Reducing incidents of misdiagnosis by raising awareness of cystic fibrosis

This past month (May) was Cystic Fibrosis Awareness Month, which encourages education in the battle against this disease which is usually diagnosed in childhood.

A paediatric pulmonologist and head of the Cystic Fibrosis Clinic at the Red Cross Children’s Hospital Dr Marco Zampoli said cystic fibrosis occurs in people of all races and backgrounds throughout the world. It is estimated that there are approximately 700 people with cystic fibrosis in South Africa. This disease is said to affect all parts of the body, but mainly the lungs and the digestive system by clogging it with thick and sticky mucus.

An unknown number of people with cystic fibrosis have been misdiagnosed with conditions such as asthma, tuberculosis or malnutrition, and subsequently, they do not have access to proper treatment. These people, many of them children, often die from malnutrition or pneumonia. Because treatment is available, it is important to reduce the incidents of misdiagnosis by raising awareness of the disease in all parts of South Africa.

Prominent symptoms which often appear in infancy and childhood, include:

• Frequent lung infections that may lead to problems with breathing, lung damage, prolonged courses of antibiotics and respiratory failure requiring support by a ventilator. Repeated infections and blockages can cause irreversible lung damage.
• Mucus that blocks the tiny ducts of the pancreas which supply enzymes required for digestion – consequently food is not properly digested and absorbed and nutritional value is lost in the process.
• Salty-tasting skin – the sweat glands are affected and the body may lose an excessive amount of salt during exercise or hot weather.
• Cystic fibrosis can also lead to frequent sinus infections, diabetes mellitus, difficulty with digestion and fertility problems.

Zampoli said that the most important aspect of therapy in cystic fibrosis is preventing malnutrition and lung damage that is caused by thick mucus and infection, so as to maintain a good quality of life.

Improved life expectancy

In a statement released by Kerry Simpson on behalf of Abbott Laboratories, it is mentioned that there is no cure for cystic fibrosis. However, people with cystic fibrosis can now live longer because of the advancements in treatment. Among these is Creon, a pancreatic enzyme replacement therapy (Pert) in the form of capsules. These capsules enable people with cystic fibrosis to absorb nutrients from food. It is specially designed to help the body absorb fats, proteins and carbohydrates.

According to the statement, “Treatment is particularly important for children, as ensuring adequate growth is important. By treating cystic fibrosis patients with this replacement therapy, nutritional management of patients with cystic fibrosis is improved, and future growth failure and chronic malnutrition can be prevented.”

Abbott Laboratories said that in studies of children with cystic fibrosis, Creon has been shown to improve the rate of fat absorption with good safety and tolerability, helping to control the symptoms of cystic fibrosis and enabling the patients to achieve satisfactory weight gain.

“Pert comes in the form of capsules containing specially coated enzyme beads (microspheres). For infants and younger children, who cannot swallow capsules whole, the capsules can be opened and the coated microspheres mixed with fruit gel. This can be fed to the infant before and during their feed. Older children can have microspheres mixed with yoghurt or pureed fruit. For children and adults, Pert capsules should be taken with every meal, including snacks,” said their statement.

Speak to your doctor or pharmacist who will assess your need for pancreatic enzyme replacement therapy.

Related article:

https://midrandreporter.co.za/214441/medical-evidence-suggests-vaping-harmful-cigarette-smoking/