Sickle cell anemia

Sickle cell anemia - Acute

 

Definition  

A group of inherited disorders of hemoglobin synthesis that includes (in decreasing order of frequency), sickle cell anemia (SS disease), sickle cell hemoglobin C disease (SC disease), and sickle cell thalassemia (SB (+) and SB (-) disease), in which diminished red blood cell deformability leads to increased red blood cell destruction and occlusion of the microvasculature

 

Medical History  

* Pelvic infection, acute

* Menstruation finding

* Exposure to cold

* High altitude illness

 

Findings  

* Hematuria - Acute

* Hypotension

* Bone pain

* Depression

* Dyspnea - Acute

* Proliferative retinopathy due to sickle cell disease

* Psychotic disorder - Acute

* Syncope - Acute

* Tachypnea

* Abdominal pain - Acute

* Abnormal breath sounds

* Abnormal fourth heart sound, S4

* Abnormal second heart sound, S2

* Abnormal third heart sound, S3

* Acholic stool

* Asthenia

* Backache

* Chest pain - Acute

* Cyanosis

* Dysphagia

* Dysuria

* Fatigue

* Flank pain

* Headache

* Hepatomegaly - Acute

* Increased body temperature

* Increased heart rate

* Jaundice

* Joint swelling

* Malaise

* Mid-systolic murmur

* Musculoskeletal tenderness

* Myalgia

* Nausea and vomiting - Acute

* Nocturia

* Nuchal rigidity

* Pallor

* Paralysis

* Periorbital edema

* Peripheral edema

* Point of maximal impulse finding

* Polyuria

* Seizure

* Skin ulcer

* Splenomegaly

* Sputum finding

* Swelling of skeletal muscle

* Urinary incontinence

* Widened pulse pressure

 

Tests  

Suspected or known anemia  

* Reticulocyte count: The reticulocyte count is essential for differentiating between anemias arising from nonfunctional versus functional bone marrow .

 

Suspected sickle cell disease  

* Peripheral blood smear examination, light microscopy: The peripheral blood smear is helpful in establishing the diagnosis of sickle cell disease.

 

Suspected and known sickle cell disease  

* Complete blood count: Baseline steady-state blood counts for adults and children with sickle cell disease may change during the acute alterations that interrupt the otherwise chronic course of SS disease.

 

Suspected and known sepsis  

* Blood culture: In patients with suspected sepsis, at least 2 sets of blood cultures should be obtained, preferably from peripheral venipuncture before antimicrobial therapy is initiated, if obtaining such cultures does not cause a significant delay in antibiotic administration .

 

Suspected hypoxia  

* Pulse oximetry: An oxygen saturation of at least 90% is acceptable in most patients .

 

Acute chest syndrome  

* Plain chest X-ray: Chest x-ray is useful in the evaluation of sickle cell patients presenting with chest pain and respiratory symptoms.

 

Initial immediate imaging evaluation for suspected acute ischemic stroke  

* CT of head: A noncontrast CT scan accurately identifies most cases of intracranial hemorrhage and helps discriminate nonvascular causes of neurological symptoms .

 

Suspected osteomyelitis  

* Radiographic imaging of bone: The first changes seen include soft-tissue swelling and periosteal elevation. Radiolucency and sequestrum formation may be seen later in the disease.

 

Dactylitis  

* Radiographic imaging of bone: Films of the hands and feet in sickle cell patients with dactylitis show periostitis, bone reabsorption, and extensive osteolysis .

 

Sickle cell anemia  

* Pulmonary function test: Restrictive respiratory patterns in adults (associated with acute chest syndrome) and airway hyper-reactivity in children are findings in sickle cell disease .

 

Differential Diagnosis  

* beta Thalassemia

* Red blood cell sequestration in spleen

* Hemoglobin SS disease with vasoocclusive crisis

* Aplastic crisis

* Sickle cell trait

* Glucose-6-phosphate dehydrogenase deficiency anemia

* alpha Thalassemia

* Hemolytic crisis

* Dactylitis

* Deficiency of pyruvate kinase

* Hereditary spherocytosis

* Autoimmune hemolytic anemia

* Acute chest syndrome

* Ischemic infarction of muscle

* Iron deficiency anemia - Chronic

 

Treatment  

Drug Therapy  

 

Suspected and known vaso-occlusive crisis  

 

MORPHINE SULFATE  

Adults: 2 to 10 mg IV over 4 to 5 minutes

Pediatrics: 0.1 to 0.2 mg/kg subQ or IV every 2 to 4 hours as needed (maximum 15 mg/dose)

 

HYDROMORPHONE HYDROCHLORIDE  

Adults: 1 to 2 mg subQ, IM, or IV every 2 to 3 hours as needed

Pediatrics: 0.015 to 0.020 mg/kg IV every 3 hours as needed

 

KETOROLAC TROMETHAMINE  

Adults (<65 years): 60 mg IM or 30 mg IV as a single dose OR 30 mg IV or IM every 6 hours (maximum 120 mg/day)

 

ACETAMINOPHEN/CODEINE PHOSPHATE  

Adults: 1 to 2 tabs orally every 4 hours as needed (maximum 360 mg codeine/4 g acetaminophen/day)

Pediatrics (3 to 6 years): 5 mL (12 mg codeine/120 mg acetaminophen per 5 mL) orally every 6 to 8 hours as needed

Pediatrics (7 to 12 years): 10 mL (12 mg codeine/120 mg acetaminophen per 5 mL) orally every 6 to 8 hours as needed

 

FENTANYL  

 

 

Empiric therapy for sepsis  

 

CEFTRIAXONE SODIUM - VANCOMYCIN HYDROCHLORIDE  

Pediatrics (>28 days): Ceftriaxone 100 mg/kg IV every 24 hours AND vancomycin 15 mg/kg IV every 6 hours

 

CEFOTAXIME SODIUM - VANCOMYCIN HYDROCHLORIDE  

Pediatrics (>28 days): Cefotaxime 50 mg/kg IV every 8 hours AND vancomycin 15 mg/kg IV every 6 hours

 

IMIPENEM/CILASTATIN - VANCOMYCIN HYDROCHLORIDE  

Adults: Imipenem/cilastatin 500 mg IV every 6 hours AND vancomycin 1 g IV every 12 hours

 

 

Empiric therapy for suspected Salmonella or Staphylococcus aureus osteomyelitis  

 

CEFTRIAXONE SODIUM - VANCOMYCIN HYDROCHLORIDE  

Pediatrics (>28 days): Ceftriaxone 50 to 75 mg/kg IV once daily AND (methicillin-resistant S aureus possible) vancomycin 10 to 15 mg/kg IV every 6 hours

 

CIPROFLOXACIN  

Adults: 400 mg IV every 12 hours

 

LEVOFLOXACIN  

Adults: 750 mg IV every 24 hours

 

 

Aplastic crisis  

 

IMMUNE GLOBULIN  

Adults: 0.4 mg/kg IV infusion

 

 

Prevention of pneumococcal infection  

 

PNEUMOCOCCAL 7-VALENT VACCINE, DIPHTHERIA CONJUGATE  

Pediatrics (primary series): 0.5 mL IM x 4 doses given at 2 months (minimum age of 6 weeks), 4 months, 6 months, and 12 to 15 months

Pediatrics (catch-up dose, healthy children) 0.5 mL IM x one dose for children aged 24 to 59 months who have not received at least one dose of pneumococcal conjugate vaccine (PCV) on or after age 12 months

Pediatrics (catch-up dose, underlying medical conditions): 0.5 mL IM x one dose for children aged 24 to 59 months who have received 3 prior doses of PCV

Pediatrics (catch-up dose, underlying medical conditions): 0.5 mL IM x 2 doses, at least 8 weeks apart, for children aged 24 to 59 months who have received less than 3 prior doses of PCV

 

PNEUMOCOCCAL VACCINE POLYVALENT  

Adults (primary vaccination): 0.5 mL IM x one dose given to all adults 65 years of age and older

Adults (revaccination): 0.5 mL IM x one dose given to all adults 65 years of age and older who were vaccinated before the age of 65 and at least 5 years prior

Adults (at high-risk): 0.5 mL IM x one or 2 doses, at least 5 years apart, for adults less than 65 years of age who have medical indications for vaccination or are part of high risk populations

Pediatrics (2 to 18 years): 0.5 mL IM or subQ as a single dose; may be repeated once after 5 years in selected cases

 

PENICILLIN V POTASSIUM  

Pediatrics (<3 years): 125 mg orally twice daily

Pediatrics (>3 years): 250 mg orally twice daily

 

AMOXICILLIN  

Pediatrics: 20 mg/kg/day orally

 

 

Prevention of influenza  

 

INFLUENZA VIRUS VACCINE (SUBVIRION)  

Adults: One dose of 0.5 mL IM annually

Pediatrics (6 to 35 months): One dose of 0.25 mL IM annually. If receiving the vaccine for the first time, the dose should be repeated once after 4 or more weeks .

Pediatrics (3 to 8 years): One dose of 0.5 mL IM annually. If receiving the vaccine for the first time, the dose should be repeated once after 4 or more weeks .

Pediatrics (9 to 18 years): One dose of 0.5 mL IM annually

 

 

Prevention of aplastic crises  

 

FOLIC ACID  

Pediatrics (infants): 0.1 mg/day orally

Pediatrics (<4 years): Up to 0.3 mg/day orally

Pediatrics (>4 years): 1 mg/day orally

 

 

Reduction of painful episodes in severe sickle cell disease  

 

HYDROXYUREA

Adults: 10 to 15 mg/kg/day in a single daily dose for 6 to 8 weeks; based on hematologic monitoring; may increase dose by 5 mg/kg/day every 6 to 8 weeks to the maximum tolerated dose or to 35 mg/kg/day (maximum recommended dose)

 

 

Adjunct in chronic pain management  

 

AMITRIPTYLINE HYDROCHLORIDE  

Adults: 25 to 100 mg orally daily; 90% of patients achieve optimal effect with 50 to 75 mg/day; therapy usually begins with 25 mg at bedtime and is increased in 25 mg increments at weekly intervals

Adolescents: 25 mg orally at bedtime; increase to 50 mg at bedtime after six weeks as needed (maximum 100 to 200 mg/day)

 

 

Procedural Therapy  

 

Dehydration  

* Intravenous fluid replacement: Intravenous fluid replacement is indicated for patients with dehydration when oral replacement cannot be accomplished .

 

Hypoxemia  

* Oxygen therapy

 

Severe anemia caused by aplastic crisis  

* Transfusion of blood product: Urgent replacement of blood is indicated for treatment of sudden severe anemia associated with severe or long-lasting aplastic crisis .

 

Hemolytic crisis  

* Transfusion of blood product: A simple transfusion of packed red blood cells usually raises the hemoglobin level to baseline (6 to 10 g/dL).

 

Severe anemia caused by acute splenic sequestration  

* Transfusion of blood product: Acute splenic sequestration requires emergent correction of hypovolemia with red blood cell transfusion

 

Vaso-occlusive crisis  

* Transfusion of blood product: Transfusions may be useful for treatment of refractory or protracted painful crises but probably are not indicated for patients with uncomplicated painful episodes .

 

Acute chest syndrome with hypoxia  

* Transfusion of blood product: Transfusion is indicated in acute chest syndrome when PaO2 is below 70% on room air or baseline PaO2 decreases by 10% .

 

Acute ischemic stroke in children with sickle cell disease  

* Exchange transfusion: Immediate exchange transfusion is indicated for treatment of ischemic stroke in children with sickle cell disease .

 

At risk of stroke  

* Transfusion of blood product

 

Cholecystitis  

* Laparoscopic cholecystectomy: Laparoscopic cholecystectomy is an alternative to open cholecystectomy and is more frequently performed in otherwise healthy patients.

 

Sickle cell disease  

* Bone marrow transplant: Allogenic bone marrow transplant (BMT) from a matched sibling donor without sickle cell disease may provide a cure in severe disease .

 

Non-Procedural Therapy  

 

Dehydration  

* Oral rehydration

 

Bone necrosis  

* Crutches