medical respiratory conditions

Question 1

COPD

Answer 1

COPD is common, preventable and treatable disease that is characterised by persistent respiratory symptoms and airflow limitation that is due to airway and/or alveolar abnormalities usually caused by significant exposure to noxious particles and gases.

The chronic airflow limitation is a mixture of small airway disease and parenchymal destruction, the relative contributions of each vary from person to person and develop at different rates

Question 2

emphysema
Chronic bronchitis
asthma

Answer 2

Emphysema: the destruction of the gas exchanging surface of the lung.

Chronic bronchitis: the presence of cough and sputum production for 3 months in two consecutive years.

Asthma: characterized by symptoms of breathlessness and wheezing, which vary in severity and frequency, due to inflammation of the air passages in the lungs and affects the sensitivity of the nerve endings in the airways so they become easily irritated.

Question 3

factors influencing disease development and progression

COPD

Answer 3

Smoking is the strongest risk factor for COPD

Exposure to pollutants

Age

Sex (women tend to have more severe disease)

Genetic factors (Alpha anti trypsin deficiency accounts for a small genetic predisposition)

Socioeconomic status

Infections

Question 4

symptoms

COPD

Answer 4

Shortness of breath: progressive over time, worse with exercise, persistent

Cough: often first symptom encountered. May be intermittent.

Sputum production: cough with sputum production is only present in 30% of patients

Wheezing and chest tightness: variable

Fatigue and weight loss

Question 5

morbidity

COPD

Answer 5

COPD is associated with significant systemic abnormalities

Cardiovascular disease

Diabetes mellitus

Musculoskeletal disorders, muscle wasting

Renal abnormalities

Hormonal abnormalities

Lung cancer

Question 6

diagnosis

COPD

Answer 6

COPD should be considered in any patient who has dyspnea, chronic cough or sputum production, and/or history of exposure to risk factors for the disease i.e. smoker

Detailed medical history

Spirometry is required to make the diagnosis; the presence of a post-bronchodilator FEV1/FVC < 0.70 confirms the presence of persistent airflow limitation and thus of COPD in patients with appropriate symptoms and significant exposures to risk factors

FEV1: Forced Expiratory Volume in one second – measures thevolume of expiredair over the firstsecond of an FVCmanoeuvre.
Healthy lungs will exhale >80%

FVC: Forced Vital Capacity- is the amount of air that can be forcibly exhaled from your lungs after taking the deepest breath possible, as measured by spirometry.
Healthy lungs: 4-6 seconds
Severe obstruction can take up to 15 seconds

Measured in litres.

GOLD stage 1: mild FEV>/= 80% predicted
GOLD stage 2: moderate 50% = FEV1% <80% predicted
GOLD stage 3: Severe 30% = FEV1<50% predicted
GOLD Stage 4: Very severe FEV1 <30%

Question 7

Borg breathlessness scale

Answer 7

0 - nothing at all 
0.5 very v slight 
1 v slight 
2 slight 
3 moderate 
4 somewhat severe
5 severe
6 
7 v severe 
8
9 v v severe 
10 maximal

Question 8

CAT assessment of symptoms

Answer 8

add up scores to get total 
Impact level of COPD on health status 
<10 - low 
10-20 medium 
21-30 high 
>30 very high

Question 9

management

COPD

Answer 9

The management strategy for stable COPD should be predominantly based on the individualised assessment of symptoms and future risk of exacerbations.

The main treatment goals are reduction of symptoms and future risk of exacerbations.

Pharmacological and non pharmacological treatments

Pharmacological treatment
Bronchodilators (long acting and short acting, combination therapy)
Anti muscarinic agents
Anti inflammatory (Corticosteroids)
Mucolytics
Theophylline (mainly acts as a bronchodilator)
Oxygen therapy

Non pharmacological treatment
Pulmonary rehabilitation 
Smoking cessation
Non invasive ventilation 
Nutrition 
Airway clearance techniques
Surgery (lung volume reduction surgery, lung transplant)
 Bronchoscopic interventions (valve, lung coils, vapour ablation)

Question 10

goals of management

COPD

Answer 10

Relieve symptoms
Improve exercise tolerance
Improve health status

Prevent disease progression
Prevent and treat exacerbations
Reduce mortality

Question 11

COPD vs asthma

Answer 11

COPD

• onset = midlife
• symptoms slow progressive
• long smoking history
• dyspnoea during exercise
• largely irreversible airflow limitation

Asthma

• onset in early life
• symptoms vary day to day
• may have associated allergic disease
• largely reversible airflow limitation

Question 12

asthma

Answer 12

“Asthma is a chronic inflammatory disorder of the airways in which many cells and cellular elements play a role: in particular, mast cells, eosinophils, T lymphocytes, macrophages, neutrophils, and epithelial cells. In susceptible individuals, this inflammation causes recurrent episodes of wheezing, breathlessness, chest tightness, and coughing, particularly at night or in the early morning. These episodes are usually associated with widespread but variable airflow obstruction that is often reversible either spontaneously or with treatment. The inflammation also causes an associated increase in the existing bronchial hyper responsiveness to a variety of stimuli. Reversibility of airflow limitation maybe incomplete in some patients with asthma.”

Question 13

asthma Causes and prevalence

Answer 13

causes
allergies - pollen animals
irritants - pollution
exercise

prevalence The prevalence of asthma in children in 21.5% and 7-9.4% in adults in Ireland

<75 people per year die of asthma, 70% of those are over 70.

As it typically begins earlier in life than many other chronic diseases, it can impose a high lifetime burden on individuals, caregivers and the community.

Question 14

asthma diagnosis

Answer 14

A history of variable respiratory symptoms

Evidence of variable airflow limitation
At least once during the diagnostic process, document that FEV1/FVC ratio is below normal (0.75-.8 in adults and <0.85 in children)

Document that variation in lung function is greater than in healthy people
FEV1 increased by >200ml or 12% of baseline value (or 12% of predicted value in children) after inhaling a bronchodilator
Average daily diurnal PEF variability is >10% and >13% for children
FEV1 increases by more than 200ml and 12% from baseline (or 12% of predicted value for children) after 4 weeks of anti inflammatory treatment

The more variation see the more confident you can be in your diagnosis of asthma

Physical examination (is often normal) but may hear wheezing on auscultation

Question 15

Asthma treatment

Answer 15

Inhaled corticosteroids: Treatment of asthma with short-acting bronchodilators alone is no longer recommended for adults and adolescents.

Long acting bronchodilator

Short acting bronchodilator (reliever): Treatment of asthma with short-acting bronchodilators alone is no longer recommended for adults and adolescents

Breathing retraining exercises

Question 16

non -CF bronchiectasis

Answer 16

term used for permanent dilation of one or more bronchi

Question 17

bronchiectasis

Answer 17

The bronchii become dilated and the wall of the bronchii becomes damaged and functions less effectively.
The cilia on the wall of the affected bronchus are destroyed, so that mucus is no longer cleared away and collects. The collected mucus obstructs the flow of air through the affected bronchi, and eventually becomes contaminated with bacteria, causing bronchial infection.
The infection causes inflammation in the bronchi. These damages the bronchial wall even further, and so we have a vicious circle of damage leading to infection leading to further damage and infection.
The inflammation spreads to surrounding bronchi and lung tissue.
Bronchiectasis can affect a small area of one lung (focal bronchiectasis) or affect widespread and different areas of the lungs (diffuse bronchiectasis)

Question 18

bronchiectasis prevalence

Answer 18

True prevalence of bronchiectasis is unknown
Estimated to 210,000 in 2012 in the UK but could be in excess of 300,000
There is perception that the prevalence of bronchiectasis is declining with antibiotics and immunisation
The prevalence for people aged 60 and over is increasing due to improved diagnostics

Question 19

bronchiectasis causes

Answer 19

Acquired 
Lung infection (TB, pneumonia, whooping cough)
Immunodeficiency (AIDs, rheumatic disease)
Local airway obstruction (tumor/foreign body)
Environmental exposures (ammonia, drugs)

congenital
CF
primary cilary dyskinesia
>50% are idiopathic with underlying cause not identified

Question 20

bronchiectasis symptoms

Answer 20

sputum
chronic cough
shortness of breath
weight loss

Question 21

bronchiectasis diagnosis

Answer 21

High resolution computed tomography (HRCT) is the most definitive method of diagnosing bronchiectasis

Chest X-ray will detect less than 50% of cases

Sputum specimens

Lung function

Bronchoscopy

Gene mutation to exclude rare mutations of CF

Question 22

spirometry

Answer 22

A higher number of infectious exacerbations was noticed among patients with lower FEV1, FVC and FEV1%FVC. Radiology score was found significantly higher in patients with a mixed pattern compared to normal and obstructive. Moreover, patients with a mixed pattern presented a higher number of infectious exacerbations per year compared to those with normal spirometry and also a more long-standing disease compared to both normal and obstructive.

*A higher radiological score indicates more widespread bronchiectasis

Question 23

bronchiectasis management

Answer 23

Pharmacological

Mucoactive agents
Antibiotics
Corticosteroids
Bronchodilators 
Oxygen therapy

non pharm
airway clearance techniques
pulmonary rehab
surgery

Question 24

cystic fibrosis

Answer 24

CF is genetic
affects lungs and digestion
1/19 irish people carry gene (1 copy) causing CF
7/10,000 people in ireland have CF

Question 25

Causes

Answer 25

A genetic mutation causes abnormalities in the production and function of a protein called the cystic fibrosis transmembrane conductance regulator (CFTR)
CFTR acts as a chloride channel and a regulator of sodium chloride and bicarbonate transport.
The CFTR is present throughout the body hence why CF is a multi system condition affecting many organs
The lungs and the gastrointestinal tract are most commonly affected

Inactive or compromised functioning of CFTR leads to impaired chloride transport and increased sodium absorption across airway epithelial cells, with increased water absorption.

This results in dehydration of the airway surfaces, and more viscid mucus.

In turn, the cilia become impaired due to the very sticky mucus, and airway clearance is affected, resulting in a build up of secretions, which provides a favourable environment for bacterial infection.

Altered CFTR has also been associated with reduced phagocytosis of some bacteria.

The overall affect is a predisposition to chronic infection and bronchiectasis

Question 26

diagnosis

Answer 26

Genetic testing

Sweat test:

• Altered CFTR in the sweat glands restricts the amount of reabsorption of chloride in the duct, reducing salt reabsorption.
• High concentrations of electrolytes in the sweat are a hallmark of CF.
• Sweat chloride levels remain a standard method for diagnosing CF.

Question 27

CF classification

Answer 27

FEV1 % predicted

Classification
≥ 85%
Normal

70-84%
Mild

40-69%
Moderate

<40%
Severe

Question 28

CF treatment

Answer 28

pharm 
- antibiotics 
-bronchodilators 
-corticosteroids 
-mucoactive agents 
- orkambi 
- nutritional support 
non-pharm 
- airway clearance 
exercise - physio
- surgery

Question 29

interstitial lung disease

examples

Answer 29

Interstitial lung disease (ILD) is an umbrella term for a large group of disorders that cause scarring (fibrosis) of the lungs. The scarring causes stiffness in the lungs which makes it difficult to breathe.

Examples: idiopathic pulmonary fibrosis, hypersentitivity pneumonitis, asbestos, sarcoidosis

IPF, is the most common type of interstitial lung disease (65%), it is a chronic and progressive fibrosing interstitial lung disease of unknown aetiology

Question 30

idiopathic pulmonary fibrosis

Answer 30

True prevalence not known

Estimated to be 400 new cases each year with a prevalence of >1000 cases

Median survival rate 3 -5 years from diagnosis

Men > women

> 50 years

Question 31

Idiopathic pulmonary fibrosis
symptoms
diagnosis

Answer 31

symptoms - shortness of breath
non-productive cough
fine crackles on asucultation

Diagnosis - CT scanning
biopsy
pulmonary function test

Question 32

daily home spirometry for detecting progression of idiopathic pulmonary fibrosis

Answer 32

Variability inherent in the measurement of FVC, combined with the unpredictable nature of IPF disease progression, hinders the use of serial FVC measurement in the assessment of individual patients and limits its usefulness.

Question 33

idiopathic pulmonary fibrosis

management

Answer 33

pharmacological

• corticosteroids
• immunosuppressive agents
• antifribrotic therapy
• ocygen therapy

non pharm

• pulmonary rehab
• surgery
• mgmt of breathlessness