Jason Conk

ALS- Lou Gehrigs Disease

FACTS

PURPOSE

Progressive
neurodegenerative
disease affecting nerve
cells in the brain and
spinal cord.

The purpose is to evaluate the best available

Evidence-Based Practice (EBP) to guide fundamental
nursing care in order to provide the most
comprehensive care possible for individuals living
with ALS.

Death of motor neurons

leads to loss of muscle
movement.

Progression of disease
eventually leads to total
paralysis.

Progressive degeneration
of the motor neurons
eventually leads to death.

Evidence for Care

Stats

Manifestations

30,000 Americans affected

Muscle Weakness

Can affect anyone

Fasciculation & cramping of

muscles

Caucasians at highest risk

Onset 40-60 y/o
No risk factors
Riluzole only drug for care
Average lifespan only 2-4
years

Difficulty projecting speech

and dysphagia
Hyperreflexia
Incoordination
Muscle atrophy

Nursing Theory
l

Hildegard Peplau
Theory of Interpersonal Relationships
The Seven Nursing Roles
The Stranger Role
The Resource Role
The Teaching Role

The Counseling Role

The Surrogate Role
The Active Leadership Role
The Technical Expert Role

Evaluation of the Evidence

Methods
Longitudinal Study
9 month study
Zung Depression Scale (ZDS)
McGill Quality of Life (MQoL)
Caregiver Burden Inventory (CBI)

Self-Perceived Burden Scale (SPBS)

Evaluation of the Evidence

Methods

Results

Longitudinal Study

9 month study

Zung Depression Scale

(ZDS)

McGill Quality of Life

(MQoL)
Caregiver Burden
Inventory (CBI)
Self-Perceived Burden
Scale (SPBS)

ZDS

Patient= Even
Caregiver = Increase

MQoL

Patient= Increase
Caregiver= Decrease

CBI

Patient= Increase
Caregiver= Increase

SPBS

Patient = Increase
Caregiver= Increase

Evaluation of Evidence
Article Critique

Not a nursing journal

Not performed by nurses

Is applicable to nursing

Ordinal Level of Evidence

QSEN Level 2

Threats to validity: Testing, Maturation, Mortality, Hawthorne Effect

Limitations

Small sample size

Many threats to validity

Deterioration of mental function

Patient Care Design

Patient

OT

Nurses

Psychiatry

Nurse Aides

Respiratory Therapists

PCP

Social Workers

Neurology

Clergy

Radiology

Hospice/ Palliative
Care

Case Study
A 51-year-old woman developed an unusual gait related to "calf
stiffness." After several months of progressive weakness, she
developed a right foot drop. Within 15 months, she also described
difficulty in writing, weakness of the right hand and arm, and diffuse
muscle twitching with painful muscle cramps. Assessment confirmed
weakness of all limbs, more marked on the right side, with muscle
atrophy. Reflexes were hyperactive and fasciculations were observed
in the right leg.
Within two years, the patient became severely disabled because of
generalized weakness. She experienced no sensory problems,
intellectual deterioration or skin breakdown in spite of her degree of
immobility. Difficulty in swallowing necessitated the use of a
gastrostomy tube for feeding. The patient died of respiratory failure
approximately three years after reporting the initial symptoms.

Patient Care Design- TRUEPIC

Patient Care Design- TRUEPIC cont.

Patient Care Design

Primary Nursing Diagnosis:
Death anxiety r/t impending loss of function leading to
death.
Impaired swallowing r/t weakness of muscles involved in
swallowing.

Overall approach to care:

Palliative/ hospice care is the primary care.
Referrals for spiritual/ psychiatric care.
Suctioning and tube feeding in later stages of disease.

Advocate Role

Riluzole
Suctioning
Tube feeding
Respiratory care
Palliative care
Referrals

Emotional
Support

References
Colley, S. (2014). Nursing 441: Nursing Theory [Syllabus] p. 6.
Gauthier, A., Vignola, A., Calvo, A., Cavallo, E., Moglia, C., Sellitti, L.,
Mutani, R., Chi A. (2007). A longitudinal study on quality of life
and depression in ALS patientcaregiver couples. Neurology,
923-926.
Ladwig, G.B., & Ackley, B.J. (2011). Mosbys guide to nursing diagnosis (3 rd
ed.). Maryland Heights, MO: Mosby Elsevier.
Smeltzer, S. C., Bare, B. G., Hinkle, J. L., Cheevver, K. H. (2010). Brunner &
Suddarths textbook of medical-surgical nursing (12 th ed.).
Philadelphia, PA: Wolters Kulwer Health Lippincott Williams &
Wilkins

Taylor, C., Lillis, C., LeMone, P., and Lynn, P. (2011). Fundamentals of
nursing: The art and science of nursing (7 th ed.). Philadelphia, PA:
Lippincott Williams & Wilkins