Assessment & Intervention of Amyotrophic Lateral Sclerosis (ALS) Rachel Rombalski Amy Londry Rachel Csatari Alyssa Kolanowski

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

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. Which subtype of ALS does this person have???????? Specifiy? PMSA?

Stages of ALS Stage I Stage II Stage III Stage IV Stage V Stage VI 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.

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”

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

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 and occupational therapy in the treatment of amyotrophic lateral sclerosis. NeuroRehabilitation, 22, 451-461.

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)

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

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

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 and occupational therapy in the treatment of amyotrophic lateral sclerosis. NeuroRehabilitation, 22, 451-461.

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.

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/