Cystic Fibrosis- How to deal with it? try 5 Various treatments

Cystic Fibrosis

Overview

Cystic fibrosis occurs in children and adults and causes progressive organ damage, including the lungs, digestive system, and other organs. As a result, the lungs become infected and breathing becomes difficult. The cells that produce mucus, sweat, and digestion juices are affected by this. However, Cystic Fibrosis patients suffer from a defective gene that causes the secretions to become sticky and thick. The secreted fluids are typically thin and slippery.

Cystic Fibrosis Symptoms and severity can vary. In some cases, the disease does not appear until teens or young adults are in the prime of their lives. There can be few symptoms at the beginning, but later more symptoms may appear. As part of the lungs, the mucus clogs the airways and traps bacteria. As a result, infections, inflammation, respiratory failure, and other complications occur. As part of the pancreas, the mucus buildup can prevent the release of digestive enzymes, which is essential for absorbing nutrients and food. This leads to malnutrition and slow growth.

The diagnosis of cystic fibrosis is made through a variety of tests, including blood, urine, and sweat tests. Cystic fibrosis is incurable, but treatments have dramatically improved in recent years. Cystic Fibrosis is a progressive disease that requires daily care. However, most patients are able to work and attend school. The quality of life for people with cystic fibrosis today is often better than it was in previous decades. Children and teenagers were the most frequently affected by cystic fibrosis in the past. With improved treatments, some cystic fibrosis patients are now living well into their fifties and sixties. Various types of treatments, such as chest physical therapy, nutrition, respiratory treatments, medicines, and exercise, can be used.

Symptoms and signs

A person’s symptoms and signs of cystic fibrosis vary depending on how severe the disease is. It is possible for a person’s symptoms to worsen or improve as time goes by. Symptoms may not start until a person reaches their teenage years or early adulthood. If the disease is not discovered until adulthood, it has milder symptoms and the patient may also experience atypical symptoms, such as recurring episodes of pancreatitis, infertility, and recurring pneumonia.

Sweat from people with cystic fibrosis contains more salt than usual. Sometimes parents can taste the salt in their children’s kisses. The respiratory system and the digestive system are usually affected by the other symptoms of cystic fibrosis.

There are several symptoms associated with cystic fibrosis, including:

• Skin with a very salty taste

• A persistent cough, often accompanied by phlegm

• Infections of the lungs, including pneumonia and bronchitis

• Breathing problems or wheezing

• Even though you have a good appetite, you do not gain weight or grow

• Constipation or greasy, bulky stools frequently

• Fertility problems in men

• Having recurrent sinusitis
• Blockages in the intestines, especially in newborns (meconium ileus)
• Constipation of such a severity that frequent straining during bowel movements can eventually result in part of the rectum sticking out of the anus 

Causes

Genes are involved in the development of cystic fibrosis. Gene defects can take many forms. The severity of the condition is determined by the type of gene mutation. A child must inherit one copy of the gene from each parent in order to have the condition. Cystic fibrosis won’t develop in children who inherit one copy. Yet, they will still be carriers, and their children could carry the gene too.

Complications

There are several organs that are affected by cystic fibrosis, including the respiratory system, digestive tract, and reproductive system. The following may be included:

• Damaged airways
• Chronic infections
• nasal polyps
• Hemoptysis
• Pneumothorax
• Respiratory failure
• Acute exacerbations
• Nutritional deficiencies
• Diabetes
• Liver disease
• Intestinal obstruction
• Distal intestinal obstruction syndrome
• Infertility in men
• Reduced fertility in women
• Osteoporosis
• Electrolyte imbalances and dehydration
• Mental health problems

When to see a doctor

Discuss cystic fibrosis testing with your doctor if you or someone in your family has symptoms of the disease. Consult a physician who is familiar with cystic fibrosis. If you suffer from cystic fibrosis, follow up with your doctor regularly, at least every three months. If you notice any changes or worsening symptoms, such as more mucus than usual, a change in the color of the mucus, or loss of weight or constipation, contact your doctor. If you’re coughing up blood, experiencing chest pain or difficulty breathing, or feeling severely sick, seek medical attention immediately.

Various treatments

Individuals with cystic fibrosis are likely to experience different types and severity of symptoms. Since treatment plans contain many similar components, they are tailored to a specific individual’s needs.

Patients with CF undergo the following treatments every day:

1. Clearing the airways can help loosen and eliminate thick mucus that accumulates in the lungs.

2. A medicine is inhaled to open the airways, thin mucus, or reduce congestion. A mist or aerosol of liquid medicine is inhaled through a nebulizer, which includes antibiotics that fight lung infections and therapies that help keep the airways open.

3. A pancreatic enzyme capsule that improves nutrient absorption. Almost all meals and snacks include these supplements. Multivitamins are also usually taken by people with CF.

4. A fitness program designed to boost energy, lung function, and overall health.

Modulators of the cystic fibrosis transmembrane conductance regulator (CFTR).In order to target the underlying defect within the CFTR protein, As different mutations cause distinct defects in the protein, medications that have so far been developed are only effective in patients who have certain mutations.

Prevention

Before having children, you and your partner may choose to undergo genetic testing if you or your partner have close relatives with cystic fibrosis. This blood test, which is performed in a lab on a sample of blood, can help determine your risk of having a child with CF. If you are already pregnant, you can have an additional genetic test performed on your child if the genetic test indicates that the baby is at risk of cystic fibrosis. Genetic testing may not be for everyone. A genetic counselor can advise you about the psychological implications of the test results before you decide to undergo testing.