Second Verse Different from the First!
I first met Tyra about two years ago. At that time, she was sent to me by her personal physician for management of the acute onset of severe hypertension in pregnancy. Unfortunately, she did not just have hypertension, she had one of the most severe forms of preeclampsia (pregnancy-induced hypertension), HELLP syndrome, and was only 22 weeks’ gestation. HELLP stands for Hemolysis (break down of red blood cells), Elevated Liver enzymes (secondary to damage to liver cells), and Low Platelets (secondary to consumption of these blood clotting factors). Not only was she just 22 weeks’, but the baby was severely growth restricted, had very little amniotic fluid, could barely push blood through the placenta, and was basically dying from placental insufficiency in utero. Despite our efforts to stabilize her disease, she deteriorated rapidly, and we had no choice but to inform her that she had to be delivered. We also had to tell her that we would not be able to do anything to save her baby. Using a prostaglandin drug called misoprostol, we were able to quickly accomplish this without a surgical procedure in a very short period of time. The baby’s heart rate was not monitored during her induction and he died sometime during the labor process. Other than being only half the size he should have been at 22 weeks, the baby was perfectly normal, and we all had tears in our eyes as she and her husband held their tiny stillborn child.
A few weeks afterwards, she returned for a consultation to review the events surrounding her delivery. I learned many years ago that only a small portion of any explanation is absorbed coincident to such tragic events, so it is usually worthwhile to repeat and expand upon any previous discussion when the patient is ready. All of her laboratory studies had returned to normal, but her blood pressure was still slightly elevated, and I suspected that she had some degree of chronic hypertension. Indeed, during the course of her hospital stay with us, we had found (unbeknownst to her previously) that she only had one kidney and it was thought that this might be the source of underlying hypertension that had contributed to the severe superimposed preeclampsia at such an early gestational age. We reviewed the placental pathology report and found changes that are often seen in abnormalities of placentation associated with autoimmune disorders or thrombophilias (conditions associated with an increased tendency to form or slowly dissolve blood clots). She had no family history, or other past medical history, to suggest she might have one of these conditions.
Baseline kidney studies were done and returned remarkably normal, despite her single kidney (and what it had recently been through!). Screens for subclinical autoimmune disorders and thyroid disease were also normal. She was found to be a heterozygote for a mutation in the methylenetetrahydofolate reductase (MTHFR) gene, but had no other evidence for a thrombophilia, and single gene abnormalities in MTHFR are rarely associated with the severe problems she had had. With this reassuring information in hand, I asked her to return when she thought she was ready to get pregnant again, and we would discuss options for ‘empiric’ therapy since she wanted to do “everything possible that was safe” to reduce the risk of recurrence for the problems she’d had and if there is nothing else I have learned through the years, it is that history does tend to repeat itself in the pregnancy complications arena.
About nine months later, she did return. She was “thinking about getting pregnant again” but wanted to know the unanswerable question, “Am I going to get sick and lose another baby?” My only response was that she was at greater risk, but in the absence of a specific medical problem or laboratory abnormality, I honestly did not know what that risk was (I usually tell folks 5-25% under these circumstances to account for the ‘unknowns’ we haven’t yet learned to look for), although I did tell her that if it happened again, it was highly likely it would happen a third time. She told me that she “appreciated my honesty” and asked what we could do to minimize her risks. To make a long story short, we started her on a prenatal vitamin, high-dose folic acid (4 mg per day), a baby aspirin (81 mg), and I told her to return within two weeks of a missed period and we would add prophylactic heparin therapy (5,000 U SQ twice per day) to this empiric regimen.
So she left and returned two months later, pregnant again. We confirmed fetal viability by ultrasound, did a few more laboratory studies, started her on the heparin, and embarked on another pregnancy with our fingers crossed. All the follow-up studies were normal, including a “quad screen” at 16 weeks, with specific attention to the alpha-fetoprotein level (frequently elevated in severe abnormalities of placentation such as she had previously). The baby had no gross abnormalities seen by ultrasound, grew well, and had no Doppler flow abnormalities detected on serial ultrasound examinations. At 28 weeks’, I recommended that we discontinue the heparin (which she did with some reluctance) and we simply plugged her into frequent office visits with ‘antepartum fetal surveillance.’ At 38 weeks’, her blood pressure began to rise and we admitted her (yesterday) for induction of labor which resulted in the outcome noted in paragraph one.
Tyra and her husband asked that I be present for the delivery “if at all possible” and I agreed. Indeed, I was grateful for the opportunity. We had been through a lot together with the first delivery, we had taken a chance with another pregnancy, we had tried a relatively ‘safe’ approach to empiric therapy, and we now had a good outcome (even if we will never know if the medical therapy was the difference). I was there today as much for me as for Tyra and her husband, not for the ego stroke, but to bring closure to the previous experience by sharing in the joy that they and their families (and many friends) got to share today - one of the rewards of this profession that I will always consider to be “priceless.”