Kokilaben Dhirubhai Ambani hospital gives new lease of life to a 13year old Iraqui girl, bedridden from last 8 years

Mumbai, September 14, 2015:  Kokilaben Dhrubhai Ambani Hospital has achieved yet another milestone with the successful treatment of a bubbly 13 year old girl from Iraq, who suffered from a rare genetic disorder. Osteogenesis Imperfecta (OI) is a rare genetic bone disorder with an incidence ranging from 1:20,000 to 1:50,000 births. It affects the body’s ability to make strong bones and results from a genetic defect in the formation of collagen. Collagen is a major protein of the body’s connective tissue and forms the building block along with the main framework which supports bone and other body structures.  This genetic defect in collagen results in extremely fragile bones that break easily


Dr. Alaric Aroojis, MBBS, D'Orthopaedics, MS (Orthopaedics), DNB (Orthopaedics), Bone and Joint /Orthopaedics Dept, Kokilaben Dhirubhai Ambani Hospital, said “the very name, Osteogenesis imperfecta – implies ‘bone that is imperfectly made since the beginning of life’. It is a crippling & disabling disorder with a vicious cycle of repeated fractures causing severe bone deformities, which further predisposes the child to further fractures. Combined with muscle weakness, the gradual deformation of the bones ensure that the affected children are unable to bear weight on their legs and gradually become bedridden & home-bound”.

The patient, Zahraa Thani, is a bubbly 13 year old girl from Iraq, who loves to dance & play like all other teenagers of her age. However, since the past 8 years, she has been confined to her home and unable to stand or walk. Zahraa suffers from Osteogenesis Imperfecta (OI) or ‘brittle bone disease’ – a genetic bone disorder that causes severe bone fragility & repeated fractures. 

Zahraa’s condition was not diagnosed at birth and her development & growth were normal until the age of 2 years, when she sustained her first fracture after a trivial injury. Her parents started getting concerned when Zahraa started sustaining 2 to 3 fractures every year – most often following very trivial injuries.

After detailed consultations & investigations by a panel of doctors in her home country, she was finally diagnosed to be suffering from a serious bone disorder called Osteogenesis imperfecta. By the age of 4 years, the bones of both her legs had deformed considerably due to repeated fractures, and she underwent two surgeries in Iraq to try to correct the deformities. Unfortunately, both surgeries were unsuccessful and by the age of 5 years, Zahraa was bedridden and confined to her home. She would manage her daily activities by crawling around the house and with help from her parents & siblings. Due to her extreme bone fragility she was unable to attend regular school like children of her age, and consequently had to learn to read & write at home with help from her older siblings.

In a bid to seek out the best medical care for Zahraa since doctors in Iraq were not fully equipped and did not have the necessary expertise, her parents approached the Orthopaedic Department of Kokilaben Dhirubhai Ambani Hospital and consulted, Paediatric Orthopaedic Surgeon, Dr. Alaric Aroojis who agreed to take up this challenging case.

Commenting on Zahraa’s treatment, at Kokilaben Hospital, Dr Aroojis said, “Being a genetic disorder, the disease itself is incurable – but several treatment strategies are available that can re-strengthen the bones and improve the quality of life. One of the exciting modern developments in treatment of this condition is the use of a special class of drugs called Bisphosphonates, that help to add strength to these fragile bones. The other treatment is surgical – and involves cutting the bone in several places to straighten out the deformity and then threading the pieces on a metal rod – a surgery popularly known as the ‘sheekh-kebab operation’.”

Dr. Alaric Aroojis, is trained in this technique of operation at world-class centres in USA. This surgery is a complex operation requiring a high level of expertise to execute safely. The bone is extremely fragile and can shatter if not handled carefully. The bone must be cut into several pieces and then threaded skillfully over a metal rod without damaging its basic structure or blood supply.

In Zahraa’s case, the complexity was compounded by the fact that her bones were severely bowed and had the length and diameter of a 5-year old child’s bones.  The medical team used a special type of rod customized for Zahraa’s bones which was only recently launched in India. The Fassier-Duval Telescoping Rod is a custom-made metal rod which has two sleeves that slide one over the other. The two ends of the rod are anchored within the growing ends of the bones & telescope on each other to expand in length as the child grows. This serves as an internal support to strengthen the fragile bone and reduces the need for frequent rod revision with growth.

Zahraa underwent this surgery on her right leg on 20th June this year and a similar surgery on her left leg two weeks later. Her legs were immobilized in a plaster cast for 2 months to allow the bones to heal, after which she was allowed to stand with the help of braces and with the support of a walker. Her bones have been straightened completely and she is able to stand and walk for the first time since 8 years.

An excited Zahraa said, “I am very happy to be able to walk for the first time after 8 years and wish to return to Iraq and resume school and play with my friends”.

Dr. Aroojis concluded, “We are happy with her progress, she now needs to administered medication twice a year, intravenously, to further boost up her bone strength. Her right leg is also short due to repeated fractures and she will need limb lengthening surgery, which will be performed at a later date. However it is incredibly fulfilling to see the confidence these children develop once they are able to stand and walk on their own two feet after years to being bed-ridden. This is a life-changing surgery, not only for the affected child but for the entire family. Such a surgery would only be possible in a tertiary-care institution such as Kokilaben Hospital, where patients have access to the latest technology and expertise in the management of complex bone disorders, and a multi-disciplinary team of Paediatric Orthopaedics, Paediatric Endocrinology and Paediatric Rehabilitation to provide 360-degree care to the child.”

Princess Zahraa has become a mini-celebrity at the hospital where she has spent 3 months recuperating. All that she wants to do now is to return home to Iraq, walk independently into her own home, resume her studies and live like any other ordinary teenager.

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