S. Doe

Provision of contraception services and advice for women with cystic fibrosis

Background and methodology As the prognosis of patients with cystic fibrosis (CF) improves, issues of sexual health, fertility, pregnancy and contraception are increasingly important. In order to plan the provision of a contraception and sexual health service for women with CF we studied their sexual and reproductive history, their current usage of contraception, the sources and quality of advice they had received, and their particular needs using a confidential questionnaire sent to all women over 16 years of age attending a regional CF centre. Results Of 55 women (mean age 29.7 years) surveyed, 42 (76%) responded. Thirty-three women (79%) were sexually active and 13 (31%) had experienced 19 pregnancies, five (26%) of which were unplanned. Only half of the women who responded were using contraception. No woman used female sterilisation, the progestogen implant, intrauterine system (IUS) or copper-bearing intrauterine device (IUD) for contraception. Twenty-six (62%) women reported not having received contraceptive advice specific to CF and 24 (57%) said that they had not been warned about the potential interaction between broad-spectrum antibiotics and the combined pill. Discussion Women with CF have a relatively high rate of unplanned pregnancy and do not receive optimal advice or use the full range of contraceptive methods. CF teams lack training in contraception and contraceptive services may not have a detailed knowledge of CF and its complications. New strategies are needed to focus the knowledge and skills of both teams in providing better services for women with CF.

Microbiological profiles of sputum and gastric juice aspirates in Cystic Fibrosis patients.

Gastro-Oesophageal Reflux (GOR) is a key problem in Cystic Fibrosis (CF), but the relationship between lung and gastric microbiomes is not well understood. We hypothesised that CF gastric and lung microbiomes are related. Gastric and sputum cultures were obtained from fifteen CF patients receiving percutaneous endoscopic gastrostomy feeding. Non-CF gastric juice data was obtained through endoscopy from 14 patients without lung disease. Bacterial and fungal isolates were identified by culture. Molecular bacterial profiling used next generation sequencing (NGS) of the 16S rRNA gene. Cultures grew bacteria and/or fungi in all CF gastric juice and sputa and in 9/14 non-CF gastric juices. Pseudomonas aeruginosa(Pa) was present in CF sputum in 11 patients, 4 had identical Pa strains in the stomach. NGS data from non-CF gastric juice samples were significantly more diverse compared to CF samples. NGS showed CF gastric juice had markedly lower abundance of normal gut bacteria; Bacteroides and Faecalibacterium, but increased Pseudomonas compared with non-CF. Multivariate partial least squares discriminant analysis demonstrated similar bacterial profiles of CF sputum and gastric juice samples, which were distinct from non-CF gastric juice. We provide novel evidence suggesting the existence of an aerodigestive microbiome in CF, which may have clinical relevance.

A new era in the treatment of cystic fibrosis.

#### Key points Cystic fibrosis (CF) is the most common, life-limiting, autosomal-recessive genetic disease. In the UK, it affects about 10,000 people (one in 2,500 live births).[1][1] The -traditional treatments available for patients with CF focus on the consequences of this genetic abnormality.

Ivacaftor and symptoms of extra-oesophageal reflux in patients with cystic fibrosis and G551D mutation

Background Extra-oesophageal reflux (EOR) may lead to microaspiration in patients with cystic fibrosis (CF), a probable cause of deteriorating lung function. Successful clinical trials of ivacaftor highlight opportunities to understand EOR in a real world study. Methods Data from 12 patients with CF and the G551D mutation prescribed ivacaftor (150 mg bd) was collected at baseline, 6, 26 and 52 weeks. The changes in symptoms of EOR were assessed by questionnaire (reflux symptom index (RSI) and Hull airway reflux questionnaire (HARQ)). Results Six patients presented EOR at baseline (RSI > 13; median 13; range 2–29) and 5 presented airway reflux (HARQ > 13; median 12; range 3 to 33). Treatment with ivacaftor was associated with a significant reduction of EOR symptoms (P < 0 ∙ 04 versus baseline) denoted by the reflux symptom index and Hull airway reflux questionnaire. Conclusion Ivacaftor treatment was beneficial for patients with symptoms of EOR, thought to be a precursor to microaspiration.

Current characteristics, challenges and coping strategies of young people with cystic fibrosis as they transition to adulthood

ABSTRACT This study provides detailed data on the current characteristics, perceptions and outcomes of 45 young people with cystic fibrosis (CF) as they transition into adulthood. Although many had severe disease, they generally coped well, found attendance at a transition clinic helpful and welcomed the increased independence of an adult healthcare environment. Levels of psychological distress were low with only 15.6% having anxiety and 6.7% depression. The main psychological coping strategy used was optimistic acceptance. Overall, most remained stable after transfer but 33% had some decline in lung function and 9% in nutritional status, requiring intensification of treatment. They had high levels of satisfaction with their relationships and life situations and 76% were in employment or education. These results are encouraging and as life expectancy improves, young adults with CF are coping well with transition into adulthood.

Breath-holding in a marijuana smoker.

It is vital to ask about illicit drug smoking in the respiratory history as marijuana smoking augments the detrimental effects of tobacco. We describe the case of a 28 year old marijuana smoker who developed a pneumothorax during a breath-holding competition. Pneumothorax is a common clinical entity that every physician should be aware of how to manage and lifetime risk is considerably increased by smoking and in exposure to barotrauma.

A service evaluation of an integrated model of palliative care of cystic fibrosis.

Background: Patients with advanced cystic fibrosis have severe symptoms with a complex trajectory of exacerbations and recovery. They are often awaiting lung transplantation, and many die without receiving specialist palliative care. Aim: We introduced an integrated model whereby palliative specialists joined the cystic fibrosis team to provide palliative care in parallel with standard care. Design: A service evaluation of this model of care was undertaken in a prospective case series documenting symptoms and outcomes, the views of the cystic fibrosis team and the experience of the palliative specialists. Setting/participants: Over 3 years, 28 (10%) of 282 patients attending the cystic fibrosis centre had specialist palliative care. Results: They had advanced lung disease (mean forced expiratory volume in 1 s (FEV1) = 0.86 L (25% predicted)), and 17 died: 6 were on a transplant waiting list at death; 10 were unsuitable and 1 died post transplantation. All who died over these 3 years had specialist palliative care. Four patients had successful transplants. Assessment showed a high prevalence of breathlessness, cough, pain, vomiting and fatigue, with a significant impact on daily life. The cystic fibrosis team rated this model of care highly, felt that palliative care should be members of the team, and thought that patients had found it helpful. The palliative specialists gained knowledge of cystic fibrosis, found it beneficial to meet patients earlier in the disease, and identified unmet needs in managing bereavement and the effects of deaths on other patients with cystic fibrosis. Conclusion: This model has been successful in overcoming the difficulties in access to specialist palliative care for patients with cystic fibrosis.

Blood in a chest drain

A 21-year-old male with Ehlers-Danlos type IV (EDS IV) presented to the emergency department with sudden onset right-sided pleuritic chest pain. On examination, some of the typical features of EDS IV including large eyes and small chin were noted, although other features such as sunken cheeks, thin nose and lips, and lobeless ears were not noted [1]. His respiratory rate was 20 breaths·min−1, saturations were 93% on air increasing to 98% on 28% oxygen via a venturi mask. Pulse was 115 beats·min−1 in sinus rhythm and his blood pressure was 112 over 64 mmHg. There was decreased air entry in the right hemithorax, with decreased vocal resonance. The trachea was central and there was no surgical emphysema. ### Task 1 1. What is the best description of the chest radiograph (fig. 1)? Figure 1 Chest radiograph on admission. 1. A right-sided tension hydro-pneumothorax 2. A right-sided hydro-pneumothorax 3. A right-sided cavitatory pneumonia 4. A right-sided pneumo-peritoneum 2. What would the next therapeutic step be? 1. Simple observation and high flow oxygen 2. Aspiration of pneumothorax 3. Insertion of a small 12-French bore Seldinger chest drain 4. Insertion of a large-bore chest drain ### Answer 1 The chest radiograph shows a right-sided hydro-pneumothorax and the next therapeutic step would be insertion of a small 12-French bore Seldinger chest drain. Local anaesthetic was instilled into the second intercostal space and it was possible to aspirate air via the green needle. A 12 French intercostal drain was inserted using the Seldinger technique with no immediate complications and connected to an underwater seal bottle which …

Nontuberculous mycobacteria in gastrostomy fed patients with cystic fibrosis

Multi-drug resistant Mycobacterium abscessus complex (MABSC) is a form of Nontuberculous mycobacteria (NTM) of special, international concern in Cystic Fibrosis (CF). We hypothesised that gastric juice and percutaneous endoscopic gastrostomy (PEG) feeding devices might yield MABSC isolates. Gastric juice and sputa from sixteen adult PEG fed CF patients and five replaced PEG tubes were studied. Bacterial and fungal isolates were cultured. Mycobacterium were identified by rpoB, sodA and hsp65 gene sequencing and strain typed using variable number tandem repeat. Bacteria and/or fungi grew from all gastric juice, sputa and PEG samples. MABSC were detected in 7 patients. Five had MABSC in their sputum. Two had an identical MABSC strain in their sputum and gastric juice and one had the same strain isolated from their PEG tube and sputum. Two patients who were sputum sample negative had MABSC isolated in their gastric juice or PEG tube. MABSC were therefore identified for the first time from a gastric sample in a minority of patients. We conclude that gastric juice and PEG-tubes may be a potential source of MABSC isolates in CF patients, and these findings warrant further study.

Travelling abroad with cystic fibrosis: Assessment of risks and healthcare requirements

There is limited information on the risks and healthcare requirements of patients with cystic fibrosis (CF) undertaking travel abroad. Of 100 patients (mean age 24.7 years, mean FEV1 57.3 %predicted) attending a UK adult CF Centre, 96% had travelled abroad but 14% now limited travel on medical advice. They travelled frequently and widely, often undertaking adventurous activities on holidays, but because of the costs involved, 18% travelled without travel insurance and 23% with insurance which did not cover CF. Of those who had ever had an illness abroad 10% had a CF-related illness (7 chest infection, 2 dehydration, 1 pancreatitis) and 12% a non-CF-related illness (4 sunburn, 3 gastroenteritis, 3 ear infection, 1 fall, 1 gastro-oesophageal reflux). There is a wide range of disease severity and assessment of the medical risks and the travel insurance premium to be charged should be based on the individuals health status rather than generically on the basis of a diagnosis of CF.