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SCHOLARLY REPORT

Role of a Domiciliary Physiotherapist in the Treatment of Children with Cystic Fibrosis Diane Rogers Mary C Goodchild

Key Words Cystic fibrosis, domiciliary physiotherapist, physiotherapy, domiciliary management.

Summary This article describes the contribution to patient care made by a part-time physiotherapist with domiciliary responsibilities who has worked exclusively with paediatric cystic fibrosis (CF) patients over the past nine years as a member of the CF Unit, Department of Child Health, University Hospital of Wales (UHW), Cardiff. The article outlines the physiotherapy input both to the CF clinics held at UHW and at shared-care clinics throughout Wales. It also describes the main indications for the use of the domiciliary service, its responsibilities and possible benefits.

Introduction During the past decade, much has been written concerning the provision of domiciliary care to a variety of specialties, including paediatrics, mental health and the elderly. This article is an evaluation of t he service provided by a physiotherapist with domiciliary responsibilities who has worked exclusively with paediatric cystic fibrosis (CF) patients over the past nine years, as a member of the CF Team operating from t h e CF Unit (Department of Child Health) but responsible to the physiotherapy department, University Hospital of Wales (UHW), Cardiff.

The CF Unit provides total care for about 60 patients aged from a few months to 18 years. All the patients live in South Wales but some 55% outside South Glamorgan. Shared-care is given to a further 45 patients during visits to other CF clinics which are mainly in South Wales.

Detailed annual assessments are carried out on our 60 total-care patients and for an additional 25 pa t ien ts who were identified on the Wales/West Midlands Neonatal Screening Survey for CF (Chatfield et al, 1991). These 25 pat ients a t tend various clinics throughout Wales and are visited for assessment from the Cardiff CF Unit.

The part-time senior I physiotherapist post held

by one author (DR) was established at UHW in October 1986. The aims were to complement the service already existing at the UHW clinics, provided by the department of physiotherapy, to allow some domiciliary support, and to set up links with physiotherapy colleagues attending shared-care clinics elsewhere in Wales. Other members of t he CF team at UHW are the associate specialist i n CF (MCG); CF Unit secretary; CF clinical nurse specialist and CF dietician (the latter two are part-time but all these members are funded by South Glamorgan District Health Authority). In addition there is a CF clinical Fellow (registrar) who is usually full time and funded via a grant from the Cystic Fibrosis Trust, UK. Psychological and social work support is given as needed from the relevant departments at UHW.

Physiotherapy on the wards is organised from the physiotherapy department by staff working under the senior paediatric physiotherapist.

In addition to its clinical responsibilities, the CF Unit conducts a number of research projects, many in conjunction with basic scientists based at the Departments of Medical Biochemistry and Medical Microbiology, UHW, and the Institute of Medical Genetics, UHW.

Physiotherapy Service Assessment and Advice Given at UHW Clinics Attended by the CF Physiotherapist Two clinics a re held each week. Following measurement of weight and height, and urine tests (the latter for those ten years or older) performed by the out-patient clinic nurse, the patients are seen by the CF physiotherapist, before any medical consultation. The following assessment is made.

The case notes are examined for any recent changes in medical management including any recent investigations or surgical procedures. @ The patients and their families are asked to describe current symptoms and any functional l imitations thought t o be due to the chest disease.

Inspection is made for posture, colour, hands (for clubbing), chest shape, breathing rate and

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breathing pattern. Exceptionally, peripheral oedema may be palpable. 0 More specific tests for respiratory function are as below. Lung function tests: SVC, FVC, FEV,, FEF 50%, FEF 25%-75% a n d PEFR, a l l as percent predicted. FEV,IF’VC is expressed as a ratio. The measurements are made using a Vitalograph Compact spirometer and a Dial Wright’s peak flow meter. Pulse oximetry (SaO,) using a Nellcor 200 pulse oximeter with both adult and paediatric finger probes. Sputum analys i s : Samples (procured when possible in preference to cough swabs) a re collected in a universal container and cough swab samples are obtained either using dry swabs or Stuart’s transport medium. Specimens are sent for microscopy, culture and sensitivity. Exercise tolerance is tested as appropriate. Currently this test is a six-minute walk but we are examining the possibility of using different exercise tolerance tests including the shuttle walking test (Singh et al, 1992). Assessment of patients’ currerlt home physiotherapy regime is helped by knowledge of current chest X-ray appearance (for most patients chest X-rays are done six-monthly) and by auscultation for normaVabnormaYdiminished breath sounds. At intervals of approximately three months, parentdpatients are invited to demonstrate their physiotherapy management to the CF physiotherapist at the clinic visit. Any problems which cannot be solved are deferred for a home visit, for which arrangements are made either at the clinic or later by telephone. Assessment of current nebulisation therapy and inhalation therapy is helped by the use of home peak flow monitoring and recording of PEFR data by patients and their families.

Assessment and Advice given at Shared-care CF Clinics Attended by the CF Physiotherapist Follow-up of patients identified in Wales on the Wales/West Midlands Neonatal Screening Survey, 1985-1990 (Chatfield et al, 1991) has been the basis for travel t o various clinics throughout Wales. These visits are carried out by one of the two medical members of the team, accompanied on most occasions by other members including the CF physiotherapist. Medical and paramedical staff from the ‘local clinic’ attend also whenever possible.

Physiotherapy assessments at these shared-care clinics take the same form as those at UHW. Information shared with the use of a database

includes medical data, physiotherapy assess- ments and social aspects, the aim being to provide a clear transfer of information, more appropriate t reatment regimes and ration- alisation of physiotherapy services.

Annual Assessment The health and survival of people with CF depend largely on the s tandard of the i r t rea tment . Over the past 50 years be t te r treatment has resulted in a steady improvement in morbidity and mortality (RCP, 1990). As the prognosis is so dependent on treatment, i t is important t o review a patient’s condition regularly t o ensure that the treatment is the most appropriate available in the l ight of present knowledge and t h a t t he regimen prescribed is being carried out by the patient. This detailed evaluation of condition and therapy has been termed the ‘Comprehensive CF Assessment’ (Littlewood, 1993).

At Cardiff we have attempted to provide this same level of service to all our patients. The following assessment is arranged and co- ordinated by the CF nurse specialist.

1. Patients are sent for chest X-ray, with which they return to the clinic.

2. Routine height, weight and urine samples are taken by the clinic nurse.

3. Pa t ien ts a r e then seen by each of t he following in rotation.

Physiotherapist: The physiotherapy assessment consists of the routine clinic assessment with the addition of pre- and post-nebulisation challenge and lung function readings; also the completion of a questionnaire examining shortness of breath, cough, sputum production, physiotherapy treatment and exercise patterns.

Dietician: The dietetic assessment includes a four-day dietary assessment with pancreatin dosage for meals and snacks and a three-day faecal fat assay, both requested prior t o this clinic visit.

Social worker: This provides an opportunity to discuss housing and benefits, allowances and any psychosocial issues.

Doctor: This involves routine examination and assessment plus blood samples which are taken as below.

Random blood glucose level and glycosylated haemoglobin (HbAlc).

Serum tests of liver function (including gamma GT), urea, creatinine and electrolytes.

0 FBC and coagulation tests.

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0 Tests for allergic bronchopulmonary aspergillosis (ABPA).

0 Plasma levels of vitamins A, E and cholesterol.

Serum immunoglobulins.

Patients are offered any up-to-date literature that may be applicable and informed when the results of investigations will be available (most within a few days, some within one month). After a clinic the entire CF team assembles for a review meeting to discuss each patient’s current condition and to plan treatment protocols for the coming year.

The Domiciliary Service Home care for CF patients plays an important role in their management. With parents (and older patients) taking an increasing part in all aspects of care, it is vital to ensure that home physiotherapy treatment is as appropriate and effective as possible.

Review of Home Physiotherapy Management Physiotherapy can help in the treatment of excess bronchial secretions, chest wall stiffness and reduced exercise tolerance (Webber and Pryor, 1993). Regular review of home physio- therapy management is done by domiciliary visit, complementing the clinic assessment and ward t reatment . Phillips and David (1987) have stated that physiotherapy techniques for CF patients should be reviewed every three months and re-education provided if necessary.

Treatments are tailored t o meet the needs of individual patients, depending on type and severity of chest disease; social and home circumstances; ages of patient and family and the i r unders tanding of t he physiotherapy management of cystic fibrosis.

At Cardiff, physiotherapy is introduced from the time of diagnosis regardless of whether the child is clinically symptomatic. A minimum of two t reatments daily is encouraged with some children requiring i t more frequently. Techniques employed should be those which aim either to clear excess bronchial secretions or to improve lung volume. They include the active cycle of breathing techniques, including the forced expiration technique (Pryor et a l , 1979); postural drainage; chest percussion; positive expiratory pressure mask (Falk et a l , 1984); exercise; nebulisation therapy; chest vibration and breathing exercises, sometimes combined with single percussion.

Additional treatments which can decrease the work of breathing are advice on poor sleeping

positions, general positioning, relaxation, and breathing control.

The ultimate aim of physiotherapy management for this condition is to ensure that the patients themselves are able to carry out physiotherapy independently a t the appropriate age. Indep- endent treatment is introduced when both the family and the CF team feel that the time is most appropriate for each individual patient. At Cardiff the age at which fully independent physiotherapy is achieved can vary from nine to 16 years, with the majority of children in this age range being semi-independent.

Provision of Physiotherapy Equipment While limited instruction on the use and safety of new equipment can be given at clinic visits (with written guidelines), provision of such equipment is always a reason for a domiciliary visit.

Physiotherapy equipment a t home consists of postural drainage wedges and frames which are made available t o families when drainage becomes difficult over a parent’s lap. The equipment we use is illustrated opposite (fig 1).

Nebulisation therapy in Cardiff is used t o deliver a variety of drugs for the treatment of CF (see table) . Mucolytic agents such as acetylcysteine (Parvolex), dornase alfa (Pulmozyme) or 5% saline are usually delivered before physiotherapy, in our unit.

Bronchodilators are usually delivered before and steroids after physiotherapy; both can decrease airway obstruction and bronchial hyper- activity. Antibiotics for patients colonised with PseuQomonas aeruginosa are delivered after physiotherapy. Antifungal agents for the treat- ment of ABPA are delivered after physiotherapy as an adjunct to other treatments.

The most commonly prescribed drugs and equipment used at Cardiff are set out below. Combinations of equipment a re supplied according t o the drugs prescribed and the physiotherapist’s assessment. Pat ients are routinely challenged with nebulisation drugs at onset of treatment and at annual assessment. Lung function tests are compared before and after nebulisation to monitor safety and drug response.

Where appropriate, inhalers with or without spacer devices are also used for delivery of many of the medications. Careful assessment is made of each patient’s need and routine monitoring of inhaler techniques is made during home visits.

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(a) Small foam wedge (b) Large foam wedge

(c) Postural drainage frame

Fig 1 : Postural drainage equipment in use at Cardiff

(d) Postural drainage frame

The most commonly used drugs for nebulisation therapy and combinations of nebulisation equipment provided by the CF Unit and Physiotherapy Department, University Hospital of Wales

Drug Proprietary Compressors Nebuliser Accessories name used by patients chambers used (dependent on drug

at home for drug delivery used and patient’s age)

Bronchodilator Salbutamol Ventolin Portaneb 50 System 22 acorn Paediatric mask Terbutaline Bricanyl or System 22 or System 22 or mouthpiece

lpratropium Atrovent sulphate CR 50 Sidestream

bromide

Prophylactic for asthma Sodium lntal

cromoglycate Portaneb 50 System 22

acorn Paediatric mask or mouthpiece

Portaneb 50 or System 22 CR 50

System 22 CR 50 or CR 60 or Medix Turboneb

Steroid Budesonide Pulmicort System 22 Paediatric mask

respules Mizer + acorn or mouthpiece or Ventstream

Antibiotic Colistin Colomycin Ventstream + System 22 Paediatric mask for

aerosol hose or Gentamicin System 22 patients < 5 years.

antibiotic T- attachment Tobramycin with acorn neb used by older patients

and aerosol hose whenever possible Mucolytic Dornase alfa Pulmozyme System 22 System 22 modified Mouthpiece

Acetylcysteine Parvolex or disposable

Antifungal Amphotericin Amphotericin B System 22 System 22 Mouthpiece

infants and generally

A mouthpiece should be

CR 50 Sidestream

Sidestream

CR 60 Sidestream

Equipment suooliers

Medic-Aid Ltd Medic-Aid Ltd Medic-Aid Ltd and Medix Ltd

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Newly Diagnosed Patients The incidence of CF, Europe’s most common inherited respiratory disease, is approximately one in 2,500 live births. With a birth rate in Wales of about 38,000 births per annum, this should give about 15 new cases a year. At the Cardiff Paediatric CF Unit, we see three or four newly-diagnosed patients per year, some of whom are looked after by shared care. I t is important that good treatment practices are set up in the early months after diagnosis. Although frequent clinic visits are necessary, home visits are very important, where quiet discussion and demonstration of techniques and physiotherapy management can take place not only with parents but often with the extended family as well. We introduce physiotherapy immediately after the diagnosis is made, using techniques taught by a physiotherapist experienced in the management of CF. Drainage positions and physiotherapy methods are carefully explained and demonstrated; parents are asked to per- form t h e techniques and required t o show a n appropriate level of understanding of physiotherapy management . In the ear ly months following diagnosis frequent follow- up visits a re made to ensure tha t effective physiotherapy is maintained at home.

Patients Receiving Intravenous Antibiotic Therapy at Home The past several years have seen a marked increase in the number of patient-days of intravenous (IV) antibiotic treatment which are given at home from the CF Unit (Rogers et a l , 1995). From 1986 we have offered the choice of home or hospital IV treatment for those patients for whom we felt tha t IV treatment at home would be suitable. Support has been provided by the domiciliary CF physiotherapist and later by the CF nurse specialist as well. After initial tui t ion on IV therapy techniques given in hospital, the family returns home to continue treatment. Written guidelines a re provided which cover most of the medical and technical aspects.

1,000

800 m

Contact with t h e CF Uni t is encouraged whenever necessary, and a comprehensive pulmonary assessment at the s tar t of the IV course is carried out at the clinic or on the ward. Physiotherapy techniques and management are adjusted to reflect the patient’s clinical condition.

A follow-up visit to the patient’s home to adjust or to reinforce altered regimes is performed as needed. Additional home visits are done only if a problem arises.

The patient is seen again at succeeding clinic visits (usually half-way through and at the end of the two-week course of treatment) for repeat assessments and advice.

Over the past nine years, 26 CF patients have received 141 IV antibiotic courses between both home and hospital. Analysis of patient-days on such treatment has shown tha t numbers of these days at home have risen each year from none in the year 1985 (when all IV courses were in hospital) to a peak so far of 364 patient-days at home in 1990 (fig 2). For the clinic as a whole, from 1989 to the end of 1993, approximately one-third of patient-days on IV treatment were spent at home and two-thirds in hospital.

Care of Patients Before and After Transplantation At Cardiff we have had limited experience of t ransplantat ion. Four pa t ien ts have been assessed for heart and lung transplant (HLT) or for bi-lateral lung transplant (BLT) and six patients for liver transplant. Three operations have been carried out: HLT on a ten-year-old; BLT on a 16-year-old and liver transplant on a 12-year-old. As inevitable before such oper- ations, patients tend to be .frail and malnour- ished. In these circumstances, we have gained the impression that home visits are as import- ant as clinic visits to maintain treatment and morale.

Post-operatively, a different set of problems may emerge, not least (in the case of HLT or BLT) a

Hospital IV days Home IV days

.& C a, .- 5 400 a

200

0 Fig 2: Comparison of patient-days 1985 1986 1987 1988 1989 1990 1991 1992 1993 on home and hospital intravenous

therapy from March 1985 to Years February 1993

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reluctance to carry on with physiotherapy of the chest (when this is appropriate). For both lung and liver transplant patients, the use of immunosuppressants can mask infection and rejection may also be a problem. Physio- therapists play an important role in detecting respiratory problems which occur af ter transplant and they are often involved with their treatment.

Cure of the Terminally Ill It is a sad fact that CF is a progressive disease. Even now, in the 199Os, a few patients will reach a terminal stage in childhood. With the provision of a domiciliary service from the CF Unit, and in association with the patient’s GP and community services, it has been possible to provide the families with specialist physio- therapy during terminal care at home. Physiotherapy input is both specific to CF and general for dying patients. Treatment may be modified but is hardly ever stopped completely. This may include the following. 0 Relief of hypoxia with domiciliary oxygen therapy and opiates.

Pain relief using both drug therapy and trans- cutaneous electrical nerve stimulation (TENS). 0 Relief of breathlessness using positioning and drug therapy. 0 Dealing with generalised discomfort and skin care. 0 Emotional support for patients and their families, which is a major par t of care and involves the entire CF team.

In recent years we have cared for three such patients a t home: the ten-year-old (mentioned above) who died six months after a heart and lung transplant, whose death was mainly due to obliterative bronchiolitis; a 16-year-old whose serious lung disease was complicated by haem- optysis and diabetes mellitus and a 16-year-old who died while on the active waiting list for a heart-lung transplant. We continue to offer this option of home terminal care to patients and families.

Education on CF and its Management 0 Families: Time available a t clinic visits is often too limited to allow a full discussion of CF, the mechanisms which produce the chronic obstructive respiratory disease and the benefits of regular physiotherapy. Domiciliary visits by a physiotherapist can help to develop a n understanding of CF and its physiotherapy management, not only for patients and their immediate family, but for the extended families as well.

0 Schools: The vast majority of our CF patients attend state schools and receive mainstream education. Litt le if any instruct ion on CF appears to be given at teacher training colleges. Many aspects of the condition can disturb and concern the affected children, other children and teachers; especially the cough, the need to take various medications including inhalers , physiotherapy if given a t school and decisions regarding games, recreation and school trips. Visits to schools, both in support of individual patients and for lectures to teachers, have been well received and have resulted in improve- ments (Dyer and Morais, 1995). Our aim is for the children t o participate in a s many activities as possible and t o enjoy a normal school life.

Discussion There is ample evidence now that CF care is of a higher standard and more effective if provided from a CF Centre by a CF team (WHO, 1983; RCP, 1990; Walters et a l , 1993). Within the framework of the CF team, physiotherapy is essential from the time of diagnosis, t o try to prevent (or at least to delay) progressive lung damage due to abnormal mucus production and recurrent chest infection (Webber and Pryor, 1993). A physiotherapist with a specific interest in CF is an integral part of the team.

Provision of a domiciliary physiotherapy service for CF has been a major development, allowing patients and their families increased access to physiotherapy, both in the clinic and at home. Early identification of problems with rapid adjustments of treatment and approach should increase compliance, which is always an actual or potential problem in CF, as in any chronic disease. We feel that compliance with physio- therapy has been improved by discussion and demonstration in the home.

However, we should not assume that all is well with domiciliary treatment, even though it is much appreciated by the families (Rogers et al, 1995) and becoming more common. More objective assessments should be made to try to demonstrate its effectiveness and to find ways by which it could be improved.

Useful information could be obtained via a detailed questionnaire, possibly conducted at the end of a course of IV antibiotics which has been given at home. Several factors could be examined, individually o r in combination. A review of physiotherapy could explore tech- niques according to patients’ ages, maturity, family support and various aspects of the

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disease (Phillips and David, 1987). Specific interventions such as courses of IV antibiotics, the insertion of a gastrostomy o r a totally implantable venous access device (such as a Portacath) , a l l necessitate adjustments of physiotherapy a s do disease complications including bronchospasm, chest and/or abdominal pain, haemoptysis and many others.

Questions could be asked about usage of medical equipment. I t is unwise t o assume that i t is being used effectively o r even at all. Unnecessary o r inappropriate equipment, detr imental in itself, may lead t o non- compliance with physiotherapy in general. The domiciliary visit is a good opportunity to assess such equipment and i t s relevance t o t h a t particular patient and the household.

Another issue which deserves further study and which could be explored by questionnaire, is the stress which this disease engenders, not only in the individuals affected, but to their immediate and extended families. From the t ime of diagnosis, when families experience feelings of bereavement and grief, they go through a series of significant events including the first isolation of Pseudomonas species, the first course of IV antibiotics, the insertion of various devices (as mentioned above), a variety of abdominal symptoms and disease complications, possibly an assessment for organ transplant, and, finally, the concept of terminal illness. So far, our impression has been that this stress is reduced when some of these treatments are carried out at home. However, while the children may be happier , i t may be t h a t such benefits a r e achieved at the cost of increased stress to other family members, perhaps most particularly to the mothers, who are likely to carry out most of these treatments. Such s t ress should be evaluated.

For our par t in Cardiff, from spontaneous comments by the families and from the results of a short questionnaire ( Rogers et al, 1995 ) we have concluded that the domiciliary service for CF patients is useful and should be continued. It has led us to believe that advice and inform- ation are better retained in the more relaxed atmosphere of a home, and that this outreach service provides a valuable link between hosp- ital and home. At the same time, we have no doubt t h a t t he service could be improved; therefore we are examining the possibility of a more detailed questionnaire, devised with the assistance of a psychologist, in the hope that this would reveal further information on home treatments which could be of benefit t o CF patients and their families.

Acknowledgment Acknowledgment is made to Miss J Y James, clinical director of therapy services, physiotherapy department, University Hospital of Wales, Cardiff, for her support in establishing the domiciliary service. We thank Mrs E Mackie for typing the manuscript.

Authors Diane Rogers MCSP is a domiciliary physiotherapist based at the University Hospital of Wales, Cardiff. Mary C Goodchild MD MB BS DCH is associate specialist in cystic fibrosis, CF Unit (Department of Child Health), University Hospital of Wales, Cardiff. Both authors are funded by South Glamorgan District Health Authority. This article was received on September 14, 1995, and accept- ed on March 26. 1996.

Address for Correspondence Mrs D Rogers, Cystic Fibrosis Unit, Department of Child Health, University Hospital of Wales, Cardiff CF4 4XW.

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Dyer, J and Morais, J A (1995). ‘Children with CF need normal schooling: The role of the cystic fibrosis clinical nurse specialist‘, Professional Nurse, 11, 51 8-520. Falk, M, Kelstrup, M, Andersen, J B, Kinoshita, T, Falk, P, Stouring, S and Gothgen, I (1984). ‘Improving the ketchup bottle method with positive expiratory pressure, PEP, a controlled study in patients with cystic fibrosis’, European Journal of Respiratory Disease, 65, 423-432. Litt lewood, J M (1993). ‘The value of comprehensive assessment and investigations in the management of cystic fibrosis’ in: Escobar, H, Baquero, F and Suarez, L (eds). Clinical Ecology of Cystic Fibrosis: The value of comprehensive assessment and investigation in the management of cystic fibrosis, Elsevier Science, Amsterdam, pages 181 -1 87. Phillips, B W and David, T J (1987). ‘Management of the chest in cystic fibrosis’, Journal of the Royal Society of Medicine, 80, supplement 15, 30-37. Pryor, J A, Webber, B A, Hodson, M E and Batten, J C (1979). ‘Evaluation of the forced expiration technique as an adjunct to postural drainage in treatment of cystic fibrosis’, British Medical Journal, 2, 417-418. Royal College of Physicians (1990). ‘Cystic fibrosis in adults: Recommendations for the care of patients in the UK, Royal College of Physicians of London, November. Rogers, D, Morais, A, Millar-Jones, L, Knowles, R and Goodchild, M C (1995). ‘Cystic fibrosis: Comparison of home and hospital intravenous antibiotic treatment‘, Journal of the Association of Chartered Physiotherapists in Respiratory Cafe,

Singh, S J, Morgan, M D L, Scott, S, Walters, D and Hardman, A E (1992). ‘Development of a shuttle walking test of disability in patients with chronic airways obstruction’, Thorax, 47,101 9-24. Walters, S, Britton, J and Hodson, M E (1993). ‘Demographic and social characteristics of adults with cystic fibrosis in the United Kingdom’, British Medical Journal, 306, 549-552. Webber, B A and Pryor, J A (1993). ‘Bronchiectasis, prlmary ciliary dyskinesia and CF‘ in: Webber, B A and Pryor, J A (eds) Physiotherapy in Respiratory and Cardiac Problems, Churchill Livingstone, London, pages 399-417. World Health Organisation/lnternational Cystic Fibrosis (Mucoviscidosis) Association (1983). Report of a Joint Meeting on Cystic Fibrosis, WHO, Geneva, HMGllCF (M) 83.8.

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