ALS: DISEASE TRAJECTORY AND HOSPICE ELIGIBILITY Terri L. Maxwell PhD, APRN VP, Strategic Initiatives Weatherbee Resources Inc Hospice Education Network Inc Course Handouts & Disclosure To download presentation handouts, click on the attachment icon Presenter discloses no financial relationships with a commercial entity producing healthcare-related products and/or services. Conflict of interest disclosure and resolution statement is on file with HEN. This presentation is for educational and informational purposes only. It is not intended to provide legal, technical or other professional services or advice. Learning Objectives Describe the clinical course of ALS State symptoms experienced by patients with ALS Identify secondary conditions commonly associated with ALS Explain end-of-life issues experienced by ALS patients and their caregivers Name the clinical data points necessary to substantiate hospice eligibility for patients with ALS 1
Amyotrophic Lateral Sclerosis (ALS) Disease of the nerve cells in the brain and spinal cord that control voluntary muscle movement (degenerative motor neuron disease) Upper (brain) and lower (spinal cord) neurons eventually die, ceasing to send messages to the muscles Muscles eventually weaken, atrophy, twitch and voluntary movement is lost Cognition, eyesight, and bladder and bowel control are not usually impaired ALS Usually occurs at random (genetic defect associated with 10% of cases). Life expectancy 3-5 yrs, but some pts live much longer. Death usually a result of respiratory failure ALS Symptoms Early: Muscle stiffness/ weakness Slurred speech Difficulty swallowing Cramps Fasciculations Late: Progressive muscle atrophy/weakness Swallowing/chewing problems Respiratory difficulty Emotional labiality 2
Secondary conditions Weight loss Aspiration Pressure sores Pneumonia Pain Constipation Lung failure Drooling of secretions Symptoms of chronic hypoventilation (am headache, anorexia, wtloss, depression/anxiety, severe fatigue) ALS Management Supportive care (interdisciplinary approach): Medical management Speech therapy Nutrition assessment/swallow evaluation PT/OT/Rehab Respiratory therapy/ pulmonology Social Worker Home care/hospice Support groups Palliative care Medical management: Riluzole (rilutek) Reduces damage to motor neurons by decreasing the release of glutamate Used to delay disease progression and may delay need for tracheostomy or onset of ventilator dependence May prolong survival by 3-4 months, Cochran review 2005 Side effects include fatigue, nausea, diarrhea and liver toxicity 3
Triggers for Initiating Discussion about Hospice/Palliative Care The patient or family asks or opens the door for end-of-life information and/or interventions, or Severe psychological and/or social or spiritual distress or suffering, or Pain requiring opiates, or Dysphagia requiring feeding tube, or Dyspnea or symptoms of hypoventilation, forced vital capacity at 50% or less, or Loss of function in two body regions Reference: Promotingexcellence.org ALS & Hospice Utilization O.4% of hospice admissions (NHPCO Facts & Figures, 2012) End-of-Life Issues/Challenges Totally dependent on others for care May require nursing home placement Decisions regarding use of PEG tubes, mechanical ventilation Advance care planning- important to discuss wishes early on 4
Amyotrophic Lateral Sclerosis How should a swallowing impairment be documented? Common documentation: Swallowing difficulty Suggested documentation: New onset of upper digestive tract impairment noted; patient chokes on thin liquids; patient has inadequate cough to clear secretions post swallow. LCD Guidelines for Hospice Eligibility and Recertification for ALS NGS LCD Number L25678 CGS LCD Number L32015 NHIC LCD Number L29881 Part III Disease-Specific Guideline Note: These guidelines are to be used in conjunction with the Non-disease specific baseline guidelines described in Part II of the LCD A. KPS / PPS < 70% B. Two or more ADL dependence C. Co-morbidities that contribute to life expectancy of 6 months or less 5
General Considerations Disease tends to progress in a linear fashion, but progression is variable History of rate of progression is important Multiple clinical factors are important to judge the progression of ALS Ability to breathe and to a lesser extent, ability to swallow are critical determinants of prognosis Mechanical ventilation and gastrostomy tube significantly alter 6 month prognosis. Examination with a neurologist within 3 months of assessment for hospice is recommended to assist with prognosis. Non-disease Specific Guidelines Both A & B must be met: A. Impaired functional status- KPS <70 or PPS <70 B. Dependence on assistance for 2 or > ADLs C. Presence of co-morbidities that contribute to disease burden HF Diabetes Dementia, etc. Disease-specific Criteria Patients will be considered to be in the terminal stage of ALS (life expectancy of six months or less) if they meet the following criteria. (Should fulfill 1 and/or 2). Patient should: 1. Do not elect tracheostomy and invasive ventilation and demonstrate critically impaired breathing capacity 2. Demonstrate critical nutritional impairment (with or without gastrostomy tube). 6
Disease-specific Criteria 1. Critically impaired breathing capacity as defined as: Vital capacity (VC) less than 40% of normal and 2 or more of the following*: Dyspnea at rest Orthopnea Use of accessory respiratory musculature Paradoxical abdominal motion Respiratory rate >20 * If unable to perform FVC test patients should manifest 3 or > symptoms Disease-specific Criteria 1. Critically impaired breathing capacity definition continued: Reduced speech/vocal volume Weakened cough Symptoms of sleep disordered breathing Frequent awakening Excessive daytime sleepiness Unexplained: headaches, confusion, anxiety, nausea Disease-specific Criteria 2. Demonstrate critical nutritional impairment defined as : Dysphagia with progressive weight loss of at least 5% of body weight with or without election for gastrostomy tube insertion 7
Clinical Indicators of Progressive ALS Progression from independent ambulation to wheelchair to bed-bound status; Progression from normal to barely intelligible or unintelligible speech; Progression from normal to pureed diet; Progression from independence in most or all ADLs to needing major assistance by caretaker in all ADLs. Admission Documentation Answer the question Why Hospice, Why Now? Decline in health and functional status in months preceding referral to hospice Changes in nutritional status and associated weight loss Changes in functional status (include, but don t limit to PPS or KPS) Respiratory status Cognitive status History of hospitalizations, ED visits, etc. Goals of care Ongoing Documentation for Recertification Descriptions of: Declining functional status/disability Weight loss or indicators of such Respiratory status, aspiration, secretions, etc. Symptoms, including pain Secondary conditions- pressure ulcers, infections, emotional labiality, depression Medication changes Use of assistive devices Caregiver burden 8
Documentation sample (admission) Allen, age 53 was diagnosed with ALS 16 mosago after noticing difficulty lifting weights in the gym. His condition has progressed and he recently d/c d rilutek. He now requires total assist to transfer from bed to w/c and is completely dependent in 5/6 ADLs. He is continent of urine/stool, but has bouts of severe constipation despite a bowel program. His speech is largely unintelligible, although his wife and son are able to understand some of what he says. He is learning to communicate with a computer-assisted device. Documentation sample (admission cont d) He is alert and oriented x 3, but has become anxious due to worsening difficulty breathing. He s lost 30 lbs (5 10, 158lbs) over the past 8 weeks. He tolerates his pureed diet poorly and in the past 3 weeks, is having frequent choking episodes. Over the past 2 weeks, he s become increasingly dyspneic at rest, RR 26. He sleeps 4/8 hours during the day and is awake frequently at night. He is married with 2 sons ages 19 & 21-both attending college. He refuses mechanical ventilation, but uses C-PAP at night. He does not want a PEG and has an advance directive requesting comfort measures only Summary ALS is a progressive, incurable condition although disease progression varies. Patients experience physical decline, nutritional deficiencies, loss of communication, respiratory compromise, and emotional changes that benefit from supportive care provided by hospice. History of rate of progression is an important predictor of prognosis as are decisions to institute artificial ventilation or feeding. 9
Course Evaluation & Post Test Thank you for viewing this course on the Hospice Education Network The course evaluation and post test are available from your course catalog page THANK YOU! Terri Maxwell PhD, APRN VP, Strategic Initiatives Weatherbee Resources Inc. Hospice Education Network tmaxwell@weatherbeeresources.com 10