A 9-month-old with Tay-Sachs disease is admitted due to seizures. Which assessment is most important for the nurse to obtain?

Ability to crawl.

Eyes with cherry-red spot.

Difficulty with swallowing.

Exaggerated startle reaction

Answer and Explanation

The Correct Answer is D

Choice A reason: The ability to crawl is not the most important assessment for the nurse to obtain. Tay-Sachs disease causes progressive loss of motor skills, so the infant may not be able to crawl or may have regressed from crawling. However, this is not a specific sign of the disease and does not indicate the severity of the condition.

Choice B reason: The eyes with cherry-red spot are not the most important assessment for the nurse to obtain. Tay-Sachs disease causes accumulation of gangliosides in the retina, which results in a cherry-red spot in the center of the macula. However, this is not a specific sign of the disease and does not indicate the severity of the condition.

Choice C reason: The difficulty with swallowing is not the most important assessment for the nurse to obtain. Tay-Sachs disease causes muscle weakness and spasticity, which may affect the infant's ability to swallow. However, this is not a specific sign of the disease and does not indicate the severity of the condition.

Choice D reason: The exaggerated startle reaction is the most important assessment for the nurse to obtain. Tay-Sachs disease causes increased sensitivity to sound and touch, which results in an exaggerated startle reaction. This is a specific sign of the disease and indicates the severity of the condition. The exaggerated startle reaction may also trigger seizures, which can be life-threatening. The nurse should monitor the infant's vital signs, seizure activity, and neurological status closely.

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Correct Answer is A

Explanation

Choice A reason: Measuring abdominal circumference provides immediate, objective data about the degree of abdominal distention, which is a key sign of intestinal obstruction in a neonate who hasn’t passed meconium and is vomiting bilious secretions. Establishing a baseline girth measurement guides further assessment, helps detect rapidly worsening distention, and informs decisions about decompression and imaging studies.

Choice B reason: Although initiating an IV infusion is critical for fluid and electrolyte replacement in any vomiting infant, securing intravenous access should follow a focused assessment. Prioritizing assessment ensures you understand the severity of the obstruction and tailor fluid resuscitation and other interventions appropriately.

Choice C reason: Monitoring strict urinary output is not the first action that the nurse should take. This is because urinary output is not the most sensitive indicator of fluid status in infants, especially those with renal insufficiency or oliguria. Monitoring urinary output may also delay more urgent interventions, such as fluid resuscitation and decompression.

Choice D reason: Preparing for anorectal manometry is not the first action that the nurse should take. This is because anorectal manometry is a diagnostic test that measures the pressure and function of the anal and rectal muscles. It is not indicated for infants with suspected meconium ileus, which is a mechanical obstruction of the bowel by thick and sticky meconium. Preparing for anorectal manometry may also delay more urgent interventions, such as fluid resuscitation and decompression.

A. Rice. B. Oats. C. Barley. D. Rye.

Correct Answer is A

Explanation

Choice A reason: Rice is a gluten-free grain that is safe for people with celiac disease. Rice does not contain the protein gluten that triggers an immune reaction and damages the small intestine in people with celiac disease.

Choice B reason: Oats are generally not recommended for people with celiac disease because they are often contaminated with gluten from other grains during processing. Some people with celiac disease may also react to a protein in oats called avenin that is similar to gluten. Only certified gluten-free oats may be safe for some people with celiac disease, but they should consult their health care provider before consuming them³.

Choice C reason: Barley is a grain that contains gluten and is not safe for people with celiac disease. Barley can cause inflammation and damage to the small intestine in people with celiac disease. Barley is also used to make malt, which is a common additive in many processed foods and beverages.

Choice D reason: Rye is a grain that contains gluten and is not safe for people with celiac disease. Rye can cause the same symptoms and complications as wheat and barley in people with celiac disease. Rye is often used to make bread, crackers, and cereals.

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