Imagine your lungs are filled with a thick, sticky substance that makes every breath a challenge. That's what people with cystic fibrosis face every day, say respiratory therapists from the American Association for Respiratory Care (AARC).
"Cystic fibrosis is a disease that is inherited from your parents," explains Dr. Jerry Edens from Cincinnati Children's Hospital in Ohio. "The main symptoms involve pulmonary insufficiency and a general malnourishment, but other aspects can include diabetes, digestive disorders and chronic sinusitis."
Since cystic fibrosis is a genetic condition (Edens notes a child must inherit defective copies of the gene from both parents in order to develop the disease), it is usually diagnosed in the very young. Children who suffer from CF face lifelong treatment to manage the respiratory and digestive components.
Edens says respiratory therapists play a key role in helping with the former. "At the basis of the pulmonary condition lies a problem with salt exchange. This imbalance causes the mucus in the airways to become very thick and tenacious."
That, coupled with a condition called brochectasis-which results in a deterioration of the airways that makes it difficult for people to clear the mucus from their lungs-is the main cause of the respiratory problems.
"Respiratory therapists are very knowledgeable in many different therapy modalities that will help enhance the patient's ability to get rid of the mucus," registered respiratory therapist Edens continues. The key, he emphasizes, is to "adapt the airway clearance therapy to the patient, not the patient to the therapy. A modality is only good if the patient is in compliance."
Respiratory therapists work closely with the patient's physician and family, trying out different modalities to see what works best for that particular patient. "Lifestyle, activity levels, and availability of assistance are topics discussed," Edens says.
Luckily, new therapies have emerged to make treatment easier for patient and family alike. "There are now devices available that are very portable, and patients can take them with them and use as needed," Edens adds. "Breathing techniques have also been developed that enhance mucus clearance without involving any assistive device-only proper training of the patient."
As better and better therapies are developed, more and more cystic fibrosis patients are defying the odds and living well into adulthood. Edens says the average lifespan for someone with the condition is now the early 30s.
The key to determining how long someone will survive appears to be when he or she first develops lung complications and infections that fail to respond to antibiotics. However, Edens says, "research is continuously coming up with new advances and treatment options. We are very hopeful that the survival trend will continue on the rise."
Respiratory therapists (RTs) are specially trained and licensed respiratory health care professionals assisting physicians in diagnosis, treatment and management of respiratory diseases. RTs provide care in hospitals, outpatient centers, physicians' offices, skilled nursing facilities and patients' homes.
The American Association for Respiratory Care (AARC) is a not-for-profit, professional organization, consisting of 40,000 respiratory therapists, physicians and other health care professionals. AARC is dedicated to assisting persons with respiratory diseases receive safe and effective respiratory care.[[In-content Ad]]