The ALS Association

ALS Ice Bucket Challenge Progress

Charting Your Respiratory Care Plan

As each individual’s experience with ALS can vary greatly, so too will your decisions regarding respiratory care. Know your multidisciplinary care team or health care provider is prepared to help guide you through the process. These health care professionals can provide recommendations to enhance quality of life as the disease progresses.

If you feel you’re ready to begin shaping your respiratory care plan, these six tips will provide an overview of the steps to take and questions to discuss with your family and health care providers.

1. Know Your ALS Respiratory Basics

ALS will eventually weaken the respiratory muscles, making breathing a more labored process. It’s not uncommon for people with ALS to experience shortness of breath, which can lead to fatigue and anxiety. Headaches, light-headedness, poor sleep and bad dreams are also signs that often present with deteriorating lung function.

“As the disease progresses and the muscles in your arms and legs become weaker, your diaphragm muscle also becomes weaker, and so you’re unable to take as deep a breath as you might previously have been able to take,” explained Cynthia Knoche, RRT, Director, Chapter Care Services for The ALS Association.

This muscle weakness leads to hypoventilation, a state in which the lungs fail to adequately expand. Often the airways at the base of the lungs become plugged with secretions making breathing increasingly difficult.

2. Learn How to Make Lighter Work of Breathing

There are a host of exercises and supportive equipment that help relieve the burden of hypoventilation. For example, techniques such as “breath stacking” taught by a physical or respiratory therapist can help you take a deeper breath and produce a stronger cough.

For some with ALS, lying flat in bed brings discomfort and makes breathing more difficult. Knoche suggests keeping the head elevated whenever possible. Even the size and fit of your power wheelchair can make a significant difference. Consider having your ALS multidisciplinary care team or health care provider check to see if your wheelchair is providing proper support to both your torso and upper limbs.

“Many people choose to utilize the benefits of part-time respiratory support devices,” said Knoche. “For example, bi-level or respiratory assist devices provide increased ventilation typically during the nighttime hours and decrease the work of breathing.” A cough assist machine is also a commonly used apparatus that can help clear the airways and provide relief and should be discussed with your multidisciplinary care team or health care provider.

3. Take Proper Precautions

As lung function begins to decline, preventing the transmission of cold and flu germs is essential. Good hygiene is often the best defense and simplest order of business. Frequent hand washing by caregivers combined with a strict daily oral hygiene routine help prevent the spread of illness that can be devastating to a person with ALS.

Knoche explained, “People cough and clear their throat numerous times during the day without even thinking about it, but when your respiratory system is affected by ALS, we’re not able to take as deep a breath. And we’re not able to cough as effectively as we had previously, so anything that would increase secretions is a problem. That’s why it’s important to touch base with the ALS multidisciplinary team or health care provider about flu or pneumonia shots.”

4. Decide if Invasive Ventilation is Right for You

Eventually the need for additional, round-the-clock respiratory support may be required. Knoche said, “Once someone requires 24-hour respiratory support, a tracheostomy is generally recommended.”

Knoche goes on to explain, “The tracheotomy is a surgical procedure that provides for an opening directly into the windpipe where a tracheostomy tube is placed. Ventilator tubing is then connected to the tracheostomy tube providing 24-hour ventilation support.”

Although a ventilator can prolong life, it does not slow the progression of ALS. The decision to undergo a tracheostomy is quite personal and individuals with ALS should be afforded ample time to weigh all options and discuss their preferences with their family and caregivers.

“Some people evaluate their own idea of quality of life with invasive ventilation and may decide that it’s a challenge they don’t want to deal with, or they don’t want their family to have to deal with,” said Knoche. “One major challenge is the expense associated with hiring a 24-hour attendant or caregiver. However, a significant number of people have goals that they haven’t yet met and decide that they want to take advantage of the benefits of portable, fulltime ventilation. Developing a strategic care plan with their family and health care team can enable them to continue to live at home using tracheostomy ventilation and at the same time, being able to go out in to the community, to the theater, to their children’s ballgame or to other activities that are important to them.”

Ronaldo R. Pelchat of Lancaster, New Hampshire, operates a website documenting his life with ALS and his decision to move forward with a tracheostomy. Pelchat will share his decision process and insights on living life to the fullest with tracheostomy ventilation in an interview to be featured in the November edition of ALS Insight.

5. Know You Can Always Change Your Mind

It’s important to know that planning ahead by developing a strategic health care plan can ease the stress and strain of having to make these decisions when your response time may be extremely limited.

“It’s important to learn what options are available and how the benefits of those options fit into a person’s specific situation and their goals,” said Knoche. “Keep in mind that ultimately each person has the right to initiate, continue, or even discontinue any of the respiratory support options they decide to take advantage of.”

6. Prepare the Proper Legal Paperwork

If you don’t have a plan, a decision could be made for you. To ensure your wishes are followed, especially in the event of an emergency, it’s helpful to have the right legal paperwork in place.

Advance Directive
In the event you are not able to make medical decisions for yourself, advance directive documents can help ensure your wishes regarding important end-of-life decisions are met. The ALS Association offers specific considerations for people with ALS, which can help aid an informed visit with a qualified legal professional.

*Note that in many states, an advance directive can be completed without the aid of an attorney or other legal professional.

Durable Power of Attorney for Health Care
Selecting a power of attorney for health care or a health care proxy is an important step in creating an advance directive. Often a family member or close friend is identified to make critical health care decisions on your behalf should you become unable or deemed incapable of doing so on your own.

*Note that selecting someone to serve as your financial power of attorney is a separate and distinct process.

Additional Resources

The ALS Association in partnership with the Les Turner Foundation and the Forbes Norris MDA/ALS Research and Treatment Center produced a three part video series that will help you 1.) Develop a care plan, 2.) Understand your respiratory support options, and 3.) Consider how your decisions may impact your quality of life.

Special Note: Whenever possible, it is recommended that ALS professionals be present when people and families coping with ALS view the videos so that questions pertaining to the individual’s unique circumstances can be addressed in a timely manner. It is also recommended that ALS professionals consider each person’s stage of progression to gauge the most appropriate time to view each video.

View the videos by visiting:

Powered by Blackbaud
nonprofit software