guidelines on the management of cystic fibrosis in the adult - professor francesco blasi

Guidelines for the management of cystic fibrosis in the adult

Prof. Francesco Blasi, MD, FERSDepartment of Pathophysiology and Transplantation,

University of Milan, Italy

Head Cardio-Thoracic Unit and Cystic Fibrosis Adult Center,

Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico Milan, Italy

Disclosures

I have accepted grants, speaking and conference invitations from Angelini, AstraZeneca, Almirall, Bayer, Chiesi, Dompè, GSK, Guidotti-Malesci, Menarini, Novartis, Pfizer, Sanofi, Teva and Zambon.

I have had recent or ongoing consultancy with Angelini, AstraZeneca, Chiesi, GSK, Menarini, Mundipharma, Novartis, Teva, Zambon.

METHODS

• Demographic data were obtained from the ECFS PatientRegistry and from the Population Reference Bureau, and gross national incomes (GNIs) per capita in currentinternational dollars ($) were obtained from the World Bank

• According to availability of longitudinal data (i.e. data linked across the years at individual patient level) in the ECFS Patient Registry, coverage of their national CF population and GNIs per capita, 34 European countrieswere grouped into four groups (A, B, C and D)

• FORECASTS FOR 2025 IN 16 EUROPEAN COUNTRIES, WITH WELL-ESTABLISHED DATA COLLECTIONS, INDICATE THAT NUMBER OF CF PATIENTS WILL INCREASE BY 50%.

• THE NUMBER OF CF ADULTS WILL INCREASE BY 75%, A FINDING THAT MOSTLY RESULTS FROM THE TRANSITION OF CHILDREN TO ADULTS, WHEREAS THE NUMBER OF CF CHILDREN WILL SHOW A 20% INCREASE

Specific Management considerationsfor adults with CF

• Transition

• Adherance

• Psychosocial support

• Late diagnosis of CF

• Lung Transplantation

• End of Life Care

WHAT WE NEED TO CONSIDER• In CF parents become experts in caring for their child

and in their knowledge of the disease

• Parents play an important and time-consuming role in the health and welfare of their child with CF

• The move to an adult service is met with trepidation and fear from both the child and the parents

• Barriers to transition are being erected— consciously or unconsciously— not only by parents, but also by the adolescent and to a degree, from the cliniciansresponsible for their care


• Transition

• Adherance





CFF report 2014

Main psychosocial issues

• Family relationships

• Employment

• Education

• Anxiety and depression

• Relationships and issues of fertility and reproduction

• New diagnoses, such as diabetes, respiratory failure, oxygen requirement


• Transition

• Adherance





LUNG TRANSPLANTATIONTiming of referral in CF

FEV1 <30% (or rapid decline)

Females with advanced CF lung disease

CF lung disease with NTM / BCC infection

6-min walk test distance <400m

Development of PH in absence of hypoxic exacerbation

Clinical decline characterized by frequent exacerbations

Weill D. J Heart Lung Transplant 2015

Timing of listing in CF*

Chronic respiratory failure

With hypoxia alone (PaO2

<60mm Hg)

With hypercapnia (PaCO2

>50mm Hg)

Long-term non-invasive ventilation

Pulmonary hypertension

Frequent hospitalization

Rapid lung function decline

WHO Functional Class IV

• All adult CF centres should have ready access to a lung transplantation service and, when required, liverand renal transplantation services

• CF adult centres should have knowledge of, and haveaccess to, supportive ventilation, including (ECMO) or extracorporeal life support (ECLS)

• The adult with CF should remain under the primarycare of the transplantation team


• Transition

• Adherance





End of life care

• Adequate skills and facilities to deal with palliative care

• End of life care is ideally delivered in an adult centre

Should all adults be managed in an adult CF centre?

• The best model considered by the task force is the establishment of a separate adult service with a referralrelationship with pediatric centre

• Some centres have a different model merging adult and paediatric services into one programme.

• It is important to have appropriately trained physicians, ideally with a background in adult pulmonology asleaders for delivery of the service to adults with CF

Where should specialist adult CF centres be situated?

• Most of the mortality and morbidity in CF relates to the lungdisease

• The medical care of adults with CF should therefore be delivered by pulmonologists who have had specific training in CF

• The need for management of pulmonary complicationssuggests adult CF care is best based in adult pulmonologyservices

Is there an ideal size for a CF centre?

• The optimal size of an adult CF centre has not beenaddressed in the scientific literature

• ECFS standards of care: a designated specialist adultcentre should usually be a minimum of 100 patients(but not below 50 patients)

• No research to indicate if there is an optimal maximum size

How frequently should adults with CF be reviewed?

• Managing the chronic complications of CF can be very challenging for young adults

• Regular review every 2–3 months is suggested

• Measures to enhance communication betweenpatients, local healthcare providers and the CF centre should be strongly considered

WHAT DO WE NEED• The rapidly changing challenges of CF management

requires that the curriculum committee keeps pace with these changes with a continuing medicaleducation to meet the needs of healthcare workersdealing with CF

• A core curriculum has to be implemented and training centres selected and accredited by the ECFS and ERS with the same modalities applied for the HERMES programme

Core Competences

(procedural skills)

Core syllabus Curriculum

PG coursesHands-on workshops

E-learning modules

Other teaching materials

(books, videos, etc)

EUROPEAN EXAMINATION

TRAINING CENTERS(FELLOWSHIPS)

guidelines on the management of cystic fibrosis in the adult - professor francesco blasi

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