Amyotrophic lateral sclerosis (PowerPoint)

					Amyotrophic Lateral Sclerosis

  Lou Gehrig’s Disease
   A Progressively neurodegenerative disease, which causes death to
    upper motor neurons located in the cerebral cortex, as well as
    peripheral lower motor neurons, originating in the spinal cord.
   Death of motor neurons causes the brain to lose the ability to
    produce and control motor muscle movements. As ALS progresses
    the patient becomes paralyzed and eventually dies.
Causes continue…
   The initial cause of ALS is unknown. The only identified
    cause, which only accounts for 10% of all cases of ALS is
    hereditary/ familial ALS (FALS). The remaining 90%
    causes of ALS are unknown.

   Familial (FALS) is autosomal dominate with mutation on
    chromosome 21, which is called superoxide dismutase
    ◦ Free Radicals may be the cause of this mutation

   The 90% unknown causes of ALS may be due to:
    ◦ High Glutamate levels: Glutamate a chemical messenger found in
      the brain. High levels of glutamate can be found in ALS patient’s
      spinal cord.
    ◦ Autoimmune Response: Self attack self.
Incidence & Prognosis:
   ALS occurs in 1 to 3 people per 100,000.

   The onset is usually between 40-70 years of age

   It is more common in men than women by 2:1

   Most people with ALS die from respiratory failure, usually
    within 2 to 6 years after diagnosis.
Assessment findings:
   Initial symptoms are often very vague and can vary from
    person to person, they may even be so slight in the
    beginning that they are overlooked.

   Symptoms can begin in the muscles that control the
    hands, arms, feet, legs, or speech. Although not all
    people with ALS experience symptoms in the same
    order or have the same pattern of progression. But
    progressive muscle weakness is a universal experience.

   Muscle weakness is a hallmark sign.
Symptoms include:
   Difficulty lifting the front      Abnormal fatigue of the legs
    part of the foot and toes          and/or arms
    (foot drop)                       Muscle cramps and
   Weakness in the ankles and         twitching (fasciculation)
    feet                              “Thick” or slurred speech
   Tripping over small things         and difficulty projecting the
    objects.                           voice
   Hand weakness and                 Uncontrollable periods of
    clumsiness                         laughing or crying
   Dropping things often             Dysarthia
   Trouble using the hands for       Dysphagia
    simple things such as
    washing, dressing, and
    buttoning clothes
Assessment finding continue…
   As the disease progresses muscles become weaker and weaker until they
    are paralyzed.

   Ventilatory support will eventually be needed as breathing muscles are
    affected and paralyzed

   As ALS progresses, muscles that control swallowing are affected,
    dehydration and malnutrition can become problems, along with aspirating
    secretions, food, or liquid into the lungs can cause a PNA.

   The most common cause of death for people with ALS is respiratory

   ALS only affects the motor neurons so there are no problems with
    sight, touch, hearing, taste, or smell.

   Bowel and bladder control are not directly affected. Constipation and skin
    breakdown may occur due to immobility.
Diagnostic Evaluation:
   ALS is often difficult to diagnose early because it is very similar
    to other neurological diseases.There is no one test or
    procedure that can be done to establish the diagnosis of ALS.

   It is often through a series of diagnostic tests to rule out other
    diseases that a diagnosis of ALS can be established.

   The initial appointment may include testing of:
    ◦   Coordination
    ◦   Balance
    ◦   Muscle strength
    ◦   Muscle tone
    ◦   Senses of touch and sight
Diagnostic Evaluation continued…
Workup to rule out other neurological diseases includes:

   A thorough neurological examination
   Electrodiagnostic tests including, electromyography
    (EMG) and nerve conduction velocity (NCV)
   X-Rays including MRI
   Myelogram of the cervical spine
   Blood and urine studies including, thyroid and parathyroid hormone
    levels, 24 hour urine for heavy metals, and high resolution serum
    protein electrophoresis
   Spinal tap (lumbar puncture)
   Muscle and/or nerve biopsy
   Riluzole (Rilutek) extends survival and/or time to invasive
    breathing assistance. Rilutek is not curative.

   Rilutek works by decreasing your body's levels of glutamate,
    an amino acid that affects nerves that send messages from
    your brain to your muscles. People with amyotrophic lateral
    sclerosis may have very high levels of glutamate, which can
    damage these nerve cells.

   Side Effects include: nausea, lung function decrease, headache,
    dizziness, nasal symptoms, muscle tightness, abdominal pain,
    high blood pressure, and liver dysfunction.
Medication continued…
Special considerations:
   Liver function will need to be checked with blood tests every
    month during the first 3 months of treatment.

   Do not smoke while taking medication as smoking decreases
    the effectiveness of Rilutek.

   When taking Rilutek, avoid drinking excessive amounts of
    alcohol; alcohol may contribute to compromising liver
    function and may be associated with an increased risk of liver
Medication continued…
Special considerations:
   Avoid coffee, tea, cola, or other products that contain
    caffeine. Caffeine may cause too much Rilutek to build up in
    your body.

   Take this medicine with a full glass of water. Rilutek has
    reduced absorption when taken with meals that are high in
    fat. It is therefore recommended that the drug is taken at
    least one hour before a meal or two hours after to avoid
    reducing the absorption of the drug.
   Treatments are based on symptomatic control. This includes:
   Physical and Occupational Therapy to preserve mobility and
    promote muscle strength.
   Usage of cane, supportive brace, walker, and wheelchair.
   Speech and language therapy to help control the loss of
    speech and ability to swallow.
   Speech-generating device to maintain communication.
   NG tube, PEG
   Respiratory therapist, noninvasive ventilation (BiPAP), trach
   Support groups: local MDA
   Medication: riluzole (Rilutek)
Nursing Considerations:
   Psych/Social issues play a HUGE role in caring for ALS patients.
      1.    Assess patient’s level of acceptance and understanding of the ALS diagnosis.
      2.    Assess patient’s level of coping for loss of independence.
      3.    Evaluate level of support system the patient has.

   Management of psych/social issues.
      1.   Provide a therapeutic environment for the patient and family to express their
           concerns and fears with each other and members of the health care staff.
      2.   Allow patient to participate in care to their maximum potential to support a sense of
      3.   Provide patient and family information for ALS support groups in their community.
      4.   Possibly refer patient and family to a counselor, psychiatrist, or psychologist for
           further supportive needs.
      5.   Provide encouragement and anticipate unasked questions.
      6.   Discuss changes in body image and how they affect the patient’s self-esteem.
      7.   Encourage patient to focus on the positive aspects of life.
      8.   Allow time for the patient/family to grieve over the diagnosis.
      9.   Discuss advanced directives and DNR code status with patient/family.
Nursing Considerations continued…
   Muscle Weakness and Skin Integrity
      1.   Assess motor strength and measure level of spasticity/flaccidity.
      2.   Assess for muscle contractures.
      3.   Assess skin integrity daily for breakdown.

   Management of Muscle status/Skin Integrity.
      1.   Encourage patient to participate in daily activities as tolerated.
      2.   Patient should follow an exercise routine to preserve muscle tone.
      3.   Range-of Motion exercises to prevent contractures, may be active or
           passive with Physical Therapy involved in care.
      4.   Reposition patient every 2 hours, while assessing skin with each turn.
      5.   Wheel chair repositioning is key. Wheelchair should have a cushion to
           prevent breakdown.
      6.   Educate caregiver on how often the patient should be turned and how
           to assess skin for changes.
Nursing Considerations continued…
   Nutrition/Elimination Needs
      1.   Assess ability to swallow and presence of gag reflex.
      2.   Assess diet/fluid intake.
      3.   Assess elimination patterns.
      4.   Assess for incontinence and ability to transfer to a commode/toilet.
      5.   Assess for s/s of UTI.

   Management of Nutritional and Elimination needs.
      1.   Elevate HOB to prevent aspiration for patients with dysphagia.
      2.   Have a Dietician evaluate the patient’s caloric needs.
      3.   Encourage fluid intake of 2500 mL/day unless contraindicated by
           difficulty swallowing.
      4.   Educate patient about need for increased dietary fiber intake to
           prevent constipation.
      5.   Encourage patient/care givers to document bowel movements.
      6.   Daily stool softeners and Metamucil are recommended to prevent
Nursing Considerations continued…
   Respiratory status.
      1.   Obtain history from patient/caregivers that indicates SOB or difficulty breathing.
      2.   Assess patient’s respiratory status including: respiratory rate, effort of breathing,
           use of accessory muscles, nasal flaring, skin color, gag/swallow reflex, and
      3.   Auscultate lungs to assess for adventitious sounds

   Management of Respiratory status.
      1.   Suction oral secretions to prevent aspiration.
      2.    Administer medications that reduce secretions as ordered by the MD.
      3.   Encourage TCDB and use of Incentive spirometer.
      4.   Administer Oxygen PRN.
      5.   Elevate HOB to allow for easier breathing and decrease the risk of aspiration.
      6.   Educate caregiver on how to prevent aspiration.
      7.   Monitor/Assess need for mechanical ventilation.This may occur as condition
           worsens. Life support measures should be discussed prior to the need of
Nursing Considerations continued…
   Mobility/Safety
     1.   Assess current level of mobility (i.e.: use assistive devices and
          ability to perform own ADL’s).
     2.   Teach patient/family to remove all slippery rugs from home and
          replace with slip-resistant rugs.
     3.   Teach patient/family to remove all cords and wires from walk ways.
     4.   Ongoing Physical Therapy is needed to maintain mobility and
          muscle function.
     5.   Educate caregiver/family on how to safely transfer patient.

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