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newly diagnosed 11-month-old with Sickle Cell Anemia to your pediatric unit. The parents appear very anxious.

newly diagnosed 11-month-old with Sickle Cell Anemia to your pediatric unit. The parents appear very anxious.

Writing Assignment: The assignments must be written in paragraph format. Remember to write to the audience. Write as if you are talking to the person and teaching them. For example, “Well, Sally, I’m glad you asked about …” and end with “Do you have any questions?”

1. You are admitting a newly diagnosed 11-month-old with Sickle Cell Anemia to your pediatric unit. The parents appear very anxious. What would you say and do to prepare the parents and the infant for hospitalization? Please include at least 5 specific examples of admission teaching as well as some brief information on pain management.

Nurse Mary: Good morning Mr. & Mrs. Brown, let’s take a seat in the children’s play room so I can talk to you for a few minutes. (Allow child to sit on the parent’s lap, as this is the trust/mistrust phase)

Mr. Brown: That would be great; we don’t know what is going on and, quite frankly, nobody has really explained anything to us. What is going on with my little Jackie?

Nurse Mary: As the doctor mentioned, Jackie has anemia, specifically sickle cell anemia. This means that the red blood cells are not healthy enough to carry oxygen throughout her body. Jackie’s red blood cells are becoming rigid, sticky, and are shaped like crescent moons. These irregular red blood cells can get stuck in small blood vessels, which can slow or block blood flow and oxygen to other parts of the body.

Mrs. Brown: But how did she get this, am I not providing her with proper nutritious food?

Nurse Mary: Actually, this condition is genetically inherited. More specifically, because Jackie is carrying this active abnormal hemoglobin gene, she must have inherited it from both of you. If only one parent had passed it down, Jackie would have one normal gene and one “defective” gene. However, because she has this specific type of anemia, it indicates she received the gene from both of you.

Mr. Brown: So, how do we know for sure that this is what Jackie has? Could it be something else? What are the complications that Jackie is at risk for?

Nurse Mary: Initially, we do a sickle-turbidity test, a blood test, and check for the presence of hemoglobin S, which is the defective form of hemoglobin that underlies this anemia. If the test result is positive, hemoglobin electrophoresis, or “fingerprinting” of the protein, is necessary to distinguish between those that are carriers and those with the disease. In Jackie’s case, the results were positive for the initial test and the disease itself.

As far as complications of the condition, pain is the most common complication and this can be treated with an analgesic. Furthermore, Jackie is also at risk for infection, leg ulcers, and vision loss. The life-threatening complications are acute chest syndrome and splenic sequestration, and this needs to be treated in a hospital. Acute chest syndrome is a lung-related complication of sickle cell anemia that lowers oxygen levels in the blood and tends to develop, more often, in young children. Splenic sequestration is a complication in which too many abnormal blood cells become trapped in the spleen. As trapping continues, the spleen gets enlarged, damaged, and fails function as it should. As a result, a high risk of life-threatening infections develop.

Ms. Brown: So is there a cure for this? What can we do to make this go away?

Nurse Mary: Unfortunately, there is no cure for this because it is an inherited disease. Jackie’s treatment will be focused on supportive and symptomatic care. First, she needs to rest to minimize energy uptake, which will improve oxygen utilization. Next, we will keep Jackie hydrated, either orally and/or with IV therapy.

This condition may decrease electrolyte balance, which promotes sickling. However, it can be treated by directly replacing the needed electrolytes. Also, blood replacement may be needed to treat the anemia and to reduce the thickness of the sickled blood. In addition, because of the pain that results from the disease, we will give her analgesics, as well as antibiotics to treat any existing infection. I would highly recommend that Jackie gets immunized with the pneumococcal and meningococcal vaccine because she is susceptible to infection due to functional asplenia. Functional asplenia is when the spleen stops functioning as an organ.

Mr. Brown: So what about when we go home, how do we take care of her then?

Nurse Mary: That’s a great question. First, you need to seek immediate medical attention if Jackie gets a fever of 38.5C. Next, you will need to be able to recognize any respiratory problems that can lead to hypoxia. Also, if prescribed by the doctor as preventive care for infections, Jackie will need penicillin. Although Jackie can get sick in ways that other children cannot, don’t forget that she is a normal child and should be treated as such in all aspects possible.

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