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Respiratory System > Cystic Fibrosis - Adults and Children > Flashcards

Cystic Fibrosis - Adults and Children Flashcards

1
Q

What sort of genetic condition is cystic fibrosis?

A

Autosomal recessive

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2
Q

What does the CFTR (cystic fibrosis conductance regulator) do?

A

Transport channel for chloride:
Regulated liquid vol. on epithelial surface (pumps Cl out)
Cilia collapse (needs fluid around them)
Excessive inflammation

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3
Q

In CF what happens to the mucous in the respiratory tract?

A

Becomes thicker as it contains less water - causing cilia collapse and excessive inflammation

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4
Q

What are the common presentations of CF in infants ad young children?

A

Recurrent chest infections

Failure to thrive

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5
Q

Is CF screened for?

A

Yes, in neonates 5 days after birth:
Bloodspot test for immune-reactive trypsinogen
If positive, mutation analysis
Referred for sweat test

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6
Q

What are 2 cardinal features of CF?

A

Pancreatic insufficiency

Recurrent bronchopulmonary infection

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7
Q

Describe the abnormal stool associated with the pancreatic insufficiency in CF

A

Pale and orange
Very offensive
Greasy or oily

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8
Q

Why do CF patients produce abnormal stools?

A

Their pancreases are abnormal and don’t produce the appropriate digestive enzyme, especially lipid digesting enzymes

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9
Q

What are the two signs of pancreatic insufficiency?

A

Abnormal stool

Failure to thrive

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10
Q

How is the pancreatic insufficiency treated?

A

Enteric coated enzyme pellets
High energy diet
Fat-soluble vitamin and mineral supplement
H2 antagonist or proton pump inhibitor (to help get vitamins through stomach to small intestine)

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11
Q

What are four effect of recurrent bronchopulmonary infections?

A

Pneumonitis
Bronchiectasis
Scarring
Abscesses

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12
Q

What is the management of CF on early years (pre-infection)?

A 
Annual influenza vaccination
Prophylactic antibiotics
Segregation/cohorting to prevent cross infection
Airway clearance and adjunct
Mucolytics
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13
Q

What are the common organisms that cause infections?

A

Staph areus
H influenzae
Psuedomonas aeruginosa (later years)

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14
Q

What is the treatment of CF patients with chronic infection?

A

Suppress bacterial load
Treat infective exacerbations
Anti-inflammatory drugs: ibuprofen, azithromycin, prednisolone

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15
Q

What are the GI effects?

A

Dysmobility: meconium ileus, gastro-oesophageal reflux, constipation
Co-existent disease: crohn’s and coeliac
Hepatopathy

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16
Q

What is meconium ileus?

A

Bowel obstruction that occurs when the meconium in the intestine is thick and sticky

17
Q

Name other manifestations of CF

A 
Diabetes
Bone: osteopenia 
Heat exhaustion 
Bilateral absence of vas deferent 
Vaginal candidacies
18
Q

Where does the CF gene lie?

A

On the long arm of chromosome 7

19
Q

How many mutations can occur on chromosome 7?

A

Over 1800, with different mutations resulting in different phenotype of disease

20
Q

How does abnormal CTFR lead to increased thickness of mucous on the epithelium?

A

Impaired Cl transport and enhance Na transport in so drawn water to inside of epithelial cells, causing thick mucous in the airways

21
Q

Does CF affect fertility in men?

A

Yes, blocked/absent vas deferens (95% male infertile)

22
Q

How does CF affect fertility in women?

A

They are still fertile, although poor nutrition may affect chances of conception

23
Q

What steps must be taken before a women with CF becomes pregnant?

A

Careful planning
Screening partner
Counselling for long term impact of condition on family
Optimum health

24
Q

What are indications to carry out lung transplant?

A 
FEV1 < 30% predicted 
Weight loss 
Hypoxia at rest 
Hypercapnia 
Recurrent worsening sepsis 
Worsening QOL
25
Q

What are three questions that must be considered before lung transplant?

A

Is the condition bad enough?
Is the rest of the body healthy enough?
Does the patient want to take risk?

26
Q

What are contraindications to lung transplant?

A 
Organ failure 
Malignancy within 5yrs 
PVD
Drug, nicotine, alcohol dependency 
Systemic infection 
Smoking - must prove they've quit for over 6 months
27
Q

What are relative contraindications to lung transplant?

A

Steroid > 20mg daily
No social support
Osteoporosis
Low BMI

28
Q

What are the psychosocial issues associated with lung transplant?

A

Hope
Fear
Guilt/conflict
Stress

29
Q

What are the end of life issues?

A

Deteriorating health/death hard to talk about
Important to be open and available for discussion
Patient/family to plan and prepare

30
Q

Are there any screening for antenatal screening for CF?

A

No

Respiratory System (29 decks)

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