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Diycult Asthma: Beyond the Guidelines Arch Dis Child 1999;80:201–206 201 Arch Dis Child: first published as 10.1136/adc.80.2.201 on 1 February 1999. Downloaded from PERSONAL PRACTICE DiYcult asthma: beyond the guidelines Ian Balfour-Lynn The current guidelines for prophylactic treat- should be considered, some more rare than ment of paediatric asthma culminate in the others. Relevant investigations should be car- addition of regular oral corticosteroids after a ried out when the history or examination stepwise increase in treatment.1–3 However, suggests one of these other diagnoses (table 1). there are still a few patients whose asthma is In particular, the presence of finger clubbing is not controlled despite such maximal conven- incompatible with the lone diagnosis of tional treatment and these children are often asthma. An asthmatic child may be aVected by referred to a tertiary paediatric respiratory another condition that contributes to or centre. Childhood asthma has several manifes- worsens the asthma symptoms. In particular, tations and diVerent approaches may be gastro-oesophageal reflux should be looked for required for diVerent patterns of asthma. and treated although its significance is not Unfortunately, some of these children are diY- always clear.5 Immunodeficiencies should also cult to treat. These include infants with severe be excluded; an initial screen should include recurrent viral wheezing who respond poorly to serum immunoglobulins and IgG subclasses, medication but generally have a good progno- complement concentrations, and antibody re- sis. There are those said to have “brittle sponses to common antigens (diphtheria, teta- asthma”; this has been classified into those with nus, Haemophilus influenzae type b, and pneu- a wide peak flow variation despite maximal mococcus) (Jones A, personal communication, treatment (type 1), and those who are well 1998). controlled in between attacks, which when they 4 occur are often sudden and severe (type 2). VOCAL CORD DYSFUNCTION This article concentrates on another group— Vocal cord dysfunction frequently mimics or children who have severe chronic background complicates asthma, and is characterised by a symptoms with acute exacerbations superim- paradoxical adduction of the vocal cords on http://adc.bmj.com/ posed. inspiration.6 The resultant airflow obstruction produces wheezing or stridor (usually loudest Confirming the diagnosis over the larynx), chest tightness, breathless- The first question when confronted by a child ness, and cough. Symptoms can be produced with severe symptoms despite conventional throughout the respiratory cycle, so they may treatment is: does the child really have asthma? be inspiratory, expiratory or both7 but they are There are several alternative diagnoses that never present during sleep. Although patients on September 29, 2021 by guest. Protected copyright. with vocal cord dysfunction are usually women Table 1 Some of the alternative and concomitant diagnoses with relevant investigations for children aged 20–40 years, the condition is well presenting with diYcult asthma recognised in children and adolescents.8 There are often underlying psychological stresses but Diagnosis Investigations it is not factitious as patients do not consciously 6 Cystic fibrosis Sweat test, DNA analysis control the process. Patients with vocal cord Primary ciliary Ciliary brushings for structure and dysfunction have often been misdiagnosed with Department of dyskinesia function, nasal nitric oxide asthma (subsequently found to be unrespon- Congenital lung Paediatric Respiratory abnormalities Chest x ray, CT chest scan sive to bronchodilators and corticosteroids), Medicine, Royal Tracheobronchomalacia Flexible bronchoscopy but the condition may coexist with asthma.9 Brompton & Harefield Vascular ring Barium swallow Spirometry is poorly reproducible, but the NHS Trust; Bronchiectasis Chest x ray, CT chest scan Department of Obliterative flow–volume loop may show evidence of Paediatrics, Chelsea bronchiolitis CT chest scan, viral titres variable extrathoracic obstruction or be and Westminster Chest x ray, inspiratory and 7 expiratory chest imaging (older normal. Diagnosis is confirmed by laryngos- Hospital, London, UK Inhaled foreign body children), rigid bronchoscopy copy, which shows adducted cords relieved by I Balfour-Lynn Chest x ray, bronchoalveolar lavage sedation.7 Treatment evolves around a clear for fat laden macrophages, explanation of the syndrome, stopping unnec- Correspondence to: Recurrent aspiration radiolabelled milk scan Dr I Balfour-Lynn, Congenital heart Echocardiography, essary medication, speech therapy, and psycho- Department of Paediatric disease electrocardiography logical support. Medicine, Royal Brompton Vocal cord dysfunction Laryngoscopy & Harefield NHS Trust, Gastro-oesophageal 24 hour pH study or radiolabelled Sydney Street, London reflux milk scan Confirming the severity SW3 6NP, UK. Immune problems Immune function testing email: i.balfourlynn@ In many cases, severity can be gauged from the ic.ac.uk CT, computed tomography. history and physical examination. Simple 202 Balfour-Lynn spirometry, which can be performed in the PSYCHOSOCIAL ASPECTS Arch Dis Child: first published as 10.1136/adc.80.2.201 on 1 February 1999. Downloaded from outpatient clinic, will provide measures of It is not uncommon for psychosocial aspects to airflow limitation, and a look at the flow– play a large role in the wellbeing of a child and volume loop can be helpful. Day to day peak particularly an adolescent with asthma. DiY- flow variability is a useful measure assuming cult home circumstances may lead to a that the home diaries are accurate. Unfortu- worsening of symptoms and sometimes the ill- nately, experience shows that these diaries can ness is used as a weapon or a cry for help. It rarely be relied on, compliance is poor, and may even be the mechanism for school refusal. measurements are often fabricated.10 Formal Admission to hospital is often needed to assess lung function testing in a laboratory may also the true functional status of the child and to be necessary for full evaluation, and would gain an impression of family interactions, albeit include measures of airway resistance, lung in unfamiliar surroundings. As well as simple volumes and air trapping, bronchodilator observations from the nursing staV on the responsiveness, and the eVects of exercise. A ward, help can be enlisted from the clinical directly observed exercise test may be useful to psychologist, teachers and play leaders, and help diVerentiate whether it is the patient’s sometimes social workers. Psychosocial prob- perception of breathlessness, general muscle lems do not mean the child does not have fitness, or true exercise induced asthma that is asthma (a point that must be emphasised to the causing problems with exercise. Patients often child), but the severity of symptoms are often start to complain of breathing diYculties the out of keeping with the actual disease severity. minute they start exercising, in which case it is The asthma and psychological disturbance unlikely this is caused by asthma itself. must both be treated on their own merits with- Measurement of exhaled nitric oxide may also out necessarily deciding which is primary and which secondary. be used to monitor the eVects of cortico- steroids on the underlying inflammation, al- though the usefulness of this measure is Reasons for treatment failure Assuming the patient genuinely has severe uncertain.11 12 It has been suggested that asthma responding poorly to treatment, it may assessment of bronchial hyperresponsiveness be possible to improve matters by simple (methacholine or histamine challenge) is the means. single most useful test of asthma severity,13 but in practice it is rarely useful in children. ALLERGENS AND OTHER AVOIDABLE FACTORS Inconsistencies with the clinical picture There may be allergens in the home that are should raise suspicions that the asthma severity providing a constant source of immunological is not as great as the child and his or her family stress to the airways. While house dust mites (and perhaps the referring doctors) perceive. are almost impossible to eradicate, their eVect The history should then be treated with may be reduced by various methods, including caution, and while reliable measures in the regular ventilation of the bedroom, mite proof examination include chest hyperinflation and allergen covers on bedding, and the use of an http://adc.bmj.com/ Harrison sulci, added sounds on auscultation eYcient vacuum cleaner with an adequate may not be genuine. Spirometry becomes filter.15 It is surprising how many asthmatic harder to interpret as measures are so eVort children live in homes with a multitude of furry dependent, although the shape of the flow– pets, and horse riding seems to be a favourite volume curve may give clues to poor eVort. pastime of teenage girls with severe asthma. Other measures that may be useful when the Rather than being dictatorial, it is best for the situation is in doubt include a straight and lat- families to come to decisions about removing eral chest x ray for hyperinflation, and in pets themselves. This may be helped by on September 29, 2021 by guest. Protected copyright. extreme cases a ventilation–perfusion scan. providing objective evidence, and if the history During a prolonged exacerbation the latter is suggests worsening symptoms after exposure to likely to show patchy areas of ventilation– a particular animal, skin prick testing or RAST perfusion mismatch and a normal scan should (radioallergosorbent)
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