COPD AND ALPHA-1-ANTITRYPSIN Flashcards

Question

What is the predicted FEV1%?

Answer 1

refers to how the patients' FEV1 compares to the average FEV1 in a population of similar age, sex and body composition There are standard formulas available to determine the predicted FEV1 for men and women at different ages. This is then used alongside the patients' FEV1 to work out the predicted FEV1 %.

Answer 2

Medical research council (MRC) dyspnoea scale 1. Breathlessness on strenuous exercise. 2. Breathlessness on hurrying or slight hill. 3. Walks slower than contemporaries on ground level due to breathlessness OR have to stop to catch breath when walking at own pace. 4. Stops to catch breath after 100 metres OR a few minutes of walking 5. Breathlessness on minimal activity (dressing) or unable to leave the house due to breathlessness

Answer 3

Smoker or ex-smoker Symptoms in older adults (> 35 years old) Chronic productive cough Persistent/progressive breathlessness Night time waking with symptoms uncommon Variability uncommon (diurnal or day-to-day)

Answer 4

Hyperexpanded/inflation Hyperlucent lung fields Flattened hemidiaphragms Hypodense Saber-sheath trachea

Answer 5

Symptoms disproportionate to spirometric assessment Alternative diagnosis suspected (e.g. bronchiectasis, fibrosis) Lung cancer suspected or to investigate abnormalities on chest x-ray

Answer 6

If lung cancer is suspected Diagnostic uncertainty If COPD is very severe (<30% predicted) or rapidly declining If cor pulmonale is suspected If they’re <40 or there is FHx of alpha-1-antitrypsin deficiency If they have frequent infections Others - assessing need for oxygen therapy, long term ventilation, steroids, nebuliser therapy or lung surgery

Answer 7

People with… Peripheral oedema. Raised JVP Systolic parasternal heave. A loud pulmonary second heart sound (over the second left intercostal space). Hepatomegaly.

Answer 8

in people with early onset of symptoms, minimal smoking history or a positive family history

Answer 9

Spirometry should be carried out 15–20 minutes after the person has inhaled a short-acting bronchodilator (for example 400 micrograms salbutamol delivered via a spacer device — local protocols may vary). Airflow obstruction is defined as a post bronchodilator ratio of forced expiratory volume in 1 second (FEV1) to forced vital capacity (FVC) of less than 0.7.

Answer 10

Asthma Bronchiectasis HF Interstitial lung disease Anaemia TB CF Upper airway obstruction

Answer 11

RTI - commonly rhinovirus Smoking Environmental pollutants

Answer 12

Increased breathlessness, cough, sputum production or change in sputum colour Increased wheeze, chest tightness, URTI symptoms, reduced exercise tolerance, ankle swelling, increased fatigue, acute confusion

Answer 13

This refers to a multidisciplinary programme that aims to optimise the physical and social performance of patients suffering from long-term chronic lung conditions like COPD. It involves exercise training, health education, and breathing techniques over a series of sessions. Nutritional education and behavioural techniques are also utilised. Pulmonary rehabilitation is offered to any patient with COPD who feels disabled by their condition.

Answer 14

If they are functionally disabled by COPD i.e. MRC grade 3 or above If they have had a recent hospitalisation for an acute exacerbation

Answer 15

Oxygen is a treatment for hypoxaemia (not breathlessness). Long-term oxygen therapy (LTOT) can improve survival in people with stable COPD and chronic hypoxia. Inappropriate oxygen therapy in people with COPD may cause respiratory depression.

Answer 16

Oxygen saturations of 92% or less breathing air. Very severe (FEV1< 30% predicted) or severe (FEV1 30–49% predicted) airflow obstruction. Cyanosis. Polycythaemia. Peripheral oedema. Raised jugular venous pressure.

Answer 17

Pneumococcal vaccine Seasonal influenza vaccine

Answer 18

Explain diagnosis, risk factors etc Give patient information from the British lung foundation and NHS websites Offer support to stop smoking at every opportunity Offer pneumococcal and influenza vaccines Offer pulmonary rehabilitation if indicated Develop a personalised self-management plan Optimise treatment for comorbidities

Answer 19

Step 1 - SABA or SAMA to use as needed Step 2 - no asthmatic or steroid responsive features - combined LABA plus a LAMA. Asthmatic or steroid responsive features - LABA plus an ICS Step 3 - No asthmatic or steroid responsive features - 3 month trial of LABA, LAMA + ICS and if no improvement at 3 months then go back to LABA and LAMA Asthmatic or steroid responsive features - if person continues to have day-to-day symptoms adversely affecting quality of life or has 1 severe or 2 moderate exacerbations of COPD within a year, offer LABA plus LAMA plus ICS.

Answer 20

Increased risk of pneumonia

Answer 21

Bind to β2 adrenergic receptors on bronchial smooth muscle cells which activates the enzyme adenylyl cyclase which leads to increased cAMP production that ultimately cause relaxation of the smooth muscle. β2-agonists also stimulate the β2 receptors on immune cells like mast cells and decrease the release of inflammatory mediators like leukotrienes and prostaglandins. This, in turn, decreases the inflammation, swelling, and irritation in the respiratory tract. Ultimately, all these events help dilate the narrowed airways and improve air flow.

Answer 22

albuterol (aka salbutamol), metaproterenol, and terbutaline.

Answer 23

Arrhythmias; headache; palpitations; muscle cramp; tremor: hyperglycaemia (uncommonly). Bronchospasm paradoxical (very rare)

Answer 24

Salmeterol Formoterol Olodaterol

Answer 25

They enter the lungs and bind to M3 muscarinic receptors on the tracheal and bronchial smooth muscles. This blocks acetylcholine from binding to the receptors, leading to less smooth muscle constriction.

Answer 26

ipratropium bromide and oxitropium bromide

Answer 27

tiotropium, aclidinium, umeclidinium, and glycopyrrolate (also called glycopyrronium)

Answer 28

a dry mouth constipation a cough headaches feeling sick (nausea)

Answer 29

Beclometasone Fluticasone

Answer 30

work by reducing inflammation within the lungs. They are thought to reduce the number of exacerbations, improve the efficacy of bronchodilators and decrease dyspnoea in stable COPD.

Answer 31

Salmeterol and fluticasone I.e. a LABA and ICS combined

Answer 32

Indacaterol and glycopyrronium I..e LABA and LAMA combined

Answer 33

formoterol/glycopyrronium/beclometasone I.e. LABA, LAMA and ICS combined

Answer 34

Inhalers Spacers Nebulisers

Answer 35

Spacers are plastic devices with a mouthpiece at one end and a hole for a metered-dose inhaler (MDI) to be inserted at the other: They require less coordination than use of a MDI alone and allow carers to help people with cognitive impairment or functional problems. They slow down the particles of the drug and allow more time for evaporation of the propellant, so that more of the drug can be inhaled. They increase the proportion of the drug delivered to the airways and reduce the amount of drug deposited in the oropharynx (thereby reducing local adverse effects and systemic absorption). They are useful in people with poor inhalation technique.

Answer 36

people with distressing or disabling breathlessness despite maximal therapy using inhalers. Nebulised therapy should not be prescribed without an assessment of the person's and/ or carer's ability to use it.

Answer 37

Oral corticosteroids Oral theophylline (slow release) Oral mucolytic therapy Oral prophylactic antibiotic therapy Oral phsophodiesterase-4 inhibitors

Answer 38

relaxes the smooth muscles located in the bronchial airways and pulmonary blood vessels by phosphodiesterase 3 inhibition

Answer 39

if a person with stable COPD develops a chronic cough productive of sputum.

Answer 40

Carbocisteine and acetylcysteine

Answer 41

people with COPD who have had more than three exacerbations requiring steroid therapy and at least one exacerbation requiring hospital admission in the previous year

Answer 42

Treatment with azithromycin 500 mg three times per week, should be considered for a minimum of 6–12 months to assess evidence of efficacy in reducing exacerbations.

Answer 43

Optimise non-pharmacological and pharmacological therapies, including smoking cessation, optimised inhaler technique, optimised self-management care plan, airway clearance techniques, and attendance at pulmonary rehabilitation course. Perform ECG (electrocardiogram) to assess QTc interval. Perform baseline liver function tests. Counsel people with COPD about potential adverse effects, including gastrointestinal upset, hearing and balance disturbance, cardiac effects, and microbiological resistance. Arrange microbiological assessment of sputum before therapy, including investigation for non-tuberculous mycobacteria (NTM). Consider accurate assessment of baseline exacerbation rate and a CT scan to exclude a possible diagnosis of bronchiectasis.

Answer 44

If adverse gastrointestinal effects occur at this higher dose of azithromycin clinicians can consider a dose reduction to 250 mg three times per week. Liver function tests should be checked 1 month after starting treatment and then every 6 months. An ECG should be performed 1 month after starting treatment to check for new QTc prolongation. If present, treatment should be stopped. Subsequent follow up at 6 and 12 months should determine whether benefit is being derived from therapy by using objective measures such as the exacerbation rate, CAT score or quality of life as measured by a validated assessment tool. If there is no benefit treatment should be stopped. It is not necessary to stop prophylactic azithromycin during an acute exacerbation of COPD unless another antibiotic with potential to affect the QT interval has also been prescribed.

Answer 45

Advice and info on… COPD and its symptoms Non-pharmacological measures Importance of vaccines Apporpate use of inhaled therapies Early recognition and management of exacerbations Details of local and national organisations and online resources that can provide more information and support such as the British Lung Foundation

Answer 46

Involves asking the person to walk for a standardised length of time (usually 6 or 12 minutes). Failure to complete the test and/or moderate to severe respiratory distress measured on a visual analogue scale indicates a possible need for in-flight oxygen.

Answer 47

Is performed in a specialist lung function unit and is used to assess whether a person needs in-flight oxygen. Measures the person's response to a simulated aircraft cabin environment. In this investigation, 15% oxygen is administered and the person is monitored continuously by pulse oximetry. A partial pressure of arterial oxygen (PaO2) of 6.6 kPa or an oxygen saturation of 85% is used as the cut-off value below which supplemental oxygen is recommended for air travel.

Answer 48

Ensure they have an advanced care plan and discuss end-of-life issues including advanced decisions Coordinate care with a respiratory nurse specialist, district nurse, palliative care team, and social services as appropriate. Optimise treatment Discuss hospice admission Consider what practical and emotional support can be provided for family and carers

Answer 49

Advise the pt to increase doses or frequency of short acting bronchodilators (not exceeding maximum dose) Consider oral corticosteroids for those with significant increases in breathlessness that interfered with daily activities - 30mg oral prednisolone Consider need for Antibiotics - amoxicillin, doxycycline or clarithromycin are fist line (if not improvement after 3 days then send a sputum sample for culture and suspectibility testing)

Answer 50

When pt breathe supplementary oxygen for at least 15 hours a day

Answer 51

Measure arterial blood gases on 2 occasions at least 3 weeks apart in pt with stable COPD on optimal management If pO2 is <7.3kPa or 7.308 with secondary polycythemia/peripheral oedema/pulmonary hypertension, then offer LTOT

Answer 52

NICE advise the following: do not offer LTOT to people who continue to smoke despite being offered smoking cessation advice and treatment, and referral to specialist stop smoking services.

Answer 53

NICE suggest that a structured risk assessment is carried out before offering LTOT, including: the risks of falls from tripping over the equipment the risks of burns and fires, and the increased risk of these for people who live in homes where someone smokes (including e‑cigarettes)

Answer 54

any previous, secure diagnosis of asthma or of atopy a higher blood eosinophil count - note that NICE recommend a full blood count for all patients as part of the work-up substantial variation in FEV1 over time (at least 400 ml) substantial diurnal variation in peak expiratory flow (at least 20%)

Answer 55

After trials of short and long-acting bronchodilators or to people who cannot used inhaled therapy

Answer 56

Reduce the dose As these antibiotics can inhibit it the breakdown of theophylline in the liver leading to an accumulation of the drug in the body. If taking both then monitor theophylline levels and reduce dose accordingly to prevent toxicity. This is particularly important if pt have pre-existing conditions that increase the risk of theophylline toxicity e.g. liver disease, kidney disease, COPD, congestive HF

Answer 57

Loop diuretic and consider long term oxygen therapy

Answer 58

Smoking cessation Long term oxygen therapy in pt who fit the criteria Long volume reduction surgery in selected patients

Answer 59

bacteria 70% Haemophilus influenzae (most common cause) Streptococcus pneumoniae (most common cause of pneumonia) Moraxella respiratory viruses 30% human rhinovirus is the most important pathogen

Answer 60

Oxygen therapy Nebulised bronchodilator (IV theophylline if not responsive) Steroid therapy Non invasive ventilation may be used

Answer 61

a method of delivering mechanical ventilation to patients with respiratory failure without the need for invasive procedures such as tracheostomy or endotracheal intubation. NIV is often used as a treatment option for patients who experience acute exacerbations or chronic respiratory failure. NIV involves the use of a machine that delivers a mixture of air and oxygen through a mask that covers the nose or mouth (or both) of the patient. The machine can provide different levels of positive airway pressure to support the patient's breathing and reduce the work of breathing. This helps to improve gas exchange, increase oxygen levels in the blood, and reduce carbon dioxide levels.

Answer 62

avoiding the need for intubation reducing the risk of ventilator-associated pneumonia improving patient comfort and quality of life It can reduce the length of hospital stay and the need for mechanical ventilation It may reduce mortality in some cases.

Answer 63

when COPD is with a respiratory acidosis pH 7.25-7.35 (can be used for those who are more acidosis but a greater degree of monitoring is required)

Answer 64

Continuous Positive Airway Pressure: CPAP provides a constant level of positive pressure throughout the respiratory cycle to keep the airways open and improve oxygenation. It is commonly used to treat obstructive sleep apnea and respiratory distress syndrome in premature infants. CPAP is not typically used to treat COPD, but it may be used in some cases to support breathing during acute exacerbations. Bilevel Positive Airway Pressure (BiPAP): BiPAP provides two levels of positive pressure, one during inhalation and a lower level during exhalation, to support breathing and improve oxygenation. BiPAP is commonly used to treat acute exacerbations of COPD, chronic respiratory failure, and other respiratory conditions. BiPAP can be adjusted to provide different levels of pressure support, depending on the patient's needs.

Answer 65

Lung reduction surgery Bullectomy Lung transplantation

Answer 66

a surgical procedure in which a bullectomy is a surgical procedure in which a bulla, which is an abnormally large air-filled space in the lung, is removed.

Answer 67

Hypercapnic failure Lungs are unable to adequately remove CO2 from the blood leading to hypercapnia Those with COPD are at risk of it PaO2 <8kPa and raised PaCO2

Answer 68

oxygen therapy should be trialed using a venturi mask for 1 hour with repeat ABG. If there is persistent evidence of T2RF, or they decline during the trial period, then non-invasive ventilation should be considered

Answer 69

IPAP: inspiratory positive airway pressure (usually 10-15 cmH20) EPAP: expiratory positive airway pressure (usually 4-5 cmH20)

Answer 70

Cigarette smoke Inhales irritants - smog, industrial pollutants, toxic chemicals, dust Repeated exposure to infections - influenza, staphylococcus, streptococcus, mycoplasma pneumonia Those with other respirairt disease have a higher predisposition Chronic GERD

Answer 71

Smoke leads to chronic irritation Its toxicity also destroys the ciliates epithelium and replaces it with squamous cells which ultimately reduces mucus clearance

Answer 72

The loss of CFTR function leads to increased viscosity of mucus, increased mucus production and decreased mucus clearance. The recurrent respiratory infections and structural changes in the airways due to chronic inflammation can also lead to chronic bronchitis.

Answer 73

Chronic bronchitis typically presents with a productive cough Emphysema almost exclusively presents with SOB

Answer 74

Prednisolone and an antibiotic - so they can start these meds whilst contacting the GP (if by the time they have seen GP e.g. 24 hours later and still bad they should probably be sent into hopsital as often deteriorate very fast)

Answer 75

A protease inhibitor normally produced by the liver It’s role is to protect cells from enzymes such as neutrophil elastase. This is important as left unchecked neutrophil elastase leads to lung damage

Answer 76

Autosomal recessive disorder with co-dominant expression (both alleles are expressed but the clinical phenotype is only seen with inheritance of 2 abnormal genes)

Answer 77

Caused by a mutation in the SERPINA1 gene on chromosome 14 There are >100 genetic mutations that can lead to reduces or absent AAT. The major 3 types are the following: M allele - normal S allele - in 2-3% population Z allele - most severe and found in 1% population

Answer 78

PiMM - homozygous for normal alleles PiZZ - homozygous for abnormal Z allele - 80-100% chance of developing A1AD PiSZ - compound homozygous - variable phenotype - 20-50% chance of A1AD PiMZ - heterozygous - 1 abnormal gene so unlikely to have the clinical phenotype (Pi - Protease Inhibitor)

Answer 79

Z allele - Abnormal AAT protein polymerises and accumulates within hepatocytes leading to cellular destruction, chronic scarring and fibrosis Mutations that lead to a complete deficiency of AAT will not cause liver disease as no protein is created If AAT is absent or significantly reduces, elastase isnt broken down so it breaks own elastin which is important for the integrity of alveoli = emphysema = oxygen transfer is reduced = chronic hypoxia and breathlessness

Answer 80

COPD features - Dyspnoea, wheeze, cough, ankle swelling (from cor pulmonale) CLD features - jaundice, bruising, spider navel, palmar erythema,a hepatomegaly, ascites, leuconychia, confusion, asterixis

Answer 81

Measure serum alpha-1-antitrypsin levels Genotyping to look for the most common mutations Others may include - Pulmonary function tests, pulmonary imaging, liver imaging

Answer 82

Stop smoking Pulmonary rehabilitation Prompt treatment of respiratory infections Manage according to COPD guidelines e.g. inhaled therapy, antibiotics and short courses of corticosteroids for infections If established cirrhosis, 6 monthly liver USS and AFP monitoring Consideration for a lung or liver transplant

Answer 83

Emphysema is most prominent in the lower lobes in A1AT deficiency and the upper lobes in COPD

Answer 84

increase the frequency of bronchodilator use and consider giving via a nebuliser give prednisolone 30 mg daily for 5 days Consider antibiotics

Answer 85

Purulent sputum Clinical signs of pneumonia

Answer 86

amoxicillin or clarithromycin or doxycycline.