Celiac Disease & Cleft Lip/Palate (AW)

many processed foods...

contain gluten as a thickener or filler

steatorrhea

fat in the feces; frothy, foul-smelling fecal matter

foods NOT allowed with celiac disease

oats, wheat, barley, rye

foods allowed with celiac disease

rice, corn, soy, potato, eggs, millet, buckwheat, nut flours

diagnosis of celiac disease

- biopsy of small intestine - genetic and serologic testing

celiac disease complications

- celiac crisis (anemia, severe weight loss) - If left untreated: -- lactose intolerance -- vitamin deficiencies -- impaired growth -- osteomalacia -- Non-Hodgekin's Lymphoma

Cleft Lip Repair: Cheiloplasty

surgery 2-3 months (need enough tissue for repair)

pathophysiology of celiac disease

- characterized by villous atrophy in the small bowel in response to the protein gluten, specifically gliadin component of gluten - leads to malabsorption - believed to be autoimmune

cleft palate repair: palatoschisis

surgery 6-12 months (best outcome with speech development before 12 mo)

cheiloplasty post op

- cleansing of the suture line - keep moist (petroleum jelly) - elbow immobilizers - pain control - resume feeds as tolerated - avoid hard objects or suction in the oral cavity - home within 24 hrs

cleft lip and cleft palate

- facial malformations that occur during embryonic development - may appear separately or together

Cleft Lip

- failure of fusion of upper lip - often associated with facial abnormalities - may be unilateral or bilateral - may elicit severe emotional reactions from parents - feeding difficulties

celiac disease: autoimmune

- genetic predisposition - CD4+ T cells play a critical role in the immune response characteristic of celiac disease

When is celiac disease diagnosed?

typically noticed several months after induction of gluten in diet (btwn 1-5 yrs)

feeding the child with CL/CP

- hold infant in upright position during feeding - special needs feeder - breastfeeding (use a pump) - obturator - burping

clinical manifestations of celiac disease

- impaired fat absorption (diarrhea/steatorrhea) - general malnutrition: muscle wasting, anemia, anorexia - abd distention

celiac disease

- intolerance to the dietary protein gluten - intestinal tolerance that produces mucosal lesions - chronic disease with severity varying greatly among children

therapeutic management of celiac disease

- primarily dietary - low gluten or gluten free diet - corn and rice become substitute grain foods - possible lactose restrictions

cleft palate

- results from failure of the hard and soft palates to fuse because of failure of the tongue to descend soon enough - may be midline or bilateral

causes & pathophysiology of cleft lip/palate

- risk factors (familial link, folic acid, cigarettes, drugs, alcohol) - defect in cell migration that results in a failure of the maxillary and premaxillary processes to come together (btwn 4-10 week of development)

palatoschisis post op

- watch for respiratory difficulties - elbow immobilizers - no hard or sharp objects in mouth - monitor suture line - feeding modifications (syringe feeding)

associated problems with CL/CP

1. feeding - inability to form anterior seal (CL) - decreased ability to suck (CP) 2. secretions 3. long term considerations - dental/orthodontic - speech - hearing - improper drainage of middle ear - otitis media

the nurse is caring for an infant whose cleft lip was repaired. important aspects of this infant's postoperative care include: A. arm restraints, suctioning of drainage, and mouth irrigations B. cleansing of suture line, supine position, and arm restraints C. mouth irrigations, prone position, and cleansing of suture line

B