Sickle Cell Anemia: Diagnosis and Management (Pediatric Nursing)

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.