The information on this website is not intended for diagnosis or treatment. Please consult your physician for health concerns.

+90 850 321 50 00

Cystic Fibrosis Lung Treatment

Modern multimodal management of CF airway disease

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Göğüs Hastalıkları department. Book Appointment →

What is Cystic Fibrosis Lung Treatment?

Cystic fibrosis is caused by mutations in the CFTR gene producing thick, dehydrated airway secretions, chronic bacterial infection (Pseudomonas, Staphylococcus aureus, Burkholderia cepacia), and progressive bronchiectasis.

CFTR modulators (elexacaftor/tezacaftor/ivacaftor) revolutionized care for those with eligible mutations, restoring partial CFTR function, improving lung function, and reducing exacerbations and hospitalizations.

Daily care includes airway clearance (positive expiratory pressure, high-frequency chest wall oscillation), nebulized hypertonic saline and dornase alfa, inhaled tobramycin or aztreonam, and prompt treatment of pulmonary exacerbations.

Symptoms

Chronic productive cough
Recurrent pneumonia and exacerbations
Progressive dyspnea
Hemoptysis
Failure to thrive in children

Risk Factors

Two CFTR pathogenic variants
F508del homozygous or heterozygous
Persistent Pseudomonas colonization
Pancreatic insufficiency
Family history

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Increased cough or sputum
  • Decreased lung function
  • New hemoptysis
  • Weight loss or anorexia
  • Fever during exacerbation

Treatment Methods

01
CFTR modulator therapy
02
Twice-daily airway clearance
03
Inhaled hypertonic saline and dornase alfa
04
Cycled inhaled antibiotics
05
IV antibiotics for exacerbations
06
Lung transplantation for end-stage disease

Which Department to Visit?

You can visit our Göğüs Hastalıkları department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Göğüs Hastalıkları Department

Let us help you

You can make an appointment with our specialists or contact us for your concerns.

Book AppointmentContact Us

Related Health Topics

Other articles from the same department you may want to explore.

Asthma

Göğüs Hastalıkları

Asthma is characterized by wheezing, coughing and shortness of breath attacks; with proper treatment it can be kept under control.

COPD (Chronic Obstructive Pulmonary Disease)

Göğüs Hastalıkları

COPD is an irreversible lung disease characterized by shortness of breath and chronic cough; quitting smoking slows its progression.

Pneumonia

Göğüs Hastalıkları

Pneumonia presents with high fever, cough and shortness of breath; the vast majority recover with appropriate antibiotic treatment.

Tuberculosis (TB)

Göğüs Hastalıkları

Tuberculosis presents with weeks-to-months of cough, fever, and night sweats; early diagnosis and treatment lead to full recovery.

Pleural Effusion

Göğüs Hastalıkları

Pleural effusion is the accumulation of excess fluid in the pleural space, resulting from imbalances in fluid production and removal, and represents a manifestation of diverse cardiopulmonary, infectious, and malignant disorders.

Pneumothorax

Göğüs Hastalıkları

Pneumothorax is the presence of air in the pleural space resulting in partial or complete lung collapse, classified as spontaneous (primary/secondary), traumatic, or iatrogenic, with tension pneumothorax representing a life-threatening emergency.

Bronchitis (Acute and Chronic)

Göğüs Hastalıkları

Acute bronchitis is mostly viral and resolves spontaneously, while chronic bronchitis is a smoking-related component of COPD.

Bronchiectasis

Göğüs Hastalıkları

Bronchiectasis is a chronic respiratory disease characterized by permanent, abnormal dilation of bronchi with associated destruction of muscular and elastic components of airway walls, resulting in impaired mucociliary clearance and recurrent infection.

Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.