HELLP Syndrome

Overview: 

HELLP syndrome: A syndrome featuring a combination of “H” for hemolysis (breakage of red blood cells) “EL” for elevated liver enzymes, and “LP” for low platelet count (an essential blood clotting element). HELLP syndrome is a recognized complication of preeclampsia and eclampsia (toxemia) of pregnancy, occurring in 25% of these pregnancies. About 70% of cases occur in the third trimester of pregnancy, and the remainder occurs within 48 hours of delivery. 

1. Pathophysiology

The pathophysiology of HELLP syndrome involves a complex interplay of systemic endothelial dysfunction, coagulation abnormalities, and inflammation. It is often associated with preeclampsia, although it may occur independently. Here's a breakdown:

1. Endothelial Damage and Inflammation

   • Placental ischemia (reduced blood flow to the placenta) leads to the release of antiangiogenic factors, causing widespread endothelial dysfunction.

   • This dysfunction results in vasoconstriction, increased vascular permeability, and activation of the coagulation cascade.

2. Hemolysis (H)

   • Microvascular endothelial damage triggers the formation of microthrombi in small blood vessels, causing

     red blood cells to be fragmented as they pass through, leading to hemolysis.

   • This contributes to anemia and elevated levels of lactate dehydrogenase (LDH).

3. Elevated Liver Enzymes (EL)

   • The microvascular obstruction in hepatic circulation causes liver ischemia and

     damage to hepatocytes, releasing liver enzymes (AST and ALT) into the bloodstream.

   • Severe cases can lead to hepatic rupture or hematoma.

4. Low Platelets (LP)

   • Platelets aggregate at sites of endothelial injury and are consumed in the formation

     of microthrombi, leading to thrombocytopenia.

   • This increases the risk of bleeding and can lead to complications like disseminated

     intravascular coagulation (DIC).

5. Systemic Effects

   • The interplay of hemolysis, liver dysfunction, and thrombocytopenia creates a systemic

     inflammatory response, exacerbating organ dysfunction.

   • Patients may also experience hypertension, proteinuria, and multisystem organ failure

     in severe cases.

2. Symptoms:

Common symptoms in women with the HELLP syndrome include a general feeling of feeling unwell (malaise), nausea and/or vomiting, and in pain in the upper abdomen. Increased fluid in the tissues (edema) is also frequent. Protein is measurable in the urine of most women with the HELLP syndrome. Blood pressure may be elevated. Occasionally, coma can result from seriously low blood sugar (hypoglycemia) 

3. Treatment: 

The first order of treatment of HELLP syndrome is management of blood clotting issues. If fetal growth is restricted, urgent delivery can be required. If the HELLP syndrome develops at or after 34 weeks of gestation or if the fetus’ lungs are mature or mother’s health is in jeopardy, urgent delivery is the treatment. 

Medications have been investigated for the treatment HELLP, but evidence is conflicting as to whether magnesium sulfate decreases the risk of seizure and progress to eclampsia. In mild cases, corticosteroids may be sufficient. 

Most often, the definitive treatment for women with HELLP Syndrome is the delivery of their baby. During pregnancy, many women suffering from HELLP syndrome require a transfusion of some form of blood product (red cells, platelets, plasma). Corticosteroids can be used in early pregnancy to help the baby’s lungs mature. 

4. Prognosis: 

The prognosis of children of mothers with HELLP is not as bad as assumed. With treatment, maternal mortality is about 1%, although complications such as a placental abruption, acute renal failure, sub capsular liver hematoma, permanent liver damage, and retinal detachment occur in about 25% of women. Perinatal mortality (stillbirths plus death in infancy) is 73-119 per 1000 babies of women with HELLP, while up to 40% are small for gestation age. In general, however, factors such as gestational age are more important than the severity of HELLP in determining the outcome in the baby. 

5. Complications: 

HELLP syndrome can lead to severe maternal and fetal complications if not promptly recognized and managed. Maternal complications include liver hematoma or rupture, which can result in internal bleeding and is potentially fatal. Acute kidney injury may occur due to reduced blood flow and thrombotic microangiopathy. Disseminated intravascular coagulation (DIC) is another critical complication, leading to widespread clotting and bleeding. Pulmonary edema may result from endothelial damage and fluid overload. Neurological issues, such as stroke or seizures, can arise due to severe hypertension. For the fetus, complications include premature birth, intrauterine growth restriction (IUGR), or stillbirth, as HELLP syndrome often necessitates early delivery to protect maternal and fetal health. Multidisciplinary care is essential to minimize these risks.

6. What should RT’s prepare for in Mothers with HELLP Syndrome? 

• Emergency Cesarean Section (C-Section): In severe cases of HELLP syndrome, the health of both the mother and the baby may be at risk, necessitating an emergency C-section. The RT must be prepared for a sudden change in the birthing plan and ready to assist with any respiratory needs that may arise during or after the surgery. 

• Blood Pressure Management: HELLP syndrome often involves hypertension. Medications may be administered to manage blood pressure, and the respiratory therapist may need to monitor the respiratory impact of these medications, as some antihypertensive drugs can influence respiratory function of the neonate. 

• Neonatal Respiratory Distress: Babies born to mothers with HELLP syndrome may face respiratory challenges due to factors such as prematurity or complications during delivery. The RT in the neonatal unit should be ready to provide specialized respiratory care to newborns experiencing respiratory distress. 

• Monitoring for Complications: HELLP syndrome is associated with various complications, including liver dysfunction and coagulation abnormalities. The RT may need to address respiratory implications associated with these complications and work collaboratively with the medical team to manage potential challenges. 

• Communication with the Obstetric Team: Collaboration between the respiratory therapist and the obstetric team is essential. Clear communication is needed to coordinate care, especially if there are unforeseen complications related to HELLP syndrome that impact both maternal and neonatal respiratory well-being.