This document summarizes the anesthetic management of a 22-year-old male patient with sickle cell anemia who underwent bilateral hip replacement surgery. Key points include: - The patient underwent an exchange transfusion the night before surgery to reduce his HbS level from 68% to 31%. - He received combined spinal-epidural anesthesia for the first surgery and spinal anesthesia for the second surgery to adjust the prosthesis. - Intraoperatively he experienced tachycardia, blood pressure fluctuations, and low oxygen saturation which were managed with various medications. - Postoperatively he developed an allergic reaction to morphine which was treated. His blood counts decreased requiring filgrastim and multiple

1. Anaesthesia in Sickle Cell
Disease
A Case Presentation
Dr. Sunder Chapagain
Dept. of Anaesthesia and
Surgical ICU
Patan Hospital

2. Particulars
• Name: Mr. Chaudhary
• Age: 22 years
• Sex: Male
• Address: Kailali, Nepal (Terai region)
• Occupation: Student
Date of admission: 19th
Chaitra 2074 (2nd
April, 2018)
Date of Surgery: 21st
Chaitra 2074 (4th
April, 2018)
Diagnosis: Bilateral Avascular Necrosis of femoral head with secondary
osteoarthritis with known case of Sickle Cell Anemia

3. • Chief Complains:
• Pain over left hip and knee for about 10 years, aggravated since 3 months.
• Past Medical History:
• Known case of Sickle Cell Anemia diagnosed 4 years back under medication.
• History of blood transfusion done 4 years back
• History of repeated hospital admission (about 1 time/month) due to bone
pain.
• No history of chronic diseases like hypertension, diabetes or tuberculosis

4. • Drug History:
• Tab. Hydroxyurea 500 mg PO BD X 4years
• Tab. Folic acid 5 mg PO OD X 4 years
• Past Anaesthetic History:
• No history of any surgeries and anaesthetic exposure in past
• Allergic History:
• No history of allergy to any drugs, food or substances.
• History of Bleeding Disorders:
• None

5. • Personal History:
• Non vegetarian by diet, does not consume alcohol, non-smoker
• Family history:
• Cousin sister – Sickle cell anemia
• No history of similar disease in his other family members
• Socioeconomic History
• Middle class family

6. Examination
• General Condition - fair
• No pallor, icterus, lymphadenopathy, dehydration or thyroid swelling
• Vital signs:
• BP: 120/70 mm Hg
• Pulse: 82 bpm
• RR: 14/min
• Temperature: 98.4 Degree Fahrenheit
• Weight: 40 kg
• Airway Assessment:
• Mouth Opening : >2 fingers
• Thyromental Distance : > 3 fingers
• Neck mobility: Free
• Thyromental Joint: Free
• Mallampati Grade: II
• No Loose or False teeth

7. • Spine:
• No any infection at the site of injection
• No superficial skin disease, swelling or local tenderness
• No any obvious deformity
• Respiratory : Bilateral equal air entry, normal vesicular breath sounds,
no added sound
• Cardiovascular: S1S2M0

8. Routine Investigations
• Complete Blood Count
• Hb: 12.9 g/dl
• HCT: 39%
• TC: 4100/uL
• Platelets: 150000/uL
• Renal Function Test
• Urea: 22 mg/dl
• Creatinine: 0.9 mg/dl
• PT/INR : 12/1.0
• ESR: 5 mm/1st
Hour
• LDH: 166
• Liver Function Test:
• Total Bilirubin: 3.3
• Direct Bilirubin: 1.8
• SGOT: 19
• SGPT: 20
• Blood Grouping: B Positive
• Serology: Non- reactive
• Urine RME: Within Normal Limits
• Chest X-ray: Normal
• ECG: Sinus rhythm, Ventricular
rate: 70/min

9. Investigations for diagnosis
• X-ray of bilateral hips: Avascular necrosis of bilateral femoral head
• CT scan bilateral hip:
• Multiple areas of osteonecrosis in pelvic bone and proximal femurs
• Mild degenerative changes in the right hip joint and severe degenerative
changes in the left hip joint.
• Hemoglobin Electrophoresis done 2 weeks prior to Surgery date:
• HbS : 68%
• HbA2 : 2.5%
• HbF : 29.5%

10. • Anaesthetic Plan: Regional Anaesthesia
• Combined Spinal Epidural Anaesthesia with adjuncts
• ASA Grading : ASA I
• Surgey: Left Sided Total Hip Replacement

11. Preoperative Preparation
• Pre-anaesthetic evaluation done on 20th
Chaitra
• All Preoperative investigations sent and reports reviewed.
• Advice
• NPO for 8 hours before surgery
• High Risk Consent
• Arrange and crossmatch Blood and blood products according to the need
• Hematologist consulted:
• Planned for exchange transfusion on the night before surgery
• Shifted to Surgical ICU
• Central venous access and a peripheral wide bore (16 G) canula opened.
• Exchange transfusion (manually) started at 9 pm on night before surgery
• Total exchange volume calculated as 1200 ml
• Goal: to decrease HbS level from 68% to upto 30%

12. Pre Exchange transfusion
• Hemoglobin : 12.9g%
• HCT : 39%
• Platelets : 150000/uL
• Total Count : 4100/uL
• HbS : 68%
• HbF : 29.5%
Post exchange transfusion
• Hemoglobin : 13.6g%
• HCT : 41%
• Platelets : 150000/uL
• Total Count: 2600/uL
• HbS : 31.4%
• HbF : 9.7%

13. Intraoperative management
• Regular monitoring parameters attached
• NIBP, Pulse Oxymetry, ECG, Temperature
• Anaesthesia: Combined Spinal Epidural Anesthesia with adjuncts
• Epidural Anaesthesia:
• Sitting Position
• L3-L4 level
• By Loss Of Resistance technique
• Test dose 3ml of 2% xylocaine with adrenaline
• Spinal Anaesthesia:
• Sitting Position
• L4-L5 level
• Inj. 0.5 % Heavy Bupivacaine 3.2 ml + 10 mg (0.2ml) pethidine

14. Intraoperative issues
• Tachycardia: 130-160bpm- managed with
• Inj. Midazolam 1 mg 5 times (anxiety)
• Inj. Fentanyl 100mcg boluses 2 times (Pain)
• Esmolol 10 mg bolus 11 times
• Blood pressure Fluctuating: From 80/40 mm Hg to 120/70 mm Hg
• Inj. Phenylephrine 100 mcg bolus (total 1500 mcg given)
• Total 4 pint IV fluids (RL) given
• Oxygen Saturation maintained at 100% with O2 at 6 liters/min via mask.
• GRBS after 2 hours of starting Surgery was 120 mg/dl
• Urine output : 600 ml
• Blood loss: 500 ml

16. Immediate Postoperative Period
• Post Operative Nausea and vomiting (PONV) prophylaxis
• Inj. Ondansetron 4 mg IV
• Postoperative Pain
• Inj. Morphine 2mg via epidural catheter
• Complained of itching all over the body within 2 minutes of giving morphine
• Inj. Hydrocortisone 100 mg Iv stat given for allergic reaction

17. Postoperative Management
• Day of Operation:
• Shifted in SICU for observation after Operation
• Vital signs monitored and quick examination
• Inj. Tramadol and Inj. Paracetamol for pain management.
• Tachycardia : Tab. Metoprolol 12.5 mg and Inj. Esmolol 10 mg boluses
• Hypotension: managed with warm IV fluids (low CVP)
• Hypothermia prevented using warm electric blankets, warm fluids, warm
ambient room temperature. Strict hourly temperature monitoring done.
• No complains of acute chest pain, shortness of breath or blurring of vision in
postoperative period.

18. Postoperative Management
• Post operative Investigations and management
• Hb: 9 g/dl and Hematocrit 27%
• 2 pint Packed Cell transfused
• Arterial Blood Gas:
• pH – 7.44
• pO2- 71 mm Hg (↓)
• pCO2- 22.5 mm Hg (↓)
• HCO3– 18.3 mmol/L (↓)
• Kept in Oxygen at 5L/min via normal mask.

19. Postoperative Management
• 1st
Postoperative day:
• No fresh complains
• Total counts decreased to 1900/uL
• Medicine consutation done
• Adviced to stop Hydroxyurea

20. • 2nd
Postoperative day:
• Total count dropped to 1500/uL
• Inj. Filgrastim 300 mcg SC OD for 2 days
• Hemoglobin : 8.6 g/dl
• 1 Pint PRBC transfused
• His total counts increased gradually
• Shifted to ward on his 4th
Post Operative day.
• In Ward:
• Check X-ray bilateral hip
• Misalignment of Prosthesis
• Hemoglobin electrophoresis
• HbS : 20.6%

21. Anesthetic management for Second
Operation
• Next operation was planned 1 week later to 1st
Operation
(Readjustment of prosthesis for misalignment)
• Anesthesia : Spinal anesthesia
• 3.2 ml 0.5% heavy bupivacaine + Inj. Pethidine 10 mg (0.2ml)
• As the patient was more anxious and complaining of pain:
• Inj. Midazolam 2mg
• Inj. Fentanyl given as per need (total 150 mcg)
• Inj. Ketamine

22. • Patient was shifted to SICU for observation after surgery.
• No immediate postoperative complications noted.
• Post operative hemoglobin on the same day : 8.6 g/dl
• Total of 1 Pint PRBC transfused.
• No fresh complains by patient and he was clinically stable
• Shifted to ward on the next day of Surgery.

23. Thank You