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Maternal/Fetal Paper

HELLP Syndrome

Lynette Barnhart

University of Texas Medical Branch at Galveston School of Nursing

NNP I

GNRS 5631

Leigh Ann Cates, MSN, RN, NNP-BC, RRT-NPS

Debra Armentrout, RN, MSN, NNP-BC, PhD

March 20, 2014

2RUNNING HEAD: Maternal/Fetal paper

HELLP Syndrome

Introduction

There are multiple high risk maternal conditions that can affect both the mother and the

infant’s health during the pregnancy, delivery, and after. As an advanced practice nurse

managing the infant after delivery requires an understanding of the high risk perinatal conditions

that occur as well as the effects on the neonate. The following paper will discuss the maternal

clinical manifestations and diagnostic evaluations, maternal and fetal pathophysiology of HELLP

syndrome, the impact of HELLP on the fetus, clinical manifestations and diagnostic evaluations

of the neonate, therapeutic approaches and treatment options, pertinent theories, evidenced based

practice, and the economic, emotional, and social implications on the family unit.

Maternal Pathophysiology

According to Khan (2013), HELLP is due to microvascular endothelial activation and

cell injury. Although there are multiple theories, the pathophysiology of HELLP is poorly

defined (Khan, 2013). Preeclampsia is thought to precede HELLP and that HELLP is in fact a

variant of preeclampsia and the pathophysiology is therefore the same (Khan, 2013). During the

16-22weeks of pregnancy there is defective remodeling of the vasculature which results in

inadequate placental perfusion (Khan, 2013). The placenta then becomes hypoxic and releases

vascular endothelial growth factor receptor-1, binding of vascular endothelial growth factor and

placental growth factor results in the inability to bind to endothelial cell receptors (Khan, 2013).

This causes the symptoms of hypertension, proteinuria, and platelet activation/aggregation

(Khan, 2013). Once this occurs, the coagulation cascade is activated and platelets are consumed,

as well as hemolysis from the shearing of erythrocytes traveling through the capillaries (Khan,

2013).


Another potential cause of HELLP syndrome arises from maternal immune rejection

when the maternal cells contact the fetus and there is an immune imbalance resulting in

endothelial dysfunction, arterial hypertension, and platelet activation/aggregation (Khan, 2013).

Fetal pathophysiology

An additional theory includes inborn errors of metabolism of the fetus, causing maternal

liver damage and placental –instigated acute inflammatory conditions (Khan, 2013). According

to Zinn (2011) there is significant evidence suggesting that long-chain 3-hydroxyacyl-CoA

dehydrogenase deficiency (LCHAD) is associated with HELLP syndrome. Mothers often show

significant improvement once the infant is delivered, which leads to the belief that the fetus is

causing a toxic effect on the mother similar to those with inborn errors of fatty acid oxidation

(Zinn, 2011). Regardless multiple theories remain and the true pathophysiology of HELLP

syndrome continues to be unknown (Khan, 2013).

LCHAD deficiency is the result of a mutated HADHA gene that is autosomal recessive

inherited (Heese, 2013). The infant is unable to break down long chain fatty acids into ketones

with low levels of 3-hydroxyacyl-CoA dehydrogenase (Heese, 2013). The resulting build-up of

fatty acids may cause anorexia, vomiting, abdominal pain, and jaundice during the third

trimester, leading to HELLP syndrome or acute fatty liver of pregnancy (Heese, 2013).

Impact on the fetus

Perinatal mortality and morbidity are higher in the fetus than the mother (Haram et al,

2009). According to the Preeclampsia Foundation (2014) the stillbirth rate is 51 per 1000

HELLP pregnancies and a 7.7-60% perinatal mortality (combined stillbirth and neonatal deaths)

in developed countries.


The leading causes of perinatal mortality associated with HELLP are prematurity, Intra

Uterine Growth Restriction (IUGR), asphyxia, placental insufficiency, and abruption (Haram et

al, 2009). Neonatal thrombocytopenia is also seen in 15-38% of maternal HELLP cases,

increasing the infant for risk of Intravascular Hemorrhage (IVH), and long term neurological

complications (Haram et al, 2009).

Although these are recognized as potential fetal complications of HELLP it is also

suggested that the mortality is based not on the HELLP itself, but more so on the complications

associated with the gestational age at birth. Therefore an infant born at 32 weeks gestational age

with a maternal HELLP risk factor is not at increased risk for mortality compared to an infant

born at 32 weeks to a healthy mother (Haram, et al, 2009).

Maternal clinical manifestations and diagnostic evaluations

HELLP syndrome is a maternal condition diagnosed and characterized by three main

factors; hemolysis (abnormal peripheral smear), elevated liver enzymes (serum AST > 70 U/L),

and a low platelet count (Platelet count <100,000u/L) (Haram, Svendsen & Abildgaard, 2009).

Women develop HELLP most often between 27 and 37 gestational weeks and postpartum within

the first 48 hours (Haram et al, 2009). According to the Preeclampsia Foundation (2013), an

estimated 48,000 women develop HELLP each year.

HELLP is classified into three categories using the Mississippi classification system. This

classification system allows practitioners to determine the severity of the disease. Class 1 is

defined as severe thrombocytopenia with a platelet count below 50,000/mm3, Class 2 is moderate

thrombocytopenia with a platelet count between 50,000-100,000/mm3, and Class 3 is mild

thrombocytopenia with a platelet count between 100,000-150,000/mm3 (Preeclampsia

Foundation, 2014).


HELLP may include one or more of the following physical symptoms; headache, nausea,

vomiting, indigestion with pain after eating, epigastric tenderness, sub sternal tenderness, right

upper quadrant pain, shoulder pain, bleeding, visual disturbances, swelling, elevated blood

pressure and protein in the urine (Preeclampsia Foundation, 2014). Although these signs and

symptoms are more often associated with pregnancy induced hypertension (PIH) they may also

be early signs of HELLP. If left untreated, HELLP progresses to liver rupture and/or stroke

which results in a critically ill mother or maternal death.

Therapeutic approaches and treatment options

Managing HELLP syndrome involves careful assessment of both the mother and the

fetus. Common treatment approaches include, glucocorticoids, intravenous magnesium sulfate,

systolic blood pressure control, and delivery within 24-72 hours of diagnosis (Martin, 2013).

According to Martin (2013) the following management is recommended:

Refer to a tertiary care facility if the gestational age is less than 35 weeks

Admit to L&D for maternal/fetal monitoring

Administer IV Magnesium sulfate

If Systolic blood pressure is greater than or equal to 160 mmHg, or the diastolic blood

pressure is greater than or equal to 105mmHg an antihypertensive will be administered.

The decision to deliver the infant is based on a series of questions, if the provider can

answer yes to any of the following questions delivery is imminent.

Is the infant less than 23 weeks or greater than 34 weeks?

Is the infant in fetal distress?

Is there maternal distress?

o Eclampsia


o Abruption

o DIC

o Renal failure

o Respiratory distress

o Suspected liver hematoma

If all of the above questions can be answered with a no and the infant is 23-34 completed

weeks the mother will receive a steroid course with 24-48 hours latency, followed by delivery.

Glucosteroids were initially administered for the benefit of the fetus to reduce hyaline

membrane disease and respiratory distress syndrome (RDS), however it was discovered that not

only did it benefit the infant it also proved to stabilize the mother’s disease process in both

undelivered and postpartum mothers (Martin, 2013).

Intravenous magnesium sulfate is administered to the mother for a multitude of benefits.

Magnesium sulfate causes central and peripheral microvascular dilation while reducing systemic

vascular resistance, protection of the blood brain barrier by reducing cerebral edema and causing

a neuroprotective /anticonvulsant action for the mother (Martin, 2013). The careful control of

systolic blood pressure is a result of multiple studies demonstrating that cerebral hemorrhage was

the most important single cause of maternal death with HELLP syndrome (Martin, 2013).

Martin (2013) refers to the Copenhagen Heart Study that states the risk for hemorrhagic stroke is

directly related to systolic blood pressure alone.

Once the diagnosis of HELLP is made delivery should occur within 24-72 hours (Martin,

2013). While awaiting delivery careful control of systolic blood pressure, IV magnesium sulfate,

and glucocorticoids are used to support the patient. In addition, platelet transfusion prior to a


caesarean section for class 1 HELLP patients is recommended as well as for a vaginal birth with

a platelet count below 25,000 (Haram, 2009).

Impact of maternal treatment on the neonate

Maternal treatment strategies have a subsequent impact on the neonate immediately after

birth or during their hospital stay. Magnesium crosses the placenta and achieves steady state

levels within hours of administration (Fanaroff, 2013). Cord magnesium levels of 4-11mg/dL

can cause hypotonia, hypotension, hyporeflexia, seizures, respiratory depression,

hypoventilation, apnea, bradycardia, poor suck, feeding intolerance, decreased GI motility,

abdominal distention, intestinal perforation, urinary retention, delayed stooling, and cardiac

arrest (Gomella, 2013). In addition long term administration greater than 7 days has been known

to cause fetal bone loss of the proximal humerous (Fanaroff, 2013).

The antenatal administration of glucocorticoids improves neonatal outcomes by reducing

neonatal morbidity, RDS, ventilator support, and use of surfactant (Fanaroff, 2013).

Glucocorticoids cause neonatal lung structural maturation that in turn increases the surface area

available for gas exchange (Jobe, 2013).

The decision to deliver the infant as a treatment option for HELLP syndrome has a direct

effect on the infant. The effect of HELLP on the infant is directly related to the gestational age of

at birth. If the infant is born <32 weeks gestation they are at the highest risk with a mortality of

32%, and infants > 32 week have a mortality rate of 8% (Haram, et al, 2009).

Neonatal clinical manifestations and diagnostic evaluations

At birth and during the first 36 hours of life, the infant of a mother with HELLP may

present with a variety of clinical manifestations. During the pregnancy and birth infants can

present with placental insufficiency (IUGR), asphyxia, and abruption. At birth and immediately


following they may present with prematurity, thrombocytopenia, and IVH (Haram et al, 2009).

All of these complications come with their own risks, diagnostic tests, and treatment options.

Infants whom are born prematurely face multiple complications. Complications include

but are not limited to; respiratory distress syndrome, electrolyte imbalances, metabolic

imbalances, infection, necrotizing enterocolitis, PDA, apnea, bradycardia, anemia, and IVH

(Gomella, 2013). In addition long term complications include but are not limited to chronic lung

disease, retinopathy of prematurity, visual problems, hearing impairment, developmental delays,

and learning difficulties (Gomella, 2013). Clinical manifestations and diagnostic evaluations are

focused on the individual complication associated with prematurity.

IUGR infants are at increased risk for complications such as perinatal asphyxia, cold

stress, polycythemia, cholestasis associated with parenteral nutrition (PN), and hypoglycemia

(Gomella, 2013). Persistent pulmonary hypertension (PPHN), respiratory distress and patent

ductus arteriosus (PDA) may also be seen in IUGR infants who experience asphyxia during

birth. Diagnostic tests include central hematocrit, blood glucose, and liver function tests with

long term PN. If PPHN or a PDA is suspected, an ECHO will be obtained for confirmation. An

arterial blood gas and chest x-ray can be obtained for diagnostic purposes. An MRI or head

ultrasound should also be obtained to assess the total brain volume which is known to be reduced

when compared to appropriate for gestational age (AGA) infants. In addition in preterm IUGR

infants the white matter and gray matter can also be reduced (Gomella, 2013).

Asphyxiated infants can present with varying degrees of distress at birth. These infants

are at risk for hypoxic-ischemic encephalopathy (HIE) (Gomella, 2013). Diagnostic tests and

treatment for these infants depend on the degree of insult at delivery. Management is focused on

supportive care including resuscitation using NRP guidelines, assisted ventilation as needed,


perfusion, acid-base management, fluid management, blood glucose management, and seizure

prevention (Gomella, 2013).

Abruption can cause neonatal thrombocytopenia and platelet dysfunction as well as

hypovolemia (Gomella. 2013). If an abruption is suspected, diagnostic tests that should be

performed include; continuous pulse oximetry, cardiac/respiratory monitoring, blood pressure

monitoring, a CBC with platelet count and type and cross in preparation for a potential platelet or

PRBC transfusion.

Neonatal thrombocytopenia may be seen following a maternal HELLP diagnosis. It can

range from mild to severe, as well as be symptomatic or asymptomatic. Diagnostic tests include

a CBC with differential and platelet count (Gomella, 2013). Treatment for thrombocytopenia is

based on treating the underlying cause, in the case of HELLP treatment will be based on the

platelet count and if the infant is symptomatic. The practitioner will observe for sings of

petechiae, and active bleeding. In addition a head ultrasound will be obtained to assess for IVH.

Although there is no relationship between the severity of thrombocytopenia and IVH, it has been

shown that along with other risk factors such as prematurity, asphyxia, and IUGR, infants are at

an increased risk for IVH (Lindern, Bruele, & Walther, 2011).

Inborn Errors of Metabolism (IEM) with an acute onset such as Fatty acid oxidation

(FAO) disorders in the infant are thought to be a factor with HELLP syndrome (Gomella, 2013).

FAO disorders include long-chain acyl-CoA dehydrogenase (LCHAD) and short-chain acyl-CoA

dehydrogenase (SCAD) (Gomella, 2013). Fatty acid oxidation disorders present with impaired

cardiac function, occasional cardiac arrhythmias, encephalopathy, impaired liver function,

rhabdomyolysis, muscle weakness, and/or retinopathy (Gomella, 2013). As an advanced

practitioner it is important to assess for severe cardiomyopathy resulting in cardiac failure,


encephalopathy, myopathy, hepatomegaly with low glucose intake or intercurrent illness and

development of hypoketotic hypoglycemia (Gomella, 2013).

When a FAO disorder is suspected the practitioner should evaluate for liver dysfunction,

although it may be mild in comparison with other IEM, hypoalbuminemia and coagulopathy may

be seen (Gomella, 2013). Hyperammonemia may present if liver dysfunction progresses and

urine organic acids may have tyrosine metabolites present (Gomella, 2013). An Acyl carnitine

profile is typically included in the newborn screen and helps to determine the levels of fatty acid

metabolites with different carbon lengths for diagnosis. A total and free carnitine plasma level

can be obtained for diagnostic purposes (Gomella, 2013).

Pertinent theories and evidenced based management guidelines

The decision to treat with maternal corticosteroids is based on the evidenced based

research of multiple studies. With antenatal treatment, RDS is reduced by 50%, as well as a

reduction in severity of disease in those that do develop RDS (Jobe, 2011). Although maternal

corticosteroid treatment is used for lung maturation it also has an effect on other organs;

decreasing incidence of PDA, IVH, and necrotizing enterocolitis, while increasing kidney tubular

function and blood pressure (Jobe, 2011).

Administration of magnesium sulfate is thought to not only provide neuroprotection for

the mother, but also for the premature fetus (Fanaroff, 2011). Three observational studies

suggested a decrease in cerebral palsy in very low birth weight infants who received magnesium

sulfate (Fanaroff, 2011). Additional case controlled studies concluded a decrease risk of

mortality among this population. According to Fanaroff (2011) a systematic review completed

by Conde-Agudelo and colleagues as well as Doyle and associates concluded that antenatal

magnesium sulfate therapy was associated with a reduction in the risk of moderate or severe


cerebral palsy and gross motor dysfunction. Although multiple studies suggest a reduction in

cerebral palsy it remains unclear if evidenced based medicine will support the use of magnesium

sulfate for the sole purpose of neuroprophylaxis for preterm infants (Fanaroff, 2011).

Economic, emotional and social implications on the family unit

HELLP has an economic, emotional, and social impact on the family. The burden is not

only associated with the financial and emotional requirements of the mother’s diagnosis, but also

related to the potential complications of the infant.

The health of women during pregnancy and childbirth impacts the health, development

and well-being of the entire family economically, emotionally, and socially (Koblinsky,

Chowdhury, Moran & Ronsmans, 2012). The obstetrical complication of HELLP increases the

risk for morbidity, and physical and mental disabilities, which in turn increases the economic and

social burden. Such burdens can further increase through impoverishment, violence, isolation,

divorce, and remarriage (Koblinsky et al, 2012). Following a severe obstetric complication there

are secondary consequences such as mental health problems, loss of physical strength, family

stability issues, loss in community status and impoverishment (Koblinsky et al, 2012).

As the infant’s gestational age decreases the financial and emotional burden significantly

increases (Hodek, Schulenburg, & Mittendorf, 2011). It is important to recognize the

perspective of the family in relation to the direct medical costs, non-medical costs, indirect costs,

and intangible costs HELLP has on the family (Hodek et al, 2011). Intangible costs include

emotional distress and reduced quality of life (Hodek et al, 2011).

Actual neonatal hospital costs vary according to the complications and degree of illness.

March of Dimes estimates the average Neonatal Intensive Care Unit (NICU) stay is $76,164,

while infants born less than 32 weeks gestational age average increases to $280,811 which is


approximately nine times more than the cost of an infant born at 37-41 weeks (“Special care”,

2011). Additional costs include; travel expenses if the NICU is not in their town, childcare for

siblings, overnight accommodations, and long term medical care.

In addition to the financial burden and stress of an infant in the NICU, Hodek et al (2011)

recognizes that the emotional stress significantly increases with decreasing gestational age. The

emotional burden of an infant in the NICU doesn’t cease once the infant is discharged home, in

fact the largest part of the burden occurs once the infant is discharged home (Hodeck et al

(2011).

Conclusion

Although the exact pathophysiology of HELLP syndrome is still unknown, and multiple

theories exist it is important to recognize the potential factors that contribute to HELLP. Early

recognition and diagnosis of HELLP is important in the management of both the mother and

infant to reduce complications, mortality and morbidity. Understanding the impact of HELLP on

the fetus, the clinical manifestations and diagnostic evaluations of the neonate, as well as

therapeutic approaches and treatment options help the neonatal team to care for the infant after

delivery. In addition as an advanced health care provider it is also important to recognize the

emotional, economic, and social implications that are associated with a maternal illness and/or an

infant in the NICU. Understanding the entire illness allows the providers to provide the best and

most appropriate care to the mother, infant, and family.


References

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M.C. Walsh. Neonatal-Perinatal medicine: Diseases of the fetus and infant (9th edition

pp.303-334). Volume 1. St. Louis, Missouri: Elsevier Mosby

Gomella, T.L. (2013) Neonatology: Management, procedures, on-call problems, diseases, and

drugs (7th edition). New York: McGraw Hill Education.

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