Understanding Interstitial Lung Diseases (ILDs)
What is an ILD?
ILD is short for Interstitial Lung Disease. This group of rare diseases makes it difficult to breathe because of damage to the tissue that surrounds the lung’s air sacks, blood vessels and airways. Inflammation or scarring of this tissue leads to stiffness which affects the function of the lung.
Patients with ILD experience shortness of breath, a dry cough, and other connected symptoms. ILD is a complex disease and therefore difficult to diagnose. There are more than 200 causes for this disease with different treatments and outlooks.
While it affects more men than women, smokers are at a higher risk of developing an ILD.
What is Pulmonary Fibrosis?
Pulmonary fibrosis is a disease that causes the walls of the tiny air sacks in the lung to become thick, stiff, and permanently scarred. As a result, less oxygen can pass from the lung into the bloodstream to be distributed throughout the body. Pulmonary fibrosis is often caused by Interstitial Lung Disease (ILD). Patients suffer of a dry cough, difficulties to take deep breaths, feeling dizzy and tired.
They sometimes also get out of breath while doing simple tasks like climbing stairs. If this condition gets worse over time, this could be a sign that the lung function is decreasing and the scar tissue is growing. Specialists call this a Progressive Fibrosing ILD. PF can be of known cause or of unknown origin, like Idiopathic Pulmonary Fibrosis (IPF). PF typically occurs in people who are more than 45 years of age and the average patient age is 65 years old.
Common types of Pulmonary Fibrosis
• Idiopathic Pulmonary Fibrosis
• Non-Specific Interstitial Pneumonia
• Chronic Hypersensitivity Pneumonitis
• Rheumatoid Arthritis – ILD
• Scleroderma – ILD
• Fibrotic sarcoidosis
• Unclassifiable ILD
What are the symptoms?
The symptoms of PF develop over time and can vary from person to person. At diagnosis, the most common symptoms of PF are shortness of breath, persistent dry cough and fatigue. Over time, the cough often becomes productive, and in the later stages of the disease, other factors such as loss of appetite, aching joints and muscles and gradual, unexplained weight loss develop.
About half of people with PF may also experience finger clubbing (widening and rounding of the tips of the fingers or toes). When someone uses a stethoscope to listen to the lungs of a person with PF, they may hear ‘velcro-like’ crackles in your lungs. These are ‘opening’ sounds which are made by the small airways when inhaling and can be heard in both lungs.
Where do patients with ILD get help?
How is ILD diagnosed?
A physical exam:
This is usually the first step to find a diagnosis. A doctor asks if there are problems or disorders that affect the whole body. He will also ask if any exposure to harmful substances has occurred.
A lung function test:
Patients breathe into a tube to check the strength of their lungs. A spirometry measures the amount of air the lung can hold and how forcefully one can empty air from the lung.
An imaging test:
All patients with the suspicion of an ILD undergo X-ray or computed tomography scans of their chest. The high resolution images can show even gentle changes in the tissue of the lung.
Signs of pulmonary fibrosis and it’s progressions are also detectable with imaging tests.
A bronchoscopy:
This procedure lets doctors take a close look at a patient’s lung and air passages. During a bronchoscopy, a thin tube is passed through the nose or mouth, down the throat and into the lung.
A lung biopsy:
To further identify the type of ILD, doctors can chose to perform a lung biopsy. This is a procedure in which small samples of lung tissue are removed using a special biopsy needle.
How is ILD treated?
How is ILD treated?
Lung transplant:
Patients with a severe condition of ILD might have to undergo surgery to get a lung transplant. This measure is often taken as a last resort and meant to prolong the life of a patient if all other treatment is failing. Not every patient is eligible for this option. There is also a risk that a lung transplant might not necessarily cure the condition.
These consider a range of factors such as a patient’s age, general health, severity and progression of PF. You can find them here
Medications:
Several drugs are able to reduce inflammation including steroids and other rheumatologic drugs. This can help to improve the function of the lung. There are also medications to prevent further stiffening of the lung tissue.
Oxygen therapy:
Since patients with ILD do not get enough oxygen from their lungs into their bloodstream, extra delivery of oxygen through a tube in their nose helps them to get through life more easily.
Immunosuppressants:
In case of an Autoimmune ILD a possible treatment can be medications that stop the immune system from attacking the lung and other organs. This can reduce or stop additional damage to the lung.
Pulmonary and exercise therapy:
Lung fitness can be improved by regular breathing exercises and increased physical activity.
NON-PHARMACOLOGICAL TREATMENTS
While a lung transplant is the only effective cure for someone with fILD, there are several non-pharmacological treatments which can help improve patients‘ quality of life:
Pulmonary rehabilitation
Pulmonary rehabilitation improves functional exercise capacity, dyspnoea and quality of life in the short term, with benefits also probable in fILD. Although short-term benefits of pulmonary rehabilitation are well documented, the longevity of those benefits remains unclear. The challenge of sustaining long-term behaviour change for exercise is likely a contributor. Patients with less severe disease (e.g., higher FVC) are more likely to maintain benefits at 6 months after program completion, reinforcing
the need for early referral of people with fibrotic ILD.
Oxygen therapy
Patients may experience hypoxemia while resting, sleeping or exercising. To deal with the effects of hypoxemia, they may be given extra oxygen through a concentrator (home-based or mobile), oxygen cylinders (portable or on wheels) or liquid oxygen.
Lung transplantation
Lung transplantation is the only surgical intervention procedure which can reverse the progression of fILD, improve quality of life and life expectancy. Some 30% of lung transplants worldwide are performed on patients with fILD. Unfortunately, only 5% of all patients with fILD qualify for a lung transplant, since the criteria for selection as a viable transplant candidate are quite strict. These consider a range of factors such as a patient’s age, general health, comorbidities, severity and progression of fILD. In case of lung transplantation, timely referral to a transplant center is crucial as the procedure takes time.
Post-transplant treatment
After lung transplantation, patients must take antimicrobial, antifungal, and antiviral medications in the initial phase and additional immunosuppressive medications for the rest of their lives.
Palliative and end-of-life care
Palliative care is a holistic approach to care, given to improve the quality of life of patients throughout the course of their disease. It aims to provide you with relief from both the physical pain, and the broader stresses and problems associated with your condition.
For ILD patient key aspects of palliative care include advance care planning, managing side effects of medications and symptoms like cough and breathlessness, psychological support, pulmonary rehabilitation and end-of-life care.
HOW CAN PEOPLE AFFECTED TAKE CARE OF THEMSELVES?
FILD is a highly complex condition which needs to be managed along with its symptoms and it is essential that patients manage some parts of the condition themselves. This allows them to control their care, set realistic goals and prepare for their future.
A doctor should advise patients on how to deal with the challenges associated with their condition, but there are lifestyle changes that can improve their quality of life. Giving up smoking or avoiding exposure to second-hand smoke can increase oxygen levels in the blood, lower blood pressure and heart rate and reduce the risk of cancer and heart diseases.
Stay active
Regular, moderate exercise not only helps maintain a healthy weight, but also strengthens the muscles and keeps the body working efficiently. This can include guided exercise with a physiotherapist, but also exercising independently. Daily activity should be adapted to the patient’s performance status and lung function. For patients with respiratory failure receiving supplemental oxygen, physical exertion should be minimal.
Eat healthily
A balanced, nutritious diet is usually recommended. In patients taking antifibrotic drugs, different side effects related to the gastrointestinal tract, such as diarrhea, nausea, bloating, decreased appetite and weight loss which is associated with worse outcomes of fILD, may occur. In such a situation, changing the diet may be crucial, e.g. avoiding raw vegetables or diary products. In addition, diet adjustments may be advised depending on treatment (e.g., risk of diarrhea with Nintedanib).
Patients need to work closely with their care team to take an active and positive role in their
treatment pathway and outcomes.
Get plenty of rest
Sleep is essential for boosting the immune system and improving the overall sense of well-being. What is more, the risk of obstructive sleep apnea is increased in IPF, even in the absence of obesity.
Stay up to date with vaccinations
It is especially important that patients stay up to date with influenza, pneumonia, pneumococcus and Covid-19 vaccines. They should also avoid exposure to infections as they can make fILD worse. Patients taking immunosuppressants should keep their herpes zoster virus vaccinations up to date.
Avoid stress
Being physically and emotionally relaxed may help patients to avoid excessive oxygen consumption. Relaxation techniques can also teach patients to manage the panic that can occur with shortness of breath.
Relaxation and mindfulness
There are different relaxation techniques that can help patients cope with the emotional and psychological challenges that may come with the fILD diagnosis. Mindfulness can help manage the physical and lifestyle challenges of living with fILD and to calm patients when they are distressed, discouraged, scared, or in pain.
Yoga and similar activities
Yoga or other activities, for example Pilates, Tai Chi or Qi Gong, are beneficial for patients with fILD as they can stimulate the lungs through focused breathing and also train the diaphragm. Simple breathing exercises can improve lung function and relieve breathing problems.
What is the outlook for patients with ILD?
Since ILD can not be cured, patients have to learn to live with the complications and symptoms that come along with it. The scarring of the lung tissue can not be reversed, but slowed down in order to preserve as much quality of life as possible.
How much quality of live can be preserved depends widely on the severity of the disease. Cases of mild ILD usually allow patients to keep on living a normal life without treatment and impairment.
Patients suffering with severe cases of ILD often struggle with the disease getting worse. A lifelong need for treatment and therapy is often needed to help manage their symptoms.