Hard Question - An Article -1

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Survival without transplant I. Götz  University Children's Hospital,

Währinger Gürtel 18-20, Vienna 1090, Austria Patients with severe cystic

fibrosis who

face lung transplant proposal react in different ways to the offer of this

potentially life prolonging therapy. Little is known about those patients who

refused the operation. This article describes various behaviour patterns and

reactions following the transplant proposal. The major reasons for refusal of

lung

transplantation are based on personal experiences and encounters and on

psychological and medical considerations. Only a subgroup of patients sticks to

the

initial decision of refusing the operation. Other patients question their

decision and fluctuate between refusal and acceptance. A third group of patients

reverses the decision mostly as a result of a significant life event. The

decision not to undergo lung transplantation needs to be viewed as a reversible

one and in the context of extreme physical and psychological vulnerability on

the part of the patients. Health professionals should recognise the potential

for change, remain non-judgemental and flexible, and adjust their services to

the respective circumstances.  Introduction

This paper focuses on persons with cystic fibrosis (CF) who do not wish to

undergo lung transplantation (LTx) but who do not want to die. This seems to be

an unsolvable problem because physicians only suggest transplantation when

they fear a patient may die within the foreseeable future. One may argue that

there are persons with CF who would accept the possibility of a dignified death.

Perhaps this is correct in a few cases. More often such persons are primarily

tired of suffering. If one would offer them a life without suffering most, if

not all, would accept. The topic of refusing lung transplantation thus far has

not drawn significant research attention. In fact, a literature search proved

unsuccessful in identifying any appropriate papers. Studies that addressed

referral and evaluation of transplant candidates only refer to the number of

patients not accepted or refusing the operation [1, 2, 3, 4 and 5]. No study so

far pursued patients with CF who voluntarily refused LTx. In addition, a

methodological problem aggravates a systematic study of patients who were

offered

LTx. Those who were considered candidates in childhood or adolescence were later

transferred to adolescent or adult care and therefore lost to follow-up.

Given the lack of empirical studies this paper is based on a 15 year clinical

experience with heart-lung and lung transplantation in children, adolescents,

and

adults with CF. The aim is to describe the various patterns of how patients

proceed after they have refused LTx.  Reasons for refusing lung transplantation

So why do people with severe CF who face progressive health deterioration and

significant impairment of quality of life refuse a life prolonging therapy? I

have attributed the reasons for refusal to three categories, although the

boundaries should not be viewed as clear-cut.  Reasons that are based on

experiences and encounters

This category refers to individual experiences and the influence of

significant others including fellow-patients, friends, family members and staff

members. A particularly dreadful experience is the death of a fellow patient,

which

may bring with it discouragement and the anticipation of an unfavourable

outcome for oneself. The opinions, attitudes and beliefs of significant others

are

strong and determining factors. If based on comprehensive reflection they may

well support the patient in decision-making. If based primarily on subjective

arguments with an emphasis on only the risks they are counterproductive. In

light of their mental and physical weakness transplant candidates are

susceptible

to any advice and often unable to recognise undue pressure such as the clear

recommendation `don't do it'. Psychological and psychosocial reasons

Some patients are afraid of the necessity to rely on and become dependent on

even more health professionals (HP), which is associated with feelings of

being abandoned by their familiar care providers. Some patients simply believe

that they will make it without transplantation or that they even will live

longer

without it. A number of patients seem to be able to accept a given life-span

on the grounds of a religious belief. Most patients express fears and

anxieties of any kind, including the unknown outcome. Medical reasons

Sometimes there is a discrepancy between the physician's and the patient's

judgement. The patients may find their health state acceptable or less severe

than the doctors or superior to patients who are not considered for LTx. A very

common reason refers to the fear of acquiring a new disease which is based on

the knowledge that transplantation involves trading part of one disease for an

entire second disease. In addition to possibly painful procedures the

anticipation of many complications and the perspective of life-long treatment

may be

discouraging. The knowledge that nerves will be severed and that this may

result in a lack of control over one's health is another stressful aspect.

Behaviour patterns and decision-making following refusal

Irrespective of which reason contributed most to the decision to refuse LTx

there are major patterns of reacting and proceeding. In general a process that

is characterised by innumerable discussions of pros and cons precedes and

follows the initial refusal of LTx. It is obvious that decision-making on a life

improving, life saving or life threatening operation often exceeds the coping

abilities and resources of those concerned. Therefore and together with

increasing confrontation with LTx over time a refusal that may have thought to

be

irreversible in the beginning can take unexpected turns. Whether or not a

patient

who withdraws his/her refusal undergoes LTx is predominantly dependent upon

health state, organ availabilty, and with respect to the different health care

systems, access to coverage of cost. Sustained refusal: the `no–no' group

Patients in this group stick to their decision, the initial refusal remains,

but they pursue two very different strategies (Fig. 1). A subgroup of patients

significantly changes their lifestyle in as much as they mobilise all their

willpower and improve nutrition, to increase physiotherapy and to set up an

exercise program. They now follow medication precisely and they often reduce

their workload. These patients view the proposal of LTX as a temporary setback

and

are determined not to lose a hard-fought battle. In the other subgroup no

major changes in lifestyle are made. Rather, these patients try to pursue their

normal life often coupled with the desire to even undertake enterprises that

seem to be far beyond their present physical capabilities. In contrast to the

patients who increase adherence these patients want to savour every moment, they

want to enjoy life to the brim, to live as best they can, and they vow to

fight to their last breath.

 

 Reactions to transplant proposal. 

Doubts and untertainty: the `no–perhaps' group

These patients' initial `no' turns, after a while and at times into a

`perhaps'. Typical comments circulate around time and particular events (e.g.

`Not

now, maybe later'; `After my holidays'; `After Christmas'; `Not before my 30th

birthday'). These patients change their mind often, they make decisions and

withdraw them again. They refuse LTx when their health improves and they are

near

to acceptance in case of deterioration. They may be on the waiting list and

withdraw themselves or even reject an available organ. They are plagued by

doubts and uncertainty and at times they do not want to hear anything at all

about

LTx. These patients are at high risk of waiting too long and coming in too

late. Whether or not their life is eventually prolonged through LTx is

predominantly a matter of luck and immediate organ availability. Changing mind:

the `no–

yes' group

Individuals in this group may stick to the initial refusal for a while and

show hardly any doubts regarding their decision. Then, all of a sudden,

something significant happens inducing these patients to reverse their decision.

This

can be a dramatic deterioration that brings them so close to death that they

now know that they really do not want to die. It may be due to a tremendous

loss of quality of life that prevents them from pursuing daily activities.

Sometimes a religious belief has lost is relevance and the potential

prolongation of

life does not conflict with the respective faith anymore. But also pleasant

events like falling in love or finding an exciting job may shed a promising new

perspective on life and encourage a patient to make any effort to go on

living. Mainly the two latter groups, and only to a smaller extent the first

group,

exhibit a behaviour that may greatly challenge the health professionals. It

is the enormous fluctuation around the decision toward LTx (Fig. 2). Often the

slightest improvements in health status support and nurture refusal (a). There

are patients with advanced CF who are able to live and even to work despite

an FEV1% pred that circulates around 15. Such severely ill patients may feel

that they benefited from therapy during the hospital admission though their lung

function hardly improved. But the subjective impression of improvement may be

more influential in the belief that survival without LTx is possible than the

objective medical parameters. The fluctuation regarding decision-making can

be so pronounced that health professionals may have difficulty tracking the

patient's views. Even within hours a patient may switch from yes to no and vice

versa ( and a decision on Friday afternoon to undergo assessment may have

turned into refusal by Saturday morning ©.

 

Fluctuations around decision-making. 

How can health professionals support patients who refuse LTx?

Given that many CF health professionals have known their patients for years

and contributed constantly to prevent deterioration or to prolong life, a

refusal of the last therapy possible may represent a severe blow to a dedicated

team. Yet the patients who refuse LTx need at least as much attention and

support

as before. It is those who are under extraordinary stress, who are threatened

with death, and who are extremely vulnerable, both medically and

psychologically. It is normal and understandable that terminally ill patients

are

frightened, sad, desperate and confused. Therefore, health care providers need

to

respect a patient's refusal and to convey their understanding and acceptance.

Instead of automatically providing a predetermined set of interventions the

patients should be asked what kind of support they think they might need. There

is

no need to withhold potentially helpful interventions including psychotherapy,

but the offers should be tailored to the patients' needs and acceptance, or

non-acceptance must be respected. Information is a crucial issue. Health

professionals should be willing and available to inform about any item, in

particular

those that deal with the fear of suffocating. The patients must know that

they do not have to suffer in the terminal phase since they may be treated with

opiates for terminal pain and dyspnea. The patients must be reassured that they

are free to change their mind at any point in time and they should be

encouraged to initiate repeated discussions.  Conclusions

The necessity to confront LTx results in a process that is associated with

changing attitudes and temporary decisions. Patients who refuse LTx may stick to

this decision or change their mind. In the beginning neither the patients nor

the health professionals know what direction the initial decision will take.

Therefore, it is very important that the CF team remains non-judgemental and

flexible, recognises the potential for change, respects contradictory

decisions, and adjusts its service to the respective circumstances. (Source:

Copyright

© 2003 European Cystic Fibrosis Society. Published by Elsevier Science B. V. )

> <A HREF= " mailto:medneck@... " >medneck@...</A>

Joanne M. Schum

Cystic Fibrosis

Bi-lateral Lung Transplant Recipient

September 12, 1997

University of North Carolina Hospitals Chapel Hill

Residence: Upstate New York

email: luckylungsforjo@...

Manager of: Transplant Support - Lung, Heart/Lung, Heart

http://groups.msn.com/TransplantSupportLungHeartLungHeart

" Taking Flight - Inspirational Stories of Lung Transplantation "

Compiled by Joanne Schum

Authored by lung recipients around the world

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