Pulmonary infections are the major cause of mortality and morbidity in cystic fibrosis (CF); patients frequently have to take antibiotics which often cannot be given orally or by aerosol but have to be administered intravenously. In order to reduce the number of venepunctures, totally implanted venous access devices (TIVAD) or Ports have been used to administer antibiotics and other infusions.

The use of Port systems has been increasing in recent years, especially for those patients requiring frequent intravenous treatments. Having a TIVAD in place makes venous access quicker and also reduces trauma, suffering and pain. However, there are important complications associated with TIVADs which can be early (pneumothorax, arterial puncture, severe bruising) or late (infections, thromboembolic complications and occlusion).

Although the use of TIVADs in CF is increasing, there is little CF-specific literature available on the epidemiology and risk of TIVAD complications. Also, literature is scarce about clinical criteria for deciding to insert a TIVAD. Therefore, so far clinical decisions were based mainly on experiences of TIVAD use in other diseases, such as cancer.

With this prospective observational study we will survey a large population of Italian CF people with TIVAD in order to: collect data about current clinical conditions of CF people with TIVAD; investigate about clinical criteria that led to the decision of positioning a TIVAD; observe the possible onset of late complications.

To be included in the study, subjects need to fulfil the following requirements:

• Diagnosis of CF, made accordingly to the CF Foundation Guideline;
• Ability to consciously express owns informed consent, if aged 18 or more. If minor, presence of at least one legally authorised parent able to consciously express informed consent;
• Have a TIVAD implanted;
• Have no current complications in the TIVAD (infection, thromboembolic complications or occlusion) Outcome Measures

Measured outcomes will be:

1. Incidence of TIVAD infections (Port pocket infection, Cutaneous site infection,Catheter-related infection;
2. Incidence of TIVAD thrombosis;
3. Incidence of TIVAD occlusion.

The observational phase will last 12 months for each subject involved

Inclusion Criteria:

• Diagnosis of CF, made accordingly to the CF Foundation Guideline
• Ability to consciously express owns informed consent, if aged 18 or more. If minor, presence of at least one legally authorised parent able to consciously express informed consent
• Have a TIVAD implanted
• Have no current complications in the TIVAD (infection, thromboembolic complications or occlusion)

People with CF and with a TIVAD implanted