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What is Idiopathic Pulmonary Fibrosis?
Idiopathic pulmonary fibrosis (IPF) is an incurable lung disease in which fibrosis, or scarring, occurs in the lungs making them thick and stiff. As lung tissue thickens, the lungs have difficulty moving oxygen into your bloodstream, resulting in a decreased amount of oxygen to your brain and other organs. Sometimes doctors can determine what’s causing IPF. When the cause is unknown, they call it idiopathic.[1]
Known Causes of Pulmonary Fibrosis
Known causes of pulmonary fibrosis include:1
- Exposure to environmental pollutants including silica, hard metal dusts, bacteria and animal proteins.
- Certain medicines such as nitrofurantoin (an antibiotic), amiodarone (a heart medicine), methotrexate and bleomycin (both chemotherapy medicines) and a wide variety of other medications.
Risk Factors for IPF
You’re more likely to get IPF if you:1
- Smoke cigarettes.
- Have certain viral infections including the Epstein-Barr virus (the cause of mononucleosis), influenza A virus, hepatitis C virus, HIV and herpes virus 6.
- Have a family history of IPF.
- Have gastroesophageal reflux disorder (GERD).
Signs and Symptoms of IPF
The signs and symptoms of idiopathic pulmonary fibrosis are progressive, meaning they develop over time. Initially, you may not even notice them. Once you do, chances are serious lung damage may have already occurred. Because the disease is progressive, symptoms generally worsen over time.1
The most common signs and symptoms of IPF include the following:1
- Shortness of breath that initially may only occur when you exercise, but may eventually occur even at rest.
- A dry hacking cough that doesn’t go away. In time, you may experience repeated bouts of coughing that you won’t be able to control.
Other signs and symptoms that may develop gradually include:1
- Shallow, rapid breathing
- Unintended weight loss
- Fatigue (different than ordinary tiredness) and malaise (feeling generally unwell)
- Aching joints and muscles
- Clubbing (widening and rounding) of the fingertips
Complications of IPF
As IPF worsens, it may lead to the following complications, some of which are life-threatening:1
- Collapsed lung
- Blood clots in the lung
- Lung cancer
- Respiratory failure
- Pulmonary hypertension (high blood pressure in the blood vessels of the lungs)
- Heart failure
Treatment of IPF
There are 4 treatments used in the management of IPF including:
Medications
To date, there are no medications known to slow the progression of IPF. Prednisone (a corticosteroid), azathioprine (suppresses the immune system) and N-acetylcysteine (a cough medicine) used alone or in combination have been utilized to treat IPF, however, more research is needed to support their use.1
Other treatments that may help people with IPF include flu and pneumonia vaccines (to prevent infections), cough medicines that include codeine (to control cough) Vitamin D, calcium and bone- building medications (to help prevent bone loss caused by corticosteroids) and anti-reflux therapies to help reduce the symptoms of GERD (because most people with IPF also have GERD).1
Oxygen Therapy
If the level of oxygen in your blood decreases, you may need supplemental oxygen (oxygen therapy) to restore your blood oxygen level to normal. Oxygen is usually delivered through nasal prongs or an oxygen mask. Initially, you may only need supplemental oxygen during exercise or when you sleep. As your disease progresses, you may need it continuously.1
Pulmonary Rehabilitation
Pulmonary rehabilitation (PR) is now the gold-standard of care for people who have chronic (ongoing) lung disease. PR uses a multi-disciplinary approach to treatment that usually involves a team of specialists from a wide variety of disciplines including doctors, nurses, respiratory therapists, psychologists, physical therapists, occupational therapists, dietitians and more.1
PR is used in conjunction with your medical therapy and may include:1
- Exercise training
- Nutritional counseling
- Education on lung disease and how to manage it
- Energy conservation techniques
- Breathing techniques
- Psychological counseling and/or group therapy
- Help with smoking cessation
Lung Transplant
If your condition progresses rapidly and is unresponsive to standard medical care, your doctor may recommend a lung transplant. A lung transplant may help you live longer and improve your quality of life. Only a select group of patients who meet very specific criteria are eligible for a lung transplant. For more information about lung transplants and to see if you qualify, talk to your primary care provider.1
Prognosis
IPF usually affects people who are middle-aged or elderly. The progression of IPF varies from person to person. For example, in some people, fibrosis occurs rapidly. In others, the progression of IPF is much slower. Still, in others, the disease stays the same for many years.1
Because idiopathic pulmonary fibrosis is progressive and has no cure, many people live only about 3 to 5 years after being diagnosed. But everyone’s outlook is different. Some people live 10 years after diagnosis.[2]
Respiratory failure is the most common cause of death related to IPF. Other causes of death include pulmonary hypertension, pulmonary embolism (blood clot in the lungs), heart failure, pneumonia and lung cancer.1
For more information about managing idiopathic pulmonary fibrosis and your own, individual prognosis, talk to your primary care provider.
[1] National Heart, Lung and Blood Institute. What is Idiopathic Pulmonary Fibrosis? Last updated September 20, 2011.
[2] WebMD. Idiopathic Pulmonary Fibrosis. Last reviewed January 3, 2017.
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You need to update your blog. There are two new drugs to slow the progression of Idiopathic Pulmonary Fibrosis OFEV (nintedanib) & Esbriet (pirfenidone).
Hi Jo, Thank you for bringing this up to us. We will do some research and adjust as needed.
Hurry up Inogen and get the two medicines as indicated by Jo above onto your blog. Ofev and Esbriet are now commonly used drugs for IPF.
Hi Rene, We're working on it. As a medical device company we need to do our research and have our regulatory team review everything before we post it. It can sometimes be a slow moving process but I assure you we are looking into these medications.
I have been on Ofev for around 3 months now and as Jo said it's not a cure but hopefully a way to slow it down. In my case Ofev comes from a specialty pharmacy and is very expensive. I was told my annual cost would be in excess of $100,000. and I said no thanks. Between my Dr., my Medicare Insurance (Humana) and another
group they are covering the cost for at least the first two years.
You also need to include the use of CellCept/Mycophenolate. The use of this medication helps slow the progression of IPF in some but not in all patients. I have been using Mycophenolate since 2011, and it has helped me in keeping my IPF stable. Also, the protocol in the use of perdinisone, azathioprine/imuran, and NAC/N-acetylcysteine together is no longer used. It was noted to have made IPF worse in patients that took that regimen. I was on that and it made me sicker and was taken off that protocol after 2 months and placed on Mycophenolate. Please update your blog.