Posters / Journal of Cystic Fibrosis 22S2 (2023) S64–S199
Results: Three broad themes were identified during the 6 month maternity
leave period; neglect of self care (PP recovery, poor daily hygiene,
inadequate nutritional intake, dehydration, poor medication adherence),
life style (sleep pattern, sleep deprivation, reduced social life, changes to
spouse/ physical relationship), mental health (baby blues, exhaustion,
mood swings, loneliness, parent guilt, resentment, worry, self doubt, body
image). Considerations to new CF parents PP: nursing; home IVs, in-patient
weekend leave, home visits, flexible and remote appointments, medication
review, referral to psychosocial teams. Physiotherapy; non adjunct ACTs,
minimal nebuliser equipment to reduce washing demand, consider DPI
therapies or combining treatments, modified physical activity, MSK advice.
Dietetics; nutritional/diet support, weight management especially for
mothers breastfeeding, body image advice, diabetic control, hydration
options, bowel management.
Conclusion: A new found insight into motherhood gave all 3 MDT
members a new perspective of CF care postpartum. Babies are demanding
and become the focus of your day, leaving little time for self care. CF teams
should consider how to make treatment and ward admissions easier for the
patient, when the parents own health may no longer be the priority.
P389
Evolving gender identification within the cystic fibrosis population and
considerations on potential effects on lung function results
A. Darby1, A. Scholz1, A. Hiller2, P. Goble1, J. Duckers1, L. Speight1. 1University
Hospital of Llandough, All Wales Adult Cystic Fibrosis Centre, Cardiff, United
Kingdom; 2University Hospital of Wales, Physiotherapy, Cardiff, United
Kingdom
Introduction: The All Wales Adult Cystic Fibrosis Centre, a service with
∼330 patients, has noted a number identifying as transgender. Biological
sex influences prognosis in cystic fibrosis (CF), with suggestions oestrogen
may affect the pulmonary system. Sex registered at birth is used to calculate
predicted FEV1 and body composition values and chest binding can affect
lung function results.
Aims: Evaluate the demographics of those who have notified the adult CF
team that they identify as transgender and consider possible implications
for their CF care.
Methods: Clinical letters of those known to identify as transgender were
searched for terms such as “transgender,” “gender dysphoria,” “bind” &
previously used first name, and data collected on age, how they identify,
and any gender affirming treatments.
Results: Five adult patients were identified, with a paediatric patient due to
transition to the adult team soon. The average current age is 19.2 years old,
range 15 to 21 years. Four identified as trans men (female to male) and 2 as
trans women (male to female). Age transgender was first mentioned in
letters was predominantly between 14–16, with 1 having the first mention
at age 21, and 1 had moved from abroad at 19 already identifying as trans.
Half are on gender affirming hormone therapy. Chest binding was
mentioned in only 1 letter.
Conclusions: There is a significant population (1.7%) identifying as
transgender within our service compared to UK prevalence estimate of
0.7%. Surveys of UK centres will help identify if Wales is an outlier, or this is
a growing area. Impacts on health and lung function are unknown. Further
research is needed to explore effects of gender affirming treatments on
physiological parameters within CF. It is important to consider how we
record gender and sex at birth within medical records and assessments.
Involvement of patients will be vital.
P390
Ageing with cystic fibrosis: challenges for patients and team members
S. Braun1, H. Eyns1, S. Vincken1, E. De Wachter1, E. Vanderhelst1. 1UZ Brussel,
Jette, Belgium
Intro: Older(40+)people with cystic fibrosis(PWCF) were not expected to
survive into adulthood. Life expectancy has improved to an mean survival
age of50+ due to a multidisciplinary approach and modulator therapy.
Along with these new developments an improved quality of life(QoL) has
been observed. With an increased survival certain issues related to
longevity are likely to emerge.
S183
Method: A cross-sectional observational study was conducted in the UZ
Brussel (UZB) CF Center.
Results: 146 PWCF have a regular follow-up at the UZB.103 patients(70.6%)
are adult with a mean age of 33.3y. 24.3% of our adult CFpatients is 40+,
9.7% is50+ and 2.9% is60+. Mental health issues(64%), diabetes(40%),
osteopenia(28%)/osteoporosis(20%), arthropathy(28%) and hypertension
(20%). Other comorbidities(<10%) were cancer, hyperthyroidism and
Parkinson’s disease.
Discussion: The most common comorbidities in elderly are heart disease,
hypertension, cerebrovascular disease, diabetes, arthropathy and mental
health issues, some of which are also known to be related to CF. Our results
show that PWCF do develop conditions of the elderly. With the
introduction of the modulators we expect a shift from normal to
overweight adding to the risk of heart disease and diabetes. With an
increase in survival existential and ethical questions and challenges arise
for this first generation of elder PWCF and the CF team. All these changes
will affect both the patient and the CF team. The role of all the specialist
team members will change in the future. Besides the physical changes, the
psychologist will need to focus on the mental changes that come with
advancing age and will need to touch on more taboo themes such as sexual
dysfunctioning, menopause and memory problems.
Conclusion: PWCF develop conditions of the elderly. As survival continues
to increase, new challenges –physical and mental– will arise for the
patients and their families. It is up to the specialist CF team to move along
this new road.
P391
“I’m different”: reviewing outpatient psychology support for people of
colour in an inner London adult cystic fibrosis centre
G. Green1, E. Mckenzie-Howat1, N. Shafi1, D. Watson1. 1St Bartholomew’s
Hopsital, Respiratory, London, United Kingdom
Objectives: CF affects people from different ethnic backgrounds. People of
colour (PoC) may experience unique psychosocial challenges in living with
CF. Since the introduction of modulator therapies, and an associated fall in
inpatient admissions, outpatient Psychology has become an increasingly
important part of CF psychological care. The project aimed to investigate
whether outpatient Psychology was accessible to PoC. It also aimed to
consider themes discussed during therapeutic work with PoC, to help
inform future care priorities.
Methods: Data was extracted from routine Psychology databases and
annual review CF registry data. Accessibility was determined by whether
the proportion of PoC attending outpatient Psychology was equivalent to
the proportion who of PoC who had an annual review. Overarching
qualitative themes were extracted from Psychology session notes.
Results: The total number of patients seen by CF Psychology in 2021 was 44
and in 2022 was 64. More patients were seen in 2022 due to an increase in
staffing levels. The number of PoC seen in outpatient Psychology was 9
(20.45%) in 2021 and 10 (16.18%) in 2022. Table 1 shows further breakdown
of ethnic categories.
Table 1.
Number of people seen in outpatient Psychology by ethnicity (%)
White Asian Asian Asian Black Mixed
Bangladeshi Indian Pakistani African White/Black
Caribbean
2021 N (%) 35 (79.55) 4 (9.09) 2 (4.55) 2 (4.55) 0 (0) 1 (2.27)
2022 N (%) 54 (85.94) 6 (9.38) 1 (1.56) 1 (1.56) 1 (1.56) 1 (1.56)
In 2021 215 patients had an annual review, of which 31 were PoC (14.42%).
Data was not compared for 2022, because the annual review data was
incomplete at the time of analysis.
Themes of power and social context were key in outpatient Psychology
work with PoC. We identify the Power Threat Meaning Framework (BPS,
2018) as a potential tool to help understand the lived experience of PoC
with CF.
Conclusion: Outpatient Psychology was accessed proportionally by PoC in
our CF centre. Issues related to power, including epistemic injustice,
S184 Posters / Journal of Cystic Fibrosis 22S2 (2023) S64–S199

highlight the need for CF care initiatives that afford PoC opportunity to Bonferroni correction was used to analyze physical activity data based on
make sense of their experiences. gender.
Results: We were unable to reveal any significant associations between
P392 physical activity participation and scores on the GAD-7 or PHQ-8. We did
Medical and mental status of refugees with cystic fibrosis from Ukraine note that there were trends toward significant negative associations
C. Schwarz1,2, D. Kostiukova2, J.G. Mainz3,4, T. Erler1,5, H. Schütte6, between increased physical activity intensity with both GAD-7 and PHQ-8
P. Eschenhagen2. 1HMU-Health and Medical University Potsdam, Potsdam, scores. The trend of negative correlation with increasing physical activity
Germany; 2Klinikum Westbrandenburg, Division of Cystic Fibrosis, Potsdam, also existed with composite FIVE score.
Germany; 3Universitätsklinikum Brandenburg an der Havel, Abteilung Päd. Table 1.
Pneumologie, Allergologie und Mukoviszidosezentrum, Brandenburg an der Spearman rho correlation between Physical Activity and Mental Health Survey Scores
Havel, Germany; 4Universitätsklinikum der Medizinischen Hochschule
Brandenburg (MHB),, CF Zentrum Westbrandenburg, Brandenburg an der Physical Activity Spearman GAD- PHQ- FIVE CFQ-R CFQ-R
Havel, Germany; 5Klinikum Westbrandenburg, Klinik für Kinder- und rho 7 8 Composite Physical Respiratory
Jugendmedizin, Potsdam, Germany; 6Ernst von Bergmann Klinikum, Low-Intensity Correlation −.110 .017 −.143 −.300 .007
Pneumologie und Beatmungsmedizin, Potsdam, Germany MET-min/ week Coefficient
Sig. .608 .936 .506 .889 .973
Objectives: The war in Ukraine has led to a flight of people with CF. Since N 24 24 24 24 24
the beginning of the war on February 24, 2022, people with CF have come Moderate Intensity Correlation −.228 −.190 −.215 .107 .143
to Potsdam, Germany, seeking medical, social and psychological support. MET-min/ week Coefficient
Methods: Mental health screening, psychological interviews and medical Sig. .284 .375 .314 .619 .240
records were used to elucidate the medical and mental status of Ukrainian N 24 24 24 24 24
people with CF. Vigorous Intensity Correlation −.314 −.374 −.296 .427** .328
Results: Currently, 10 patients and their families have been admitted to the MET-min/ week Coefficient
Sig. .135 .071 .160 .037 .118
CF Center in Potsdam. In total 24 people including family members and one
N 24 24 24 24 24
dog from Ukraine have been treated in our clinic since February 24, 2022.
Total MET-min/ Correlation −.241 −.157 −.261 .184 .162
The mean age of people with CF was 21.6 (range: 4–36 years). 7 patients week Coefficient
were >18 years and 3 <18 years. FEV1 was 55.0 percent predicted (range: Sig. .257 .462 .219 .388 .451
29–95pp). 8/10 (80%) of the patients were at the time of initial contact N 24 24 24 24 24
bronchopulmonary exacerbated and were receiving immediately antibiotic
treatment. In 10/10 (100%) patients, the standard therapy according to
Conclusion: Although no significant associations between physical activity
guidelines was not established in their home country. Also in 10/10 (100%)
and mental health scores were found, there was a trend of negative
patients, recommended diagnostics have not been performed in their
associations with increasing effect size. This finding suggests that future
home country. As far as assessable, 10/10 (100%) were traumatized by flight
efforts aimed at increasing physical activity in CF patients should consider
and war and had very high scores on the PHQ-9 (mean 15) and the GADS-7
mental health status including fear of viral illness.
(mean 19). In all patients, standard therapy was established upon arrival at
the CF center and in 8/10 (80%) patients, highly efficient CFTRm therapy
P394
with elexacaftor/tezacaftor/ivacaftor and Ivacaftor could be started.
Comparisons of the personal well-being score (PWS) and health-
Conclusion: In total, since the beginning of the war in Ukraine, 10 people
related quality of life for people with cystic fibrosis before and during
with CF and 14 relatives have been successfully provided with medical and
the COVID-19 pandemic
psychosocial help and care. In order to be able to care for these highly
traumatized and seriously ill people with CF and their relatives, R. Altabee1,2, S.B. Carr3,4, J. Abbott5, R. Cameron1,6, D. Office7,
interdisciplinary cooperation - also with institutions outside the CF N.J. Simmonds4,7, J.A. Whittey1,6,8, D. Turner1, G. Barton1. 1University of East
Center - will be necessary. Anglia, Health Economics Group, Norwich Medical School, Norwich, United
Kingdom; 2King Saud Bin Abdulaziz University for Health Sciences, College of
P393 Applied Medical Sciences, Jeddah, Saudi Arabia; 3Royal Brompton Hospital,
Physical activity participation and mental health status of patients Department of Paediatric Respiratory Medicine, London, United Kingdom;
4
with cystic fibrosis during the COVID-19 pandemic: a single centre Imperial College London, National Heart and Lung Institute, London, United
experience Kingdom; 5University of Central Lancashire, School of Psychology, Preston,
United Kingdom; 6National Institute for Health Research (NIHR) Applied
S. Delgado1, J. Russo2, H. Vincent3, D. Fedele4. 1University of Florida,
Research Collaboration (ARC) East of England (EoE), Cambridge, United
Pediatrics, Gainesville, United States; 2University of Virginia, Medicine,
Kingdom; 7Royal Brompton Hospital, Adult Cystic Fibrosis Center, London,
Charlottesville, United States; 3University of Florida, Physical Medicine &
United Kingdom; 8Evidera, London, United Kingdom
Rehabilitation, Gainesville, United States; 4University of Florida, Clinical &
Health Psychology, Gainesville, United States Background: Well-being scores are used across Europe; the personal well-
being score (PWS) is a 4-question survey used by the Office of National
Objective: The COVID-19 pandemic has posed a significant mental health
Statistics (ONS) in the UK annually on the general population. It asks about
challenge to individuals worldwide. Little is known about the association
satisfaction, happiness, anxiety and feeling worthwhile, scored from 0–10.
between mental health and physical activity participation in patients with
The COVID-19 pandemic impacted many; especially those with cystic
Cystic Fibrosis (CF) who are encouraged to exercise as part of their
fibrosis who were at high-risk and asked to isolate. This study aimed to
treatment regimen. This exploratory study aimed to determine physical
evaluate the differences in well-being and health-related quality of life
activity levels, mental health status (anxiety and depression symptoms)
(HRQoL) pre and during the pandemic.
and fear of illness (Fear of Illness and Viral Evaluation; FIVE) score among a
Method: A UK observational pilot study collected data ( pre-pandemic,
sample of CF patients from our academic health center.
2016–18) from 5 adult CF centres. The during-pandemic cohort was
Methods: A cross-sectional survey study was performed between February
collected from a single adult centre (2020–21). The surveys contained 3
1st 2021 to May 30th, 2021 with 24 CF patients ages ≥ 12 years who
measures; PWS and 2 HRQoL measures: EQ-5D-5L & the CF Quality of Life
completed a series of validated questionnaires exploring mental health
(CFQoL).
status (GAD-7, PHQ-8, CFQ-R) including fear of viral illness (FIVE) and
Results: The pre (n = 66) and during-pandemic (n = 213) cohorts had 47%
physical activity participation (IPAQ-7). Spearman rho analysis was used to
vs. 53.1% females; a median age of 35.5 vs. 36.5 yrs, respectively. No
detect associations between physical activity in units of MET-min/week
significant difference was observed between both cohorts for PWS (table).
and mental health scores. Independent Kruskal-Wallis analysis with
EQ-5D-5L’s index score, Visual Analogue Scale, and CFQoL domains also