# Cystic Fibrosis - Category: chronic - Review status: reviewed (every claim checked against a document named on this page) - Sources: Cystic Fibrosis - StatPearls (NCBI Bookshelf NBK493206) - https://www.ncbi.nlm.nih.gov/books/NBK493206/ · ICPC-3 (WONCA International Classification of Primary Care, 3rd edition) class RD99.02 - condition scope only, no dose · No dose - referral pathway, no medicine given in primary care · Egyptian National Drug Formulary - Respiratory Medicines 2026 (Ipratropium Bromide monograph) · Egyptian National Drug Formulary - Respiratory Medicines 2026 (Salbutamol monograph) - Verified date: 2026-08 ## Verified against - No dose - referral pathway, no medicine given in primary care ## Treatment metadata - Referral & safety-netting (no drug therapy) - Salbutamol — oral.inhalation - Ipratropium bromide — oral.inhalation ## Complete treatment card ```text CYSTIC FIBROSIS Sources: Cystic Fibrosis - StatPearls (NCBI Bookshelf NBK493206) - https://www.ncbi.nlm.nih.gov/books/NBK493206/ · ICPC-3 (WONCA International Classification of Primary Care, 3rd edition) class RD99.02 - condition scope only, no dose · No dose - referral pathway, no medicine given in primary care · Egyptian National Drug Formulary - Respiratory Medicines 2026 (Ipratropium Bromide monograph) · Egyptian National Drug Formulary - Respiratory Medicines 2026 (Salbutamol monograph) Review status: REVIEWED against the source listed above (2026-08) IS IT THIS? - reference only, to read alongside your own examination SYMPTOMS - what the patient reports (7) - Newborn screening can identify cystic fibrosis before any clinical symptoms appear - Some newborns present with meconium ileus, prolonged jaundice, or difficulty thriving [jaundice] - Infants and children may show failure to thrive, anemia, undescended testes, and recurrent sinopulmonary infections [anaemia · failure to thrive] - Median age at diagnosis is 6 to 8 months, though some are not diagnosed until later - Adults may report chronic sinusitis, postnasal drip, and nasal polyps [nasal polyps] - Salty-tasting sweat is a classic feature - Male infertility from an absent vas deferens; thickened cervical mucus affects female fertility SIGNS - what you find (3) - Digital clubbing and cyanosis may be seen on exam [cyanosis · finger clubbing] - Exaggerated palm wrinkling occurs after brief water immersion (the water immersion test) - A pulmonary exacerbation brings breathlessness, tiredness, a productive cough, and fever [breathlessness · cough · fatigue · fever] TESTS (7) - Sweat chloride above 60 mEq/L meets the laboratory criterion for cystic fibrosis - Finding two disease-causing CFTR mutations confirms the diagnosis - Diagnosis starts with sweat chloride testing, repeated if normal but symptoms persist - Immunoreactive trypsinogen (IRT) testing improves newborn screening sensitivity for cystic fibrosis - Chest x-ray may show hyperinflation, bronchiectasis, abscesses, or atelectasis - Bronchoalveolar lavage shows many neutrophils and often grows Pseudomonas or other organisms - Low FEV1 with preserved FVC on spirometry indicates obstructive disease with air trapping IF NOT THIS - what else fits (3) - Asthma is on the differential list - Bronchiectasis is a differential to distinguish from cystic fibrosis - Primary ciliary dyskinesia is on the differential list Source StatPearls "Cystic Fibrosis" - disease-level clinical article Status traced to the source above Rx: Main treatment | Short-acting bronchodilator for wheeze | Added to salbutamol when wheeze persists MAIN TREATMENT 1. REFERRAL & SAFETY-NETTING (NO DRUG THERAPY) [1st line] Adult A genetic disorder causing thick mucus secretions that lead to recurrent lung infections and pancreatic insufficiency; rare but not absent in Egypt given the effect of consanguinity on recessive-disease risk. Management is specialist, at a paediatric respiratory or CF centre, with enzyme replacement and airway clearance, so the GP's role is recognition and referral. - Refer, with advice Peds Children follow the same pathway: recognise and refer. No primary-care medicine is implied. Source No dose - referral pathway, no medicine given in primary care Why A genetic disorder causing thick mucus secretions that lead to recurrent lung infections and pancreatic insufficiency; rare but not absent in Egypt given the effect of consanguinity on recessive-disease risk. Management is specialist, at a paediatric respiratory or CF centre, with enzyme replacement and airway clearance, so the GP's role is recognition and referral. Caution Rare in Egyptian primary care. Referral is the pathway; the medicines listed alongside are what primary care can give before or while it happens. RED FLAG - Recurrent chest infections with failure to thrive in an infant, meconium ileus in a newborn, or salty-tasting skin reported by parents: refer for specialist assessment. SHORT-ACTING BRONCHODILATOR FOR WHEEZE 2. SALBUTAMOL [1st line] Adult One or two inhalations of 100 micrograms up to 4 times a day; no more than 8 inhalations in 24 hours and usually not repeated inside 4 hours Peds Under 12 years: one or two inhalations of 100 micrograms up to 4 times a day, not exceeding 800 micrograms daily and not more than 6 times in 24 hours. Source Egyptian National Drug Formulary - Respiratory Medicines 2026 (Salbutamol monograph) - verbatim: "Oral inhalation, one inhalation (100 mcg) or two inhalations up to 4 times a day for persistent symptoms." Why Cystic-fibrosis airways obstruct and wheeze like asthma, and an inhaler is the one thing a primary-care doctor can hand the patient the same day while specialist care is arranged. Caution Tremor and jitteriness are common and dose-related, and children may become noticeably excitable. Palpitations and a fast pulse can occur; check the pulse if the patient is using it often. Needing it more and more often means the disease is worsening - that is a reason to escalate care, not to increase the inhaler. Do not use it in anyone hypersensitive to salbutamol or to any part of the formulation. Egypt AEROLIN 100MCG/DOSE INHALER EIPICO > 3M H... 7.30 EGP BUTALIN 100MCG/ACTUATION 200 DOSES JULPHAR 12.00 EGP SALBOVENT RESPIRATOR 5MG/ML SOLN. 20 ML ALEXANDRIA 17.50 EGP BRONCHOLIN S 0.5% RESPIRATOR SOLN. 30 ML EIPICO 37.00 EGP ALVEOTRACK 5 MG/ML INH. SOLN. 30 ML MDI PHARMA 49.00 EGP VENTAL INHALER 100 MCG/DOSE 200 DOSES ARAB DRUG COMPANY (ADCO) 72.00 EGP VENTOLIN DISKUS 200MCG/DOSE ACCUHALER GLAXO SMITHKLINE 86.00 EGP VENTOLIN EVOHALER 100MCG/ACTUATION INHALER GLAXO SMITHKLINE 86.00 EGP OPICHESTAL SYRUP 120ML EL-OBOUR 2.00 EGP -> ? strength differs, ? different route - not oral.inhalation SALBUTAMOL 2MG/5ML SYRUP 120ML SEDICO 3.50 EGP -> ? strength differs, ? different route - not oral.inhalation ADDED TO SALBUTAMOL WHEN WHEEZE PERSISTS - give alongside 3. IPRATROPIUM BROMIDE [add-on - not a substitute] Adult Nebulised 250 to 500 micrograms 3 to 4 times a day; 500 micrograms for an acute episode Peds 6 to 12 years: 250 micrograms a dose, up to 1 mg in total each day. No dose under 6 years in the sources opened. Source Egyptian National Drug Formulary - Respiratory Medicines 2026 (Ipratropium Bromide monograph) - verbatim: "Maintenance therapy: 250 - 500 micrograms 3 to 4 times daily." Why Adding an anticholinergic to the beta-2 agonist widens the airway a little further in cystic-fibrosis airway obstruction; it is a supportive measure, not a substitute for specialist care. Caution Do not use it in anyone allergic to ipratropium or to atropine. Keep the nebuliser mist out of the eyes - it can blur vision and raise eye pressure in glaucoma. This is symptom relief only. Cystic fibrosis is managed by a specialist centre and the referral still stands. Egypt ASMATROPIM 250MCG/ML 20 NEBULIZER VIAL SOLN. GLOBAL ADVANCED PHARMACEUTI... 70.00 EGP IPRATROPIUM VIATRIS 0.25MG/ML (CHILDREN) 10 UNIT DOSE VIAL LABORATOIRE U... 74.00 EGP IPRATROPIUM VIATRIS 0.5MG/2ML (ADULTS) 10 UNIT DOSE VIAL LABORATOIRE UNI... 80.00 EGP LUNGOTROPIUM 250MCG/1ML 20 UNIT DOSE VIAL NEBULIZER SOLN EVA PHARMA 152.00 EGP ASMATROPIM 500MCG/2ML 20 NEBULIZER VIAL SOLN. GLOBAL ADVANCED PHARMACEUT... 164.00 EGP LUNGOTROPIUM 500MCG/2ML 20 UNIT DOSE VIAL NEBULIZER SOLN EVA PHARMA 164.00 EGP ATROVENT 250MCG/2ML 20 UNIT DOSE INH. VIAL. BOEHRINGER INGELHEIM 286.00 EGP ATROVENT 500 MCG/2ML 20 UNIT DOSE INH. VIAL. BOEHRINGER INGELHEIM 286.00 EGP Prices are indicative (dataset snapshot 2026-06); verify with the pharmacy. ``` --- Dawaa Reference is a reference for prescribers, not a medical device, and does not replace clinical judgement. [Privacy policy](/privacy)