HomePEN at the End of Life

Lyn Howard, MB, FRCP, FACP, Medical & Research Director, The Oley Foundation

Editorial Comment: From time to time we receive requests for our thoughts on the subject of death, dying and the termination of nutritional support. In response to one such request, we asked Dr. Lyn Howard, who has spent a long and distinguished career caring for patients on nutritional support, to write about her experience with this issue. We at Oley have avoided publishing information on this most difficult topic for obvious reasons. Even people in good health avoid it, and we in the HomePEN community who expend extraordinary effort to survive have more reason for not wanting to dwell on death and its particulars. Recognizing this is a difficult and controversial issue, we sent this article to a number of longterm consumers for review and comment before going to press. We thank everyone involved for sharing their opinions freely. Oley does not intend to take a position on this subject, but will remain a neutral conduit for the exchange of information. If this exchange is to produce meaningful results we must hear from you, our readers, on our handling of this topic as well as comments on the paper itself. We hope you find the article helpful.

Recently Joan Bishop, executive director of the Oley Foundation, asked me to share some thoughts about dying while on home parenteral and enteral nutrition (HPEN). I think Joan saw that I'm getting close to the end of my long career supporting persons and families on HPEN and felt I should tackle this difficult topic before I topple over! It's not an easy topic since we traditionally spend our days defeating death and promoting a full and active life. But it's true all of us die at some point and I think there are special issues for persons on HPEN to consider.

HPEN Not a Likely Cause of Death
First a few facts; who dies on HPEN and what is death due to? The chief factor determining mortality is the underlying diagnosis. HPEN is not so much a disease treatment as a treatment of a disease complication - intestinal failure. The disease itself may stabilize, as in many individuals with short bowel due to Crohn's disease, or it may progress, as in unresectable cancer. Table 1 lists the primary diagnoses where HPN is commonly used. Table 2 gives percent survival at 1, 3 and 5 years for persons who stay on parenteral therapy. Because table 2 largely reflects adult outcome,I have been given permission to share figure 1, the unpublished outcome data in 302 children treated in France on PN. The majority of HEN users have a swallowing disorder, commonly associated with a stroke or cancer in adults, and cerebral palsy in children. Seven percent of HEN users have an intestinal absorption disorder. Most of these individuals started out on HPN and progress to HEN, their survival at 1 year is 99%.

Table 2 survival percentages do not include persons who graduate off HPN recovering full intestinal autonomy. As shown in table 3, a large percentage of persons starting HPN and HEN do indeed recover and resume oral nutrition. This is true for 75% of HPN short bowel (<150 cms.) patients with non-malignant diagnoses. Recovery, if it occurs, is almost always in the first two years of therapy in adults, but in children recovery may take much longer - probably because children have much higher energy requirements (60 - 80 kcals/kg/d compared to 25-35 kcals/kg/d for adults). As children develop they continue to grow their bowel and eventually reach nutritional autonomy once their high growth demands subside. "Permanent" HPEN dependence is about one in eight persons who starts therapy in the USA, currently about 4000-6000 persons. These individuals are the backbone of the Oley Family.

Short-term users of HPEN rarely die of an HPEN therapy complication (1%). Death, if it occurs, is almost always due to the primary disease, occasionally to some other medical illness.

Sepsis Rarely Fatal
Long-term HPN users, after many years on therapy, may die from an HPN complication. Over several decades this adds up to 15-20% of all deaths in HPN subjects. Even in this setting, 80-85% of deaths are due to the primary disease or another medical illness. If an HPN-related death does occur, it is most frequently due to sepsis, occasionally to liver failure, venous thrombosis or some other metabolic disorder. It should be emphasized that while sepsis is the cause of 70% of deaths due to HPN, overall sepsis is rarely fatal. In the first few years on HPN, the average adult has a septic event once every 2 years, and the average child has a septic event once a year. Studies show that septic complications are less common in long-term survivors, suggesting a learning curve during which veteran HPN users get better at handling their catheters in a consistent aseptic manner.

HEN therapy results in fewer therapy complications, leading to only one-third of the HPN re-hospitalization rate. Mortality over decades has not been studied in this population. These statistics point to the fact that overall HPEN is a remarkably safe therapy.

Small bowel transplantation is not considered in HEN users at this point. It is potentially life saving for only a small percentage of long- term HPN users.

Planning Ahead
If death is approaching and cannot be checked, certain issues arise with HPEN that are best planned for early on. Artificial nutrition, like artificial respiration, may thwart an untimely death, but can prolong a natural death. Most of us, in my experience, want to die peacefully and without undue suffering when our time has come; we rarely want to prolong the dying process or worst still, survive when we are permanently unable to relate to our family and friends. In New York State, and many other states, only an advanced directive, written and signed by the patient allows us to retain some control over our dying process. Only if we havedesignated a health care proxy can a close friend or family member act on our behalf, keeping our wishes foremost, when we can no longer do it for ourselves. An advanced directive is easily written when one is well and not under stress. A form can be obtained from any hospital or clinic. A copy should be kept with your medical records and by the person who agrees to be your health care proxy. Advance directives are advisable for everybody, but especially for persons on HPEN or other forms of artificial life support.

HPEN seldom benefits persons on the edge of death and can, in fact, create unwanted complications. A terminal patient is defined as a person who will die in three months or less from their debilitating fatal illness. Recognizing the terminal phase is not always easy. Often it is more recognizable to the dying person than to their clinician. At some point there needs to be a discussion between the person and their close caregivers about steps in the dying process. If the plan is to die at home, is there a circle of friends who can help? When is it best to transfer from artificial nutrition to more simple hydration? There are very few nursing homes who can manage a patient on parenteral nutrition, but tube feeding or simple IV hydration is not usually a problem. In the last month or two it is often helpful to involve a hospice service. In my experience, hospice nurses are more available than the usual visiting nurses and can make the dying process more comfortable. Hospice nurses are very experienced with managing pain symptoms and take responsibility for indicating to the physician what the home patient needs. In many situations the hospice organization can only get involved when active treatment, such as HPN, has ceased. At the very end, intravenous fluids need to be scaled back to make breathing more comfortable.

Hospice nurses are familiar with this aspect of care.
In my experience, if a person on artificial nutrition understands these end-of-life issues, they will signal when they are ready to stop artificial nutrition to simplify his or her care. The most important element is for the clinician to know the person and their family and stay in close contact. For me, without exception, helping someone I've cared for, for a long time, to have a peaceful dying process, is the end of a long privileged partnership.

Articles on other end of life issues such as health care proxies and estate planning, are available from the Oley office, call (800) 776-OLEY.