Case Study HELLP Syndrome HELLP syndrome is a life-threatening obstetric (severe), between 50,000 and 100,000 is class II complication usually considered to be a variant of pre- (moderately severe) and >100,000 is class III (mild). This eclampsia. Both conditions occur during the later stages system is termed the Mississippi classification. of pregnancy, or sometimes after childbirth. Pathophysiology HELLP is an abbreviation of the main findings: The exact cause of HELLP is unknown, but general Hemolytic anemia activation of the coagulation cascade is considered the Elevated Liver enzymes and main underlying problem. Fibrin forms crosslinked networks in the small blood vessels. This leads to a Low Platelet count microangiopathic hemolytic anemia: the mesh causes destruction of red blood cells as if they were being forced through a strainer. Additionally, platelets are consumed. As the liver appears to be the main site of this process, downstream liver cells suffer ischemia, Signs and symptoms leading to periportal necrosis. Other organs can be similarly affected. HELLP syndrome leads to a variant Often, a patient who develops HELLP syndrome has form of disseminated intravascular coagulation (DIC), already been followed up for pregnancy-induced leading to paradoxical bleeding, which can make hypertension (gestational hypertension), or is suspected emergency surgery a serious challenge. to develop pre-eclampsia (high blood pressure and proteinuria). Up to 8% of all cases present after delivery. Treatment There is gradual but marked onset of headaches (30%), The only effective treatment is prompt delivery of the blurred vision, malaise (90%), nausea/vomiting (30%), baby. Several medications have been investigated for the "band pain" around the upper abdomen (65%) and treatment of HELLP syndrome, but evidence is conflicting paresthesia (tingling in the extremities). Edema may as to whether magnesium sulfate decreases the risk of occur but its absence does not exclude HELLP syndrome. seizures and progress to eclampsia. The DIC is treated Arterial hypertension is a diagnostic requirement, but with fresh frozen plasma to replenish the coagulation may be mild. Rupture of the liver capsule and a resultant proteins, and the anemia may require blood transfusion. hematoma may occur. If the patient gets a seizure or In mild cases, corticosteroids and antihypertensives coma, the condition has progressed into full-blown (labetalol, hydralazine, nifedipine) may be sufficient. eclampsia. Intravenous fluids are generally required. Hepatic hemorrhage can be treated with embolization as well if Disseminated intravascular coagulation is also seen in life-threatening bleeding ensues. about 20% of all women with HELLP syndrome, and in 84% when HELLP is complicated by acute renal failure. Epidemiology Patients who present symptoms of HELLP can be Its incidence is reported as 0.2-0.6% of all pregnancies, misdiagnosed in the early stages, increasing the risk of and 10-20% of women with comorbid preeclampsia. liver failure and morbidity. Rarely, post caesarean HELLP usually begins during the third trimester, and patient may present in shock condition mimicking either usually in Caucasian women over the age of 25. (Padden, pulmonary embolism or reactionary haemorrhage. 1999) Rarely cases have been reported as early as 23 weeks gestation. The outcome for mothers with HELLP Diagnosis syndrome is generally good. With treatment, maternal mortality is about 1 percent. However complications In a patient with possible HELLP syndrome, a batch of have been observed, including placental abruption, acute blood tests is performed: a full blood count, liver renal failure, subcapsular liver hematoma, and retinal enzymes, renal function and electrolytes and coagulation detachment. studies. Often, fibrin degradation products (FDPs) are determined, which can be elevated. Lactate dehydrogenase is a marker of hemolysis and is elevated (>600 U/liter). Proteinuria is present but can be mild. A positive D-dimer test in the presence of preeclampsia has recently been reported to be predictive of patients who will develop HELLP syndrome. D-dimer is a more Source: http://en.wikipedia.org/wiki/HELLP_syndrome sensitive indicator of subclinical coagulopathy and may be positive before coagulation studies are abnormal. Classification The platelet count has been found to be moderately predictive of severity: under 50,000/mm3 is class I PATHOPHYSIOLOGY Risk Factors: Primigravidas and multigravidas, maternal age greater than 25, white race (Caucasian), history of poor pregnancy outcome, & history of PIH/hypertension. Cause: Unknown. Usually a complication of PIH (Pregnancy Induced Hypertension). Pregnancy Induced Hypertension * (More particularly Severe Preeclampsia) Vasoconstriction of blood vessels RBCs pass through narrow blood vessels Hemolysis of RBCs Obstruction of liver ducts Fibrin deposits (Forms a mesh in blood vessels) Ischemia (Low blood supply) to liver Fibrins run to damaged blood vessels Elevated Liver Enzymes Microangiopathic hemolytic anemia (SGPT, SGOT, ALT, AST) Periportal necrosis (Death of tissue) Low circulating platelet Intrahepatic hemorrhage - Increased tendency to bleed Subcapsular Hematoma - Pallor (Skin, mucous membranes) Formation - Weakness/Activity intolerance/Fatigue - Shortness of breath (Inc. HR & RR) Hepatic failure - Fainting spells - Right sided upper abdominal pain - Epigastric pain - Nausa & vomiting - Jaundice - Shoulder & back pain * Signs & Symptoms of Severe Preeclampsia: - Elevated BP of 160/110 mmHg - 5+ Proteinuria (5 gm/dl) - Edema on face and extremities - Weight gain of 5lbs/week - Oliguria (400ml/24 hours) - Epigastric pain - Headache, paresthesia, changes in vision - General body malaise - Decreased fetal movements CASE STUDY ABC is a 30 year-old primigravida at 32 weeks gestation with a history of hypertension. She complained of epigastric pain, headache, blurring of vision, and tingling sensation at the extremities that have been bothering her for 3 days already. She appeared pale and weak, but was oriented and conscious. Her abdomen was noted to be slightly distended. Upon assessment, her blood pressure was 160 mmHg, respiratory rate of 25 cpm, heart rate of 105 bpm, and temperature of 36.7°C. Her reflexes were brisk, edema was noted on her face and ankles, and uterine fundal height was less than dates. Laboratory results revealed: Patient Results Normal Values Hematocrit- 35% 38-47% Hemoglobin- 10 g/dl 12-16 g/dl Platelet count- 55,000/mm3` 150,000-450,000/mm3 Plasma fibrinogen level- 300 mg/dL AST- 40 U/L 7-34 U/L ALT- 35 IU/ml 10-30 IU/ml Protein (u/a) 5 gm/ml None ABC was diagnosed with moderately severe HELLP syndrome, and was subsequently admitted for closer observation. The following were ordered by the doctor: - Infuse PNSS 1L @ 15 gtts/min - To administer following drugs: Magnesium Sulfate 5g IVTT q 4hrs, then 2g/hr continuous infusion. Hydralazine (Apresoline) 10mg PO QID initially, then 25 mg QID for 1 week. Dexamethasone (Decadron) 10mg IVTT q 12hrs. - For blood transfusion: Fresh frozen plasma @ 20 gtts/min. PRBC @ 20 gtts/min. Name of Patient: ABC Patient’s Health Profile: Received patient conscious, oriented, pale, and dyspneic, with PNSS 1L @ 15 Age: 30 Sex: F gtts/min, infusing well @ right hand. Occupation: Teacher Initial Complaints: Epigastric pain, headache, blurring of vision, & tingling sensation @ extremities Date of Admission: September 3, 2009 Status: Married Religion: Roman Catholic Medical Diagnosis: Moderately Severe HELLP Syndrome NEEDS/NURSING SCIENTIFIC ANALYSIS OBJECTIVES NURSING INTERVENTIONS RATIONALE EVALUATION DIAGNOSIS/CUES Physiologic Need: Ineffective tissue perfusion Ineffective tissue perfusion After 8 hours of nursing - Monitor vital signs, especially - Elevated BP and temperature After 8 hours of nursing related to decreased is the decrease in oxygen interventions, the client will BP and temperature. is indicative of poor blood interventions, goal was hemoglobin concentration in resulting in the failure to be able to demonstrate circulation. partially met as evidenced by blood. nourish the tissues at the increased perfusion as balanced I & O, alertness & capillary level. evidenced by BP <140/90 and > - Assist in monitoring FHT. - To check fetal health. oriented state of patient, Subjective Cues: HELLP Syndrome is a or equal to 90/60 mmHg, BP=150/110 mmHg, RR= “Sakit jud kaayo akong tiyan, multisystem disease affecting RR=12-20cpm, HR= 80-100bpm, - Monitor I & O. - To check hydration state of 22cpm, HR= 100 bpm, and unya akong panan-aw haphap. pregnant women. It is temp= 36.5-37.5°C, balanced I patient. temp= 36.9°C. Labad pajud akong ulo. Lisud attributed to abnormal vascular & O, and being alert and pud iginhawa.” tone, vasospasm and oriented. - Elevate HOB & maintain - To promote coagulation defects. Due to the head/neck in midline/neutral circulation/venous drainage. Objective Cues: coagulation defects, hemolysis position. - Received patient conscious, occurs and thus decreasing the oriented, pale, and dyspneic, oxygen supply to the tissues of - Encourage quiet, restful - Conserves energy & lowers with PNSS 1L @ 15 gtts/min, the body. atmosphere. tissue oxygen demands. infusing well at right hand. - Lab exams of Hgb= 10g/dl, Sources: - Encourage bed rest. - To conserve energy and Hct of 35%, and platelet count= Doenges, Marilynn. NURSE’S decreases cardiac workload. 55,000/mm3 POCKET GUIDE. Philadelphia - BP= 160/110 mmHg 2007. Edition 11. p705 - Encourage rest after meals. - To maximize blood flow to RR= 25cpm HR= 105bpm Padden, Maureen. HELLP stomach, enhancing digestion. Temp= 36.7°C SYNDROME: RECOGNITION & - Slight abdominal distention PERINATAL MANAGEMENT. - Assist patient in range-of- - To enhance venous return noted California 2008. motion exercises. especially at extremities. - - Patient noted to be pale on elevation Own analysis: - Instruct patient to refrain - Might be at risk for emboli. - Restlessness & weakness The patient manifested all from massaging the legs. noted cardinal signs of HELLP - Capillary refill >3 seconds. Syndrome and/or severe - Administer anithypertensive - To treat underlying cause. - Edema noted on face and preeclampsia, which is also drugs and corticosteroids as ankles suggestive of ineffective tissue indicated. - Uterine fundal height less perfusion. Vasoconstriction due than dates. to hypertension also leads to - Assist in blood transfusion of - To improve Hgb, Hct, and narrowed blood vessels, PRBC and fresh frozen plasma. platelet levels. decreasing the blood supply to the different organs, and thus resulting to decrease oxygen supply to the tissues. NAME OF DRUG CLASSIFICATION & MECHANISM INDICATION & DOSAGE CONTRAINDICATIONS SIDE EFFECTS/ADVERSE EFFECTS NURSING RESPONSIBILITIES OF ACTION Magnesium Sulfate Mineral & electrolytes Treatment of hypertension. Hypermagnesemia, CNS- Drowsiness. -Monitor PR, BP, RR. & ECG (Generic Name) -Essential for the activity of many Anticonvulsant. hypocalcemia, anuria, active Resp- Decreased RR. frequently during enzymes. Plays an important role 5g IVTT q 4hrs, then 2 g/hr labor within 2hr delivery CV- Arrythmias, bradycardia administration. RR should be at in neurotransmission and muscular continuous infusion. GI- Diarrhea least 16/min before each dose. excitability. MS-Muscle weakness -Monitor I & O. - Monitor neurologic status before and throughout therapy. Check patellar reflex before each parenteral dose. If response is absent, no additional dose should be administered until (+) response is obtained. Hydralazine (GN) Antihypertensive Moderate to severe Hypersensitivity to tartazine. CNS-Drowsiness, dizziness, - Monitor BP and PR frequently Apresoline (BN) - Direct-acting peripheral hypertension headache. during initial dose adjustment arteriolar vasodilator. 10 mg PO QID initially, then CV- tachycardia, edema, and during therapy. 25 mg QID for 1week. arrythmias, orthostatic hypotension - Weigh patient daily and assess GI-diarrhea, n & v. feet and ankles for fluid F&E- Sodium retention. retention. Neuro- Peripheral neuropathy - Caution patient to avoid sudden changes in position to minimize orthostatic hypotenstion. Dexamethasone (GN) Corticosteroids For hematologic disorders. Known alcoho, bisulfite, or CNS- Depression, euphoria, - Monitor I & O, and weigh Decadron (BN) - Suppress inflammation and the 10mg IVTT q 12hrs. tartazine hypersensitivity. headache, restlessness. daily. Observe patient for normal immune response. EENT- increased intraocular peripheral edema. pressure. - Instruct patient to avoid CV-hypertension people with known contagious GI-anorexia, n & v disease. Derm-decreased wound healing - Discuss possible effects on MS- muscle wasting and pain. body image. Explore coping mechanisms.