Cystic fibrosis (CF) is the most common genetic disease affecting the Caucasian population. It is caused by a mutation in a particular protein in a person’s DNA. This mutation causes a malfunction in the sodium and chloride transport channels. As a result, large amounts of salt are secreted in the sweat of a person with cystic fibrosis. Additionally, the body produces abnormally thick, sticky mucus which creates problems for nearly every major body function, particularly the respiratory and digestive systems.
People with cystic fibrosis are prone to frequent bouts of pneumonia and other lung infections. This is because bacteria become easily trapped in the sticky mucus that blocks the airways. Airway clearance devices and preventive maintenance medications are typically used to avoid infection. Such methods include inhaled antibiotics, bronchodilators, aerosolized medications, and chest physiotherapy.
Another complication of CF involving the lungs is hemoptysis. In its simplest definition, hemoptysis is blood in the sputum. In patients with CF, the source of the bleed is typically from the bronchial arteries rather than the pulmonary arteries. When it happens for the first time, hemoptysis is more than a little frightening. Hemoptysis is usually a symptom of something else going on in the lugs. Any of the following may be the underlying cause of the bleedingInfectio
-Dryness
-Reaction to a particular nebulizer treatment (e.g. Pulmozyme or Hypertonic saline)
-Bronchiectasis
-Vitamin K deficiency
-Overexertion
Infection is the leading cause of hemoptysis. When the bronchial arteries become swollen and inflamed in response to an infection, they may burst and bleed. Unless the bleeding is massive (i.e. more than a few tablespoons in an hour) then this is rarely cause for alarm. It’s just one of the frightening and unpleasant side effects of cystic fibrosis. Bleeding in the lungs may also occur when the tissue and blood vessels of the lungs becomes particularly damaged, after many subsequent infections or bouts of pneumonia.
Hemoptysis may also be the result of an adverse reaction to Pulmozyme or hypertonic saline inhalation treatment. Some cystic fibrosis patients simply cannot tolerate Pulmozyme. There are a number of studies that have demonstrated that a specific component of Pulmozyme, (DNase I) triggers lung hemorrhage.
Occasionally the bleeding is simply the result of overexertion during the usual process of airway clearance. Coughing too hard or too long may cause a small bleed. This type of bleeding clears up on its own in a relatively short period of time. The same is true for bleeding that is the result of the lungs being exposed to dry air. Some women also report brief episodes of hemoptysis during menstruation.
Hemoptysis that does not resolve on its own requires medical intervention. A process called bronchial artery embolization (BAE) can effectively stop a minor bleeding episode. This is a more favorable approach than surgery, since anesthetising persons with CF poses a certain degree of risk. A study published in Chest states “Early BAE in patients with CF who have nonmassivehemoptysis is an effective, safe therapeutic approach offeringbetter long-term control of recurrent bleeding and quality oflife than medical therapy alone.” (Chest. 2002;121:796-801.) © 2002 American College of Chest Physicians
When an episode of hemoptysis has occurred, be sure to mention it to the doctor at your next appointment. Your doctor can adjust your medications if they are triggering the bleeding, or prescribe something to address an underlying infection. When describing the episode to your health care provider, be sure to note whether you coughed up dried blood streaked through your sputum, or if it was only bright red blood. Always be able to give a good estimation of how much blood was present.
Reference:
- Pulmonology Channel www.pulmonologychannel.com/hemoptysis/
