Unlocking the secrets of locked in syndrome By

Unlocking the secrets of locked -in syndrome By Rachel L. Palmieri, RN-C, ANP, MS Nursing 2009, July 2009 2. 4 ANCC contact hours Online: www. nursingcenter. com © 2009 by Lippincott Williams & Wilkins. All world rights reserved.

Locked-in syndrome (LIS) ¡ ¡ Complete paralysis of voluntary muscles in all parts of the body except those that control blinking and vertical eye movements Patients with the classic form are conscious and can think and reason but can’t speak or move anything except their eyes

Causes ¡ Rare neurologic disorder ¡ Caused by primary vascular or traumatic brainstem injury ¡ Usually a ventral pons lesion from injury or obstruction of basilar artery ¡ Traumatic brain injury can be a result of direct brainstem contusion or vertebrobasilar axis dissection

Other causes of LIS ¡ ¡ ¡ ¡ Brain tumors that invade ventral pons Prolonged hypoglycemia Damaged nerve cells, especially myelin sheath (such as in MS) Hemorrhage in pons Ischemia Overdose End stages of ALS

LIS signs and symptoms/characteristics ¡ ¡ Preserved consciousness with upper motor neuron quadriplegia Paresis Hyperreflexia Clonus ¡ ¡ ¡ Initial contralateral flaccid paralysis Paralysis of cranial nerves VII, IX, X, XII produces facial, tongue, pharynx paralysis Severe difficulties in swallowing and speech

LIS characteristics ¡ ¡ Sensation remains intact Temporary LIS can be pharmacologically induced Prognosis is unpredictable Early aggressive treatment promotes best outcomes

Incidence of LIS ¡ ¡ ¡ Difficult to detect; often misdiagnosed as coma, persistent vegetative state, or minimally conscious state 367 patients from 1997 -2004 have been registered by the Association du Locked-In Syndrome based in France Neurologists believe many more cases are undetected

Categorizing LIS ¡ Classified into three categories: - classic: quadriplegia, with eye movement - incomplete: remnants of voluntary movement (arm, hand, face) - total: total immobility, conscious, inability to communicate

Diagnosis ¡ ¡ ¡ Key is assessing for voluntary vertical eye movement MRI is preferred method for detecting pons lesions Neurologist should reexaimne patient once a lesion is confirmed

Diagnosis ¡ ¡ ¡ EP (evoked potential) testing can provide information about brain function EEG is controversial Definitive diagnosis can take months to years; family/healthcare providers must observe patient closely

Assessment tools ¡ ¡ Glasgow Coma Scale (GCS) used to be gold standard Full Outline of Unresponsiveness (FOUR) score developed in 2005 at Mayo Clinic supplements GCS

FOUR Score Coma Scale ¡ ¡ FOUR Score Coma Scale includes four subscales to score separately for eye response, motor response, brainstem reflexes, respiration Lower scores indicate more severe signs and symptoms

FOUR Score Coma Scale Eye response (E) ¡ Try to elicit best level of alertness by using at least 3 trials, then grading best response ¡ If patient’s eyes are closed, open them and see if patient tracks a finger or object

FOUR Score Coma Scale ¡ ¡ ¡ In cases of eyelid edema or facial trauma, tracking with only one open eyelid will suffice If horizontal tracking is absent, examine patient for vertical tracking Alternatively, document 2 blinks on command, which indicates LIS (patient is fully aware)

FOUR Score Coma Scale ¡ ¡ ¡ E 4: Eyelids open or opened, tracking, or blinking to command E 3: Eyelids open but not tracking E 2: Eyelids closed, open to loud voice, not tracking E 1: Eyelids closed, open to pain, not tracking E 0: Eyelids remain closed with pain

FOUR Score Coma Scale Motor response (M) ¡ Grade best possible response of arms ¡ If patient demonstrates at least 1 of 3 hand positions with either hand, score is M 4

FOUR Score Coma Scale ¡ ¡ If patient touches or nearly touches examiner’s hand after painful stimulus compressing the temporomandibular joint or supraorbital nerve, score is M 3 If patient has any flexion movement of the upper limbs, including withdrawal or decorticate posturing, score is M 2

FOUR Score Coma Scale ¡ ¡ ¡ M 4: Thumbs up, fist, or peace sign to command M 3: Localizing to pain M 2: Flexion response to pain M 1: Extensor posturing M 0: No response to pain or generalized myoclonus status epilepticus

FOUR Score Coma Scale Brainstem reflexes (B) ¡ Examine pupillary and corneal reflexes ¡ Preferably, test corneal reflexes by instilling few drops of saline on cornea from distance of several inches to minimize corneal trauma from repeated exams, or use cotton swabs

FOUR Score Coma Scale ¡ ¡ Test cough reflex to tracheal suctioning only when both reflexes are absent Score of B 1 indicates both pupil and corneal reflexes are absent but cough reflex (using tracheal suctioning) is present

FOUR Score Coma Scale B 4: Pupil and corneal reflexes present ¡ B 3: One pupil wide and fixed ¡ B 2: Pupil or corneal reflexes absent ¡ B 1: Pupil and corneal reflexes absent ¡ B 0: Absent pupil, corneal, and cough reflex ¡

FOUR Score Coma Scale Respiration (R) ¡ For ventilated patients, use respiratory patterns shown on ventilator monitor to identify patient-generated breaths ¡ Don’t adjust ventilator while patient is graded; try to ensure patient has a Pa. CO 2 within normal limits

FOUR Score Coma Scale ¡ ¡ To assess breathing drive, may need to disconnect ventilator for 1 -2 minutes while providing oxygenation Standard apnea test may be needed when patient is breathing at ventilator rate

FOUR Score Coma Scale R 4: Not intubated, regular breathing pattern ¡ R 3: Not intubated, Cheyne-Stokes breathing pattern ¡ R 2: Not intubated, irregular breathing pattern ¡ R 1: Breathes above ventilator rate ¡ R 0: Breathes at ventilator rate or apnea ¡

Finding a way to communicate ¡ Establish a blinking pattern for communication ¡ Establish good rapport with patient ¡ Give patient control over care ¡ Validate patient’s fear, anxiety, pain

Finding a way to communicate ¡ Involve patient’s family ¡ Educate hospital staff ¡ Work with speech therapist ¡ Point board system or Morse code may be used to expand patient communication

Nursing care ¡ ¡ ¡ Family education regarding patient’s care is one of the biggest needs Stimulating the mind of the patient with music, being read to, etc. Coordinating interdisciplinary team regarding patient’s care

Following a plan for patient care Respiratory function ¡ Place patient in lateral recumbent position, keeping neck in neutral position ¡ Elevate head of bed 30 degrees unless contraindicated ¡ Oxygenate with 100% oxygen before and after suctioning

Following a plan for patient care ¡ Suction oropharyngeal airway or via endotracheal/tracheostomy tube every 1 -2 hours to clear drainage. Limit suctioning to 10 seconds or less, 1 insertion per attempt ¡ Provide tracheostomy care every 4 hours ¡ Frequently monitor rate, depth, pattern of respirations

Following a plan for patient care ¡ Observe frequently for signs and symptoms of respiratory distress ¡ Auscultate chest every 2 hours for adventitious sounds ¡ Monitor ABG values periodically, continue pulse oximetry ¡ Administer supplemental oxygen as ordered

Following a plan for patient care ¡ ¡ ¡ Provide mouth care every 2 -4 hours, brush patient’s teeth every 8 hours If patient is mechanically ventilated, provide “sedation vacation” with spontaneous breathing trial as ordered Institute VTE prophylaxis as ordered

Following a plan for patient care Cardiovascular function ¡ Monitor vital signs frequently ¡ ¡ Monitor rate, rhythm, quality of apical and peripheral pulses Document any dysrhythmias

Following a plan for patient care ¡ ¡ Don’t use foot gatch under patient’s knees or place constricting objects behind knees Position patient so each joint is higher than previous joint; distal joints will be highest

Following a plan for patient care Integumentary system ¡ Use lubricants, protective dressings, proper lifting techniques to avoid skin injury from friction/shear when transferring/turning patient ¡ Use pillows or other devices to keep bony prominences from direct contact with each other

Following a plan for patient care ¡ ¡ Optimize nutrition and hydration Conduct pressure ulcer admission assessment, reassess risk daily; inspect skin daily ¡ Provide pressure-relieving devices but not donut-type devices ¡ Use protective barriers on fragile or irritated skin

Following a plan for patient care ¡ ¡ ¡ Don’t massage bony prominences Perform risk assessment with a reliable, standardized tool (Braden Scale) Clean skin at time of soiling; avoid hot water and irritating cleaning agents; use moisturizers on dry skin

Following a plan for patient care ¡ Keep patient’s heels off bed at all times ¡ Turn and reposition patient at least every 2 hours ¡ Protect skin of incontinent patients from exposure to moisture

Following a plan for patient care Musculoskeletal function ¡ Perform passive range-of-motion exercises at least 5 times/day ¡ ¡ Position patient in proper body alignment, using trochanter roll, splints, slings, pillows, etc. Collaborate with physical therapist

Following a plan for patient care Urologic function ¡ Monitor intake and output ¡ ¡ Follow strict aseptic technique in care of patient’s urinary catheter Remove urinary catheter as soon as possible

Following a plan for patient care ¡ ¡ ¡ Consider intermittent catheterization program Provide perineal care Monitor urinalysis and urine culture and sensitivity results for signs of infection

Following a plan for patient care Gastrointestinal function ¡ Monitor and record character and frequency of bowel movements ¡ ¡ Auscultate bowel sounds Use peptic ulcer prophylaxis as ordered

Following a plan for patient care Neurologic function ¡ Provide sensory stimuli by talking to patient; explain surroundings, treatments ¡ ¡ Encourage family to touch, talk to patient Use orientation instruments (clock, window, favorite objects, etc. )

Following a plan for patient care Pain ¡ Assess for nonverbal pain indicators ¡ ¡ ¡ Assess for distended bladder, fecal impaction Assess foreign object on/under skin Administer analgesics, provide alternatives

Following a plan for patient care Nutrition and hydration ¡ Request nutritional consultation ¡ ¡ Maintain accurate intake/output record; include daily calorie count Monitor skin turgor, mucous membranes for dryness

Following a plan for patient care ¡ Monitor urine specific gravity, serum osmolality values ¡ Provide hydration as ordered ¡ Weigh patient daily

Research ¡ ¡ Infrared eye movement sensors and computer voice prosthetics being developed Tissue plasminogen activator (t-PA) administered to patients with evolving LIS has shown to reverse quadriparesis when given within 3 hours