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Cystic Fibrosis
Cystic Fibrosis
Cystic Fibrosis
Cystic Fibrosis
Cystic Fibrosis
Cystic Fibrosis
Cystic Fibrosis
Cystic Fibrosis
Cystic Fibrosis
Cystic Fibrosis
Cystic Fibrosis
Cystic Fibrosis
Cystic Fibrosis
Cystic Fibrosis
Cystic Fibrosis
Cystic Fibrosis
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Cystic Fibrosis

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  • For cystic fibrosis to occur, a child’s parents must both be carriers of a defective gene on chromosome 7. If both parents are carriers, a child then has a 50% chance of also becoming a carrier, a 25% chance of contracting cystic fibrosis and a 25% chance of not being a carrier and not contracting cystic fibrosis.
  • This gene on chromosome 7 contains information for a protein called the cystic fibrosis transmembrane conductance regulator (CFTR). The protein CFTR controls the movement of salt and water in and out of the cell. When the gene is defective, like in CF, the CFTR doesn’t work properly or may be completely missing. This causes sweat to be up to five times saltier than normal. It also causes thick, sticky mucus to be produced on the outside of the cell (Genetic Science Learning Center, 2009).
  • The two organs that are mainly affected by this mucus are the lungs and the pancreas. The mucus builds up and obstructs the airways in the lungs. This build up of mucus can lead to bacterial growth, increasing risk of infections and the probability of lung damage. The pancreas produces enzymes that assist with digestion but the build up of mucus can also block the ducts in the pancreas, stopping the enzymes from reaching the intestines. Without these enzymes the intestines are unable to digest food properly, leading to a loss of vitamins and nutrients.
  • Social / emotional:            A child with cystic fibrosis ability to interact with children may be hindered from absence from the centre due to health reasons. It is important to monitor this and help to facilitate interactions when needed.          The child may experience some emotional distress while at the centre. It is important to watch for signs of depression, withdrawal, becoming over dependent. A child may feel embarrassed by the effects of their CF such as coughing. It is important to encourage them to cough and provide them with tissues for any phlegm they bring up. Allow them to leave the play space if they wish to cough around fewer children. Explain to the other children that it is okay to cough and encourage procedures such as covering their mouth, using a tissue and washing hands to all children at the centre.
  • Physical:            It is important for children with CF to exercise regularly so this should be encouraged at the centre. Exercise helps to loosen mucus, in conjunction with inhalers or nebulizers.          A child’s ability to fully participate in physical activities may be hindered depending on their current health status and any infections they may have at that time. It is still important to encourage participation at these times and the child may need rest breaks to cough and have a drink of water. Developmental delays may occur if the child has fewer opportunities to participate in physically active play if absent from the centre for a period of time due to illness.
  • Cognitive:            A child’s cognitive development is not usually affected by cystic fibrosis. . Brain development is crucial in the first few years of life. Research conducted by The American Academy of Paediatrics suggests that because children with CF are at risk of malnutrition they will be at risk of poorer cognitive development. This suggests that children with CF must meet their own nutritional requirements to maintain optimal cognitive development.
  • Language:            A child’s language development is not usually affected by cystic fibrosis.          If the child is sick in bed at home or the hospital, reading stories to them is a great opportunity to promote early language and literacy skills.
  • Partnerships with families are always important for a childcare centre. The family is a primary source of information about their child and the staff need to form partnerships and develop effective communication with the families to gain important knowledge and understanding of each child (National Childcare Accreditation Council Inc, 2005). This is even more vital for a child with an additional need such as cystic fibrosis.   Just as every child is different, each child with cystic fibrosis is also different and this needs to be taken into consideration. It is important to talk to the family and child about what characteristics of cystic fibrosis they display, rather than making assumptions.
  • Including the child’s family during their child’s daily routine in the centre is important. Parents may need to come to the centre to assist the child with their daily physiotherapy. This physiotherapy is done to help clear the airways of the mucus in the lungs and is important in helping to reduce the risk of infections. The amount of physiotherapy sessions each day varies with each child and each day. There are a number of physiotherapy techniques a child with CF may use, some include: chest percussion, vibrations and breathing techniques.
  • The child will have a mealtime routine as they have certain nutritional requirements. Children with CF need to eat extra calories and protein than other children. This is so they can replace the fat and protein they pass and be well nourished to assist in preventing infections. Children with CF also require supplements at mealtimes. Supplements may include: -         Vitamins to replace vitamins lost in digestion -         Salt supplements, especially in hot weather or when exercising to replace salt lost through sweat Pancreatic enzymes to assist with digestion
  • Transcript

    • 1. Children with Cystic Fibrosis
    • 2. What is Cystic Fibrosis? Cystic fibrosis (CF) is a hereditary disease of the mucus, saliva and sweat glands . It affects organs such as lungs, pancreas, liver, intestines, sinuses, and sex organs (MedicineNet Inc, 2009).   “ Cystic fibrosis is the most common inherited, life limiting, incurable condition amongst Caucasians” (Royal Children’s Hospital)
    • 3. What Causes CF?
      • Cystic Fibrosis is an inherited disease.
      • For a child to inherit CF, both parents must be carriers of a defective gene on chromosome 7.
      • They then have a 50% chance of becoming a carrier.
      • A 25% chance of getting CF
      • A 25% chance of not being a carrier and not having CF
    • 4. What is it about Chromosome 7 that causes CF?
      • A chromosome carries genetic information
      • Chromosome 7 carries the cystic fibrosis transmembrane conductance regulator (CFTR)
      • CFTR controls salt and water movements in and out of cells
      • When CFTR is defective, cystic fibrosis occurs because the CFTR doesn’t work or is completely missing.
      • When salt and water don’t move in and out of cells properly, sweat becomes 5 times saltier and a thick, sticky mucus is produced outside the cell.
    • 5. What does the thick, sticky mucus do? It affects the… Lungs Pancreas
      • Mucus builds up and obstructs airways
      • Build up also makes a suitable environment for bacterial growth
      • Bacterial growth increases risk of infections
      • Repeated infections cause lung damage
      • Pancreas produces enzymes that help with digestion
      • Build up of mucus blocks ducts in pancreas, stopping enzymes form reaching intestines
      • Without enzymes, intestines can’t digest food properly
      • Leads to loss of vitamins and nutrients
    • 6. Symptoms of CF
      • Persistent cough, often with phlegm or possibly blood streaking
      • Difficulty breathing / wheezing
      • Fatigue
      • Fleshy growths inside the nose (nasal polyps)
      • Weight loss / poor weight gain
      • Frequent lung infections, pneumonia, bronchitis
      • Abdominal pain
      • Salty tasting skin (salt loss when sweating) leading to dehydration
      • Greasy, light coloured, foul smelling stools or diarrhoea
    • 7. Implications For Development: Social / Emotional
      • Child may have difficulty interacting with other children due to periods of absence from centre
      • Child may experience some emotional distress – depression, withdrawal, becoming over dependent
      • Child may feel embarrassed by effects of their CF. For example – coughing a lot and bringing up phlegm.
    • 8. Implications For Development: Physical
      • Children with CF are very capable of participating in normal physically active play and activities.
      • Regular exercise is important – it helps to loosen mucus
      • Child’s current health status may not allow them to fully participate in physical activities. They can still participate but may need more frequent breaks to cough and have a drink.
    • 9. Implications For Development: Cognitive
      • A child’s cognitive development is not usually affected by cystic fibrosis
      • Malnutrition can stunt cognitive development. Research conducted by The American Academy of Pediatrics suggests children with CF are at risk of malnutrition because of their loss of vitamins and nutrients. This is mainly a concern when CF hasn’t yet been diagnosed.
    • 10. Implications For Development: Language
      • A child’s language development is not usually affected by cystic fibrosis.
      • If the child is sick in bed at home or the hospital, reading stories to them is a great opportunity to promote early language and literacy skills.
    • 11. Modifications to the Service
      • No structural modifications are usually required.
      • There are a few modifications needed to be made to the program to ensure the health and safety of the child with CF, mainly during routine times.
      • Resources to assist the child while at the centre may include their nebuliser. Tissues are also helpful to reduce embarrassment when coughing up phlegm.
    • 12.
      • Involving a child’s family in the service and maintaining effective communication is vital in developing an individualised routine for a child with CF.
      • The family will be able to tell you about their child’s cystic fibrosis and how it effects their child - Just as every child is different, each child with cystic fibrosis is also different!!
      Involving Family in the Modifications
    • 13. Individual Routines - Physiotherapy
      • Parents may need to come to the centre to assist the child with their daily physiotherapy.
      • Physiotherapy helps clear the airways of the mucus in the lungs and is important in helping to reduce the risk of infections.
      • The amount of physiotherapy sessions each day varies with each child and each day.
      • There are a number of physiotherapy techniques a child with CF may use, some include: chest percussion, vibrations and breathing techniques.
    • 14. Individual Routines - Mealtimes
      • Children with CF need to eat extra calories and protein than other children. This is so they can replace the fat and protein they pass and be well nourished to assist in preventing infections.
      • Children with CF also require supplements at mealtimes. Supplements may include:
      • - Vitamins to replace vitamins lost in digestion
      • -  Salt supplements, especially in hot weather or when exercising to replace salt lost through sweat
      • - Pancreatic enzymes to assist with digestion
    • 15. Reference List 1. American Academy of Pediatrics. (2004), Cognitive Function of Children With Cystic Fibrosis: deleterious Effect of Early Malnutrition, accessed on 24 August 2009 <http://pediatrics.aappublications.org/cgi/content/full/113/6/1549> 2. Cold Spring Harbour Laboratory. 2002, Cystic Fibrosis: What causes it?, accessed on 11 August 2009 <http://www.yourgenesyourhealth.org/cf/cause.htm> 3. Cystic Fibrosis Australia. 2002, Cystic Fibrosis Teachers, accessed on 11 August 2009 < http://www. cysticfibrosis .org.au/ pdf /CF_Teachers_Guide. pdf > 4. Cystic Fibrosis Australia. 2009, Cystic Fibrosis in Australia > Nutition > For Children, accessed on 11 August 2009 <http://www.cysticfibrosis.org.au/nutrition/nutritionforchildren> 5. Genetic Science Learning Center. 2009, Cystic Fibrosis, accessed on 18 August 2009 < http://learn.genetics.utah.edu/content/disorders/whataregd/cf/ > 6. MedicineNet Inc. 2009, Cystic Fibrosis Causes, Symptoms, Diagnosis and Treatment, accessed on 19 August 2009 < http://www.medicinenet.com/cystic_fibrosis/article.htm > 7. National Childcare Accreditation Council Inc. 2005, QIAS Quality Practices Guide. Accessed on 19 August 2009 < http://www.ncac.gov.au/publication_extracts/qias_qpg_preambles.pdf >          
    • 16. 8. National Library of Medicine. 2008, Cystic Fibrosis – Genetics Home Reference, accessed on 19 August 2009 < http:// ghr . nlm . nih . gov /condition= cysticfibrosis > 9. PSCQ. Cystic Fibrosis General Overview, accessed on 20 August 2009 < http://www.noahsark.net.au/PDF/cystic_fibrosis.pdf > 10. Royal Children’s Hospital. Cystic Fibrosis, accessed on 23 August 2009 < http://www.rch.org.au/emplibrary/edinst/Cystic_Fibrosis_Sheet.pdf> 11. The Nemours Foundation. 2009, Cystic Fibrosis, accessed on 18 August 2009 < http://kidshealth.org/teen/diseases_conditions/digestive/cystic_fibrosis.html# > 12. Web Ring Inc. 2007, Cystic Fibrosis Symptom.com – Cystic Fibrosis Foundations, Resources, Symptoms and Information, accessed on 11 August 2009 < http://www.cystic-fibrosis-symptom.com/symptoms.htm >

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