Idiopathic pulmonary fibrosis (IPF) is a rare lung disease, with only about three to nine cases per 100,000 people in Europe and North America. So you’ll likely find that many people have never heard of IPF.
The rarity of this disease leads to a great deal of misunderstanding. If you or someone close to you has been diagnosed with IPF, you might have encountered a lot of questions from well-meaning but confused friends and family members. Here’s a guide to help you answer the questions people close to you might have about IPF.
What is IPF?
Given the rarity of IPF, you’ll likely have to start your conversation by explaining what IPF is. In short, it’s a disease that causes scar tissue to form deep inside your lungs. This scarring — called fibrosis — stiffens your lung’s air sacs so they can’t deliver enough oxygen into your bloodstream and out to the rest of your body. Explain that this chronic lack of oxygen is why you cough a lot, feel tired, and get short of breath whenever you walk or exercise.
Did you get IPF from smoking?
With any lung disease, people have a natural tendency to wonder if smoking is to blame. If you smoked, you can respond that the habit might have increased your risk of getting the disease.
Yet cigarette smoking doesn’t necessarily cause IPF. Other factors, including pollution, certain medicines, and viral infections, could have also increased your risk. Let the person know that in most cases, IPF isn’t due to smoking or any other lifestyle factors. In fact, the word “idiopathic” means that doctors don’t know exactly what caused this lung disease.
How does IPF affect your life?
Anyone who is close to you might have already witnessed the symptoms of IPF. Let them know that because IPF prevents enough oxygen from getting out to your body, it’s harder for you to breathe. That means you might have trouble doing even the most basic activities — like taking a shower or walking up and down stairs. Tell them that even talking on the phone or eating could become difficult for you as the condition worsens.
You might have to skip out on some social events when you don’t feel well.
If you have clubbing of your fingers, you can explain that this symptom is also due to IPF.
Is there a cure?
Let the person know that, although there is no cure for IPF, treatments like medicine and oxygen therapy can help manage symptoms like shortness of breath and coughing.
If the person asks why you can’t just get a lung transplant, tell them this treatment isn’t available to everyone with IPF. You have to be a good candidate and be healthy enough to undergo surgery. And even if you meet these criteria, you’ll have to get on an organ transplant waiting list, which means waiting until a donor lung becomes available.
Are you going to die?
This is one of the hardest questions to answer, especially if a child is asking it. The prospect of death is as hard on your friends and family as it likely is on you.
A quick search of the internet will turn up statistics showing that the average person with IPF survives just two to three years. While these numbers sound scary, explain that they’re misleading. Though IPF is a serious disease, everyone who gets it experiences it differently. Some people live for many years without having any real health problems. Treatments — especially a lung transplant — could improve your outlook dramatically. Reassure the person that you’ll do what you can to stay healthy.
How can I learn more about IPF?
If your doctor’s office gives out pamphlets on IPF, have some on hand to provide. Point people to web resources like the National Heart, Lung, and Blood Institute, American Lung Association, and Pulmonary Fibrosis Foundation. These organizations offer educational resources and videos that describe IPF, and its symptoms and treatment.
Encourage the person to attend a support group meeting with you to find out what it’s like to live day to day with IPF. If you’re close to them, you might encourage them to also join you at one of your doctor visits. Then they can ask your doctor any outstanding questions they might have about your condition.