Amyotrophic Lateral

Sclerosis

Presented by

Professor Kathy Mitchell

Algonquin College

Ottawa Ontario Canada

COURSE CONTENT

1. What is ALS

2. Community Resources

3. Health care team

4. Assessment of client’s needs

5. Physiological and Psychosocial needs

6. Managing the illness at home

7.Ethical issues and decision making

8.Research and hope for the future

ALS

Lou Gehrigs’ disease-June 2 1941-United States-age 37

Motor Neurone Disease-Europe

Maladie de Charcot -1840

ALS

Progressive degenerative neuromuscular disease

Attacks motor neurons which control voluntary muscles-movement of arms legs

Muscles used in swallowing, breathing and talking

Does not affect: Cognitive status

Vision and eye movement

Sensory system

Bowel/bladder/sexual function

Types

Sporadic-90-95%

No family history

Familial-5-10%

Adult Familial-gene defect on chromosome 21

20% have deficiency of SOD1-super oxide dismutase enzyme

Physiology

No known cause or cure

Not contagious or infectious

Age dependent-80% of all cases between 40-70

Life expectancy 18 months-5 years

Men 1.5 ratio to 1.0 women

Diagnosis

Done by excluding other illnesses

El Escorial ALS diagnostic criteria

History, Physical , Neurological exam

Signs of upper or lower motor neuron degeneration with progressive spread

Clinical symptoms

Muscle weakness

Muscle atrophy

Shortness of breath

Slurred speech

Occasional choking

Limb Onset

Weakness

Fasciculations-involuntary muscle twitches

Atrophy-muscle deterioration

Dysarthria-inability to articulate words

Respiratory impairment

Progressive muscular weakness

Bulbar onset

Speech disorder-weakness in tongue,jaw lips-dysarthria

Difficult swallowing-dysphagia

Respiratory problems-dyspnea

Excessive salivation-sialorrhea

Fatigue

Disease Progression

Client specific- Length of illness unpredictable

Paralysis of voluntary muscles

Atrophy

Cause of death-respiratory failure

Interdisciplinary Team

All team members contribute from their own professional expertise to support the client and family

Nursing Model of Assessment is based on the work of Calista Roy

Health Team

Person with ALS

Family

Physician/physiatrist

Registered Nurse

Physiotherapist

Occupational Therapist

Speech Therapist

Nutritionist

Health Team

Social Worker

Pastoral care Worker

Home Care worker

Community based care-mobility

Respiratory Therapist

Palliative care

Holistic Care

Disease progress is unpredictable

Continuous assessment, implementation and evaluation of needs of client and family

Communication with health care team is ongoing

Physical and psychosocial needs create major challenges

Physiologic Needs

Oxygenation

Nutrition

Fluids and electrolytes

Elimination-Bowel and Bladder

Activity and Rest

Protection

Psychosocial Needs

Self concept

Role function

Interdependence

Current Treatments

Home care based

Long term management of client problems

Medication-to alleviate symptoms

Physical therapy-maximize capabilities

Speech therapy augmented communication

Nutrition-p.e.g. tube

Ventilation-non-invasive vs invasive

Hope for the Future

Longer prognosis

Live beyond five years

Physical impairment will move slowly

Assistive devices will maintain independence

Minimum of suffering

Drug therapy will progress-Riluzole

Effective symptom management

Hope for the Future

Positive attitude

Firm belief system

Support of family and friends

Spirituality

Hiroshi Mitsumoto Columbia University

Спасибо доктору Льву Брылеву и Фонду «Живи Сейчас»!

Contact details Ottawa

Kathy Mitchell R.N. B.N.

613 727-4723 ext 5389

www.algonquincollege.com

Email: mitchek@algonquincollege.com