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Pulmonary exacerbations
For minor respiratory exacerbations ( where FEV 1 typically decreases by less than 10 % predicted ), hospital
admission is not usually required . 32 Management includes sending a sputum sample for culture , a viral PCR , starting oral antibiotics ( usually based on the most recent sputum sample ) and increased physiotherapy . Review in 5-7 days is recommended , and if there is no improvement , contact the CF team .
Major exacerbations include deterioration in respiratory status combined with systemic symptoms , such as high fever , anorexia and weight loss . 32 FEV 1 typically decreases by more than 10 % predicted . An admission to hospital ( or hospital in the home ) for IV antibiotics , based on recent sputum culture , and intensive physiotherapy are appropriate .
Haemoptysis
Haemoptysis is a relatively common complication of CF and is a major cause of morbidity and mortality . 33 It is believed to occur because of persistent airway inflammation and infection weakening the blood vessel wall and promoting proliferation of tortuous fragile bronchial arteries closely associated with bronchi . 33 Massive haemoptysis occurs in about 1 % of patients with CF each year and is more common in adult patients with severe pulmonary disease . 34
Managing haemoptysis can be challenging , and consensus guidelines recommend treating mild , moderate and massive haemoptysis with antibiotics . However , beyond bronchial artery embolisation in severe or persistent haemoptysis , other treatment data are limited . 34 Systemic antifibrinolytic therapy has been shown to reduce hospital admission , with no serious adverse events . 34 Encourage patients to contact their CF team for advice when haemoptysis occurs . They are often advised to cease their regular chest physiotherapy until bleeding resolves .
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Respiratory
Gastrointestinal
Endocrine
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• Chronic suppurative lung disease with airflow obstruction and chronic airway infection
• Bronchiectasis ( see figure 5 )
• Pneumothorax
• Haemoptysis
• Allergic bronchopulmonary aspergillosis ( see figure 6 )
• Respiratory failure and pulmonary hypertension
• Chronic rhinosinusitis and nasal polyposis ( see figure 7 )
• Digital clubbing
• Meconium ileus
• Rectal prolapse
• Constipation , delayed gastric emptying and recurrent distal intestinal obstruction syndrome
• Gastro-oesophageal reflux
• Intussusception
• Inflammatory bowel disease
• Colorectal cancer and colonic polyposis
• Pancreatic insufficiency : steatorrhoea , fat-soluble vitamin deficiency ( vitamins A , D , E , K ), faltering growth
• Recurrent acute pancreatitis ( in patients with pancreatic sufficiency )
• Focal biliary or multilobar cirrhosis complicated by portal hypertension
• Biliary sludge / cholelithiasis
• CF-related diabetes ( microvascular complications 10 or more years from diagnosis )
• Functional hypogonadism
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Infants and toddlers
Preschoolers
School-age children
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• Persistent moist cough or cough that recurs after antibiotics are ceased
• Weight loss ; failure to thrive ; oily , offensive stools
• Less commonly , haemolysis due to vitamin E deficiency , prolonged neonatal conjugated jaundice
• Persistent moist cough
• Finger clubbing
• Hyponatraemia and dehydration ( children with CF are at risk of developing electrolyte abnormalities . Pseudo-Bartter syndrome — which is defined as hypokalaemic hypochloraemic metabolic alkalosis in the absence of renal tubular pathology — may occur )
• Oedema from prolonged malabsorption
• Rectal prolapse
• Typically , thin children who are mislabelled as having severe asthma and are thought to be steroid resistant
• Finger clubbing
• Recurrent chest infections isolating S . aureus and / or Pseudomonas aeruginosa
• Sinusitis and / or the presence of nasal polyps
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Musculoskeletal |
• CF-related bone disease : osteoporosis
• Rib and vertebral fractures
• Kyphosis
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Genitourinary |
• Male : congenital bilateral absence of vas deferens
• Female : urinary incontinence , subfertility ( 85 % can conceive naturally within a year of ceasing contraception )
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Renal • Nephrolithiasis ( oxalate stones )
• Nephrotoxicity ( eg , aminoglycoside , CFRD related )
Other features of CF and / or its treatment
• Allergic reactions
• Drug toxicity
• Depression / anxiety
• Venous access devices and their complications
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Source : Bell SC et al 2020 6 , Fitzgerald D 2008 14 |
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Table 3 . Common regular medications used in cystic fibrosis
System
Medication
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Cystic fibrosis – related diabetes
In the pancreas , the abnormal CFTR
protein results in both exocrine and endocrine dysfunction . In 2021 , 18.3 % of Australians with CF were reported to have CF-related diabetes ( CFRD ); the prevalence of diabetes increases with age , with the condition present in 30 % of those with CF who are over 30 . 9
The development of CFRD often leads to poorer outcomes , including increases in pulmonary exacerbation , poorer lung function and early mortality . 35 International CF guidelines recommend screening for CFRD starting at age 10 using the oral glucose tolerance test . 36 There is , however , emerging evidence suggesting that non-diabetic early glucose abnormalities may also be important in lung function and nutrition and need to be identified and treated to optimise patient outcomes . 35 Insulin therapy is the only recommended treatment for CFRD and is typically given in a basal – bolus format via injection or insulin pump . 37
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Respiratory
Gastrointestinal
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• Airway clearance — Hypertonic saline ( 3 % or 6 %) — Dornase alpha — Mannitol
• Antibiotic prophylaxis — Oral flucloxacillin ( in some clinics given in first 1-3 years of life or if frequent exacerbations )
— Oral trimethoprim / sulfamethoxazole ( sometimes used in older children with chronic S . aureus culture and repeated exacerbations )
• Airway inflammation treatment — Azithromycin ( dose taken three times a week )
• Treatment for allergic bronchopulmonary aspergillosis — Itraconazole plus oral prednisone — Omalizumab : anti-IgE monoclonal antibody
• Treatment for chronic pseudomonas infection — Inhaled antibiotics ( eg , tobramycin or colistin )
• Pancreatic insufficiency — Pancrelipase ( Creon or Panzytrat ) — Fat-soluble vitamins ( A , B , D , E , C , K )
• Constipation and distal intestinal obstruction syndrome ( see figure 10 ) — Macrogol with electrolytes
• Gastric acid – reducing medication — Omeprazole
• Salt replacement — Sodium chloride tablets
• Liver disease — Ursodeoxycholic acid
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Allergic bronchopulmonary aspergillosis
Aspergillus sp . are found in water ,
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Endocrine |
• CF-related diabetes — Insulin
• Bone disease — Oral or IV bisphosphonates
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Figure 5 . CT scan images showing bilateral bronchiectasis in an 18- year-old male with cystic fibrosis . |