Johannesburg, 10 June 2019: May was National Cystic Fibrosis Awareness Month, encouraging education in the battle against this disease which is usually diagnosed in childhood. An inherited life-threatening disorder, cystic fibrosis (CF) affects all parts of the body, predominantly the lungs and digestive system.

1 According to the South African Cystic Fibrosis Association (SACFA), there is currently no accurate information of cystic fibrosis patients in South Africa.2 “CF 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,” says Dr Marco Zampoli, Paediatric Pulmonologist and Head of the CF Clinic at the Red Cross Children’s Hospital, who has been working on a national CF registry launched in 2018 which will hopefully provide more accurate information in the near future.

However, due to the complex nature of the disease, limited access to diagnostic tests outside the main cities and low levels of CF awareness in society, an unknown number of people with CF are likely being misdiagnosed with conditions that resemble CF such as asthma, tuberculosis or malnutrition, and subsequently do not have access to proper treatment. These people, children more specifically, often die from malnutrition or pneumonia.

Because treatment is available, it is important to reduce the incidence of misdiagnosis by raising awareness of the disease in all parts of South Africa.2 Symptoms of cystic fibrosis According to SACFA, CF affects a number of organs in the body, primarily the lungs and pancreas, by clogging them with thick and sticky mucus.2 Prominent symptoms, which often appear in infancy and childhood, include:2

• Frequent lung infections that may lead to problems 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.
• CF can also lead to frequent sinus infections, diabetes mellitus, difficulty with digestion and fertility problems.

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

“The damaging effects of CF begin in early life and accumulate over time. Early diagnosis and appropriate medical treatment in early life is critical to ensure people born with CF can lead normal, healthy productive lives.” Improved life expectancy There is no cure for CF1. However, advancements in treatment have made it possible for people with CF to live much longer than ever before1.

Among these is Creon, a pancreatic enzyme replacement therapy (PERT) in the form of capsules, that enables people with CF to absorb nutrients from food. It is specially designed to help the body absorb fats, proteins and carbohydrates.

3,4 Because around 85% of CF patients have pancreatic exocrine insufficiency (PEI) by early childhood, maldigestion and poor growth resulting from PEI is a characteristic feature of cystic fibrosis.5 Prolonged, untreated PEI is associated with a poorer prognosis in the long term.

5 Treatment is particularly important for children, as ensuring adequate growth is important.5,6 By treating CF patients with PERT, nutritional management of patients with CF is improved, and future growth failure and chronic malnutrition can be prevented.5 In studies of children with CF, Creon has been shown to improve the rate of fat absorption with good safety and tolerability, helping to control the symptoms of CF and enabling the patients to achieve satisfactory weight gain.

6 PERT comes in the form of capsules containing specially coated enzyme beads (microspheres).3,4 For infants and younger children, who cannot swallow capsules whole, the capsules can be opened and the coated microspheres mixed with fruit gel.

7 This can be fed to the infant before and during their feed. Older children can have the microspheres mixed with yoghurt or pureed fruit. 7 For children and adults, PERT capsules should be taken with every meal, including snacks.3,4,7 Speak to your doctor or pharmacist who will assess your need for PERT.

For more information, visit the South African Cystic Fibrosis Association: http://www.sacfa.org.za/about-sacfa/

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References: 1. South African Cystic Fibrosis Association. What is CF? 2019. Available from: www.sacfa.org.za/about-cystic-fibrosis/. 2. South African Cystic Fibrosis Association. About CF. 2019. Available from: www.sacfa.org.za/about-cystic-fibrosis/. 3. Creon® 10000 Approved Package Insert, September 2005. 4. Creon® 25000 Approved Package Insert, February 2016. 5. Toouli J, et al. Management of pancreatic exocrine insufficiency: Australasian Pancreatic Club recommendations.

Med J Aust 2010;193:461–467. 6. Munck A, et al. Pancreatic enzyme replacement therapy for young cystic fibrosis patients. Journal of Cystic Fibrosis 2009;8:14–18. 7. South African Cystic Fibrosis Association. Medication. 2019. Available from: www.sacfa.org.za/about-cystic-fibrosis/.