HELLP Syndrome: A rare, scary pregnancy illness you should know about

In the summer of 2013, as I headed into the third trimester of my first pregnancy, I started to feel not quite right. Like any other anxious expectant woman, I turned to Google. Topics I searched over the following weeks included: “Headaches third trimester.” “Nausea third trimester.” “Nose bleed third trimester.” “Bruising third trimester.” “Swelling third trimester.” And on and on. According to my research, all these symptoms were normal on their own, and it didn’t occur me that they could be connected by anything other than being pregnant. Despite feeling progressively tired and unwell, I didn’t actually think anything was wrong. I assumed this last trimester was hitting me hard or that maybe I simply sucked at this pregnancy thing.

The night before my 33-week appointment, the nagging heartburn I’d had on and off turned into a searing pain that radiated across my chest, through my back and stabbed my right shoulder. It kept me up most of the night, but I wasn’t concerned enough to go to the ER as I was seeing my doctor in the morning. Exhausted and barely coherent, I reported my symptoms to my OB/GYN, who prescribed Zantac for heartburn. As I gazed up at a poster listing the warning signs of preeclampsia (and deciding, nope, not me), the nurse noted my blood pressure was a little higher than my last visit, but still in the normal range. She also made a passing comment about watching out for rib pain, which totally didn’t register—probably because I was focused on my terrible chest pain. (Looking back though, I’d specifically had rib pain a few weeks earlier.) All I could think about was how I would get through another six weeks of feeling this awful. I called in sick to work that day and the next, since the Zantac had done nothing to ease my suffering. I lay down for a nap before lunch and woke up in a puddle of fluid—my water had broken. After calling my husband in a state of shock, I walked over to my hospital (only a block from my home) to get checked out.

It took another five fearful days of bedrest in hospital, myriad blood tests and blood pressure monitoring, even an assessment for preeclampsia, and then a transfer to another hospital, before I received the diagnosis, a pregnancy illness I’d never heard of: HELLP syndrome.

What is HELLP? A variant of preeclampsia, HELLP stands for Hemolysis, Elevated Liver Enzymes, Low Platelet Count. It’s a life-threatening hypertensive disorder of pregnancy and like preeclampsia, it typically occurs after 20 weeks’ gestation (in the late second or third trimester) and in rare cases up to six weeks postpartum. Rohan D’Souza, a maternal-fetal medicine physician at Mount Sinai Hospital and assistant professor, obstetrics and gynaecology at the University of Toronto, distinguishes between HELLP and preeclampsia: “HELLP syndrome is a complication of pregnancy in which women show signs of liver damage and abnormalities of blood clotting; [whereas] Preeclampsia, women have high blood pressure, often accompanied by protein in their urine.” According to D’Souza, both these conditions are believed to originate in the placenta.

We don’t hear about HELLP Syndrome a lot because it’s pretty rare: It occurs in 0.5 to 0.9 percent of all pregnancies and in approximately 15 percent of women who develop preeclampsia.

Women most at risk of HELLP may be overweight or obese, over 40, or have preexisting hypertensive disorders. It is also more common in first pregnancies and in women who have previously developed preeclampsia. The only cure for HELLP is to deliver the baby—consequently many babies are born prematurely, which, depending on their gestational age and birthweight, puts them at risk. According to D’Souza, if HELLP syndrome is not treated early, up to 25 percent of women may develop serious complications and one to three percent of mothers could die. However, once baby and placenta are delivered, D’Souza says most patients will stabilize within 24 to 48 hours.

Tracey MacCharles, a founding member of Preeclampsia Foundation Canada, knows the risks of HELLP first-hand: in 2012, during her third pregnancy, her daughter Jonah Wynn was stillborn at 37 weeks due to complications of the illness. “During the ordeal I had congestive heart, liver, and kidney failure, DIC (a blood clotting issue), pulmonary edema, and temporarily lost my eyesight,” she says. MacCharles suffered from preeclampsia in her second pregnancy, but was unaware of her increased risk for HELLP. “Before the heartbreaking experience of losing my daughter, I didn’t truly understand the severity of the condition.”

Diagnosis and HELLP syndrome treatment The problem with diagnosing HELLP is that the symptoms are often vague and seemingly unconnected, and many women, like me, don’t realize they’re unwell until the illness has progressed dangerously. “Thirty to 60 percent of women report headaches and up to 20 percent report visual symptoms,” says D’Souza, “however, some report non-specific symptoms such as malaise, fatigue, abdominal pain, nausea, vomiting, easy bruising or flu-like symptoms.” He stresses the importance of regular prenatal visits and informing care providers about previous high-risk pregnancies or family history of HELLP or preeclampsia. “Understand the warning signs and seek help promptly, even when something just doesn't feel right.”

The definitive treatment for HELLP syndrome, D’Souza says, is delivering your baby—but the plan really depends on how far along you are when you get a diagnosis. After 34 weeks, most women will be induced immediately, depending on the severity of their symptoms. Before 34 weeks, expectant mothers will be given injections of corticosteroids to help their baby’s lungs mature faster, to prepare for an emergency delivery. Beyond birth, there are several interventions that may be needed to prevent seizure, stroke or organ damage in the mother, D’Souza says, including medications to control blood pressure. 

My son Rory and I were very lucky. Because my water broke early, even before my diagnosis, I had been given corticosteroid injections for his lungs (and because my doctors believed it would also help ease my symptoms). Once diagnosed, I was induced and immediately put on magnesium sulfate to help control my blood pressure. The 24 hours after delivery were a bit rocky, and I narrowly escaped needing a blood platelet transfusion. I was in hospital for four days post-partum and recovered fully. Rory, born six weeks prematurely at 4lb. 6oz., spent just two weeks in the NICU, with no lasting health problems—he’s now an active, energetic, often mischievous, three-year-old. After delivery, my placenta was sent for analysis—it was badly damaged and only in the 10th percentile for size; I have no idea how it supported my son’s life for as long as it did.

Hope after HELLP In the days after my ordeal, I told my husband I would never do that again. But the truth was, we wanted another child. When Rory was a year old, we decided to explore our options and weigh the risks. With no hypertensive problems outside of pregnancy and being in general good health, a specialist in placental health and HELLP syndrome told me I had a five percent risk of developing it again. Pretty typical odds, according to D’Souza, who says the chance of HELLP occurring in subsequent pregnancies can range from two to 27 percent. Add to that increased risk of preeclampsia, preterm delivery and delivering a smaller baby on average, and risk of developing hypertension and cardiovascular disease later on in life.

Nonetheless, I got pregnant in the summer of 2015, and entered a special pregnancy program focused on placental health at Mount Sinai hospital. Starting at 12 weeks, I was put on low-dose aspirin, which is believed to help with blood flow to the placenta and prevent high blood pressure, and had ultrasounds with each regular prenatal visit to monitor my placenta and baby’s growth. As I neared my third trimester, we had a plan: If there was any sign of vascular damage to the placenta or baby’s growth wasn’t on track, then we’d discuss the need for blood thinners to stave off the illness; if there were any of these signs or increased blood pressure after 34 weeks, we would consider induction.

As with my first pregnancy, I felt great until I hit my third trimester—except this time I was way more vigilant. I didn’t have the same symptoms, but I knew at 31 weeks something was off. Although I showed no clinical signs of preeclampsia or HELLP, my doctor recommended I start my mat leave early and rest. I began to experience symptoms of high blood pressure (including headaches) at 33 weeks, but it remained within the normal range. The headaches increased, along with swelling and fatigue. At 36 weeks, my blood pressure was on the rise. My doctor sent me for monitoring over a four-hour period and sure enough it was intermittently spiking; there was also protein in my urine, something that wasn’t present in my first pregnancy. This time, I was diagnosed with preeclampsia and induced the next day. Thanks to the close monitoring, my second son, Archie, was born healthy, at 6 lb. 2 oz., and didn’t have to spend any time in the NICU. Five days later though, I was back in hospital for two days with post-partum preeclampsia and put on medication to get my blood pressure under control. Within two weeks, I was fully recovered and off medication. Once again, I felt very lucky, but nonetheless perturbed by these complex, frightening and still relatively obscure pregnancy illnesses. While I’ve been told I would be able to support another pregnancy (with close monitoring and blood pressure medication), my husband and I agree that we’re incredibly grateful for our two healthy boys. We won’t be testing those odds again.

Symptoms of HELLP Syndrome Contact your doctor or go to your labour and delivery triage if you experience any of these symptoms, usually after 20 weeks. • Headache that won’t go away • Nausea/vomiting/indigestion with pain after eating • Abdominal or chest tenderness and upper right upper side pain (from liver distention) • Shoulder pain or pain when breathing deeply • General bleeding that doesn’t stop easily • Changes in vision • Swelling

Signs to also look for that are related to Preeclampsia include: • High blood pressure (above 140/90) • Protein in the urine

Learn more and get involved Preeclampsia Foundation Canada is actively seeking volunteers to support its mission to reduce maternal and infant illness and death due to Preeclampsia, HELLP syndrome and other hypertensive disorders of pregnancy. For more information or to get involved, visit preeclampsiacanada.ca.

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