During my years practicing and teaching as a Family Physician, I knew intuitively that there was a ‘difference’ in caring for patients with cancer and non-cancer diagnoses. During my clinical work at the Ottawa Regional Palliative Unit and then as a physician providing uniquely near-end-of-life care in the community, I became more aware of the subtle and often, not so subtle differences. In my community near-end-of-life practice my experiences caring for a number of patients with neurodegenerative diseases, mainly Amyotrophic Lateral Sclerosis (ALS), brought these differences into focus.
A straightforward way to understand why there is such a difference between cancer and non-cancer diagnoses is to visualize the triangle of the hierarchy of the body and understand that the body is a sophisticated survival organism. It is programmed to have each essential organ run smoothly to continue to live as well as possible even as it is running out of energy.
Then visualize the cancer as a ‘monster’, that just wants to grow and grow, using all the body’s energy randomly for itself without following any of the body’s rules for survival. Cancers present two major problems for the body. First, the body needs to use energy to try to control and kill these rogue cells. Second, when the brainstem can no longer control the cancer growth, the cells multiply and spread rapidly consuming all the body’s energy needed for survival, thus hastening its path toward death.
Patients who have non-cancer diagnoses, remain under the control of the brainstem regulating the function of all the essential organs of the body until destabilization. The brainstem does not allow any wasteful use of the energy needed for survival. I will explain the progression toward death of patients with non-cancer diagnoses by describing the journey of patients with ALS.
ALS is a subgroup of neurodegenerative disease. The term ‘neurodegenerative’ implies that the patient has progressing weakness of the nerves, including those present in the brain, spinal cord and/or limbs. Patients with ALS initially progress very slowly. The patients may have the illness for a few years before being diagnosed and another few years before becoming functionally impaired. The essential organs for the survival of the body are not affected until very late in the progression of these illnesses.
However, when patients reach the final phase of their illness, the ominous signs indicating that ‘death is imminent’ occur very late leaving little time to prepare for the impending death. Most of these patients do not fit the criteria for palliative care services until very close to their deaths. Often, they die before services can be set up.
When I started my community practice, I had no experience caring for patients with ALS at near-end-of-life. As a family physician, I did have experience caring for patients with other neurodegenerative disease, suffering from strokes or Parkinson’s. While caring for patients diagnosed with ALS and their families, I was taken down a path of tremendous learning, not only for these patients but for the future care of all my patients with non-cancer diagnoses at near-end-of-life.
Greg was a middle-aged man, a teacher, who presented with severe respiratory distress, arriving at the hospital unconscious without a “Do Not Resuscitate” order. The team assessed he would die without internal ventilation assistance. After a tracheotomy and insertion of a breathing tube, he was started on a ventilator.
Greg recovered, but the physicians were unable to wean him off the ventilator. He was too well to stay in the hospital, but there was no expertise to care for him in a long-term care facility. Greg wanted to go home. His wife was prepared to care for him at home with home care resources, but the family needed a link with a physician. I was first asked to care for Greg at home, not because of my expertise, but because the palliative care team at the Ottawa hospital could not find a family physician to provide the care.
I understood why a family physician would be worried about providing the care at home. No family physician practicing in the Ottawa area close to his home had had the experience managing a patient on a ventilator in the hospital, much less being the primary medical decision-maker at home. To be clear, I did not have the experience either, but I had been visiting patients at home and providing twenty-four hours a day coverage for the last few years. As well, in Malawi, I had had experience with the inherent stress of caring for very sick patients by myself. At least in Ottawa, if a severe problem arose with the patient or the ventilator that I could not settle, I could easily call 911 for help.
If I refused to care for Greg, he would not be able to go home. If I agreed to care for him, at least he had an excellent chance to stay at home for a few weeks or months. Therefore, I agreed to take over his care.
My first visit to Greg’s home was not quite what I expected. I took my usual observer role. Greg appeared comfortable and all his equipment was running well. His wife explained the history of his illness. She clearly understood the severity of the illness and realized there would be no further active treatment. Greg had a feeding tube and was tolerating the feeds well. The couple had two adult children who visited frequently. Greg was pleased with being at home and enjoying his interactions with his children. His wife appeared very competent and understood the ventilator’s workings and the feeding tube. There was a backup ventilator in the home that was easy to access if there were problems with the machine in use.
Greg was present throughout the interview. He listened carefully and appeared to understand all the nuances of the conversation. He still had strength in his hands and communicated through writing. When I asked him what concerned him the most and if he had questions for me, he handed me a written note he had prepared for me. He wrote that he was furious. Physicians had attached him to this ‘machine’ without his permission! He was now ‘stuck’ with this situation. He wanted to be sure I made all the decisions with him and his wife. No medical choices were to be made without his input.
Greg lived for several months at a very stable functional level. There were no issues with the ventilator or the feeding tube during that time. Greg had no new symptoms. Then slowly, during the next month, he began to weaken, unable to write efficiently, more often asleep and asking for fewer feeds. His vital signs remained stable.
During one visit, with Greg awake and listening, his wife asked me about the logistics of the ventilator with Greg’s further deterioration, “Would there be a time to stop the ventilation, to remove the ventilator? How would we do that? What would be the symptoms?” Greg became very angry, and fiercely wrote, “I never want this ventilator to be removed! Do not let my wife stop the ventilation!” For a patient who had been so angry with being attached to the ventilator, I was surprised he did NOT wish the ventilator to be removed for ANY reason. His wife had not been contemplating taking any action. She was essentially asking the questions “How long?” and “What will the process be like?” We reassured Greg that no decision would be made in the future unless he was part of the discussion.
In Ottawa, starting in early 2000 and culminating in 2005 with the incorporation of the Rehabilitation Centre into the Ottawa Hospital, a greater understanding emerged of the needs of patients diagnosed with degenerative illness. In parallel, there was more recognition of the need for rehabilitation of patients with strokes and severe accidents, etc., affecting the spinal cord and the functioning of the limbs.
Outpatient clinics were set up for each category, including one clinic for patients with ALS. The ALS team consisted of several internal medicine physicians, nurses, physiotherapists, occupational therapists, social workers, dietitians, and respiratory therapists. The team assessed all the patients and offered interventions to alleviate symptoms and prevent crises, to improve the quality of care and the quality of their lives.
The ALS clinic was very successful in improving the care of patients. This clinic was enhanced with the creation and evolution of the ALS Society – a volunteer organization formed to help support patients and families with ALS. The ALS society provided psychological and educational support. They also developed an equipment ‘cupboard’ to provide equipment at no cost, e.g., electric wheelchairs, transfer equipment, external breathing support equipment, etc., to help with the day-to-day functional needs of the patients.
In my mind, I thought, “This could be a complicated discussion when Greg was very close to dying.” But the discussion was not for this moment in time. Some signs were signifying a clue that Greg was weakening, but he did not demonstrate any signs of destabilization.
A week later, his wife called me to report that Greg was very weak, not responding much to any stimulus since the morning. He had had a typical week. There had not been an obvious trigger. I visited quickly.
As soon as I observed Greg I knew he had a few minutes to a few hours to live. The ventilator was working well, but Greg made no effort to assist the breathing as I had observed before. His colour was very pale, bluish. His pulse was very weak and irregular. He was in no distress. I knew I had a little time to explain the changes and discuss the issues. I sat quietly close to Greg with his wife, and we observed his breathing while waiting until his two children arrived. Then we all took a quiet moment of reflection and talked realistically about what was happening. I let the family describe the last few weeks and what they had observed. We talked about his wishes NOT to stop the ventilator –how that was so important to him.
I then explained what I observed. The ventilator was still working, bringing oxygen to his lungs, but his heart was not working. His pulse was just barely present. Despite the oxygen going into his lungs, his colour demonstrated that the blood vessels could not bring oxygen to all parts of his body, including his brain, especially that part of the brain directing all the essential functions of his body. I stated, essentially, he had already died – not of his breathing but of the failure of his heart and brainstem. I explained the ventilator had done its work and had kept his breathing working well, but the disease had been quietly progressing, in the background, into his heart and brainstem and had caused his death.
I was sure that Greg would not be distressed if I removed the ventilator. He would not react. He would demonstrate to us that he had indeed died. I let the family digest what I was saying for a few minutes. No one had questions, so I asked everyone, “Do you wish me to remove the ventilator? Take your time. Discuss if you want. I will not remove the machine until you all agree.”
They all understood what had happened and without further discussion asked me to remove the ventilator. Greg did not respond to the machine being turned off, nor to the removal of the tracheostomy tube. He remained calm, with no signs of any effort. To his family, he looked at peace, as they remembered him.
Differences in Approach to Care for Patients with ALS
From Clues to Destabilization to Death:
During my first experience caring for a patient with ALS at home, I had a glimpse of how quickly a patient with ALS can pass from a stable condition to the destabilization to death. During later experiences I learned just how frequent rapid deterioration occurs.
Complexities of Decisions at Near-End-Of-Life:
Discussions about “Do Not Resuscitate” and the cessation of active treatment when patients are still stable and comfortable, often lead to decisions made with the ‘thinking brain,’ using logic. Patients often state, “I am making these decisions because I do not wish to be a burden to my family, I do not wish to be dependent on anyone.” But when faced with a serious premonition though still lucid, they often make very different decisions, using ‘their heart’.
Caregivers must be very aware of this possibility and allow patients facing death the time and space to reassess their previous decisions.
Benefits and Harm of Mechanical Assistance:
Equipment used to assist essential organ functions assists the body to maintain internal equilibrium. We must have the humility to understand it is the body that re-establishes its internal balance. The body benefits from the assistance of artificial methods temporarily. As the body continues to weaken and has no reserve energy left, these techniques can cause more harm than help, destabilizing the body, and leading to discomfort and imbalance, for example:
Assistive devices are effective at the appropriate time for the proper symptom, but slowing down the interventions, and eventually stopping them is not hastening death. It allows the body to continue to balance its internal environment in its most efficient manner. And, at times, the stopping of the intervention allows the body to use its tiny reserve of energy more efficiently enabling the patient to live longer and more comfortably.
A few months after Greg’s death, I was contacted by a member of the ALS Society. She knew I was providing palliative care in the community and had cared for several patients with ALS and kept them at home comfortably until their death. She also knew that I could care for French-speaking patients. She stated that the ALS Society was hosting a conference for French-speaking patients and families in the Ottawa area who were dealing with ALS. She asked if I could participate as a member of a panel discussion. Although I explained I was not an expert in caring for patients with ALS, she persisted, stating they could find no other physician in town. She reassured me that I would be part of a six-member panel including a physiotherapist, occupational therapist, social worker and case manager. I merely had to introduce myself and answer a few questions from the participants. Eventually, I agreed to participate.
I do not remember much of the discussion during that one-hour panel, but I had very vivid memories of the audience and the questions they asked. Patients were in wheelchairs with their families beside them in the room. When the session was opened for questions from the audience, suddenly all the wheelchairs and family members moved toward my direction, and every question and all comments seemed to be directed toward me! Most of the patients and families were very upset with physician care in the community and the lack of palliative care resources. The recurring theme was, “When we are diagnosed with ALS, we are palliative from the first day! We know there is no cure. Why are we denied palliative care until the very end of our illness?”
I do not remember how I answered! Still, I must have said something that resonated with the participants because I suddenly started to receive more referrals for patients with ALS, directed to me by the ALS society.
At that time, my criteria for referral still included an expected three-month prognosis. But I began to question myself and wonder seriously about what the participants at the conference had told me. By sticking to this one criterion, I would not be able to understand some of the patients’ and families’ needs before the crises occurred.
This concept was brought in sharp focus for me about one month after the conference. I was contacted by a home care case manager who asked me to visit a patient with ALS urgently, on the same day if possible, stating that profound changes had occurred in the last few days. The patient and family wished to stay at home and had no link with a physician. I visited by the afternoon. An executive member of the ALS Society was at the patient’s home by coincidence, trying to support the family through a respiratory crisis. On my arrival, I introduced myself and started my assessment. I noticed the patient’s breathing was changing, becoming much slower, with less effort. He knew I was present. He looked at me, recognized that I was a physician, then calmed without the need for any medication and, within a few minutes, died with his family beside him.
After his death, the ALS Society member and I stayed to support the family and then we stopped to talk outside for a few minutes about what had just happened. Everyone had done the best they could. The referral was requested when the first severe respiratory symptoms occurred. The case manager verbally contacted me to explain the urgency of the problem, and I was able to visit within a few hours. But the changes had occurred so quickly. The patient and family did not have the support they needed weeks before the crisis.
During that discussion, I decided to change my referral criteria for patients with ALS and all the patients with neurodegenerative illnesses. I would now accept referrals as soon as the patients and families were comfortable discussing death and dying issues, including the review of Do Not Resuscitate, and expressed the wish to stay at home or in a residential hospice if care at home became impossible. The ALS Society decided to incorporate a session introducing near-end-of-life issues in their monthly teaching sessions with patients and families.
With these changes, from 2000 to 2010, I cared for 35 patients with ALS. Of these patients: 30 died at home; two at a residential hospice; two at the Elisabeth Bruyere Palliative care unit. Only one patient was transferred to an active hospital – a young mother who needed a little time to decide if she wished to be on a ventilator.
