Success Stories

Sickle Cell Anemia induced Hip Arthritis for a 17 year old and Hip Replacement

Ayo HamzatAdewemimo. a 17 year old boy, attending A level school in Lagos, Nigeria presented to Apollo Indraprastha Hospital, New Delhi, India with complaints of pain, stiffness, shortening in right hip and difficulty in walking for the last 5 years. These problems have affected his activities of daily living significantly to the extent that he could hardly walk. He was unable to take part in the extracurricular activity of school and was mainly confined due to pain.

He is a known case of sickle cell disease which was diagnosed when was only 11/2 years. Many of his family members are Sickle cell carriers but no one has clinical manifestation of this disease.  He has 2 brothers and both are healthy.  Patient was having sickle cell crises off and on for the last 15 years. Due to the disease he had developed Avascular Necrosis (AVN) of the femoral head of both hips (R>>L), with secondary arthritis in the right hip.

Parents sought consultations of many doctors in Nigeria who had only advised him bed rest and mobilization with walker, but could not offer him a permanent solution to overcome this problem.  Since the patient was very  young,  doctors in Nigeria were apprehensive in doing the total hip replacement. Hence, Ayo had to suffer a lot of pain and was really not able to carry out his daily activity. This patient and his parents obviously wanted to get the best treatment possible for his problem and hence  “googled” the internet to choices of doctors and hospitals across India to address his right hip problem. They then decided to come to India for his total hip replacement, at Indraprastha Apollo Hospitals, New Delhi under care of Dr (Prof) Raju Vaishya , who examined him and offered total hip replacement surgery. He was operated upon on 15/07/2014. Total hip replacement was done using uncemented joint components with ceramic bearings and large diameter head.  A special care was taken to prevent sickle cell crises during peri-operative period, by providing adequate hydration and oxygenation to the patient. The intraoperative and postoperative period was uneventful. 

The preoperative pain of hip arthritis had completely gone after the surgery and now he started taking his first pain-free steps after a long time. Ayo was confident that since he has been operated successfully, he would take life head on without any pain and disability in the hip.

This case was unique in many ways, especially as he was a very young boy with hip arthritis due to sickle cell disease. Total hip replacement surgery in such young age is extremely rare and is often not required. But due to his severe hip problem there was no better solution than THR to treat it. He may require revision THR surgery in future, due to wear and tear of the prosthesis. But still he will be able to lead an active and pain free life in his youth and prime time of life.

Sickle cell disease or sickle cell anemia is a hereditary blood disorder, where the red blood cells assume an abnormal, rigid, sickle shape. This sickling decreases the cells’ flexibility and results in a risk of various life-threatening complications. The sickling occurs because of mutation in the haemoglobin gene. This disease occurs more commonly among people living in tropical and sub-tropical sub-Saharan regions. The disease may lead to various acute and chronic complications, several of which have a high mortality rate. Sickle cell disease results in anemia and crises that could be of many types including the vaso-occlusive crisis, aplastic crisis, sequestration crisis, haemolytic crisis and others. Most episodes of sickle cell crises last between five and seven days. Infection, dehydration, and acidosis (all of which favor sickling) can act as triggers.

The 2 main orthopaedic problems these patients can have include, a) Avascular necrosis (aseptic bone necrosis) of the hip and other major joints, which may occur as a result of ischaemia and b) Osteomyelitis (bacterial bone infection); the most common cause of osteomyelitis in sickle cell disease is Salmonella, followed by Staphylococcus aureus and Gram-negative enteric bacilli.