What is Pulmonary Fibrosis?

Pulmonary fibrosis is a disease that affects the air sacs of the lungs. These air sacs are necessary for tranferring oxygen from the lungs to the bloodstream. Pulmonary fibrosis causes scarring in the air sacs which, as it progresses, makes breathing increasingly difficult.

This disease has several causes:

•Environmental or occupational exposure to substances such as asbestos or metal dusts

•The formation of inflammatory cells, called sarcoidosis

•Certain medicines

•Radiation treatment for some cancers

•Disorders of connective tissue or collagen, such as rheumatoid arthritis or systemtic sclerosis

•Genetics

Sometimes the cause of pulmonary fibrosis is unknown. These cases are called idiopathic pulmonary fibrosis (IPF) and are characterized by a dry cough and shortness of breath. Both types of pulmonary fibrosis are progressive diseases and have no cure.

Over 200,000 people in the United States are affected by pulmonary fibrosis.

What is Alpha-1 Antitrypsin Deficiency?

Alpha-1 antitrypsin deficiency is caused when there is a reduced amount or, in some cases, none of a protein called alpha-1 antitrypsin (AAT) in a person’s blood. It is one of the most common genetic disorders in the world. In healthy people, AAT is made in the liver and is released into the blood. Individuals who have AAT Deficiency cannot fully release the AAT made by the liver into their bloodstream. This can result in life-threatening liver disease in children and adults, and lung disease in adults.

AAT Deficiency can cause lung diseases such as asthma, chronic bronchitis, emphysema, chronic obstructive pulmonary disease (COPD), and bronchiectasis (in adults). It can also lead to liver diseases such as elevated liver enzymes, neonatal hepatitis, chronic liver diseases, cirrhosis, liver cancer, and panniculitis (a skin disease).

AAT Deficiency can lead to lung destruction and may be misdiagnosed as Chronic Obstructive Pulmonary Disease (COPD) or asthma. The World Health Organization (WHO) recommends that all individuals with COPD (and adults and adolescents with asthma) be tested for AAT Deficiency. A diagnosis can be made with a simple blood test that measures the amount and type of AAT a person makes.

The following are signs and symptoms of AAT Deficiency:

•Family history of lung disease or infant lung disease

•Recurring respiratory infections

•Shortness of breath while at rest or during exercise

•Wheezing, persistent cough

•Persistent sputum (or phlegm) production

•Rapid deterioration of lung function without a history of significant smoking

•Decreased exercise tolerance

•Chronic liver problems

•Non-responsive asthma or year-round allergies

•Sinus infections

•Elevated liver enzymes

About 100,000 people in the U.S. have severe AAT Deficiency. It occurs in all races and genders. About 26 million people in the U.S. have the defective gene that causes the disease. It can possibly be passed on to their children.

How is Pulmonary Fibrosis or Idiopathic Pulmonary Fibrosis Treated?

Caremark is able to provide many of the therapies used in the treatment of pulmonary fibrosis.

Alpha-1 Antitrypsin Deficiency Treatment

Treatment of Alpha-1 Antitrypsin Deficiency includes:

•Behavioral and lifestyle modifications

•Drug therapy for lung problems

•Specialized drug therapy for lung disease

•Treatments for the complications of liver disease associated with AAT deficiency

•Surgery if necessary

One medicine used with in AAT Deficiency treatment is called AralastTM. This medicine is prepared from large pools of human plasma. To reduce the risk of viral transmission, the manufacturing process includes a solvent detergent and a nano-filtration step to remove unwanted viruses.

The drug is prescribed based upon the individual’s weight, and the dosage is given once a week by intravenous infusion. The infusion takes about 30 minutes. Call Caremark toll-free at 1-866-RX-CARE-1 to learn more about treatment for Alpha-1.

Caremark understands that people with pulmonary diseases have special needs. We’ll make sure you get your prescribed medicine from one of our 17 Specialty Pharmacies, along with the medically necessary supplies you need to safely and correctly administer your treatment.

Caremark has many years of experience helping people with pulmonary diseases. In addition to providing your prescribed medicine and supplies, Caremark Specialty Pharmacy Services can help you in many other ways:

Education. We’ve developed customized learning programs about the disease, medicines, lifestyle issues, ways to keep track of your disease, and the importance of following your treatment plan.

Customized care. Sticking to your treatment plan is a top priority, so Caremark assigns each plan participant a CareTeam—a pharmacist and a home patient representative. Your CareTeam will review your medicine schedule, work with you to resolve any problems with your treatment and reinforce the importance of managing your own therapy.

Ongoing communication. Your CareTeam will call you periodically to answer questions, help with treatment issues, schedule shipments of your medicine, and make sure you’re following your treatment plan.

Discreet deliveries. We know how important your privacy is, so we’ll ship your medicines in secure, plain, temperature-controlled packaging to your home, office or other requested location.

24-hour availability. We’re here when you need us. Our Specialty Pharmacy pharmacists are available, 24 hours a day, 365 days a year in case of emergencies.

With Caremark, you’ll have a team of experts on your side, helping you get the most professional, caring and cost-effective service. If you would like to find out more about our services, please call CaremarkConnect® toll-free at 1-800-237-2767.

Navigating Insurance

To inquire about or begin services with Caremark Specialty Pharmacy Services, please call or have your healthcare provider call CaremarkConnect® at 1-800-237-2767. You may also begin the enrollment process here. Once you fill out the requested information, one of our staff of specialists will contact you. Regardless of how you begin the process, our staff of specialists will work with you and your insurance carrier to confirm coverage for your treatment. We’ll conduct a full benefits investigation for you for the medicines you may need.

A specialist will assist you with:

•Securing coverage for your new prescription

•Determining your coverage and minimizing out-of-pocket costs

•Processing insurance paperwork and coordinating benefits

•Handling prior authorization issues

•Obtaining all necessary authorizations

•Communicating your financial obligations verbally and in writing

•Assisting in the coordination of home administration services

•Arranging delivery of your medicines

•Coordinating the start of therapy with your doctor

To explore financial coverage alternatives when insurance is not available to you, we offer Caremark Customer Assistance at 1-800-331-7171. Our staff of specialists will work with you to explore coverage alternatives when insurance is not an option. Please feel free to call us with questions or problems.

To inquire about or begin services with Caremark Specialty Pharmacy Services, please call or have your healthcare provider call CaremarkConnect® at 1-800-237-2767. You may also begin the enrollment process here. Once you fill out the requested information, one of our staff of specialists will contact you. Regardless of how you begin the process, our staff of specialists will work with you and your insurance carrier to confirm coverage for your treatment. We'll conduct a full benefits investigation for you for the medicines you may need.

You may find these Web sites/organizations helpful:

American Lung Association (Pulmonary Fibrosis)

Pulmonary Fibrosis Foundation

* Caremark does not operate the Web sites/organizations listed here, nor is it responsible for the availability or reliability of their content. These listings do not imply or constitute an endorsement, sponsorship or recommendation by Caremark. Information from these Web sites/organizations is not a substitute for medical advice or treatment. Discuss any information you find with your doctor or healthcare professional.

Copyright © 2005 Consumer Health Interactive

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