A condition, either intrinsic or extrinsic, causes the lungs to lose their ability to expand and contract. If the lungs can’t expand, they cannot bring in enough air for adequate oxygenation and ventilation (gas exchange). Patients will also struggle to cough because they are unable to inflate or forcefully deflate (cough) their lungs – therefore secretions may build up.
Intrinsic – this means the problem came from within the lungs themselves. There has been some sort of inflammation and scarring of the lung parenchyma (tissue) and therefore it has lost its elasticity. Oftentimes this will also cause damage to the alveoli themselves. The most common example is Pulmonary Fibrosis. This is a chronic, terminal disease of the lungs with no cure – we can only support the patient’s symptoms.
Extrinsic – this means the problem is coming from outside the lungs. Something is restricting the lungs from physically expanding like they need to. Most commonly this is caused by some sort of neuromuscular disorder like Amyotrophic Lateral Sclerosis (ALS), Muscular Dystrophy, or Quadriplegia.
Optimize oxygenation and ventilation, prevent pulmonary infections (common cause of death), and provide supportive care for patient and family.
Maintain patent airway
Some patients with neuromuscular disorders may require frequent suctioning and/or a tracheostomy to ensure adequate oxygen delivery
Obtain and evaluate labs (ABG)
This will reveal whether interventions are effective or if there is any decompensation
Complete a full respiratory assessment to detect changes or further decompensation
It’s important to know your patient’s baseline lung sounds. These diseases are progressive and it may be difficulty to recognize when something has changed. A change in lung sounds may indicate a pulmonary infection or that the patient simply requires suctioning..
Provide supplemental oxygen as appropriate
Increasing the concentration of oxygen they are taking in (FiO2) can improve their overall oxygenation capabilities.
Decreases oxygen demands if patient’s rest can be maximized
Ensure patient is in optimal position to decrease work of breathing
Sitting up in bed to enable appropriate lung expansion allows for adequate inspiration and expiration, which facilitates better gas exchange (if clinically appropriate to be sitting up)
Educate patient and family to find activities the patient enjoys and can tolerate. This is a method for distraction which can be a good coping mechanism
Restrictive lung diseases are often progressive and terminal without a cure. In later stages, this can become very isolating as patients are confined to bed or on a ventilator. Encourage positive coping strategies and activities to improve the patient’s state of mind and quality of life.
The most common cause of death with patients with restrictive lung diseases is pneumonia or lung infections. This is because they have difficulty coughing to expel any built up secretions. They are also at risk for aspiration. We need to work to prevent any buildup of bacteria and to help remove secretions and prevent aspiration.
Provide oral care
If a patient is intubated or receiving oxygen via nasal cannula/face mask or tent, or other method of delivery, oral care is essential to protect mucous membrane and prevent infection
Promote appropriate nutrition
Malnourishment is common with chronic lung disease, and appropriate nutrition provides the patient support for healing. If unable to take food orally, this may be done via NG tube short term, but long term patients will require a gastrostomy tube.
Tough to allow appropriate gas exchange in a patient if they cannot handle their own secretions and are using effort to cough/clear their airway, or if it is getting down into their trachea.
Patients with neuromuscular disorders need physical assistance coughing. This can be done manually by pushing on their chest or with a cough-assist machine that delivers positive pressure, then negative pressure to suck out secretions.
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