Cystic Fibrosis

• What is cystic fibrosis?


  Cystic fibrosis is a genetic condition.
• What causes cystic fibrosis?


  It is caused by a faulty gene that affects the movement of salt and water in and out of cells.
• What are the consequences of cystic fibrosis on mucus production?


  It results in a build-up of thick, sticky mucus in the body's tubes and passageways.
• Which parts of the body are primarily affected by cystic fibrosis?


  Particularly the lungs and digestive system.
• What problems can cystic fibrosis cause from a young age?


  It can cause problems with breathing and digestion.
• What happens to the lungs over time in individuals with cystic fibrosis?


  The lungs become increasingly damaged and may eventually stop working properly.
• On which chromosome is the gene that causes cystic fibrosis located?

  Chromosome 7.
• What type of mucus do mucus-secreting cells produce in cystic fibrosis?


  They produce a very sticky type of mucus instead of a normal runny type.
• Is there a cure for cystic fibrosis?


  No, there is no cure.
• What is the average life expectancy for people with cystic fibrosis?


  47 years.
• What must both parents have for a child to inherit cystic fibrosis?


  Both parents must have a copy of the faulty (mutated) gene.
• What happens if only one copy of the faulty gene is inherited?


  A child will be a carrier but will not have the condition themselves.
• How is cystic fibrosis typically detected in the UK?


  It is picked up at birth using the newborn screening heel prick test.
• What does the newborn screening heel prick test involve?


  A healthcare professional pricks the baby's heel and collects 4 drops of blood on a special card.
• What is the incidence of cystic fibrosis in the UK?


  About 1 in 2,500 babies born in the UK has cystic fibrosis.
• What are some early signs of cystic fibrosis in babies?


  Babies may not gain weight well and are prone to chest infections.
• How can babies with cystic fibrosis be treated early?


  With a high-energy diet, medicines, and physiotherapy.
• What is the benefit of early treatment for cystic fibrosis?


  It can help them live longer, healthier lives.
• What does screening for cystic fibrosis also find?


  It finds some babies who may be genetic carriers of the condition.
• What additional tests are needed if the screening test suggests a child may have cystic fibrosis?


  A sweat test and a genetic test.
• What does the sweat test measure?


  It measures the amount of salt in sweat, which will be abnormally high in someone with cystic fibrosis.
• What does the genetic test check for?


  It checks for the faulty gene that causes cystic fibrosis.
• Can the genetic test be used for older children and adults?


  Yes, it can be used to diagnose cystic fibrosis in older children and adults who didn't have the newborn test.
• What is the importance of the genetic test for families?


  It can determine whether someone is a "carrier" of cystic fibrosis.
• What are some main symptoms of cystic fibrosis?


  Recurring chest infections, difficulty putting on weight, frequent coughs, diarrhea, wheezing, and shortness of breath.
• What related conditions can develop from cystic fibrosis?


  Diabetes, thin weakened bones (osteoporosis), and liver problems.
• What are the available treatments for cystic fibrosis?


  • Antibiotics to prevent and treat chest infections
  • Mucus thinners to make mucus easier to cough up
  • Bronchodilators to widen airways
  • Physiotherapy to help clear mucus from lungs
  • Bowel therapy medicines for nutrient absorption
• What additional treatments may be needed for cystic fibrosis patients?


  • Special diet and supplements to prevent malnutrition
  • Lung transplant if lungs are greatly damaged
  • Oxygen therapy to improve blood oxygen saturation
  • Enzyme pills for digestion
  • Dietary advice to reduce mucus production
• What monitoring is required for cystic fibrosis patients?


  • Lung function tests to measure air inhalation and exhalation
  • Sputum culture to test for specific bacteria
  • Chest and sinus X-rays for signs of complications
• What care needs do cystic fibrosis patients have?


  • Individual care plan and specific advice
  • Exercise plan to support health
  • Regular check-ups and vaccinations
  • Dietary supplements (vitamins A, D, E & K)
• What lifestyle adjustments do people with cystic fibrosis need to make?


  • Increased calorie intake, especially from fats
  • Mindfulness of bacteria and hygiene
  • Regular exercise
  • Emotional and social support as condition advances
• Why do people with cystic fibrosis need more calories?


  They need more calories to fight infection and due to malabsorption of food.
• What dietary composition is generally recommended for people with cystic fibrosis?


  A high-fat diet with 40 percent of total calories from fat.
• What challenges do people with cystic fibrosis face in their daily lives?


  They may find it difficult to study or work full-time due to hospitalizations and fatigue.
• What emotional support might people with cystic fibrosis need as their condition advances?


  Support for depression, anxiety, and feelings of isolation.
• What physical support might be needed for advanced cystic fibrosis?


  They may need ambulatory oxygen and help with daily tasks.
• What adaptations might be necessary for individuals with advanced cystic fibrosis?


  They may need a stair lift fitted if advanced CF.