A Resource Guide for Patients with ALS in the Early, Middle, and Late Stages of Disease

 

Background:

Amyotrophic Lateral Sclerosis (ALS) is a degenerative upper and lower motor neuron disease that impacts about 15,000-21,000 individuals in the United States.¹ It is a progressive disease where patients rapidly lose function and expected life expectancy is 3-5 years. During this process of disease, patients go through a difficult and emotional grieving process, in which they are learning to cope with the loss of many aspects of their life. The role of physical therapy in this population is crucial however, research is limited regarding exercise and activity recommendations for people with ALS. Patients are also often hesitant to participate in exercise programs and are simultaneously experiencing increasing fatigue in their daily life.

 

Statement of Need:

After visiting the ALS multidisciplinary clinic a number of times, I noticed the role of physical therapy was mainly to provide energy conservation techniques, assistive devices, and regularly evaluate and document loss of function for ALS patients. After talking to my CI’s, Courtney Matrunick and Bryn Kennedy, they explained how there was a gap in care where these patients were not given any clear guidelines for exercise or sent home with an exercise program. Although research is limited, there is good evidence showing a combination of stretching, strengthening and aerobic, exercise at moderate intensities can in fact slow progression of disease and help preserve patients’ quality of life.

In addition, there is currently no advanced case study for the second year Neuro STEP UP scholars. The presentation I created aims to fill that need. It focuses on one of the patients I was able to see in the ALS multidisciplinary clinic who is in the mid-stage of ALS. The presentation challenges students to think of purposeful and appropriate interventions, as well as think ahead on what the role of PT is throughout the patient’s disease progression.

 

Purpose:

The handouts created are meant to be a simple and effective way for clinicians to provide home exercise recommendations and education for patients with ALS throughout the disease progression. The multidisciplinary clinic can be a challenging environment to complete a full evaluation with purposeful interventions, and these educational handouts are meant to make it easier on the therapists, as well as provide patients with a program they can work on until their next visit to the clinic. The HEP’s are designed as checklists so that therapists can quickly check off recommendations. There are three separate checklists which are for patients either in the early, middle, or late stages of disease. There are also pictures of each exercise to accompany the checklist as well as a fatigue tracking guide with education on over-fatigue for the patients to use daily. In addition, I created a breathing handout to improve respiratory function that can be given to patients at any stage of the disease, as well as a pressure relief handout with pictures for patients in the later stage of disease. These are meant to give the patients clear and organized instructions that they can follow at home as they typically only come to the clinic every three months.

The presentation is for STEP UP students, and is meant to provide an overview of ALS, go through a usual multidisciplinary visit, learn common outcome measures, and come up with interventions for patients in the mid stage of ALS. It is also meant to help them think ahead of how to modify care when the disease progresses. At the end of the presentation there are forum questions for them to answer and post in the STEP UP Sakai site.

 

Learning Objectives:

Patients

• To understand what safe and appropriate exercise is for patients with ALS
• To better pace activities throughout the day to improve activity tolerance
• To have an established HEP throughout the disease process

STEP UP Scholars

• To understand common signs and symptoms of ALS
• Develop an understanding of a usual course of PT care for an individual with ALS
• To learn common outcome measures used for patients with ALS
• Identify and apply evidence-based interventions for individuals with ALS

 

Products:

Exercise Guide Early^2,3,4

Exercise Guide Middle^2,3,4

Exercise Guide Late^2,9

Strengthening_ Pictures⁵

Stretch with Caregiver Pictures⁵

Independent Stretch_ Pictures⁵

Fatigue Tracking⁶

Pressure Relief Pictures⁷

Breathing Handout^8,9,10

ALS Advanced Case Study (Audio Presentation)

 

Self-assessment and Evaluation: 

Throughout my time creating these products, I was able to gain verbal feedback from a number of clinicians who work in the ALS clinic or have worked with patients with ALS. Many of them affirmed the need for these products as well as helped me to refine and tailor the exercise recommendations and educational materials. They also helped me to edit the case study and presentation for the STEP UP students. I am very pleased with each of my products and am excited to see them in use in the multidisciplinary clinic. I would love to see how patients react to the materials and would want to update them after getting feedback from patients. I also hope the presentation benefits the students coming after me. I sent out a survey to five clinicians who could potentially use these resources and 100% agreed or strongly agreed that these would be effective and appropriate recommendations and materials to give out to patients with ALS.

Link to survey: https://forms.gle/iWpkr9c6Nktb9dL7A

There are many more points of education that I would have loved to include in this project but did not have time to create such as recommendations on positioning, and energy conservation techniques, but I hope to add to these materials as I continue in my profession. Through my work with the Neuro STEP UP program, and my clinical experiences, I have grown a passion for serving patients with neurodegenerative diseases and I have witnessed how physical therapy can truly preserve quality of life for so many individuals. I am excited to continue this work after graduation and throughout my career!

 

Acknowledgements:

Jessica Cassidy PT, DPT, PhD Thank you for being my primary advisor and for meeting with me throughout the year to help refine my ideas. Thank you for being a great mentor to me through the STEP UP program. I have grown so much under your guidance and look forward to working with you through continued involvement in the program.

Karen McCulloch, PT, PhD, MS, FAPTA, FACSM, NCS Thank you for being a member of my committee and taking the time to look over my products. Your feedback and guidance along the way helped me to improve my presentation and choose which products would be most useful for patients. Thank you (and Vicki) also for giving me all of my foundational neuro education and being the beginning of my interest in the field of neurorehabilitation!

Courtney Matrunick, PT, DPT Thank you for being a member of my committee and an amazing CI. I could not have completed this project without your teaching and clinical expertise.

Amanda Beaty PT, DPT Thank you for allowing me to come shadow at the Duke multidisciplinary clinic and for helping me refine my products. Thank you also for connecting me with Lily and helping me put together the case study!

Bryn Kennedy PT, DPT Thank you for all you have taught me in and outside of clinic and for being an amazing CI. Your feedback and guidance are invaluable, and I am thankful for all of your mentorship!

Thank you to the STEP UP program and members of the VLT for cultivating my interest in neurodegenerative diseases and helping me grow as a clinician. I am so grateful for the opportunities and mentorship the program has provided.

 

References:

1. FYI: Epidemiology of ALS and Suspected Clusters | The ALS Association. Accessed April 28, 2023. https://www.als.org/navigating-als/resources/fyi-epidemiology-als-and-suspected-clusters
2. Dal Bello-Haas V, Florence JM. Therapeutic exercise for people with amyotrophic lateral sclerosis or motor neuron disease. Cochrane Database Syst Rev. 2013;(5):CD005229. doi:10.1002/14651858.CD005229.pub3
3. Kalron A, Mahameed I, Weiss I, et al. Effects of a 12-week combined aerobic and strength training program in ambulatory patients with amyotrophic lateral sclerosis: a randomized controlled trial. J Neurol. 2021;268(5):1857-1866. doi:10.1007/s00415-020-10354-z
4. Bello-Haas VD, Florence JM, Kloos AD, et al. A randomized controlled trial of resistance exercise in individuals with ALS. Neurology. 2007;68(23):2003-2007. doi:10.1212/01.wnl.0000264418.92308.a4
5. HEP2go – Online Home Exercise Program – Rehab – Physical Therapy, Occupational Therapy, Physical therapist, Occupational Therapist, Therapeutic Exercises, HEP. Accessed April 28, 2023. https://www.hep2go.com/
6. Beaty, Amanda, Good, Laura; Exercise in ALS, Duke ALS Clinic, Department of Physical Therapy and Occupational Therapy
7. How to Do Pressure Reliefs (Weight Shifts) | MSKTC. Accessed April 25, 2023. https://msktc.org/sci/factsheets/how-do-pressure-reliefs-weight-shifts
8. Learning diaphragmatic breathing – Harvard Health. Accessed April 24, 2023. https://www.health.harvard.edu/healthbeat/learning-diaphragmatic-breathing
9. Inspiratory muscle training in neuromuscular patients: Assessing the benefi…: EBSCOhost. Accessed April 24, 2023. https://web-s-ebscohost-com.libproxy.lib.unc.edu/ehost/pdfviewer/pdfviewer?vid=0&sid=e8f5fb8f-396f-4963-b415-44d429c522f5%40redis
10. How To Use Your Inspiratory Muscle Trainer – YouTube. Accessed April 25, 2023. https://www.youtube.com/watch?v=rwNfOAhm6LA&t=1s