Antiphospholipid Antibody Syndrome


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Introduction

This pamphlet is a layman’s terms summary of Antiphospholipid Syndrome (APS).  It covers such topics as diagnosis, symptoms, treatment, and coping.  It is meant for patients newly diagnosed, however would also be good for informing friends and family about your disease.

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What is Antiphospholipid Antibody Syndrome?

Antiphospholipid Antibody Syndrome or APS is an autoimmune disorder in which the body recognizes certain normal components of blood and/or cell membranes as foreign substances and produces antibodies against them. Patients with these antibodies may experience blood clots, including heart attacks and strokes, and miscarriages. APS may occur in people with systemic lupus erythematosus, other autoimmune diseases, or in otherwise healthy individuals.

APS is also known as APLS, APLA, Hughes Syndrome or "Sticky Blood."

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APS is an Autoimmune Disease

One way in which our immune system fights infections is by making antibodies.  Antibodies are proteins in the blood and body fluids that bind to foreign invaders like bacteria and viruses and help the immune system destroy and remove them.  Sometimes the immune system doesn’t function properly and makes antibodies against normal organs and tissues in the body.  These self-reactive antibodies are called autoantibodies.  The autoantibodies in APS were originally thought to recognize that recognize certain phospholipids, fatty molecules that make up part of normal cell membranes, hence the name “antiphospholipid” antibodies.  It is now known that most of the autoantibodies in APS patients actually recognize certain blood proteins that bind to phospholipids, not the phospholipids themselves.  Two blood proteins that are major targets of antiphospholipid antibodies are b2-glycoprotein I and prothrombin.

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APS: The Statistics

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Clinical Features of APS

People with antiphospholipid antibodies have an increased risk of developing one or more of the following problems:

Other features that might be associated with antiphospholipid antibodies include:

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How is APS Diagnosed?

Physicians use a combination of clinical symptoms (see above) and laboratory tests to diagnose APS. The common blood tests for antiphospholipid antibodies are as follows:

Panels of tests for antibodies to phospholipids other than cardiolipin are available but have not undergone the rigorous international standardization efforts applied to anticardiolipin assays.  A number of experts in the field question the usefulness of these panels, which may be quite expensive.

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APS Treatment

There is no cure for APS, but there is treatment.  The treatment of choice for patients with APS who have had a blood clot is anticoagulant therapy.  This is usually successful in preventing further clots.  For women with APS and recurrent miscarriages who have not had a prior blood clot, the use of anticoagulant therapy during the pregnancy significantly increases the likelihood of a successful outcome.  Some individuals may have elevated antiphospholipid antibodies but have no clinical manifestations of the syndrome.  These individuals are usually treated with aspirin.  Aspirin reduces the risk of blood clots by making the platelets less sticky.  Studies are ongoing to determine how helpful aspirin is and whether low doses of anticoagulants might be more effective. 

In general patients who have had a blood clot (i.e., stroke, heart attack, DVT) and have persistently positive tests for antiphospholipid antibodies should be treated with anticoagulants indefinitely.  Discontinuing treatment after a fixed period of time, such as six months, may be quite dangerous in such patients.  In some patients with a history of blood clots, antiphospholipid antibodies may disappear after a certain period of time.  It is not known whether it is safe to stop anticoagulation in this situation.  Consultation with a doctor experienced in treating APS is recommended for such patients.

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Doctors and Information on APS

Although APS is actually one of the more common autoimmune diseases, some primary care doctors remain uniformed about it.  When their patients have symptoms of APS, these doctors may not test for antiphospholipid antibodies soon enough or at all. Unfortunately, many patients have had to see several physicians and specialists before getting the proper diagnosis and treatment.

The type of doctor a patient sees should be determined by the symptoms the individual patient is having, and any given patient may benefit from the input of several specialists. For patients with blood clots, a hematologist would be involved, often for management of anticoagulant therapy (blood thinner). For patients with recurrent miscarriages, a high-risk obstetrician should be consulted.  For patients who also have rheumatologic symptoms, such as symptoms of lupus, a rheumatologist would be important to see. Of course, all patients would benefit from having a single physician identified as their primary care provider, to help coordinate all of their health care needs.

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Women & APS

APS and Pregnancy Complications

Women with APS may have difficulties with pregnancy. During pregnancy, women are at higher risk of developing blood clots and preeclampsia.  In APS, pregnancies are thought to be lost because blood clots form in the placenta and starve the baby of nutrition.  Some women may have trouble getting pregnant, while others may experience repeated miscarriages. Blood clots that develop in the placenta can cause fetal growth problems, fetal distress, preterm birth, or pregnancy loss.

Expert care and close monitoring of the pregnancy is essential by a doctor knowledgeable about APS.  During pregnancy, physicians may recommend low doses of aspirin and daily injections of the blood thinning drug, heparin.  This gives the fetus about an 80% chance of survival, a drastic improvement from the 1980's when fetal survival was around 20%. The therapy is started at the beginning of pregnancy and halted just before delivery to reduce the risk of bleeding during childbirth. Soon after birth, the treatment resumes for about six weeks because of an increased risk for clotting in the postpartum period. In a more serious case preeclampsia may set in towards the end of pregnancy and a planned premature birth may be necessary.  Heparin can cause bone loss, so women may need to take additional calcium during pregnancy. In addition, women need to be monitored for development of a low platelet count.

Over the long term, many doctors recommend women continue to take a low dose of aspirin to reduce the risk of developing dangerous blood clots.  Many women with APS are unaware they have the condition, but it can be diagnosed with a blood test. Doctors may consider the diagnosis when a woman has repeated, unexplained pregnancy loss.

If you are trying to get pregnant or are pregnant it is very important to let your doctor know immediately. Continued use of warfarin may cause birth defects. The doctor will change your medication to a different blood thinner that is safe. Using proper treatment, women with APS have about the same risks as other women during pregnancy.

APS pregnancies are not normal. Normal pregnancy is 40 weeks. In APS, it is more common to deliver the baby between 30-35 weeks, and between 3-5 pounds. Heparin protects the placenta partially, but not fully so that the baby gets enough nutrition to survive longer in the mother. Once born, the babies do fine.

Many women who have problems with APS during pregnancy are completely fine when not pregnant. Others do go on to develop problems with clotting.  Currently there is no way of telling which women will be unlucky, until a clot actually occurs.

Infertility has also been linked to antiphospholipid antibodies. Testing for these antibodies is becoming routine in infertility clinics.

Birth Control Pills & Hormone Replacement Therapy

Women also need to avoid estrogen therapy (such as birth control or hormone replacement therapy) because estrogen predisposes patients to clotting.

Other forms of contraception should be discussed with your doctor.

Problems with Periods

Some women taking warfarin experience problems with increased bleeding. It can lead to anemia.  Tell your doctor about this problem. The doctor can recommend several options and prevent anemia. One example is: for women who have already given birth and are not actively trying to conceive, the Mirena® IUD has been successful in reducing period blood loss. As it only releases hormones to the uterus lining and is not absorbed into the blood stream, therefore, it is safe for women with APS to use.

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Other Points to Consider

You may notice you bruise more easily or little cuts will bleed longer when you are taking warfarin (Coumadin®).  Injuries can be more serious when on anticoagulants and care should be taken during any activity that can result in injury. Contact sports are not recommended.

If, while on anticoagulants, you injure your head, go directly to the Emergency Room. Your brain is very sensitive to a bleeding while on anticoagulants.

If a serious injury does occur go directly to the Emergency Room and be sure they know you are taking anticoagulants and tell them what your most recent INR was.

You should have Medical Alert bracelet and wear it at all times. You can order one through Medic Alert at http://www.medicalert.org. A bracelet is the most visible, the easiest and the most recognized however, there are also necklaces and other types available.

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Safety When Traveling

Long trips, especially by air, have some clotting risk even for non-APS people.  It is important for people with APS to get up and walk around at least every couple of hours. On long car trip stop at least every two hours and walk. Drink plenty of water and wear compression stockings to help reduce your chance of DVT.  If you plan to be away during the time of a periodic blood test, arrange for the blood test before you leave for the trip.

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Take Your Medication

It is very important to take your medicine every day. Try to take your the medicine at the same time each day for consistency.  You may want to get a pillbox that holds at least one week’s supply of the pill(s) or to mark it on a calendar when you take your medicine.  This will help you to know when you have taken your pills.  Do not take two doses in one day if you have forgotten your dose.

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Symptoms to Watch For

If you test positive for APS Antibodies you should be aware of the symptoms caused by blood clots. If any of these symptoms occur, seek medical help immediately.  Symptoms that could be caused by a blood clot include:

Heart Attack

Chest discomfort or pain. Most heart attacks involve discomfort in the center of the chest that lasts more than a few minutes, or that goes away and comes back. It can feel like uncomfortable pressure, squeezing, fullness or pain. Generally becomes so bad that it may feel unbearable and relentless, but occasionally the pain can be milder.

Stroke

Strokes (Blood Clot in the brain) can be life threatening. Some stroke symptoms may last only minutes or a few hours and are called TIA’s. (Transient Ischemic Attack or mini stroke.) Rapid treatment (within three hours) is vital.  Medical treatments that reverse the stroke damage is available—but only if you seek immediate treatment.  Stroke symptoms are:

Clots in other locations

Muscle pain, numbness, or tingling, pale color, weakness, muscle spasm in a leg or arm.

Heart attacks, strokes and other blood clots need immediate medical attention. Go immediately to Emergency Room.

f you feel these warning signs, DIAL 911 IMMEDIATELY. Most people wait 2 or 3 hours before seeking care, yet by then the heart may suffer significant damage. Dial 911 within 5 minutes if you or someone you are with experiences these symptoms.

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Coping with APS

APS treatment is life long. Treatment of blood clots caused by APS outweighs the small side effects by treatment.

Most of the time people with APS will appear on the outside just as they did before they were diagnosed. Because of this, it may be difficult for family and friends to understand that you have a life threatening illness and that you just can’t do some of the things that you could before. They can’t see what is going on with your body and this can become quite frustrating on both ends.

In some cases you may want a therapist to help you adjust. Any person with a life threatening disease is at risk of being depressed. In some cases this progresses to clinical depression. It is very important to discuss your mental health with your doctor as well as your physical health.

The APS Foundation of America, Inc has a place on the Internet where you can communicate with others who have this disease. You can find our support forum at http://www.apsforum.com. You are the only one who knows exactly how you feel. Others with the same disease are very understanding and support groups of some kind can make your life much fuller. Your immediate family and children should be told about your disease, how it affects you, what it means to them as well as what you may have to expect of them in support.

The good news is: many people with proper treatment live normal full lives. Others may find their lives changed forever due to APS, but a positive attitude will mean that life can still be worthwhile and fulfilling as the correct treatment prevents further problem.

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Other Sources for APS Information

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How can you help?

There are many ways you can help the APS Foundation of America, Inc.  You can volunteer your time & talents such as in the area of fundraising, advocacy, finance or support group experiences, donate money or purchase APS gear through our webpage at www.apsfa.org, through our CafePress store at http://www.cafepress.com/apsfoundation or through our Zazzle store at http://www.zazzle.com/apsfoundation.

The APS Foundation of America, Inc is a non-profit organization.  Your donations are greatly needed to help us provide awareness, support, and education of this disease.  We need your enthusiasm and monetary support to help our individuals, family, friends and caregivers battle the long-term consequences caused by APS.  Thank you in advance for your support and time.  Please contact us through our website or at 608-782-2626 for more information.

Please send donations to:

APS Foundation of America, Inc
Post Office Box 801
La Crosse, Wisconsin 54602-0801

We also accept PayPal donations via our website.

We thank you for your support!

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Disclaimer

APS Foundation of America, Inc. is not intended to replace standard doctor-patient visits, physical examination, and medical testing. Information given to members is only an opinion. All information should be confirmed with your personal doctor. Always seek the advice of a trained physician in person before seeking any new treatment regarding your medical diagnosis or condition. Any information received from APS Foundation of America, Inc is not intended to diagnose, treat, or cure. This brochure is for informational purposes only.

If you think you may have a medical emergency, call your doctor or 911 immediately.

A team of people contributed to this publication. Information was adapted from various website, books and other media sources.  Please contact us through the website for a complete list of sources.  This pamphlet was assessed at draft stage by doctors, allied health professionals, an education specialist and people with APS. A non-medical editor rewrote the text to make it easy to understand and an APS Foundation of America, Inc. medical editor is responsible for the content overall. 

©Copyright 2005, 2006. All rights reserved. All content in this pamphlet is protected by U.S. and international copyright laws and may not be reprinted, reposted, or otherwise reproduced wholly or in part without prior written permission from the APS Foundation of America, Inc. Bibliography available here.

Revised October 2006

 

Page last update: 11/13/10

The APS Foundation of America, Inc. website and forums are both volunteer run and funded by donations to the APSFA.

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DISCLAIMER: APS Foundation of America, Inc. website is not intended to replace standard doctor-patient visits, physical examination, and medical testing. Information given to members is only an opinion. All information should be confirmed with your personal doctor. Always seek the advice of a trained physician in person before seeking any new treatment regarding your medical diagnosis or condition. Any information received from APS Foundation of America, Inc. website is not intended to diagnose, treat, or cure. This site is for informational purposes only. Please note that we will be listing all donor or purchaser's names on the Donor page of our foundation site. If you do not want your name listed, please contact us to opt out. If you think you may have a medical emergency, call your doctor or 911 immediately.

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