Cystic Fibrosis in Children

Living with cystic fibrosis (CF) can be challenging, as it requires daily care and attention. However, despite these challenges, individuals with CF are often able to attend school and contribute to the workforce. Over time, advancements in medical treatments have led to a better quality of life for those with CF compared to previous decades.

Understanding Cystic Fibrosis

Cystic fibrosis (CF) is an inherited disorder that can cause extensive damage to various organs, notably the lungs and digestive system. This condition impacts cells responsible for producing mucus, sweat, and digestive fluids, resulting in abnormal thickening of these secretions.

Effects of Cystic Fibrosis

In individuals with CF, thick and sticky secretions lead to blockages in the lungs, pancreas, and other bodily ducts. Despite being a progressive condition requiring daily management, individuals with CF can still lead relatively normal lives, attending school and work.

Advances in Cystic Fibrosis Care

With advancements in screening and treatment, the quality of life for people with CF has significantly improved compared to previous generations. It's now possible for individuals with CF to live well into their mid-to-late 30s, 40s, and even 50s, highlighting the positive impact of medical progress in managing this condition.

What Are The Symptoms of Cystic Fibrosis?

Cystic fibrosis symptoms can vary depending on the severity of the disease, and they can change over time, even within the same person. Some people may not experience any symptoms until their teenage years or adulthood. Those who are diagnosed with the disease in adulthood usually have milder symptoms and are more likely to have atypical symptoms such as recurring bouts of pancreatitis, infertility, and pneumonia. One of the most common symptoms of cystic fibrosis is a higher-than-normal level of salt in sweat. Parents can usually taste the salt when they kiss their children. The respiratory system and digestive system are the most affected systems in people with cystic fibrosis.

Respiratory Signs and Symptoms

The thick and sticky mucus associated with cystic fibrosis clogs the tubes that carry air in and out of your lungs. This can cause signs and symptoms such as:

•         A persistent cough that produces thick mucus (sputum)
•         Wheezing
•         Exercise intolerance
•         Repeated lung infections
•         Inflamed nasal passages or a stuffy nose
•         Recurrent sinusitis

Digestive Signs and Symptoms

The thick mucus can also block tubes that carry digestive enzymes from your pancreas to your small intestine. Without these digestive enzymes, your intestines aren't able to completely absorb the nutrients in the food you eat. The result is often:

•         Foul-smelling, greasy stools
•         Poor weight gain and growth
•         Intestinal blockage, particularly in newborns (meconium ileus)
•         Chronic or severe constipation, which may include frequent straining while trying to pass stool, eventually causing part of the rectum to protrude outside the anus (rectal prolapse)

How is Cystic Fibrosis Diagnosed?

CF is diagnosed through various tests, including newborn screening, sweat test, genetic tests, chest X-rays, sinus X-rays, lung function tests, sputum culture, Nasal potential difference tests, and Intestinal current measurement tests. Newborn screening involves taking blood from a heel prick, sweat test measures chloride levels, genetic tests detect CF-causing genes, and chest and sinus X-rays support CF diagnosis. Lung function tests use spirometry, sputum culture tests for bacteria, NPD test measures the electricity generated by the transfer of ions across nasal tissue, and ICM test measu

res chloride secretion. In people with atypical CF, the sweat test may be normal. NPD and ICM tests can help confirm a questionable diagnosis.

 

Why is a High-Calorie, High-Fat Diet Needed For People With Cystic Fibrosis (CF)?

People with cystic fibrosis require more calories and fats than those without CF. This is because they use more energy to breathe, fight lung infections and maintain their strength. Cystic fibrosis stops digestive enzymes from working correctly, which means that nutrients and fats from food aren’t fully absorbed. Maintaining a higher weight than normal is crucial for young people with CF to reach their full genetic potential, which includes getting as tall as possible with lungs as large as possible. People with CF can eat salty foods since they lose a lot of salt in their sweat. It's advisable to speak with a healthcare provider about the amount of salt needed each day.

 

How is Cystic Fibrosis Treated?

Cystic fibrosis (CF) is a condition that has no cure but can be managed. The primary aim of management is to keep airways clear, and special ways of coughing and breathing can help. Devices and therapy vests that rely on vibrations are also useful. Chest physical therapy can help loosen mucus. Medications like antibiotics, inhaled bronchodilators, anti-inflammatory drugs, pancreatic enzymes, etc., can help in certain situations. Surgery may be needed for complications and can involve bowel surgery, nose or sinus surgery, or transplantation.