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HELLP Syndrome Case Study

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					                                                        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.[6]
of pregnancy, or sometimes after childbirth.
                                                                                    Pathophysiology
HELLP is an abbreviation of the main findings:[1]
                                                               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,[2] and in
84% when HELLP is complicated by acute renal failure.[3]                             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.[4] 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.[7]
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.[5] 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.[citation needed]

                      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.

				
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