Cystic Fibrosis

Question 1

What is cystic fibrosis?

Answer 1

• it is an autosomal recessive disorder
• it is a disorder where there are abnormalities in the sodium chloride concentrations in the secretory epithelia occurring throughout the body, this may affect
  —> respiratory tract
  —> male reproductive system
  —> pancreas
  —> hepato-biliary system
  —> gastrointestinal tract

Question 2

What are some of the complications of cystic fibrosis?

Answer 2

• nasal polyps
• recurrent sinusitis
• bronchiectasis
• Haemoptysis
• intestinal obstruction
• pancreatic insufficiency
• malnutrition
• portal hypertension
• cirrhosis
• gallstones
• male infertility
• insulin-dependent diabetes
• osteoporosis
• vitamin and salt deficiency
• delayed puberty
• CF associated arthritis

Question 3

What are the goals of treatment?

Answer 3

• to maintain lung function
• to maintain a quality of life
• treatment involves daily physiotherapy and medication
• regular assessment of lung function is necessary, as is early aggressive treatment of infections
• dietary supplementation is important

Question 4

What are some of the issues in pregnancy?

Answer 4

• needs adequate dietary supplementation
• susceptible to recurrent infections
• respiratory failure
• pulmonary hypertension

Question 5

What is the role of the midwife antenatally?

Answer 5

• requires MDT approach
• booking at consultant-led unit
• support woman throughout pregnancy and reinforce adherence to treatment and ensure engagement with other professionals
• promote a healthy lifestyle with emphasis on a nutritional diet
• encourage dietary supplementation
• monitor closely noting any signs of increasing breathlessness
• ensure serial growth scans have been commenced for identification of IUGR

Question 6

What is the role of the midwife in labour?

Answer 6

• need to be mindful of potential risk to woman and baby with respect to respiratory failure and monitoring requirements
• ensure that oxygen is available in the delivery Rom and maternal oxygen saturation’s should be measured at intervals
• continuous electronic fetal monitoring
• avoid prolonged pushing and the Valsalva manoeuvre
• if necessary prepare for pre term delivery and make the paeds aware that the mother is in labour

Question 7

What is the midwives role postnatally?

Answer 7

• encourage regular management routine and offer any additional support
• be alert for signs of respiratory infection
• infant feeding may be an issue if woman is prescribed quinolones
• nutritional supplements still required