CVR scenario 2 (CF) Flashcards
What is the main aim of physio for those with CF?
To prevent secondary complications and improve QoL by removing excess mucus secretions and to maintain or improve lung function
Clinically reason treatment options for the CF pt
Positive expiratory pressure (PEP)
- an airway clearance technique
- by breathing out with a moderate force through a resistance, a positive pressure is generated in the airways, helping to keep them open. This positive pressure allows airflow to get beneath the areas of mucus obstruction and move the mucus toward the larger airways, where it can be coughed out.
Postural drainage
- study in 2006 showed that head down positions do not aid further than a normal supine position. It is even linked with gastro-oesophageal reflux
- gravity assists with draining secretions from peripheral to central airways where they can be expectorated. Also helps with increasing air movement to different parts of the lungs
- upper lobes = supine
- middle lobe = quarter turn
- lower lobe = side-lying
Percussion
- used to help loosen secretions from the chest wall by producing an energy wave. Use a towel as pt complains of pain on the left side
- use whilst having pt in postural drainage
Rectangular breathing
- would be beneficial to pt when they become breathless
- it allows more time for the air to leave your lungs as you breathe out therefore creating more room for the next breath in. The aim of recovery breathing is to calm
your breathing and maximise inspiration.
Explain the AD procedure for treatment
Clear your nose and throat by blowing your nose and huffing.
Firstly take a normal “assessment” breath
First, take a large breath in, hold it for a moment. Breathe all the way out for as long as you can. Now you are at low lung volume.
Take a small to normal breath in, and pause. Hold your breath for about 3 seconds. All the upper airways should be kept open. This improves the even filling of all lung parts. The pause allows time for the air to get behind the mucus.
Breathe out through the mouth in a sighing manner.
You hear the mucus rattling in the airways when breathing the right way. Put a hand on your upper chest, and feel the mucus vibrating. High frequencies mean that the mucus is in the small airways. Low frequencies mean that the mucus is in the large airways. Using this feedback lets you easily adjust the technique.
Repeat the cycle. Inhale slowly to avoid sending the mucus back down. Keep breathing at a low level until the mucus collects and moves upward. Signs of this are:
The crackling of the mucus can be heard as you exhale.
You feel the mucus moving up.
You feel a strong urge to cough.
The level of breathing is raised when any of the above occurs. Moving the breathing from lower to higher lung area takes the mucus with it.
Finally, the collected mucus reaches the large airways where it can be cleared by a high lung volume huff. Cough only if a huff did not move the mucus to the mouth.
You have now finished one cycle. Take a break of one to two minutes. Relax and perform breathing control before you start on the next cycle. The cycles are repeated during the session. A session lasts between twenty to forty-five minutes or until you feel all the mucus has been cleared.
Clinically reason any exercise prescription for this CF pt.
Benefits of exercise include:
- delayed onset of dyspnoea
- delay the decline in pulmonary function
- prevent the decrease in bone density
- improve QoL
Adults with CF should aim to perform exercise activities for 15-30 minutes daily. Examples could include practicing football.
Recent literature suggests that exercise produces similar benefits as airway clearance techniques.
Incorporate positioning for dyspnoea.
What questions would you ask for the subjective?
- 5 cardinal signs of respiratory disease:
- cough
- dyspnoea
- wheeze
- chest pain
- sputum
- previous treatments/therapies
- does he get help to manage his CF?
- THREADS
- Red flags
- palpable neck lumps - enlarged lymph nodes, cancer
- haemoptysis
- enlarged tonsils - cause by viruses or bacteria
- how long has he had the chest pain - aggs and eases
- physio expectations and goals
Explain an A-F assessment of the pt
A = patent, 4lO2 via nasal specs
B = observation - posture, accessory muscels, buket and pump handle, hypoxic (blue fingers). Cough assessment, sputum colour, volume, viscosity, ease of expectoration.
Thoracic expansion - 3-5 cm displacement of spinous process.
Percussion note - dull = absence of air (consolidation)
resonant = normal, presence of air
hyper-resonant = more air, emphysema, asthma
C = temperature (35.7-38degrees), fluid balance, UO, dehydration can cause viscous secretions
D = AVPU - alert
E = any MSK problems, BMI, peripheral oedema, finger clubbing ( often it is related to an inadequate oxygen-rich blood supply. However, it can be hereditary and completely normal), tremors, attatchments
F = 6/12 minute walk, stairs, muscle strength
Clinically reason outcome measures for this pt
FEV1:FVC
FVC = forced vital capacity - the amount of air a pt can quickly exhale after a deep breath
FEV1 = forced expiratory volume - vol of air pt can force from lungs in 1 second.
Both measured using spirometry.
FEV1:FVC calculates the proportion of the maximum inhaled air that can be exhaled in one second.
The higher the % of FEV1:FVC ratio the healthier your lungs. A low ratio suggests something is blocking your airways (secretions). Low ratio in adults is less than 70%.
CF pts will always have a lower ratio due to the thick secretions. However, it can be used to assess if airway clearance techniques are useful.
Accuracy and sensitivity of spirometry depends on many factors that may be difficult to control, including pts mood and motivation and relationship between pt and the technician.
Sputum weight and ease of expectoration.
While sputum volume has shown to be difficult to determine with precision, sputum weight has shown to be more accurate. It is very common to expectorate after respiratory physiotherapy sessions or to swallow secretions even during the session, which means that sputum weight expectorated during a session may underestimate the effect of airway clearance techniques. On the other hand, expectoration may be contaminated with saliva so sputum weight can, therefore, be both over- and under-estimated
CF Questionnaire
- measures impact on overall health, daily life, perceived well-being and symptoms
- 9 QoL domains including physical functioning, emotional state, body image, social limitations
- 3 symptom scales: respiratory, digestive and weight
- 1 health perception scale : health status
- each is scored out of 4
- higher scores indicating better health
- patient reported outcome measure
Describe what CF is
CF is an autosomal recessive disorder (child inherits a defective version of the gene from both parents)
Caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that results in sticky mucus building upon the lungs and digestive system. The CFTR protein made by this gene regulates the movement of chloride and sodium ions across the epithelial cell membranes. When mutations occur, ion transport becomes defective, resulting in a build-up of thick mucus throughout the body.
In healthy lungs, a fluid layer is maintained through a balance of chloride secretion and sodium absorption to cover the surface of airway epithelial cells. In CF, defective chloride secretion and sodium hyperabsorption lead to the depletion of the layer of airway surface fluid.
What are some additional issues linked with CF?
- pancreas dysfunction
abnormality in CFTR gene leads to mucus build up in pancreas ducts, clogging them, causing a drop in the amount of digestve enzymes the pancreas puts out so pt has trouble absorbing fats, some proteins and vitamins A,D,E and K. eventually pancreas is damaged as backed up digestive enzymes degrade in the cells causes local inflammation, cysts (fluid-filled sacs) and fibrosis (excess deposition of connective tissue) - posture and reduced bone density
osteopenia is a common bone disease in which bones have fewer minerals and are weak; it often leads to osteoporosis, which describes a condition where the bones are less thick or dense and are weaker, leading to a higher risk of fractures. As people with CF have difficulties in absorbing vitamin D, they have a higher risk of developing osteoporosis. Vitamin D helps the body absorb calcium, which is an essential mineral for bone density. - CF related diabetes
people who have Cystic Fibrosis develop excessive mucus, which in turn can scar the pancreas. If scarring occurs, the pancreas stops producing normal amounts of insulin, causing the person to become “insulin-deficient” like someone with Type 1 diabetes. around 40-50% of adults with cystic fibrosis develop diabetes. - transplant and end of life issues
What mucolytic agents (medicines taken orally that may loosen sputum) would you recommend to this pt and when would they be taken?
Hypertonic saline A sterile saline solution that increases the amount of sodium (salt) in your airways. This attracts water into the airways, which thins mucucs, making it easier to cough out. Inhaled through a nebulizer twice a day. May cause bronchoconstriction. Side effects include: Increased cough Sore throat Chest tightness
Should be used after his salbutamol nebuliser, which is a bronchodilator
What does Creon do for CF?
a prescription medicine used to treat people who cannot digest food normally because their pancreas does not make enough enzymes due to cystic fibrosis. Creon is a mixture of amylase, lipase, and protease. It is a form of pancreatic enzyme replacement therapy (PERT) and helps ensure that nutrients are effectively absorbed from food.
Just as the lungs produce thick, sticky mucus, the pancreas also makes thick mucus that blocks the release of enzymes needed for digestion. Taken by mouth, the enzymes go to work in the intestines so you can digest food and absorb the nutrients to keep your body healthy.
Which members of the MDT would be beneficial for the CF pt and why?
- psychologist: those with CF tend to face more difficulties in everyday life, including in maintaining jobs and relationships
- OT: any home adaptations that can make life easier?
- Dietitian: education to help with reducing risk of diabetes
What are the nebulised salbutamol and colomycin used for?
And what are the benefits of nebulised therapy?
Nebulised salbutamol:
a bronchodilator used to relax around your airways to ease chest tightness. should be taken prior to chest physiotherapy
Nebulised colomycin:
antibiotic used for the treatment of chest infections such as pseudomonas. should be taken after bronchodilator and chest physiotherapy.
Nebuliser therapy:
- the medication goes straight to where it is needed (in the lung), so reduces side effects
- compared to some inhalers, larger doses of medication can be given
What may be some barriers that you need to overcome with a CF patient?
- overprotective/ involved family
- lack of support to manage the condition
- emotional wellbeing - anxiety is one of the most common emotional issues faced by these patients. This is related to daily treatments, remembering to take medications, missing out on things they want to do, and being hospitalized for periods of time. In addition, it is known that CF patients and caregivers are more likely to suffer depression. Among the 204 respondents studied, the median number of therapies taken per day was 7, and the median number of minutes needed to complete therapy per day was 108 minutes.
What other investigations would you want for this pt?
- ABGs
- low pH = because of infection
- low pO2 = becuase of retained secretions
- high pCO2 = because of increased WoB - CXR
- can request a sinus x-ray as 90% of people with CF show signs of chronic sinusitis. in healthy sinuses they are filled with air so appear black, but sinusitis appears white as it shows a build-up of fluid
- hyperinflation due to limited expiratory airflow. shown by flattened diaphragms and dark lung fields
- possible atelectasis, shown by a whiteout of the lung due to a reduced volume
