9 years old Saudi boy known case of sickle cell anemia complaining of:- Headache and irritability. S.O.B. for 2 days prior to admission. History of presenting illness. Bader was in good health till 2 days prior to admission when he started to have headache, generalized, throbbing in character. Associated with irritability.

He gave history of right side abdominal pain, progressive, radiated to right shoulder, increased by drinking coffee and eating fatty meals. Relived by drinking fevadol. Bader also has bone pain, generalized, increased by exposure to sun light, and air condition relived by fevadol.

No history of fever, cough, runny nose, change in urine color, dysuria, heameturia, change in bowel habit, jaundice, pallor, joints swelling or redness. No history of limb weakness. On the day of admission he came to ER and received I.V Fluid but no improvement.

He is a known case of S.C.A with multiple hospitalization, around 2 admission lasts around 4 to 5 days.  No other medical illness.  No surgical history.  No history of blood transfusion.

He is on folic acid and ospen “Penicillin.V” once daily with poor compliance. Not known allergic to any drug or food.

 Mother was not following regularly during antenatal period.  No prenatal complication.  Bader full term pregnancy, N.S.V.D, birth weight: 2.8 kg.  Apgar score 7 in 1/min, 8 in 3/min and 9 in 5/min.  No postnatal complication.

Complete in up to date. And received Pneumococcaal and Meningeococcal vaccines.

He is in 4 th class elementary school with good performance.

On family diet with acceptable appetite

S.C.A 44 Yrs S.C.A Carrier 34 Yrs 9 Yrs7 Yrs S.C.A Carrier 1 Yr not investigated 5 Yrs S.C.A Disease

 Father is smoker, not working  Mother is housewife  Originally from Jizan, living in Riyadh with fair income

 On arrival, Bader was looking un well, jaundiced, pale, mild dehydrated, in pain, not distressed, not tachypnia  No skin stigmata  No lymphadenopathy  No edema

 Height: 120 cm. Weight: 20 kg  H.C: 48 cm. Chest C: 60 Abdomenal C: 55 cm.  V/S:- T: 37C, P: 130 beast/min, R: 28 breath/min, Bp: 100/62 mmgh

Conscious, alert, oriented Cranial nerves: grossly intact Power Tone Reflexes Normal

No throat or ear congestion.  Apex beat in 5 th intercostal space medial to midclavicular line  S1 + S2 +O, no added sound  Palpable peripheral pulses, good volume, regular rhythm, synchronize

 No chest deformity  Equal chest expansion bilaterally  Central trachea  Good air entry bilaterally without added sounds

 Soft, tender right upper quadrant  Liver just palpable  Spleen 3 cm below costal margin  Audiable bowel sounds

 No joints swelling, redness, tenderness or restriction of movement

 Normal skin color  Normal malgenitalia with descending testes

 S.C.A with vaso-occlusive crisis

CBC WBC = 13.7  13.6 RBC = 4.61  5.08 HB = 9  12.8 PLT = 546  399 Retic = 25.0%  6.5% Sickling test =  positive

HB HBAs = 2% HBs = 98% CRP = 12 ELECTROPHORESIS

All within normal limit CHEMISTRY & L.F.T U/S ABDOMEN  Mild enlargement of the liver  Moderate enlargement of spleen  Rest of study normal X-RAY OF ABDOMEN Normal

 Patient was admitted to general word for 5 days for rehydration and to receive pain killer medication (Voltaren Injection)  Bader was improving daily becoming more active tolerating oral intake, less abdominal and bone pain  Bader did not have any attack of fever or joints swelling

 He was discharged in a good health  For follow-up in heamatology clinic  Continues using Folic acid and Penicillin as prophylaxis

DRUGS Voltaren Tab 25 mg p.o q 12 h. PCM tab. 500 mg p.o q 6 h. Morphine Inj. 5 mg Stat.

Description A chronic, severe, hemolytic disease associated with the presence of hemoglobin.

Type  S.C disease  S.C career Pathophysiology  Abnormal Hgb (Hgbs) replaces all or part of normal Hgb; under condition oxygen tension and lowered pH, RBCs changes from round to sickle or crescent shaped.

 Resulting in clumping, thrombosis, arterial obstruction, hemolysis, tissue ischaemia and necrosis  Acute and chronic changes organs and structures

 A A symptomatic exposed to precipitating factor  Dehydration, infection, fever, cold exposure, hypoxia, or extreme  Crises typically are characterized  Fever  Severe pain in joints, back, abdomen, or extremities  Weakness  Anorexia or vomiting

 Thrombocytic Crisis  Sequestrian Crisis  Aplastic Crisis

Nursing Diagnosis  Activity intolerance  Body image disturbance  Altered family processes  High risk for fluid volume deficit  Altered health maintenance  Knowledge deficit  Pain  Social isolation  Altered peripheral tissue perfusion

Planning & Implementation  Oral and IV Fluid  Electrolyte replacement  Oxygen therapy  Bed rest  Blood replacement  Relieve pain  Monitor for signs of infection  Promote tissue oxygenation avoid overexertion or emotional stress  Support the family

 Family teaching:  D isease process, including genetic  H ome management of mild crisis  R egular dental check-ups and ophthalmic examination

 The child is free from pain and infection  Parents verbalize an understanding of the disease process and major management principles