Cystic fibrosis (CF) is a chronic condition that affects the digestive and respiratory systems. The extensive damage caused by the disease results in a buildup of mucus in multiple organs, including the lungs, pancreas, liver, and intestines. Cells that produce digestive enzymes, mucus, and sweat are affected by cystic fibrosis.
In a healthy human being, these fluids prevent the organs from getting infected by keeping them lubricated. However, these secreted fluids turn sticky and thick in people with a faulty gene who develop CF. Such fluids start behaving as passageways, tubes, and ducts, posing a threat to the patient’s life.
In this blog post, we will walk you through the detection and management of cystic fibrosis in detail:
Detection of Cystic Fibrosis
The first step of diagnosis is understanding and identifying the symptoms of cystic fibrosis. Let’s look at the respiratory and digestive symptoms below:
- A persistent cough with phlegm
- Shortness of breath
- Recurrent lung infections
- A stuffy nose
- Stuffy sinuses
- Loss of appetite
- A swollen abdomen
- Foul-smelling stools
- Delayed growth in children
A genetic predisposition is responsible for CF. The CFTR gene or the “cystic fibrosis transmembrane conductance regulator” gene is responsible for salt and water movement between cells. A mutation in this gene causes abnormal mucus buildup.
If you have a family history, you should regularly checkup and look for the signs mentioned above. Apart from that, you may need to go through specific diagnostic tests, including:
Immunoreactive Trypsinogen (IRT) Test
The immunoreactive trypsinogen (IRT) test checks for abnormal protein levels in the blood called IRT to diagnose cystic fibrosis. Higher than normal levels of IRT indicates cystic fibrosis. It is further confirmed through more tests.
Sweat Chloride Test
It’s a common test used for the detection of cystic fibrosis. In this diagnostic method, the doctor checks if your sweat has increased salt levels. A chemical and a weak electric current are used to make a person sweat, which is then analyzed for salt levels.
A combination of X-rays is used to create images of the body’s internal structures, such as the pancreas and liver. CT scan also allows the doctor to evaluate the extent of organ damage.
Pulmonary Function Tests (PFTs)
Testing for cystic fibrosis includes determining how well your lungs are functioning. Patients are evaluated for their ability to inhale or exhale properly. Apart from that, their lungs are checked whether they can effectively transport oxygen.
How to Manage Cystic Fibrosis?
Doctors may prescribe various antibiotics, mucus-thinning medications, nonsteroidal anti-inflammatory drugs (NSAIDs), bronchodilators, CFTR modulators. Patients must never take these medications without a doctor’s advice.
Apart from that, patients suffering from cystic fibrosis may need bowel surgery, feeding tube, or double-lung transplant depending on their condition. It should be noted that transplant is not the cure since the patient’s body continues to have the defective gene.
Besides, CF patients may benefit from:
Chest Physical Therapy
Management of cystic fibrosis includes chest physical therapy. It’s a technique used to loosen the mucus inside the patient’s lungs. Seek the advice of your care team to learn exactly how to do it.
Usually, you have to lie down at the edge of your bed. Following which you have to clap the sides of your chest with cupped hands. This technique should be repeated 1-4 times a day.
Besides, you can also buy mechanical devices such as a chest clapper or an inflatable vest.
People with CF find it difficult to absorb nutrients due to mucus buildup in their intestines. For this reason, they may need to consume more calories than an average person. Besides, doctors may recommend multivitamins, antacids, and pancreatic enzyme capsules as well.
For the effective management of cystic fibrosis, you should:
- Drink plenty of fluids
- Exercise regularly – swimming, biking, walking
- Avoid mold, pollen, smoke, and other irritants
- Get vaccinated for pneumonia and influenza
AdvantagePlusNurses offers chronic care management for seniors where our team makes a detailed care plan, monitors the patient every day, and intervenes in case of emergency.