Article :Survival against the odds

April 8, 2003

Journal of Cystic Fibrosis

Volume 2, Issue 1 , March 2003, Pages 46-48

Science B. V.

Survival against the odds

Marie Johannesson, , a and Laskb

a Uppsala CF-centre, Department of Womens' and Childrens' Health, Uppsala

University Hospital, 75185, Uppsala, Sweden

b Department of Psychiatry, St 's Hospital Medical School, London,

UK

While the majority of patients with cystic fibrosis (CF) survive well

into adulthood, some do so against the odds. The severity of their

illness, their psychosocial environment, or their failure to adhere to

treatment are such that survival could not have been predicted. It

appears that some patients are more resilient than others. Some specific

components of resilience include comprehensibility, manageability and

meaningfulness. These are defined and case examples provided to

illustrate their significance.

1. Introduction

The majority of patients with cystic fibrosis (CF) now survive into

adulthood [1 and 2], with an average life expectancy in Scandinavia of

more than 30 years [3]. Some survive despite poor lung function, frequent

infections, adverse psychosocial factors or poor adherence. They appear

to survive against the odds. Such survival demands a high degree of

resilience, which in itself is influenced by both biological and

psychosocial factors [4].An important contribution to the understanding

of the psychology of resilience is Antonovsk's theory of `sense of

coherence' [5]. This states that a sense of coherence is a major

determinant of maintaining one's position on the health/disease continuum

and of movement toward the healthy end. The sense of coherence is a

global orientation that expresses the extent to which one has a

pervasive, enduring, but dynamic feeling of confidence that (a) the

stimuli deriving from one's internal and external environments in the

course of living are structured, predictable and explicable; (

resources are available to one to meet the demands posed by these

stimuli, and © these demands are challenges, worthy of investment and

engagement [5].These three core components to a sense of coherence are

described as: comprehensibility, manageability and meaningfulness.

Comprehensibility is a sense that information is ordered, consistent,

structured and clear. Manageability is a sense that resources are at

one's disposal, which are adequate to meet the demands imposed.

Meaningfulness is a sense that one feels oneself to be a participant in

the processes shaping one's destiny.In this paper case examples are used

to illustrate how this sense of coherence may relate to survival among

adults with CF.

2. Case examples

2.1. Case 1: Ingrid, age 35

Ingrid was diagnosed with CF, aged 14, due to frequent pulmonary

infections and malnutrition. She remembered that she had felt sick all

through childhood and could never play with other children. In addition

she had been traumatised by the death of her brother in a car accident

and by her mother's alcoholism. The CF is currently categorised as

moderate to severe with the need for intensive medical treatment. She is

married and has two children (aged 6 and 2), the older having attention

deficit hyperactivity disorder and the younger craniosynostosis.Ingrid

has chosen to divide her CF into negative and positive components. The

negative components are that: (a) `CF is an invisible handicap'. ( `The

treatment takes a lot of time' © `I am not able to do as much as I want

to'. (d) `I may not see my kids grow up'.The positive components are that

(a) `I meet good people'. ( `I don't care about small things' © `I

treat other people well'. (d) `I never give up'. (e) `I have a full

physical check up at least once a year'. (f) `I have a good quality of

life'. (g) `I have two children through in vitro fertilisation'. (h) `I

have a sense of power and strength to have gone through everything so

far'. (9) `I am satisfied with life'.Ingrid believes that she keeps going

because there are more positives than negatives. Applying the three

components of a sense of coherence: comprehensibility is fulfilled by the

annual full physical check-ups; manageability is manifested also by the

check-ups as well as access to in-vitro fertilisation; and meaningfulness

by her sociability, social network, satisfaction with life and most

particularly by her sense of power and strength.2.2. Case 2: Bjorn, age

41

Bjorn was diagnosed in early childhood due to frequent pulmonary

infections and malnutrition. He is considered to have moderate illness.

He was finding the treatment far too intrusive and time-consuming,

especially since he loves his job as a truck driver. After he was given

an inhalation devise that he could use in his truck he was able to carry

out his inhalations more frequently with consequent considerable

improvement in his quality of life `It is my work that keeps me

going'.Manageability is the key to Bjorn's resilience.2.3. Case 3: Marie,

age 42

Marie was diagnosed with CF at 18 years of age after having recurrent

pneumonia and nutritional problems. Her mother died from cancer two years

later. She has had several severe hemoptyses treated successfully with

arterial embolisation of the bronchial arteries. Marie is married and has

given birth to four children (one of them died from sudden infant death

syndrome). Currently she is considered to have moderate disease, which

demands extensive medical treatment.When asked what keeps her going she

answered: `I see myself as healthy. CF is like a backpack. There is no

reason for you to think that I conceive that my problems are worse than

yours....'Meaningfulness is the key to Marie's coping. She sees herself

as `carrying a backpack', a task well within her capability, and a task

that many others have to carry out for themselves, so making her no

different from others.2.4. Case 4: Liv, aged 35

Liv's CF was diagnosed in early childhood due to respiratory infections

and gastrointestinal problems. She was frequently hospitalised throughout

childhood and adolescence, and was so severely affected by her disease

that she needed a wheelchair by the time she was 18. Aged 20 she moved

closer to a CF centre, where her treatment was intensified with

aggressive antibiotic treatment, nutritional guidance and physical

exercise. Her physical status improved considerably, and by the age of 22

she was working part time with no need for a wheelchair. Aged 25 she was

discovered to have mild myasthenia gravis (MG). Now, 10 years later, Liv

has moderately severe CF with recurrent peripheral thrombosis and MG,

which limits the use of antibiotics. She is married and has one daughter

(6 years old) and a dog (this was chosen instead of a second child). She

has a full time pension but is heavily involved in her child's leisure

activities.When asked what keeps her going she answered: `I see myself as

a fighter. Is there an option? I have to take every chance there is to

control my CF. I cannot let CF win over my family. I have to fight.

Sometimes I feel like a lioness. I love my activity as a gym instructor

for those kids. To see them run around me makes me happy'.Liv uses

manageability and meaningfulness to cope. She makes use of every chance

to control her CF and sees herself as having considerable control of the

processes shaping her destiny.

3. Discussion

Survival against the odds is dependent upon a high level of resilience.

According to the theory of sense of coherence resilience is determined by

the patient having a strong sense of comprehensibility (a sense that

information is ordered, consistent, structured and clear), manageability

(a sense that resources are at one's disposal which are adequate to meet

the demands imposed), and meaningfulness (a sense that one feels oneself

to be a participant in the processes shaping one's destiny). Some people

are sufficiently resilient from drawing primarily upon one component,

e.g. Bjorn who uses manageability, or Marie who uses meaningfulness;

others use two, e.g. Liv who uses both meaningfulness and manageability;

but most will draw upon all three, e.g. Ingrid.The most common replies

when patients are asked what helps them keep going relate to being part

of something such as a family, a job or the community, i.e.

meaningfulness. This gives some clues as to what sort of help our

patients may most value; i.e. finding some practical role which gives

enhanced meaning to their lives. Providing understandable information in

the right way should increase the degree of comprehensibility, and

attention to the practicalities of living with CF may increase

manageability.Clearly there is far more to resilience than these

constructs but an increased coherence will improve resilience among our

patients with CF and thereby hopefully increase their chances of

survival.

Becki

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April 8, 2003