When I opened my community practice, I visited the coordinator of the palliative care team at the Children’s Hospital of Eastern Ontario (CHEO). I explained the scope of my community clinical practice. I knew CHEO had a strong palliative care team in the hospital but did not have a full-time physician for the team. I offered my services to families who wished to care for their children at home until their death. If there was an interest, the palliative care team coordinator could contact me and send the referral. We both understood that the CHEO team would still be involved in the care of the children if their parents requested. The families would be free to return to CHEO if they wanted. I realized the referrals would be minimal. I explained that I would always accept a referral within the CHEO catchment area. I would be the primary physician for the care in the home, providing twenty-four hours a day, seven days a week coverage.
I was confident the CHEO palliative care team would continue to evolve and expand over the years and my services would no longer be needed. Therefore, I knew my involvement would be limited if needed at all. To be truthful, I did not believe I would have any referrals. But again, I was wrong. From 1996 to 2005, I was asked to provide near-end-of-life care at home for thirty-three children and their families. Most of the patients lived in Ottawa, but as agreed, I accepted referrals from patients throughout the CHEO catchment area, up to 100km outside of Ottawa. Of those referrals, thirty-two children died at home. The only patient who died in hospital was a young teenager whose parents lived in Sudbury, but he had spent most of his last few years in treatment at CHEO. He decided to stay in Ottawa until his death because he felt the most comfortable in Ottawa, especially at CHEO. He knew the staff well and knew they would take good care of him.
From my point of view, I felt it was so important to care for the children when I requested to do so. Young children were just not meant to die. It was emotionally draining, but I felt they all deserved the best care I could provide for them. As with my adult patients and their families, my experiences caring for young children and their parents, taught me more than I taught them. We successfully provided medical care and support for these beautiful and brave children with all caregivers working together as a team.
I learned parents were ferocious advocates for their children – no matter the difficulties! They wanted to know clearly, “How long?” and “What will the process be like?” If the children asked their parents to take them home, they took them home.
And when the parents decided to take a child or a young baby home to care for them until they died, they did NOT change their minds.
The only factor in the care of children simpler than adult care was the absence of the physical aspect of the medical care due to their small stature. The children were light to carry, and parents were used to handling their bowel and bladder issues.
The following three journeys illustrate the major issues of caring for children and supporting their parents while highlighting the insight these young children gave me.
I was called by the CHEO palliative team asking me to see a baby in the neonatal intensive care unit. Elizabeth was born with serious lung and heart problems. Despite the best efforts of the physicians, she was not improving. Her physicians explained to Elizabeth’s parents that her death was imminent. Her parents expressed the desire to take their baby home, wishing to care for her at home until she died. In the ICU, she was constantly monitored for pulse and oxygen levels.
I was present with the ICU nurse, palliative care nurse, and her parents for the final pre-discharge discussion. At one point, her father asked the nurse. “Could I have an oxygen measuring machine at home?” Before he explained why he wanted this equipment, the nurse quickly stated, “You will not need that. There will be no active treatment to try to correct her oxygen if it goes down.” The nurse went out for a few minutes. I looked at her father and quietly asked, “Why do you want the oxygen monitor?” He replied. “I am legally blind. I cannot tell if my baby is ‘blue’. If she lives longer than a few days, I will not be able to stay awake and hold her all the time. I know I will need to sleep. I want the monitor to alarm if her oxygen is low. That way, I will know to hold her, so she won’t die alone.” I leaned over to him and whispered, “Don’t worry, once your baby is home, I will ask the oxygen company to leave an oximeter in the home for you and your wife.” The oximeter was set up within hours of Elizabeth’s discharge home. Elizabeth died a few days later in her parents’ arms.
At that moment, I thought, “Wouldn’t it be great if all families could have a small, simple oxygen measuring machine in the home, not for active treatment, but as a warning sign, to help predict an impending crisis, and find ways to address the issues before a significant problem?” But at that time, these oximeters were just too expensive to have available for each patient and too heavy for me to carry from home to home.
Jacob was eleven years old when, with his mouth full of blood clots, he asked his mother, “Please take me home!”
Jacob had been diagnosed at five years old with a sarcoma in his leg. He underwent very aggressive radiation and chemotherapy treatments and made a full recovery. Five years later, he developed leukemia caused by his previous radiation. He was again given treatment, but it was no longer effective. He was anemic and actively bleeding.
His parents were committed to taking him home to die. They expected it would be for just a few days. The decision was made abruptly, and resources were set up very quickly. I was asked to participate in the care. I was told the family was coping very well and had exceptional help in the home for the care. His parents expected my only role would be to confirm his death. I agreed to be involved in the care, but with the rapidity of the progression and his symptoms, I felt I needed to assess Justin before his death, not just confirm his death. I was apprehensive about a crisis leading to distress for Jacob and his parents. The decision to take Jacob home was rushed. If Justin’s comfort could not be maintained his parents could easily feel guilty and second guess their decision for a home death.
When I arrived, Jacob’s parents reassured me they were comfortable with their decision to be at home and had the care resources in place. I quietly went into my observer mode. Jacob was in the main bedroom, in bed between both his parents. He had been transferred home only a few hours before my visit. His uncle, a family physician was helping with the care. A nurse, from CHEO, who had agreed to accept shifts in the home was present (Jacob was well known and loved by the staff at CHEO).
The parents seemed very much at peace with their decision. I sensed they felt his death was imminent, perhaps a few hours to a few days. The nurse and physician appeared very worried and apprehensive – not sure exactly what to do.
I then directed my observations to Jacob and immediately understood their concerns. Jacob was lying very quietly with deep breathing for a few minutes. Then, his breathing became more and more shallow, and then stopped for about 45 seconds. I could see his mother touching him gently. It appeared that this touch stimulated him to restart breathing. With that effort, he suddenly shot up the sitting position, opened his eyes for a few seconds, then fell back down, calm, apparently sleeping, with a return to the deep regular breathing pattern. This sequential pattern of breathing happened repeatedly.
I asked Jacob’s parents, “Could I explain what is happening to Jacob with this breathing pattern?” They agreed, and I could sense everyone seemed a bit calmer.
I explained this pattern of breathing is called Cheyne Stokes breathing. “You observe a period when Jacob is breathing deeply – he appears comfortable and sleeping. Then the breathing becomes more and more shallow until he stops. This non-breathing interval seems like an eternity, but his colour remains pink. I see you gently touching him as if to stimulate him to start breathing again. In reality, Jacob’s Brainstem (i.e., the lower part of the brain controlling all essential body functions) senses he is not breathing through the rise of carbon dioxide. It automatically sends an emergency signal to his lungs to restart breathing. He suddenly takes a deep breath, then another and another, profoundly and regularly, until the carbon dioxide returns to normal. The stimulus to breathe subsides. His breathing slows until it stops again. In Jacob’s case, when the signal to restart breathing is sent, some impulses reach other parts of the brain and tell it to wake up as well. That is why Jacob sits up.”
“This type of breathing is very significant, but not life-threatening. Jacob’s brainstem continues to be vigilant and in control. Its aim is to save energy. First, it achieves this by making Jacob sleep and not eat. He gets a little fluid from the intravenous, so he does not need to drink. The breathing pattern you are observing is the brainstem taking away some of the energy usually allotted to breathing. It decides to stop breathing until the carbon dioxide reaches a critical level, the oxygen level is still high enough for survival. Then, it restarts the breathing for ten to twenty breaths until the carbon dioxide in the body returns to normal, then his breathing stops again. Energy is saved in the non-breathing phase. The brainstem does not wish Jacob to wake and sit up because that process is a waste of energy, but it does not know how to stop that. Jacob is such a fighter! When he is stimulated, he tries to awaken and help the process.”
“We need to preserve the signal Jacob receives to restart breathing but calm the stimulus that awakens him. Before my visit, I reviewed his medication regime, and I have an idea that will likely make a significant difference to his ongoing comfort. If you agree, we will give Jacob a small dose of Phenobarbital via his intravenous line. This medication acts as a sedative and has anticonvulsant properties that will calm the irritability of the brain. It will take about twenty minutes to be effective and last for twelve hours. I will stay and we will quietly wait to observe if the medication has a beneficial effect.”
The change in Jacob’s comfort was dramatic. After fifteen minutes Jacob sat up for a shorter time; then he did not sit up, but just awakened and moved his arms a little; then finally, he slept through the initiation of his breathing.
A calmness settled in the room because Jacob looked so comfortable. It was clear that the family felt they made the right decision to bring him home. From my point of view, I knew now we would be able to keep Jacob comfortable until his death.
I did notice that his parents still touched him when they could no longer stand to see him not breathing. It is so hard to believe someone who is not taking a breath for that long is not dying. Their hearts felt perhaps he needed just a little stimulation to restart breathing.
Jacob stabilized over the next few days, surprising everyone. He slept peacefully and needed the Phenobarbital regularly only at bedtime. He was calm enough, allowing the nurses to carefully do his personal care and even empty his mouth of clotted blood without causing him any distress. He awoke for a short period every few days.
About a week later, I opened the door, and while I was still in the hallway, I heard Jacob’s breathing. His pattern had changed to a deep regular rhythm. I knew the change in his breathing meant he had progressed in his near-end-of-life journey toward death. But the family interpreted this change in breathing very differently. They thought it was an improvement because he no longer had the long periods of no breathing. Jacob’s aunt came to greet me and stated the family had decided to take him back to the hospital for further treatment.
As I came into the bedroom, I saw Jacob beside his parents. Although his breathing was deep and regular with no pauses, he appeared very calm and comfortable. I asked his mother what had made her change her mind and decide to transfer Jacob back to the hospital. She answered. “We expected him to die within a few days, it has been a week, and he is still alive. His breathing is now laboured; he is trying very hard to live. I think he is asking us to take him back to the hospital for more treatment.”
I stated quietly, “I see that Jacob appears comfortable just now. Do you think we can take a moment of quiet reflection before we make any decision? I would like you to go back to Jacob’s initial diagnosis and take me through his journey. She agreed and summarized the treatments Jacob had experienced, some of which were terrifying for him when he needed to stay in isolation without his parents. I asked, “I heard Jacob had awakened a few times during this last week. Did he speak? If yes, what did he say?” She answered, “Yes, he awakened three times. The first time, he asked to see his brother. The second time, he asked to listen to his favourite music. The third time I was not present.” His aunt explained that she was present and reported, “He asked me to bring his suit. I did not understand what he meant really. I thought he might be cold and needed warmer pants.”
Immediately, his mother said, “I have changed my mind. I know what Jacob meant when asking for a suit. He does not wish to be transferred to the hospital. He knows he will die soon. He needs to stay at home with us.” I asked, “What did his statement mean to you?” She explained, “Jacob is a very fashionable child. Whenever there is a special occasion, he loves to wear a suit and tie. By his statement, he is telling us he will soon have a funeral and will be needing his suit for the special occasion.”
I then explained my scientific interpretation of the change in Jacob’s breathing to the family to confirm his mother’s intuition. “This new breathing pattern is called Kussmaul breathing. It signifies Jacob’s kidneys are failing. They can no longer balance the body’s internal environment, leading to a life-threatening elevation of acid in the body. The brainstem acts, stopping its quest to preserve. To preserve life, it prioritizes all the energy it has left to remove this acid. It recruits the lungs to increase the breathing rate and depth to blow off the acid as carbon dioxide through expiration. But the deep regular breathing pattern is an emergency response only, a last desperate effort to survive. Once the limited energy is used up over the following hours, Jacob’s breathing will become more spaced out, more shallow. The carbon dioxide will increase again, leading to loss of consciousness, and Jacob will die very quietly.” Jacob’s mother understood that the science proved her intuition for Jacob’s behaviour. Jacob died within twenty-four hours with his family beside him as he had requested. He wore his suit for the funeral as he had asked.
This experience made me realize just how important it is for families to be aware of the significance of the changes in breathing at near-end-of-life. For me, these changes were a comfort, an objective sign that helped me predict the timeline of the progression to death. For patients and families, who often saw these changes before anyone else, usually at night, the signs are very distressing and interpreted as, “My loved one is in crisis and very uncomfortable.” Panic takes over and often calls are made to 911.
After my experience with Jacob and with reflection on similar experiences with many of my patients, I realized I needed to explain these changes before they happened so that families would understand the science and the significance of the changes and participate as equal partners. In that way, the panicky calls for advice would be to me rather than to 911, followed by a needless trip to the hospital. My solution was to add the description and significance of the various breathing patterns at near-end-of-life to the back of my teaching card, outlining the natural process of the body shutting down. I left this updated card in every home if my patients and families asked, “What will this process be like?”
When I received a referral to care for young children, my interactions were mainly with their parents. If the parents talked with me in-depth and trusted me, the children automatically trusted me. The most difficult challenges were when the children could speak independently, but their parents did not permit me to discuss their understanding of the illness and the journey toward death. I understood that the parents were very protective of their children, but it often led to challenging situations. The following journey demonstrates some of these challenges.
Karl, eleven years old, had been diagnosed with a sarcoma with metastases to his bones and throughout his body. He had undergone many treatments, both radiation and chemotherapy, but the disease had continued to progress, and all active treatment for cure had stopped. Kari was referred to me for ‘two’ reasons: to prescribe his pain medication and to confirm his death.
Karl’s pain was controlled but only with high doses of Morphine which could not be administered orally as he progressed further. His family wanted to care for him at home. He lived about an hour’s drive outside of Ottawa. I was told by the referring palliative care team member that Karl did not know he was dying, and the family wanted to keep him from knowing – they did not want him to be frightened.
The palliative team decided to start him on a Morphine pump attached through an intravenous port for easy and accurate administration until he died. But he lived too far out of town for his family physician to visit, and the palliative home care nurses supported by the CHEO palliative nurses needed a physician to write the orders for the infusion of Morphine. Once prescribed, the nurses would take care of the logistics of setting up the Morphine pump.
I explained that I would accept caring for Karl, but the care could not be as limited as they described. If I was to write orders for Karl, I needed to know his history, talk with him and his family, and assess his needs. The only way I could do so effectively was to make at least one home visit before writing any orders. It would be too dangerous for Karl’s well-being to do otherwise. The nurses talked with the family, who reluctantly agreed that I could visit, but I was NOT to speak to him in any way about death. I agreed. I would not bring up any reason for my visit other than symptom control unless Karl asked me specific questions on different topics.
The visit was set up. When I arrived, the CHEO palliative nurse was present. She met me outdoors and confirmed again that I would NOT bring up the subject of death and dying.
I was to say I was the physician helping set up the Morphine pump and write the orders. Karl’s mother whispered in my ear as I entered the home, warning me NOT to talk about dying. I reassured her that I would not unless Karl brought up the subject.
I met with Karl, his mother, and the nurse. The nurse introduced me to Karl, and before she could add that I was here to help with the pump, he looked at me and smiled, saying, “Hi, you are the doctor who will help me die at home. Thanks for coming to help!”
His mother and the nurse were shocked. The tension in the room immediately dissipated. There were no more taboo subjects throughout the rest of his journey. As it turned out, the family was very competent in meeting the needs of their son. Karl understood the reality of his situation very clearly. I did not need to visit again until Karl died very comfortably at home as he wished.
In preparation for the evolution of pediatric palliative care, the CHEO team was expanded to incorporate three physicians: a pediatric anesthetist (the leader of the team); a pediatrician, and myself. I initially helped teach the logistics of palliative care in the community and helped with the on-call coverage at homes and the hospice. As expected, my role became less essential as the team gained experience and ended when the team expanded further
In 2005 Roger Neilson, a former coach for the Ottawa Senators Hockey team, died of leukemia. Roger had been involved extensively in volunteer work with young children with leukemia in the Ottawa area. In memory of their coach, the Ottawa Senator Foundation decided to work with the Ottawa community to build a residential hospice for children near the end of life, the Roger Neilson House, attached to CHEO. The hospice opened its doors in 2006.
