NOTE: I wrote to Dr. Oppenheimer to get permission for an article that he had published, I found his response very interesting and hence will add it all to this page.
Dear Mr. George Goodwin,
Thank you for your email.
Yes, you can copy those comments to your web site.
I am very understanding when people with ALS, who have been encouraged and properly informed (and provided with the needed resources too) decide that they prefer to avoid prolong their life using assisted ventilation; I feel that these people often do not get the intensive palliative care and symptom-relief that they need.
However I want to encourage people with ALS to strongly consider living with ALS as a disability and realize that it need not be a fatal disease if fully treated. Many PALS today do use noninvasive ventilation. Few know how to use assisted cough techniques to help make this work better and longer. Many are not getting optimal use from their noninvasive ventilation due to poor experience and follow-up. And, very few consider going on to use tracheostomy ventilation which can provide many many years of survival after the onset of respiratory muscle weakness. Too often physicians discourage the use of tracheostomy, or it is difficult for the PALS and family to arrange the needed resources without excessive burden on family members. My patients with ALS who have used tracheostomy ventilation continue to do well until another disease cuts short their life (just like any one else without ALS). The challenge, of course, is continuing to live and stay active socially so that life is worthwhile! There are many PALS using tracheostomy ventilation who can provide assistance to others -- networking to trade experience on how to not only manage from day to day, but to thrive.
My first patient with ALS (Jack) had respiratory muscle weakness as the first evidence of ALS in 1975. He elected to have a tracheostomy and lived quite happily at home with his wife, Shirley, and his children until 1992 when he had a sudden heart attack and died. He also was lucky to have a great family, good resources from work benefits and helping organizations (although they were of modest means economically), and he too was able to eat, swallow and talk -- there was little significant bulbar impairment (which is unusual after years of ALS). Jack, Shirley and I certainly learned a lot together over the years.
The latest ALS Digest #1187 has a reply I sent (item 3) that may be of interest too regarding tracheostomy.
Cheryl New, who wrote the great piece about ALS is a disability in the MDA newsletter, recently had a tracheostomy. She had a tough time in the ICU but is now home learning how to live again. She sent an email that discussed her experience and suggested that tracheostomy is not very glamourous... I replied as follows:
Warm best wishes,
Edward Anthony Oppenheimer, MD, FCCP
Los Angeles, California
THE ARTICLE THAT I HAD ASKED PERMISSION TO PUBLISH:
Date : 30 Jun 2003
From: "Edward Anthony Oppenheimer, MD" email@example.com
Dear Zbigniew Skotniczny,
I received your email about tracheostomy in Poland, for friend person with ALS. I have written some general comments below that may be of help. Only physicians who have actually examined him, and know all the details of his history and tests, can give him reliable personal
medical advice. Email comments must be of a more general nature.
When respiratory muscle weakness occurs in ALS, the person with ALS should have decided in advance whether to let life end, and use palliative care to relieve discomfort, or to continue living using mechanical ventilation. If the bulbar involvement due to ALS is mild or absent, then noninvasive positive pressure ventilation (NPPV) is usually used as long as it works and is comfortable. If bulbar impairment is more severe, or if NPPV is not tolerated, then some people with ALS decide to use tracheostomy positive pressure ventilation (TPPV). Tracheostomy is usually more comfortable than using a mask for noninvasive mechanical ventilation. There are
additional skills needed however, that family and other caregivers must learn, so as to take care of the tracheostomy and equipment properly and to avoid infection or injury to the tracheostomy opening (stoma) or the delicate inner lining of the trachea.
Experience with TPPV for people with ALS is that it provides the most secure and safe method of long-term ventilation (long-term survival), but of course it does not stop the progression of ALS. People with ALS who use TPPV usually develop quadriplegia (inability to move their
arms or legs) and require a feeding tube for nutrition (PEG). This disability usually requires a trained caregiver to assist the person with ALS 24 hours/day seven days/week (family, friends and paid assistants). Some are able to talk, but others lose the ability to talk due to bulbar impairment and should have help with a good alternative communication system. Many people with ALS have continued an active social life, adjusting to their disability. In my experience those who are most successful have a positive attitude, inner strength, high motivation and good support from family and friends. Resources are needed for the equipment and the caregivers -- this is easier for some people to arrange, but for others it is difficult and can become a burden for family members if there are not enough other caregivers to help. I would expect that a person who is 39 years old
certainly still has a lot to live for, and with good care and resources, should be able to be at home with his family using TPPV.
The person with ALS, family members and other caregivers need to be carefully trained as to the skills needed to provide safe care in the home. This usually requires 2-3 weeks, or more, of training. Some people with ALS are not able to go home because the family cannot do the care, and find themselves in a long-term care institution (I do not feel that this is the ideal option).
In general I advise finding and training paid helpers to assist the family with the care. Usually personal attendants can be trained to do this properly, and do other non-medical tasks, to help, in the home too. I do not recommend using paid nurses because they are so expensive, they are in short supply, and usually they will not help with housework, transportation and other needed assistance.
People with ALS using TPPV can live 5-15 or more years if they have good care at home, and have help from doctors and other healthcare professionals who are experienced and have a positive approach to ALS care. People with ALS using TPPV at home, who I have had as patients, have usually been very satisfied with the quality of their life, and they say that if they had the opportunity to do it again (or decline) that they would do it again. When people with ALS, in my experience, have not done well on TPPV this is usually due to poor care, inadequate resources, a dysfunctional family, or another illness that has caused complications or death. With good care ALS is not fatal,
but it is very disabling.
In the USA people have the legal and ethical right to refuse using mechanical ventilation, or to stop using it, if they truly understand the pros and cons and consequences of not using this (even though
death would occur if it is not used or if it is not continued). Medications should be given to relieve distress if a person is unable to breathe themselves and refuses mechanical ventilation or decides to
Since about 10-12% of people with ALS who use TPPV eventually become totally locked-in (unable to communicate in any way), I usually advise that they complete an advance directive (this is a legal document in the USA) for use by their family and doctors, that says that life-sustaining treatment should be stopped if permanent coma or the totally locked-in state develops. Each person should have the right to say when "enough is enough", and receive support and symptom-relieving
medications when only palliative care is desired. Hospices have been very helpful to many people with ALS, and their families, when palliative care is desired rather than long-term survival.
Very helpful information about ALS care and TPPV is in print (books, manuals, articles), and good material can be found on the Internet. Many people with ALS (and family caregivers) stay in touch with each other (networking) using email, by joining ALS email discussion groups (such as the ALS Digest and Living-with-ALS), and by using the Internet. Some of the information in print and on the Internet regarding TPPV for people with other neuromuscular diseases (such as post-polio), is helpful for the care of people with ALS too.
Each person is truly unique in their circumstances, their other medical conditions, values and preferences, family situation, economic and community resources, etc.. Thus there is no "best" answer that is right for every person. Each person and family must work through these issues with advice and support from doctors and friends, and then decide what is best. Some people with ALS definitely have found more meaning and intensity in the quality of their lives (then before they
had ALS) as they deal with this disability, and they find ways to continue their lives, their interests, ...to indeed thrive. It is a challenge.
With all best wishes,
Edward Anthony Oppenheimer, MD, FCCP
Los Angeles, California
"It is more important to know where you are going than to get there quickly."
© ALS Independence 2003-12