Understanding Cystic Fibrosis
Cystic fibrosis is a progressive genetic condition that primarily affects your respiratory and digestive function. More than 30,000 children and adults in the U.S. are living with cystic fibrosis. Currently, there is no cure for this condition, but it is manageable. With advances in treatment, people with cystic fibrosis are able to experience a better quality of life and live to a longer age. Here’s what to know about managing cystic fibrosis.
What is cystic fibrosis?
Our bodies all produce mucus, digestive enzymes, and other fluids that lubricate our organs and tissues. People with cystic fibrosis have a genetic mutation that increases the thickness of these fluids. This can affect many organs in your body, particularly your lungs and digestive tract. Cystic fibrosis makes it more difficult for air to pass through your lungs and for the intestine to absorb vital nutrients. In more advanced stages, cystic fibrosis may result in severely impaired respiratory function. The thicker fluids can also trap bacteria, making individuals with cystic fibrosis more susceptible to a variety of infections.
Causes of cystic fibrosis
Cystic fibrosis is caused by a mutation to a gene — the cystic fibrosis transmembrane conductance regulator (CFTR) gene — that affects how water and salt move through your body’s cells. Both parents must carry a mutated copy of this gene in order for a child to develop cystic fibrosis. However, having two carrier parents does not guarantee that a child will have cystic fibrosis; there is a 25% chance.
A child with just one carrier parent will not have cystic fibrosis, but they may become a carrier themselves, increasing their own children’s risk for the condition. Genetic testing can identify a child’s risk of developing cystic fibrosis or becoming a carrier. Tests may also be performed during pregnancy if you have concerns about your baby’s potential risk for cystic fibrosis.
There is a spectrum of severity in cystic fibrosis, and early symptoms may vary for different individuals. In some cases, symptoms first develop early during childhood or infancy, but in others, symptoms appear later in life. Many primary symptoms are respiratory or digestive-related, including:
- A persistent cough, often with thick mucus or blood
- Wheezing or shortness of breath
- Frequent lung or sinus infections
- Poor weight gain during childhood
- Bad-smelling stools
- Loss of appetite
- Abdominal swelling
Another key trait of cystic fibrosis is very salty sweat, since the condition affects how salt moves through the body. If an individual has symptoms of cystic fibrosis, a sweat test, which measures the amount of salt in a person’s sweat, may be used to confirm a diagnosis.
Having an effective treatment plan for your individual needs is a big part of managing life with cystic fibrosis and will improve your comfort and quality of life. Your treatment plan may include airway clearance exercises, medications taken orally or via inhalation, and proper nutrition and fitness.
Airway clearance techniques
Airway clearance techniques use coughing or huffing to loosen the mucus in your lungs. These exercises are performed multiple times each day, often alongside medications like inhaled bronchodilators and antibiotics. A respiratory therapist can demonstrate different airway clearance exercises and help you find the best fit for your needs.
Several types of medications may be used to treat symptoms of cystic fibrosis or related health complications. Bronchodilators relax airway muscles and are available as an inhaler or in a vial for nebulizer use. Inhaled and oral corticosteroids can help reduce inflammation in the airways.
Mucus-thinning medications such as hypertonic saline and dornase alfa (Pulmozyme®) may be used to help you cough up mucus and clear your airways. Nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen are sometimes used to treat airway inflammation. Some research indicates that NSAIDs are most effective in cystic fibrosis treatment for those between ages 6 and 17. Your doctor may also prescribe antibiotics to treat lung infections.
Many people with cystic fibrosis experience gastroesophageal reflux disease (GERD), commonly referred to as heartburn or acid reflux. Proton pump inhibitors including omeprazole (Prilosec®), lansoprazole (Prevacid®), and esomeprazole (Nexium®) and H2 blockers including ranitidine (Zantac®), famotidine (Pepcid®), and nizatidine (Axid®) may also be used to treat this condition.
An over-the-counter medication called polyethylene glycol (MiraLAX®, GoLYTELY®, and NuLYTELY®) can help reduce constipation, a common symptom of cystic fibrosis.
Nutrition and fitness
People with cystic fibrosis have to pay extra attention to what they eat. It isn’t uncommon for individuals with the condition to consume twice as many calories as others of a similar age, weight, and height, since they use more energy to breathe, fight lung infections, and compensate for poor digestion. Your doctor will make recommendations about nutrition and diet tailored to your individual needs, which may include treatments or medications to help you digest food, absorb nutrients, and maintain a healthy weight.
A consistent exercise routine is also an important part of managing cystic fibrosis, as it provides more energy for daily activities and improves lung function. Work with your care team to develop an appropriate fitness routine for your health needs.
Living with a chronic condition like cystic fibrosis affects many aspects of life, so it’s important to build a strong support system and take care of your emotional health. In addition to support from friends, family, and mental health professionals, there are many resources that can help you navigate life with cystic fibrosis and connect with others who share your experiences. As a starting point, explore what these leading cystic fibrosis organizations can offer:
Cystic Fibrosis Lifestyle Foundation
The right pharmacy for your cystic fibrosis treatment
Here at Alto, we make it as easy as possible to follow the treatment your doctor has recommended. We offer free hand-delivery of your medication, automatic savings investigations, and easy medication management tools through our website and app. And our team of pharmacists is always here to answer your questions by phone, text, or chat.
Reach out any time through in-app secure messaging or by phone at 1-800-874-5881 to learn how Alto can support your cystic fibrosis treatment plan.
This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or another qualified health provider with any questions you may have regarding a medical condition.
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