Rhinosinusitis and nasal polyps in adults with Cystic Fibrosis

The pathophysiology of Cystic Fibrosis predisposes patients to the development
of sinonasal disease, i.e. rhinosinusitis and polyposis nasi. Dysfunction of
the chloride transport channel also results in viscous mucus in the upper
airways, which leads to bacterial overgrowth and infection. However, little is
known about the actual prevalence and the influence of sinonasal pathology on
the general health of a CF patient.
Nowadays CF patients have an increased life expectancy because of better
therapeutic modalities and centralised care. As a consequence therapy focuses
increasingly on improvement of quality of life. Sinonasal pathology has a great
negative influence on general quality of life. Previous research shows that
these symptoms have a greater negative influence compared to symptoms of
congestive heart failure, COPD and back pain.
Moreover previous studies hypothesize that the upper airways can be a reservoir
for bacteria which leads to cross-infection between the upper airways and the
lower airways. Thus, in theory pneumonias can be prevented by adequately
treating the upper airway infection.
Research in the prevalence of rhinosinusitis and nasal polyps in patients with
CF is scarce. Besides sinonasal disease in patients with Cystic Fibrosis are
currently underdiagnosed and therefore undertreated. This shows that it is
important to do solid research in the prevalence of this condition.
More knowledge of the actual prevalence, the impact of the symptoms on quality
of life and the severity of these symptoms, is essential to make a first step
in accurate treatment and a national protocol regarding sinonasal disease in
CF. In conclusion this can lead to improvement of the general health of the CF
patient.

Investigate the prevalence of rhinosinusitis and/or nasal polyps in adult
patients with Cystic Fibrosis. Also several aspects of sinonasal disease are
investigated; quality of life, correlation between phenotype and genotype,
microbiology, anatomy of the paranasal sinuses and nasal airway resistance.

Cross-sectional multicenter study.

In this study the patient will visit the hospital once, preferably subsequently
to a regular visit. The total time of this visit is estimated two hours. At
this visit the subjects will fill in a questionnaire. Also they will undergo
physical examination determined by ENT examination, nasendoscopy, nasal lavage,
middle meatal cultures, sputum culture, measurement of NAR and CT scan of the
sinuses. Radiation risk of the CT sinus is considered to be small compared to
the annual background radiation. The relative risk of radiation-induced cancer
is small. Possible discomfort of the nasendoscopy and the middle meatal
culture, will be prevented by the administration of local anaesthesia.
Theoretically it could be possible that nasal bacteria are spread as a
consequence of the nasal lavage. Although this has never been reported in
practice. This spread of bacteria could cause pulmonary infections. The
researchers will carefully record complications that occur after the nasal
lavage.