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Cystic Fibrosis
Treatment

Cystic Fibrosis Treatment

Because cystic fibrosis affects several body organs, people are treated by a multidisciplinary team that is experienced in cystic fibrosis care. The core team is comprised of the following disciplines:

  • Pediatrician or internist
  • Dietician
  • Respiratory therapist
  • Social worker

Additional team members may include:

  • Exercise physiologist
  • Gastroenterologist (digestive problems)
  • Genetic counselor
  • Physical therapist
  • Pulmonary and respiratory therapists
  • Pulmonologist (lung and respiratory problems)

Specialists may be needed for people who develop cystic fibrosis-related diabetes, or require a lung or liver transplant.

Early diagnosis and treatment can significantly improve the survival and quality of life for people with cystic fibrosis. Common cystic fibrosis treatments include:


Prescriptions

Prescription medications for cystic fibrosis help:

  • Fight infections
  • Improve breathing
  • Maintain sufficient nutrition
  • Prevent intestinal blockages

Prescription medications include:

  • Antibiotics
  • Assorted respiratory medications
  • Bronchodilators
  • Mucus thinners
  • Respiratory medications
  • Vaccinations
  • Pancreatic enzymes

It's important for people to ask their healthcare provider or pharmacist in advance for possible drug side effects or interactions.

The following list of medications isn't inclusive. Additional information, side effects, and drug interactions can be obtained by clicking the name of highlighted medications.

Antibiotics

People with cystic fibrosis often develop bacterial lung infections. Antibiotics destroy or inhibit the growth of bacteria, and have been shown to improve lung function and delay lung deterioration.

Antibiotics are available in the following forms:

  • Oral (pill or solution)
  • Aerosol (inhaled mist)
  • Intravenous (into a vein for severe cases)

The choice of antibiotics depends on the strain of bacteria causing the infection. Common bacteria infecting the lungs of people with cystic fibrosis include:

  • Staphylococcus aureus (variety of infections)
  • Haemophilus influenzae (acute infections)
  • Pseudomonas aeruginosa (most of the respiratory infections in people with cystic fibrosis)

People with cystic fibrosis usually take antibiotics for long periods of time and at high doses. Although oral and aerosol antibiotics can treat mild infections, severe infections may require prolonged therapy, hospitalization, or intravenous treatment. Because long-term use of antibiotics may result in drug-resistant bacteria, the antibiotic chosen also depends on a person's history of antibiotic use.

Common antibiotics include:

  • azithromycin (Zithromax)
    Azithromycin treats Pseudomonas aeruginosa. It is not advisable for children.
  • cephalexin (Keflex).
    Cephalexin treats Staphylococcus aureus. It is not advisable for people allergic to penicillin.
  • ciprofloxacin (Cipro)
    Ciprofloxacin treats Staphylococcus aureus. It is not advisable for children.
  • tobramycin (TOBI)
    Tobramycin treats Pseudomonas aeruginosa. The medication is inhaled into the lungs using a nebulizer (delivers medication in mist form to a person's airways). Tobramycin isn't advisable for people with impaired hearing, impaired kidney or neuromuscular function, or pregnant women.

Bronchodilators

Bronchodilators help widen air passages in the lungs and make it easier for people to breathe. Bronchodilators can be inhaled or taken by mouth.

People inhale bronchodilators in aerosol form using a nebulizer (delivers the medication to the deep part of the lungs) or metered dose inhaler (delivers specific amounts of the medication to the lungs).

  • albuterol (Proventil, Ventolin)
    Albuterol dilates airways in the lungs by relaxing the surrounding muscles. Albuterol is taken by mouth as a tablet (regular or extended-release) or syrup. It can also be inhaled in aerosol form.

Mucus thinners

Mucus thinners work by reducing the quantity and thickness of mucus, making the mucus easier to cough out. Mucus thinners also have been shown to reduce the number of lung infections and improve airflow in people with cystic fibrosis.

  • dornase alfa (Pulmozyme)
    Dornase alfa is inhaled into the lungs using a nebulizer. Bacteria attacked by the immune system release DNA, which thickens the mucus in the lungs and makes it difficult for people to breathe. Dornase alfais a synthetic form of the enzyme DNase, which breaks down DNA in the mucus. This action thins the mucus and makes it easier for people to cough it out.

Assorted respiratory medications

The following prescription medications help relieve respiratory symptoms.

  • Cough suppressants prevent or stop coughs.
  • Expectorants thin and loosen thick mucus which makes it easier to cough up.
  • Nasal sprays relieve nasal congestion. Nasal sprays include fluticasone (Flonase).

Vaccinations

Pneumonococcal and annual influenza vaccinations help protect people from developing pneumonia or catching the flu. Killed vaccines are advisable for people with cystic fibrosis due to the possibility of developing a secondary infection when live vaccines are used.

Pancreatic Enzymes

Pancreatic enzyme products (PEP) help people with cystic fibrosis break down and digest the fats, carbohydrates, and proteins in their food.

The Food and Drug Adminstriction (FDA) requires all pancreatic insufficiency products to be available by prescription only. In April 2004, the FDA required that PEPs get FDA approval (prove the safety and effectiveness of the medications) within the next four years. Until then, people can continue to use their current prescribed pancreatic enzymes.

New treatment guidelines

In 2007 the Cystic Fibrosis Foundation developed new cystic fibrosis treatment standards. Due to the limited studies on treating younger children, the standards only apply to people age 6 and older.

The treatment recommendations with the strongest, most consistent results are:

  • Inhaled tobramycin to suppress chronic Pseudomonas aeruginosa infections in people with moderate to severe cystic fibrosis
  • Dornase alfa to improve lung function and reduce exacerbations

Other recommendations include:

  • Inhaled tobramycin to suppress chronic Pseudomonas aeruginosa infections in people with mild cystic fibrosis or who are asymptomatic
  • Dornase alfa to improve lung function and reduce exacerbations in people with mild cystic fibrosis or who are asymptomatic
  • Hypertonic saline to improve lung function and reduce exacerbations
  • Beta 2-adrenergic receptor agonists to improve lung function

The guidelines recommend against:

  • Systemic corticosteroids for children
  • Inhaled corticosteroids
  • Prophylactic anti-Staphylococcal antibiotics
  • All other treatments that lack sufficient evidence to recommend for or against their use

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Over-the-Counter Medications

Several over-the-counter medications help relieve cystic fibrosis symptoms. People who take over-the-counter medications in combination with prescription medications should check with a healthcare provider for possible side effects and drug interactions.

Assorted respiratory medications

Several over-the-counter respiratory medications are available.

  • Cough suppressants include dextromethorphan (Robitussin).
  • Expectorants include guaifenesin (Mucinex).
  • Nasal sprays include oxymetazoline hydrochloride (Afrin). To avoid "rebound congestion," nasal sprays should be taken no longer than three consecutive days. Saline nasal sprays are less likely to cause rebound congestion.

Pain relievers

Over-the-counter or prescription ibuprofen (Motrin, Advil) may slow lung damage in some children with cystic fibrosis by reducing inflammation (swelling) in the lungs.

Stool softeners and enemas

Stool softeners and enemas help people prevent or clear intestinal blockages. Enemas are intended for occasional use. Stool softener products include docusate sodium(Colace). Enema products include bisacodyl (Ducolax, Fleet Bisacodyl).

Nutritional supplementation

The following supplements help replace nutrients that aren't absorbed from food:

  • Fat soluble vitamins (A, D, E, K)
  • High-calorie, high-fat foods
  • Nutritional drinks

Oxygen Therapy

People with low blood oxygen levels may require supplemental oxygen, which increases oxygen levels throughout the body. A nasal cannula (breathing device with tubes that extend into a person's nostrils) or face mask provides oxygen to the lungs.

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Non-Drug Therapies

Non-drug therapies used in addition to medications help improve cystic fibrosis symptoms. Common non-drug therapies for cystic fibrosis symptoms include:

Chest Physiotherapy

Chest physiotherapy is designed to loosen mucus in the lungs, making it easier for people to cough out. Popular techniques include:

  • Postural drainage
    People position their bodies so their head and windpipe are both leaning downward, and below their chest. In this position, gravity is now working with them instead of against them as they attempt to drain their lungs of the excess mucus.
  • Manual or mechanical chest clapping
    An assistant or an electric chest clapper (mechanical percussor) claps on a person's chest to loosen the mucus.
  • High frequency chest compression vest
    An inflatable vest connected to a pulse generator vibrates the chest to loosen the mucus.
  • Positive expiratory pressure (PEP) mask
    A PEP mask helps keep the airways open while a person breathes in and out. The mask is often used with bronchodilators.
  • Flutter device
    A person breathes in and out of the flutter device, which creates vibrations that help loosen the mucus.
  • Breathing techniques
    People are trained in series of specialized breathing techniques that help loosen the mucus.

Counseling

Counseling helps people with cystic fibrosis understand their abilities and limitations, reduce their emotional stress, and learn to feel good about themselves and their lives. Support groups, counseling, and education about cystic fibrosis can be very helpful both for people who have the disease and their families. Counseling is especially helpful for children and teenagers who have cystic fibrosis.

Occupational therapy

Occupational therapy helps improve productivity and reduce fatigue, enabling people with cystic fibrosis to continue working as long as possible.

Surgery

The following surgical procedures may be performed to treat cystic fibrosis complications:

  • Respiratory repair procedures
    Chronic lung infections may require surgery to repair any bleeding or restricted areas. Surgery is also needed for pneumothorax (air in the cavity surrounding the lungs).
  • Lung transplantation
    Replacing a diseased lung with a healthy donor lung may be the only option for people with severe breathing problems or when antibiotic resistance threatens a person's life. Because both lungs are affected by cystic fibrosis, they both need to be replaced. People with small chests often receive the lower lobes donated by two living donors.
    Lung transplantation is not a cure since the defective genes are present in all of the cells of the body. The complications of lung transplantation include post-surgical infection, organ rejection, and the need to take anti-rejection medications for life.
  • Nasal polyp surgery
    Nasal polyps are growths that develop in the nasal passages. Nasal polyps that interfere with breathing may have to be removed surgically.
  • Intestinal repair
    Surgery may be needed to repair intestinal obstructions, intussusception (one part of the intestine slides into another), or rectal prolapse (the rectum protrudes through the anus).
  • Feeding tubes
    Tube feeding helps people get the nutrients they need while they sleep. The tube goes into the stomach either through the nose (nasogastric tube) or skin of the upper abdomen (gastrostomy tube). A minor surgical procedure is required to insert the gastrostomy tube and remove it when it's no longer needed. Although tube feeding might seem drastic, the health benefits are often dramatic.
  • Blood transfusions
    People who lose large quantities of blood due to excess coughing or blood in the stool may require blood transfusions.
  • In vitro fertilization (IVF)
    IVF helps couples with cystic fibrosis who have trouble conceiving. The female's eggs are surgically removed and mixed with the male's sperm in a laboratory dish. Fertilization typically takes place within a few days. The fertilized eggs are then placed in the female's uterus where they continue to develop.

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Complementary and Alternative Medicine

Complementary and alternative medicine refers to those medical and healthcare treatments that are not part of traditional medicine. Whereas, complementary medicine is used with traditional medicine, alternative medicine is used in place of traditional medicine.

Some people try the following complementary and alternative medicine therapies to reduce their cystic fibrosis symptoms:

Herbs

The following herbs are believed to help people with cystic fibrosis fight infection:

Massage Therapy

Therapeutic massage helps drain mucus from the lungs.

Supplements

  • Beta-carotene is believed to help prevent lung infections.
  • Fat-soluble vitamins (A, D, E, and K) help replace lost vitamins.
  • L-arginine may aid the immune system of people with cystic fibrosis and is believed to prevent colds.
  • Omega-3 fatty acids (EPA and DHA from fish oils) may help fight inflammation caused by cystic fibrosis.
  • Probiotics contain live microorganisms that are believed to have health benefits. Lactobacillus acidophilus, bifidobacterium, and other probiotics may help gastrointestinal problems caused by cystic fibrosis.

Some of these therapies haven't been well studied, and little information is available about their effectiveness, side effects, and drug interactions. It's important to discuss any of these therapies with your doctor before trying them. 

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Lifestyle Changes

The following lifestyle changes can help relieve some of the symptoms of cystic fibrosis:

Exercise

Regular aerobic exercise helps people with cystic fibrosis:

  • Build and strengthen muscles to aid mucus clearing.
  • Decrease shortness of breath.
  • Improve a person's physical well being.
  • Keep the airways open.
  • Move air deep into the lungs.

Hand washing

Frequent hand washing with soap and water removes bacteria and other microorganisms from the skin. Alcohol-based hand sanitizers are useful when soap and water aren't available.

Healthy diet

People with cystic fibrosis should eat healthy meals in addition to taking supplemental pancreatic enzymes and vitamins. Children are advised to eat high-calorie, high-fat diets to make up for the nutrients lost during digestion. To avoid dehydration, it's important to drink plenty of fluids, especially during hot weather or when symptoms worsen. Adults with chronic pancreatitis (inflammation of the pancreas) may develop diabetes.

Palliative care

There is no cure for cystic fibrosis. Eventually a person's condition worsens to the point where palliative care is required. The goal of palliative care is to relieve the symptoms of a disease without curing it. It helps people manage their emotions, as well as their symptoms and side effects. Palliative care also helps people manage and plan their finances.