5 year old girl with a very rare blood clotting disorder successfully treated by doctors in Mumbai

Story by Sagaya Fernando

Mumbai: January 19, 2018

 

A five year old girl was successfully treated for a very rare birth defect with a very rare blood clotting disorder by doctors at Bai Jerbai Wadia Hospital in Mumbai.

The child was diagnosed one among 200 rare medically recorded cases globally.

Deepika Yadav from Raichur district in the south Indian state of Karnataka was having right sided brachial plexus injury and Factor VII deficiency. Brachial plexus injury is about nerves of the upper limb are stretched inadvertently during difficult delivery leading to poorly functioning arm.

Factor VII deficiency is a blood clotting disorder that causes excessive or prolonged bleeding after an injury or surgery. With Factor VII deficiency, the body either doesn’t produce enough Factor VII, or something is interfering with factor VII, often another medical condition. Factor VII is a protein produced in the liver that plays an important role in helping blood to clot. It’s one of about 20 clotting factors involved in the complex process of blood clotting.

The brachial plexus is the network of nerves that sends signals from your spine to your shoulder, arm and hand. A brachial plexus injury occurs when these nerves are stretched, compressed, or in the most serious cases, ripped apart or torn away from the spinal cord. Babies sometimes sustain brachial plexus injuries during birth.

“Deepika had a very limited ability to lift her right arm above shoulder and on MRI it was found that she had a posterior dislocation of the humeral head. She was evaluated and advised surgery of the right shoulder by shoulder muscle transfer. During a routine work up investigations for surgery, it was revealed a persistent deranged bleeding profile (prolonged Prothrombin time) which was not corrected even on medication. After further thorough evaluation by our medical team it was notified that Deepika had a disorder in which Factor VII was deficient,” said Dr. Minnie Bodhanwala, Chief Executive Officer of Wadia Hospitals.

The hematologists advised Factor VII administration whose cost is very expensive. A 1 mg vial of rNOVO7 costs Rs 48,000 and as per estimate by hematologists she would require at least 18 to 19 vials which cost around Rs 9 lakhs. Due to financial constraints of the labourer father who had 6 dependents, the surgery was deferred.

“After consultation, the hematologists of our hospital played a vital role of making a protocol for the surgery. This surgery was performed under cover of Factor VII infusion (rNOVO7) uneventfully. The rNOVO7 was given every 4th hourly for the 48 hours and then reduced to 8th hourly after that. It has now been stopped and there is no bleeding. Without this treatment she would have bled uncontrollably, and surgery would have been impossible for this child. This child needed corrective surgery to reconstruct the shoulder, or which would result in inability to use the arm,” said Dr. Bodhanwala.

Deepika’s father Tirumala said, “We had approached many hospitals in Karnataka but was unsuccessful in our endeavor. B J Wadia hospital stepped up to this challenge and treated my daughter free of cost and we are very grateful to them.” 

 “This case was one of its kinds and the rarest one. Fewer than 200 cases of such cases are reported globally till date,” said Dr. Minnie Bodhanwala.

She further added that the child is doing well now, with a plaster cast being put on her right hand to maintain the position of her shoulder.

“After six weeks, we will give her physiotherapy to teach her how to use her upper limb,” she added.