Sickle Cell Diease

sickle cell disease (SCD)

a group of inherited, autosomal recessive disorders characterized by an abnormal form of hemoglobin in the RBC.

Median survival for sickle cell disease is...

now exceeds 45 years

types of sickle cell disease

- sickle cell anemia

- sickle-cell thalassemia

- sickle cell Hgb diease

- sickle cell trait

sickle cell results in

irreversible damage to the lungs, kidneys, brain, retina, bones

Sickled RBCs

are stiff and angular, causing them to become stuck in small capillaries

sickle cell hemoglobin

forms long inflexible chains

Sickling episodes are triggered by

low O2 tension in the blood

- a bacterial or viral infection ( most common cause)

- dehydration (common cause)

Events that can cause sickling

- Increased hydrogen ions (acidosis)

- Decreased plasma volume

- Low body temp

- High Altitudes

- Emotional or physical stress

- Surgery and blood loss

- Sometimes no obvious reason

Initial course of sickling episodes

- sickling can be reversed with re-oxygenation

- eventually becomes irreversible

- vaso-occlusive phenomena and hemolysis are clinical hallmark signs of SCD

sickle cell crises

- severe painful, acute exacerbations (pain)

- blood flow to tissues is impaired (perfusion)

- severe hypoxia (gas exchange)

- tissue ischemia (tissue integrity)

- hemoconcentration (clotting)

sickle cell crises characteristics

- can have a sudden onset and last for days

- severity depends on percent of HBGs present

sickle cell disease symptoms

- typically in good healthy mostly

- the typical client has asymptomatic anemia except during episodes

- occurs more frequently in dark-skinned individuals

-- assess pallor in mucous membranes

-- skin may appear grayish

-- jaundice

-- increased gallstones

- pain in back,chest, extremities, and abdomen

- fever, swelling, tenderness, tachypnea, hypertension, N&V

peripheral blood smear

reveals sickled cells and increased amount of reticulocytes

Hemoglobin electrophoresis

- determines the amount of hemoglobin S

- skeletal X-rays show bone/joint deformities and flattening

Nursing Management goals for sickle cell disease

- prevent sequelae from diease

- alleviate manifestations from complications

- minimize end organ damage

- promptly treat serious sequelae

-- acute chest syndrome

Sickle cell causes morbidity and mortality

- infection- failure of spleen to phagocytize foreign substances (usually 2-4 years of age)

- spleen becomes small r/t repeated scarring (auto splenectomy)

- pneumonia- the most common infection

-- can trigger crisis (temporary shutdown of RBC production in bone marrow

Acute Chest Syndrome (ACS)

- acute pulmonary complications

- includes: pneumonia, tissue infarction, and fat embolism

- characteristics: fever, chest pain, cough, lung infiltrates, dyspnea

-- can lead to pulmonary HTN, MI, cor pulmonale and heart failure

retinal vessel obstruction

- results in hemorrhage, scarring, retinal detachment, and blindness

- increased blood viscosity and decreased oxygen- kidney damage

- pulmonary embolism/stroke can be a result of thrombosis and infarction of cerebral blood vessels

- bone osteoporosis and osteosclerosis-- chronic leg pain

- chronic leg ulcers from hypoxia around ankles

- priapism- may occur if the penile vein becomes occluded

splenic sequestration

- may require splenectomy as a life-saving measure in children

- usually atrophies by 6 years of life

nursing care for sickle cell disease

- assessment- auscultate lungs

-- note SPO2 levels, adventitious sounds

- diagnose-identify problems

Sickle cell disease interventions

- restore and maintain oxygen saturations greater than 90%

-- acute stage: high flowers, supplemental oxygen, adminster fluids as prescribed

-- ongoing interventions: encourage IS, encourage rest, prevent DVT, treat priapism with nifedipine

- Transfusion-indicated with aplastic crisis occurs

-- RBC exchange programs may be initated for individuals with frequent crises or serious complications such as acute chest crises

- done sparingly because client's develop antibodies for RBC and iron overload

iron chelation therapy

if thalassemia to reduce transfusion-produced ion overlaod

Pain management- often undertreated

- R/T lack of understanding of the severity

- Client may develop tolerance- requires larger doses of pain medication

- Optimal pain includes large doses of continuous opioids during crises

-- delivered as patient-controlled analgesia (PCA) morphine and hydromorphone

-- NSAIDs, anti neuropathic

Febrile client is an emergency

treat ulcer infections with rest, antibiotics, warm saline socks, mechanical or enzyme debridement, and grafting if needed

hydroxyurea (Hydrea)

only beneficial medication (is a chemotherapy)

-- increases production of Hemoglobin F (fetal hemoglobin)

-- reduces hemolysis

-- increases hemoglobin concentration

-- decreases sickle cells and painful crises

dietary supplementation

-- oral glutamine (an amino acid)- can reduce frequency and pain crises and reduce hospitalizations

hematopoietic stem cell transplant (HSCT)

- effective life-saving procedures, but may have delayed complications that impact the quality of life

- only treatment that can cure some clients

- not widely available

- selection of recipients, scarcity of appropriate donors, risk and cost limit the use

patient and parent education for sickle cell disease

- avoid high altitudes

- maintain adequate hydration

- encourage immunizations

- encourage visual assessments for retinopathy beginning at age 10

- develop a reproductive life plan

- teach pain control

Special Pediatric Considerations for sickle cell disease

- educate parents on specific instructions on how many daily fluids

- parents who are unaware may restrict fluids at night to prevent bedwetting

- increase fluids during exercise/strenuous activities, warm weather

- increase fluids during times of emotional stress

- presence of dactylitis, severe anemia, and leukocytosis can predict severity