Pathophysiology of HELLP Syndrome

A maternity unit is generally filled with happy moments; however, when an obstetrical emergency walks through the door, those times can be hectic and chaotic. All persons involved need to be part of a delicate team who work together in order for the woman and her child to receive the best care and to ensure the most optimum outcome as possible. The preceding case study will show how to effectively manage HELLP syndrome, the acronym for hemolysis, elevated liver enzymes and low platelet count.

N.N. is a 26-year old, gravida 2, para 1 at 37 weeks gestation per last menstrual period and early ultrasound. She presented to the labor and delivery unit with a direct statement of “I had HELLP syndrome with my last pregnancy and I think I have it again.” Her vital signs were: temperature 99.4 F, pulse was 126, respirations 26 and blood pressure was 156/93. Her weight is 185 pounds with a prepregnancy weight noted to be 167 pounds.
This patient was placed on external fetal monitoring. The fetal heart rate was noted to be 145 beats per minute with moderate variability and shown to have a reactive strip. There were appropriate accelerations noted and no decelerations. Uterine contractions were shown to be irregular, 3-9 minutes apart lasting 40-60 seconds in duration. The uterine contractions palpated mild. Reflexes were tested and were abnormal at 3+ with clonus, and +2 pitting edema was noted in lower extremities. A cervical exam revealed a soft, thick closed cervix. An IV was started and lactated ringers was hung and infusing at 150 ml per hour. Labs were drawn for CBC, ABO & RH and type and screen, comprehensive profile, PT, PTT, and a urinalysis. An ultrasound was ordered to rule out a hematoma on her liver and to check for a placental previa or abruption as well as to check for fetal well being.

Her abdomen was soft, but tender to touch with pain in her upper right quadrant. She denied leaking of amniotic fluid and tested negative for nitrazine and ferning. She complained of a headache that she states she has had for two days. Her liver enzymes were elevated with an AST of 906 and ALT of 1042 and hemolysis of LDH at 1123. Her platelets were at 43,000. The urinalysis showed a trace amount of protein as well as ketones and bilirubin. The rest of the urinalysis was within normal limits. The ultrasound was negative for a liver hematoma. It also showed an active, apparent well fetus. She presented with 3+ pitting edema in her lower extremities. The PT, PTT and fibrinogen were within normal limits. The physician on call was immediately called and was on his way in.
Because this patient had had HELLP syndrome in the past, it was likely she had it again. There is up to a 25% chance that a woman who has HELLP in previous pregnancies, she will have it again in subsequent pregnancies (Hagl-Fenton, 2008, p. 23.) Women who have had pregnancies complicated with preeclampsia have a 2-12 percent chance of developing HELLP in their pregnancy (Lealbe, Niebyt, & Simpson, 2002, p. 956.) Many women who come into a labor and delivery unit are misdiagnosed because the symptoms mimic preeclampsia. The platelet count is one of the key indicators that it is more than preeclampsia, (O’Hara Padden, 1999, p. 829) as well as an increased bilirubin (Lealbe et al., p. 956.)
There is not a definitive pathogenesis of HELLP syndrome. The discoveries of this multisystem disease are characterized by “abnormal vascular tone, vasospasm and coagulation defects” (O’Hara Padden, 1999, p. 830.) HELLP appears to be the last indicator of some attack that leads to microvascular endothelial impairment and “intravascular platelet activation” (O’Hara Padden, p. 830.) With the activation of the platelets, serotonin and thromboxane A are released, thus resulting in platelet agglutination and aggregation and then continued damage of the epithelium (O’Hara Padden, p. 830.) This can only be corrected by the delivery of the baby, if the gestation is greater than 32 weeks (O’Hara Padden, p. 32.)
If the woman presents to a labor and delivery unit and is less than 32 weeks in gestation, medicinal therapeutics are first tried to help keep the patient pregnant as long as safe for her and her baby. Betamethasone 12mg IM is given twice in 24 hours to help mature the lungs of the premature infant. Giving Decadron, 10 mg IV every twelve hours, has been shown to improve many of the laboratory irregularities identified with HELLP syndrome (O’Hara Padden, 1999, p. 33.) Magnesium Sulfate, 4-6 gram bolus, intravenously, should be administered over a 20-30 minute time frame. A 2 gram maintenance dose of magnesium should be continued after the bolus is given. This can help lower the elevated blood pressure of the patient. A magnesium flow sheet should always be used to monitor for signs of magnesium toxicity. Reflexes should be tested each hour to assess for slow, sluggish or hyper reflexes. Visual disturbances, headaches and urinary output should also be assessed each hour.

Platelet activation is believed to participate to the pathogenesis of preeclampsia and HELLP syndrome, which results in hemodynamic changes, vasoconstriction and endothelial injury (Sheu, Shen, Lin, & Tzeng, 2002, p. 132.) Formation of Thromboxane A in platelets generally rises during pregnancy (Sheu et al., p. 133) and it has also shown that it is exacerbated when combined with a decrease in prostacyclin (Sheu et al., p. 133.)
The hemolysis in HELLP syndrome is microangiopathic hemolytic anemia (O’Hara Padden, 1999, p. 830.) The red blood cells become odd shaped, known as traumatic hemolytic anemia. These red blood cells are seen in the peripheral blood when they are exposed to a large amount of turbulence and may been seen as schistocytes or echinocytes (burr cells) (Lowdermilk, Perry, & Bobek, 1997, p. 792.)
The persistent vasoconstriction that occurs causes fibrin deposits in the hepatic sinusoids (Leaton & Martin, 2001, p. 32.) This block hepatic blood flow and changes liver function (Leaton & Martin, p. 32.) The liver swells causing the pain in upper right quadrant as well as epigastric pain. ALT and AST are chief enzymes that initiate intracellular protein metabolism and are generally found in small quantities in the extracellular serum. An elevated AST may occur when there is liver ischemia. An increase in the ALT, found primarily in liver cells, is when the liver fails to function normally in its role of hemoglobin degradation. An increase in the bilirubin may also take place because of the effects on the liver (Lowdermilk et al., 1997, p. 792.)

Vasoconstriction of the blood cells can cause limitations on the blood flow, thus hindering the blood from getting to the vital organs of both the mother and the fetus in the complication of decreased organ perfusion. If the placenta is not receiving sufficient blood, the baby can have complications that can be assessed by the fetal monitor. These complications can be seen as variables or decelerations. If the baby has run out of fetal reserves, the baby may not survive due to the lack of placental perfusion. Vasoconstriction has also been associated with intrauterine growth retardation (IUGR) (Robillard, Chaline, Chaouat, & Hulsey, 2003, p. 131.)

The obstetrician on-call that night was in the hospital within 12 minutes of the call he received. He assessed the patient and looked at the labs that were taken. He rechecked her cervix which showed no change in dilatation. Because of her gestation age of 37 weeks, the decision for an immediate cesarean was brought forth. He was concerned for the possibility of a long labor. The fact that she did not have a favorable cervix and using pitocin to augment her labor, could have had a poor outcome for both the mother and her baby. The time between decision for the cesarean section to incision was 17 minutes. N.N. had a 7lb. 13oz. baby boy. His apgars were eight at one minute and nine at five minutes. APGAR is an acronym for the scale or scoring used to determine how well the baby did after delivery. APGAR stands for activity (muscle tone), pulse, grimace (reflex ability), appearance (color of the newborn), and respirations. Each score rates either 0, 1, or 2. Two is the best a baby can achieve with each section of the scoring tool. This baby had one point taken off for color and tone at one minute and one point taken off for color at five minutes. There was no need for further intensive care for this baby.

During the post partum period, the patient recovered without any further problems. The AST and ALT were back within normal limits by the second post partum day. Another ultrasound was done on her liver the second post partum day which showed no evidence of enlargement, bleeding or rupture. There was no evidence of post partum hemorrhage. Urinary output was greater than 30ml per hour after her cesarean section. By the third post partum day, the edema in the lower extremities was down to 1+ edema with no evidence of pitting. Her post op pain was controlled by as needed opioid medications as well as NSAID’s.

The first nursing diagnosis that could have been used for this patient was risk for injury: maternal, related to vasospasm, high blood pressure. A second nursing diagnosis could have been anxiety related to fear of disease process and possible outcomes. A third nursing diagnosis could have been altered protection related to decreased platelet count as evidenced by platelet count of 43,000. A fourth nursing diagnosis could have been pain related to post operative procedure.
The interventions used for this patient were used to help ensure the positive outcome for herself and her newborn. She was assessed and properly diagnosed within a very short period of time. The interventions that were done were to promote the patients comfort from the pain she was experiencing from both her abdomen and her head. She was placed on her side in order to not obstruct the blood flow from the vena cava; this can happen if the patient is lying flat on her back. She was assured as well as comforted to help to reduce the anxiety that she was feeling from the fear of what was happening to her. From the time she arrived on to the unit, until the time of her cesarean section, the time lapsed was only approximately 45 minutes.
Had she not stated that she had, had HELLP syndrome with her first pregnancy, she may have been diagnosed with preeclampsia or another pregnancy related issue. The fact that she told the nursing staff when she arrived helped us by knowing what to order, what to look for when the results came back and how to properly treat her. The physician on call was not her regular physician and had never seen this patient before. As he was assessing this patient, he was also looking through her prenatal to view her history. With the patient’s symptoms, the fact she has had HELLP syndrome in the past and the labs and the radiology reports that came back, this patient had the best possible procedure that gave her the best outcome for her and her baby.

Had this patient been under 36 weeks, she would have been transported to a level three hospital. Our hospital is a level one, community hospital and do not generally deliver babies under 36 weeks unless there is a risk to the mother or fetus. If this is the case, the neonatal intensive care unit (NICU) team from the level three hospital is called and informed.

HELLP syndrome is a very traumatic and scary disease process for the patient. Without thorough evaluation and assessment of the patient and her fetus, both she and her baby could have been subjected to a very different outcome. Through team work by the medical staff, the nursing staff as well as the rest of the ancillary members that were involved with this patients care, the patient went home, with a healthy newborn, both with no residual damage.

Hagl-Fenton, D. J. (2008, March 2008). Beyond Preeclampsia. RN, 71(3), 22-25.
Lealbe, S. G., Niebyt, J. R., & Simpson, J. L. (2002). Obstetrics-Normal and Problem Pregnancies (4th ed.). Philadelphia, Pa.: Churchill Livingstone.
Leaton, M. B., & Martin, P. S. (2001). Dealing with coagulopathies of PIH. Nursing2001, 31(3), 32-35. Retrieved from ProQuest September 16, 2008
Lowdermilk, D. L., Perry, S. C., & Bobek, I. M. (1997). Maternity & Women’s Health Care (6th Ed.). New York, NY: Mosby.
O’Hara Padden, M. (1999, September 1, 1999). HELLP syndrome: Recognition and perinatal management. American Family Physician, 60(3), 829-836. Retrieved from ProQuest September 16, 2008
Robillard, P. Y., Chaline, J., Chaouat, G., & Hulsey, T. C. (2003). Preeclampsia/eclampsia and the evolution of the human brain. Current Anthropology, 44(1), 130-134. Retrieved from ProQuest September 30, 2008
Sheu, J. R., Shen, G., Lin, M. Y., & Tzeng, W. Y. (2002). The hyperaggregability of platelets from normal pregnancy is mediated through thromboxane A2 and cyclic AMP pathways. Clinical & Laboratory Haematology, 24(2), 121-129. Retrieved from ProQuest September 30, 2008