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

Bereavement Midwives – Experiences and Opinions

My own experience with the hospital bereavement midwife wasn’t a very positive one and I wanted to discover whether more can be done to ensure that people are more supported by the bereavement midwives after they leave the hospital. I went about asking for experiences and opinions from other loss parents; and to be honest it is completely mixed! Some had amazing support, while others had awful, or non-existent support!

My Experience

After we lost Sophie we were assigned a bereavement midwife by the hospital; she wasn’t working the day that Sophie was born and therefore we didn’t meet her before the birth; however, we were induced two days after we were told that Sophie had died so she really could have made contact during that time to offer support and explain what would happen… it’s a very scary time and that would have been helpful! She was working the day after Sophie was born, but she still didn’t come to the bereavement suite to meet us before we left as she was ‘really busy’, so the hospital chaplain came in her place.

In fact the first time I spoke to her was the day after we got home when I wanted to go back to the hospital to see Sophie before she went for her post mortem; I telephoned her number and left a message for her to call back… which she did and then she met me at the hospital. The first time I met her she seemed pleasant, she asked how we were coping and if there’s anything we need help with. I asked for a referral to bereavement counselling for my son (I am still waiting for her to do this and ended up going through my GP!). After I visited Sophie she explained about how long the post mortem would take etc, and said that she would keep in contact to see how we were; and to please phone if I need to talk to someone as that’s what she’s there for.

I did telephone a few times (always got the answering machine!), and it always took 24 – 48 hours to receive a call back, and then she was nice on the phone but didn’t action anything she said she was going to!, she has never once visited me or invited me to any appointments, she did not attend Sophie’s funeral and she has not acknowledged Sophie since… I have heard from some people who, for instance, received a letter or a ‘thinking of you’ card on their baby’s birthday… we didn’t even get a phone call! I personally think she’s in the wrong job!

Experiences by others

I asked members of the Sophie’s Angels support group for their experiences and opinions, and the comments were completely mixed! Some positive and some negative. I thought this would give an idea as to what more can be done to support families who have lost a baby.

Positive

I’m always pleased to hear positive experiences about bereavement support… these are some of the comments that were made:

‘So far… AMAZING. I had a side room and the same 2 lovely nurses who looked after me from being admitted to being discharged. Nothing was too much trouble, pain was well managed. Small acts of kindness like letting my husband come/go and stay as much as I/he wanted without restricting us to visiting hours. Giving him a pass to the car park so we didn’t rock up a huge parking debt. They fed my husband and brought him cups of tea/coffee. The way they were with Dexter, how they dressed him and complemented him, the gorgeous memory box and ‘birth certificate’ (not an official one as he was born at 20 weeks), the way they brought him to me as many times as I wanted, the pass they gave me to come back to the ward to come and see him as much as I liked after I was discharged. The photographer they got to come and take pictures of the 3 of us, the chaplain they arranged to come and see us, the bereavement midwife who came to see us… if it’s possible to have a ‘positive’ experience whilst going through this I certainly did’

‘My son died at the children’s hospital where they had a group of people specialist in bereavement. They took hand and prints as well as a foot cast of my son, gave us a box with a candle, an angel, seeds to plant a flower, a box to put some of his hair in. The phoned every so often to check on us and they were wonderful’

‘I could write so much about all of the amazing care I received especially my amazing bereavement midwife Nikki. She was my rock when we lost Amelia last year. This time when we lost Sophia in June The care at the hospital was fantastic again. We were in the snowdrop suite again which is nice, my husband never had to leave they set up a bed so we could sleep together. They gave us our beautiful memory box and took hand and footprints for us. Nikki the bereavement midwife came straight up to the snowdrop suite to see me when she heard I was back. I could tell you so many things she’s done for me. She has gone above and beyond. Today she came to visit me and she knew I wanted to go back to the hospital to hold my baby as I didn’t have chance when she was born. My husband didn’t want to see her again and as she didn’t want me to go alone she took me. Sat with me when I cried and hugged me when I needed it the most. She then took me for a hot chocolate and a chat’

Negative

Unfortunately there are also many negative experiences, and more can most definitely be done to help with bereavement support; either before, during, or after the birth:

‘My experience wasn’t so good I was on labour ward for a whole week and I wasn’t allowed in the quiet room till the last night. The midwife’s where lovely, gave me a memory box hand and foot print had a cold cot were really respectful of him asked his name etc and talked to him which was comforting. I can’t fault the midwives, they gave me a lot of emotional support while I was in the labour wars for 7 days hearing births; another lady came in during that time at 39 weeks to deliver a stillborn baby which was heart-breaking all the more. I was given some leaflets and that was it when I was discharged, SANDS send me a letter to attend a candle lighting event every 6 months, but that’s all aftercare I have received’

‘My bereavement care was so poor. The whole care from admission to discharge was disgusting actually. Felt really let down and totally robbed of all the things, the little memories I never got to do that I will never get back. They only had 1 bereavement midwife for the hospital, and whilst she was off the week I was admitted no one stepped in and took her place to guide me and my partner through the process of it all. I had a different midwife and Dr every day and night literally no continuity of care what so ever. The memory box was left outside the room, they all avoided the room like the plague avoided all the questions I had. The midwife who delivered my angel was nice but she didn’t do the care to her full potential, she covered my baby with a towel as if she was a bit of trash, I was totally rushed with my baby, I was given 8 hours with her which I will forever treasure, but I was kept in that night and my baby sent to the mortuary even though they had a cold cot there. Also I wasn’t offered to see my baby again by the hospital, the bereavement midwife txt me and called me when she got back off holiday and explained that the staff hadn’t given me a bereavement info pack that I should’ve got which I later got posted out to me. Also I had to figure a lot of things out on my own. The staff were all so under trained with bereavement care (and I wasn’t even a difficult patient). I was 37 weeks pregnant when I had my little girl who was born sleeping, with no complications what so ever and no cause of death from a full post mortem’

‘I never had one. Wasn’t even offered one. I’ve just struggled through on my own’

‘My care was horrific! My bereavement midwife was none existent. Counselling was a great help but wasn’t offered until 12 weeks after, and by that time I had already attempted suicide! Nothing got explained, because of this we missed out on a lot of memory making’

‘Mine was terrible. I lost my daughter on the 8th April at 17+2 and I’m still trying to get help. I have taken an overdose too. I was assigned a bereavement midwife but only spoke to her once and that was to tell me Elsie had had her post mortem. I’m still waiting for the results. The care I received whilst suffering my miscarriage was on another level of shocking. I’ll never go back to the hospital concerned if I’m ever lucky enough to have my rainbow’

Conclusion

As far as I can see there are some bereavement midwives who go above and beyond to really support the families, while others could do with retraining so that people feel supported and cared for. Small gestures make all the difference… phoning or visiting to see how you are, offering help and support, referring to outside agencies who may be of benefit, attending the funeral, becoming a friend, being approachable and easy to talk to, returning telephone calls, visiting before the birth, organising the birth photographer, hand and foot prints etc, offering to help with any arrangements, recommending funeral directors… there are many things that can be done to help the grieving family and as a bereavement midwife or support worker they really should be doing everything that they can to make this difficult time slightly easier.

Please feel free to join Sophie’s Angels, it can really help to connect with other parents who are going through loss.

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Foetal Development – An Overview

There are three trimesters in pregnancy and each has different developmental characteristics. The estimated due date (EDD) is set by calculating 40 weeks from the first day of the last monthly period (LMP); which means that when fertilisation occurs the woman is already two weeks pregnant.

For a detailed view of foetal development week by week – with pictures! Please click here.

In the first trimester, when an ovum (egg) is released it travels down the fallopian tube towards the uterus; the corona radiata (outer layer of the ovum) contains follicular cells that are difficult to penetrate; spermatozoa (sperm) must then secrete a digestive enzyme in order to weaken the corona radiata. Once the sperm have penetrated the egg the chromosomes combine to form a zygote (a one-celled body which contains 46 chromosomes); hCG is then released to increase progesterone levels and stop your period. The zygotes’ cells divide rapidly until there is a cluster of 16 cells (a morula); fluid collects within the morula creating a separate outer layer which encapsulates the inner cluster of cells, the inner layer will eventually become the embryo and the outer layer will form the placenta; there are now 58 cells in the structure and it is now called a blastocyst.

The blastocyst then burrows into the uterine wall (implantation). During the embryonic period (weeks five to ten) the major structures begin development; there are three layers to the embryo; the outer layer (ectoderm) forms the outer layer of skin, nervous systems, eyes, inner ears, and connective tissues; the middle layer (mesoderm) forms the heart and circulatory system, along with the bones, muscles, kidneys and the reproductive system; and the inner layer (endoderm) becomes a tube lined with mucous membranes ready for the development of the lungs, intestines and bladder.

While the placenta is forming the embryo is nourished by the yolk sac. The brain forms and the heart is starting to pump blood through the main blood vessels; the tissue which will become the spine is growing and has developed somites; the eyes are beginning to form, and arm and leg buds are developing. The neural tube then closes and the ears and nostrils begin to develop; the lungs are also forming. By the seventh week the arm buds look like paddles which will develop into fingers. The spine eventually begins to straighten and as the arms continue to grow they can bend at the elbows; toes then start to form and all of the essential organs have begun to grow. The eyelids then fuse shut, and the intestines rotate. At the end of week ten the embryo is termed a foetus. Red blood cells begin to form in the liver, tooth buds appear and the external genitalia starts to develop into either a penis or clitoris. By the end of this trimester the embryo has the appearance of a miniature human.

During the second trimester the intestines (which have been growing in the umbilical cord) return to the abdomen and bone tissue is developing. The ovarian follicles start to form in females and the prostate appears in males. Red blood cells are developing in the spleen and bones have also begun developing, with movements becoming better coordinated; the ears move near to their final position and the foetus can hear sounds outside of the womb; the mouth now makes sucking motions. Fat stores form under the skin, and vernix (a greasy coating) covers the foetus offering protection from abrasions and chapping due to the amniotic fluid. The next stage of development is the swallowing reflex; the foetus swallow’s amniotic fluid and then urinates before swallowing again, this helps to mature the lungs; lanugo (soft, fine hair) covers the foetus helping to keep the vernix in place; and meconium is now made in the intestinal tract. The foetus begins to show signs of rapid eye movements, fingerprints are starting to form and taste buds are developing. In females the uterus and ovaries which contain a lifetime supply of immature eggs are in place and in males the testes have started to descend from the abdomen. Bone marrow is now making blood cells and the startle reflex is developing; the foetus may respond to sounds with movement. Surfactant is now being produced in the lungs, allowing the air sacs to inflate and deflate. 

In the third trimester the foetus rapidly gains weight; the bones are fully developed although still soft, and red blood cells have formed in the bone marrow. The eyes open and the pupils can now detect light; the brain is growing rapidly and during the thirty-first week the central nervous system can control body temperature. The lungs begin to practice breathing, lanugo starts to disappear and the foetus begins to absorb minerals (iron and calcium) from the intestinal tract. At thirty-seven weeks the foetus is classed as early term, all organs are able to function and the head may begin its descent into the pelvis. The foetus is considered full term at 40 weeks.