For Other Nurses: Chronic Pulmonary Fibrosis?

My husband's father was just diagnosed with Chronic Pulmonary Fibrosis. He's a non-smoker so we think it's environmental from where he used to work. He's in his 60's and has been on oxygen since before January.

Although I'm a nurse, respiratory diseases are not my speciality. They've told him that the only treatment option is Prednisone and that he would be required to live a sedintary lifestyle because of both the disease and the side effects of the Prednisone. I've not met him, but from what my husband says, this is NOT within his father's character.

We live about 24 hours from his father, so this is very stressful for us in that we cannot be near him and give him the support he needs and I feel helpless as I'm not familiar with the treatment options for this.

Is Prednisone the only option for him or are there other options we can look into?

CPF is a disorder where the alveoli (air sacs) in the lungs harden and limit the amount of air that can be transmitted to the body. In short, the patient suffocates little by little until the lungs can no longer function.

Many times treatment is limited only to treating the inflammatory response that occurs in the lungs with the pulmonary fibrosis. This is done in the hope that stopping the inflammation will prevent the laying down of scar tissue or fibrosis in the lungs and thus stop the progression of the disease. Corticosteroids are the drugs which are usually administered in an attempt to stop the inflammation. The advantage of this treatment has not been proven in every case, although it does appear that if the drugs are given early on in the course of the disease, there is a better chance of improvement. Corticosteroid medications can have various side effects and so patients taking these medications must be frequently reassessed by their physicians in order to judge the safety and benefit of this therapy.

Other drugs have been tried but convincing evidence of their efficacy is lacking.

Although drug therapy of pulmonary fibrosis may not always be successful, there is much that can be done in the way of supportive therapy that will ease the breathlessness that accompanies this condition.Pulmonary Rehabilitation and education programmes can help considerably in teaching patients how to breathe more efficiently and to perform their activities of daily living with less breathlessness. Sometimes supplemental oxygen therapy is required in order to treat breathlessness. Early treatment of chest infections is required. Smoking must be discontinued, as the effects of tobacco will aggravate the shortness of breath.

Ask you doctor about pulmonary rehab prgrams in his area.

WHATS THAT

um... Prednisone is the medication of choice, but I pretty sure you can take nitro glycerin too.

Matt is right on with his answer. I have suffered with IPF for 4 years - one year longer than the doctors gave me to live. I took prednisone for a while, but the side effects and toxic nature of the drug actually did more harm than good. I gained about 60 pounds and became sedentary and oxygen dependent because of prednisone. After spending some quality time with a transplant surgeon, I was weaned off steroids. For the past 4 years I have become an advocate for the Pulmonary Fibrosis Foundation and kept myself busy by trying to educate as many people as possible about this disease.

I lost 40 of the 60 pounds and went through pulmonary rehab to help my breathing technique. I also moved from the city (too much pollution and a high altitude - 5,000 feet above sea level) to a town on the Pacific Coast. The move allowed me to wean myself off full time oxygen. I now use supplemental oxygen only while exercising and when I sleep at night. The O2 at night is administered through a CPAP. This allows my lungs to rest during the night and oxygenates my tissues and organs.

IPF is a progressive disease that kills 100% of those afflicted with it, usually within 5 years of diagnosis. In the US, 50,000 people die from IPF every year and the number is actually climbing. The only "treatment" available at this time is a lung transplant. I have explored that option and decided it is not for me (I am 53 yrs. old). I would only be trading my current set of problems for another set with no guarantee of the length or quality of my life.

Unfortunately, your husband's father is terminally ill. As a nurse you realize that hope is essential, but terminal means terminal. He needs to remain active. I still walk 2 miles every day using 3 liters of supplemental oxygen. I don't break any land speed records, but the exercise is necessary to my overall health. Every day is a victory and a gift. Encourage your father-in-law to remain active. If he needs oxygen full time, he can still remain ambulatory using a portable system, but he needs to get up and get going. He needs to get pulmonary rehab from someone like Matt so he learns to use every lung cell available. The disease will kill him, but he can postpone the inevitable by fighting this hideous disease. It is a horrible way to die, but the past 4 years of my life have been the best 4 years of my life.

He is lucky to have you and your husband for support. Even if you cannot visit, you can call, email, send videos via webcam. The bottom line is to encourage, support, and love him.

I am sorry to hear he is going through this. For more information you can go to one of the following websites for information. Good luck to you all.

www.pulmonaryfibrosis.org
www.coalitionforpf.org