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TITLE:

Early Pulmonary Fibrosis Symptoms Are Easy to Ignore

ORIGINAL URL: http://healthlink.mcw.edu/article/1031002711.html

ABSTRACT:

Pulmonary fibrosis, a hardening of the lungs caused by scarring, progressively

impairs one's ability to breathe. Patients typically have symptoms for five

years

before they seek a doctor's advice, says Dr. Rose Franco.

ARTICLE:

Pulmonary fibrosis, a hardening of the lungs caused by fiber-like scarring, is a

condition that progressively impairs one's ability to breathe. It is fortunately

very rare, occurring in less than 1% of adults, but accounts for about 40,000

deaths a year in the US.

With pulmonary fibrosis, " The lungs lose their sponginess and become very stiff,

impairing their ability to deliver oxygen to the bloodstream, " explains Rose

Franco, MD, Assistant Professor of Pulmonary/Critical Care Medicine and

Associate Director of the Sleep Disorders Program at the Medical College of

Wisconsin. She is a pulmonary specialist who often cares for patients with this

condition. Dr. Braza practices at the Froedtert & The Medical College of

Wisconsin Pulmonary Medicine Clinic.

Unfortunately, patients typically have symptoms for five years before they seek

a doctor's advice, says Dr. Franco. " Our bodies are so well constructed that you

can lose more than half of your lung capacity before you notice any problem, "

she notes.

" In the vast majority of cases, the doctor will have to carefully review the

patient's work history, medical history and medication history, then follow up

with a variety of tests to determine the cause of the fibrosis and what course

to follow, " she says.

Inflammation Leads to Scarring

Pulmonary fibrosis occurs when lung cells or the spaces between them fill up

with either proteins or inflammatory cells, blocking the flow of oxygen through

the blood vessels. Fibrosis first shows up in the very narrow " interstitial "

walls between lung cells, and eventually involves the alveoli (air sacs), the

bronchioles (small, branching tubes that conduct oxygen to the air sacs), and

the capillaries (the tiny blood vessels that carry oxygen) of the lungs.

" Over time, the inflammatory cells will cause the destruction of air sacs and

blood vessels, " says Dr. Franco. " Then you have more trouble getting oxygen into

the system and getting rid of carbon dioxide. The air sacs and blood vessels

have become scarred and non-functional. "

The primary symptoms of pulmonary fibrosis are quite general, and could be

explained by a number of health problems. Symptoms include:

-Fatigue and weakness

-Discomfort in the chest

-Loss of appetite

-Rapid weight loss

But while some of these symptoms may appear minor, it is critical to be alert to

early signs of the disease. Seeing a doctor is very important if the symptoms

persist, stresses Dr. Franco. " Be especially aware of a persistent cough that

lasts for more than three months, or shortness of breath that is out of

proportion to that of your peers with the same level of exertion. "

Varieties of Pulmonary Fibrosis

Pulmonary fibrosis is a large category, covering a variety of severe lung

conditions that lead to inflammation and possibly scarring, says Dr. Franco.

Among them:

-Pneumoconiosis: Some occupational dusts cause intense inflammation of the

lungs. The most common lung diseases are associated with regular contact with

organic dusts such as coal (producing " brown lung " ), asbestos, or silica.

-Systemic Collagen Vascular Diseases: Fibrosis can also be the outcome of such

systemic diseases as lupus or rheumatoid arthritis (collagen is a tough,

glue-like protein; malfunctioning can cause a hardening of lung tissue). These

diseases can result in progressive scarring of the lung and loss of function.

-Wegener's Granulomatosis: This rare condition results in destructive

inflammation of the blood vessels (vasculitis), which damages the lungs and

kidneys by impairing blood flow and destroying normal tissue.

-Hypersensitivity Pneumonitis: This is a condition that usually occurs among

farmers working with moldy, wet hay. It occurs much more frequently in whites

than in African-Americans, where the incidence is very rare. People with this

condition may develop hypersensititivity to the bacterium or mold; recurrent

episodes of pneumonitis eventually progress to scarring (fibrosis) if not

recognized early and therapy instituted.

-Sarcoidosis: This condition produces inflammatory clusters of cells called

granulomas, which often occur in the lung tissue. Scarring of the lungs will

develop in only about a third of people with sarcoidosis. Sarcoidosis is found

in disproportionately high numbers among African-Americans in the Midwest and it

is thought that some cases may be due to a genetic predisposition.

-Idiopathic Pulmonary Fibrosis: This category covers those cases where no clear

cause can be determined. Most pulmonary fibrosis cases fall into this category.

Testing

" If pulmonary fibrosis is suspected, we will do CAT scans to determine where the

lungs may be inflamed and where they are scarred. We will also perform breathing

tests, both at rest and with activity, " Dr. Franco notes. This testing allows

the doctor to measure how the lungs are functioning and can also be used to

monitor the progress of the disease. In fibrosis the lungs will shrink as they

harden, leading to decreased total lung capacity and decreased diffusion

capacity for gases.

Frequently, in the early stages of disease, testing the patient during activity

is especially revealing. " A test involving walking and simultaneously measuring

the oxygen level through the skin (pulse oximetry) may be the only place where

the scarring shows up, " says Dr. Franco.

" We will also do blood tests for collagen vascular disease. It turns out that

arthritic conditions such as scleroderma, lupus, and rheumatoid arthritis not

only affect the joints, but also the lungs, " she states. " We often also do an

echocardiogram to look at the heart structure and make sure that the heart isn't

the source of the breathing problem. "

In many cases, making the diagnosis requires a lung biopsy. There are two

varieties. A bronchoscopy is used when sarcoidosis is suspected. In this

procedure a lighted scope will be inserted through the throat into the air sacs

to retrieve a tiny sample of tissue about the size of the tip of a pen.

The other type of biopsy is surgical, and is conducted by a cardiothoracic

surgeon. A small incision is made in the chest and a tiny scope is slid between

the ribs to take tissues from two or three spots. " This yields much better

results and gives us a better set of samples, " says Dr. Franco. " But this option

is used when we know it isn't sarcoidosis and the patient is not too ill to

tolerate general anesthesia and a lung surgery. "

Prevention

A substantial number of pulmonary fibrosis cases cannot be explained by

hereditary factors. " In most cases, its sheer bad luck, " says Dr. Franco.

However, there are definite preventive steps that can be taken:

-Be proactive about the possible threats to your lung health from your workplace

and hobbies. Learn about the possible health risks of the tools and materials

you use. At work, these can include lubricants used in the auto industry,

asbestos, silica, and coal dust from metal or wood shavings. Hobbies that

involve the use of propellants for spray painting or coating projects can also

pose a threat.

-One of the most important steps in preventing lung disease is using a

respirator to protect your lungs from substances that could cause scarring. In

some cases, patients might need to make the move to a less risky occupation.

-Be alert to any cough that lasts more than three months, or unusual shortness

of breath after activity.

" Surprisingly, the condition is not associated with smoking by itself, " says Dr.

Franco, while stressing that smoking strongly aggravates pulmonary fibrosis and

is to be strictly avoided. " In the majority of cases we see, something else is

contributing. "

Lung Transplants

For some pulmonary fibrosis patients, a lung transplant can be a viable

solution. But the wait for a lung transplant can be long, and Dr. Franco notes

that there is " a very limited group of donors " whose lungs will produce a

sufficient match.

Despite the twin difficulties of a short supply of donor lungs and finding the

right match, the process of transplantation has become more successful over the

past two decades. " Lung transplants have been done since the 1980s, and

long-term success for these transplant recipients has improved greatly as we

have become smarter about controlling the complications of transplantation, "

observes Dr. Franco.

To increase the availability of transplanted lungs to the living, Dr. Franco

urges everyone to sign the donor statement on the back of their drivers'

licenses.

Courses of Treatment

" We haven't found anything that will cure pulmonary fibrosis, " says Dr. Franco.

" That is especially true for idiopathic cases because we simply have no proven

treatment. Most patients have progressive decline in their lung capacity despite

treatment and many patients succumb to the disease within two years because of

pneumonia or cardiac problems. In every case, one of our goals is to improve the

patient's quality of life. "

" Oxygen treatment is good for relieving breathlessness and reduces the strain on

the heart. Typically, oxygen helps patients to stay active so that they don't

become deconditioned " (so unaccustomed to movement that the muscles weaken, lose

tone, and atrophy).

For patients with less acute, non-specific inflammation, the steroid prednisone

can be effective. " Prednisone is able to slow down the inflammation, and in some

cases puts the scarring process into remission, " she says.

" If we have any hope of improving the chances of saving lung function for people

suffering from pulmonary fibrosis, it's before their lungs are scarred and still

in an inflammatory stage, " Dr. Franco says. " We can't resurrect scar tissue into

normal tissue, so we have to fight the battle to keep the scarring from

progressing. "

There are numerous current national trials being undertaken to study new

therapies for pulmonary fibrosis; most of them require that patients are

enrolled when their condition is not far advanced.

" The benefit of these drugs may only be seen when patients are diagnosed and

treated early in the disease, " says Dr. Franco. " Early diagnosis so very

important and patient participation in national trials is our only hope for

finding an effective cure for this disease. "

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