Cultural issues in cystic fibrosis

[Guest guest]

Journal of Cystic Fibrosis

Volume 2, Issue 1 , March 2003, Pages 38-41

Copyright © 2003 European Cystic Fibrosis Society. Published by Elsevier

Science B. V.

Cultural issues in cystic fibrosis

Alistair J. A. Duff,

Department of Clinical and Health Psychology, Wing Extension, St

's University Hospital, Leeds LS9 7TF, UK

Although current numbers of non-Caucasian CF patients are small,

collectively they represent sizeable groups and increasingly more is

becoming known about the spectrum of mutations in the CFTR gene in

different populations. As such it is important to acknowledge that there

will be certain sociocultural challenges in interacting with and managing

such patients. This paper explores such developments and considers some

of the current and future challenges facing those who treat the disease.

Techniques applied in other illness contexts for establishing and

maintaining effective patient–professional relationships, reviewing

patient education, resolving treatment conflicts and improving adherence

are discussed, as are the implications for future research.

1. Introduction

Engaging individuals in modern health care systems depends on their

intrinsic health beliefs. These not only vary over time and depend on

gender, socio-economic status and education, but also stem from other

values, cultural and spiritual, which influence how patients' define and

perceive their health (e.g. health as; `not ill', the absence of disease,

leading a socially and emotionally healthy life and being physically fit)

[1]. Implications of poor health can have great cultural significance and

thus have major implications for how patients access services, interact

with health care professionals and adhere to treatment. Patient

information initiatives and in-patient care are also culturally

influenced.It is argued that cystic fibrosis (CF) is so rare outside

populations of European descent, that screening should not be offered to

these groups [2]. Yet, identification of an increasing number of genetic

mutations in different populations ( Fig. 1), has led others to consider

whether or not CF has been under-reported in certain groups, being

mis-diagnosed as chronic pulmonary infection, malnutrition, tuberculosis,

or failure to thrive [3]. In some multi-ethnic countries significant

differences in prognosis and clinical status have been found. In the USA,

patients of African–American decent, have significantly poorer

nutritional status and pulmonary function than their Caucasian

counterparts [4]. In South Africa, Cape Coloured children became infected

with Pseudomonas aeruginosa infection significantly earlier than those of

European decent and had a worse 5-year survival rate [5]. Although

socio-economic factors were implicated in both studies, ethnicity and

under-recognition were also linked.

  (12K)Fig. 1. Distribution of known CFTR mutations amongst different

ethnic populations (reproduced with kind permission of Garry Cutting

M.D., s Hopkins, Baltimore MD, USA).

In the UK people from many ethnic backgrounds may have CF, for example,

Asians, particularly those whose families originate from northern India

(Sikhs and Hindus) Pakistan and Bangladesh (Muslims) and Jews (both

Ashkenazi and non-Ashkenazi). Whilst numbers may currently be small, the

introduction of national CF screening programmes could raise incidence

rates, particularly in multi-ethnic areas. The `strength' of religious

beliefs varies within these groups, just as in others, ranging from the

non-practising to the fanatical. Consequently, whilst it is wrong to

assume that there will necessarily be cultural barriers to treating

Muslims and Jews with CF, as management needs a multi-systemic approach,

carers need to be aware of potential obstacles and negotiate how these

can be overcome.

2. Challenges

There are four potentially important and inter-linked aspects of managing

CF that may present teams with sociocultural challenges; establishing and

maintaining patient–professional relationships, patient education,

adherence to treatment and care of the dying and death.2.1.

Patient–professional relationships

The basis for effective treatment is good patient–professional

relationships. It is important to continually reflect on how differing

sociocultural beliefs and values affect the establishment of these and

the maintenance of the boundaries between them. Increasing access to

health services for individuals' from ethnic minority groups is a

national priority in the UK. It is important that people feel able to

engage with service providers and are motivated to do so. CF team

members, themselves will come from a variety of different ethnic and

religious backgrounds. At times this too may need to be reflected on if

strong patient–professional are to be formed. Ultimately it is this that

enables effective liaison and mutual learning and so, CF team members

need to feel comfortable asking questions about different sociocultural

beliefs (as do all health carers), if they are to become truly

`culturally informed'. This rarely results in offence being caused and

indeed, in most cases would probably be viewed positively or even

necessary.Using family members as interpreters is inappropriate as it is

crucial to try and communicate directly with each individual to properly

hear `their views'. As such, team members need to be trained in how to

properly use interpreting services if good relationships are to be

maintained over, what is usually, long periods of time. Discussion of how

cultural values may impinge on treatment, for example, issues of gender

and touch and taking detailed family histories, can only truly take place

within a trusting professional therapeutic relationship if difficulties

are to be resolved or negotiated.2.2. Patient education

Whilst language barriers can usually be overcome, when combined with the

conflicting views of some parents about Western medicine, for example

Asians, explaining CF to children may become difficult [6]. It has been

reported that, just like other patients in the UK, some young Asians with

CF enter adulthood knowing little about the condition. CF-related

problems such as decreasing lung function, infertility and poor mobility,

can raise many cultural issues for Asian adults. Although not all want

arranged marriages, these too can present dilemmas which are rooted in

perceptions of `acceptability', parents being reluctant to seek out

partners for their children for fear of them being rejected. This largely

depends on how much parents understand CF and communicate the seriousness

of the disease to prospective parents-in-law (i.e. its treatment and

longer-term implications). If this is minimised there is the potential to

create unrealistic expectations of female patients in their new role as

wife or daughter-in-law [6].Considering family dynamics and discussing

these and the impact of CF openly, is vital for teams. Having a range of

CF literature in different languages may be difficult to prioritise at a

local level, however, co-ordinating such resources nationally may be less

onerous. Equally, where appropriate, involving the support of local

religious or spiritual leaders and groups may be helpful in facilitating

discussion and understanding, particularly where aspects of treatment

potentially contravene certain beliefs or values.2.3. Adherence to

treatment

Whilst there is no doubt that advances in CF treatment have significantly

improved patients' longevity and QoL, the burden of care has also

undeniably increased. Introducing new therapies and treatments to

patients and the extent to which they adhere to what is prescribed, will

again depend on their relationships with CF team members and how

treatment is negotiated. In order to consider how cultural beliefs may

impact on adherence rates, carers may need to spend greater time ensuring

that explanations about treatment rationales are properly conveyed and

understood.Dietetic management poses many challenges for different ethnic

and religious groups (e.g. during religious periods of fasting and the

need to avoid certain foods) and many excellent reviews of these exist

[7]. In CF management there are some particular challenges for Muslims

and Jews. Periods of religious fasting for people may need to be

negotiated and compromises reached. Pancreatic enzyme replacement therapy

is central to the nutritional management of CF. Capsules containing

porcine-derived digestive enzymes are taken orally with all meals and

snacks and without clear guidance from spiritual leaders, this can be

problematic for some patients. Whilst less commonly problematic, the

prescription of some nutritional supplements and certain vitamins also

need to be considered.

Case example

A 17-year-old male patient, who was a devout Muslim, had been under

dietetic review for poor weight gain. As the assessment progressed, it

became evident that he had stopped taking enzyme replacement capsules

(Creon) on discovering they were porcine-derived. Despite the fact that

the pharmaceutical company issues a routine statement from the London

Central Mosque Trust & Islamic Cultural Centre, stating that Creon can be

taken by Muslims (on the grounds that no alternative exists), the

patient's detailed knowledge of the Holy Qur'aan was such that he still

questioned taking it. Considerable discussion took place with the team

dietitian, focussing on compromise, the lack of an alternative enzyme

replacement therapies and the effect of non-adherence on the patient's

long-term health. Guidance and discussion took place with another

spiritual leader, the local Mufti,1 with whom the patient had close ties

with and much respect for. His consideration and subsequent permission

was sufficient for the patient to accept the therapy. He began to gain

weight shortly after recommencing Creon.

In-patient care too can pose significant sociocultural challenges for

Muslims. Traditional gender divisions can lead to worry and consternation

about male carers carrying out physical examinations and physiotherapy on

females, or stays on mixed-sex wards. These situations must be handled

with great diplomacy if anxieties are to be reduced and compromises

reached.

For those whose lung function has deteriorated to the point where

transplantation is considered, again views about organ donation and blood

products will need to be considered. Spending time on developing good

relations and understanding and respecting cultural differences, will

pave the way for effective discussion and adaptation.2.4. Care the dying

and death

Most religions have strict and specific funeral rituals, however, only

some have guidelines for care during the process of dying. As with all

patients, open discussion with them and their relatives about their

wishes must take place. However, for certain groups, (e.g. Jews and

Muslims), operational policies exist within most NHS Trusts which address

how death in normal and abnormal circumstances, autopsy and burials,

should be followed whenever possible.

Muslims with CF who are dying should be made to sit or lie facing the

Qiblah (the first house of worship in Mecca) if possible. Family members

should be permitted to recite prayers around their relative's bed even if

this is situated in a bay.

After death, there are explicit instructions about how the body should be

handled and released, with burial taking place as soon as possible after

death. The body of a Jewish patient who dies must be handled with equal

care and most NHS Trust protocols include how teams should respond during

the Sabbath and other religious festivals and holidays.

3. Conclusions

Different cultures present different challenges in managing CF. Increased

screening programmes and the identification of new genetic CFTR mutations

will lead to an increased understanding of CF in non-Caucasian

populations and maybe the recognition of a higher incidence. In the UK,

although current numbers of non-Caucasian CF patients are relatively

small, when aggregated throughout the country, they represent sizeable

groups. The challenges in managing such patients are to consider

developing and maintaining good therapeutic relationships, improving

patient-education and negotiating certain aspects of treatment. To date

most studies of ethnic diversity in CF have focused on screening and

incidence. Very few have explored the experiences of such groups and how

CF impacts on their lives. There is a real need to understand more about

this, particularly with increasing knowledge of identification and

possible incidence.

Becki

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