This article presents a case report that discusses the anaesthetic management of a patient with sickle-thalassemia scheduled for total hip replacement.

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Sickle cell disease is a congenital condition, and its most common clinical manifestation is anaemia due to chronic hemolysis. β-thalassemia is a quantitative issue of β-globin chain synthesis. Sickle syndromes can result from inheriting HbS from one parent and another hemoglobinopathy, such as β-thalassemia or HbC, from the other parent.

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Radiological examination findings

1. X-ray chest: Generalised osteopenia, Rib in Rib appearance
2. X-ray B/L hip: Avascular necrosis of the left hip, medullary widening with cortical thinning, reduced joint space, generalised osteopenia, a periosteal reaction in the greater trochanter
3. X-ray L-S spine: Wedge compression fracture of L2 vertebra with a collapsed body, generalised osteopenia, and kyphosis at mid-lumbar level

Treatment

1. The patient received multiple blood transfusions preoperatively, with a final haemoglobin level of 9.8 g%.
2. Six hours of fasting were ensured, during which Ringer's lactate solution was administered to prevent dehydration and sickling.
3. On the day of surgery, after obtaining written informed consent, a prophylactic antibiotic (Inj. Cefoperazone + Sulbactam) was administered 1 hour before the surgical incision.
4. A femoral nerve block was administered using 10 ml of 0.5% Bupivacaine to facilitate patient positioning for regional anaesthesia.

Combined spinal-epidural anaesthesia was performed at the L3-L4 level using 0.5% heavy Bupivacaine, and an epidural catheter was placed 5 cm into the epidural space. Intraoperatively, oxygen was administered via a face mask, intravenous fluids were continued, and a warmer was applied to prevent hypothermia. The total duration of surgery was 2 hours and 30 minutes, with an estimated blood loss of 550 ml. One pint of packed red blood cells (300 ml) was transfused. The intraoperative period was uneventful.

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Discussion

Sickle cell syndromes are caused by a mutation in the β-globin chain of haemoglobin, where valine replaces glutamic acid at position 6. These syndromes include sickle cell anaemia, sickle cell trait, haemoglobin SC disease, and sickle β-thalassemia, which result from inheriting HbS from one parent and another hemoglobinopathy (e.g., β-thalassemia or HbC) from the other.

Sickling of erythrocytes occurs in conditions such as hypoxia, dehydration, acidosis, infections, cold temperatures, high altitudes, stress, and anaemia. These factors reduce solubility and cause reversible polymerisation of sickle haemoglobin (HbS), making the red blood cells rigid and deformed. This leads to increased intracellular viscosity, obstructing microvessels, accelerating red blood cell destruction, and causing organ damage.

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Anaesthetic concerns and management

Preoperative:

• Severe anaemia
• Infection, fever
• Blood transfusion complications
• Pain crises
• Hydration

Intraoperative:

• Choice of anaesthesia
• Preventing hypoxia, acidosis, and hypothermia
• Hydration and blood loss management

Postoperative:

• Pain management
• Avoiding hypoxia and hypothermia
• Hydration
• Monitoring for complications like acute chest syndrome, thromboembolic events, and infections

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Collaborative care

Sickle thalassemia requires a multidisciplinary approach for optimal patient outcomes, involving haematologists, anesthesiologists, surgeons, intensivists, and physiotherapists.

1. Preoperative preparation

Blood transfusions are essential to raise haemoglobin levels to 9-10 g/dL and reduce HbS levels. Proper hydration and infection screening, particularly for respiratory infections, are crucial. A thorough evaluation of end-organ function (e.g., spleen, heart, kidneys) guides perioperative care.

2. Intraoperative management

Regional anaesthesia (spinal or epidural) is preferred to minimise stress and hypoxia. For general anaesthesia, careful monitoring of oxygenation and ventilation is critical. Hypothermia is managed with warming measures, and adequate IV hydration with crystalloids prevents hemoconcentration. Preventing hypoxia, acidosis, and hypothermia is key.

3. Postoperative care

Postoperative care includes pain management, oxygen therapy, early mobilisation, and monitoring for complications like acute chest syndrome, thromboembolic events, and infections.

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Disclaimer- The views and opinions expressed in this article are those of the author and do not necessarily reflect the official policy or position of M3 India.

About the author of this article: Dr N Chinna Siddeswarappa is an Assistant professor (Anaesthesiology dept.) at Meenakshi Medical College Hospital And Research Institute, Kanchipuram.