According to the American Lung Association, interstitial lung disease (ILD) describes a group of over 200 conditions that damage the lungs and make it hard to breathe. The scarring and inflammation that occur in ILD are usually irreversible. They can have lasting effects on a person’s health, quality of life, and ability to carry out their daily routine. 

In this article, we will explore interstitial lung disease, its symptoms, and strategies to best support someone diagnosed with this lung condition. Check here to see if you have free access to Trualta’s library of resources on all aspects of caregiving, including caregiving for someone who has a lung disease.

What is Interstitial Lung Disease? 

To fully understand ILD, we must first understand how the lungs work: 

• Our lungs are made up of tiny air sacs called alveoli.  
• When we breathe in oxygen, it travels down our airways to the alveoli, where it exchanges with carbon dioxide.  
• The alveoli send oxygen into the bloodstream, and it goes out to supply the body.
• Carbon dioxide is exhaled through our airways, and that is how we breathe.  

In interstitial lung disease, inflammation causes damage and scars the alveoli, which makes it difficult or even impossible for oxygen exchange to happen. This leads to difficulty getting oxygen out to our bodies and can lead to progressive breathing problems, permanent lung damage, infections, and, in extreme cases, eventual respiratory failure. 

Examples of interstitial lung diseases include: 

• Hypersensitivity pneumonitis
• Idiopathic pulmonary fibrosis
• Auto-immune-related interstitial lung diseases

Causes of Interstitial Lung Disease

There are both known and unknown causes of ILD. 

The known causes of interstitial lung disease are: 

• Environmental factors, such as inhalation of chemicals, including asbestos, tobacco, or other toxic substances can lead to lung inflammation.
• An autoimmune disease or connective tissue disease, like lupus, scleroderma, and rheumatoid arthritis, can be a segway into ILD. 
• Medications or medical treatments can damage the lungs, leading to interstitial lung disease. Examples include radiation therapy, amiodarone, or methotrexate.
• Genetics can play a role in susceptibility to interstitial lung disease. 

There are some cases of interstitial lung disease where the cause is unknown, or idiopathic. In these cases, symptoms can develop without known exposure to any of the above conditions or factors. 

Symptoms of Interstitial Lung Disease

Signs and symptoms of interstitial lung diseases can start quite mild and progress to become more severe over time. 

Interstitial lung disease symptoms include: 

• A dry cough that is persistent and does not produce mucus
• Shortness of breath that gets worse with exertion
• Chest pain 
• Fatigue
• Clubbing, or rounding of the fingertips, can happen over time with prolonged shortness of breath.

Because the initial symptoms are mild, people often mistake them for being out of shape or getting older. These symptoms continue to get worse over time with disease progression, including worsening shortness of breath that doesn’t get better with rest. 

If you notice any of these symptoms in your care recipient, follow up with their doctor to have them evaluated. 

Testing and Assessment of Interstitial Lung Disease

If your care recipient begins to show symptoms of interstitial lung disease, their doctor will likely recommend a series of tests to figure out what is going on. Here are some common assessments that can give an idea of how the lungs are functioning: 

• Pulmonary Function Tests assess overall lung function by having the patient blow into a device that measures lung capacity, breathing efficiency, and gas exchange. 
• Blood tests are used to look at different autoimmune factors and look at blood gas results to get an idea of the gas exchange happening in the lungs. 
• A bronchoscopy is when a tube is inserted down the airway of the patient to allow doctors to take pictures and get a better look at both the airway and the lungs. 
• Bronchoalveolar lavage is a procedure where a scope is inserted into the airways, and a small amount of saline is released and sucked back into the tube to get cells for testing from the lung tissue. 
• Chest X-rays allow for a clear picture to be taken of your lungs. ILD can be identified by net-like markings on the lower part of the lungs.
• A lung biopsy may be done to take a sample of the tissue and test it to confirm if your loved one has ILD or not. 

How is Interstitial Lung Disease Treated? 

Interstitial lung disease care includes addressing both the underlying cause and the symptoms to help your loved one have a better quality of life. 

Medical Treatment

• Anti-fibrotic medications can reduce scarring that leads to lung fibrosis and hardening in the lung tissue, and can slow the progression of the disease.
• Corticosteroids are often given to decrease lung inflammation and improve symptoms.
• Lung transplantation is considered in extreme cases when symptoms have progressed and lung function has become significantly impaired.

Supplemental and Lifestyle Treatment

Doctors often prescribe lifestyle adjustments or rehabilitation therapy after a diagnosis of interstitial lung disease. 

• Supplemental oxygen is recommended in some cases to deliver extra oxygen to the lungs and make sure they get enough oxygen. 
• Pulmonary rehabilitation might be recommended by a healthcare provider to encourage cardiovascular exercise to keep the heart and lungs strong. 

What is the Prognosis of Interstitial Lung Disease? 

The prognosis of interstitial lung disease depends on many factors:

• Severity and type of the condition
• Age
• Overall health of the individual 
• How quickly the condition was caught and how much it has progressed

Some types of ILD have a slow progression, while others progress quickly, leading to worse lung function, increased shortness of breath, critical care hospitalizations, and failure of the respiratory system. 

Because ILD includes different types of conditions, the type of ILD matters. If the interstitial lung disease was caused by inflammation, treating it with steroids and anti-inflammatory medications can help keep symptoms from getting worse and minimize the effects. If the condition leads to fibrotic changes causing the lungs to harden, then these individuals usually have more progressed symptoms and more shortness of breath.

Interstitial lung disease is a progressive condition that can eventually lead to other heart problems, pneumonia, pulmonary hypertension (increased pressure in the vessels supplying the lungs), and overall respiratory failure.

What to Expect When Caring for Someone with Interstitial Lung Disease

Physical Activity

Depending on the severity of the condition, someone with ILD will likely experience some shortness of breath during activity, but that doesn’t mean they shouldn’t exercise.

Here are some exercise-related tips for caring for someone with interstitial lung disease: 

• Talk to their doctor or physical therapist to get an idea of how much walking they should be doing and which strengthening exercises to do.
• Using a pulse oximeter can help determine their blood oxygen level and make sure they are safe when they exercise.
• In some cases, the care recipient’s supplemental oxygen should be increased for exercise purposes. Ask their doctors or other healthcare providers for these recommendations.

Sleep

Sleep can be a challenge for people with interstitial lung disease because of how much and how often they have a dry cough or difficulty breathing during the night.

Here are some tips to help your loved one with ILD get more restful sleep: 

• Maintain a routine sleep schedule to help minimize difficulty sleeping
• Use a wedge or pillows to elevate the head of the bed. This can help relieve shortness of breath discomfort and minimize the irritating, dry cough.
• Cough suppressants can occasionally help reduce how much and how often they cough.
• Use of an air humidifier can help moisten the air to minimize irritation of the airways, leading to dry coughs.

Daily Tasks 

Your care recipient may have trouble with daily tasks and get tired quickly. Simple things like taking a shower or cleaning the dishes may take longer because they need to take breaks and catch their breath. 

As a caregiver to someone with this condition, here are some activity-related tips to help your care recipient manage throughout their day:  

• Plan out the day, choosing to do the most difficult or tiring task first to save energy
• Place chairs around the house as resting points if the care recipient gets tired while walking from room to room.
• Consider purchasing a chair for the shower to save energy and make this task easier.
• Many tasks can be done while sitting to conserve energy, such as cleaning the dishes or folding the laundry. 

Oxygen Therapy

As mentioned above, oxygen therapy can be prescribed for people with interstitial lung disease if they need extra support getting oxygen to their lung tissue. You might need to assist with managing the tubing or the concentrator. Sometimes, their overall flow may need to be increased or decreased depending on what they are doing and how they are feeling that day.

Emotional 

There is a large emotional toll that comes with a diagnosis of a chronic illness. Because you are caring for someone with this condition, the best thing you can do is be an open, safe space for them to share their thoughts and feelings. Consider encouraging them to reach out to support groups or seek professional help if you feel it could be beneficial. 

Conclusion

A diagnosis of interstitial lung disease will result in an overall change in lifestyle, physical capabilities, and overall well-being for your care recipient. A new diagnosis like this can be overwhelming and frightening. By understanding what is happening in the body, how it can be managed, and strategies to support day-to-day tasks, you are well-equipped to care for your loved one and keep them at ease as they navigate this new health journey. 

References

1. American Lung Association. (n.d.). Interstitial lung disease (ILD). American Lung Association
2. Cleveland Clinic. (n.d.). Interstitial lung disease: Stages, symptoms & treatment.
3. Mayo Clinic. (n.d.). Interstitial lung disease.
4. Pulmonary Fibrosis Foundation. (n.d.). For the caregiver. 
5. Wijsenbeek, M., & Cottin, V. (2020). Interstitial lung disease. In StatPearls. StatPearls Publishing. NCBI Bookshelf