CELIAC DISEASE (CD):

Also known as Gluten- induced enteropathy (GSE ) and celiac sprue, is a disease of the small intestine  characterized by abnormal mucosa and permanent intolerance to gluten.CD is second  only to cystic fibrosis as a case of malabsorption in children

Frequency

·         It varies from 1 in 300 to 1 in 4000 , and appears to be declining , possibly in relation to environmental factors

·         It is seen more commonly in Europe than in America  and is rarely reported in Asians and Africans

·         The exact cause of CD is unknown , but that appears to be an inherited predisposition with an influence by environmental factors

·         seen between 1-5yrs of age

Etiology

·         Gluten was identified as causative agent in 1953

·         Gluten is found in wheat, rye, barley & oats. Alpha glandin fraction is responsible factor having strong association with HLA a8, DRW3 histocompatibility antigen

·         Environmental trigger appears to be necessary

·         an inherited disease. Celiac disease effects those with a genetic predisposition.

Pathogenesis

Factors suggesting immunological basis-

·          Antibodies to alpha glandin demonstrated in serum and intestinal secretions

·         Serum IgA are high & IgM are low

·         Favorable response to immunosuppressive agents

Clinical manifestations

The first evidence of the disease may be FTT and iarrhea

·         Impaired fat absorption

®    Steatorrhea (excessively large , pale, oily, frothy stools)

®    Exceedingly fowl smelling stools

·         Impaired absorption of the nutrients

®    Malnutrition

®    Muscle wasing(especially prominent in legs and buttocks)

®    Aneamia

®    Anorexia

®    Abdominal distension

·         Behavioural changes

®    Irritability

®    Fretfulness

®    Uncooperativeness

®    Apathy

·         Celiac crisis

®    Acute, severe episodes of profuse watery diarrhea and vomiting

®    May be precipitated by

¾    Infections(especially GIT)

¾    Prolonged fluid and electrolyte depletion

¾    Emotional disturbances

Diagnosis:

¾    Clinical picture

¾    H/o improvement with exclusion of wheat  from diet and recurrence of diarrhea on reintroduction of wheat , rye, barley, or oats in the diet

¾    Increased excretion of fats

¾    Serum xylose levels less than 20 mg / 100ml 2 hours after administration of 0.5 gm of xylose per kg of body weight

¾    Jejuna biopsy shows characteristic villous atropy

¾    Evidence of secondary lactose deficiency

Complication

·         osteoporosis

·         lymphoma of the small intestine

·         infertility

·         autoimmune liver disease

Treatment

Ø  Exclusion of all cereals containing gluten(wheat, rye, barley, oats)

Ø  Gradual introduction of foods in acute cases with simple sugar, banana, skimmed milk rice& lentil gruel(khichri)

Ø  Milk and lactose containing diets withheld for first few weeks as secondary lactose deficiency is common

Ø  Celiac crisis to be treated energetically with rehydration therapy

Ø  Corticosteroids can be used in celiac crisis and in patients who are symptomatic in spite of gluten withdrawal for 1 week

Ø  Iron and vitamine suppliments to counteract aneamia and vitamine deficiencies

Ø  Prolonged i/v alimentation required in very severe chronic cases before they start tolerating oral feeds

Ø  Iron and folate suppliments

Ø  Vitamine A and D in water soluble forms

Ø  In children with chronic diarrhea secondary to bile acid malabsorption, the use of cholestyramine (Questran) to bind bile acids may help to reduce the duration and severity of the diarrhea.

Nursing care

Nursing assessment

• steatorrhea
• Chronic diarrhea or constipation or both
• Anorexia
• Generalized malnutrition and failure to thrive
• Coagulaton difficulty
• Irritability
• Anemia
• Clubbed fingers
• Dental enamel defect
• Vomiting rash
• Electrolyte study for ca, protein(hypocalcemia, hypoalbuminemia)
• Blood study for HB, BT, CT(hypothrombinemia)
• Stool specimen for high fat

Nursing diagnosis

1. Imbalanced nutrition less than body requirement related to poor absorption of the nutrients
2. Chronic pain – abdomen related to the disease condition
3. Fluid electrolyte imbalance related to underlying pathology
4. High risk for complication – anemia, bleeding related reduced thrombin level
5. Alteration in comfort related to the disease condition
6. Anxiety related to the unexpected outcome of the disease
7. Altered parental coping related to the need for long term care

Nursing interventions

This can be divided as :

1. Assisting with the diagnosis
2. Assisting parents in their adjustment to the diagnosis
3. Providing nutritious diet
4. Correcting nutritional deficiencies
5. Prevention and care during celiac crisis
6. Educating the child and parents during longterm follow up care

In genrral;

• Eliminate all gluten from the food

• Give the child corn and rice product , soy and potato flour, breast milk or soy – based formula, and fresh fruits
• Replace vitamins and calories ; give small frequent meals
• Monitor for staetorrhoea its disappearance is a good indication that the child’s ability to absorb nutrients is improving