Cystic Fibrosis Treatment

A patient’s treatment plan for cystic fibrosis depends on several factors, such as the severity of the disease and what symptoms and complications develop.

People with cystic fibrosis usually need a medical care team to manage  the complex disease’s various physical and emotional effects.

Besides a primary care physician, a patient’s multidisciplinary team may include a pulmonologist, respiratory and physical therapists, dietitian, and psychologist. Depending on his or her symptoms, the patient may also need to consult physician specialists such as an otolaryngologist (ear, nose, and throat doctor), a gastroenterologist, and an endocrinologist. (1)

Cystic fibrosis is not curable. But as treatments for the disease continue to improve, so does life expectancy for people who have it. Today, people with cystic fibrosis are living into their 40s, 50s, and longer. (2)

Treatments for cystic fibrosis and its complications range from nutritional and respiratory therapies to medications and lifestyle changes to surgery.

The goals of cystic fibrosis treatment include:

• Treating and preventing lung infections
• Loosening and removing mucus from the lungs
• Maintaining lung function
• Treating and preventing blockages in the intestines
• Providing nutrition and preventing dehydration

Medications for cystic fibrosis may be inhaled, swallowed, or injected.

Some drugs that are widely used to treat cystic fibrosis include:

• Antibiotics to treat and prevent lung infections
• Bronchodilators to keep airways open
• Mucus thinners to help you expel mucus
• Anti-inflammatories to reduce swelling and open up airways

Three cystic fibrosis drugs approved by the Food and Drug Administration (FDA) correct the function of the defective protein produced by the cystic fibrosis gene. These drugs are known as cystic fibrosis transmembrane conductance regulator (CFTR) modulators.

These following drugs help people with specific genetic mutations:

• ivacaftor (Kalydeco)
• ivacaftor and lumacaftor (Orkambi)
• ivacaftor and tezacaftor (Symdeko) (3, 4,5)
• elexacaftor, ivacaftor, and texacaftor (Trikafta)

Some forms of therapy used to treat cystic fibrosis include:

Chest Physical Therapy (CPT)

This airway clearance technique (ACT) loosens thick mucus in the lungs so it can be coughed or huffed out of the body.

The patient lies or sits in a position to encourage drainage of mucus from the lungs (postural drainage).

Therapy is done by manually clapping on the back, chest, and areas under the arms (percussion) with a cupped hand. CPT can also be achieved using a flattened hand to create a shaking motion (vibration). (6)

Devices such as an electric chest clapper, a vibrating vest, or a specialized mask can assist in the performance of CPT.

Airway Clearance Devices

There are several devices available to improve airway clearance in people with cystic fibrosis. Patients can use these devices independently without the need of a healthcare professional or caregiver, as opposed to manually administered chest physical therapy.

These include positive expiratory pressure (PEP) devices, in which the patient breathes through a mask or handheld mouthpiece; and oscillatory devices, which combine using resistance when breathing out with vibrations to move mucus from the lungs.

Pulmonary Rehab

Designed to improve your lung function, pulmonary rehab usually includes exercise, breathing techniques, nutritional guidance, education, and counseling.

This medically supervised approach is used to help people with other conditions as well as cystic fibrosis, including chronic obstructive pulmonary disease (COPD). (7)

Surgeries and other therapies are needed to treat complications associated with cystic fibrosis. These may include:

Oxygen Therapy

Your doctor may recommend supplementary oxygen if your blood-oxygen level declines.

Feeding Tube

You may need a temporary feeding tube if your body is not adequately absorbing nutrients from your food.

Nasal Polyp Surgery

This procedure removes nasal polyps that may affect your breathing.

Endoscopy and Lavage

This treatment involves suctioning mucus from your airways through a tube called an endoscope.

Lung Transplant

You may need to receive healthy donor lungs if you have severe breathing problems or complications from your disease. Lung transplantation is used for other conditions, such as emphysema, but only patients with severe lung disease are candidates for the surgery.

Bowel Surgery

Constipation and intestinal blockages, known as meconium ileus in newborns or distal intestinal obstructive syndrome (DIOS) in adolescents and adults, are common cystic fibrosis complications. In cases where the condition does not respond to medications, surgical intervention may be required. (8)

The pancreas produces enzymes that help the body digest and absorb protein and fats. In people with cystic fibrosis, mucus buildup in the pancreas can impair the ability to get enough dietary protein, fat, and calories; and that can lead to malnutrition.

To help the body absorb more protein and calories, people with cystic fibrosis often have to take pancreatic enzymes. Supplements of vitamins A, D, E, and K can also help. (9)