Cystic Fibrosis (CF) is a genetic disorder that affects the digestive system, respiratory system, and various other parts of your body. The organs most frequently impacted by Cystic Fibrosis are the lungs, stomach, pancreas, and intestines. Thick, viscous mucus accumulation in the organs is often among the most common Cystic Fibrosis causes, and this disease can affect children, as well as adults. 

Cystic Fibrosis may be managed with regular, everyday care, but there is no standard treatment option that works for everyone. However, treatment has improved with recent medical innovations, and even the children affected by severe CF now live longer. Let’s take a look at the effective medicines that work wonderfully on CF patients.

What is Cystic Fibrosis?

Cystic Fibrosis is a hereditary condition that causes thick, sticky mucus to accumulate in your organs and harm them. Some consider CF to be a lung disease because it affects the lungs and airways, and causes breathing difficulties, however, the reason it's termed Cystic Fibrosis is that it also results in pancreatic cysts and scarring. Children and adults with this disease often become weak because it can be difficult to obtain nutrients from the digestive tract due to blockages caused by this thick mucus. 

The accumulation of sticky mucus in the lungs raises the risk of lung infections, makes breathing difficult, and the lungs may eventually cease to function properly. Moreover, mucus blocks the pancreas, an organ that aids in digestion, and this prevents digestive enzymes from reaching the stomach. As a result of this problem, most Cystic Fibrosis patients require higher calorie intake to prevent malnutrition since they are unable to efficiently absorb nutrients from meals.

Cystic Fibrosis causes gene mutations, and that’s why patients with Cystic Fibrosis have low amounts of certain proteins or malfunctioning proteins in their body. These malfunctioning proteins generate minerals that trap mucus within cells, and make it viscous and thick. Cystic Fibrosis is a congenital condition that worsens over time, and most CF patients don't live as long as those without the disease.

Symptoms of Cystic Fibrosis

The most common Cystic Fibrosis symptoms include chronic chest infections, breathing difficulties, coughing, wheezing, airway damage, jaundice, and weight gain. A variety of other illnesses, such as diabetes, weak bones (osteoporosis), and liver issues, can also occur in people with the disorder. 

Medications for Cystic Fibrosis Treatment  

Cystic Fibrosis treatment can help a patient manage the condition and its symptoms, and improve quality of life.  

1. CFTR Modulators for Cystic Fibrosis

CFTR modulators are drugs that act on specific imitation to help enhance the quantity of functional proteins on the surface of your cells and fix problems with proteins produced by mutant CFTR genes. CFTR modulators cannot cure CF, however, there have been notable improvements in some patient’s symptoms and life expectancy by using these medications. Kalydeco, Orkambi, Symdeko, and Trikafta are the most commonly used CFTR modulators. 

2. Other Medications for Cystic Fibrosis

Your doctor can recommend drugs to treat infections, lessen inflammation, and control the other symptoms of the disease. 

• Antibiotics - Antibiotics may be prescribed by your healthcare practitioner to treat or prevent infections.
• Inhaled bronchodilators - Breathing becomes easier with bronchodilators because they relax and widen your airways.
• Anti-inflammatory drugs - These include nonsteroidal anti-inflammatory medications (NSAIDS) and corticosteroids that lessen edema.  
• Pancreatic enzymes - These aid with food digestion and nutrient absorption to make up for the calorie deficit caused by CF.
• Inhaled hypertonic saline - Saline solutions' salt draws water to it, thins mucus, and facilitates mucus clearing. 

Other Treatments for Cystic Fibrosis

1.Airway Clearance Techniques for Cystic Fibrosis

If you have Cystic Fibrosis, you may follow this Cystic Fibrosis treatment to maintain clean airways.

• Coughing and breathing techniques - You can learn methods to expand your airways and release mucus from a physical therapist who specializes in treating cystic fibrosis.
• Positive expiratory pressure (PEP) - PEP devices are worn on the face or in the mouth like a mask, and they make it difficult for you to exhale, pushing mucus out of your airways when you do so. Certain PEP models, such as Flutter, Acapella, and AerobikA, oscillate to help release mucus. 
• Airway clearance vests - An inflated vest that fastens to a machine is known as an airway clearing vest or high-frequency chest wall oscillation device, and they help release mucus through vibration. 
• Postural drainage and percussion - In this kind of physical therapy, you adopt specific postures to allow your lungs to empty, and to assist in releasing the mucus, another person claps their hand on your back, and chest.  

2. Surgeries for Cystic Fibrosis

Numerous bodily areas are impacted by CF, and your physician could advise surgery to address specific CF issues. You need to remember that complications with CF surgery can include infections, hemorrhage, breathing issues, and organ rejection and infections in the case of transplant surgeries.  

• Lung transplant

You could be a candidate for a lung transplant if your lung function has significantly decreased, you have lung problems that could be fatal, or your medications are no longer effective for treating lung infections. After a lung transplant, the new lungs won't have cystic fibrosis issues, but post-lung transplant problems, such as infections, diabetes, and pancreatitis, can still happen.

• Liver transplant

A liver transplant may be advised for cases of severe cirrhosis or other liver diseases linked to CF, and sometimes, a liver transplant may be paired with a pancreatic or lung transplant in certain individuals. 

• Nasal and sinus surgery

During this treatment, nasal polyps (growths) that impede breathing can be removed, and you may also need sinus surgery if you get sinusitis frequently.

• Feeding tube placement

CF impedes the breakdown and absorption of nutrients from meals, even when pancreatic enzymes are used, and maintaining weight may be challenging as a result. Any supplement that your doctor recommends can be given through a feeding tube to give you additional calories and nourishment. This tube can be surgically placed in the belly, and it won't prevent the patient from eating with their mouth. 

Final Remarks

The genetic condition known as Cystic Fibrosis causes an accumulation of viscous mucus in the organs that can lead to permanent harm to the body. The severity of this disease can vary from patient to patient, and the treatment options vary as well depending on the extent of the symptoms. Some patients are diagnosed with CF in their childhood, but many others get diagnosed in their teens or even as adults. Remember that this is a very serious disease, and regular treatment under the supervision of a doctor is extremely important.