By BRYAN WOODFORD
The following scenario occurred in a small rural town with a population under 1,000, serviced by a volunteer fire department basic life support (BLS) ambulance. Advanced life support (ALS) is 30 minutes away, and the closest hospital is more than 40 minutes away.
A 26-year-old female was pregnant with her first child and had been extremely healthy and active for most of her life. Her obstetrician was confident that she would have few complications with her pregnancy.
The patient’s first trimester went as planned; the mother and the fetus progressed as anticipated. Shortly after the beginning of the second trimester, the mother developed swelling in her feet. Edema rapidly progressed and, within two weeks, swelling was evident in her feet, ankles, hands, and face. The mother visited her obstetrician, who expressed his concerns with her current presentation and ordered the mother to reduce her work hours. The obstetrician and his staff monitored the mother’s vital signs from this point forward.
At 25½ weeks gestation, the mother awoke from sleep one morning with a headache and complained of extreme sensitivity to light and sound. Her husband, a registered nurse (RN), took her vital signs and noted her blood pressure (BP) as 154/104 mmHg, heart rate (HR) at 104 beats per minute, and respiratory rate (RR) at 18 breaths per minute. The patient and her husband were concerned, as this was the first reported headache and high blood pressure reading during the pregnancy. The couple called 911 for expeditious transport to the local hospital.
On the local BLS ambulance crew’s arrival, the patient was awake and alert, complaining only of a mild headache. Because of the call’s nature, ALS was dispatched simultaneously with BLS and was in transit to the couple’s location. The BLS crew performed a rapid primary assessment and checked vital signs on-scene and began its transport to rendezvous with ALS. Initial vital signs taken by emergency medical services (EMS) were BP 158/98, HR 92, RR 20, and pulse oximetry was 98 percent on room air.
Approximately 20 minutes into the transport, the mother’s symptoms worsened. She complained of a worsening headache, dizziness, and mild right upper quadrant pain, which she described as a “gas-like” discomfort. Repeat vital signs were BP 98/52, HR 84, RR 20, and pulse oximetry 98 percent on room air. The BLS crew placed the patient on her left side and started her on three liters per minute of nasal oxygen. BLS provided a patient update by radio to the incoming ALS crew, who advised their estimated time of arrival (ETA) at five minutes. The BLS crew continued to monitor and reassess the patient.
Once the ALS unit arrived on-scene with the BLS unit, the emergency medical technician gave the paramedic a brief medical report. An 18-gauge intravenous catheter was inserted into the patient’s left antecubital fossa and 0.9 percent normal saline solution (NSS) was initiated at 150 ml/hr. Personnel applied a cardiac monitor and completed a more advanced physical assessment, which revealed normal deep tendon reflexes without any signs of clonus. Maternal vital signs were BP 121/70 mmHg [mean blood pressure (MAP) 87], HR 78 with a sinus rhythm, RR of 20, and oxygen saturation 98 percent on three liters of nasal oxygen. Lung sounds were clear and equal bilaterally with symmetrical chest wall expansion. Peripheral pulses easily palpated bilaterally. The patient presented with marked edema of the face, hands, ankles, and feet, which the patient reported had progressively worsened since its onset several weeks ago. The abdominal exam revealed a symmetrical abdomen that was free from surgical scars with minor striae gravidarum notes and a normal fundal height of 27 centimeters.
The patient reported a urinary output decrease that began around the 20th week of gestation. The edema to the lower extremities was symmetrical, and the patient denied any complaints of pain associated with the swelling. The onboard paramedic contacted the accepting hospital personnel and provided them with a detailed patient report and an updated ETA. Medical command did not send any additional orders at this time.
Approximately 25 minutes into the 40-minute transport, the patient complained of a sharp stabbing pain in the center of her chest radiating to the right upper quadrant of her abdomen. She initially rated her pain only a 5/10 in severity. However, the patient became very anxious and difficult to calm. Repeat vital signs were BP 164/110 (MAP 128), HR 112 (sinus tachycardia), RR 24, and pulse oximetry 99 percent.
The patient was placed on 15 liters of oxygen through a nonrebreather mask. Approximately five minutes later, the patient was writhing in pain and was barely able to stay secured to the stretcher because of her extreme anxiety. She stated, “It hurts so bad I just can’t get comfortable.”
The patient rated her headache, chest, and abdominal pains all a 10/10 in severity. A reassessment of the vital signs showed BP 210/112 (MAP 144), HR 121 (sinus tachycardia), RR 28, with the pulse oximetry unchanged. Personnel again contacted and advised medical command of the patient’s change in condition. Personnel received orders for four-mg morphine IV and four-g magnesium sulfate IV over five to 10 minutes with an infusion of two grams per hour. The orders were carried out without any significant changes in the patient’s condition.
On arrival at the tertiary care center, the patient was taken to the emergency department and was met by a team of high-risk obstetricians, who quickly determined that the patient was suffering from severe preeclampsia and required an emergency cesarean section (C-section). The patient remained in the emergency department for another 10 minutes while the obstetricians attempted to treat her hypertension.
None of the interventions were successful at treating the hypertension; the patient was immediately transported to the labor and delivery unit, where personnel prepared her for the emergency C-section. Approximately 45 minutes after the patient arrived at the tertiary care facility, the preterm infant was born. The 25½-week-old infant weighed 698 grams, required immediate endotracheal intubation, and presented with APGAR (Appearance, Pulse, Grimace, Activity, Respiration) scores of 5 (one minute) and 7 (five minutes). The infant was then taken to the neonatal intensive care unit, where he underwent aggressive life-saving interventions. The infant remained hospitalized for the next three months and was discharged to home with low flow oxygen. The child is now five years old and remains healthy without any residual complications from his prematurity.
Days after her son’s delivery, the mother was diagnosed with a thrombophilia, known as Factor V Leiden. This particular thrombophilia is an inherited blood-clotting disorder named after a specific mutation associated with an increased risk of miscarriage, pregnancy-induced hypertension (preeclampsia), slow fetal growth, and early placenta separation from the uterine wall (placental abruption).1 Initially, the high-risk obstetricians believed the Factor V Leiden caused microclot formation within the placenta, resulting in a blood flow decrease to the fetus, causing the severe preeclampsia. This theory proved inaccurate once the placenta pathology report showed no signs of microclot formation within the placenta.
Days after her fetus’ delivery, the mother’s blood pressure remained slightly elevated; she was discharged to home with a prescription for an oral antihypertensive and was ordered to follow up with her obstetrician at the tertiary care center to discuss her newly diagnosed thrombophilia and to evaluate her mildly elevated blood pressure. At the time of discharge, it was still unclear what caused the mother to experience severe preeclampsia.
ANALYSIS
These events raise the following questions.
What was the likely cause of this patient’s deteriorating clinical picture and severe pain while in transport? The patient was diagnosed with preeclampsia after testing positive for proteinuria, hypertension, and elevated uric acid levels at the referring facility. Ten percent of women diagnosed with preeclampsia see their condition worsen, causing them to develop HELLP (Hemolysis, Elevated Liver enzymes, and Low Platelet count) Syndrome. HELLP carries a very high mortality rate. If HELLP is not treated aggressively during its early onset, studies show that up to 25 percent of affected women will suffer serious complications.2 A small number of women may even die if their signs and symptoms are not treated. In the above case, the patient’s symptoms developed rapidly without warning and required emergency surgery to extract the fetus from the mother. This is the only definitive treatment for a HELLP-affected mother, as liver function rapidly worsens, causing significant complications for the mother and the fetus.3,4
Although the maternal laboratory values from the tertiary care facility are not available, HELLP syndrome is a likely suspect. Once the mother arrived at the tertiary care center, a group of high-risk obstetricians performed a rapid physical assessment and found severe hypertension, headache, and right upper quadrant abdominal pain with hepatomegaly, epigastric pain, and blurred vision. Recent literature regarding laboratory efforts to diagnose HELLP based on various liver enzyme elevations [asparate aminotransferase (AST), alanine aminotransferase (ALT), and lactic acid dehydrogenase (LDH)] is varied and inconsistent, as interlaboratory differences are a major problem.
What could have been done differently in the transport and treatment of this patient? In this particular transport, the preeclampsia symptoms could have rapidly worsened and required immediate life-saving interventions. At the referring facility, the mother was diagnosed with a mild case of preeclampsia, which rapidly progressed to severe preeclampsia during transport.
Severe preeclampsia is associated with increased rates of maternal mortality (0.2 percent) and morbidity (five percent), which is why transport personnel need to continuously reassess their patient for worsening symptoms.(2) Potential life-threatening conditions include convulsions, pulmonary edema, acute renal or liver failure, liver hemorrhage, disseminated intravascular coagulopathy, and stroke. (2) Each of these complications is typically seen in women who develop severe preeclampsia prior to their 32nd week of gestation.
Critical care transport personnel must obtain “what if” orders from medical command if they do not have a liberal set of protocols for readily treating signs and symptoms related to certain diseases. The transport program in this case did not have a protocol or obtain “what if” orders that would have allowed personnel to treat the aforementioned signs and symptoms without contacting the medical command physician, and thus they did not demonstrate meticulous behavior to ensure a safe and efficient transport.
In this scenario, the transport personnel had little difficulty contacting medical command. However, delays were possible and might have caused undue harm to the mother. Many women cannot tolerate the acute onset of severe preeclampsia. Maternal and perinatal outcomes are largely dependent on issues such as gestational age, severity of the disease, presence of multifetal gestation, and presence of preexisting medical conditions such as diabetes and renal disease. (1)5
What is the best treatment for HELLP?There are two algorithms to follow when treating a mother suspected of having HELLP. The first involves a clinically stable mother who presents with minimal or no stress to herself or the fetus. This patient requires immediate hospitalization with continual observation in the labor and delivery unit, must be treated for severe preeclampsia, and should receive magnesium sulfate prophylactically to prevent seizures.6
Next, confirm or exclude the diagnosis of HELLP from the other conditions that mimic HELLP. Laboratory tests must include a complete blood count; peripheral smear; coagulation studies; serum AST levels; and creatinine, glucose, bilirubin, and LDH levels. If you confirm a HELLP diagnosis, decide how soon the fetus will need to be delivered. If the mother is at less than 35 weeks gestation, she will require immediate transportation to a tertiary care facility that specializes in high-risk obstetrics. If the clinical condition of the mother and fetus is stable, decide whether the delivery must occur immediately or if both the mother and fetus could tolerate waiting up to 48 hours for corticosteroid therapy.
The second algorithm follows the path experienced by the mother in this scenario, whose clinical condition rapidly deteriorated during transport to the tertiary care facility. Aggressive attempts at lowering her life-threatening blood pressure were unsuccessful. On arrival to the tertiary care facility, the mother was not clinically stable and showed no signs of improvement. Obstetricians performed an emergency C-section, which ultimately saved the life of this mother and her fetus.
What is the most common complication associated with preeclampsia? Eclampsia, which is the development of convulsions or unexplained coma during pregnancy with patients who display signs and symptoms of preeclampsia,7 is reported to range from one in 2,000 pregnancies to one in 3,448 pregnancies, with the higher incidence in geographic locations with little or no prenatal care, multifetal gestation, and tertiary referral centers.8,9,10 Eclampsia is also known for its very high mortality rate and often requires a magnesium sulfate infusion to assist in stopping the seizures. In this scenario, transport personnel started a magnesium sulfate infusion during the transport phase to prevent any seizures, and it was continued postoperatively until the mother’s blood pressure was stable.
Seizures in any patient require aggressive treatment. The majority of in-hospital and prehospital protocols use benzodiazepines as a first line agent to terminate active seizures. In a pregnant patient, especially with suspected or known preeclampsia, administer magnesium, but do not delay using benzodiazepine as needed to terminate a seizure.
The mother in this case was not necessarily out of the woods after delivery. Eclampsia can occur during the antepartum, intrapartum, or postpartum periods. (10)11,12,13 Most postpartum eclampsia cases occur within the first 48 hours after delivery, but some cases are reported between 48 hours and 23 days postpartum. (11,13)14 In cases that occur days to weeks after the delivery, the mother should undergo a comprehensive neurological evaluation to rule out any other possible cerebral pathologies. (13,14)
Does pregnancy-induced hypertension make this patient more susceptible to reoccurrence in future pregnancies? Yes. After the patient recovered from her emergency C-section, her doctors performed an array of outpatient laboratory tests to discover a cause for her preeclampsia. Studies show that the preeclampsia’s exact cause is unknown, but some possibilities are clotting disorders and chromosomal abnormalities. In the above case, the detailed laboratory studies revealed that the patient indeed had the thrombophilia disorder Factor V Leiden, often the cause of small clots that form inside the placenta, which, in turn, caused HELLP, which accounts for a 60 percent reoccurrence of preeclampsia in future pregnancies. The high-risk obstetricians ordered a pathology study on the placenta, which didn’t reveal any blood clots. This ruled out Factor V Leiden as the cause, but it still leaves the question of what was the cause for this preterm delivery. It is likely that if the patient in this case study was to become pregnant again, she would require daily anticoagulant therapy with a low molecular weight heparin, such as enoxaparin, to further prevent clot formation.
Studies show that women with preeclampsia have a recurrence risk of 7.5 to 18 percent.15-18 Women with severe preeclampsia during the second trimester, such as the one in this case study, have a recurrence risk of 65 percent.19 If a female is diagnosed with HELLP, her risk of recurrence is two to six percent.20-22 Several studies also suggest that women who develop preeclampsia during pregnancy are more likely to develop cardiovascular complications later in life.23-27
What infrequent complication of preeclampsia is associated with high mortality? HELLP syndrome, the final diagnosis for this particular patient, which occurs in only three percent of women diagnosed with preeclampsia or eclampsia but results in a much higher mortality rate.
REFERENCES
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BRYAN WOODFORD, RN, MSN, MBA, NREMT-P, CMTE, is a flight nurse at STAT MedEvac in Pittsburgh, Pennsylvania. He is also a career firefighter/paramedic with Cumberland Trail Fire District in St. Clairsville, Ohio; an Ohio EMS instructor; and an assistant professor for a local Ohio nursing program. He has edited two critical care transport textbooks.
