With the passing of another International Rare Disease Day, the spotlight has yet again been turned towards uncommon conditions and rare disorders. One of those little-known, rare diseases is a condition known as idiopathic pulmonary fibrosis (IPF). IPF is a very uncommon disease, affecting about 5 million people worldwide, or about 13 per 100,000 people. In the United States, IPF affects about 100,000 people or 0.03% of the population. With less than one percent of the population living with IPF, chances are, you’ve never heard of it. So, what is IPF, and what causes this extremely rare condition? Here’s everything you need to know.
What Is Idiopathic Pulmonary Fibrosis?
Idiopathic pulmonary fibrosis is a serious, rare lung disease. When you breathe in, oxygen enters your lungs, and across tiny air sacs known as alveoli. From there, it moves into your bloodstream and travels to your organs. This process is vital to a proper functioning, healthy body. In people with IPF, the lungs build up scar tissue, making it hard to breathe and pass oxygen to your organs.
IPF gets worse over time. The thick scar tissue that forms in the lungs is similar to the scars you get on your skin after a cut. These slow the flow of oxygen from your lungs to your blood, which can keep your body from functioning properly.
There’s no cure for IPF, and most healthcare professionals consider the condition terminal. That said, everyone’s outlook on IPF is different. For most people, the symptoms of IPF won’t get better, but certain treatments can slow the damage to the lungs. In some cases, symptoms can progress quickly, with a life expectancy of only a few years. Other cases of IPF can last for nearly a decade or longer.
There are several treatments for IPF, most of which are meant to help you breathe easier and manage other symptoms associated with the degenerative condition. In some cases, a lung transplant may be necessary.
What Causes Idiopathic Pulmonary Fibrosis?
The word idiopathic refers to the fact that the condition has no known cause. Other types of pulmonary fibrosis have causes associated with them, such as pollution, certain medicines, or an infection. However, in the case of IPF, doctors are at a loss for what could cause the chronic condition.
That said, several risk factors are thought to make you more susceptible to IPF. These include:
- Age: Almost everyone diagnosed with IPF is over 50
- Gender: About 75% of people diagnosed with IPF are men
- Genetics: Around 20% of people who have IPF have a family member with a similar lung disease
- Breathing in wood or metal dust at work or home
- Having acid reflux disease
- Smoking cigarettes
IPF can be very difficult to diagnose, as it shares many of the same signs as other lung diseases. Some people can have IPF for years without knowing. It may years of doctor visits to get the right diagnosis. If you have trouble breathing that doesn’t get better, or if you believe you are at risk, you’ll probably need to see a pulmonologist (a doctor who treats lung problems).
As previously mentioned, IPF can go undetected for years with little to no symptoms. The symptoms of IPF develop over time as the scar tissue continues to build up. Symptoms may be different from person to person and can develop more rapidly or slowly depending on the case.
Generally speaking, there are signs and symptoms of IPF that everyone can experience at some point. These may include:
- A dry, hacking cough that persists and won’t go away
- Chest pain or tightness
- Shortness of breath, especially when you walk or exercise
- Loss of appetite
- Swelling in your legs
- Aching muscles and joints
- Gradual, unintended weight loss
Some people with IPF develop other serious lung conditions, including lung cancer, blood clots in the lungs (pulmonary emboli), pneumonia, or high blood pressure in the blood vessels that supply the lungs (pulmonary hypertension).
Most people with IPF have an average life expectancy of around three to five years after their diagnosis. This makes early diagnosis your best bet for effective treatment, which could prolong life expectancy and improve your symptoms.
Treatment for Idiopathic Pulmonary Fibrosis
Treatment for IPF won’t cure the disease, but it can make it easier to breathe and ease other associated symptoms. Some treatments may keep slow lungs from building up scar tissue, while others may try to irradicate the buildup that currently exists. Since IPF looks different for everyone, treatment is also prescribed on an individual basis. Depending on your condition, the severity of the damage to your lungs, and several other factors, your doctor may recommend the following treatment options.
- Medicine: There are currently two drugs that are approved by the Food and Drug Administration (FDA) for IPF. Nintedanib (Ofev) and pirfenidone (Esbriet) are both used to treat symptoms associated with IPF. While scientists are still trying to figure out how these drugs work, they have found that they slow the damage caused by scarring. Doctors may also prescribe corticosteroids such as prednisone to help reduce inflammation during a flare-up of the disease.
- Oxygen Therapy: Oxygen therapy can help you breathe easier if the scarring in your lungs has become severe. During oxygen therapy, you breathe oxygen through a mask or prongs that go in your nose. This boosts the oxygen in your blood, which can help reduce shortness of breath and make you more active. The level of oxygen therapy you need will depend on the severity of your condition. Your doctor may recommend oxygen therapy only while you sleep, or for 24 hours a day in more serious cases.
- Pulmonary Rehabilitation: Pulmonary rehabilitation is a type of care designed to help manage your IPF symptoms. Think of it as physical therapy for your lungs. During pulmonary rehab, you will work with a team of doctors, therapists, and pulmonologists to manage your symptoms and make life easier. You may focus on things like excises to help build your lung reserves, healthy eating, relaxation, and destressing techniques, and waves to save your energy. This can be done either at the hospital or at home.
In cases where IPF becomes very severe or develops rapidly, your doctor may recommend a lung transplant.
Lung Transplant For IPF
A lung transplant is a major surgery that can help you live longer but can carry serious risks. If you fit the criteria, your doctor will put you on the waiting list for a lung transplant from a donor. It is impossible to predict how long the waiting period will last for a lung to become available. That said, the average waiting time for patients with IPF or similar diseases is about three to six months, although some cases could take years.
After your surgery, you may need to stay in the hospital for a month or longer. You will also need to take drugs for the rest of your life to ensure that your body does not reject your new lung. You’ll also need to have routine tests to see how well your lungs are functioning and will need regular physical therapy to help you recover.
A lung transplant is also a very emotionally challenging surgery. If you’re thinking about a transplant, you may find that finding support from family and friends can help you both before and after your procedure. Support groups are also another great way to cope with the challenges of a lung transplant. They can help you connect with other people who have had transplants, and talk to you about their journeys. Talk with your doctor about programs that can help explain what to expect before and after the surgery.
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