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(Chest. 2000;118:80-84.)
© 2000 American College of Chest Physicians

Cystic Fibrosis*

End-Stage Care in Canada

Ian Mitchell, MB, FCCP; Edina Nakielna, MB, FCCP; Elizabeth Tullis, MD, FCCP and Carol Adair, PhD

* From the Department of Paediatrics (Dr. Mitchell) and the Department of Community Health Sciences (Dr. Adair), University of Calgary, Canada; the Department of Medicine (Dr. Nakielna), University of British Columbia, Vancouver, Canada; and the University of Toronto (Dr. Tullis), Canada.

Correspondence to: Ian Mitchell, MB, FCCP, Alberta Children’s Hospital, 1820 Richmond Rd SW, Calgary, Alberta, Canada T2T 5C7; e-mail: ian.mitchell{at}crha-health.ab.ca


   Abstract
TOP
 Abstract
Introduction
 Materials and Methods
 Results
 Discussion
 References
 
Objectives: To determine the circumstances in which individuals with cystic fibrosis (CF) die, the role of different caregivers, and the extent of palliative care for CF patients.

Design: Mailed survey of CF physicians.

Setting: CF centers in Canada.

Patients: All CF deaths in 1996 known to centers in Canada.

Results: The mean age (± SD) at death of the 45 individuals included in the study was 25.8 ± 13.5 years. The major cause of death was respiratory (34 patients; 75.5%). Nutritional concerns were common. Lung transplantation was considered in 42 patients (93.2%), with 7 patients (17.1%) being entered on a list, but it was carried out in only 2 patients (4.4%). Autopsies were performed on only 10 patients (22.2%). Most patients died in hospital (37 patients; 82.2%), and 7 patients (15.6%) died in ICUs while receiving intermittent positive-pressure ventilation. Palliative care was never discussed in 10 patients (25%). In a further 16 patients (40%), it was not discussed until the last month before death.

Conclusions: Respiratory disease remains the most common cause of death in CF patients. Lung transplantation is frequently considered, but most patients die without having had a transplant. Discussions on end-of-life care could be considered sooner.

Key Words: autopsy • cystic fibrosis • dying • end-stage care • lung transplantation • palliative care


   Introduction
TOP
 Abstract
 Introduction
Materials and Methods
 Results
 Discussion
 References
 
Cystic fibrosis (CF) is the most common genetic disease among white patients.1 It usually presents in infancy with respiratory or nutritional symptoms, but it can present later. The major systems affected are the respiratory and GI tract, and over time, chronic pulmonary infection and progressive pulmonary impairment dominate the clinical features. The current management of CF is thus directed toward the control of pulmonary infection, clearance of bronchial secretions, correction of the malabsorptive defect, and improvement in nutrition.1 There has been considerable improvement in survival, and although death is now uncommon in childhood,2 it still occurs early in life. In Canada, the median age at death is currently 31.0 years3 ; in the United States, the median age at death was 23 years in 19914 and 31.3 years in 1996.2 In a recent study of all CF patients in the United Kingdom, the median age of death was 31 years.5

CF care teams have been developed over the last 30 years and are composed of physicians, nurses, nutritionists, physiotherapists, respiratory therapists, psychologists, and social workers. Evidence has been presented that care in CF centers does indeed improve the outcome of patients with the disease.6 Adult clinics have been set up in most Canadian centers to meet the new reality that there are almost as many adults with CF as there are children.

New treatments such as DNase 7 and transplantation 8 also have been added. With the description of the CF gene, new approaches toward therapy are being investigated, although none are in active use at present.9

Along with the goal of long-term survival of patients with CF and the adoption of new technology and treatments, caregivers would like to provide quality end-of-life care. Formal palliative/hospice care programs in medicine have been developed by oncologists for the terminal care of cancer patients. However, the treatment for cancer tends to be quite invasive and is associated with severe and unpleasant side effects. At the terminal stage, all treatment, other than for the control of pain, is discontinued. This philosophy is not applicable to CF patients, a disease in which oxygen, IV antibiotics, and physiotherapy need to be continued. Few publications have dealt with issues of end-of-life care in CF, and it is not clear how such care is presently provided for CF patients.10 11 12 13

In this study, we asked some specific questions, such as the location of death for CF patients (at a designated palliative care unit, in hospital, or at home). We were interested in the role of the different caregivers in the CF team, and whether they felt capable of providing appropriate terminal support, as it is not clear whether advances in palliative care are included in CF care. Some patients may be on a list for transplantation, and we wanted to know whether this had an impact on end-of-life care. We also were interested in determining whether there were respiratory or nonrespiratory complications that might have accelerated death.

Thus, we report a cross-sectional survey of physicians on the circumstances surrounding the death of individuals with CF who died in Canada over a period of 1 year. The information obtained will be used to plan more detailed studies of some aspects of end-of-life care for patients with CF, and this will include relatives and all caregivers.


   Materials and Methods
TOP
 Abstract
 Introduction
 Materials and Methods
Results
 Discussion
 References
 
This was a cross-sectional study of 36 CF clinics that provided care for approximately 3,000 individuals with CF. The questionnaire was developed by a group of experienced clinicians, covering issues such as medical history, disease complications, details surrounding the death, and desirable support, according to standard questionnaire design recommendations.14 15 This was distributed to the 36 directors of all CF clinics in Canada by the Canadian Cystic Fibrosis Foundation and was translated into French by staff members at the Canadian Cystic Fibrosis Foundation for francophone clinics. The identity of those clinics that returned forms was known to the Canadian Cystic Fibrosis Foundation but not to the investigators, so the overall response rate cannot be calculated. Reminders were sent by the Foundation to those clinics that had not returned the forms. Data were entered and scrutinized for discrepancies, then descriptive statistics were prepared. The results are presented as a percentage of the number of responders (clinic directors). Denominators vary due to incomplete responses for some questions. The study was approved by the Conjoint Medical Ethics Committee at the University of Calgary.


   Results
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 Abstract
 Introduction
 Materials and Methods
 Results
Discussion
 References
 
Questionnaires were returned for 45 individuals who had died of CF in 1995. The Canadian Cystic Fibrosis Foundation keeps statistics on a fiscal year basis, and there were 44 deaths in the period from July 1, 1995, to June 30, 1996, suggesting that our coverage of deaths was virtually complete.

Gender was stated in 42 cases; 21 were female. The early history of the patients is shown in Table 1 , with mean age of onset of symptoms (14.9 months, SD, 3.77 months) and mean age at diagnosis (6.4 months, SD, 4.07 months). The median age at death was 24 years (mean 25.8; SD, 13.57).


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  		Table 1. Presentation at Time of Diagnosis

 
Care was most often given by a CF physician or pediatrician (89.9%), followed by an internal medicine specialist (4.4%) or a family physician (6.7%)

The most common cause of death was respiratory causes (34 deaths [75.5%]), including two patients with massive hemoptysis. Two patients (4.4%) died of sepsis, and the following were other causes of death: cardiac arrest, cerebral edema, liver cancer, infection, epileptic seizure, suicide, possible suicide, uncontrolled bleeding, and electrocution. We accepted the clinicians’ statement of cause of death, but some of the "causes" are modes of death and may be secondary to a respiratory cause. Two patients had either a pneumothorax or hemoptysis in the month before death. Thirty-one patients (69%) were on long-term oxygen therapy (> 1 month). Pseudomonas aeruginosa, Burkholderia cepacia, and Staphylococcus aureus were the most common organisms seen in the sputum (Table 2 ).


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  		Table 2. Distribution of Organisms in Sputum by Patient at the Time of Death*

 
Nutritional issues were common, and only two patients were described by their clinicians as having good nutritional status at the time of death.

Multiple organ failure was noted in two patients. Four patients had positive results for blood cultures (B cepacia in two patients, B cepacia and Gram-negative rods in one patient, and Enterobacter and Candida spp in another patient).

Liver disease was present in six patients (13.3%), and one patient died following liver transplantation. Diabetes mellitus was present in 14 patients (31.1%), and a number of other complications (or disorders) were present, including arthropathy in six patients and gastroesophageal reflux in four patients. It was noted that one patient had osteoporosis and another had pancreatitis. Major mental health problems occurred in six patients, four with drug and alcohol abuse and two with major psychosis.

Lung or heart/lung transplantation would seem to be a standard of care for these patients, as it was considered in 41 (93.2%) Only seven patients were accepted for transplantation, and surgery was performed in two patients before death occurred. Of course, this survey would not include those in whom transplantation was successful. Thirty-four of those patients referred were not accepted for transplantation or did not wish to have treatment (or perhaps they died before being accepted), and the responses to the questionnaire did not allow us to determine why acceptance did not occur.

Autopsies were requested for 16 deaths (35.5%), and permission was given in 10 cases (62.5%). This results in autopsies for only 22.2% of the total number of deaths

Most patients died in a hospital ward, including an ICU (35 patients [77.8%]) (Table 3 ). Nine patients died while receiving assisted ventilation, but we are unable to determine whether ventilation was invasive or noninvasive or whether the patients were awaiting transplantation.


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  		Table 3. Place of Death

 
Palliative care/comfort measures were used in 29 cases (76.3%) and included morphine (given to 15 patients [34.1%]) and anxiolytics (used for 1 patient). In some cases, palliative care never was discussed (10 patients [25%]) or only discussed in the last month of life (16 patients [40%]).

There were many support personnel available; the social worker was the most commonly mentioned, followed by the CF team as a whole (Table 4 ). Members of the ICU and transplantation staff also provided support for some patients. Only seven clinicians felt that additional psychosocial support might have been helpful, and they specified better home care or better hospice care. It is not known whether this response is related to a lack of knowledge of possible services.


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  		Table 4. Psychosocial Supports for Patients With CF*

 
As this group of dying individuals was mainly young adults, they had few surviving children. Only two of the women had families. We do not know whether any of the men with CF had children who were either adopted or who had been born through artificial insemination. We have no information on the support offered to these bereaved children.


   Discussion
TOP
 Abstract
 Introduction
 Materials and Methods
 Results
 Discussion
References
 
CF is the most common genetic disease in the white population and leads to early death. Despite these facts, there are few studies that have focused on end-of-life care.10 11 In a study 11 of individuals who had died in one institution over a period of 5 years, the authors described (end-of-life) care as preventative, therapeutic, and palliative and pointed out the difficulty of deciding when CF care moved from therapeutic to palliative. The issue of transplantation was not explored. Nevertheless, this study, like ours, raises important questions on this aspect of CF care.

The patients in our study were managed in a variety of ways as they came from a number of different centers. The information submitted had been collected by a responsible clinician shortly after each patient’s death. The demographics of the patients, in terms of mode and age of presentation and age of death, were typical; as in other publications, the deaths were mainly due to respiratory causes. These patients had often received supplemental oxygen, were commonly infected with P aeruginosa, and a substantial minority had liver disease and diabetes mellitus, problems that increase in frequency as patients with CF age. Transplantation was almost always considered but was only carried out in a minority of patients. Autopsies were infrequently requested. The physicians who completed the questionnaire indicated a desire for assistance in providing palliative care. As noted, active treatment is continued in CF patients up to the end of life, and this may explain why discussion about palliative care often came late in the patient’s life.

Our cross-sectional study asked only broad questions and could not answer any question in depth. The questions were answered by physicians and nurses only. We limited the questions to those that could be readily answered by the responsible clinician. We recognized that there were other questions that might be of interest but would require a much longer and more detailed questionnaire, the input of other team members, the input of other professionals who were not members of the CF team, and, most important of all, the input of CF patients and families. Although all the patients were in a publicly funded health program, they were treated in 36 different medical centers, and there would have been regional differences in the services available. More detailed studies may need to be limited to one or two centers.

The consideration of heart/lung transplantation involves patients and their families as well as clinicians. It is not always clear when it is time to refer patients for transplantation. Early studies suggested than an FEV1 of <= 30% predicted might be a useful measure,16 although this has not been confirmed in other studies.17 It is not surprising that transplantation was considered in > 90% of cases. Our information is only on those who died, not on those who received transplants and are alive. Thus, the reasons why so many are referred and yet are not transplanted is an issue to explore.

We did not offer a definition of palliative care to those completing the questionnaire but assumed that this was care designed to relieve symptoms in dying patients. As has been pointed out,12 palliative care services have been based on care provided to patients with cancer and AIDS, where treatment has unpleasant side effects. In these diseases, a decision is often made to discontinue active treatment and to use measures solely for the comfort of the patient. In CF, measures such as morphine certainly were used. However, in the last few months of life, a patient may be admitted several times to a hospital with a pulmonary exacerbation that responds to antibiotic treatment and even to short-term assisted ventilation. As a result, even when the patient is at a terminal stage, there is always a chance of a successful response to treatment, which makes it difficult for both clinicians and for families to decide when to switch from active treatment to palliative care.

Many clinicians answering the questionnaire felt that terminal care could be improved, but there was no information on the kind of services needed. The quality care of dying CF patients would involve continuing the connection with their long-term clinicians while being supplemented by others skilled in terminal care, including palliative care specialists, psychologists, and social workers. The spiritual needs of the patients also would have to be addressed.

Autopsies were not often requested, perhaps because physicians feel the only role of an autopsy is to "find a diagnosis" and that this is already known in the case of CF patients. However, autopsies also may be effective in determining complications of disease and complication of treatment, especially in long-term disease.18 Consent for an autopsy is dependent on the family, and if a request is not made, the family will not have an opportunity to discuss this option. The person who requests the autopsy and the mode of making this request will influence the likelihood that families will give consent.19 The information obtained from an autopsy should be given to families and is best done in a personal discussion between the clinician and the family. Assuming that the percentage of acceptance would not change if autopsies were requested in all of the patients in our survey, there would have been 28 autopsies carried out instead of 10.

We expected this survey to raise questions for further detailed studies, which would include the assessment of the role of transplantation and agreement on a definition of palliative care. The details of palliative care would be raised; for instance, while morphine is traditionally used to relieve dyspnea, there may be other, better treatments. It would also be important to assess whether the needs of dying CF patients and their families are identified and how they should be met.


   Footnotes
 
Abbreviation: CF = cystic fibrosis

Supported by the Division of Paediatric Respirology, Calgary Regional Health Authority, Canada.

Received for publication October 22, 1999. Accepted for publication February 24, 2000.


   References
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 Abstract
 Introduction
 Materials and Methods
 Results
 Discussion
 References
 

  1. Hay, WW Groothuis, JR Hayward, ARet al eds. Cystic fibrosis in current pediatric diagnosis and treatment 13th ed. 1997,446-448 Appleton & Lange Stamford, CT.
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  3. Canadian Cystic Fibrosis Foundation. Canadian Patient Data Registry, National Report, 1995. Toronto, Canada: Canadian Cystic Fibrosis Foundation, 1997
  4. Halliburton, CS, Mannino, DM, Olney, RS (1996) Cystic fibrosis deaths in the United States from 1979 through 1991. Arch Pediatr Adolesc Med 150,1181-1185[Abstract]
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  6. Mahadeva, R, Webb, K, Westerbeek, RC, et al (1998) Clinical outcome in relation to care in centers specialising in cystic fibrosis: cross sectional study. BMJ 316,1771-1775[Abstract/Free Full Text]
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  9. Collins, FS (1992) Cystic fibrosis: molecular biology and therapeutic implications. Science 256,774-779[Abstract/Free Full Text]
  10. Chapman, JA, Goodall, J (1979) Dying children need help too. BMJ 1,593-594
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  12. Westwood, ATR (1998) Terminal care in cystic fibrosis: hospital versus home [letter]? Pediatrics 102,436[Free Full Text]
  13. Liben, S, Goldman, A (1998) Home care for children with life-threatening illness. J Palliat Care 14,33-38[ISI][Medline]
  14. Sudman, S, Bradburn, NM (1983) Asking questions. Jossey-Bass San Francisco, CA.
  15. Aday, L (1989) Designing and conducting health surveys. Jossey-Bass San Fransisco, CA.
  16. Kerem, E, Reisman, J, Corey, M, et al (1992) Prediction of mortality in patients with cystic fibrosis. N Engl J Med 326,1187-1191[Abstract]
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  18. Beckwith, JB (1989) The value of the pediatric postmortem examination. Pediatr Clin North Am 36,29-36[ISI][Medline]
  19. McPhee, SJ (1996) Maximizing the benefits of autopsy for clinicians and families: what needs to be done. Arch Pathol Lab Med 120,743-748\[ISI][Medline]




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