Cystic Fibrosis Treatment In Mumbai

Cystic fibrosis disease is a chronic and inherited condition that affects the lungs and digestive system. This causes severe respiratory and nutritional challenges due to the accumulation of sticky mucus that clogs the airways, making breathing difficult. This also increases the risk of lung infections. The condition has significantly reduced the quality of life and, in severe cases - shortened life expectancy.

The condition has also led to daily challenges such as - frequent hospital visits, difficulty in gaining weight, and persistent coughing. However, with the advancement in cystic fibrosis medication, the symptoms can be managed effectively. However, it is important to note that the condition has no cure. Early diagnosis and checkups can help slow disease progression. Also, a diet change, exercise, and infection prevention measures such as vaccination and proper hygiene maintenance can help combat the condition. 

Cystic fibrosis (CF) is a disease that is passed down in families and causes damage to the lungs, in addition to the digestive system and other organs in the body. It is known as cystic fibrosis because it causes cysts and fibrosis (also known as scarring) in the pancreas. The condition affects the cells that make sweat, mucus, and digestive juices. These fluids, often known as secretion, protect the body’s internal organs, tubes, and ducts. It also affects the liver, sinuses, intestines, and sex organs. Researchers are still studying the cystic fibrosis cure.

There are two types of cystic fibrosis: 

• Classic Cystic Fibrosis - It often affects multiple organs and is usually diagnosed in the first few years of life.
• Atypical Cystic Fibrosis - It is a milder form of CF, which only affects one organ, and the symptoms may fluctuate.

CF is caused by a mutation in the CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) gene - called variants or mutations. This gene makes a protein that works an ion channel on the cell surface. 

It is important to note that a cystic fibrosis cure is not available as of yet. However, doctors suggest certain plans to manage cystic fibrosis. It is treated after conducting a range of tests with a few drops of blood from the newborn’s heel immediately after birth and a few weeks later. The test looks for immunoreactive trypsinogen (IRT) - a chemical made in the pancreas. Individuals with high cystic fibrosis have a higher IRT level in their blood.

Sometimes, most newborn screening tests do not detect increased CF levels. So, if the child or the mother shows symptoms of CF, a healthcare provider usually performs sweat tests and follows up with additional tests, such as - pancreatic biopsy, sputum culture, imaging, genetic tests, nasal potential difference, etc. 
Here are a few cystic fibrosis management plans as advised by our specialists at Gleneagles Hospitals:

• Keeping the airways clear and open using breathing techniques to loosen mucus
• Use of medication that helps manage issues with CFTR proteins
• Medications to reduce certain symptoms
• Having proper calorie food

Suppose the issue persists even after following management protocol and cystic fibrosis medication. In that case, surgery might be needed for a liver and lung transplant, in addition to surgery on the nose or sinuses and bowel surgery to remove blockages. Also, researchers are exploring ways to deliver a healthy CFTR gene into cells to replace the faulty ones. Moreover, genetic editing (CRISPR-Cas9) is also done to correct the CFTR gene mutation at the DNA level. Though research is still ongoing, they have shown some promising results, making them viable treatment options for cystic fibrosis. 

Cystic fibrosis treatment usually does not require any preparation unless surgery is recommended. This is done in case of severe damage to the lungs and other organs. It is important to adhere to the planning and medical guidelines. Here’s how to prepare for transplant surgery:

• The patient needs to undergo a series of diagnostic tests, including - imaging scans, lung function tests, and blood work.
• After the test, the patient is placed on a waitlist till the healthcare provider finds a donor.
• During the waiting period, the doctors may recommend pulmonary rehabilitation, a high-calorie diet, and strategies to prevent infection.
• The patient is prescribed certain medications to manage symptoms.

It is important to follow the guidelines as provided by the doctors - this helps minimise the discomfort and increase the chance of success. 

Before the surgery begins, the healthcare provider will conduct some tests such as - blood pressure, blood tests, cardiovascular examination, checking fluid balance, etc. Once done, the patient will be given an IV (intravenous injection) to administer anaesthesia and other required medication. Next:

• Surgeons make an incision in the chest to access the damaged lung to remove it.
• Healthy donor longs are placed and connected to the patient’s airways.
• Then, the blood vessels and bronchi are attached to the new lungs to restore proper oxygen flow.
• Once the lung is connected to the body, the chest is closed using stitches and a sterile bandage is wrapped over it.
• The patient is moved to an ICU (Intensive Care Unit) for monitoring, and one or more drainage tubes will be placed in the chest to let excess fluid, air, and blood flow out from the surgical site.
• They are connected to a ventilation system that breathes for them until the new lung functions properly.

After the surgery, some pain and discomfort are expected. Doctors prescribe medications required to help relieve pain through an IV. Immunosuppressive medications are also provided to prevent the body from rejecting the transplanted lungs. The ventilation is then removed after a few days, and the doctors will gradually transition the patient to eating solids - depending on the patient's current condition.

In addition, nurses and physical therapists also help prepare the patient to move out of the ICU with the help of therapy. Once the doctors are confident enough, they discharge the patient and advise on management of how to take care of the surgery and recover properly. Certain challenges arise that may vary depending on the patient and are managed and taken care of by the specialists. 

Here are some risks and complications that are caused after cystic fibrosis lung transplant surgery:

• Bleeding
• Infections
• Blood clots
• Organ rejection
• Respiratory insufficiency

There is no way to prevent cystic fibrosis. However, there are certain ways to manage the risks - 

• Genetic counseling helps to understand if there is a CF condition in the family.
• Carrier genetic testing can help identify the CFTR gene mutations before pregnancy.

While the disease cannot be avoided, early diagnosis through infant screening can help in timely Cystic Fibrosis Treatment in Mumbai. This improves quality of life through proper medical care, a healthy lifestyle, infection prevention strategies, etc.

Gleneagles Hospital is a leading facility for a Cystic Fibrosis Treatment in Mumbai because of the advanced technologies used to identify CFTR gene mutation. With a team of pulmonologists, genetic specialists, respiratory therapists, and highly skilled surgeons, we offer comprehensive treatment for CF.

We are equipped with the latest diagnostic tools and high-tech ICUs for managing CF. We understand cystic fibrosis prevention is not possible, so our doctors offer a treatment plan that includes medication, nutrition guidance, and physiotherapy, and in case an organ is damaged, we advise organ transplantation.