Pulmonary fibrosis is a serious disease that can possibly lead to increasing scarring of the lung tissues. The disease starts with injuries to the tissues, both within and between the alveoli (tiny air sacs) in the lungs. Continued damage leads to further scarring, which in turn can cause the lungs to become stiff.
A normal lung is flexible and pliant so that it can expand and contract for full breaths. The continued fibrosis (the medical term for scarring) makes breathing difficult and can lead to shortness of breath and a dry cough. Medications and therapy are usually used to treat pulmonary fibrosis, with the aim of improving lung function and quality of life for the sufferer. For those with advanced cases of pulmonary fibrosis or those who are not responding to treatment, there might need to be a lung transplant.
Timothy is concerned about his health. He has several problems that make him wonder if he does not have a severe problem developing. He goes to the doctor eventually with symptoms that include shortness of breath and a dry cough. In addition, he is always very tired and has lost almost twenty pounds although he has had no change in his diet at all. He has started feeling out of breath even when he is simply getting dressed and he can no longer do any of the things that he has previously enjoyed doing.
There are 300 million alveoli in each of the lungs, for a total of 600 million in the body. In each of these tiny air sacs, a small blood vessel removes oxygen from the blood and replaces it with carbon dioxide. This process is repeated every time that you breathe in and out and is necessary to keep the body supplied with oxygen.
Pulmonary fibrosis allows scarring to build up and make the lungs stiffer than they should be so that breathing is very difficult. There are several reasons that this might happen, including a problem with the nutrients inside of the body and a misfire in the healing process.
What Causes Pulmonary Fibrosis
After being diagnosed, considered to be a medical challenge in most cases, Tim will look at the factors that may have contributed to the damages to his lung. There are hundreds of factors that can damage the lungs and lead to pulmonary fibrosis, but some of the most common include:
– Environmental or occupational factors: there are a number of toxins and pollutants that you can come in contact with both in your job and daily life. Grain dust, sugar cane and animal droppings can also cause these problems as well.
– Radiation: lung damage can occur in people who are given radiation treatment for lung or breast cancer. This damage may not show up for months or even years, after the first treatment was done. The use of chemotherapy can also increase the risk of damages to the lung.
– Medications: certain drugs can be harmful to the lungs, including some chemotherapy drugs, some heart medications, some psychiatric medications and some antibiotics.
– Gastroesophageal reflux disease, also called GERD: when stomach acids back up into the esophagus, it can lead to damages not only to this structure but to the lungs as well.
– Lung infections and other medical conditions: some lung diseases, such as tuberculosis and pneumonia, can lead to the scarring that increases the risk for pulmonary fibrosis. Other conditions that can lead to this disease: systemic lupus erythematosus, rheumatoid arthritis, dermatomyostitis, poliomyelitis, Sjogren’s syndrome and acidosis. It may also be associated with scleroderma.
(Source: The Mayo Clinic)
There are some times when the disease has no known causes or risk factors. In these cases, it is called idiopathic pulmonary fibrosis. Timothy has never been a smoker nor has he ever had to have chemo or radiation treatment for any reason. The only factor that may have contributed to the condition is the work that he does in a factory. In addition, he has also had several bouts with pneumonia in the past few winters, but was never that worried about it until now.
After diagnosis, which involved a chest X-ray and a bronchoscopy (a method that allowed for a biopsy), Timothy is being given his treatment options for pulmonary fibrosis. One of the things that he is going to have to work on is improving his nutrition.
He will need to make sure that he is getting all of the nutrition that his body needs to stay strong and keep his lung function where it needs to be. He has already lost some weight and the doctor would like to keep him from losing more. While he wants to comply as much as possible, it is not easy for him to eat because he just does not feel up to it. One of the suggestions that the doctor makes for him is to use a protein supplement. He tries one, but it is too much for him and he gives up. He finally finds another option, which is small enough for him to consume without a lot of effort. For every serving, he gets 25 grams of protein without carbohydrates or fat. The additional protein is needed to prevent any further damage to his lungs and to keep his immune system working at its highest level.
Timothy is shocked though when his doctor tells him that he needs to start exercising more frequently. At first, it makes him feel even worse to move around, but after a few weeks, he finds out that he is actually feeling a little better and can breathe a little easier. He tries to make sure that he is also getting enough rest each night.
Once pulmonary fibrosis starts it cannot be reversed. It also cannot be stopped from progressing further. The treatments that are used are meant to slow this progression and to help keep the quality of life high.
Medications that are used to treat this disease include prednisone, a corticosteroid. Others include methotrexate or cyclosporine (both are immunosuppressants), and N-acetylcysteine, a derivative of a naturally occurring amino acid that may be added to the prednisone, which can slow the progression in some people. Another drug, pirfenidone, is being used in clinical trials and is showing some promise. (Source: The Mayo Clinic).
These medications can cause serious side effects, which can include diabetes, glaucoma, skin cancer and lymphoma. They may also cause the body to produce fewer red blood cells. Because these side effects are potentially as dangerous as the condition which the medication is treating, it is important that the doctor monitor for signs of improvement and discontinue the drugs if there is none after six months time. There are some people who will have no response on the medications at all. The treatment option of last resort for pulmonary fibrosis is a lung transplant.
In addition to these, there are therapies that are used to improve the quality of life. These include oxygen therapy and pulmonary rehabilitation. The wait for lungs to become available can be very long and emotionally draining for most people. In addition, they must make sure to stay as healthy as possible so that they can be ready for surgery whenever the call comes in.
Mayo Clinic Staff Pulmonary Fibrosis Mayoclinic.com