Thursday- March 30, 2006 2:30pm- LUPUS ANTICOAGULANT

Today at 2:15 as I sat in my office preparing for a 2:30 meeting with some big clients, Dr. O Sitti calls. She is a blood specialist I went to see several weeks ago because Aunt Sissy had been diagnosed with a genetic blood disorder. I decided to get tested just as a precaution. Well, as I said, Dr. Sitti called me herself. She is from Thiland and speaks with an accent. She said "Stephy,I need talk you about test. First, you know you pregnant? You not tell me you pregnant!" I told her well, I didn't know I was when I came in to see you but yes I just found out. She said "well, this confusing to me but,you tested positive for Lupus Anticoagulant." Things got very quiet on the phone. I was basically like freaking out. She said "You okay? You calm down. We take care you and everything be okay." I am still sitting there in silence. I don't know what Lupus is so I am thinking the absolute worst (this is some form of cancer), I come out with... "Am I dying from this?" She says "No! Goodness you not die! We going to test you some more and we gona get to the bottom of this. We treat this and we get it taken care of." WHEWWWW... I am not dying... that is all I can think or hear at this point. She tells me she will have orders for me Friday am for more test. I need to have them done asap on Monday. I agree! My nerves are completely SHOT and I have totally missed my 2:30 appointment. Thank goodness for my assistant Chrystie who got on the phone with the clients rescheduled our appointment and called Kevin to come to my office. I am blessed to have her on my team and a saint for a husband! Talk about shock...I think I don't like surprises anymore. This surprise thing is getting to the point that it is not funny anymore. Please keep us in your prayers as we go throught this series of test and search for some answers.

I must admitt have been a little hesitant to look up these terms on the internet and I just keep hoping the test are wrong. But, I will post here some information to give you all a better understanding of what is going on. If there is anyone who is reading this who has been through this disorder while pregnant please share with me. It would really help put my mind at ease.

The term "lupus" is used when talking about many different forms of the disease. When someone says, "I have lupus," he or she could be affected in many different ways depending on the type of lupus present.

TYPES:
Cutaneous Lupus: ACLE, SCLE, CCLE, or DLE
Systemic Lupus: SLE
Drug- Induced Lupus
Overlap: RA, Myositis, Sjogren's, Scleroderma
Neonatal Lupus (rare)
Even within the same type of lupus, each case is unique, symptoms range from mild to severe and no two cases exact the same toll.

Cutaneous (skin) lupus: affects primarily the skin, but may also involve the hair and mucous membranes. It is frequently referred to as discoid lupus. Within lupus of the skin, there are types that cause different looking rashes and symptoms. These include: Acute cutaneous lupus erythematosus (ACLE)
Subacute cutaneous lupus erythematosus (SCLE), Chronic cutaneous lupus erythematosus (CCLE) or Discoid lupus erythematosus (DLE). Other terms used to describe specific forms of chronic cutaneous lupus include: verrucous DLE, lupus profundus, mucosal DLE, palmar-plantar (hands and feet) DLE, and lupus tumidus.

Systemic lupus erythematosus (SLE): can affect any system or organ in the body including the joints, skin, lungs, heart, blood, kidney, or nervous system. Symptoms of SLE can range from being a minor inconvenience to very serious and even life threatening. A person may experience no pain or they may experience extreme pain, especially in the joints. There may be no skin manifestations or rashes that are disfiguring. They may have no organ involvement or extreme organ damage. Most often when people mention "lupus," they are referring to the systemic form of the disease.

Drug-induced lupus erythematosus (DILE): is a side effect of long-term use of certain medications. Some symptoms overlap with those of SLE. Once the suspected medication is stopped, symptoms should decline within days and usually disappear within one or two weeks.

Neonatal lupus: is a rare condition acquired from the passage of maternal autoantibodies, specifically anti-Ro/SSA or anti-La/SSB, which can affect the skin, heart and blood of the fetus and newborn. It is associated with a rash that appears within the first several weeks of life and may persist for about six months before disappearing. Congenital heart block is much less common than the skin rash. Neonatal lupus is not SLE. "Lupus and Pregnancy" by T. Flint Porter, MD, MPH and D. Ware Branch, MD. Lupus News, Vol. 20, No. 5 Winter 2000.

Lupus in Overlap: The majority of people with lupus have lupus alone. Between five and thirty percent of people with lupus report having overlap symptoms characteristic of one or more connective tissue diseases. There are several well-recognized overlaps that may affect people with lupus including: lupus and rheumatoid arthritis (RA), lupus and myositis, lupus and systemic sclerosis (SSc or scleroderma), lupus and Sjogren's syndrome (SS). Blood disorders are common in lupus and can be very important. Hematologists, who are specialists in blood disorders, are often asked to be involved in the evaluation and treatment of patients with systemic lupus erythematosus (SLE). The principal hematological (blood) issues of interest are: Anemia: low hemoglobin or red blood cells ,
Thrombosis: excess blood clotting , Blood transfusion , and Bone marrow testing

Thrombosis

The body's blood is normally in a liquid state. When a person is injured or has surgery, blood thickens and plugs up the spot that is bleeding in a process called hemostasis , also known as coagulation or clotting . Hemostasis is a normal, vital function of the body. Sometimes in lupus, however, the processes of hemostasis are too strong, and a blood clot forms where it is not needed—or wanted. This condition is called thrombosis . It may be said that the difference between hemostasis and thrombosis is that the latter is too much of a good thing. If a thrombus , or clot, breaks off and travels elsewhere in the circulation, it is called embolus . Thromboembolism is fortunately not common, but it is always significant. Blood clots may affect the leg veins (sometimes with embolism going to the lungs), or the arteries to the arms, legs, or brain, as well as other places in the body. During pregnancy, blood clots can lodge in the placenta and disrupt nutrition to the fetus. A baby may be born prematurely with low birth weight, or may not survive to be delivered. Some women lose pregnancies over and over until a proper diagnosis is made and treatment given. Most thrombosis in lupus is associated with antibodies in the blood called antiphospholipid antibodies . The two blood tests most often used to detect antiphospholipids are the anticardiolipin test and the lupus anticoagulant test . (The lupus anticoagulant has a paradoxical name, since it is not really an anticoagulant in the body—it just looks like one in the laboratory.)

Diagnosis and treatment

The best treatment is anticoagulation (blood thinning) medication, such as warfarin (Coumadin and generics). Warfarin cannot safely be used during pregnancy because of a risk of birth defects in the middle of the first trimester, and a risk of fetal bleeding in the third trimester. Thus a woman who takes warfarin must switch to the injectable anticoagulant heparin or low molecular weight heparin as soon as she is pregnant. These are safe for the fetus. Aspirin and other anti-inflammatory drugs are not very effective antithrombic treatments when the problem is antiphospholipids. The best management of antiphospholipid pregnancy is not yet established, but often involves a combination of drugs. Careful, frequent monitoring of both mother and fetus by the obstetrician is an important part of overall care.

Lupus anticoagulants and Pregnancy

LA have clearly been associated with an increased risk of fetal loss due to pre-eclampsia, placental abruption, intrauterine growth retardation, and stillbirth. Some evidence suggests that this may be due to antibodies against the placental anticoagulant protein, annexin V. Placental infarction has been suggested as the cause of the failure to carry to term but pathological analysis has not definitely supported this contention. What about treatment? Well, we are looking at a thrombotic disease so we need anticoagulation, and as you would expect for acute thrombosis. You treat as you would any patient, usually with heparin followed by Coumadin. This anticoagulation should be continued for at least six months and it may be very important to continue it for a longer time depending on the status of the patient. If the lupus anticoagulant and underlying syndrome that perhaps the patient has disappears, you may not need longer anticoagulation. Now during pregnancy when this certainly occurs as well, what should one be doing? Patients who have recurrent pregnancy losses should, during their next pregnancy, receive low molecular weight heparin and again, oftentimes, low dose aspirin is added. The increase in fetal survival is about 50-80%, so this has been fairly effective treatment in these patients. I should add that there has been some debate about the level of Coumadin, Coumadin-ization that should be maintained in these patients. There is evidence in some people’s hands that they feel that higher dose or higher doses sufficient to cause the INR to be 3.5 or above is important. I think the answer isn’t entirely known.