Management of Respiratory Failure in ALS Laura Binneboese University of Iowa: College of Nursing

Objectives Give a short overview of ALS End of Life Care Early signs of ALS Prognosis End of Life Care Interventions Treatment Options Spark interest in the targeted audience Provide the means to further research and explore a disease that is often misunderstood, unfamiliar, and in major need of a cure.

ALS Overview Amyotrophic Lateral Sclerosis (Also known as Lou Gehrig's Disease) “A” meaning absence of, “Myo” meaning muscle, “Trophic” meaning nourishment, “No Muscle Nourishment,” (ALS Association) Without muscle nourishment, these nerves begin to atrophy. Neurodegenerative disease that affects the nerve cells in the brain and spinal cord. Causes progressive loss of muscle movement affecting ones ability to speak, eat, and move. Eventually leads to loss of respiratory function, causing death. Approximately 5,600 are diagnosed each year (2 in 100,000). There is no cure for this disease.

Early signs of ALS Gradual onset, initial s/s varying among patients Unilateral weakness and/or fatigue in hands, arms, or legs (then progresses to remaining side) Different tone or pitch in voice Tripping, dropping things, slurred speech Motor neurons are affected, while senses are still intact: hearing, smell, taste, sight, and touch all remain present throughout entire course of disease. Even uncontrollable laughing or crying can be an early sign of ALS.

Prognosis On average, people live 2-5 years from initial diagnosis. Although rare- there have been cases of ALS symptoms stopping progression, and some that have been reversed. Thus allowing individuals with the diagnosis to live beyond the average expectancy.

Focus on End of Life Care for Patients with ALS ALS patients eventually become bedridden, completely dependent on their care takers, and essentially trapped inside their paralyzed bodies fully aware of their situation and surroundings. Respiratory failure (almost always) occurs Patients need to be properly prepared for this

Early Palliative Care is Imperative Intended to ease and prevent suffering for patient and family while also trying to improve their quality of life. Involves physical, spiritual, social, and emotional care and therapy. Educating the patient on the disease course, prognosis, complications, and treatment options is crucial in providing the best quality of care. Family education is just as important! Loved ones often suffer with the individual affected. Its important to be honest, open, and available for questions and concerns from family members throughout the entire process.

Patient Education They need to be aware of what’s happening to their body. They need to be told the truth about what to expect. They need support, a chance to express their fears and concerns, and they need to be comfortable enough to ask even the most sensitive or embarrassing questions. This is not a disease that offers much hope for the patient, and they often feel responsible for their family's emotions as well. ALS patients NEED unlimited access to resources.

Educating the family There are support groups for families. Focus needs to remain on the patient at all times, but the family will also need emotional support and possibly care of their own. WATCH: You’re Not You Starring Hilary Swank, a woman diagnosed with ALS Available on Netflix Provides an extraordinary take on every aspect on the disease! Patient, family, life, work, relationships, etc. Excellent movie for patients and family members to watch!

End Stage ALS The patient is considered to be terminal or nearing the end when respiratory function is compromised. Respiratory Failure eventually causes death, because muscles that help move the lungs are no longer able to assist in gas exchange. Also, other systems may be affected due to complications of immobility such as: Pulmonary (embolism) Cardiac (arrhythmias) Malnutrition (inability to swallow, eat/drink)

Hospice There is specific criteria to be met in order to receive hospice care and be covered by Medicare. Click link for more details: http://www.cms.gov/medicare-coverage-database Also, a physician needs to recommend Hospice care when they feel the patient has 6 months or less to live.

Management of Respiratory Care: Reason for early intervention : Weakness affects muscles required for inspiration, expiration, accessory muscles used for breathing, the diaphragm, and muscles of the upper airways. Positioning the patient can help prevent further restriction to these muscles Rather than lying supine while sleeping, raise the head of bed at least 45 degrees to alleviate orthopnea during the night. Reduces pressure on the diaphragm facilitating easier breathing during sleep.

Monitoring Lung Function AGB, Bicarb levels, and Pulse Oximetry are monitored There are numerous tests available to measure lung function (specifically muscle function) Inspiration, Expiration, Forced Vital Capacity (FVC), Maximal Inspiratory Pressure (MIP), Peak Cough Expiratory Flow (PCEF), Maximum Expiratory Pressure (Pemax) Sniff Nasal Pressure (SNP): used for individuals with severe muscle weakness of the mouth, which could make the above mentioned tests as inaccurate assessment. These tests help the patient, family, and care team know the level of respiratory compromise that’s occurring

Respiratory Care Interventions: Ensuring patient is current on vaccinations such as Flu and Pneumococcal, to prevent further risk of respiratory related infections and distress. Routine suctioning to prevent secretions from entering the lungs, causing either infection or aspiration. Proper positioning to ensure improved breathing. Raise head of bed during sleep, and position upright during time awake.

THE DISCUSSION Although hard, there must be a discussion early on about measures to take when respiratory failure is near. Continue this conversation throughout course of disease as the patient and family may change their mind or need further education. Non Invasive Ventilation (NIV) is a common recommendation early on when respiratory insufficiency is apparent. NIV doesn’t prevent progression of muscle weakness, BUT it does help provide relief of fatigue, episodes of breathlessness, and helps patients get better rest at night. This has been shown to IMPROVE QUALITY OF LIFE in patients with ALS. NIV is available in different forms, depending on the comfort level of each patient. Video supporting the benefits of NIV in ALS patients: https://www.youtube.com/watch?v=osH-C8-no4Q

Long-term mechanical ventilation (LTMV) This needs to be discussed prior to reaching the need for this type of respiratory assistance. Generally the LAST option for patients Not favorable in the US, mostly due to COST and the QUALITY of Life one can have with LTMV Can be done in the home if available to patient (depending on insurance, hospice team, physician recommendation, and location).

Continue research and educate yourself… ALS needs more attention The following links (references) are great places to start your research. My intention of this presentation is to spark interest on the topic of ALS. I highly suggest the movie “You’re Not You” starring Hilary Swank. Released in 2014. Available for live streaming on Netflix! This movie inspired me to research and really get into the topic of ALS.

One last video to get your attention “Only 32,000 people in the US have ALS,” (Anthony Carbajal). Perhaps this is the reason we don’t hear much about it? Please watch.. This four minute video will open your eyes at what it is like to live with ALS, knowing that soon you wont have any control of your body while your mind is still completely intact. Please watch this video: https://www.youtube.com/watch?v=DrsJrIcX9lw

References ALS Association. (2010). Facts you should know. Retrieved from http://www.alsa.org/about-als/facts-you-should-know.html American Medical Association. (2011). Local coverage determination for hospice determining terminal status. Retrieved from http://www.cms.gov/medicare-coverage-database MND care. (2014). Non-invasive ventilation (NIV). Retrieved from http://www.mndcare.net.au/Living-with-MND/Symptom- management/Respiratory/Dyspnoea/Non-invasive-ventilation- %28NIV%29.aspx Torres, Andrea L. (2012). The management of respiratory insufficiency in patients with ALS at or near the end of life. Home Healthcare Nurse, (30)3. Retreived from www.homehealthcarenurseonline.com Youtube.com. (2014). ALS disease must watch video- anthony carbajal’s story. Retreived from https://www.youtube.com/watch?v=DrsJrIcX9lw Youtube.com. (2013). Deborah gelinas: optimizing NIV in patients with ALS/MND. Retrieved from https://www.youtube.com/watch?v=osH-C8-no4Q