Assessment & Intervention of Amyotrophic Lateral Sclerosis (ALS)

1. Assessment & Intervention of Amyotrophic Lateral Sclerosis (ALS) OT 510/515 Rachel Rombalski Amy Londry Rachel Csatari Alyssa Kolanowski

2. Introduction • Brief overview of ALS • Stages of ALS • Case Study: Roberto • Areas of Occupation Affected • Role of Occupational Therapy in Treating ALS • Assessment Protocol • Expected Assessment Results • Rehabilitative Frame of Reference • Client-Centered Intervention

3. Amyotropic Lateral Sclerosis (ALS) (Lou Gehrig’s Disease) • A group of progressive, degenerative neuromuscular diseases. • Destruction of motor neurons (upper and lower) within the spinal cord, brain stem, and motor cortex. • 2 types: Familial ALS and Sporadic ALS • Progressive bulbar palsy, Progressive spinal muscular atrophy, and Primary lateral sclerosis • Focal weakness beginning in arm, leg, and bulbar muscles. • The individual may trip or drop things, may have slurred speech, abnormal fatigue, and uncontrollable periods of laughing or crying. • As the disease progresses there is noted muscle atrophy, weight loss, spasticity, muscle cramping, and fasciculations (twitching of the muscle fascicles at rest). • **Does not affect cognition, but overall prognosis is difficult to predict because it varies from person to person.

4. Stages of ALS • Stage I • Mild weakness, clumsiness, ambulatory, independent with ADLs • Stage II • Moderate, selective weakness, slightly decreased independence in ADLs • Stage III • Severe, selective weakness in ankles, wrists, and hands. Moderately decreased independence in ADLs. Tendency to become easily fatigued with long-distance ambulation. • Stage IV • Hanging-arm syndrome with should pain and sometimes edema in the hand. Wheelchair dependent. Severe lower extremity weakness. Able to perform some ADLs, but fatigues easily. • Stage V • Severe lower extremity weakness. Moderate to severe upper extremity weakness. Wheelchair dependent. Increasingly dependent in ADLs. At risk for skin breakdown caused by poor mobility. • Stage VI • Dependent, with all positioning in bed and wheelchair. Completely dependent in ADLs. Extreme fatigue.

5. Case Study: Roberto • 60 y/o married male, diagnosed with SALS 5 months ago, currently in stage II • Golfer, plays with grandchildren, cooking, takes trips, breakfast club with friends • Client wants to continue doing these activities as long as possible • Willing to use adaptive equipment, but never wants to be dependent with feeding • Currently receiving outpatient rehab with an OT • OT is using a completely client centered approach with the rehabilitative fame of reference • Things noted in occupational profile: • Abnormal fatigue • Uncontrollable periods of laughing or crying • Muscle weakness in arms and legs • Has trouble keeping balance in his life between activities • He “doesn’t know where his day goes”

6. Areas of Occupation Affected • ADLs • Bathing, dressing, eating, feeding, functional mobility, personal device care, hygiene/grooming • IADLs • Care of others, care of pets, community mobility, health management and maintenance, home establishment and management, meal prep and clean up, shopping • Rest and Sleep • Leisure • Participation in • Social Participation • Family and friends

7. Role of Occupational Therapy in Treating ALS • Maximize ADL and IADL independence • Equipment and Modifications • Educating patient and family • Prescribing appropriate exercise, ROM, MMT • Preventing complications of immobility • Eliminating or preventing pain • Energy conservation Lewis, M. & Rushanan, S. (2007). The role of physical therapy andoccupational therapy in the treatment of amyotrophic lateralsclerosis. NeuroRehabilitation, 22, 451-461.

8. Assessment Protocol • **Interview + Occupational Profile + Skilled Observation of Functional Tasks • FIM • Canadian Occupational Performance Measure (COPM) • Klein-Bell Activities of Daily Living Scale • Scoreable Self-Care Evaluation • Self-Identification Goals Assessment (SIGA)

9. Expected Assessment Results • The need for: • Adapted equipment • Built up eating utensils • Built up golf club • Shower stool • Handrails • Frequent rest breaks • Fatigue • Functional mobility • Wheelchair

10. Rehabilitative Frame of Reference • ALS is a degenerative disease • 80% of individuals with ALS die within the first 3-5 years of diagnosis • OT’s maximize independence and will focus on the here and now • Compensatory methods and adaptive equipment

11. Client-Centered Intervention • Stretching/Strengthening/ROM • Environmental modifications • Energy conservation • Assistive technology/Adaptive equipment • Wheelchair assignment Lewis, M. & Rushanan, S. (2007). The role of physical therapy andoccupational therapy in the treatment of amyotrophic lateralsclerosis. NeuroRehabilitation, 22, 451-461.

12. Conclusion • ALS is a progressive degenerative disease • Although more research is needed, it is evident that occupational therapy intervention is beneficial in managing the symptoms of ALS. • The main goal of OT intervention for a client with ALS is to enhance the quality of his/her life.

13. References • Cup, E. H. C., Pieterse, A. J., Knuijt, S., Hendricks, H. T., Van Engelen, B. G. M., Oostendorp, R. A. B., & Van Der Wilt, G. J. (2006). Referral of patients with neuromuscular disease to occupational therapy, physical therapy and speechtherapy: Usual practice versus multidisciplinary advice. Disability and Rehabilitation, 29(9), 717-726. • Lewis, M. & Rushanan, S. (2007). The role of physical therapy and occupational therapy in the treatment of amyotrophic lateral sclerosis. NeuroRehabilitation, 22, 451-461. • Pendleton, H. M. & Schultz-Krohn, W. (2006). Pedretti’s occupational therapy: Practice skills for physical dysfunction. St. Louis Missouri: Mosby Elsevier. • MDA ALS Division (May, 2009). Everyday Life With ALS: A Practical Guide. Retrieved from http://www.als-mda.org/