Cystic Fibrosis (Mucoviscidosis): Symptoms, Diagnosis, and Treatment | Clinique Omicron<\/title>\n<meta name=\"description\" content=\"Cystic fibrosis is a genetic disease affecting the lungs and pancreas. Neonatal screening, CFTR modulators, physiotherapy, and management in Quebec.\">\n<meta name=\"keywords\" content=\"fibrose kystique traitement, mucoviscidose Qu\u00e9bec, fibrose kystique sympt\u00f4mes, fibrose kystique modulateurs CFTR, Trikafta fibrose kystique, fibrose kystique diagnostic, fibrose kystique mucoviscidose, fibrose kystique d\u00e9pistage n\u00e9onatal\">\n<link rel=\"preconnect\" href=\"https:\/\/fonts.googleapis.com\">\n<link href=\"https:\/\/fonts.googleapis.com\/css2?family=Cinzel:wght@600&family=Poppins:wght@400;500;600;700&display=swap\" rel=\"stylesheet\">\n<style>\n.co-wrap * { font-family: 'Poppins', sans-serif; box-sizing: border-box; }\n.co-wrap { max-width: 1100px; margin: 0 auto; padding: 30px 0 60px; }\n.co-label { font-family: 'Cinzel', serif; font-size: 14px; font-weight: bold; letter-spacing: 1px; text-transform: uppercase; color: #4D6577; margin-bottom: 14px; display: block; }\n.co-wrap h1 { font-size: 32px; font-weight: 500; color: #323C52; margin: 0 0 22px; 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line-height: 1.7; }\n.co-urgence p:last-child { margin-bottom: 0; }\n.co-disclaimer { font-size: 13px; color: #8a9aaa; font-style: italic; border-top: 1px solid rgba(77,101,119,.15); padding-top: 24px; margin-top: 40px; line-height: 1.6; }\n<\/style>\n<\/head>\n<body>\n<div class=\"co-wrap\">\n <span class=\"co-label\">Pulmonology & Medical Genetics & Family Medicine<\/span>\n <h1>Cystic fibrosis (cystic fibrosis)<\/h1>\n<style>.oc-fiche-cta{margin:8px 0 34px;padding:22px 24px;border:1.5px solid rgba(50,60,82,.15);border-left:4px solid #FF611C;border-radius:12px;background:#F4F6F8;font-family:Poppins,sans-serif}.oc-fiche-cta-t{font-size:18px;font-weight:600;color:#323C52;margin:0 0 4px;line-height:1.35}.oc-fiche-cta-x{font-size:15px;color:#4D6577;margin:0 0 14px;line-height:1.55}.oc-fiche-cta-b{display:flex;flex-wrap:wrap;gap:10px}.oc-fiche-cta-b a{display:inline-flex;align-items:center;justify-content:center;min-height:44px;padding:10px 18px;border-radius:8px;font-size:14px;font-weight:600;text-decoration:none;box-sizing:border-box}.oc-fiche-cta-b .oc-p{background:#FF611C;color:#fff}.oc-fiche-cta-b .oc-p:hover{background:#E04E0C}.oc-fiche-cta-b .oc-s{background:#fff;color:#323C52;border:1.5px solid rgba(50,60,82,.25)}@media (max-width:600px){.co-wrap h1{font-size:24px;line-height:1.25;text-transform:none}.oc-fiche-cta{padding:18px 16px}.oc-fiche-cta-b a{flex:1 1 100%}body .co-wrap > .co-table,body .co-wrap > .co-table tbody,body .co-wrap > .co-table tr,body .co-wrap > .co-table td{display:block;width:100%;box-sizing:border-box}body .co-wrap > .co-table{min-width:0;table-layout:auto}body .co-wrap > .co-table thead{display:none}body .co-wrap > .co-table tr{margin:0 0 10px;border:1px solid rgba(77,101,119,.18);border-radius:8px;overflow:hidden}body .co-wrap > .co-table td{padding:9px 12px;border:0}body .co-wrap > .co-table td:first-child{font-weight:600;color:#323C52;background:rgba(77,101,119,.07)}#ocw{transform:scale(.8);transform-origin:bottom right}}<\/style><div class=\"oc-fiche-cta\" data-oc-cta=\"fiche-haut-consult\"><p class=\"oc-fiche-cta-t\">Obtenir un avis adapt\u00e9 \u00e0 votre situation<\/p><p class=\"oc-fiche-cta-x\">Consultation en clinique ou en t\u00e9l\u00e9consultation, partout au Qu\u00e9bec.<\/p><div class=\"oc-fiche-cta-b\"><a class=\"oc-p\" href=\"https:\/\/cliniqueomicron.ca\/rendez-vous\/\">Make an appointment<\/a><a class=\"oc-s\" href=\"https:\/\/cliniqueomicron.ca\/consultation-en-ligne\/\">Consult online<\/a><a class=\"oc-s\" href=\"tel:+15146063350\">514 606-3350<\/a><\/div><\/div>\n\n\n <div class=\"co-intro\">\n Cystic fibrosis\u2014known by this name in Quebec and Canada, and referred to as mucoviscidosis in Europe\u2014is the most common serious inherited genetic disorder among people of European descent. It is caused by mutations in the CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) gene, located on chromosome 7, which encodes a chloride channel present on the surface of epithelial cells in many organs. The defective CFTR protein leads to abnormal transport of chloride and sodium ions across cell membranes, resulting in abnormally thick, viscous, and dehydrated secretions in the lungs, pancreas, bile ducts, intestines, sweat glands, and genital tract. In Quebec, the incidence is approximately 1 in 3,600 births, and it is estimated that more than 4,200 people are living with cystic fibrosis in Canada. The most common mutation, deltaF508 (or F508del), accounts for approximately 70% of mutated alleles in North America\u2014it causes a folding defect in the CFTR protein, which is degraded before reaching the cell membrane. More than 2,000 mutations in the CFTR gene have been identified to date, with clinical consequences that vary widely depending on their functional class. Long considered a fatal pediatric disease affecting young children, cystic fibrosis has undergone an unprecedented therapeutic revolution since the 2010s with the advent of CFTR modulators\u2014particularly elexacaftor-tezacaftor-ivacaftor (Trikafta\/Kaftrio) \u2014 which directly correct the functional defect in the CFTR protein in the majority of patients, radically altering the course of the disease. Median life expectancy now exceeds 50 years in Canada for patients born today, and continues to rise.\n <\/div>\n\n <h2>Pathophysiology, diagnosis, and organ damage<\/h2>\n <ul class=\"co-list\">\n <li><strong>Genetics and CFTR mutation classes:<\/strong> autosomal recessive inheritance \u2014 two mutated alleles required to develop the disease \u2014 healthy carriers (heterozygotes) represent 1\/25 people of European descent; mutation classes according to their functional impact: class I (nonsense mutations \u2014 W1282X, G542X): complete absence of CFTR protein synthesis \u2014 severe form; class II (processing mutations \u2014 F508del: the most frequent): CFTR protein synthesized but misfolded \u2192 degraded in the endoplasmic reticulum before reaching the membrane \u2014 severe form \u2014 target of CFTR correctors (lumacaftor, tezacaftor, elexacaftor); class III (gating mutations \u2014 G551D): CFTR protein present at the membrane but channel does not open properly \u2014 moderate to severe form \u2014 target of CFTR potentiators (ivacaftor); class IV (conduction mutations): channel open but reduced conductance \u2014 moderate form; class V (splicing mutations): reduced amount of normal protein \u2014 moderate to mild form; class VI: unstable protein at the membrane \u2014 variable form<\/li>\n <li><strong>Pulmonary involvement (main cause of morbidity and mortality):<\/strong> Thick, dehydrated mucus in the bronchi \u2192 stasis + obstruction of the small airways \u2192 progressive chronic bacterial colonization: Staphylococcus aureus (from childhood onward) \u2192 Haemophilus influenzae \u2192 Pseudomonas aeruginosa (chronic colonization in 70\u201380% of adults \u2014 a marker of disease progression) \u2192 Burkholderia cepacia complex (genovar III = B. cenocepacia \u2014 cepacia syndrome \u2014 fulminant necrotizing pneumonia \u2014 high mortality) + Stenotrophomonas maltophilia + Achromobacter xylosoxidans + nontuberculous mycobacteria (NTM \u2014 Mycobacterium abscessus \u2014 difficult to treat); excessive neutrophilic inflammation of the bronchi \u2192 release of neutrophil elastase + metalloproteases \u2192 progressive destruction of the lung parenchyma \u2192 diffuse bronchiectasis + chronic respiratory failure; pulmonary complications: acute exacerbations (increased cough + increased purulent sputum production + dyspnea + decreased FEV1 + fever) + hemoptysis (20\u201330% of adults \u2014 sometimes massive) + pneumothorax + allergic bronchopulmonary aspergillosis (ABPA \u2014 total IgE + anti-Aspergillus antibodies + infiltrates) + end-stage chronic respiratory failure<\/li>\n <li><strong>Extrapulmonary manifestations:<\/strong> exocrine pancreas (85\u201390% of patients): obstruction of the pancreatic ducts \u2192 autodigestion \u2192 exocrine pancreatic insufficiency \u2192 malabsorption of fats and fat-soluble vitamins (A, D, E, K) \u2192 steatorrhea + growth and weight failure + vitamin deficiencies \u2192 pancreatic enzyme replacement therapy (PERT); endocrine pancreas: cystic fibrosis-related diabetes (CFRD) \u2014 affects 40\u201350% of adults \u2014 mixed mechanism (destruction of the islets of Langerhans + peripheral insulin resistance during exacerbations) \u2014 distinct from type 1 and type 2 diabetes \u2014 preferred treatment: insulin; liver and biliary tract: focal biliary cirrhosis (5\u201310% of adults with CF) + gallstones + hepatic steatosis; intestine: meconium ileus (10\u201320% of newborns with CF \u2014 first clinical manifestation) + distal intestinal obstruction syndrome (DIOS \u2014 formerly meconium equivalent \u2014 adults) + chronic constipation + rectal prolapse (children); genitourinary system: obstructive azoospermia due to congenital bilateral absence of the vas deferens (CBAVD \u2014 97\u201398% of men) \u2192 near-constant male infertility (female fertility preserved but reduced); sweat glands: sweat test (sweat chloride \u226560 mmol\/L = diagnosis); ENT: nasal polyposis (50% of adults) + chronic sinusitis + sensorineural hearing loss (aminoglycosides)<\/li>\n <li><strong>Diagnosis :<\/strong> Universal newborn screening in Quebec since 2019: measurement of immunoreactive trypsin (IRT) on a blood blot (Day 3) \u2192 if IRT is elevated \u2192 CFTR genetic testing + confirmatory sweat test; sweat test (Gibson-Cooke method): pilocarpine iontophoresis + measurement of sudoral chloride \u2014 chloride \u226560 mmol\/L = confirmed CF diagnosis; chloride 30\u201359 mmol\/L = borderline result (CFTR-RD \u2014 related disorder); chloride <30 mmol\/L = normal; extended genetic analysis (CFTR mutation panel): confirms the diagnosis + identifies mutations to guide targeted therapy (eligibility for modulators); spirometry: FEV1 (forced expiratory volume in one second) \u2014 key prognostic marker \u2014 FEV1 <40% predicted = indicator of advanced severity + evaluation for transplantation; High-resolution chest CT: extent of bronchiectasis + bronchial wall thickening + mucoid impaction + consolidation<\/li>\n <\/ul>\n\n <h2>Treatment<\/h2>\n <table class=\"co-table\">\n <colgroup><col style=\"width:200px;\"><col style=\"width:42%;\"><col><\/colgroup>\n <thead>\n <tr><th>Treatment<\/th><th>Mechanism, molecules, and modalities<\/th><th>Effectiveness, results, and precautions<\/th><\/tr>\n <\/thead>\n <tbody>\n <tr>\n <td>CFTR protein modulators<br><small style=\"font-weight:400;color:#7a8fa0;\">Therapeutic Revolution \u2014 Treatment of the Molecular Cause<\/small><\/td>\n <td>CFTR modulators are small molecules that directly target the functional defect in the CFTR protein\u2014they do not correct the DNA mutation but partially or fully restore the protein\u2019s function; two main classes: correctors (correct the folding and intracellular trafficking of the CFTR protein, class II\u2014e.g., lumacaftor, tezacaftor, elexacaftor) + potentiators (increase the opening time of the CFTR channel at the membrane\u2014e.g., ivacaftor); ivacaftor (Kalydeco)\u2014potentiator alone: gating mutations (G551D and 8 other responsive mutations) \u2014 FEV1 +10.6 L\/s vs. placebo (STRIVE 2011 NEJM) \u2014 sudoral chloride \u221248 mmol\/L \u2014 Health Canada approval 2012 \u2014 4\u20135 L\/s in eligible patients; lumacaftor-ivacaftor (Orkambi): first-generation corrector + potentiator \u2014 F508del homozygotes \u2014 FEV1 +2.6 to +4 L\/s \u2014 modest improvement \u2014 pulmonary side effects (initial bronchospasm) + drug interactions (CYP3A4) \u2014 largely replaced by Trikafta; tezacaftor-ivacaftor (Symdeko\/Symkevi): second-generation corrector + potentiator \u2014 FEV1 +4% \u2014 better tolerated than Orkambi; elexacaftor-tezacaftor-ivacaftor (Trikafta\/Kaftrio): triple combination \u2014 next-generation corrector (elexacaftor) + second-generation corrector (tezacaftor) + potentiator (ivacaftor) \u2014 dosage: 2 tablets in the morning + 1 tablet in the evening \u2014 eligibility: \u22651 F508del copy + any responsive mutation \u2014 covers >90% of patients with CF in Canada<\/td>\n <td>Trikafta \u2014 clinical results (VX19-445-102 trial, Middleton 2019 NEJM): FEV1 +14.3 L\/s at 24 weeks vs. placebo + sudoral chloride \u221241.8 mmol\/L + quality-of-life score (CFQ-R respiratory) +20 points + 63% reduction in pulmonary exacerbations at 24 weeks + BMI +1.0 kg\/m\u00b2; long-term follow-up (AURORA Registry, Cystic Fibrosis Foundation Registry): sustained improvement at 2\u20133 years + reduced lung transplant rate + improved survival; Trikafta reimbursement in Canada: approved by Health Canada in June 2021 (\u226512 years) then extended to \u22656 years (2022) and \u22652 years (2023) \u2014 reimbursed in Quebec (RAMQ) since November 2021 for patients \u226512 years old (F508del homozygotes or heterozygotes + responsive mutation) \u2014 exceptional access application process for other genotypes; Trikafta side effects: elevated transaminases (10\u201315 % \u2014 monthly liver monitoring for the first 3 months) + lens opacities (cataracts \u2014 ophthalmological screening before treatment in those <18 years) + CYP3A4 interaction (rifampicin, azithromycin \u2014 dose adjustment) + skin rash; Trikafta cost: approximately 350,000 CAD\/year without reimbursement \u2014 RAMQ reimbursement radically changes accessibility in Quebec<\/td>\n <\/tr>\n <tr>\n <td>Respiratory physiotherapy and mucociliary clearance<br><small style=\"font-weight:400;color:#7a8fa0;\">Pillar of treatment \u2014 daily for life<\/small><\/td>\n <td>Respiratory physical therapy (RPT) is a fundamental and irreplaceable treatment for cystic fibrosis\u2014its goal is to mobilize and clear thick bronchial secretions that obstruct the airways and serve as a substrate for chronic bacterial colonization; airway clearance techniques (ACT): autogenic drainage (AGD): a technique involving controlled active exhalation at different lung volumes\u2014learned with a physical therapist specializing in CF\u2014the gold standard in Europe and Canada\u2014can be performed independently without equipment; high-frequency oral oscillation (OOFE \u2014 Acapella, Flutter): device creating expiratory oscillatory resistance \u2192 intrabronchial vibrations \u2192 loosening of secretions + improvement in mucociliary clearance; Positive expiratory pressure (PEP): mask or mouthpiece providing resistance to exhalation \u2192 recruitment of collateral alveoli \u2192 mobilization of secretions from the small airways; high-frequency chest oscillation vest (ThAIRapy vest): used primarily in North America + in young children or uncooperative patients; frequency: 1\u20132 sessions\/day of 20\u201340 min \u2014 increased during exacerbations (3\u20134 sessions\/day); mucoclearance-facilitating medications inhaled prior to PTR: hypertonic saline solution (3\u20137 M NaCl \u2014 4 mL nebulized) \u2192 rehydration of the bronchial mucosal surface \u2192 improvement in viscosity; inhaled mannitol (Bronchitol): inhaled osmotic agent \u2014 approved by Health Canada in 2021<\/td>\n <td>PTR should ideally be taught and supervised by a physical therapist specializing in cystic fibrosis at an accredited CF center\u2014in Quebec, adult and pediatric CF centers (CHU Sainte-Justine, CHUM, H\u00f4pital Maisonneuve-Rosemont, CHU de Qu\u00e9bec\u2014IUCPQ) have multidisciplinary teams that include experienced physical therapists; rhDNase (dornase alfa \u2014 Pulmozyme): mucolytic enzyme \u2014 cleaves extracellular DNA released by necrotic polymorphonuclear cells in bronchial secretions \u2192 reduces mucus viscosity \u2014 administered via nebulizer prior to PTR (2.5 mg \u00d7 1\/day) \u2014 reduction in exacerbations of 29% and improvement in FEV1 of 5.8% (Fuchs 1994 NEJM) \u2014 covered by RAMQ; since the introduction of Trikafta, secretion volume and viscosity have decreased significantly within 4 to 8 weeks of initiation \u2014 adjust the duration and intensity of PTR based on clinical response (but maintaining a daily routine is recommended)<\/td>\n <\/tr>\n <tr>\n <td>Antibiotic therapy - infections and exacerbations<br><small style=\"font-weight:400;color:#7a8fa0;\">Maintenance and Exacerbation Strategies<\/small><\/td>\n <td>Antibiotic therapy in cystic fibrosis\u2014three distinct strategies depending on the goal: early eradication of Pseudomonas aeruginosa (primary colonization detected by sputum culture): inhaled tobramycin (TOBI) 300 mg twice daily for 28 days + oral ciprofloxacin 750 mg twice daily for 4 weeks \u2014 eradication rate 75\u201385% % \u2014 objective: to delay chronic colonization; long-term anti-Pseudomonas antibiotic therapy (established chronic colonization): alternating inhaled tobramycin (TOBI \u2014 1 month ON \/ 1 month OFF) OR inhaled aztreonam (Cayston) \u2014 maintenance of pulmonary stability + reduction in exacerbations + slowing of FEV1 decline; oral azithromycin 250\u2013500 mg \u00d7 3\/week: anti-inflammatory + immunomodulatory effect (non-antibiotic) \u2014 reduces exacerbations by 35\u201340% in patients colonized with P. aeruginosa \u2014 precaution: do not use if active nontuberculous mycobacteria (masks and promotes resistance); treatment of acute pulmonary exacerbations: hospitalization criteria \u2014 FEV\u2081 drop >10% + significant increase in secretions + hemoptysis + SpO\u2082 <95% + patient unable to maintain outpatient care; IV antibiotics: based on antibiotic susceptibility testing (recent sputum cultures required) \u2014 P. aeruginosa: piperacillin-tazobactam 4.5 g \u00d7 4\/day IV + tobramycin IV (once daily \u2014 10\u201312 mg\/kg\/day \u2014 pharmacokinetic monitoring required \u2014 nephrotoxicity + ototoxicity) \u2014 duration: 14\u201321 days IV or until return to baseline FEV1<\/td>\n <td>Managing bacterial resistance is a major challenge in cystic fibrosis\u2014patients are exposed to dozens of courses of antibiotics over the course of their lives \u2192 emergence of multidrug-resistant (MDR) P. aeruginosa + B. cepacia complex + nontuberculous mycobacteria (M. abscessus\u2014complex treatment: IV amikacin + imipenem + cefoxitin \u00b1 azithromycin \u00d7 12 months); segregation of patients in hospitals and clinics: patients colonized with B. cepacia complex (particularly B. cenocepacia) must never come into contact with other CF patients \u2014 single rooms mandatory \u2014 risk of interpatient transmission and fulminant cepacia syndrome + relative contraindication for lung transplantation (B. cenocepacia \u2014 very high post-transplant mortality) ; ototoxicity of aminoglycosides (tobramycin): annual audiometric screening in patients receiving inhaled or IV tobramycin \u2014 nephrotoxicity: creatinine + clearance before each IV course + peak and trough pharmacokinetic measurements; Trikafta significantly reduces the frequency of exacerbations requiring IV antibiotics (63% reduction in % trials) \u2192 decreased cumulative exposure to aminoglycosides \u2192 reduced risk of ototoxicity<\/td>\n <\/tr>\n <tr>\n <td>Nutritional and pancreatic support<br><small style=\"font-weight:400;color:#7a8fa0;\">PERT \u2014 pancreatic enzymes \u2014 fat-soluble vitamins<\/small><\/td>\n <td>Exocrine pancreatic insufficiency (85\u201390% of patients): pancreatic enzyme replacement therapy (PERT): Creon (pancreatin\u2014lipase + amylase + protease in the form of enteric-coated, gastro-resistant microspheres)\u2014take at the start of a meal or snack\u2014dosage: 500\u20132,500 IU lipase\/kg\/meal (max 10,000 IU lipase\/kg\/day) \u2014 titrate based on steatorrhea and weight gain; nutritional goals in FK: target BMI \u226522 kg\/m\u00b2 (women) and \u226523 kg\/m\u00b2 (men) \u2014 1\u20133 times the expected weight \u226590% of the recommended intake in children; high-calorie, high-fat diet (caloric intake 110\u2013200% of recommended intake) \u2014 no fat restriction (unlike standard pancreatitis); mandatory fat-soluble vitamin supplementation: vitamin A (10,000 IU\/day) + vitamin D (800\u20132,000 IU\/day \u2014 annual 25-OH-D measurement \u2014 target 75\u2013120 nmol\/L) + vitamin E (100\u2013400 IU\/day) + vitamin K (coagulopathy prevention \u2014 1\u201310 mg\/day); additional salt in summer or during intense exercise (excessive NaCl loss in sweat); nocturnal enteral nutrition via nasogastric tube or gastrostomy: if BMI <18 or weight <85 kg despite optimal oral management \u2014 often prescribed for adolescents during growth spurts; CF-related diabetes (CRD): rapid-acting insulin therapy at meals + nocturnal basal insulin if fasting hyperglycemia \u2014 avoid oral antidiabetics (except metformin as a second-line treatment) \u2014 specialized endocrinological follow-up; osteoporosis: bone densitometry starting at age 18 + bisphosphonates if T-score <\u22122.5<\/td>\n <td>Nutrition is a major independent prognostic factor in CF \u2014 nutritional status is directly correlated with lung function and survival (BMI <18 associated with accelerated FEV1 decline and increased mortality); since Trikafta: significant improvement in nutritional status in the first months of treatment (increase in body weight + improved fat absorption) \u2014 possible reduction in PERT doses needed in some patients (adjustment guided by clinical steatorrhea and nutritional status, not empiric cessation); CF-related diabetes (CFRD) is often asymptomatic initially \u2014 annual screening with oral glucose tolerance test (OGTT 75g \u2014 2h) recommended from age 10 in all patients \u2014 screening with HbA1c is insufficient in CF (accelerated erythrocyte turnover + anemia) \u2014 use postprandial glycemia; liver transplantation: indicated in decompensated cirrhosis (Child-Pugh C) \u2014 can be performed in combination with lung transplantation in some patients<\/td>\n <\/tr>\n <tr>\n <td>Lung transplantation<br><small style=\"font-weight:400;color:#7a8fa0;\">End-stage \u2014 FEV1 <30% of predicted or rapid decline<\/small><\/td>\n <td>Bilateral lung transplantation (double-lung transplant) is the last-resort treatment for end-stage respiratory failure associated with cystic fibrosis\u2014CF is the third most common indication for lung transplantation worldwide (after COPD and idiopathic pulmonary fibrosis); Transplantation criteria (Cystic Fibrosis Foundation + ISHLT): FEV1 <30% of predicted (or decline >20% of predicted value within 12 months) + resting SpO\u2082 <90% on oxygen or during exercise + hypercapnia (PaCO\u2082 >50 mmHg) + frequent exacerbations requiring hospitalization + severe treatment-resistant malnutrition + PAH + recurrent massive hemoptysis; relative or absolute contraindications: colonization with B. cenocepacia (genotype IIIA \u2014 post-transplant mortality of 50\u201375% at 1 year \u2014 contraindication in most centers, including the IUCPQ) + active, uncontrolled M. abscessus + severe obesity (BMI >35) + severe uncorrectable renal or hepatic insufficiency + documented non-adherence to therapy + major comorbidities; lung transplantation program in Quebec: IUCPQ (Quebec University Institute of Cardiology and Pulmonology) \u2014 the only lung transplantation center in Quebec + pre-transplant follow-up centers (CHUM, CHU Sainte-Justine); post-transplant survival FK: 80 % at 1 year \/ 55\u201360 % at 5 years \/ 40\u201345 % at 10 years (ISHLT registry 2022) \u2014 better outcomes in FK patients than for other indications (younger population + fewer comorbidities); complications: chronic rejection (CLAD\u2014bronchiolitis obliterans) + opportunistic infections (bacterial, viral [CMV], fungal) + immunosuppressant toxicity<\/td>\n <td>Since the introduction of Trikafta, the number of CF patients on transplant waiting lists has decreased significantly in countries where Trikafta is covered\u2014some patients awaiting transplantation have even been removed from the list after starting Trikafta due to the clinical improvement achieved; however, Trikafta does not reverse irreversible lung damage that has already occurred (bronchiectasis, parenchymal destruction)\u2014patients with very low FEV1 (<30\u201340% of predicted) may have a partial response and remain candidates for transplantation even while on Trikafta; Management of CF medications post-transplant: CFTR modulators (Trikafta) are generally continued after transplantation\u2014they continue to benefit non-transplanted organs (pancreas, sinuses, intestines, genital tract) \u2014 interactions with immunosuppressants (tacrolimus + cyclosporine \u2014 CYP3A4 substrates) require adjustment of immunosuppressant doses (a 50% reduction in tacrolimus is recommended upon initiation of Trikafta) + monitoring of residual levels; palliative care: for patients ineligible for transplantation or in the terminal phase \u2014 specialized palliative care program + cystic fibrosis team + ambulatory oxygen therapy + non-invasive ventilation + advance discussion of care directives<\/td>\n <\/tr>\n <\/tbody>\n <\/table>\n\n <div class=\"co-infobox\">\n <span class=\"ico\">\u2139\ufe0f<\/span>\n <span><strong>Trikafta and access in Quebec \u2014 an accessible revolution:<\/strong> Elexacaftor-tezacaftor-ivacaftor (Trikafta) has been reimbursed by the RAMQ in Quebec since November 2021 for patients aged 12 and over with at least one F508del mutation, and since 2023 for children aged 2 and over according to Canadian guidelines. For eligible patients with other non-F508del mutations, an exceptional access request (exception drug) is possible through the RAMQ upon recommendation from a CF-specialized pulmonologist. Cystic Fibrosis Canada (cysticfibrosis.ca) offers access support resources and a patient assistance program. Quebec patients whose genotype is not covered by Trikafta are followed in compassionate access programs or ongoing clinical trials for new modulator combinations (vanzacaftor-tezacaftor-deutivacaftor \u2013 VX-522 \u2013 in phase 3).<\/span>\n <\/div>\n\n <div class=\"co-urgence\">\n <div class=\"co-urgence-titre\">Emergency \u2014 Severe exacerbation and massive hemoptysis<\/div>\n <p>Dial <strong>911<\/strong> or go immediately to the emergency room if : <strong>Sudden shortness of breath + SpO\u2082 <90% % + rapid decline in respiratory function<\/strong> Severe pulmonary exacerbation - IV antibiotics + intensive inpatient PTR.<\/p>\n <p><strong>Massive hemoptysis (>240 mL\/24h or massive hemoptysis)<\/strong> Call 911. Affected lateral position. No anticoagulants. Emergency bronchial artery embolization at a specialized center.<\/p>\n <p><strong>Sudden chest pain + sudden shortness of breath + unilateral absence of breath sounds<\/strong> \u2192 pneumothorax \u2014 surgical emergency \u2192 chest drain.<\/p>\n <\/div>\n\n <h2>Consult at Clinique Omicron<\/h2>\n <p>Les m\u00e9decins de Clinique Omicron assurent le suivi de premi\u00e8re ligne des patients atteints de fibrose kystique en collaboration avec les centres sp\u00e9cialis\u00e9s FK au Qu\u00e9bec : gestion des infections intercurrentes, renouvellement des ordonnances, suivi nutritionnel, d\u00e9pistage du diab\u00e8te li\u00e9 \u00e0 la FK et coordination des demandes de remboursement des modulateurs CFTR (RAMQ). Des consultations sont disponibles dans nos points de service au Qu\u00e9bec et via la t\u00e9l\u00e9m\u00e9decine. Pour prendre rendez-vous, <a href=\"https:\/\/cliniqueomicron.ca\/rendez-vous\/\" data-oc-cta=\"fiche-consulter\">choisissez votre service en ligne<\/a>.<\/p>\n\n <p class=\"co-disclaimer\">The content of this page is provided for informational purposes only and does not substitute for the advice of a qualified healthcare professional specializing in cystic fibrosis. Optimal management of cystic fibrosis requires a multidisciplinary team at an accredited center\u2014pulmonologist, physical therapist, nutritionist, specialized nurse, and social worker.<\/p>\n<\/div>\n<\/body>\n<\/html>\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>","protected":false},"excerpt":{"rendered":"<p>Fibrose kystique (mucoviscidose) : sympt\u00f4mes, diagnostic et traitement | Clinique Omicron Pneumologie & G\u00e9n\u00e9tique m\u00e9dicale & M\u00e9decine de famille Fibrose kystique (mucoviscidose) La fibrose kystique \u2014 d\u00e9sign\u00e9e au Qu\u00e9bec et au Canada par ce terme, et connue sous le nom de mucoviscidose en Europe \u2014 est la maladie g\u00e9n\u00e9tique h\u00e9r\u00e9ditaire grave la plus fr\u00e9quente dans… <a href=\"https:\/\/cliniqueomicron.ca\/en\/fibrose-kystique\/\" rel=\"bookmark\">Read More \"<span class=\"screen-reader-text\">Cystic Fibrosis (Mucoviscidosis): Symptoms, Diagnosis, and Treatment | Clinique Omicron<\/span><\/a><\/p>","protected":false},"author":1,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"om_disable_all_campaigns":false,"neve_meta_sidebar":"","neve_meta_container":"","neve_meta_enable_content_width":"off","neve_meta_content_width":100,"neve_meta_title_alignment":"","neve_meta_author_avatar":"","neve_post_elements_order":"","neve_meta_disable_header":"","neve_meta_disable_footer":"","neve_meta_disable_title":"","_themeisle_gutenberg_block_has_review":false,"_metasync_otto_title":"","_metasync_otto_description":"La fibrose kystique est une maladie g\u00e9n\u00e9tique touchant les poumons et le pancr\u00e9as. 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Neonatal screening, CFTR modulators, physiotherapy, and management in Quebec.\">\n<meta name=\"keywords\" content=\"fibrose kystique traitement, mucoviscidose Qu\u00e9bec, fibrose kystique sympt\u00f4mes, fibrose kystique modulateurs CFTR, Trikafta fibrose kystique, fibrose kystique diagnostic, fibrose kystique mucoviscidose, fibrose kystique d\u00e9pistage n\u00e9onatal\">\n<link rel=\"preconnect\" href=\"https:\/\/fonts.googleapis.com\">\n<link href=\"https:\/\/fonts.googleapis.com\/css2?family=Cinzel:wght@600&family=Poppins:wght@400;500;600;700&display=swap\" rel=\"stylesheet\">\n<style>\n.co-wrap * { font-family: 'Poppins', sans-serif; box-sizing: border-box; }\n.co-wrap { max-width: 1100px; margin: 0 auto; padding: 30px 0 60px; }\n.co-label { font-family: 'Cinzel', serif; font-size: 14px; font-weight: bold; letter-spacing: 1px; text-transform: uppercase; color: #4D6577; margin-bottom: 14px; display: block; }\n.co-wrap h1 { font-size: 32px; font-weight: 500; color: #323C52; margin: 0 0 22px; 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line-height: 1.7; }\n.co-urgence p:last-child { margin-bottom: 0; }\n.co-disclaimer { font-size: 13px; color: #8a9aaa; font-style: italic; border-top: 1px solid rgba(77,101,119,.15); padding-top: 24px; margin-top: 40px; line-height: 1.6; }\n<\/style>\n<\/head>\n<body>\n<div class=\"co-wrap\">\n <span class=\"co-label\">Pulmonology & Medical Genetics & Family Medicine<\/span>\n <h1>Cystic fibrosis (cystic fibrosis)<\/h1>\n<style>.oc-fiche-cta{margin:8px 0 34px;padding:22px 24px;border:1.5px solid rgba(50,60,82,.15);border-left:4px solid #FF611C;border-radius:12px;background:#F4F6F8;font-family:Poppins,sans-serif}.oc-fiche-cta-t{font-size:18px;font-weight:600;color:#323C52;margin:0 0 4px;line-height:1.35}.oc-fiche-cta-x{font-size:15px;color:#4D6577;margin:0 0 14px;line-height:1.55}.oc-fiche-cta-b{display:flex;flex-wrap:wrap;gap:10px}.oc-fiche-cta-b a{display:inline-flex;align-items:center;justify-content:center;min-height:44px;padding:10px 18px;border-radius:8px;font-size:14px;font-weight:600;text-decoration:none;box-sizing:border-box}.oc-fiche-cta-b .oc-p{background:#FF611C;color:#fff}.oc-fiche-cta-b .oc-p:hover{background:#E04E0C}.oc-fiche-cta-b .oc-s{background:#fff;color:#323C52;border:1.5px solid rgba(50,60,82,.25)}@media (max-width:600px){.co-wrap h1{font-size:24px;line-height:1.25;text-transform:none}.oc-fiche-cta{padding:18px 16px}.oc-fiche-cta-b a{flex:1 1 100%}body .co-wrap > .co-table,body .co-wrap > .co-table tbody,body .co-wrap > .co-table tr,body .co-wrap > .co-table td{display:block;width:100%;box-sizing:border-box}body .co-wrap > .co-table{min-width:0;table-layout:auto}body .co-wrap > .co-table thead{display:none}body .co-wrap > .co-table tr{margin:0 0 10px;border:1px solid rgba(77,101,119,.18);border-radius:8px;overflow:hidden}body .co-wrap > .co-table td{padding:9px 12px;border:0}body .co-wrap > .co-table td:first-child{font-weight:600;color:#323C52;background:rgba(77,101,119,.07)}#ocw{transform:scale(.8);transform-origin:bottom right}}<\/style><div class=\"oc-fiche-cta\" data-oc-cta=\"fiche-haut-consult\"><p class=\"oc-fiche-cta-t\">Obtenir un avis adapt\u00e9 \u00e0 votre situation<\/p><p class=\"oc-fiche-cta-x\">Consultation en clinique ou en t\u00e9l\u00e9consultation, partout au Qu\u00e9bec.<\/p><div class=\"oc-fiche-cta-b\"><a class=\"oc-p\" href=\"https:\/\/cliniqueomicron.ca\/rendez-vous\/\">Make an appointment<\/a><a class=\"oc-s\" href=\"https:\/\/cliniqueomicron.ca\/consultation-en-ligne\/\">Consult online<\/a><a class=\"oc-s\" href=\"tel:+15146063350\">514 606-3350<\/a><\/div><\/div>\n\n\n <div class=\"co-intro\">\n Cystic fibrosis\u2014known by this name in Quebec and Canada, and referred to as mucoviscidosis in Europe\u2014is the most common serious inherited genetic disorder among people of European descent. It is caused by mutations in the CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) gene, located on chromosome 7, which encodes a chloride channel present on the surface of epithelial cells in many organs. The defective CFTR protein leads to abnormal transport of chloride and sodium ions across cell membranes, resulting in abnormally thick, viscous, and dehydrated secretions in the lungs, pancreas, bile ducts, intestines, sweat glands, and genital tract. In Quebec, the incidence is approximately 1 in 3,600 births, and it is estimated that more than 4,200 people are living with cystic fibrosis in Canada. The most common mutation, deltaF508 (or F508del), accounts for approximately 70% of mutated alleles in North America\u2014it causes a folding defect in the CFTR protein, which is degraded before reaching the cell membrane. More than 2,000 mutations in the CFTR gene have been identified to date, with clinical consequences that vary widely depending on their functional class. Long considered a fatal pediatric disease affecting young children, cystic fibrosis has undergone an unprecedented therapeutic revolution since the 2010s with the advent of CFTR modulators\u2014particularly elexacaftor-tezacaftor-ivacaftor (Trikafta\/Kaftrio) \u2014 which directly correct the functional defect in the CFTR protein in the majority of patients, radically altering the course of the disease. Median life expectancy now exceeds 50 years in Canada for patients born today, and continues to rise.\n <\/div>\n\n <h2>Pathophysiology, diagnosis, and organ damage<\/h2>\n <ul class=\"co-list\">\n <li><strong>Genetics and CFTR mutation classes:<\/strong> autosomal recessive inheritance \u2014 two mutated alleles required to develop the disease \u2014 healthy carriers (heterozygotes) represent 1\/25 people of European descent; mutation classes according to their functional impact: class I (nonsense mutations \u2014 W1282X, G542X): complete absence of CFTR protein synthesis \u2014 severe form; class II (processing mutations \u2014 F508del: the most frequent): CFTR protein synthesized but misfolded \u2192 degraded in the endoplasmic reticulum before reaching the membrane \u2014 severe form \u2014 target of CFTR correctors (lumacaftor, tezacaftor, elexacaftor); class III (gating mutations \u2014 G551D): CFTR protein present at the membrane but channel does not open properly \u2014 moderate to severe form \u2014 target of CFTR potentiators (ivacaftor); class IV (conduction mutations): channel open but reduced conductance \u2014 moderate form; class V (splicing mutations): reduced amount of normal protein \u2014 moderate to mild form; class VI: unstable protein at the membrane \u2014 variable form<\/li>\n <li><strong>Pulmonary involvement (main cause of morbidity and mortality):<\/strong> Thick, dehydrated mucus in the bronchi \u2192 stasis + obstruction of the small airways \u2192 progressive chronic bacterial colonization: Staphylococcus aureus (from childhood onward) \u2192 Haemophilus influenzae \u2192 Pseudomonas aeruginosa (chronic colonization in 70\u201380% of adults \u2014 a marker of disease progression) \u2192 Burkholderia cepacia complex (genovar III = B. cenocepacia \u2014 cepacia syndrome \u2014 fulminant necrotizing pneumonia \u2014 high mortality) + Stenotrophomonas maltophilia + Achromobacter xylosoxidans + nontuberculous mycobacteria (NTM \u2014 Mycobacterium abscessus \u2014 difficult to treat); excessive neutrophilic inflammation of the bronchi \u2192 release of neutrophil elastase + metalloproteases \u2192 progressive destruction of the lung parenchyma \u2192 diffuse bronchiectasis + chronic respiratory failure; pulmonary complications: acute exacerbations (increased cough + increased purulent sputum production + dyspnea + decreased FEV1 + fever) + hemoptysis (20\u201330% of adults \u2014 sometimes massive) + pneumothorax + allergic bronchopulmonary aspergillosis (ABPA \u2014 total IgE + anti-Aspergillus antibodies + infiltrates) + end-stage chronic respiratory failure<\/li>\n <li><strong>Extrapulmonary manifestations:<\/strong> exocrine pancreas (85\u201390% of patients): obstruction of the pancreatic ducts \u2192 autodigestion \u2192 exocrine pancreatic insufficiency \u2192 malabsorption of fats and fat-soluble vitamins (A, D, E, K) \u2192 steatorrhea + growth and weight failure + vitamin deficiencies \u2192 pancreatic enzyme replacement therapy (PERT); endocrine pancreas: cystic fibrosis-related diabetes (CFRD) \u2014 affects 40\u201350% of adults \u2014 mixed mechanism (destruction of the islets of Langerhans + peripheral insulin resistance during exacerbations) \u2014 distinct from type 1 and type 2 diabetes \u2014 preferred treatment: insulin; liver and biliary tract: focal biliary cirrhosis (5\u201310% of adults with CF) + gallstones + hepatic steatosis; intestine: meconium ileus (10\u201320% of newborns with CF \u2014 first clinical manifestation) + distal intestinal obstruction syndrome (DIOS \u2014 formerly meconium equivalent \u2014 adults) + chronic constipation + rectal prolapse (children); genitourinary system: obstructive azoospermia due to congenital bilateral absence of the vas deferens (CBAVD \u2014 97\u201398% of men) \u2192 near-constant male infertility (female fertility preserved but reduced); sweat glands: sweat test (sweat chloride \u226560 mmol\/L = diagnosis); ENT: nasal polyposis (50% of adults) + chronic sinusitis + sensorineural hearing loss (aminoglycosides)<\/li>\n <li><strong>Diagnosis :<\/strong> Universal newborn screening in Quebec since 2019: measurement of immunoreactive trypsin (IRT) on a blood blot (Day 3) \u2192 if IRT is elevated \u2192 CFTR genetic testing + confirmatory sweat test; sweat test (Gibson-Cooke method): pilocarpine iontophoresis + measurement of sudoral chloride \u2014 chloride \u226560 mmol\/L = confirmed CF diagnosis; chloride 30\u201359 mmol\/L = borderline result (CFTR-RD \u2014 related disorder); chloride <30 mmol\/L = normal; extended genetic analysis (CFTR mutation panel): confirms the diagnosis + identifies mutations to guide targeted therapy (eligibility for modulators); spirometry: FEV1 (forced expiratory volume in one second) \u2014 key prognostic marker \u2014 FEV1 <40% predicted = indicator of advanced severity + evaluation for transplantation; High-resolution chest CT: extent of bronchiectasis + bronchial wall thickening + mucoid impaction + consolidation<\/li>\n <\/ul>\n\n <h2>Treatment<\/h2>\n <table class=\"co-table\">\n <colgroup><col style=\"width:200px;\"><col style=\"width:42%;\"><col><\/colgroup>\n <thead>\n <tr><th>Treatment<\/th><th>Mechanism, molecules, and modalities<\/th><th>Effectiveness, results, and precautions<\/th><\/tr>\n <\/thead>\n <tbody>\n <tr>\n <td>CFTR protein modulators<br><small style=\"font-weight:400;color:#7a8fa0;\">Therapeutic Revolution \u2014 Treatment of the Molecular Cause<\/small><\/td>\n <td>CFTR modulators are small molecules that directly target the functional defect in the CFTR protein\u2014they do not correct the DNA mutation but partially or fully restore the protein\u2019s function; two main classes: correctors (correct the folding and intracellular trafficking of the CFTR protein, class II\u2014e.g., lumacaftor, tezacaftor, elexacaftor) + potentiators (increase the opening time of the CFTR channel at the membrane\u2014e.g., ivacaftor); ivacaftor (Kalydeco)\u2014potentiator alone: gating mutations (G551D and 8 other responsive mutations) \u2014 FEV1 +10.6 L\/s vs. placebo (STRIVE 2011 NEJM) \u2014 sudoral chloride \u221248 mmol\/L \u2014 Health Canada approval 2012 \u2014 4\u20135 L\/s in eligible patients; lumacaftor-ivacaftor (Orkambi): first-generation corrector + potentiator \u2014 F508del homozygotes \u2014 FEV1 +2.6 to +4 L\/s \u2014 modest improvement \u2014 pulmonary side effects (initial bronchospasm) + drug interactions (CYP3A4) \u2014 largely replaced by Trikafta; tezacaftor-ivacaftor (Symdeko\/Symkevi): second-generation corrector + potentiator \u2014 FEV1 +4% \u2014 better tolerated than Orkambi; elexacaftor-tezacaftor-ivacaftor (Trikafta\/Kaftrio): triple combination \u2014 next-generation corrector (elexacaftor) + second-generation corrector (tezacaftor) + potentiator (ivacaftor) \u2014 dosage: 2 tablets in the morning + 1 tablet in the evening \u2014 eligibility: \u22651 F508del copy + any responsive mutation \u2014 covers >90% of patients with CF in Canada<\/td>\n <td>Trikafta \u2014 clinical results (VX19-445-102 trial, Middleton 2019 NEJM): FEV1 +14.3 L\/s at 24 weeks vs. placebo + sudoral chloride \u221241.8 mmol\/L + quality-of-life score (CFQ-R respiratory) +20 points + 63% reduction in pulmonary exacerbations at 24 weeks + BMI +1.0 kg\/m\u00b2; long-term follow-up (AURORA Registry, Cystic Fibrosis Foundation Registry): sustained improvement at 2\u20133 years + reduced lung transplant rate + improved survival; Trikafta reimbursement in Canada: approved by Health Canada in June 2021 (\u226512 years) then extended to \u22656 years (2022) and \u22652 years (2023) \u2014 reimbursed in Quebec (RAMQ) since November 2021 for patients \u226512 years old (F508del homozygotes or heterozygotes + responsive mutation) \u2014 exceptional access application process for other genotypes; Trikafta side effects: elevated transaminases (10\u201315 % \u2014 monthly liver monitoring for the first 3 months) + lens opacities (cataracts \u2014 ophthalmological screening before treatment in those <18 years) + CYP3A4 interaction (rifampicin, azithromycin \u2014 dose adjustment) + skin rash; Trikafta cost: approximately 350,000 CAD\/year without reimbursement \u2014 RAMQ reimbursement radically changes accessibility in Quebec<\/td>\n <\/tr>\n <tr>\n <td>Respiratory physiotherapy and mucociliary clearance<br><small style=\"font-weight:400;color:#7a8fa0;\">Pillar of treatment \u2014 daily for life<\/small><\/td>\n <td>Respiratory physical therapy (RPT) is a fundamental and irreplaceable treatment for cystic fibrosis\u2014its goal is to mobilize and clear thick bronchial secretions that obstruct the airways and serve as a substrate for chronic bacterial colonization; airway clearance techniques (ACT): autogenic drainage (AGD): a technique involving controlled active exhalation at different lung volumes\u2014learned with a physical therapist specializing in CF\u2014the gold standard in Europe and Canada\u2014can be performed independently without equipment; high-frequency oral oscillation (OOFE \u2014 Acapella, Flutter): device creating expiratory oscillatory resistance \u2192 intrabronchial vibrations \u2192 loosening of secretions + improvement in mucociliary clearance; Positive expiratory pressure (PEP): mask or mouthpiece providing resistance to exhalation \u2192 recruitment of collateral alveoli \u2192 mobilization of secretions from the small airways; high-frequency chest oscillation vest (ThAIRapy vest): used primarily in North America + in young children or uncooperative patients; frequency: 1\u20132 sessions\/day of 20\u201340 min \u2014 increased during exacerbations (3\u20134 sessions\/day); mucoclearance-facilitating medications inhaled prior to PTR: hypertonic saline solution (3\u20137 M NaCl \u2014 4 mL nebulized) \u2192 rehydration of the bronchial mucosal surface \u2192 improvement in viscosity; inhaled mannitol (Bronchitol): inhaled osmotic agent \u2014 approved by Health Canada in 2021<\/td>\n <td>PTR should ideally be taught and supervised by a physical therapist specializing in cystic fibrosis at an accredited CF center\u2014in Quebec, adult and pediatric CF centers (CHU Sainte-Justine, CHUM, H\u00f4pital Maisonneuve-Rosemont, CHU de Qu\u00e9bec\u2014IUCPQ) have multidisciplinary teams that include experienced physical therapists; rhDNase (dornase alfa \u2014 Pulmozyme): mucolytic enzyme \u2014 cleaves extracellular DNA released by necrotic polymorphonuclear cells in bronchial secretions \u2192 reduces mucus viscosity \u2014 administered via nebulizer prior to PTR (2.5 mg \u00d7 1\/day) \u2014 reduction in exacerbations of 29% and improvement in FEV1 of 5.8% (Fuchs 1994 NEJM) \u2014 covered by RAMQ; since the introduction of Trikafta, secretion volume and viscosity have decreased significantly within 4 to 8 weeks of initiation \u2014 adjust the duration and intensity of PTR based on clinical response (but maintaining a daily routine is recommended)<\/td>\n <\/tr>\n <tr>\n <td>Antibiotic therapy - infections and exacerbations<br><small style=\"font-weight:400;color:#7a8fa0;\">Maintenance and Exacerbation Strategies<\/small><\/td>\n <td>Antibiotic therapy in cystic fibrosis\u2014three distinct strategies depending on the goal: early eradication of Pseudomonas aeruginosa (primary colonization detected by sputum culture): inhaled tobramycin (TOBI) 300 mg twice daily for 28 days + oral ciprofloxacin 750 mg twice daily for 4 weeks \u2014 eradication rate 75\u201385% % \u2014 objective: to delay chronic colonization; long-term anti-Pseudomonas antibiotic therapy (established chronic colonization): alternating inhaled tobramycin (TOBI \u2014 1 month ON \/ 1 month OFF) OR inhaled aztreonam (Cayston) \u2014 maintenance of pulmonary stability + reduction in exacerbations + slowing of FEV1 decline; oral azithromycin 250\u2013500 mg \u00d7 3\/week: anti-inflammatory + immunomodulatory effect (non-antibiotic) \u2014 reduces exacerbations by 35\u201340% in patients colonized with P. aeruginosa \u2014 precaution: do not use if active nontuberculous mycobacteria (masks and promotes resistance); treatment of acute pulmonary exacerbations: hospitalization criteria \u2014 FEV\u2081 drop >10% + significant increase in secretions + hemoptysis + SpO\u2082 <95% + patient unable to maintain outpatient care; IV antibiotics: based on antibiotic susceptibility testing (recent sputum cultures required) \u2014 P. aeruginosa: piperacillin-tazobactam 4.5 g \u00d7 4\/day IV + tobramycin IV (once daily \u2014 10\u201312 mg\/kg\/day \u2014 pharmacokinetic monitoring required \u2014 nephrotoxicity + ototoxicity) \u2014 duration: 14\u201321 days IV or until return to baseline FEV1<\/td>\n <td>Managing bacterial resistance is a major challenge in cystic fibrosis\u2014patients are exposed to dozens of courses of antibiotics over the course of their lives \u2192 emergence of multidrug-resistant (MDR) P. aeruginosa + B. cepacia complex + nontuberculous mycobacteria (M. abscessus\u2014complex treatment: IV amikacin + imipenem + cefoxitin \u00b1 azithromycin \u00d7 12 months); segregation of patients in hospitals and clinics: patients colonized with B. cepacia complex (particularly B. cenocepacia) must never come into contact with other CF patients \u2014 single rooms mandatory \u2014 risk of interpatient transmission and fulminant cepacia syndrome + relative contraindication for lung transplantation (B. cenocepacia \u2014 very high post-transplant mortality) ; ototoxicity of aminoglycosides (tobramycin): annual audiometric screening in patients receiving inhaled or IV tobramycin \u2014 nephrotoxicity: creatinine + clearance before each IV course + peak and trough pharmacokinetic measurements; Trikafta significantly reduces the frequency of exacerbations requiring IV antibiotics (63% reduction in % trials) \u2192 decreased cumulative exposure to aminoglycosides \u2192 reduced risk of ototoxicity<\/td>\n <\/tr>\n <tr>\n <td>Nutritional and pancreatic support<br><small style=\"font-weight:400;color:#7a8fa0;\">PERT \u2014 pancreatic enzymes \u2014 fat-soluble vitamins<\/small><\/td>\n <td>Exocrine pancreatic insufficiency (85\u201390% of patients): pancreatic enzyme replacement therapy (PERT): Creon (pancreatin\u2014lipase + amylase + protease in the form of enteric-coated, gastro-resistant microspheres)\u2014take at the start of a meal or snack\u2014dosage: 500\u20132,500 IU lipase\/kg\/meal (max 10,000 IU lipase\/kg\/day) \u2014 titrate based on steatorrhea and weight gain; nutritional goals in FK: target BMI \u226522 kg\/m\u00b2 (women) and \u226523 kg\/m\u00b2 (men) \u2014 1\u20133 times the expected weight \u226590% of the recommended intake in children; high-calorie, high-fat diet (caloric intake 110\u2013200% of recommended intake) \u2014 no fat restriction (unlike standard pancreatitis); mandatory fat-soluble vitamin supplementation: vitamin A (10,000 IU\/day) + vitamin D (800\u20132,000 IU\/day \u2014 annual 25-OH-D measurement \u2014 target 75\u2013120 nmol\/L) + vitamin E (100\u2013400 IU\/day) + vitamin K (coagulopathy prevention \u2014 1\u201310 mg\/day); additional salt in summer or during intense exercise (excessive NaCl loss in sweat); nocturnal enteral nutrition via nasogastric tube or gastrostomy: if BMI <18 or weight <85 kg despite optimal oral management \u2014 often prescribed for adolescents during growth spurts; CF-related diabetes (CRD): rapid-acting insulin therapy at meals + nocturnal basal insulin if fasting hyperglycemia \u2014 avoid oral antidiabetics (except metformin as a second-line treatment) \u2014 specialized endocrinological follow-up; osteoporosis: bone densitometry starting at age 18 + bisphosphonates if T-score <\u22122.5<\/td>\n <td>Nutrition is a major independent prognostic factor in CF \u2014 nutritional status is directly correlated with lung function and survival (BMI <18 associated with accelerated FEV1 decline and increased mortality); since Trikafta: significant improvement in nutritional status in the first months of treatment (increase in body weight + improved fat absorption) \u2014 possible reduction in PERT doses needed in some patients (adjustment guided by clinical steatorrhea and nutritional status, not empiric cessation); CF-related diabetes (CFRD) is often asymptomatic initially \u2014 annual screening with oral glucose tolerance test (OGTT 75g \u2014 2h) recommended from age 10 in all patients \u2014 screening with HbA1c is insufficient in CF (accelerated erythrocyte turnover + anemia) \u2014 use postprandial glycemia; liver transplantation: indicated in decompensated cirrhosis (Child-Pugh C) \u2014 can be performed in combination with lung transplantation in some patients<\/td>\n <\/tr>\n <tr>\n <td>Lung transplantation<br><small style=\"font-weight:400;color:#7a8fa0;\">End-stage \u2014 FEV1 <30% of predicted or rapid decline<\/small><\/td>\n <td>Bilateral lung transplantation (double-lung transplant) is the last-resort treatment for end-stage respiratory failure associated with cystic fibrosis\u2014CF is the third most common indication for lung transplantation worldwide (after COPD and idiopathic pulmonary fibrosis); Transplantation criteria (Cystic Fibrosis Foundation + ISHLT): FEV1 <30% of predicted (or decline >20% of predicted value within 12 months) + resting SpO\u2082 <90% on oxygen or during exercise + hypercapnia (PaCO\u2082 >50 mmHg) + frequent exacerbations requiring hospitalization + severe treatment-resistant malnutrition + PAH + recurrent massive hemoptysis; relative or absolute contraindications: colonization with B. cenocepacia (genotype IIIA \u2014 post-transplant mortality of 50\u201375% at 1 year \u2014 contraindication in most centers, including the IUCPQ) + active, uncontrolled M. abscessus + severe obesity (BMI >35) + severe uncorrectable renal or hepatic insufficiency + documented non-adherence to therapy + major comorbidities; lung transplantation program in Quebec: IUCPQ (Quebec University Institute of Cardiology and Pulmonology) \u2014 the only lung transplantation center in Quebec + pre-transplant follow-up centers (CHUM, CHU Sainte-Justine); post-transplant survival FK: 80 % at 1 year \/ 55\u201360 % at 5 years \/ 40\u201345 % at 10 years (ISHLT registry 2022) \u2014 better outcomes in FK patients than for other indications (younger population + fewer comorbidities); complications: chronic rejection (CLAD\u2014bronchiolitis obliterans) + opportunistic infections (bacterial, viral [CMV], fungal) + immunosuppressant toxicity<\/td>\n <td>Since the introduction of Trikafta, the number of CF patients on transplant waiting lists has decreased significantly in countries where Trikafta is covered\u2014some patients awaiting transplantation have even been removed from the list after starting Trikafta due to the clinical improvement achieved; however, Trikafta does not reverse irreversible lung damage that has already occurred (bronchiectasis, parenchymal destruction)\u2014patients with very low FEV1 (<30\u201340% of predicted) may have a partial response and remain candidates for transplantation even while on Trikafta; Management of CF medications post-transplant: CFTR modulators (Trikafta) are generally continued after transplantation\u2014they continue to benefit non-transplanted organs (pancreas, sinuses, intestines, genital tract) \u2014 interactions with immunosuppressants (tacrolimus + cyclosporine \u2014 CYP3A4 substrates) require adjustment of immunosuppressant doses (a 50% reduction in tacrolimus is recommended upon initiation of Trikafta) + monitoring of residual levels; palliative care: for patients ineligible for transplantation or in the terminal phase \u2014 specialized palliative care program + cystic fibrosis team + ambulatory oxygen therapy + non-invasive ventilation + advance discussion of care directives<\/td>\n <\/tr>\n <\/tbody>\n <\/table>\n\n <div class=\"co-infobox\">\n <span class=\"ico\">\u2139\ufe0f<\/span>\n <span><strong>Trikafta and access in Quebec \u2014 an accessible revolution:<\/strong> Elexacaftor-tezacaftor-ivacaftor (Trikafta) has been reimbursed by the RAMQ in Quebec since November 2021 for patients aged 12 and over with at least one F508del mutation, and since 2023 for children aged 2 and over according to Canadian guidelines. For eligible patients with other non-F508del mutations, an exceptional access request (exception drug) is possible through the RAMQ upon recommendation from a CF-specialized pulmonologist. Cystic Fibrosis Canada (cysticfibrosis.ca) offers access support resources and a patient assistance program. Quebec patients whose genotype is not covered by Trikafta are followed in compassionate access programs or ongoing clinical trials for new modulator combinations (vanzacaftor-tezacaftor-deutivacaftor \u2013 VX-522 \u2013 in phase 3).<\/span>\n <\/div>\n\n <div class=\"co-urgence\">\n <div class=\"co-urgence-titre\">Emergency \u2014 Severe exacerbation and massive hemoptysis<\/div>\n <p>Dial <strong>911<\/strong> or go immediately to the emergency room if : <strong>Sudden shortness of breath + SpO\u2082 <90% % + rapid decline in respiratory function<\/strong> Severe pulmonary exacerbation - IV antibiotics + intensive inpatient PTR.<\/p>\n <p><strong>Massive hemoptysis (>240 mL\/24h or massive hemoptysis)<\/strong> Call 911. Affected lateral position. No anticoagulants. Emergency bronchial artery embolization at a specialized center.<\/p>\n <p><strong>Sudden chest pain + sudden shortness of breath + unilateral absence of breath sounds<\/strong> \u2192 pneumothorax \u2014 surgical emergency \u2192 chest drain.<\/p>\n <\/div>\n\n <h2>Consult at Clinique Omicron<\/h2>\n <p>Les m\u00e9decins de Clinique Omicron assurent le suivi de premi\u00e8re ligne des patients atteints de fibrose kystique en collaboration avec les centres sp\u00e9cialis\u00e9s FK au Qu\u00e9bec : gestion des infections intercurrentes, renouvellement des ordonnances, suivi nutritionnel, d\u00e9pistage du diab\u00e8te li\u00e9 \u00e0 la FK et coordination des demandes de remboursement des modulateurs CFTR (RAMQ). Des consultations sont disponibles dans nos points de service au Qu\u00e9bec et via la t\u00e9l\u00e9m\u00e9decine. Pour prendre rendez-vous, <a href=\"https:\/\/cliniqueomicron.ca\/rendez-vous\/\" data-oc-cta=\"fiche-consulter\">choisissez votre service en ligne<\/a>.<\/p>\n\n <p class=\"co-disclaimer\">The content of this page is provided for informational purposes only and does not substitute for the advice of a qualified healthcare professional specializing in cystic fibrosis. Optimal management of cystic fibrosis requires a multidisciplinary team at an accredited center\u2014pulmonologist, physical therapist, nutritionist, specialized nurse, and social worker.<\/p>\n<\/div>\n<\/body>\n<\/html>\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>","protected":false},"excerpt":{"rendered":"<p>Fibrose kystique (mucoviscidose) : sympt\u00f4mes, diagnostic et traitement | Clinique Omicron Pneumologie & G\u00e9n\u00e9tique m\u00e9dicale & M\u00e9decine de famille Fibrose kystique (mucoviscidose) La fibrose kystique \u2014 d\u00e9sign\u00e9e au Qu\u00e9bec et au Canada par ce terme, et connue sous le nom de mucoviscidose en Europe \u2014 est la maladie g\u00e9n\u00e9tique h\u00e9r\u00e9ditaire grave la plus fr\u00e9quente dans… <a href=\"https:\/\/cliniqueomicron.ca\/en\/fibrose-kystique\/\" rel=\"bookmark\">Read More \"<span class=\"screen-reader-text\">Cystic Fibrosis (Mucoviscidosis): Symptoms, Diagnosis, and Treatment | Clinique Omicron<\/span><\/a><\/p>","protected":false},"author":1,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"om_disable_all_campaigns":false,"neve_meta_sidebar":"","neve_meta_container":"","neve_meta_enable_content_width":"off","neve_meta_content_width":100,"neve_meta_title_alignment":"","neve_meta_author_avatar":"","neve_post_elements_order":"","neve_meta_disable_header":"","neve_meta_disable_footer":"","neve_meta_disable_title":"","_themeisle_gutenberg_block_has_review":false,"_metasync_otto_title":"","_metasync_otto_description":"La fibrose kystique est une maladie g\u00e9n\u00e9tique touchant les poumons et le pancr\u00e9as. 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