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TL Cystic Fibrosis
Nursing of the child with Cystic Fibrosis (HESI)
| Question | Answer |
|---|---|
| Define Cystic Fibrosis. | Autosomal-recessive disease that causes dysfunction of the exocrine glands so that tenacious mucus production obstructs vital structures |
| Define exocrine glands. | Exocrine Glands are glands that secrete their products into ducts that lead to the external surface like sweat, mucous, and saliva glands |
| List 3 complications that develop from exocrine dysfunction. | Lung insufficiency (most critical), Pancreatic insufficiency, increased loss of sodium and chloride in sweat |
| Which race is most likely to have cystic fibrosis? | whites |
| What sign is present at birth in 10 – 20 % of cases of cystic fibrosis? | meconium ileus |
| What finding would the nurse expect to find on assessment of the client with Cystic Fibrosis? (RRI PC S FSBS DG PWG STS) | recurrent respiratory infection, pulmonary congestion, steatorrhea, foul-smelling bulky stools, delayed growth and poor weight gain, skin tastes salty when kissed |
| What are the later signs of cystic fibrosis? (CNC) | cyanosis, nail-bed clubbing, congestive heart failure |
| Give two nursing diagnoses that are applicable to the client with Cystic Fibrosis. | Ineffective airway clearance, Imbalanced nutrition less than |
| Name some nursing interventions that address ineffective airway clearance. (MRS, ASOI, AAODP, MVA, EERT) | Monitor respiratory status, assess for signs of infection, administer IV antibiotics, oxygen, DNase, or Pulmozyme as prescribed, manage vascular access, evaluate effectiveness of respiratory TX, |
| How does recombinant human deoxyribonuclease (DNase) help kids with Cystic Fibrosis? | because white blood cells accumulate in the mucus, and, when they break down, they release DNA, which adds to the 'stickiness' of the mucus. DNase enzymes break down the DNA, and the mucus is much easier to clear from the lungs. |
| How does dornase alfa (Pulmozyme) help kids with Cystic Fibrosis? | hydrolyzes the DNA present in sputum/mucus and reduces viscosity in the lungs, promoting improved clearance of secretions. (produced in Chinese hamster ovarycells ) |
| What nursing interventions help with imbalanced nutrition less than body requirements? | administer pancreatic enzymes (Cotazym-S, Pancrease) with food, Administer fat soluble vitamins in water soluble form (ADEK), teach diet high in calorie, protein, and moderate to high in fat, moderate to low in carbohydrates |
| What techniques will the nurse teach the family to help the child with lung congestion? | Postural drainage and percussion |
| What nursing intervention will address the hospitalized child’s developmental needs? | provide age appropriate activities |
| What nursing intervention addresses the fact that cystic fibrosis is an inherited disorder? | refer family for genetic counseling |
Created by:
Perseverandovercome
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