Carmel Moriarty

Contraception, communication and counseling for sexuality and reproductive health in adolescents and young adults with CF.

With survival now into the fourth decade and rapid growth of the adolescent and adult population of people with cystic fibrosis CF sexual and reproductive health issues are integral to the management of adolescents and adults with CF. Education and counseling for sexual health related issues must be included in the daily routine of CF care. With advances in genetic counseling, contraception, assisted reproductive technology and collaborative management adolescents and young adults with CF realizing their sexual and reproductive potentials safely and realistically can be possible .

Pregnancy and Cystic Fibrosis

The management of a pregnancy in a woman with cystic fibrosis is usually achieved with successful outcomes for mother and child with appropriate multidisciplinary care. The process begins prior to conception and requires frequent monitoring of the mothers respiratory status, level of glycaemic control and obstetric wellbeing. Recent reports have suggested that pregnancy can be managed without a persisting decrement in lung function beyond what may be expected in women with cystic fibrosis who are not pregnant. With the increasingly positive outcomes for people with cystic fibrosis, it is likely that more couples will chose to pursue pregnancy, cognisant of the risks and longer term issues for mother, child and family. This review will address the current issues in the management of pregnancy in women with cystic fibrosis.

Clinical profile of adult cystic fibrosis patients with frequent epidemic clones of Pseudomonas aeruginosa.

Background and objective:  Earlier reports suggested that Pseudomonas aeruginosa frequent epidemic clones circulating in cystic fibrosis (CF) centres had increased virulence. However, recent data show no consistent associations with virulence, and suggest attenuation of virulence in chronic infection. Changes to infection control programmes in relation to frequent epidemic clones should be based on their frequency, virulence across all age groups and mode of acquisition. The Australian epidemic strain‐1 (AES‐1) (or the Melbourne epidemic strain) and AES‐2 are common in CF clinics in mainland eastern Australia, but not in the environment. Both have shown increased virulence, but there are no data specifically in adults. This study examines the frequency and virulence of P. aeruginosa frequent epidemic clones in the adult CF clinic at Royal Prince Alfred Hospital, Sydney, Australia.

Effect of a short course of rhDNase on cough and mucociliary clearance in patients with cystic fibrosis

The aim of the study was to measure the effect of a short course of recombinant human deoxyribonuclease I (rhDNase) on ciliary and cough clearance in a group of cystic fibrosis patients, using a radioaerosol and gamma camera technique. Patients were initially randomized to receive either rhDNase (2.5 mg qd) or placebo. Following the measurement of baseline clearance, patients were given a 7‐day course of either rhDNase or placebo. The patient then returned on the seventh day for follow‐up clearance measurements. This was followed by a 2‐week washout period before the whole process was repeated with the alternative inhalation solution. On each of the study days, mucociliary clearance was initially measured for a period of 60 min (IC). This was followed by cough clearance (CC) measurements for 30 min, during which patients were requested to cough a total of 120 times. Post‐cough clearance (PCC) was then measured for a further 60 min.

Effect of Burkholderia cepacia infection in the clinical course of patients with cystic fibrosis: a pilot study in a Sydney clinic

Background: Colonisation with Burkholderia cepacia complex in patients with cystic fibrosis (CF) has been associated with adverse outcomes. The aim of the present study was to determine the actuarial survival of CF patients colonized with B. cepacia and to evaluate the efficacy of the Royal Prince Alfred Hospital segregation policy. A secondary aim was to characterize the specific genomovars and strains of B. cepacia isolated in an Australian clinic.

Pregnancy outcomes in the current era of cystic fibrosis care: a 15-year experience.

Background:  With improvement in clinical care and longer survival of patients with cystic fibrosis (CF), pregnancy has become commonplace. However, the impact of pregnancy on maternal health and fetal outcomes requires ongoing review.

Recombinant Activated Factor VII for Massive Hemoptysis in Patients With Cystic Fibrosis

Massive hemoptysis is a common complication in patients with cystic fibrosis (CF) and is associated with significant morbidity and mortality. Conventional treatment with antibiotic therapy and early bronchial artery embolization (BAE) is usually successful in achieving hemostasis in the majority of patients. Recombinant activated factor VII (rFVIIa), originally developed for use in patients with hemophilia, has emerged as a general hemostatic agent that is potentially useful in the management of many life-threatening bleeding conditions. In this article, we present four patients with CF lung disease and massive hemoptysis who were treated successfully with rFVIIa. We suggest that in patients with CF who present with massive hemoptysis, the use of rFVIIa can be considered in patients with refractory hemoptysis despite conventional therapy or as a temporizing therapy when BAE is not immediately available.

Granulomatous diseases in a patient with cystic fibrosis

We report the case of an adult with Crohns disease and pulmonary sarcoidosis on the background of cystic fibrosis (CF). There is a recognized association between Crohns colitis and CF, but cases of pulmonary sarcoidosis in CF are rare. There may be a pathogenic link between the two granulomatous disorders and CF with chronic immune stimulation leading to hyperimmunoglobulinemia, circulating immune complexes and subsequent granuloma formation.

Hypoglycaemia in cystic fibrosis: An analysis of a single centre adult cystic fibrosis clinic

BACKGROUND Hypoglycaemia in cystic fibrosis (CF) is known to occur during oral glucose tolerance tests (OGTT) and continuous glucose monitoring, however demographic, clinical and mechanistic data are limited. The aims of this study were to review patient electronic medical records (EMR) in order to 1) describe patient characteristics of a university teaching hospital CF clinic, 2) determine the prevalence of hypoglycaemia on OGTT and explore associations with demographic and clinical characteristics, and 3) explore patient reported symptoms suggestive of hypoglycaemia documented in the EMR. METHODS Adults who attended the RPA CF clinic between January 2009 to April 2016 were included in the study. The prevalence of hypoglycaemia on OGTT was determined and clinical and demographic data were compared to age, sex and glucose tolerance matched controls. Reported symptoms suggestive of hypoglycaemia documented in EMR were qualitatively explored. RESULTS Hypoglycaemia on OGTT was prevalent in 25 (3 fasting and 22 reactive) of 169 patients who had an OGTT. They were heavier, less likely to have pancreatic insufficiency and had a lower insulin response at 2-h. Another 14 patients reported symptoms suggestive of hypoglycaemia in their EMR. No patient appropriately suppressed insulin at 2-h on OGTT. CONCLUSIONS This study identified two potentially different presentations of hypoglycaemia occur in different clinic sub-populations. Knowledge gaps in the aetiology and triggers of hypoglycaemia remain.

Use of extracorporeal membrane oxygenation in cystic fibrosis in an Australian cystic fibrosis centre

Extracorporeal membrane oxygenation (ECMO) support is used in selected patients with cystic fibrosis (CF) as a bridge to transplantation. Our aim was to describe briefly treatment and outcomes of six CF patients who received ECMO. One patient received a lung transplant and another recovered from acute respiratory failure. Four died despite ECMO support. Lack of timely availability of suitable donor lungs and patient selection are contributing factors.