form. It cannot be used for long term treatment due to an increased risk of osteoporosis. It is, however, used in some Antiphospholipid Syndrome pregnancies, as warfarin is potentially toxic to the developing baby.

Aspirin 

  • aps5Low dose aspirin (baby aspirin) – at a dose of between 75mg to 100mg a makes the blood platelets less sticky.
  • Side effects are rare (indigestion; allergy especially in asthmatics)
  • Safe in pregnancy

Heparin

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  • Newer “low molecular weight” heparin (such as Fragmin) has replaced older preparations
  • Only available as injection
  • Used to treat thrombosis, prior to warfarin use
  • Safe in pregnancy

Warfarin (Coumadin)

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  • Warfarin is a safe and effective medicine; despite being dismissed as ‘rat poison’ by some journalists
  • 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 ‘International’ scale called ‘INR’ ‘Normal’ is “1” – half thick blood is “2” and one third blood is “3”
  • Not allowed in pregnancy

Placental Insufficiency

Overview

The placenta is the baby’s lifeline during pregnancy, it’s an organ which grows in the womb and is connected to the baby via the umbilical cord; it provides baby with Oxygen and nutrients which pass from your blood supply into the placenta and are then carried to your baby via the umbilical cord; carbon dioxide and other waste products are also carried away from the baby by the umbilical cord to the placenta and then into your bloodstream for disposal. Hormones produced by the placenta help your baby grow and develop; It offers your baby protection against bacterial infections while in the womb, and towards the end of pregnancy it passes antibodies from you to your baby which should give him or her immunity for about three months after birth. HowevPlacental+insufficiency1er, it only passes on antibodies that you already have. Alcohol, nicotine and other drugs can also cross the placenta and can cause damage to your unborn baby.

Placental insufficiency (also called placental dysfunction or uteroplacental vascular insufficiency) is a serious pregnancy complication, and usually occurs when either the placenta does not develop properly or it is damaged.

With placental insufficiency, the placenta is unable to provide the baby with adequate oxygen and nutrients; and without this the baby cannot grow and thrive. This can lead to low birth weight, premature birth, birth defects and stillbirth; therefore diagnosing placental insufficiency early is vital for improving the outcome for both mum and baby.

Causes

It is often linked to blood flow problems. While maternal blood and vascular disorders can trigger it, medications and lifestyle habits are also possible triggers. You are more at risk of having placental insufficiency if you are overdue, have diabetes, chronic high blood pressure (hypertension), blood clotting disorders, anaemia, are a smoker, or take drugs (especially cocaine, heroin, and methamphetamine); it may also occur if the placenta doesn’t attach properly to the uterine wall.

There may be areas of dead tissue, called infarcts, within the placenta which result in reduced blood flow in those areas; often these are caused by a problem with the vessels within the placenta. Certain conditions are known to increase the number of infarcts within the placenta, such as pregnancy-induced hypertension. Infarcts don’t usually affect the unborn baby; however, in certain cases and especially in women with severe hypertension, the reduced placental blood flow may be enough to cause poor growth and even stillbirth.

Symptoms

Regular antenatal (prenatal) appointments are essential as there are no maternal symptoms for placental insufficiency. However, the mother may notice that her bump appears small or her baby may be moving less than expected.

Complications

A pregnant lady who has placental insufficiency is at greater risk of preeclampsia, placental abruption, and preterm labour and delivery. However, the risks are far greater to the growing baby and the risks for the baby include:

  • Oxygen deprivation at birth (which can cause hypoxic ischemic encephalopathy (HIE), seizures, brain damage and cerebral palsy)
  • IUGR
  • Learning disabilities
  • Hypothermia
  • Low blood sugar
  • Low blood calcium levels
  • Excess red blood cells
  • Premature labour
  • Death

Diagnosis and Management

Regular antenatal care can improve outcomes as it helps your provider to diagnose placental insufficiency earlier. A range of Tests are used to diagnose placental insufficiency; these include: taking measurements of the fundal height during routine antenatal appointments, pregnancy ultrasound scans to measure the size of the placenta, the size of the baby, and check the placental blood flow, non-stress test to measure the baby’s heart rate, movements and contractions, and sometimes you may have a blood test to check the alpha-fetoprotein levels in the mother’s blood.

If you have high blood pressure or diabetes then treating these can help to improve the baby’s growth. Placental insufficiency can’t be cured but if it’s detected early enough then it can be managed with regular antenatal care. The consultant may recommend looking for any signs of preeclampsia, more frequent appointments, regular ultrasound and foetal doppler scans and if necessary admission to hospital so that it can be continuously monitored. You should also pay very close attention to your baby’s movements and kicks, and any changes in these should be assessed straight away. If there is concern about premature birth then you may receive steroid injections; these dissolve through the placenta and strengthen the baby’s lungs.

Future Pregnancies

In future pregnancies, you are likely to be monitored much more frequently with many more ultrasound and doppler scans and more antenatal appointments. Your consultant may suggest you take low dose aspirin, although you should speak to your doctor first about this, and may also suggest anticoagulant injections such as Fragmin or Lovenox… this will however depend on the reasons for your placental insufficiency. If you have diabetes then this will be carefully monitored and a suggested treatment plan will be put in place; and any bad habits such as smoking or drug misuse should be addressed.

There really isn’t very much that can be done to prevent this from happening. However, careful monitoring and good antenatal should improve the outcome.

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