Survival without transplant

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Journal of Cystic Fibrosis

Volume 2, Issue 1 , March 2003, Pages 55-57

Copyright © 2003 European Cystic Fibrosis Society. Published by Elsevier

Science B. V.

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. 1. 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.

2. 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.2.1. 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'.2.2. 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.2.3. 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.

3. 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.3.1. 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.

  (4K)Fig. 1. Reactions to transplant proposal.

3.2. 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.3.3. 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 ©.

  (3K)Fig. 2. Fluctuations around decision-making.

4. 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.

5. 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.

Becki

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