Psychosocial Distress and Knowledge Deficiencies in Parents of Children in Ireland Who Carry an Altered Cystic Fibrosis Gene.

Significant gaps have been identified in parental understanding of CF newborn screening and the consequences of carrying an altered CF gene. Seven potential causes of psychosocial adversity arising from false positive newborn screening for CF have been identified. The current study aimed to increase parents understanding of CF, reduce their levels of stress, and investigate psychosocial adversity arising from false-positive screening. This national study was run over one year in the Republic of Ireland. Parents were recruited for the study following a diagnostic sweat test confirming their child carried a single altered CF gene. Parents were randomly assigned into a control and intervention group, with those in the intervention group receiving a carefully designed information pack. All parents took part in semi-structured interviews. Parents (n = 16) who received an information pack had significantly higher CF knowledge scores than parents (n = 16) in the control group. 66% of parents in the control group misunderstood the health implications of carrying an altered CF gene, no parents in the intervention group had the same misunderstanding. There was no significant difference in stress scores between the groups. Parents of infants who had more than one sweat test due to insufficient sweat quantity had higher overall stress percentiles (50%), than parents of infants who had one sweat test (30%), indicating greater parental stress. The combination of written and audio-visual information contained in the information pack successfully increased parents comprehension of CF. The study also evaluates the potential for psychosocial adversity following false positive newborn screening for CF.

Confidence and understanding among general practitioners and practice nurses in the UK about diagnosis and management of COPD.

In order to assess the confidence of healthcare professionals in diagnosing and managing COPD telephone interviews were conducted with 60 practice nurses and 46 general practitioners (GPs) in 2001 and 61 nurses and 39 GPs in 2005. The nurses all ran respiratory clinics. 80% of GPs were confident about diagnosing COPD and this had increased from 52% in 2001. Fifty five percent of nurses were confident and there was no change from 2001. In 2005, 79% of GPs and 70% of nurses were confident about differentiating asthma and COPD. Smoking history, breathlessness, age of onset, lack of response to asthma therapy and cough were reported as features differentiating COPD from asthma. Most respondents stated that spirometry is essential to diagnose COPD and in 2005 nearly all practices had access to a spirometry service. GPs were more confident about interpreting spirometry results in 2005 than nurses and their confidence had increased significantly from 2001. In 2005, nearly all respondents had heard of pulmonary rehabilitation, and significantly more had a programme in their area in 2005 than 2001 (69% vs. 49% p=0.05). Fifty four percent of GPs were confident about which patients to refer for long term oxygen therapy in 2005 but nurses were less confident. There had not been any significant change between 2001 and 2005. In 2005 only 35% of respondents had access to a pulse oximeter. When presented with case scenarios, GPs self-reported confidence was not reflected in their diagnoses or investigation and management strategies and they seem to favour cardiac over respiratory diagnoses.

Abnormal ventilation scans in middle-aged smokers. Comparison with tests of overall lung function.

We have assessed the uniformity of regional ventilation during tidal breathing using continuous inhalation of krypton-81m in 43 male, lifelong nonsmokers and 46 male, current cigarette smokers (mean daily consumption 24.1 cigarettes/day) between 44 and 61 yr of age and with mild or no respiratory symptoms. All subjects had normal chest radiographs. The results of the ventilation scans were compared with tests of overall lung function (spirometry, maximal expiratory flow-volume curves, and single-breath N2 test). Diffuse abnormalities of the ventilation scan were found in 19 (41%) of the 46 smokers but in none of the nonsmokers. Focal abnormalities were found in 7 smokers and 3 nonsmokers. Smokers showed the expected abnormalities in overall lung function (reduced FEV1 and VC, increased single-breath N2 slope, and closing volume), but in individual smokers there was only a weak relation between the severity of abnormality of overall lung function and an abnormal ventilation scan. Abnormal scans could be found when overall lung function was normal and were not invariably found when significant abnormalities in FEV1/VC or N2 slope were present. There was no relation between the presence of chronic expectoration and an abnormal scan. The prognostic significance of an abnormal ventilation scan in such smokers remains to be established.

Postural changes in rib cage and abdominal volume-motion coefficients and their effect on the calibration of a respiratory inductance plethysmograph.

Volume-motion coefficients were determined for the rib cage and abdomen in normal human subjects in upright, supine, and semirecumbent postures by the isovolume calibration technique of Konno and Mead (J Appl Physiol 1967; 22:407-422, using the respiratory inductive plethysmograph (RIP) to measure displacements of rib cage and abdominal walls. Volume motion coefficients changed systematically with posture; those for the rib cage were smallest in the upright posture, and for the abdomen, greatest in the upright posture. These volume motion coefficients were then used to estimate tidal volume during resting breathing in the different postures, and compared with estimates of tidal volume derived from calibration by the change in posture technique reported by Sackner and coworkers (American Review of Respiratory Disease 1980; 122:867-871). Estimates of tidal volume derived from RIP signals using both calibration techniques were compared with independently measured spirometric volume changes. Errors in tidal volume averaged 6% with the isovolume technique and 9 to 23% with the change in posture technique (depending upon whether the calibrating postures were upright, supine or semirecumbent supine). The larger errors with the change in posture calibration method are attributable to both the change in volume motion coefficients with posture and the change in distribution of tidal volume between rib cage and abdomen compartments with change in posture.

The effects of nebulized salbutamol on lung function and exercise tolerance in patients with severe airflow obstruction.

The effects of 10 mg of nebulized salbutamol on lung mechanics and exercise tolerance in 10 patients with severe airflow obstruction are described. All patients were previously considered to have irreversible airflow obstruction as demonstrated by little or no improvement in expiratory flow rates either during a corticosteroid trial or after inhalation of 100 micrograms of isoprenaline. There was a small improvement in expiratory flow rate after nebulized salbutamol but the greatest increases were seen in slow vital capacity, inspiratory capacity, inspiratory flow rates, dynamic compliance during tidal breathing, mid-inspiratory and expiratory pulmonary resistance and exercise tolerance. The mechanism of these effects and their therapeutic significance is discussed.

Domiciliary nebulised salbutamol solution in severe chronic airway obstruction.

Nine patients with severe chronic airway obstruction secondary to chronic bronchitis and emphysema all preferred nebulised salbutamol solution to placebo in a double-blind controlled trial. Four of the patients who had previously received domiciliary nebulised salbutamol failed to complete the placebo period, though all completed the active period. Five others improved subjectively on active therapy, and showed a significant improvement in morning and evening peak flows. Symptom scores for breathlessness, wheezing, and sputum production were lower in the active treatment period and standard aerosol usage fell, although these changes might have been due to chance. Patients with severe chronic airway obstruction who do not respond to conventional bronchodilator therapy should be considered for this form of treatment.

Chronic eosinophilic pneumonia complicating long-standing rheumatoid arthritis.

A 54-year-old patient with long-standing rheumatoid arthritis developed chronic eosinophilic pneumonia in association with a relapse of his arthritic condition. There was a rapid and complete response to oral steroid therapy.

Hydatid disease presenting as tension pneumothorax.

The case of stockfarmer with hydatid disease which presented as a left tension pneumothorax is described. The diagnosis was not made until the patient underwent a thoracotomy for a complicating empyema.