"...Now I am thinking about getting pregnant again..."

Q: I was diagnosed with HELLP syndrome last August.  I started feeling what I thought was morning sickness soon after I found out I was pregnant.  My doctor prescribed B-12 and Benadryl.  It worked for the most part, but on occasion I would get a very sudden extremely painful feeling in my upper stomach. Soon after, I would vomit and then feel better.  I have been pregnant before without complications or morning sickness so I didn't realize this pain was not normal.  I simply suffered through. I then went to my regular appointment where my doctor routinely tested me for preeclampsia.  I felt fine but my blood pressure was 180 over 110.  My protein levels were elevated.  I had swelling around my ankles.  They sent me to the hospital. I was then transferred by ambulance to another hospital in
Denver, Presbyterian St. Lukes.  They have a high-risk maternity department.  Within 5 days my condition had progressed to the point where I was screaming in pain (from my liver). My liver enzymes were doubling by the hour. They sedated me and the next morning performed a C-section. Our baby, Rachel Eileen, lived for about 30 minutes. It was the most horrible thing I have ever had to go through. 
Fortunately we already had a child, Trent Alexander, who was 2 at the time.  We were forced to cope and move on for his sake.  Now I am thinking about getting pregnant again.  I just found out that I have two genes where 1 of the pair is abnormal which affect blood clotting. I am wondering what this means for me.

A: Clotting abnormalities, (thrombophilias), have received a great deal of attention with regard to preeclampsia and HELLP Syndrome. Some have reported a strong association and recommend treatment with heparin.  Others have not found this association.  To date there have not been trials that indicate that heparin treatment prevents HELLP.

In your case, it depends what these abnormalities are.  Some, (e.g. Factor V Leiden), where one gene is abnormal and the other is not is very common.  Another, MTHFR deficiency, can be treated with folic acid supplementation.  Thrombophilias, such as a lupus inhibitor, (not inherited) can be a very serious medical condition.

You are at risk for HELLP and preeclampsia in another pregnancy.  This does not mean that you cannot have a successful outcome.  Continue to see your high risk specialist and coordinate your care with them.

Thomas Easterling M.D.
University of Washington

Q. I was wondering if you were able to put me in touch with anyone who might be able to answer a question for me regarding antiphospholipid syndrome. Given that all other lab values are normal are elevations in the following labs enough to make a diagnosis of APS or do you need other labs to be positive?
 
Elevations in:ATIII, Protein C, Anti-B2GPI Antibodies IGA and IGM,  Anti-phospholipid antibodies IGM., and plasinogen and MTHFR Gene 677c t (positive heterozygous), MTHFR Gene 1298s c (positive Heterozygous), Plasminogen Activator inhibitor 1 (positive heterozydous).
 
Any help you could provide would be appreciated.

A. These are tests for thrombophilia ( not all APS):

- Elevations in:ATIII,  - low is abnormal
- Protein C, - low is abnormal
- Anti-B2GPI Antibodies IGA and IGM,  related to antiphospholipid  usually accompanied (+) lupus inhibitor or lupus anticoagulant
- Anti-phospholipid antibodies IGM., and related to antiphospholipid usually accompanied (+) lupus inhibitor or lupus anticoagulant
- plasinogen and  MTHFR Gene 677c t (positive heterozygous),  heterozygote not abnormal  - corrected by taking folic acid.
- Mthfr Gene 1298s c (positive Heterozygous),
- Plasminogen Activator inhiitor 1 (positive heterozydous).


Thomas Easterling M.D.
University of Washington


Q.  My name is Jessica.  I am friends with Lauren.  She forwarded me an email about the prospective link between mothers who had HELLP Syndrome during pregnancy and metabolic disorders in children. I had HELLP and post HELLP at 33 1/2 weeks of pregnancy. I have mentioned this to my pediatrician and asked if my son could be tested for metabolic disorders. He responded that there are so many metabolic disorders it would be impossible to test for all of them. He asked if I had heard which types of metabolic disorders are suspected. Do you happen to know which ones are suspect and/or any articles discussing this topic? Any information would be greatly appreciated. 

A. There were a couple of papers that suggested that a certain rare metabolic disorder in the fetus could lead to HELLP syndrome symptoms in the mother.  From what I understand, this is a major metabolic problem (not subtle at all).  At 33+ weeks, the cause of HELLP is more likely to be secondary to preeclampsia (as opposed to early onset).  If the baby is doing well, there is little chance that a metabolic disorder is present. The specific disorder is thought to be a deficiency of acetyl-carnitine enzyme. (We have looked in several babies and not found any useful
correlation.)


Dr. Mark Polak
Department of Pediatrics
West Virginia University Children's Hospital



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