MG-GBS OT 537
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The Role of OT with Myasthenia Gravis and Guillain Barré Syndrome
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MG-GBS OT 537
- 1. OT 537
- 2. Myasthenia Gravis (MG) • Chronic autoimmune neuromuscular disorder that affects the neuromuscular junction of voluntary muscles • Acetylcholine receptor antibodies destroy ACh receptors which prevents the transmission of this neurotransmitter, resulting in severe muscular weakness and fatigue
- 4. Onset and Progression An Inside Story: Life with MG KEY TERMS: • Diplopia • Ptosis
- 6. Signs & Symptoms Experienced with MG
- 7. Diagnosis • Can be difficult to diagnose • Thorough physical exam often reveals easy fatigability • Decreased/absent/slow reflex responses may be noted • Blood test for antibodies against the acetylcholine receptor (sensitivity ~50%) • A good response to medication can also be considered a sign of this type of autoimmune pathology. • The Ice Test • Chest x-ray • Spirometry (lung function testing) • Muscle biopsy
- 8. Medical Intervention Medications: • Acetylcholinesterase inhibitors • Neostigmine • Pyridostigmine • Steroids • Prednisone • Cortisone Surgery: • Removal of the thymus gland (“thymectomy”) Other Interventions: • IVIG - Intravenous immune globulin • Plasmaphoresis There is no cure for MG, but the symptoms can be controlled.
- 9. OT Evaluation • Occupational profile – roles, routines, habits • Analysis of Occupational Performance • ROM and strength testing including grip and pinch strength, MMT • Observation of functional mobility and transfers
- 10. OT Evaluation (con’t) • Testing and observation of performance in – • ADLs/self-care routines • iADLs • Leisure interests • Home – safety and accessibility • Canadian Occupational Performance Measure (COPM) • Functional Independence Measure (FIM) • Barthel Index • Perceived Exertion Scale • Myasthenia Gravis Activities of Daily Living Profile • Quantitative Myasthenia Gravis (QMG) Scale • Assessment of Motor and Process Skills
- 17. References & Resources • Christiansen, C. & Baum, C. (2005). Occupational Therapy: Performance, Participation, and Well-Being. Thorofare, NJ: Slack. • Myasthenia.org - http://www.myasthenia.org • National Institute of Neurological Disorders & Stroke – www.nids.hih.gov/ • Paul, R. and Gilchrist, J. (2003). Psychological and social consequences of Myasthenia Gravis. In Kaminiski, H. (Ed.), Myasthenia Gravis and Related Disorders. Humana Press. • Radomski, M. V. & Trombly Latham, C. A. (2013). Occupational therapy for physical dysfunction. 7th ed. Philadelphia: Lippincott, Williams & Wilkins.
- 18. OT 537
- 19. Demographics
- 20. Signs & Symptoms Rapidly progressive weakness Typically follows illness of some sort like the flu Bladder incontinence is common in GBS but the bowel is typically not affected.
- 21. Medical Intervention and Prognosis Recovery is often prolonged, lasting after progression stops. Fatalities from GBS are rare.
- 22. Phases of GBS Plateau Phase – Significant edema and weakness, especially in LE’s, is common, along with pain and extreme fatigue. At risk of ANS dysfunction (postural hypotension) especially when changing positions Onset Phase – Includes severe edema, muscle weakness or paralysis, sensory loss, bladder incontinence, and pain. Requires total assistance for ADL’s. At risk for contractures and pressure sores as well as respiratory and psychosocial dysfunction. Recovery Phase –Strength is starting to be regained in UE/LE and trunk. Endurance continues to be very limited. Mobility and gradual re-entry to the daily living environment and routine as tolerated are primary goals of this phase. Consider: What is the role of OT during each of these phases? What ADLs/iADL s is this likely to affect?
- 23. GBS Acute inflammatory demyelinating polyneuropathy (AIDP) Miller Fisher syndrome (MFS) diplopia ptosis ataxiaChronic inflammatory demyelinating polyneuropathy (CIDP)
- 24. OT Evaluation
- 25. Developingan InterventionPlan Occupational therapy practitioners ask, "What matters to you?" … not "What's the matter with you?” • What’s important to the client (and caregivers)? • What can be done to promote safety, health and overall well-being, self-esteem, and self-satisfaction?
- 26. OT Intervention • Expand activities gradually. • Increase repetitions before resistance in order to avoid injury to muscles, tendons and joints. • Client and caregiver education
- 27. References & Resources • National Institute of Neurological Disorders & Stroke – www.nids.hih.gov/ • GBS/CIDP Foundation International - www.gbs-cidp.org • http://www.gbsnz.org.nz/guillain_barre_syndrome • Guillain-Barre Syndrome Foundation International – www.gbs- cidp.org • Radomski, M. V. & Trombly Latham, C. A. (2013). Occupational therapy for physical dysfunction. 7th ed. Philadelphia: Lippincott, Williams & Wilkins.
- 28. Practice Question An occupational therapist evaluates a client who has just entered the plateau phase of GBS. When assessing ADLs, what would the OT most likely expect to see? A. Bowel and bladder incontinence, which affects skin integrity and toileting routine B. Unilateral sensory impairments, which affect fine motor tasks like oral care C. Edema and weakness in the LEs, which makes bathing and dressing difficult D. Shoulder weakness, which results in decreased independent in UB bathing and dressing
Editor's Notes
- In Latin: grave muscle weakness
- An autoimmune disease that affects skeletal muscles. Skeletal muscle constitutes the muscle that is attached to the skeleton and controls motor movements and posture. There are a few instances where this type of muscle is restricted to soft tissues: the tongue, pharynx, diaphragm and upper part of the esophagus. Podcast series on MG: http://www.myasthenia.org/LivingwithMG/PodcastEducationalSeries.aspx
- Affects people of any age Women < 40 and men > 60 The cause of this bimodal distribution isn’t clear. The disease incidence is 3–30 cases per million per year and rising as a result of increased awareness. Genetic factors also may be associated. If a woman with myasthenia gravis gives birth, the baby may have some temporary, and potentially life-threatening, muscle weakness (neonatal myasthenia) because of antibodies that have transferred from the mother's bloodstream. Typically, during the baby's first weeks of life, the antibodies are cleared from the baby's circulation and the baby develops normal muscle tone and strength.
- Relatively sudden onset – fast progression May wake up feeling fine one day but begin to experience weakness later in the day Typically, disease affects eye and head musculature first and progression continues to limbs and sometimes respiratory muscles Progression: eyes (diplopia – double vision; or ptosis – drooping eyelids) musculature of the head LE/UE and in some cases trunk/respiratory muscles Video- 14:53
- Most patients need treatment for the remainder of their lives, and their abilities vary greatly. MG is not usually a progressive disease; the symptoms may fluctuate, but do not always get worse as the patient ages. For some, the symptoms decrease after a span of three to five years ( costanzo, 2012).
- A life threatening manifestation of the disease – occurs with the weakness affects certain muscles involved with basic life functions
- In specific situations (decreased reflexes which increase on facilitation, coexisting autonomic features, suspected presence of neoplasm, especially of the lung, presence of increment or facilitation on repetitive EMG (electromyography) testing) testing is performed for Physical examination Muscle fatigability can be tested for many muscles. A thorough investigation includes: looking upward and sidewards for 30 seconds: ptosis and diplopia looking at the feet while lying on the back for 60 seconds keeping the arms stretched forward for 60 seconds ten deep knee bends walking 30 steps on both the toes and the heels Ice Test - Applying ice for 5 mins. to weak muscle groups characteristically leads to improvement in strength of those muscles. It is thought that acetylcholinesterase is inhibited at the lower temperature and that this is the basis for this diagnostic test.
- ACh inhibitors - Stop breakdown of Ach which ends up resulting in increased concentration of Ach around the muscle cells Steroids – reduce production of harmful antibodies (auto-antibodies) - produce total remission / marked improvement in > 75% of patients Removal of thymus gland – reduces muscle weakness Specific treatment to age, overall health, and medical history and extent of the condition The goal of treatment is to prevent respiratory problems and provide adequate nutritional care to the child since the swallowing and breathing muscles are affected by this condition.
- Grip strength measurement – dynomometer May combine a bottom-up approach to address issues of impairment and client factors along with top-down performance measures (see next slide)
- Tools used for OT eval may or may not have been developed with this client population in mind Self-report questionnaires/scales to report client’s perception of function/problems Documentation should include performance in various contexts of environment and time as well as impact of medications (typically best performance occurs 1.5 – 2 hours after dosing with pyridostigmine)
- The condition leads to fluctuating muscle weakness and fatigue Muscle strength Endurance Functional balance ADL performance Safety Memory Gross and fine-motor skills - hand atrophy Environmental access Occupational performance (including work and leisure activities)
- Brochure p. 84
- Specifics of energy conservation – p. 87
- Handout p. 86 Diet Patients may experience difficulty chewing and swallowing due to oropharyngeal weakness Thickened liquids are preferred, when dysphagia arises to counteract the fear of aspiration. Asparagus should be taken as it contains steroid-like substance. Activity Patients should be as active as possible but should take rest in between. Yoga exercises to stretch the weakened muscles should be done. This not only strengthens the muscles but also provides oxygen & removes carbon dioxide from them. Teach patient/family disease process, complications, and treatments Teach patient about their medications uses dosage etc -- Teach medications to use with caution d/t muscle exacerbation Beta blockers, calcium channel blockers, quinine, quinidine, procainamide, some antibiotics, neuromuscular blocking agents Avoid certain medications -- D-penicillinamine, A-interferon, botulinum toxin