27-year-old sickle cell disease patient undergoes multiple joint replacement surgery

Fortis Hospitals Mulund, Mumbai, performed a multiple joint replacement surgery on a 27 year old young Nigerian patient. Rebacca underwent a hip and a shoulder replacement surgery by Dr. Sachin Bhonsle, Consultant Orthopedic Surgeon, after years of suffering in pain and immobility.

Rebecca was suffering from a sickle cell disease since birth; an inherited (genetic) disorder where the RBC (red blood corpuscles) assumes an abnormal shape of a sickle under certain conditions. Any stress, injury, or operation can lead to change in shape of the RBCs. But they are extremely sensitive to drop in oxygen saturation of blood. This causes immediate 'sickling'.

Sickle cells contain abnormal haemoglobin, known as 'haemoglobin S', which causes the cells to have a sickle shape. Sickle-shaped cells don't move easily through blood vessels. The clumps of sickle cells block blood flow in the blood vessels that lead to the limbs and organs. Blocked blood vessels can cause pain, serious infections, and organ damage. It can be at times life threatening.

Commonest problems encountered are: 1. Sickle crises - painful abdominal emergencies because of blockage of blood vessels to abdominal viscera. 2. Anaemia - red blood cells are easily destroyed and their life span is reduced causing anaemia. 3. Avascular necrosis (AVN) of bones - certain bones with peculiar blood supply undergo AVN because of blockage of the artery. Once the blood circulation is lost the bone crumbles and the adjoining joints undergo arthritis. Commonest are hip and shoulder joints. Remodelling of entire skeleton is affected sometimes.

In Rebecca's case the disease was severe to the degree that 93% of her haemoglobin was abnormal. This resulted in extreme abdomen pain and loss of blood supply to bones. Due to the lack of blood flow in her bones she developed avascular necrosis in her hip joint and right shoulder. "She came to us in a complete immobile state with a painful right hip and shoulder which were completely damaged. The sickle disease had affected her knees as well" said her surgeon Dr. Sachin Bhonsle, Consultant Orthopedic Surgeon.

"We had three major challenges before we decided to go ahead with the surgery. Firstly, control of her Sickle cell disease - Rebecca had 93% 'haemoglobin S' (HbS). Subjecting her to trauma of a major surgery like joint replacement, associated blood loss and stress would have precipitated a life threatening sickle crisis. To reduce her HbS to about 30% we had to perform an exchange transfusion. Exchange transfusions involved removing blood with HbS and replacing it with fresh blood of compatible donors. It took about five days to restore the HbS to a safe level, after which she was ready for surgery.

Secondly, there was the challenge of doing a shoulder and hip replacement at same time. Thirdly, when doing a joint replacement on very young patient it is important to perform a joint replacement surgery which would give her a long lasting and better quality of life."

After proper examination we decided to go ahead with the replacement of right hip and shoulder. A decision was taken to use an 'uncemented hip replacement' of a robust construction, which has a potential to last a lifetime. A ceramic bearing was chosen for this hip which again will never wear out.

Dr. Bhonsle used a minimally invasive approach to replace Rebecca's right hip whereby the hip joint was reached by minimal disruption with just a 4 inch cut. Because of avascular necrosis the bone quality was very hard and difficult to prepare, but it was achieved using careful and specialized instrumentation. Uncemented prosthesis was securely implanted and a ceramic bearing was fitted. Rebecca's hip was ready to use. Similar minimal invasive approach was taken to the shoulder joint. Cemented shoulder prosthesis was selected and secured.

Rebacca was able to walk on the 2nd day of her surgery. Following the surgery her pain has gone completely. She had a speedy recovery and was able to get back to her normal course of life within few weeks of the surgery.

SOURCE Fortis Hospitals Limited