Luigina Longo,  Susi Speri,  Gemma Sartori,  Roberta Doro
Cystic Fibrosis Center of Veneto, Verona, Italy

VAGINAL DISORDERS IN CF WOMEN: PRELIMINARY RESULTS OF A NURSE LED STUDY

The clinical course of cystic fibrosis heavily conditions the quality of life of affected people. In order to control respiratory infections, CF patients often need frequent courses of high doses antibiotics.
Little attention has been given so far by the literature to the side effects of antibiotic therapy on the genital apparatus of women with CF. Because of the considerable use of antibiotics CF women are likely to be more susceptible to genital infective disorders.
The aim of this study is:
-to provide data on the incidence of vulvo-vaginal symptoms in women with CF;
-to assess the influence of such symptoms on patients  quality of life.
The study was started on July 2001. Data was obtained through a questionnaire administered by staff nurses to CF female patients aged 10 or more, followed by the CF Centre of Verona.
The questionnaire required patients to report:
-the number of antibiotic courses in the last year and the route of administration;
-if they had vulvo-vaginal disorders during the courses;
-if and how the reported disorders affected their everyday life;
-what kind of measures were taken.
Preliminary results:
Ninety-four patients were examined. All the patients received antibiotic therapy in the last year (78% both per os and I.V.). 58% of them had more than one antibiotic course per month. (range 13-24 courses per year).
61% of the women reported vulvo-vaginal disorders.
The statistic analysis shows that I.V. administration of antibiotics caused a higher incidence of vulvo-vaginal symptoms (67% of patients who used I.V. administration vs. 27% of patients who used only oral and aerosolized administration, Fisher test p=0.007).
The effects of such symptoms were worse on sexual life (23%) than on working life (7%).
Conclusions:
The incidence of vulvo-vaginal disorders in CF women receiving antibiotic therapy is high. Specific nursing measures should be evaluated in order to prevent and relieve such disorders.

G.Esmond⁽¹⁾,  M Butler⁽²⁾,  A McCormack⁽²⁾,  D Empey⁽²⁾
(1)City University, London, ⁽²⁾ Barts and The London adult cystic fibrosis centre, UK

COMPARISON OF HOSPITAL VERSUS HOME INTRAVENOUS ANTIBIOTICS IN ADULTS WITH CYSTIC FIBROSIS

The concept of home intravenous antibiotic therapy for acute respiratory exacerbations is rapidly becoming popular, in response to economic pressures and patient choice.
Comparison of hospital and home intravenous antibiotic therapy in adults with cystic fibrosis for clinical outcomes and quality of life was studied using a quasi-experimental design. A pre and post test was used to measure clinical outcomes of Forced Expiratory Volume in one second (FEV1), Forced Vital Capacity (FVC), oxygen saturations (SaO2), Body Mass Index (BMI) and quality of life using the Cystic Fibrosis Quality of Life (CFQoL) questionnaire.
Thirty adult cystic fibrosis patients (15 hospital and 15 home) were recruited to the study once they had chosen where they undertook their treatment. There were 6 females and 9 males in the hospital group and 9 females and 6 males in the home group which was on average 4 years older. When the groups were compared for clinical outcomes the hospital group showed greater improvement in Forced Vital Capacity (p<0.01). There were no differences in quality of life when the hospital and home groups were compared (p>0.05), although changes achieved with treatment showed differences in five of the nine quality of life domains.
Lung function improved more in the hospital group, suggesting that acute respiratory exacerbations were not as effectively treated at home. Although there were no differences in quality of life between the home and hospital groups, the Cystic Fibrosis Quality of Life (CFQoL) questionnaire was able to detect transient changes in health status during the course of intravenous antibiotics.
If the patients right to choose where they receive treatment is to be supported, further research is required to determine the reasons for home care being less clinically effective in treating acute respiratory exacerbations in cystic fibrosis patients.

JJ Cottrell,  EF Burrows,  DP Heaf
Royal Liverpool Childrens Hospital, Alder Hey, Liverpool, UK

ECHOGENIC BOWEL ON ULTRASOUND SCAN LEADING TO ANTENATAL DIAGNOSIS OF CF : THE ROLE OF THE CF TEAM

In the past 3years we know of 4 cases of echogenic bowel detected at antenatal ultrasound (US) scan at 20/40 which have led to CF screening for the parents and amniocentesis of the foetus. We present the 4 cases and their different outcomes.
#1 US scan 20/40  showed echogenic bowel. Mother DF508, Father DF508, foetus DF508/DF508. Genetics referral and CF Team informed. Outcome : male infant born 29weeks +3 by emergency Caesarian section due to pre-eclamptic toxaemia in the mother. Meconium Ileus, ileostomy on Day 9. 
#2 US scan 22/40 showed echogenic bowel. Mother DF508, Father DF508, foetus DF508/DF508. Genetics referral and CF Team informed by Obstetric team. Outcome: Termination of pregnancy. 
#3   US scan 20/40 showed echogenic bowel. Father DF508, Mother showed to be negative of 20  mutations, foetus DF508/?  Referral to Genetics declined and referral to CF team not offered. Outcome: female born term, spontaneous delivery with meconium stained liquor. Baby presented 12/52 Failure to thrive and chest symptoms.
#4 US scan 20/40 showed echogenic bowel. Mother DF508, Father DF508, foetus DF508/DF508. Genetic referral made but CF team refferal not offered. Parents self-referred to CF team.
Outcome: male infant born 38weeks+2. MI, ileostomy day 2. 
All parents were seen by CF consultant and  CF nurse specialist and given a detailed explanation of CF; daily treatment, prognosis, impact on the family, meconium ileus and its management. For those pregnancies which continued, the CF nurse maintained regular contact offering support and information. The CF nurse has a key role in the support of the family and liaison between the Teams involved.It is the CF nurse who provides continuity for the parents in the antenatal and postnatal period. 
Improved communication with the Specialist Midwives will inform them that the CF Team is available to provide a service in the antenatal period and that this can be offered to any couple in this situation.