A patient’s treatment option for cystic fibrosis lies on many factors, such as how severe the disease is and what symptoms and complications arose.
Person’s living with cystic fibrosis usually need a medical care team to manage the complex disease’s various physical and emotional effects.
Other than 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)
Aims of Cystic Fibrosis Treatment
Cystic fibrosis is not curable condition. But as treatments for the disease continue to get better, so does life expectancy for people who are affected by 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 adjustments to surgery.
The aims 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
What Medications Are Given for Cystic Fibrosis?
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 drugs below help people with specific genetic mutations:
- Kalydeco (ivacaftor)
- Orkambi (combining lumacaftor and ivacaftor)
- Symdeko (combining ivacaftor and tezacaftor) (3,4,5)
What Therapies and Devices Help Cystic Fibrosis?
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.
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).
Besides a primary care physician, a patient’s medical team may include a pulmonologist, respiratory and physical therapists, a dietitian, and a psychologist.
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.
Surgeries and Other Procedures for Cystic Fibrosis
Surgeries and other therapies are needed to treat complications associated with cystic fibrosis. These may include:
Your doctor may recommend supplementary oxygen if your blood-oxygen level declines.
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.
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. (7)
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.
Cystic Fibrosis and Diet
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.