Thursday, November 20, 2008

HELLP: Tiffany's Story

This post contributed by a wonderful preemie mom whose running and cooking both impress me immensely. Thanks, Tiffany!

I distinctly remember excitedly phoning my father one winter morning to inform him that my husband and I were expecting our first child. My father, who is still very much overprotective of me even at 36, responded with something along the lines of “Oh no! I’m worried. What if you die in childbirth?” I responded with a loud guffaw and said, “Dad! You cannot be serious. Women don’t die in childbirth anymore!” After all, I believed it was only pregnant women in third world countries that risked dying, far away from modern hospitals and basic sanitation. Surely a pregnant mother’s life couldn’t be in danger, not in this day and age, and certainly not where I lived, in the heart of Silicon Valley.

My pregnancy progressed normally. I suffered from morning sickness the first trimester, and enjoyed the second trimester as the nausea faded away. I started to actually look pregnant and enjoy the small bulge forming. After the standard blood tests came back showing all was normal with the baby, we breathed a small sigh of relief and looked ahead with few worries or concerns. I was taking my prenatal vitamins, eating healthily and doing all the things you are supposed to. Labor itself was one of the only things I feared as my husband and I were 10 lbs and 9 lbs respectively!

Around my 25th week I began to have some swelling and at one of my regular exams my previously low blood pressure had begun to creep up, slowly but noticeably. Still, these were fairly common pregnancy symptoms in themselves so no alarm bells were sounded and I was simply told to come back in for a check two weeks later. By the next appointment my kidneys had just started to spill a small amount of protein, an indication of pre-eclampsia. During the next five days, I went back and forth between partial bedrest at home and back to the hospital for urine collections and monitoring. I had no symptoms other than the swelling and, in fact, felt great which made it harder to fathom what was happening to my body. Just after hitting my 28th week, I went in for a check of the baby and was immediately admitted to the hospital with full-blown pre-eclampsia.

I was put on magnesium sulfate to ward off potential seizures as is the standard protocol for pre-eclampsia patients. I tried to settle in for what I hoped would be a few more precious weeks of time. However just 48 hours later, I started to vomit and feel what I thought was indigestion but turned out to be epigastric pain just below the ribs, a sign of something even more serious. Thirty minutes later our son was delivered by C-section in a room full of more people than I can remember.

Liam was born at 10:05 pm on a warm night in June at just over 28 weeks gestation weighing 2 lbs 1 oz (925 grams). He was immediately ventilated but stabilized and whisked off to the NICU before either of us had a chance to see him. With the baby delivered, we thought at least I was now out of the woods.

At some point in the middle of that night, a doctor came in to see my husband and inform him that they were moving me immediately to ICU. My blood tests showed that my platelets were dropping and I had developed a rare but very serious variant of pre-eclampsia called HELLP syndrome. My husband asked what that meant and this doctor, with quite a gruff bedside manner, responded that “it could be fatal” and rushed out the door to page the high-risk perinatologist at home for advice. My husband now had to deal with a baby on one floor, a wife on another, both of us fairly critical and unsure if we’d get through it.

What is HELLP Syndrome?

HELLP Syndrome stands for Hemolytic anemia, Elevated Liver enzymes and Low Platelet count. It normally happens hand-in-hand with pre-eclampsia but not always.

Besides uncontrollable bleeding, a main danger with HELLP is liver rupture and we were told that HELLP patients generally get worse before they get better, thus the need to be monitored in ICU. They also had a bag of blood platelets ready to transfuse in case my platelets dropped below a certain range, I believe around 25,000. HELLP patients are classified according to 3 levels of platelet counts:
  • Class I (the most severe) is below 50,000
  • Class II is between 50,000 to 100,000
  • Class III below 150,000
At its worst, my platelets had fallen to 27,000 and had reached Class I HELLP but narrowly avoided needing a transfusion. It’s an understatement to say it was a difficult time for family. After two days in ICU and arms so black and blue from blood draws they were unrecognizable, my condition began to turn upwards and on the third day I was moved out of ICU to a normal recovery room. And, at last, I was able to visit my son for the first time.
Ultimately my son spent 76 days in the NICU. He was on ventilators for a month (conventional and oscillator), required PDA surgery for a heart ligament, fought severe pneumonia from a staph infection, and faced similar challenges of other preemies of his gestation. Though it has been a long road with challenges along the way, three years later he is a happy and healthy boy with a few scars (or battle wounds as we call them) and a wide grin.

Coming to terms with HELLP and the resulting premature delivery of my son has been a journey, one I wouldn’t wish on anyone. Yet as trite as it sounds, so much has come from this experience. The appreciation for all the small milestones our preemies make. The strength and knowledge you gain as a parent from being your child’s advocate during the NICU and beyond whether their challenges are minor or profound. And of course the fellow parents of preemies you meet along the journey. Fellow parents bound together by many shared experiences including the indescribable feeling of having to leave your child at the hospital instead of coming home together. Night after night after night.

Symptoms of HELLP Syndrome
(Please note symptoms do not always present themselves with HELLP. These, however, are common):

  • Marked onset of headaches
  • Blurred vision
  • Nausea/vomiting
  • Upper–gastric pain (which as I mentioned felt like pain under the ribs, or indigestion)
  • Tingling in the extremities
  • Edema (very typical of pre-eclampsia)

A blood test is performed to officially diagnose HELLP Syndrome. Other than delivery of the baby, there is no “cure” for HELLP syndrome. Recurrence rates are quoted as being anywhere from 5-35% with a higher risk of recurrence the earlier and more severe HELLP was.

Follow-up Testing:

HELLP survivors should be tested for a variety of autoimmune and clotting disorders. At present these include:

  • Lieden Factor V mutation R560Q
  • Hyperhomocysteinemia MTHFR Mutation
  • Prothrombin Gene Mutation 20210 (GA)
  • Protein C levels and Protein S levels; Activated Protein C activity
  • Antibodies to 6 phospholipids of the IgM, IgG and IgA classes
  • Lupus anticoagulant antibody
  • Russell Viper Venom time
  • Activated partial thromboplastin time (APTT)
  • Prothrombin time (PT)and Partial prothrom
(In my case I tested negative for all of the above disorders, though I most likely did have a genetic predisposition to pre-eclampsia)


For more information on HELLP Syndrome, please consult the following:

Yahoo Groups HELLP Syndrome
HELLP Syndrome Society
Preeclampsia Foundation:

Tiffany is the mother to Liam, born at 28 weeks gestation in June 2005. Since Liam's early birth she has done support for NICU parents and been an advocate for prematurity awareness. She enjoys cooking, gardening and running and works part-time as an internet marketing consultant.


Ryann said...

Although my severe pre-eclampsia didn't turn into HELLP, I think we went through similar experiences. I'm glad both you and your son have recovered and are living a happy, healthy life. Thank you so much for sharing your story.

Anonymous said...

JessieLeigh, thanks for providing a forum for people to share their stories! The guest posts this week have been very insightful and heartfelt.

Ryann, thanks for sharing your story too! I read your story yesterday (I remember you from BabyCenter I think?) and was thrilled to see how well Addyson is doing. She is beautiful! Pre-e and HELLP are like the double whammies of prematurity. Not only do you have to deal with a premature infant but you also face the risks to your health for that and any subsequent pregnancy. Not fun!

Take care guys,

Tiffany (Liam's mom)

Amy @ Finer Things said...

Another moving experience. I had never heard of HELLP. Thank you for sharing!