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The Respiratory System
Naturally occurring AAT plays an important part in protecting lungs from damaging enzymes. Learning about the respiratory system is an important step in understanding how AAT works and why inadequate AAT can result in lung damage.
The respiratory system allows gas exchange between air and blood, enables speech, provides the first line of defense against infection, and helps regulate the pH of blood.
Components of the Respiratory System
The respiratory tract consists of the nose, nasal cavity, pharynx, larynx, trachea, bronchi, bronchioles, and alveoli. The nose and pharynx make up the upper respiratory system, and the larynx, trachea, bronchi, and lungs comprise the lower respiratory system.
How Does the Respiratory System Work?
The respiratory tract can be divided into a conducting portion that conducts air into the lungs, and a respiratory portion where gas exchange occurs. As air travels through the conducting portion—the nose, pharynx, larynx, trachea, bronchi, and bronchioles—air is filtered, warmed, and humidified.
The respiratory portion is composed of the smallest bronchioles and alveoli. It is in the alveoli that gas exchange takes place. There are approximately 150 million alveoli in each lung. The respiratory membrane of the alveoli is very thin and covers a large surface area – about the size of a tennis court. This large thin membrane allows oxygen and carbon dioxide to diffuse rapidly and efficiently.
A deficiency of the naturally occurring Alpha-1 protein may result in destruction of alveoli, which leads to reduced surface for gas exchange and less oxygen in the blood. Destruction of alveoli also can cause loss of elasticity and air trapping in the lungs.
GLASSIA [Alpha1-Proteinase Inhibitor (Human)]
GLASSIA is indicated for chronic augmentation and maintenance therapy in individuals with emphysema due to congenital deficiency of alpha1-proteinase inhibitor (Alpha1-PI), also known as alpha1-antitrypsin (AAT) deficiency.
- The effect of augmentation therapy with GLASSIA or any Alpha1-PI product on pulmonary exacerbations and on the progression of emphysema in Alpha1-PI deficiency has not been demonstrated in randomized, controlled clinical trials.
- Clinical data demonstrating the long term effects of chronic augmentation and maintenance therapy of individuals with GLASSIA are not available.
- GLASSIA is not indicated as therapy for lung disease in patients in whom severe Alpha1-PI deficiency has not been established.
Detailed Important Risk Information for GLASSIA
- GLASSIA is contraindicated in IgA deficient patients with antibodies against IgA. GLASSIA is contraindicated in individuals with a history of severe immediate hypersensitivity reactions, including anaphylaxis, to Alpha1-PI products.
- GLASSIA is made from human plasma. It may carry a risk of transmitting infectious agents, such as viruses, and theoretically, the Creutzfeldt-Jakob disease (CJD) agent.
- Administer GLASSIA at room temperature at a rate not greater than 0.04 mL/kg body weight per minute. If anaphylactic or severe anaphylactoid reactions occur, discontinue the infusion immediately.
- Administer GLASSIA within 3 hours of entering the vials.
- Safety and effectiveness in patients over 65 years of age have not been established.
- Two serious adverse reactions observed on two separate occasions during clinical studies with GLASSIA were cholangitis and exacerbation of chronic obstructive pulmonary disease (COPD).
- The most common product-related adverse reactions in clinical studies were headache and dizziness.
Please see GLASSIA Full Prescribing Information for full prescribing details.
ARALAST NP [Alpha1-Proteinase Inhibitor (Human)]
ARALAST NP is indicated for chronic augmentation therapy in patients having congenital deficiency of A1-PI with clinically evident emphysema.
- The effect of augmentation therapy with ARALAST NP on pulmonary exacerbations and on the progression of emphysema in alpha1-antitrypsin deficiency has not been demonstrated in randomized, controlled clinical trials.
- Clinical data demonstrating the long-term effects of chronic augmentation or replacement therapy of individuals with ARALAST NP or ARALAST are not available.
- ARALAST NP is not indicated as therapy for lung disease patients in whom congenital A1-PI deficiency has not been established.
Detailed Important Risk Information for ARALAST NP
- ARALAST NP is contraindicated in IgA deficient patients with antibodies against IgA, due to the risk of severe hypersensitivity.
- ARALAST NP is derived from pooled human plasma. It may carry a risk of transmitting infectious agents, e.g., viruses and theoretically, the Creutzfeldt-Jakob disease (CJD) agent.
- The recommended rate of administration (≤0.08 mL/kg/min) should be closely followed and vital signs monitored continuously. If anaphylactic or severe anaphylactoid reactions occur, the infusion should be discontinued immediately.
- Safety and effectiveness in patients over age 65 years of age have not been established.
- ARALAST NP should be administered at room temperature within three (3) hours after reconstitution and should be administered alone, without mixing with other agents or diluting solutions.
- The safety of ARALAST NP was evaluated with ARALAST in a crossover clinical PK comparability study. The most common adverse events deemed related to ARALAST NP included headache and musculoskeletal discomfort. No serious adverse reactions or deaths were reported in the study. In the ARALAST pivotal study, the most common adverse events were headache and somnolence.
Please see ARALAST NP Full Prescribing Information for full prescribing details.