You are preparing a child with sickle cell anemia for discharge after an acute crisis episode. Which of the following instructions is appropriate advice for the care of a child with sickle cell anemia?

Choice A reason:

A specific gravity of 1.010 indicates that the child’s urine is adequately diluted, suggesting proper hydration. Normal urine specific gravity ranges from 1.005 to 1.030. A value of 1.010 falls within this range and indicates that the kidneys are functioning well and the child is not dehydrated.

Choice B reason:

Moist mucous membranes are a sign of adequate hydration. When a child is well-hydrated, the mucous membranes in the mouth and other areas remain moist. This is a positive indicator that oral rehydration therapy has been effective in restoring the child’s fluid balance.

Choice C reason:

Capillary refill time of less than 3 seconds is another indicator of proper hydration and good peripheral perfusion. This means that the blood is circulating well throughout the body, and the tissues are receiving adequate oxygen and nutrients. A capillary refill time of less than 3 seconds is considered normal and suggests effective rehydration.

Choice D reason:

A sunken anterior fontanelle is a sign of dehydration in infants. When the fontanelle is sunken, it indicates that the child has lost a significant amount of fluid. Therefore, this finding does not indicate that oral rehydration therapy has been effective. Instead, it suggests that the child may still be dehydrated.

Choice E reason:

A heart rate of 146/min is elevated for an 8-month-old child1. Normal heart rates for infants range from 80 to 160 beats per minute. While this heart rate is within the upper limit of normal, it can also be a sign of dehydration or other stressors. Therefore, it is not a definitive indicator of effective rehydration therapy.

Choice A reason:

A low protein diet is not a therapeutic treatment for Hirschsprung disease. Hirschsprung disease is a congenital condition characterized by the absence of ganglion cells in the distal colon, leading to a lack of peristalsis and functional obstruction. Dietary modifications, such as a low protein diet, do not address the underlying issue of the absence of nerve cells in the bowel. Therefore, this choice is incorrect.

Choice B reason:

Surgical removal of the affected section of the bowel is the primary treatment for Hirschsprung disease. This procedure, known as a pull-through surgery, involves removing the aganglionic segment of the colon and connecting the healthy part of the intestine to the anus. This surgery restores normal bowel function by allowing the passage of stool through the healthy, innervated portion of the intestine. The pull-through procedure can be performed using minimally invasive techniques, resulting in shorter recovery times and fewer complications.

Choice C reason:

A permanent colostomy is not typically the first-line treatment for Hirschsprung disease. While a temporary colostomy may be performed in some cases to allow the bowel to heal before a pull-through surgery, a permanent colostomy is usually reserved for more severe cases or when other surgical options are not feasible. The goal of treatment is to restore normal bowel function, and a permanent colostomy is generally considered a last resort.

Choice D reason:

Daily enemas are not a definitive treatment for Hirschsprung disease. While enemas may be used temporarily to relieve symptoms and manage bowel movements, they do not address the underlying cause of the condition. The absence of ganglion cells in the bowel requires surgical intervention to restore normal bowel function. Therefore, daily enemas are not a long-term solution for Hirschsprung disease.