Cystic Fibrosis (CF), Causes, Symptoms, Treatment & Life Expectancy

Management of cystic fibrosis

Doctors don’t have curative treatment for cystic fibrosis. But, a complex and comprehensive management plan can ease the disease symptoms, reduce the life-threatening complications, and improve the quality of life. CF management is a complex issue; it requires a team of doctors from different specialties to manage the various affected systems in one plan.

CF management should include close monitoring and aggressive intervention to slow the disease progression and get longer life.

The management plan should aim to:

  • aid the patient to get rid of the mucous and loosen it
  • protect the lung from infections and control any existing infection
  • correct the malnutrition and provide adequate nutrition
  • prevent intestinal obstruction and treat it if it occurred
  • manage any associated condition

The management plan should also consider infertility, especially if the patient wants to get a child.

To achieve these aims, we have several options; we have medications, airway clearance techniques, pulmonary rehabilitation, surgery, and lifestyle modifications.

Medications

Medications include:

  • Medications that target the mutated genes (CFTR modulators): These medications improve the CFTR protein functions, which improves lung functions and reduces the sweat salt. These medications have several side effects; thus, doctors take some precautions before and during taking them. Doctors will do liver function tests and eye exams to check for liver abnormalities and cataracts. Only take these medications under your doctor’s supervision.
  • Antibiotics to treat and prevent the lung functions
  • Mucous-thinning drugs help the patient to expel (cough) the accumulated mucous and improve lung functions. They include hypertonic saline.
  • Inhaled bronchodilators to open the airways and relax the muscles in their wall
  • Anti-inflammatory drugs to relieve any inflammatory swelling in the airways or pancreas
  • Stool softeners to relieve constipation and prevent intestinal obstruction
  • Oral pancreatic enzymes improve digestive function and compensate the defective pancreatic enzymes. Also, anti-acids enable pancreatic enzymes to work better.
  • Medications for any associated condition, such as diabetes mellitus, osteoporosis, mental health problems, and liver diseases

Airway clearance techniques (chest physical therapy)

These techniques aim to loosen the thick mucous and make it easier to cough; thus, they relieve the mucous obstruction and reduce the risk of lung infections.

Several techniques of chest physical therapy can help the lung in CF. Also, the doctor may recommend a combination of them. These techniques may be manual or use devices.

Your doctor will choose the better technique for you and how often you should do it daily.

Pulmonary rehabilitation

It is a long-term program that improves lung functions and overall health. Your doctor may recommend this program for you; it consists of:

  • Physical exercise to improve your overall condition
  • Nutritional support
  • Breathing techniques to improve your lung performance and get rid of the mucous
  • Psychological support and counseling
  • Education about cystic fibrosis

Surgery

Some manifestations and complications of cystic fibrosis require surgical treatment, as follows:

  • Intestinal obstruction: It is a life-threatening condition that requires urgent surgical intervention to remove the obstructing agent and reconstruct the intestinal tract.
  • Nasal polyps: These polyps interfere with breathing; thus, your doctor may recommend surgery to remove them. Also, your doctor may recommend sinus surgery for recurrent and chronic sinusitis.
  • Liver transplantation: Cystic fibrosis affects the liver, causing failure and cirrhosis. When the liver can’t keep its functions, liver transplantation becomes the option to maintain life. You may also need pancreas transplantation.
  • Lung transplantation: When you can’t breathe and suffer from life-threatening lung complications, and your lung infections don’t respond to antibiotics, lung transplantation will be the option to save your life. In bronchiectasis, you need to replace both lungs.

Cystic fibrosis doesn’t recur after lung transplantation, but the other complications may still occur after it.

Other procedures

Patients with cystic fibrosis may need procedures, such as:

  • Oxygen therapy and (or) noninvasive ventilation: When the blood oxygen declines.
  • Feeding tube: To bypass the poor digestion in cystic fibrosis and get the essential nutrients to keep growing, especially in children.

Written by Martin Davis