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JOURNAL OF THE ROYAL SOCIETY OF MEDICINE
Annual review S B Carr MRCP
or
Supplement No. 27
Volume 89
1996
continuous assessment?
R Dinwiddie FRCP
J R Soc Med 1996;89(Suppl. 27):3-7
SECTION OF PAEDIATRICS, 10 OCTOBER 1995
Keywords: cystic fibrosis; vitamins; diabetes; lungfunction testing
INTRODUCTION
Cystic fibrosis (CF) is a serious life-long and ultimately lifethreatening disease. Great strides have been made in the last few years in terms of understanding the basic pathophysiology, in consolidating present treatments and developing new ones. As a result of this, life expectancy and the quality of life for CF patients has improved dramatically1. One of the main reasons why treatment has improved so much is the development of specialized CF centres that are able to support a multiprofessional team with considerable experience and expertise in the field. This specialized team can closely monitor the progress of each patient in the clinic so that any change in disease severity can be detected at an early stage and appropriate measures taken-either to minimize or hopefully reverse any adverse effects. Most large CF clinics have regular weekly meetings to review each patient seen and to discuss any management changes that may need to be made. This forum allows the various health professionals to share their findings and to make specific treatment plans. Many CF units use a stylized proforma sheet for recording clinic visits. (Available on request from RD.) This allows all aspects of the patient's physical condition and routine care to be carefully documented. Additionally, at least annually, there is a need for surveillance of other parameters, for example chest X-ray, full lung function, blood count, blood glucose and liver function tests. This allows the CF team to form an overview of each patient's general progress and can best be done at the time of measurements. In an ideal world this should happen within the above system and thus the concept of 'continuous assessment' should act as a cost effective and efficient way of long-term patient monitoring. However, 'the best laid plans o' mice an' men Gang aft a-gley' (Burns 1785). This has led, therefore, to the development of a more formalized 'annual review' process which involves a much more structured and detailed investigation of the patient, often including a day case admission to the ward once a year. This system also lends itself well to the patients who have shared care with their local hospitals and attend the CF centre 4 monthly, 6 monthly or yearly. Increasingly in present day practice, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London WC1 N 3JH, England
Correspondence to: Dr R Dinwiddie
formalized CF databases are being developed in order to facilitate clinical audit and continued research and development. It is anticipated that these will highlight continuing overall problems for the patients as a group and ultimately lead to better treatment for all. The process of annual review in a large clinic such as that at Great Ormond Street Hospital for children with 270 patientsis likely to be expensive and time consuming, not only for the staff concerned but also for the patients and their families. Is it cost effective, is it improving quality of patient care, or is the previous system of continuous assessment in fact perfectly adequate if applied properly? It is with these questions in mind that a careful analysis of the initial experience of a formalized annual review system was undertaken. The current review system has evolved over the past 20 months to involve middle grade medical staff, nursing staff, dietitian, physiotherapist and psychologist and a follow-up appointment at consultant level. A breakdown of the areas covered at review is given in Table 1. The results of an audit of 100 cases will be used as a framework to discuss these areas, the mean age of this group was 9.8 years (range 1. 1 to 16.5) and included 51 males and 49 females. RATIONALE AND METHODS
Respiratory illness is still the leading cause of morbidity and mortality in CF2, therefore a detailed assessment of respiratory function and treatment is needed. The main areas of concern are the present lung function, lung function trends, chest X-ray score, the number of infectious exacerbations, the bacterial colonization status and a review ofthe treatment modalities from both the physiotherapy aspect and medications. Respiratory function testing utilizing basic spirometry has become an accepted part of routine clinic attendance for any child old enough to perform the tests and a computerized print out of the previous 5 years is produced (Figure 1). Reviewing this on a yearly basis allows trends in declining function to be identified. Annual review allows the opportunity for more formal testing including plethysmographic lung volumes, bronchodilator response and exercise testing where appropriate. Present guidelines3 recommend an annual chest X-ray, with scoring4'5, in patients with CF. A review of sputum cultures documents lung bacterial colonization with specific organisms. The number of chest exacerbations in the preceding year allows for decision
3
Supplement No.
JOURNAL OF THE ROYAL SOCIETY OF MEDICINE
Table 1 Areas covered at annual review Respiratory
Gastrointestinal
Liver Other systems
Psychosocial
Equipment review and servicing Medication review
making about the commencement or manipulation of prophylactic antibiotics particularly via the nebulized route. Aspergillus status can also be assessed through sputum culture positivity, aspergillus precipitins, chest X-ray changes and skin tests where appropriate. Supplementary WARD/DEPT.
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treatment with bronchodilators, inhaled or oral steroids, DNase or mucolytics is also reviewed. Patients have an assessment of their chest physiotherapy technique; the annual review allows time for this and also for modification in treatment and introduction of new methods as children move from a passive to a more independent approach. Advice and education is given regarding the importance of exercise as an integral part of treatment. Malabsorption secondary to pancreatic enzyme deficiency is present in 85-90% of the cystic fibrosis population6. Malabsorption can present at birth with 10-15% of CF children having meconium ileus. Currently, at Great Ormond Street, some 25% of patients have presented in this way; this may represent a bias of surgical referrals to our hospital for specialized neonatal care. The malabsorption problems continue throughout life so careful review of the gastro-intestinal system is of great importance. Accurate measurement of height and weight must be made. At annual review all patients are seen by the dietitian. Time is afforded for a detailed review of caloric, pancreatic enzyme and vitamin intake. There is an opportunity for education and discussion about suitable energy supplementation, as well as adjustments of enzyme intake7. CF patients have increased calorie requirements, in the range of 120-150% of expected for the normal population8, therefore calculation of estimated average daily intake for age, sex and height is made. The need for supplementation of the fat soluble vitamins has long been established with reports of vitamin deficiency in CF as long ago as 19399: how to monitor this is less clear. Blood levels of vitamin A and vitamin E have been used but interpretation of results is difficult; low levels may reflect
Respiratory function testing Spirometry, lung volumes (plethysmography) + bronchodilator response, exercise testing Oxygen saturation Soutum microbiology-acute and chronic Aspergillus status Physiotherapy-technique Chest X-ray Nutritional-dietitian's assessment of dietary intake Vitamin levels and supplementation Pancreatic enzyme requirements Pancreatic endocrine function Function + Ultrasound Haematology Immunoglobulins Genotyping Ear, nose & throat Family and patient concerns Compliance Education and knowledge of disease
RESPIRATORY
Volume 89
27
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Figure 1 Long-term trend plot (6 years) of forced vital capacity (FVC) and forced expiratory volume in Is (FEV1). (Reproduced by permission of Mr A Laverty, Respiratory Laboratory, Great Ormond Street Hospital, London)
Supplement
JOURNAL OF THE ROYAL SOCIETY OF MEDICINE
No. 27
Volume
89
1996
are swallowed up in the endless task ofnebulization ofup to four different types of medication as well as fitting in several physiotherapy sessions. The review allows time to discuss these issues and new treatments that patients and parents often have questions about such as DNase.
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Figure 2 Frequency distribution of per cent predicted FEV1 (forced expiratory volume in I s)
either poor compliance or inadequate supplementation. Current recommendations for vitamin supplementation are up to 8000 i.u. for vitamin A and vitamin E 50-100mg daily for infants and 100mg daily for older children9. The prevalence of CF related diabetes increases with age'1, and so the emphasis on screening for this complication as the patient reaches adolescence is important. Routine annual urinalysis in children is a useful screen in conjunction with random blood glucose estimation. The presence of unaccountable weight loss in a patient is also a warning sign for the onset of diabetes and always triggers a urinalysis for glucose in this centre. Hepatic cirrhosis is an important complication of CF; the incidence increases with age up to adolescence1 2, and varies from 4_7%/o13, with a fall off again in the adult age range'4. The finding of hepatosplenomegaly on examination is one of the most important indicators of liver involvement; the most commonly used biochemical marker is aspartate amino transferase (AST). Raised AST in conjunction with prolonged prothrombin time is a further marker of liver disease and, even in the absence of clnical examination findings, these children should undergo ultrasound examination of their liver and spleen. Routine blood tests are taken at review and these will include full blood count, white cell differential, clotting times, urea and electrolytes, liver function tests, blood glucose, immunoglobulins, aspergillus precipitins, vitamin levels and any other tests tailored to individual patient needs. Genotyping is presently done by means of a mouth wash in the first instance. All patients and parents in our clinic are also seen at the annual review by the psychologist. This allows early contact and removes some of the stigma, still felt by parents especially, attached to seeing a psychologist. Parents and children are given the opportunity to express their concerns and fears to someone who is not actively treating them. Issues such as poor compliance can also be addressed in this forum and strategies around behavioural problems planned. Equipment maintenance is often overlooked and parents are advised to bring their nebulizer compressor and/or feeding pump equipment to annual review for servicing. Rationalization ofmedications is undertaken and their modes and times of delivery. All too often it is found that patients' days
RESULTS Lung function testing In the 77 children old enough to perform spirometry, results of forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) showed means of 66% (standard deviation 21.3%) and 77% (SD 19.5%) (predicted for height, and sex), respectively (Figure 2). In a group of 25 children who have had two annual reviews there was no significant change in their mean FEVI, by paired t-test analysis, mean at year zero was 62.4% (standard error 4.2) and 1 year 64.8% (SE 4.9). Of 54 children undergoing bronchodilator testing 18.5% showed a > 15% improvement in peak flow, 16.7% showed a worsening with bronchodilator and the remaining 64.7% showed no improvement. Oxygen saturation (n=59) median value was 95% (interquartile (IQ) range 940/o-96%).
Chest X-rays All children had chest X-rays. Eighty-six were scored with the Chrispin-Norman system with a mean score 9.7 (SD 5.7) range 1-23. A negative Pearson's correlation (r=-0.6, P
