Assessment of real life and trained inhalation of Tobramycin Inhalation Powder (TIP) and Tobramycin Inhalation Solution (TIS) TIPTIS study.

Tobramycin inhalation powder (TIP) is recently introduced in treating
Pseudomonas aeruginosa (Pa) infection in Cystic Fibrosis (CF) patients. Higher
rates of sputum concentration are found compared to tobramycin inhalation
solution. High inhalation flows are likely to lead to more oropharyngeal
deposition of tobramycin. Compared to tobramycin inhalation solution (TIS),
more patients report cough after the inhalation of TIP. Our hypothesis is that
patients inhale TIP forceful, and that this leads to cough. A slow and deep
inhalation should reduce cough and thereby allows a more effective completion
of the full inhalation maneuver. We also hypothesize that patients inhale
with a wide variation of tidal volume when inhaling from a nebulizer.

Primary objective of the study is to record TIP inhalations on video at home to
study the association between inhalation manoeuver and cough.
Secondary objectives are: To study the inhalation maneuvers of patients using
TIP in the home situation; to determine for TIP whether fast inhalations are
associated with cough; to determine for TIP whether it is feasible to instruct
patient to do a slow and deep inhalation; to determine the duration of the
inhalation of TIP; to determine the tidal volume pattern while patients are
inhaling from a conventional nebulizer; and to record inhalation profiles of
TIP and nebulizer inhalations for later computer simulations using fluid
dynamic modeling of aerosol deposition.

This is a prospective study. Patients will be observed on video using TIP and
nebulizing NaCl solution in the home situation. During two home visits, every
visit 4 inhalations of TIP will be recorded on video, and between the visits 3
unsupervised inhalations will be recorded. During the second visit patients
will be asked to inhale either the first or second pair of tobramycin capsules
forcefully using a cross-over design.

Patients will be visited at home twice to record inhalations on video and
measure inhalation flows. The second visit patients will be asked to nebulise
NaCl solution for 10 minutes and to inhale tobramycin forcefully twice. Between
the home visits patients will be asked to record 3 unsupervised inhalations on
video. The video registrations and flow recordings are not invasive.
Participating children are used to nebulisation or inhalation therapy. During
forceful inhalations cough might be triggered, if this cough occurs we expect
it to be of short duration. Patients and parents will get feedback on the TIP
inhalation technique during the routine outpatient visit following the study,
which is likely to improve efficacy of the inhalation therapy.