Sickle Cell Disease

Diagnostic Studies

- Peripheral blood smear -Sickling test(exposes to deoxygenation agent in vitro). -Hemoglobin electrophoresis (Most accurate and commonly used) Other non-diagnostics test includes: X-Rays MRI Doppler

Patient Teaching

-Avoid high altitudes -Maintain adequate fluids to avoid dehydration -Treat infections promptly -Seeking medical attention quickly( e.g.,upper respiratory infection). -Take vaccinations (e,g., Pneumonia, influenza, hepatitis)

Most Common Sites Affected by SCD Crisis Are?

-Back -Chest -Extremities -Abdomen

Chronic Leg Ulcers Treatment

-For chronic leg ulcers to treat with rest,antibiotics, warm saline soaks, mechanical or enzyme debridement and grafting may be necessary.

Other Pharmacological Treatment

-Nonsteroidal anti-inflammatory agents (NSAIDS) -Antineuropathic (e.g. tricyclic antidepressants, antiseizure medications. -Local anesthetics or nerve blocks -Broad spectrum antibiotics -Folic acid -Hydroxyuria (Drug of choice )

Sickle Cell Crisis May Require Hospitalization

1-Administer oxygen 2- Rest (to reduce metabolic requirements) 3-Administered prophylactic anticoagulant 4-Administer fluids and electrolytes to reduce blood viscosity and maintain renal function. 5- Blood transfusion may be given

Nursing Intervention

1-Alleviating symptoms 2-Minimizing organ damage 3- Treating complications promptly

Risk Factors for Sickle Cell Disease Include

1-Genetics: Having parents heterozygous for hemoglobin S 2-Ethnicity: Been of African American descent Other races that can be affected are people from Mediterranean , Caribbean, Arabian, and east Indian descent.

Acute Chest Syndrome (Complication Cont)

Acute pulmonary complications that include pneumonia, tissue infarction and fat embolism. Manifestation: Fever, chest pain, cough, pulmonary infiltrates, and dyspnea.

Hemolytic Crisis

An accelerated rate of red blood cell destruction. Manifestation: Jaundice, anemia, reticulocytosis (reticulocytes= immature blood cell).

A 13- year old patient is admitted to the hospital after presenting in sickle cell crisis. The patient reports a recent cough and fever. The nurse would question which treatment orders for a patient admitted with sickle cell crisis? Select all that apply a. Maintain Bed rest with bathroom privileges b. Administer Meperidine PRN for breakthrough pain c. Administer Oxygen for SpO2 below 90 d. Begin total RBC exchange transfusion program

B. Administer Meperidine PRN for breakthrough pain D. Begin total RBC exchange transfusion program Rationale: Meperidine contains high doses of lead and is contraindicated in SCD because of possible toxicity. Total RBC exchange transfusions are indicated for patients with frequent crisis or serious complications. This patient reports an identifiable precipitating event and there is no indication that patient experiences frequent crisis or serious complications.

The client diagnosed with sickle cell anemia comes to the emergency department complaining of joint pain throughout the body. The oral temperature is 102.4˚F and the pulse oximeter reading is 91%. Which action should the emergency room nurse implement first? A. Request arterial blood gases STAT. B. Administer oxygen via nasal cannula. C. Start an IV with an 18-gauge angiocath. D. Prepare to administer analgesics as ordered

B. Administer oxygen via nasal cannula. Rationale: A pulse oximeter reading of less than 93% indicates hypoxia, which warrants oxygen administration.

A young African American couple speak to the nurse about the desire to have a child but are concerned because the female has sickle cell anemia. Which statements indicate teaching has been effective? Select all that apply. A. I know any children we have will have sickle cell anemia but we can use hematopoietic stem cell transplantation to cure them. B. We have the option to seek genetic counseling to evaluate the likelihood of having a child with sickle cell anemia C. We really need to consider the severity of my wife's sickle cell anemia when making our decision because complications of SCD can worsen during pregnancy D. Since my husband does not have sickle cell anemia we will not pass sickle cell anemia to our child.

B. We have the option to seek genetic counseling to evaluate the likelihood of having a child with sickle cell anemia C. We really need to consider the severity of my wife's sickle cell anemia when making our decision because complications of SCD can worsen during pregnancy Rationale: Genetic counseling can evaluate the likelihood of passing sickle cell anemia to offspring and pregnancy can potentiate SCD.

Aplastic Crisis

Caused by diminished production and increased destruction of red blood cells, triggered by viral infection or depletion of folic acid. Manifestation: Profound anemia and pallor.

Hemoglobin S is Sensitive to

Changes in oxygen content in red blood cell. Insufficient oxygen causes the cell to assume a sickle shape and the cell becomes rigid and clumped together, obstructing capillary flow.

A nurse is caring for a pediatric patient with sickle cell anemia. What is the priority nursing intervention for prevention of thrombus formation, stasis of the blood and blood clotting? a. Encourage and administer fluids per orders b. Maintain bed rest per order c. Oxygen Administration per order d. Anticoagulant administration per order

Correct answer is A. Encourage and administer fluids per doctors orders Rationale: Encourage and administer fluids per doctors orders. Fluids will prevent all of the specific stated issues. Fluids will prevent and treat dehydration and fluid electrolyte imbalances which are risk factors for sickling episodes. Adequate hydration is imperative to prevent and treat sickling episodes.

A pediatric patient is being discharged from the hospital following a vaso-occlusive episode. What should the nurse include when providing discharge instructions? a. Play outdoors every afternoon to get adequate sunshine b. Pt. should wait to receive vaccinations/ immunization as they may precipitate a sickle cell crisis c. Increase fluids to 3840 ml (128 oz) daily to promote hemodilution d. Care giver should place ice packs on the joints for 15 min every four hours to limit pain and give pain medicine as needed per dr. orders

D. Care giver should place ice packs on the joints for 15 min every four hours to limit pain and give pain medicine as needed per dr. orders Rationale: Pain is the most common manifestation of a vaso-occlusive episode. Pain should be treated adequately

Folic Acid in SCD

Folic acid is often taken by sickle cell patients in order to increase the number of red blood cells in the body.

Kidney Failure (Complication Cont)

From blood viscosity and the lack of oxygen

Complications of SCD Crisis

Involvement of all body system, especially spleen, lungs, kidneys and brain.

Sickle Cell Disease

Is a group of inherited red blood cell disorders. People with SCD have abnormal hemoglobin, called hemoglobin S or sickle hemoglobin, in their red blood cells.

Hydroxyurea

Is a myelosuppressive agent. It is one of the only effective drugs proven to reduce the frequency of painful episodes. It raises the level of HbF and the hemoglobin level. It is usually decreases the rate of painful episodes by 50%. It is taken daily. This drug works by inhibiting the formation the formation of sickle shaped blood cells at the RNA, molecular level.

Sickle Cell Crisis

Is a severe , painful, acute exacerbation of RBC sicking causing a vaso-occlusive crisis. Tissue ischemia ,infarction , and necrosis eventually occurs from the lack of oxygen.

Hematopoietic Stem Cell Transplantation (HSCT)

Is the only available treatment that can cure some patients with SCD.

Autosplenectomy (Complication Cont)

Is the process where the spleen loses its function due to multiple and repeated infarctive episodes cause by sickle cells clumping and obstructing blood flow to the spleen.

Pulmonary Infarction (Complication Cont)

May cause pulmonary hypertension, MI, HF and cor pulmonale (enlargement of right side of the hearth).

Retinal Vessel Occlusion (Complication Cont)

May result in hemorrhage , scarring , retinal detachment, and blindness

Pain medication of Choice

Morphine Hydromorphone Meperidine (Demerol) is contraindicated because high doses can lead to the accumulation of a toxic metabolite, normeperidine can cause seizures.

Priapism Treatment

Pain medication fluids Nifedipine (Procardia)

Which is the Primary Symptoms Associated with SCD Crisis?

Pain, severity can range form mild to excruciating. Pain is often accompanied by fever, swelling, tenderness, tachypnea, hypertension, nausea and vomiting.,

Viral Infections (Complication Cont)

Pneumonia is the most common infection and is often pneumococcal origin. Infections can be so severe that they can cause an aplastic and hemolytic crisis and gallstones.

Other complications

Pulmonary embolism Stroke Osteoporosis Chronic leg ulcers (Prominent around the ankle) Priapism (persistent penile erection)

Sickling Episodes Can be Precipitated by

Sickling episodes are most commonly triggered by low oxygen tension in the blood. Hypoxia or deoxygenation can be caused by: -Viral or bacterial infections (Most common precipitating factors). -High altitude -Emotional or physical stress (Increases metabolic rate) -Surgery -Blood loss -Dehydration -Acidosis -Low body temp (Vasoconstriction) -Decreased plasma volume -It can happens suddenly without any reason

Clinical Manifestations

The effect of SCD vary from person to person, depending on the genetic polymorphisms. A typical patient is anemic but asymptomatic except during sicking episodes.Some symptoms can include Pallor (mainly in mucous membrane in African Americans), grayish skin, possible jaundice (can lead to gallstones).

Treatment of Sickling Episodes

The sickling episode is reversible under condition of adequate oxygenation and hydration 1-maintain adequate hydration and blood flow through oral and IV. Replace electrolytes PRN 2-Administer oxygen 3-Administer blood transfusion or exchange transfusion 4- Administer analgesic as Rx. (Best around the clock rather than PRN).

The Nursing Management for a patient in sickle cell crisis includes (Select all that apply) a. Monitoring CBC b. Optimal pain management and O2 therapy c. Blood transfusion if required and iron chelation d. Rest as needed as deep vein thrombosis prophylaxis e. administration of the IV iron and diet high in iron content

a, b, c, d