Cardio case Flashcards
Cystic fibrosis pathophysiology
-Cystic fibrosis (CF) is an autosomal, recessive disease which can be inherited if both parents contain the defective CFTR protein, leading to a 25% chance of inheritance for their offspring.
-This Abnormal CFTR protein affects the number and/or function of the ion channels that move sodium and chlorine in and out of the cell.
- This leads to excess sodium being stuck inside the cell.
-Excess sodium inside the cell initiates osmotic processes in which water is drawn into the cell in an attempt to correct the imbalance.
-The dysfunction of the ion channels further causes a loss of chloride removal, preventing the correction of low surface water volume
-This combination of excess sodium and water in the cell and loss of chloride removal dehydrates and decreases the liquid layer that lubricates the airways and cilia.
-A dehydrated and decreased liquid layer leads to dysfunction and destruction of the cilia.
-With this cilial collapse, the mucociliary transport system cannot clear dry secretions through cilial beating.
-A resultant of this cilial dysfunction, causes excess, dry secretions to linger in the airways.
-Bacteria then collects and propagates in the lungs causing persistent bacterial infections producing a cyclic effect. Often this leads to CF bronchiectasis
Respiratory signs and symptoms of CF
Signs:
-Thick sputum production
-Haemoptysis
-Hypoxaemia on ABG’s
-Shows an obstructive spirometry pattern
-Cx shows; thickening of bronchial walls, irregular shaped bronchioles, mucous plugging, patchy opacities/mucoid impaction
-Auscultation: wheezing
-Cyanosis
-Nutritional deficits
-Recurrent chest infection
-Respiratory muscle deconditioning
Symptoms:
-Cough
-Dyspnoea/SOB
-Fatigue
-Fever
-Failure to thrive
-Malaise
-Sinus pain
-Chest pain
Signs and symptoms of other systems/organs with CF
Digestive:
Signs: malnutrition, meconium ileus shown on Xray, abdominal distension
Symptoms: smelly stools, incontinence, vomiting, reduced frequency of bowel movements
Endocrine:
Signs: low insulin levels (can lead to diabetes mellitus), fibrotic tissue on pancreas on US, short stature, decreased testosterone
Symptoms: abdominal pain, bloating, nausea
Exocrine:
Signs: sticky saliva and more viscous and sweaty secretions from mucous glands
Musculoskeletal:
Signs: thoracic kyphosis, digital clubbing, low bone mineral density
Symptoms: joint pain, weight-loss, muscle atrophy
Reproductive:
Signs: irregular or absent menstruation, absent sperm canal seen through US, delayed puberty, thick cervical mucous
Hepatobiliary:
Signs: signs of liver disease such as fibrotic tissue on US or CT and abnormal blood results, enlarged liver and spleen, malnutrition
Symptoms: jaundice, bleeding (nose bleeds, bruising, visible veins)
Urinary:
Signs: incontinence, weak pelvic floor
Cardiac:
Signs: high HR, BP, MAP, increased levels of angiotensin II and aldosterone, irregular ECG,
Symptoms: chest pain, fatigue
Angela (CF patient) ICF
Activity restrictions:
-May not be able to meet physical activity guidelines due to symptoms such as pain, fatigue and SOB.
-ADL’s such as walking up stairs, getting ready for school may be affected due to symptoms such as fatigue and breathlessness.
-Sleep may be impaired due to chronic coughing, pain, night feeding or medications needed to be taken throughout the night.
Participation restrictions:
-Sporting teams or classes at school due to severity of symptoms or recent exacerbations.
-May not be able to participate in activities such as swimming or sport classes due to incontinence and lack of access.
-May not be able to attend social gatherings around meal times because of medications.
-May be excluded or stigmatised because patient’s present with respiratory/COVID symptoms such as coughing - social isolation.
-May not want to participate in physical or social activities out of fear of embarrassment (sweating and incontinence)
Environmental factors:
-Have to avoid other CF patients - avoid risk of infection.
-Air pollution and allergens may cause further inflammation of the airways, exacerbating patients secretion movement impairment.
-Exposure to tobacco smoke increases airway inflammation and further damages the cilia.
Personal factors:
-CF patients have higher incidence rates of depression
-Low BMI - can affect her self esteem and energy levels.
-decreased puberty development - can lead to feelings of embarrassment or self-consciousness.
Order of treatment for CF patient
- Mobilise secretions
- Clear secretions
- Improve ventilation and gas exchange
- Mobilise patient to improve exercise tolerance
- Exercise and diet to address malnutrition
- Manage her stress urinary incontinence
- Postural management
Gravity assisted drainage (GAD)
-Allows sputum to be drained from peripheral to more central airways. -This technique utilises gravity, allowing thick secretions to ‘unstick’ from the chest wall and peripheral airways. By putting the patient into a certain position, secretions from a specific lung segment can drain into more central airways.
-Secretions in central airways can be more easily cleared using techniques such as FET.
-MGAD - doesn’t include HDT = more safe and tolerated.
Autogenic drainage (AD)
-Autogenic drainage is a self assisted breathing and airway clearance technique
-Follows a three stage process, ‘unstick,’ ‘collect,’ and ‘evacuate.’
-Unstick: slow breaths through their nose at a normal depth with a 3 second breath hold to encourage humidification and the minimisation of airflow turbulence. Slow exhalation to remove as much air as possible from lungs
-Collect: repeating same process but inhaling 2x deeper than a normal breath. Allowing secretions to be caught in central airways
-evacuate: patient inhaling at a higher lung volume to perform high-to-low volume huffs/controlled coughs to remove the secretions.
ACBT
-Breathing control: normal breathing pattern encouraging lower chest breathing
-Thoracic expansion exercises: deeper breaths with a 3 second breath hold allowing use of collateral channels (3-4 reps)
-FET (huffing followed by coughing): low to high volume huffs to move secretions from peripheral airways and high to low volume huffs to move secretions from central airways
Manual techniques
Percussion
-Rhythmically taps patients chest to looses secretions on chest wall by changing the rheology of the sputum and enhances the frequency of the impaired ciliary beat.
-Allows sputum to be drained into central airways.
Vibrations:
-Often used after percussion to facilitate secretion clearance. application of fine oscillatory movements to a particular lobe of the lungs.
-Vibrations utilises/increases the physiological mechanisms of annular flow, allowing the sputum to move towards the larger airways. -The application of compression and oscillation during vibrations also optimises the expiratory flow rate.
Shakes:
-Similar to vibrations (oscillatory movement) but with larger amplitudes and slower frequencies to enhance expiratory flow.
-Typically used in patients who need a gentler approach
Suctioning
-Process in which a small tube is inserted through an artificial airway in the nasopharyngeal, oropharyngeal, or endotracheal orifices to vacuum the sputum out of the central airways.
-Suctioning typically is used in very weak, unconscious or mechanically ventilated patients
-Can be used in conjunction with ACBT
PEP and OPEP
Positive expiratory pressure:
-Patient breathes into a mouthpiece that has a one-way valve providing resistance.
-This resistance creates positive airway pressure within the lungs. The positive airway pressure opens the smaller, blocked or collapsed airways through collateral channel ventilation.
-This improves ventilation distribution as well as allows air to get behind secretions. Positive pressure further encourages airway splinting which stabilises the airways, preventing collapse.
-A pressure gradient builds that forces secretions to move centrally into larger airways so that expectoration can occur
Oscillatory positive expiratory pressure
-Works same way as PEP but has oscillations.
-Oscillations produce vibrations within the airways, dislodging secretions and making them less viscous
HFCC Vest
-Is a mechanically controlled vest that provides chest oscillations via a pulse generating compression.
-The intermittent inflations of the jacket create chest wall vibrations, helps facilitate airway clearance mechanisms by decreasing mucous viscosity.
Cough assist device
-Imitates a cough
-Uses positive airway pressure to increase the amount of air that is in the lungs, imitating a deep breath that is taken prior to a cough.
-Device then applies a rapid decline in pressure within the lungs to represent the fast expulsion of air from the lungs, shearing secretions to be expectorated.
Specific/general positioning
-Specific and general positioning can be used to promote oxygen movement due to the use of gravity
-Specific positions can increase lung compliance which in turn increases FRC, TV and decreases CC (sitting upright or lying prone).
-Side-lying opens up the alveoli on the topmost lungs increasing ventilation in that lung segment
NIV (non-invasive ventilation)
-Positive pressure is applied through a breathing mask to provide ventilatory assistance.
-Positive pressure creates a pressure gradient, splinting open the airways allowing more air to enter the lungs and to participate in gas exchanged in the now increased # of alveoli.
-Decreases WOB, dyspnoea, fatigue, SOB, hypoxaemia and increases breath size
CPAP: continuous single level of positive pressure
BiPAP: two levels of pressures (IPAP and EPAP). IPAP supports inspiration by increasing TV, decreasing load on respiratory muscles. EPAP is able to stabilise and hold open airway at the end of expiration. This increasing alveoli recruitment. Positive pressure is left in the lung to re-expand collapsed alveoli through collateral channel ventilation. Increases FRC and oxygenation.
