Bronchiectasis and cystic fibrosis Flashcards
What is bronchiectasis?
Chronic dilatation of one of more bronchi. The bronchi exhibit poor mucus clearance and there is predisposition to recurrent or chronic bacterial infection.
On radiological investigation, you would see that the bronchial dilation is bigger than the adjacent blood vessel and the bronchial wall is thicker.
Therefore, the gold standard diagnostic investigation is a CT.
What is a signet ring sign?
Seen in bronchiecasis - This is the bronchial wall thickening.
What are the symptoms of bronchiectasis?
Very Common:
Chronic cough
Daily septum production
Common: Breathlessness on exertion Intermittent haemoptysis Nasal symptoms Chest pain Fatigue
Less common:
Wheeze
What are some common causes of bronchiectasis?
Post infective - (whooping cough, TB) infection causes scaring
Immune deficiency - Hypogammaglobulinaemia
Mucociliary clearance defects - CF
What are common organisms causing Bronchiectasis?
Haemophilus influenzae
Pseudomonas aeruginosa
Moraxella catahhhalis
How do you find out if a patient has bronchiectasis?
Patient labelled as “asthma” without any objective evidence, particularly lifelong
History of severe chest infection earlier in life
Lifelong chest infections - genetic
Nasal / ear symptoms or admitted to SCBU at brith
Recurrent chest infections - immunodeficiency
Septum culture positive for common organsisms
IBD, RA
How do you manage bronchiectasis?
Physio / airway clearance
Sputum sampling - routine culture and NTM
Exclude immunodeficiency / treat identifiable causes
Management plan for infective exacerbations
Consider long-tern treatment therapy’s at future visits
Flu vaccine
Pulmonary rehabilitation of MRC dyspnoea score >3
An established MDT is key
How do you define an exacerbations of bronchiectasis?
A person with bronchiectasis with deterioration in 3 or more key symptoms for at least 48 hours:
Cough Sputum volume / consistency Sputum purulence Breathlessness / exercise tolerance Fatigue Haemoptysis
What is the pathophysiology of cystic fibrosis?
Autosomal recessive
Long arm chromosome 7 defect
Leads to CFTR mutation
Causes ineffective cell surface chloride transport
Leads to thick dehydrated body fluids in organs which have CFTR
How do you diagnose CF?
One or more characteristic phenotypic features:
- CF in sibling
- Positive newborn screening result
AND
- An increased sweat chloride concentration - SWEAT TEST
- Identification of two CF mutations - genotyping - may need extended genotypes
How does CF present?
Meconium lieus - newborn bowel blocked by sticky secretions. Signs of obstruction soon after brith inc bilious vomitting, abdominal distention and delay in passing Meconium.
Intestinal malabsorption
Chest infections
Newborn screening
What is Median predicted survival for CF?
46 - has improved significantly.
What are complications of CF?
Lungs - bronchiectasis, pneumothorax, ABPA, haemoptysis, respiratory failure
Nasal / upper respiratory tract - chronic sinusitis, nasal polyposis
Pancreas - Pancreatic insufficiency
GUT - DIOS, oesophageal reflux / oesophagitis
Liver - Chronic liver disease, portal hypertension
Biliary tree - Gall stones
Heart - Cardiac failure
Joints and bones - arthritis, osteoporosis
Reproductive tract - male infertility, CBVD
What is CF lifestyle advice?
No smoking
Avoid other CF patients - as can contract disease
Avoid friends / relatives with colds or infections
Avoid jacuzzi’s (pseudomonas)
Clean and dry nebulisers thoroughly
Avoid stables, compost or rotting vegetation - risk of aspergillosis fumigated inhalation
Annual flu vaccine
NaCl tablets in hot weather / vigorous exercise
How do you mange CF?
Complex - managed by CF specialist centres / MDT’s
Holistic care / multisystem (organ) focus
Key is maintaining lung health (chest, physio, infection, management) and nutritional state - BMI, pancreatic status, vitamin status
Targeted therapies
Managing other co-morbiities e.g. diabetes, liver disease
Concordance (physio and drugs) and other psychosocial factors
