Sickle Cell Anemia: Diagnosis and Management (Pediatric Nursing)

by Paula Ruedebusch

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    00:01 How do we diagnose sickle cell anemia? Well genetic counselling is available for parents known to have sickle cell disease or trait.

    00:09 Diagnosis of sickle cell anemia is also done as part of the routine newborn screening done in most states.

    00:16 There are benefits to early diagnosis.

    00:18 These patients can have prevention and relief of pain episodes.

    00:22 You can try to prevent infections, organ damage and strokes.

    00:27 You can try to treat anemia and control complications and these patients can be offered counselling, prophylaxis, increased immunizations and follow-up information regarding research.

    00:40 Managing a patient with sickle cell disease is complex.

    00:43 The patient may need the bed rest during an acute vaso-occlusive crisis and increased fluid therapy - this is key.

    00:50 Yyour patient may need one and a half or two times their normal maintenance.

    00:54 A blood transfusion of packed red cells can help in the setting of severe anemia and analgesics are used to relieve pain.

    01:01 There should be a really low threshold in treating a patient with sickle cell anemia who has an infection.

    01:07 Antibiotics are usually started early as the patient's immune system is already compromised.

    01:13 A splenectomy is the surgical removal of the spleen and this is done in order to prevent recurrence of certain complications.

    01:19 Your patient may need supplemental oxygen and this oxygen is gonna be administered because remember the red blood cells already have dysfunctional hemoglobin and this makes a decreased oxygen carrying capacity.

    01:30 Supplemental oxygen can help manage the hypoxia and crisis symptoms.

    01:36 To help prevent a crisis, patients are gonna want to try to avoid infections.

    01:41 They're also going to want to decrease their exposure to cold because of the vasoactivity that happens.

    01:47 Same thing goes with stress.

    01:50 Patients are gonna want to be non smokers and they want to avoid becoming dehydrated.

    01:56 You'll have a really low threshold for rehydrating a patient with sickle cell disease.

    02:01 A patients with sickle cell disease can go into a crisis in a hypoxic environment so they're gonna avoid going to high altitude areas or maybe climbing tall mountains.

    02:12 Patients with sickle cell anemia can also prevent a crisis by taking prophylactic antibiotics.

    02:17 All children's sickle cell disease you should take a penicillin twice a day until at least age 5 to prevent pneumococcal sepsis.

    02:25 You want to be sure your patients with sickle cell disease are up-to-date on their vaccinations and this decreases the likelihood that they'll get these vaccine-preventable diseases including influenza and pneumococcal.

    02:36 Children with severe types of sickle cell disease should be offered primary stroke prevention with annual transcranial Doppler scans and blood transfusions when resources allow.

    02:47 Hydroxyurea is a medication, it's a myelosuppressive agent that's the only effective drug proven to reduce the frequency of painful episodes.

    02:55 It raises the hemoglobin level and decreases the rate of painful episodes by about 50% Education is key in preventing a crisis as this is a lifelong chronic disorder.

    About the Lecture

    The lecture Sickle Cell Anemia: Diagnosis and Management (Pediatric Nursing) by Paula Ruedebusch is from the course Blood Disorders – Pediatric Nursing.

    Included Quiz Questions

    1. 5–6 months
    2. 2–3 months
    3. 7–9 months
    4. 10–12 months
    1. Preventing infection
    2. Avoiding exposure to the cold and high altitude
    3. Stopping smoking
    4. Maintaining hydration
    5. Having genetic counseling

    Author of lecture Sickle Cell Anemia: Diagnosis and Management (Pediatric Nursing)

     Paula Ruedebusch

    Paula Ruedebusch

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