Health Awareness blog

HELLP syndrome is one of the most severe complications caused by pre eclampsia. It might be as dangerous as eclampsia but more common. It is characterized by some degree of liver involvement together with blood clotting disorder. Basically, “H” is stands for hemolysis or rupture of the red blood cell. “EL” stands for elevation of the liver enzymes that indicate the degree of the liver damage. “LP” stands for low platelets which vital for normal blood clotting. Those having this syndrome are classified having severe pre eclampsia.

HELLP syndrome manifests itself like the other signs of pre eclampsia. Typically, the blood pressure is high and the patient has significant proteinuria associated with edema of hands, feet or face. Just like eclampsia, it sometimes arises out without any warning signs. Most of the time, the patients will present with epigastric pain, vomiting and headache. The epigastric pain (pain under the ribs) might be misleading where we might easily think of heartburn. But the pain do not manifests itself like usual heartburn. Furthermore, it is not relief by antacid. The pain usually gets very severe and the patient will also have tenderness over the liver. Sometimes the doctor might diagnose this unfortunate lady with cholecystitis.

HELLP syndrome is most likely to occur immediately after the delivery. It is the same thing with eclampsia. However, it is also might present during 20 weeks of gestation or maybe a bit later and sometimes develops with devastating speed. Rarely, it occurs earlier in the pregnancy.

There are various complications that may give rise from HELLP syndrome. The blood clotting function might be severely impaired and lead to heavy and uncontrollable bleeding particularly after surgery. The damage of the liver also might be severe enough and might result of rupture of this vital organ. The other severe complication is renal failure and the mother might present with shortness of breath. Some of them may need artificial ventilation. Stroke due to cerebral hemorrhage might present with or without eclampsia.

Whoever is suspected to have HELLP syndrome should be admitted immediately to the hospital for further investigation and monitoring. The pre evaluation should be done similar to severe pre eclampsia. The management includes evaluation of maternal and fetal well being and blood pressure stabilization. Magnesium sulfate infusion should be initiated. Other measures are correction of coagulopathy and maternal stabilization. Consider platelet transfusion before delivery.

 Once the diagnosis has been made, the delivery of the baby should be done immediately (provided the mother is stable) regardless the maturity of the baby. Delivery is the core treatment for this life threatening condition. Patients more than 34 weeks should go for immediate delivery. For those less than 34 weeks corticosteroids (dexamethasone 12mg bd for 1 day) should be given and delivery should be planned in 48 hours.

It is not uncommon for the symptoms to become worse – or to develop for the first time – in the 48 hours following delivery, and treatment in an intensive care unit may be necessary. Sometimes, the symptoms develop or worsening in the 48 hours following delivery. Admission to intensive care unit is mandatory for close monitoring and active management.

As for delivery method, whoever diagnosed with HELLP syndrome and has a favorable cervix, trial of labor should be offered to the patient. Sometimes, operative delivery may cause more harm than good. Elective cesarean section should be considered in some patients at very early of gestational ages without favorable cervices.  Serial blood monitoring should be done at the same time for monitoring.

Treatments are mean to support the failure of the mother’s systems including lung, liver, kidney and the blood clotting system. It will take some time before the systems recover and the mother can cope. Most of the time, no permanent damage occur and full recovery is expected if the condition is diagnose in a good time and proper action has been taken place. The mother might been between few days up to 2 or 3 months before the recovery can occur and not everybody will be hospitalized during the monitoring. A few visits to the clinic might be sufficient provided the patient is stable and reliable.

HELLP syndrome is a maternal condition and not directly affects the fetus. But, all the same, it might result in growth retardation or fetal distress due to shortage of maternal blood supply to the placenta. In this case, intrauterine death might occur. In most cases, the early delivery is done for the mother sake and sometimes the baby might suffer with severe prematurity and unable to survive outside the mother’s womb.

Subcapsular liver hematoma is another known potential life threatening complication of HELLP syndrome. Clinical findings that consistent with this complication include phrenic nerve pain. In haemodynamically stable patient, conservative management is appropriate (considering the hematoma is not ruptured). However, close hemodynamic monitoring should be done including serial evaluations of coagulations profiles. Serial evaluation of the hematoma using radiologic studies should be performed to monitor the illness.

The recurrence rate of HELLP syndrome for next pregnancy is high and may goes up 25%. Treatment of low dose aspirin may be recommended in some cases and should be given in earlier in the pregnancy. Close monitoring of patient’s condition at tertiary centre should be done in view of detecting the signs of recurrence at the earliest possible stage. So far, there is no way to predict the recurrence or to prevent that from happen. Preconception counseling should be offer to the patients with an expert to devise an appropriate antenatal care programme for the next pregnancy.

References:

1. Coppage, K.H. and Sibai, B.M. Hypertension Emergencies. In Foley, M.R.., Strong T.H.J and Garite, T.J. editors. Obstetric Intensive Care Manual 2^nd edition. Mc Graw-Hill Medical Publishing; 2004.p. 5 1-65
2. Minister of Health. Training Manual on Hypertensive Disorder in Pregnancy. National Technical Committee Confidential Enquiries into Maternal Deaths. 2000
3. Collier, J., Longmore, M. and Brown, T.D. Oxford Handbook of Clinical Specialties, Oxford University Press;  2001.
4. Clinical Practice Guidelines Management of Hypertension. 3^rd edition. Ministry of Health Malaysia, Academy of Medicine of Malaysia and Malaysian Society of Hypertension; 2008.
5. Wagner Lana K. Diagnosis and Management of Preeclampsia. American Family Physician, 2004; 70 (12):2317-2324
6. Gibson, P. and Carson, M.P. Hypertension and Pregnancy [Internet]. 2007. [cited 10 January 2009]. Available from: http: emedicine.medscape.com/article/261435-overview.
7. McPhee, S.J., Papakadis.M.A. and Tierney, L.M. Current Medical Diagnosis & Treatment. Forty Sixth edition. McGraw-Hill Companies Inc; 2007
8. James Denise. Therapeutic Abortion [Internet].2006. [cited 16 January 2009] Available from: http://emedicine.medscape.com/article/266440-overview .

Preeclampsia is known to be one of the most common of the dangerous pregnancy complications where both fetus and mother can be severely affected.  It is defined as pregnancy induced hypertension (systolic blood pressure > 140mmHg or diastolic blood pressure > 90 mmHg) associated with significant proteinuria (> 0.3g in a 24 hour urine collection).

The term preeclampsia is refers to a set of symptoms rather than any causative factors. The etiologic agent that is responsible for the development of preeclampsia still remains unknown.  Pathophysiology changes in preeclampsia are attributable to intense vasoconstriction that is thought to be due to increased vascular reactivity. It appears that there is a substance or substances that have been release from the placenta which may esult to the endothelial dysfunction in the maternal blood vessels of the affected women. The most visible sign of the disease is the elevation of the blood pressure or ‘hypertensive crisis’. However, it is very important to remember that it also involves generalized damage to the maternal endothelium of kidneys and liver, with the release of vasopressive factors only secondary to the original damage.  Hematology abnormality in preeclampsia is low platelet or thrombocytopenia. However, the exact mechanism for thrombocytopenia is still unknown. Vasoconstriction that occurs in pre eclampsia patients will lead to decreased renal perfusion. There will be subsequent reduction in the glomerular filtration rate (GFR) hence will cause the deteriorating of the renal function. As for hepatic damage, it ranges from mildly elevated liver enzyme levels to subcapsular liver hematoma and hepatic rupture. This is one of the severe consequences of HELLP syndrome. It might be as severe as eclamptic convulsion, which perhaps the most disturbing Central Nervous System (CNS) manifestations of pre eclampsia.

Preeclampsia is prevalent for those aged less than 18 years old and above 35 years old. In this case, Puan Julian is 33 years and there is tendency she will have this kind of complications. It may develop after 20 weeks gestation. However, it rarely occurred before the third trimester. If it happens before 32 weeks gestation, it is considered as early onset which highly associated with more severe morbidity. The progress of each case may differ and give rise to various complications. Naturally, most cases are diagnosed pre-term. However, it can also occur up to 6 weeks post partum. The core treatment is delivery of the baby and the placenta either by induction of labor, spontaneous vaginal delivery or even by caesarean section.

There are various symptoms of preeclampsia. Some common symptoms are nausea, vomiting and persistent severe headache that might mislead us and think of migraine or other cause of headache. The patient might complain of right upper quadrant or epigastric pain which some doctors might misdiagnose with acute astritis. Some patients might present with blurry of vision and scotomata. The alarming symptom will be decreased fetal movement that means the fetus already affected. The patients will be advised to come to hospital immediately if experience leaking liquor, rupture of membranes, vaginal bleeding or regular uterine contractions.

In patient having preeclampsia, the risk to the fetus is largely related to gestational age at delivery. The aim of management is to avoid cerebro vascular accident to the mother and to prolong the pregnancy enough to deliver  a live baby that is sufficient to continue his/her life outside the womb. Experienced clinicians should be involved in managing pregnant lady that has been diagnosed with preeclampsia in view of various complications that they might encounter. It is known that pre eclampsia is associated with various complications and might cause death to both mother and fetus. Renal failure is not uncommon especially in those people with severe proteinuria and deteriorating renal failure. There is also possibility to develop disseminated intravascular coagulation where the patient might bleed. Other complications are intracranial hemorrhage, retinal detachment, pulmonary edema, liver rupture and abruptio placenta. 

In mild preeclampsia, a home management with proper bed rest may be attempted. This will only be offered to selected patients that are reliable, compliance to medication and medical advices have stable home environment and have the ability to self monitoring the blood pressure. It is very important for the patients to have assistance at home, transportation and rapid access to the hospital should the associated symptoms present. As addition, a competent nurse from the health clinic may be provided to do frequent (even daily) home visits and proper assessment. Hospital management is a must in patients with moderate to severe preeclampsia and those with unreliable home situations.

Severe preeclampsia must be swiftly identified. This is important so the patient may urgently been referred to appropriate centre with specialist and facilities so close observation and timing for delivery can be done.As for the management of severe preeclampsia, the patient needs to be admitted to labor and delivery area immediately after been diagnosed with preeclampsia. Laboratory assessment should include hematocrit, platelet count, serum creatinine, aspartate aminoransferase, uric acid, urine protein and 24 hour urine collection for analysis. Maternal and fetal should be evaluated and monitored closely. Should the patient present with hypertensive crisis or symptoms of impending eclampsia with present of proteinuria, magnesium sulfate should be given within 24 hours.  Antihypertensive agents will be given to mother if systolic >180 mmHg and diastolic > 110, or MAP >130 mmHg.

At this delivery area, we will assess both mother and fetus. If there is evidence of maternal distress, non reassuring of fetal status and the pregnancy is more than 34 weeks gestation, magnesium sulfate and steroid will be given to mother and proceed with the delivery of the fetus. The same thing will be done if the mother is in labor or presented with rupture of membranes or even if there is evidence of severe intrauterine growth retardation.

If the pregnancy is within 23 to 32 weeks gestation, steroids (dexamethasone) will given to the mother for the sake of the fetus should early delivery is decided later. Antihypertensive agent will be given to mother if needed depending on the blood pressure control. A daily evaluation of maternal and fetal conditions is mandatory and we aim for the delivery at 34 weeks or at term. We will keep the fetus as long as possible in utero till we are almost sure that the fetus may survive or might do better if outside the womb unless mother’s condition is compromise. If the fetus is nonviable, the termination of pregnancy will be decided for the sake of the mother. The decision of termination of pregnancy should be done by agreement of multidisciplinary team. The team should at least include the patient herself, her obstetrician, the specialist who is expert about the disease in question, the expert person in genetic counseling and also neonatalogist .

            Pre eclampsia is very dangerous. This is one of the reasons why early and frequent atenatal check-up is mandatory for all pregnant mothers to screen them for the possible complications of pregnancy. Early intervention will lead for better outcome.

References:

1. Coppage, K.H. and Sibai, B.M. Hypertension Emergencies. In Foley, M.R.., Strong T.H.J and Garite, T.J. editors. Obstetric Intensive Care Manual 2^nd edition. Mc Graw-Hill Medical Publishing; 2004.p. 5 1-65
2. Clinical Practice Guidelines Management of Hypertension. 3^rd edition. Ministry of Health Malaysia, Academy of Medicine of Malaysia and Malaysian Society of Hypertension; 2008.
3. Wagner Lana K. Diagnosis and Management of Preeclampsia. American Family Physician, 2004; 70 (12):2317-2324
4. Gibson, P. and Carson, M.P. Hypertension and Pregnancy [Internet]. 2007. [cited 10 January 2009]. Available from: http: emedicine.medscape.com/article/261435-overview.
5. McPhee, S.J., Papakadis.M.A. and Tierney, L.M. Current Medical Diagnosis & Treatment. Forty Sixth edition. McGraw-Hill Companies Inc; 2007
6. James Denise. Therapeutic Abortion [Internet].2006. [cited 16 January 2009] Available from: http://emedicine.medscape.com/article/266440-overview .