Nursing care of children with sickle cell anemia
Definition of sickle cell anemia
Sickle cell anemia is a severe chronic anemia in people having abnormal
hemoglobin called hemoglobin –S(sickle).
People who are homozygous for the gene will be affected and those who
are heterozygous will be carriers of disease
Patho physiology
When the sickle hemoglobin loses its oxygen , it forms rigid rods called
polymers that change the red blood cells into a sickle shape .these
abnormal shaped cells are both rigid and sticky. They stick to the walls
and cannot squeeze through the capillaries. Thus blood flow becomes
slow or stopped in small blood vessels. In the immediate settings,
hypoxia causes severe pain , but overtime it leads to gradual destruction
in the organs and tissues. Cell dehydration is another factor in sickling
process. Sickle cells have a short life span of 10-20 days compared to the
120 days in normal cells. This causes a drop in the number of cells
resulting in anemia.
Signs and symptoms of sickle cell anemia
Children do not become symptomatic until first year of age
 Anemia may last for 1-2 weeks .the child may have hemoglobin of
6-8g/dl
 Loss of appetite, pallor ,weakness, fever, irritability and jaundice
 Crisis may be precipitated by hypoxia, dehydration, infection
trauma, extreme activity ,fatigue, cold exposure or acidosis
 Vaso occlusive crisis (painful crisis)-most common forms
- osteoporosis
- bone and joint pain
- dactylitis ( hand foot syndrome)
- abdominal pain and spleneomegaly
- stroke
- pulmonary infarction
- altered renal function
- altered liver function and hepatomagaly
- priapism-abnormal painful constant erection of penis
 Splenic sequestration crisis: spleen becomes massively enlarged
due to pooling of blood. circulatory collapse and death can occur
 Aplastic crisis: bone marrow stops producing cells.
 Chronic symptoms related to organ damage
-jaundice
-Gall stone
-Increased infections
-Growth retardation
-Delayed puberty
-Cardiac effects
-Chronic painful ulcers
Diagnosis
Blood tests
- sickling test
- sickledex test
- hemoglobin electrophoresis
- complete blood count
Diagnostic tests for stroke
-Trans cranial Doppler
-MRI
Management
 stimulation of the production of healthy fetal hemoglobin to inhibit
sickling e.g.; Hydroxyurea
 transfusion therapy bone marrow stem cell transplantation
 prevention of precipitating causes like hypoxia, dehydrations,
stress etc
 vitamins especially folic acid, vitamin B12,and B6
 vaccination against all common infections
 pain medication
 short term oxygen therapy
Complications
-Infections
-Liver kidney or bone and joint damage
-Visual problems
-Stroke
-Gall stones
Nursing management
Nursing diagnosis
1. Acute pain related to tissue anoxia from disease process
Interventions
 give pain medications round the clock
 avoid aspirin
 identify and use effective measures to alleviate pain
-carefully position and support painful area
-hold the child if possible
-distract the child or use Diversional activities
-encourage liberal visits
- Bed rest during the acute attack
Bath the child in warm water , apply local heat or massage
-activities as tolerated
2. Ineffective tissue perfusion related to increased blood viscosity
Interventions
-prevent hypoxia
-monitor and prevent hypoxia due to opioids
-administer oxygen
Increase the fluid intake to 150ml/kg/day
Treat infection
Restrict activities during crisis
Blood transfusion as needed
3. Impaired gas exchange related to opioids and pulmonary infarction
Interventions
Monitor vitals signs, pulse oxymetry
Encourage deep breathing and coughing
Oxygen as needed
Treat acidosis if present
4. Activity intolerance related to the decreased oxygen carrying capacity
of the blood
 Assess for signs of intolerance like tachycardia, dyspnea ,dizziness,
breathlessness, change in the skin color, sweating etc
 Look for signs of fatigue
 Assess level of tolerance for activities of daily living and play
 Provide cluster care
 Assist in activities of daily living as appropriate
 Restrict activities as necessary
 Plan activities on the basis of tolerance and the child's age
 Increase activities gradually
 Encourage good eating habits, relaxation and sleep
5. Risk for infection
 avoid contact with people having respiratory infections
 thorough hand washing
 choose a room with a non infectious child
 maintain adequate nutrition
 assess for signs of infections like fever and leukocytosis
 administer antibiotics
 give meticulous care to open wounds
6. Interrupted family process related to frequent medical care, and
chronic illness
Interventions
-Encourage parents to talk about the illness and how they feel about it
-Expect negative feelings like grief, shock or guilt
-Provide factual information to the child
-Encourage role playing
-Assure the adolescent that the sexual development may be delayed but
normal
-Tell parents to treat the child like a normal child
-The child needs the same discipline like others6
7. Risk for altered growth and development
 assess the growth and development
 encourage the parents to monitor the development regularly

8. Knowledge deficit related to the causes, management sickle cell
anemia
 educate the parents about the cause, prevention of crisis and the
management
 clarify their doubts
 refer to social workers as appropriate