HELLP Syndrome
Records show that 5% or 200,000 of pregnant women in the U.S. are affected by preeclampsia every year (Campbell 2005). Pre-eclampsia is severe form of the HELLP syndrome that has a high 25% mortality rate. HELLP means hemolysis elevated liver enzymes and low platelets. Pre-eclampsia is a multi-system disease of pregnancy, which is associated with symptoms like high blood pressure, protein in the urine, swelling, sudden weight gain, headaches, vision problem, increased perinatal and maternal morbidity and mortality (Campbell and Matchaba and Moodley 2005). The HELLP syndrome affects less than 1% of pregnant women but 4 to 12% of pregnant women with pre-eclampsia. It is rare but life-threatening, not yet fully understood and can lead to lung and heart failure, permanent liver and kidney damage, internal bleeding, stroke and other serious complications to the mother (Chen 2001). The condition can also cause early placental detachment from the uterine wall or placenta abruption and lead to fetal death. Approximately 2% of these women with the HELLP Syndrome and 8% of their babies will die of HELLP. Hemolysis involves the destruction of red blood cells (Chen).
The last trimester of pregnancy is a most exciting time for most women, but not for Karima Edmonds on her first pregnancy (Mayo Clinic 2005). Three months before due date, she developed a high fever and nausea, which she thought were due to the flue. But when symptoms got worse, she saw a doctor at the Grand Forks Air Force Base in North Dakota, who diagnosed her with a rare liver syndrome. She was airlifted to Mayo Clinic in Rochester when she went into critical condition. Her daughter was delivered by emergency caesarian section and the doctors waited for her liver condition to improve. But instead of improving, she went into a hypatic coma and entered the stage 4 of liver failure, the most severe stage, according to her attending physician, Dr. Gregory Gores, a hepatologist. Dr. Gores ecided that Karima should have a liver transplant within a week. Mayo Clinic performs up to 100 liver transplants a year, but Karima’s case was the first for a patient with the HELLP Syndrome. Only 40-50% of viable organs are donated and many patients do not receive them on time. But Karima was lucky to have received a compatible liver available at the time and which saved her life. Karima’s daughter was delivered earlier than scheduled and she said she sometimes gets tired easily. She is not sure if it is because of a new liver or a new baby. But what matters to her is that she now lives a full, active and normal life with her new child (Mayo Clinic).
Pregnant women with HELLP usually complain of fatigue, general malaise, pain in the upper right part of the abdomen, nausea, vomiting, headaches and water retention with weight gain (Chen 2001). Some have convulsions. The HELLP syndrome’s symptoms mimic those of ailments, which make HELLP hard to diagnose. These ailments include the flu, systemic lupus erythemetous or SLE, acute fatty liver of pregnancy AFL, thrombotic thrombocytopenic purpura or TTP and other collagen vascular diseases.
In addition to the risks of high blood pressure during pregnancy, which reduce the flow of blood to organs and even possible seizure, the HELLP Syndrome can cause anemia and blood clotting (New Haven Health Network 2002). A serious blood clotting complication, called disseminated intravascular coagulation or DIC, may lead to severe bleeding or hemorrhage, detachment of the placenta and pulmonary edema or fluid buildup in the lungs. A severe disease may place both the mother and fetus in danger, so that it may become necessary to deliver the baby early to prevent further complications, as in the case of Karima Edmonds. Recovery from the HELLP Sydrome can take several days after the delivery (New Haven Health Network).
In diagnosing the Syndrome, a doctor will normally order a complete blood count for signs of hemolytic anemia and a platelet count, liver function tests to detect specific enzymes that can indicate liver damage, and blood-clotting studies (Chen 2001). The first line of treatment will be delivering the baby as soon as possible by inducing labor through some cervical ripening agents or by an early caesarian section. If the pregnancy is less than 32 weeks and the symptoms are not severe, a conservative approach is usually taken. This consists of bed rest, fluids, and close monitoring to allow time for the fetus to develop and mature. During this period, the doctor may give medications to control or prevent complications, such as high blood pressure or seizure or a blood transfusion if the anemia or bleeding is severe. He may likewise give corticosteroids to help the fetus’ lungs develop faster to prepare it for early delivery (Chen).
In addition to the patient’s complete medical history and physical examination, diagnostic procedures are normally performed for blood pressure, red blood cell count to obtain the bilirubin level that will indicate the breakdown of the red blood cells, liver function tests, platelet count to control bleeding and urine tests for protein (New Haven Health Network 2002).
The physician will draw up a treatment plan based on the patient’s pregnancy, overall health and medical history, the extent of the disease, the woman’s tolerance for specific medications, procedures or therapies, expectations for the course of the disease, the patient’s opinion and preference (New Haven Health Network 2002). Treatment plan may include bed rest at home or in the hospital, hospitalization by specialized personnel and for special equipment, blood transfusions for severe anemia and low platelets, magnesium sulfate to control or prevent seizures, anti-hypertensive medications to reduce blood pressure, magnesium sulfate to prevent or control seizures, fetal monitoring to check the condition of the fetus, biophysical profiling, and Doppler flow studies. Fetal monitoring may include fetal movement counting, which will keep track of fetal kicks and movements. Frequent changes or movements may mean the fetus is under stress. Non-stress testing will measure the fetal heart rate in response to the fetus’ movements. Biophysical profiling combines non-stress test with ultrasound in observing the fetus. And Doppler flow studies are a type of ultrasound that uses sound waves to measure the flow of blood through a blood vessel laboratory testing of liver, urine, and blood medications, called corticosteroids. Changes in the liver, urine and blood may signal the worsening of the HELLP Syndrome. Corticosteroids hasten the maturing of the lungs of the fetus. Lung immaturity is a major problem of premature infants, who may develop the respiratory distress syndrome and require them to be placed in a neonatal intensive care unit for very careful monitoring. HELLP is less likely to affect the fetus if the pregnancy is close to 37 weeks and the lab results are normal or close to normal (Chen 2001). If the placenta gets detached, a lack of oxygen to the baby can lead to asphyxia.
Studies suggest that steroid treatment in HELLP Syndrome may improve maternal hematological and biochemical and biological features and also perinatal mortality and morbidity (Matchaba and Moodley 2005). A 31-year-old woman with the morbidity was at her 35 weeks of gestation and admitted at the labor and delivery ward because of elevated blood pressure at 155/90 mm Hg (Clenney and Vierra 2004). Lab test findings showed increased levels of aspartate aminotransferace and alanine aminotransferase and proteinuria. The platelet count was 68000. When she was given corticosteroids antepartum or post partum for HELLP, both she and the baby did well. One case report and five retrospective reviews, which assessed corticosteroids in women with HELLP showed that laboratory variables, like platelet counts and aminotransferase levels and clinical measures improved. These included urine output, mean arterial pressure and the length of hospital stay. A small prospective randomized controlled trial of corticosteroids in women with the HELLP Syndrome antepartum involved 25 such women participants were given 10 mg dexamethasone every 12 hours. They exhibited clinically and statistically significant increases in platelet counts and decreased levels of lactate dehydrogenase and alanine aminotransferase. These outcomes included improved urinary output, although investigators failed to note if controls at 34 weeks or less gestation received steroids for the maturation of fetal lungs. But because baseline platelet counts were significantly lower in the steroid group than in the control group, the effectiveness of randomization and allocation remains uncertain (Matchaba and Moodley).
Two other prospective and randomized controlled studies evaluated costicosteroids among women with HELLP post partum (Matchaba and Moodley 2005). In one of the studies, 40 women were randomized with two doses of dexamethasone 10 mg 12 hours apart, then by 5 mg at 24 and 36 hours at a total of 30 mg or no corticosteroids. Findings show that women in the dexamethasone group had clinically and statistically significant increases in urine output and platelet counts. Lactate dehydrogenase and aspartate anubitrabsferase levels also decreased significantly in the group. There is no mention if women in less than 34 weeks’ gestation had dexamethasone for fetal indications (Matchaba and Moodley).
A more recent prospective randomized trial involved 30 women with HELLP syndrome post partum also got two doses of dexamethasone 10 mg 12 hours apart and then 5 mg at 24 and 36 hours or no corticosteroids (Matchaba and Moodley 2005). The dexamethasone group showed meaningful improvements in several variables. After 48 hours, the women who received dexamethasone has a significantly reduced mean arterial pressure at 115 mm Hg v 94 m Hg, P < 0.05 and mean asparatate aminotransferase level at 100 IU/1 v 50 IU/1; P < 0.05. Their urine output also improved at 60 ml/h v 40 ml/h; P < 0.05 and a mean platelet count at 115-000 v 70 000; P < 0.05. The researchers concluded that their findings supported a high dose corticosteroid treatment of women with the HELLP syndrome. Although three control patients showed infectious complications, there were no statistically significant differences in morbidity.
As part of nursing and medical management, dexamethasone is often given to women with this condition and are between 24 and 34 weeks’ gestation at risk of pre-term delivery to accelerate the maturation of fetal lungs (Matchaba and Moodley 2005). While the HELLP Syndrome is an uncommon disorder, it happens frequently enough to warrant preparation and the start of proper management. Dexamethasone is cheap and available almost universally. And although the studies are comparatively small, the findings illustrate a clear benefit if corticosteroids are started early. Fetal complications, such as growth retardation and low birth weight, are unlikely in humans. There are no concerns for harm on the fetus, either. The treatment of antepartum cases with corticosteroid is relatively brief and the time to delivery is also fast. And repeated corticosteroid treatments are not an issue (Matchaba and Moodley).
The preponderance of current evidence is the basis for recommendation that women with the HELLP syndrome, both antepartum and post partum, should receive two doses of dexamethasone 10 mg 12 hours apart and then 5 mg at 24 and 36 hours (Matchaba and Moodley 2005).
Bibliography
Campbell, S. (2005). Preeclampsia Sufferers at Great Risk of HELLP Syndrome During Pregnancy. The North Scott Press. http://www.zwire.com/site/news.cfm?BRD=1839&deptAdviwarePtyLtd200/July_id_1104088newsid=13913304&PAG=461&rfi=9
Chen, P., reviewer (2004). HELLP Syndrome. University of Maryland Medical Center. http://www.umm.edu/pregnancy/specialcare/articles/hellp.html
2004). HELLP Syndrome. Medline Plus. U.S. National Library of Medicine, the U.S. National Institutes of Health and the Department of Health and Human Sciences. http://www.nlm.nih.gov/medlineplus/ency/article/00089.htm
Clenney, TL. And Vierra AJ. (2004). Costicosteroids for HELLP Syndrome, a clinical review, 329:270-272 (31 July), doi: 10.1136/bmj.329.7460.270. BMJ Publishing Group Ltd. http://bmjjournals.com/cgi/content/full/329/7468/270
Matchaba, P, Moodley J. (2005).Corticosteroids for HELLP Syndrome. The Cochrane Database of Systematic Reviews, issue 4, Art number: CDOO2076_pub2.DOI: 10.1002/14651858.CDO02076.pub2. http://www.update_software.com/Abstracts/ABO2076.htm
Mayo Clinic. (2005). A New Baby and a New Liver for Karima Edwards. Mayo Foundation for Medical Education and Research. http://www.mayoclinic.org/gi-rst/karmiaedmonds.html
New Hanover Health Network (2002). High-Risk Pregnancy. http://www.nhhn.org/1434.cfm
University of Virginia Health System. (2005). HELLP Syndrome. Rectors and Visitors of the University of Virginia. http://www.healthsystem.virginia.edu/uvahealth/pubs_hrpregnant/hellp.cfm
