Miracle Spine Surgery of Little Girl at Chennai !

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Girl gets spine corrected

Source : Deccan Chronicle , 29th June 2013

One in 3,000 girls is affected by scoliosis, the curving of the spine from side to side. But only a few visit hospitals for surgery, says Dr Nalli R. Uvaraj,  professor of spine surgery, department of orthopedics, Rajiv Gandhi government general hospital (RGGGH).

Dr V. Kanagasabai, dean, RGGGH (left) and Dr R. Uvaraj, professor of spine surgery, department of orthopedics, greet Abitha, who underwent growing rod technique procedure, on Friday.

“If left untreated, the lungs and heart get affected and one’s lifespan also comes down. Scoliosis is more common among girls than boys,” said Dr Nalli who performed dual growing rod technique procedure on nine-year-old P. Abitha from Porur.

While a few corporate hospitals perform surgery for scoliosis, this is the first time the government hospital has performed it on a nine-year-old with the new technique.

“We usually operate on 13 or 14-year-old girls. This patient visited us with acute back pain and we performed the procedure in March. While most operations for spinal deformity on young children affect their growth, this particular technique is to allow for continued, controlled growth of the spine,” informed Dr Nalli, adding that screws are inserted into the bones of the vertebral column at the level of the deformity and the correction is done.

The child needs to visit the hospital every six months to have the rods lengthened. “This is mainly to keep up with the child’s growth.  She is only nine years now and till she reaches 14 years, the rods will be lengthened every six months in a simple procedure. When she reaches 20, the rod can be removed,” explained Dr V. Kana­gasabai, dean, RGGGH.

While the surgery would have cost Rs4 lakh and the follow-up every six months more than Rs70,000, the procedure was done free of cost at the general hospital. Abitha, daughter of car driver Prabhu, said she could play well and would go back to school from Monday.



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GH docs do rare surgery on kid with bent spine

Source : The New Indian Express , 29th June 2013

Nine-year-old Abitha found it hard to run around and play her favourite games with her friends at school as she often had unbearable pain on her back. Her father Prabhu, a car driver, approached doctors at the Rajiv Gandhi Government General Hospital a few months ago after he found that his little girl was suffering from scoliosis, a spinal deformity since birth.





               Top docs at RGGGH show Abitha’s progress post surgery

Today, Abitha can run and play around just like any other child without pain. Thanks to the dual growing rod technique, a rare spine treatment given to her.

Doctors at the RGGGH performed the procedure which would otherwise cost her Rs 11 lakh in total, free of cost. “Scoliosis is the bending of the spine on its side. The girl’s father approached us after seeing newspapers about a 22-year-old woman with the same deformity we had operated on earlier,” said Dr V Kanagasabai, Dean, RGGGH.

With highly expensive equipment procured through government funds, the doctors cut and opened the lower part of her back, inserted rods with a length of about 15cms each and four screws. They then created an opening near the neck to adjust the rods up through tunneling method and fixed some hooks. Within a month after the first surgery was done on March 1, the girl was able to move around freely, doctors said.

But since Abitha was just nine, she would require four to five surgeries in intervals of about six months. While the first surgery cost  about Rs 4 lakh, the subsequent ones will cost about Rs 70,000 each if charged. “If the girl grows about one cm, we can extend the rod to keep pace with her growth. This can be performed through a small incision and will not affect the girl’s growing organs like heart and lungs. In the first surgery itself, we were able to achieve 50 to 60 per cent correction,” explained Prof Nalli R Uvaraj, Ortho Spine Surgery Unit, RGGGH.

Once the girl reaches her full height at the age of about 14 or 15, she will undergo a  complete procedure where the rods will be fused and fixed permanently, doctors said.  They added that though it was a long, difficult therapy for the child, the complication of hook dislodgement and rod breakage was low. “The size of the rod would be double the size for adults than the ones used on her. After 14 or 15, the growth rate is less and it won’t affect her or the rods. There is no reason why such a deformity occurs but it happens one in 3,000 people and more on women,” the professor explained.

The little girl said she was comfortable walking around. “I study class four and I love playing with my friends. Earlier I had pain, now I don’t. I am going to get back to school on Monday,” she said with a smile.

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Surgery straightens 9-year-old’s spine

Source : The Hindu , 29th June 2013

For years now, P. Prabhu, a car driver, has been spending sleepless nights trying to find a solution to his nine-year-old daughter’s condition – a spinal deformity. All through her formative years, Prabhu watched Abitha walk with a slight hunch and struggle with intense pain.

On Friday though, three months after the child underwent a surgery at Rajiv Gandhi Government General Hospital (GH), Prabhu was looking forward to the day she would fully recover.

“When Abitha was three, we noticed she walked with a slight hunch but we did not know the extent of the problem. She used to complain of pain. We found out that she had a spinal deformity only when she was six,” he said.

Doctors at GH diagnosed Abitha with scoliosis — a sideways curvature of the spine, and performed a surgery using the ‘growing rods’ technique, in March.

“Abitha was brought to us last November. She had an ‘s’-shaped curve in her spine. We opened up the upper end and lower end of the curve. Screws and hooks were inserted at both ends and we tunnelled four rods under the skin. The screws held on to the rods on either ends. The rods will be lengthened every six months to keep up with the child’s growth,” said Nalli R. Uvaraj, professor of spinal surgery at GH.

When the child is older, doctors will remove the rods and perform a spinal fusion operation. Abitha, who is in class IV at a school in Porur, said she felt much better after the surgery.

Foreigners’ get their backs mended in city : Chennai

A few weeks ago, a teenage girl from Nigeria with spinal deformity visited a private hospital in the city and underwent a surgery for correction. Similarly, in another private hospital, a 12-year-old girl from Iraq was treated with braces for scoliosis, a medical condition where there’s abnormal curving of the spine sideways.

With increasing number of teenage girls coming from other countries for scoliosis surgery or braces here in Chennai, doctors say fortunately there are only fewer cases reported in the city.


“Scoliosis is more idiopathic, that is, nobody knows the causes for scoliosis. It is seen mostly among adolescent girls and starts from 12 or 13 years. Scoliosis progresses rapidly and we find a spinal curvature of even more than 40 or 50 degrees. Their rib cages are seen bending that only the mother notices the deformity,” says Dr M. Siva Murugan, director of Soundarapandian Bone & Joint Hospital.


Scoliosis is more common among girls than boys. “Fortunately, there are only a few cases in Chennai. It’s very common in western countries and also on the west coast. More teenagers from Iraq come here for surgery or for braces that are used in cases of moderate scoliosis. And another important challenge we face is that scoliosis is seen more among the elderly population here in our state. Osteoporosis is also linked to scoliosis,” explains Dr Siva Murugan.


Doctors say wearing braces does not cure scoliosis and girls find it difficult to wear braces. Ms Stephanie Amatha, who came for surgery here, had said not many girls were comfortable wearing braces. As the cost of surgery is low, compared to other countries, many girls visit hospitals in Chennai for surgery. Dr Siva Murugan says that any delay in diagnosis of scoliosis can have a psychological impact on the patient.


Source : Deccan Chronicle , 18th June 2013

Robotic Spine surgery helps foreigners : Chennai

Stephanie Amata Oghenefegho, a 14-year-old from Nigeria, suffered from spinal deformity and her mother, TV host, Agatha Amata, visited England and Germany to find a solution for the deformity.

“At first, I thought my daughter was hunching and I asked her to walk erect. But only later I found that it was a deformity and through a friend, “I came to know about Dr Sajan Hegde, consultant spine surgeon at Apollo Hospitals.

He assured that after surgery she would be fine and can go back to school. His confidence and solution to my problem brought me here to Chennai,” said Agatha Amata.

Similarly, three more patients, Eric Francis Millican, 65, from California, Julia Obur Deng Lual, 44, from Sudan and Haidur Kadhum Fatay, 33, a drilling engineer from Iraq, came to Chennai last week with different spine-related problems and were treated by Dr Sajan Hegde using robotic spine surgery.

In six months, the hospital has performed 100 surgeries using the robotic spine surgery. “If you take the case of teenage girl Amata, the surgery would have cost five to six times more than the cost in India. They came from different countries and the surgeries were performed only last week,” said Dr Sajan Hegde.

Deputy minister of the Republic of South Sudan Simon Mijok Mijak, whose wife Julia underwent spinal surgery at Apollo Hospitals, said his wife had been suffering from severe lower back pain for three years.

“She was given drugs and when nothing worked, we came here,” he said. Julia was treated with minimally invasive reconstruction using the unique robotic technology to treat her unremitting back pain that distracted her from her normal routine.

Source : Deccan Chronicle , 6th June 2013 

She goes to school with a portable ventilator : Bangalore

It's nothing but the triumph of the will to live in the face of a debilitating morbidity. Fourteen-year-old Nidhi goes to school not just with her schoolbag. She carries along a portable ventilator and a suction machine by her wheelchair. It's after a long struggle that she has managed to get into a regular school and then get them to install a lift there for her.

 

 

Nidhi has one of the rarest genetic disorders which also happens to be one of the costliest. She has been diagnosed with Pompe disease that results in debilitating and progressive muscle weakness because of a defective gene she has received from one of the parents. The disease is life threatening and the treatment is ERT (enzyme replacement therapy), an expensive procedure.

Nidhi is not alone in her struggle. Her mother, Sharada Shirol, sits beside her in the classroom through the day as her daughter listens to the teacher intently. Her father, Prasanna Kumar Shirol, has quit his job at Reliance to attend to his daughter's needs.

The girl was normal when she was born. The first symptoms started when her parents realized that she could not crawl or stand up on her own. The initial diagnosis was myopathy, a muscular disease. Her parents took her to almost 45 hospitals in India before she was diagnosed with Pompe was in Davanagere in 2007.

It has been a struggle since then. She is wheelchair bound, has to be on a ventilator at night and has tracheostomy where an artificial airway has been done to help her breathe. She developed resistance to enzyme therapy and had to go through an immuno-suppression costing Rs 12 lakh.

For Prasanna and Sharada it has been a relentless fight to keep their precious one alive. "Why should we give up when she's fighting? Every time she goes to hospital, she exercises and finds a nerve for the injection. She has never cribbed. Many people have told me that I am fighting nature. But when she wants to live, how can we sit back?" asks Prasanna.

The couple's social life is nil. They have limited visitors at home, as Nidhi's immunity levels are low. Hangouts are restricted to malls, that too on rare occasions. "She has heard so much about Wonderla that she wants to visit it. We have promised her a visit this vacation," he said. Nidhi's only pastime is painting and she can do it endlessly.

It has been one disaster after another that the Shirols are handling. Nidhi has now developed Scoliosis because of being continuously confined to the wheelchair. Her spine is S shaped, and requires 35 screws to be re-aligned - that too at the risk of losing her post this procedure.

OUT OF RESOURCES

And the parents have run out of resources. "The medicines cost Rs 85 lakh per annum for her body weight of 25 kg. Thankfully it is being sponsored by a pharmaceutical company under their Charity Access Programme. But that is not all. We spend around Rs 30,000 to Rs 40,000 per month for her supportive care. The Sciolosis surgery will cost a minimum of Rs 10 lakh and we are running short of funds," he said.

Nidhi is the couple's only child. Sharada had conceived again but the pregnancy was terminated after pre-natal tests showed that the second baby too was affected by the disorder.

 Source : Times Of India  , 3rd April 2013 

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BRAVE ART ( THE WEEK )

The letters in her English notebook look like children standing in a row in a school assembly. Her paintings of kids having fun in parks and swimming pools bring in a rainbow on her bedroom wall. Except for the tablet wrappers which dot her glass paintings, there is no trace of what she is going through in her bright and cheerful artworks that have won her many awards.

Being immobile hasn't stopped Nidhi Shirol. Despite being wheelchair-bound, the 12-year-old is the busiest person in her house. She enjoys painting and one of her works recently appeared in Expression of Hope, a book published by GENZYME, a global programme for awareness and inspiration, featuring artworks by the Lysosomal Storage Disorders Community.

Nidhi was 7 years old when she was diagnosed with Pompe, a rare genetic disorder caused by a deficiency of the enzyme called acid alpha glucosidase. “When she was born, she was a normal child,” recalls Prasanna Kumar B. Shirol, Nidhi's father, showing her lovely childhood pictures on his laptop. But a few signs of development delays were seen as she grew up. She had difficulty crawling and even at 18 months, she was not able to get up on her own.
 

Shirol and his wife, Sharada, took Nidhi to the National Institute of Mental Health and Neuro Sciences in Bangalore, where the child was diagnosed with myopathy, a disease of muscle tissue for which there was no cure.
 

The symptoms of Pompe appeared after five years. The disease crippled her muscles and she had difficulty standing and sitting. Being an outdoors person, that was more than what she could bear.
 

Life became tougher for her as days passed by. She developed severe breathing problems and had an incision made in her windpipe to get a tube inserted to assist breathing (tracheotomy). At night, she needs ventilator assistance.

As Nidhi couldn't walk, the family moved from Hubli to Bangalore and put her in Gopalan International School.
 

With medication, Nidhi's condition has improved a lot. But whatever damage the body has had is irreparable. Treatment only stops further deterioration.
 

Despite all the difficulties, Nidhi never complains. She never loses her patience over the numerous dos and don'ts. She knows that she cannot go out in the rain as it can lead to respiratory problems. The quiet fortitude with which she fights the disease amazes even her doctors. Some of the procedures in her treatment are a bit painful but she takes it all without a word of complaint. Nidhi never gives any trouble to her parents, too. “In spite of her ailments, she does everything on her own. I don't have to be after her to make her do her homework,” says Sharada, who involves Nidhi in all activities, family functions and holidays.

Nidhi misses three or four classes a month for her treatment, which includes enzyme replacement therapy. She has to do physiotherapy, pranayama and hydrotherapy regularly, and so, after school, she gets very little time for her studies. However, she has managed to score well and wants to become a doctor.

Recently, Nidhi's became less responsive to the therapy she was receiving as her body developed antibodies. She then underwent a complicated and expensive treatment regimen to overcome this antibody effect on the advise of Pompe disease experts at Duke's University in the US.

For Shirol, the challenges seem unending. Availability of the main drug for Pompe can be a big problem. “The highly expensive drug was discovered in the US in 2006. Right now the company is offering it to us free of cost as part of their clinical trials. If they stop sending us the medicine, Nidhi's life will be in danger,” says Shirol. Drug costs are based on the body weight of the child. “Now she is 24kg and the medicines for her cost 085 lakh per annum,” he adds.

Supportive medical help also costs more than what a middle class family can afford. “Right now I am spending around 040,000 a month on various tests, physiotherapy and sending the samples abroad,” says Shirol. He quit his job and started his own business to find more time to take care of Nidhi.

Around 500 children in India have been diagnosed with Pompe disease. “They need to be given treatment lifelong. Unfortunately, many of them are from poor families that cannot afford the treatment even for a month. So, many children affected by this disease have lost their lives for want of treatment. But the government has not started any major programme either at the state or Central level for Pompe disease,” says Shirol, the president and Karnataka state coordinator of Lysosomal Storage Disorder Support Society. The support society has sought the help of the National Human Rights Association India to preserve the lives of children with Pompe.

Neither Shirol nor his wife had a family history of the disease. Still their second child also had Pompe. “Sharada got a prenatal test done during her second pregnancy. Pompe was diagnosed and so pregnancy was terminated,” says Shirol. He hopes that developments in gene therapy may cure her daughter some day. “But it may take decades,” says the visibly anxious father.

Mahaveer Regal apartment, where Nidhi stays, looks like a large joint family during summer holidays. Children play together from morning till evening in the corridors and the ground floor. The noise drives Nidhi into a pensive mood at times. But then she will seek refuge in her make-believe world where there are seesaws, slides and lots of playmates waiting to be animated into paintings. 

When Pompe strikes : By Dr Meenakshi Bhat

 
Pompe, a rare genetic disorder, is found in one in 40,000 people. It mainly affects muscles in the body. The initial symptoms include difficulty standing, sitting and climbing. The patient may have trouble breathing, chewing and swallowing also as more important muscles get affected. The earlier the diagnosis, the better. Those who receive treatment late may require tracheotomy and ventilator support. Heart muscles can also get affected and heart rhythm problems can happen. The muscles of the back often lose their ability to support the spine and, therefore, people with Pompe are prone to abnormal curvature in the spine called scoliosis.
 

In a family where one child is affected with Pompe disease, the likelihood of the parents having another affected child is 25 per cent (1 in 4) in each subsequent pregnancy.

Dr Meenakshi Bhat is consultant in clinical genetics and associate 
professor at Centre for Human 
Genetics at Biotech Park, Electronic City, Bangalore.

Source : The Week  , 1st June 2012 

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Heard of Pompe, Gaucher’s? Know about them and more   

Listen To Those Who Live With These Rare Genetic Disorders

 

Nidhi (left) was diagnosed with Pompe when she was seven years old, while Yatish (above) suffers from Gaucher’s

 If you have a disease, even as serious as malaria, you know there is a cure and that doctors can diagnose it. But have you heard of rare genetic disorders like Pompe, Gaucher’s, Fabry and Mucopolysaccharidosis (MPS)? These are inherited metabolic disorders that result from defects in lysomal function. Some of their incidences are among 1 in 10,000.
 

    The Centre for Human Genetics hosted a gathering of patients and parents with these rare genetic diseases on Saturday. 

    Nidhi Shirol, a third standard student of an international school, loves to paint but her life is not the same as any other student of her class. Her mother waits outside her class helping her in case she has difficulty in breathing. She is put on ventilator throughout the night. Her father Prasanna Kumar Shirol, a marketing executive and now president of Lysosomal Storage Disorders Support Society (LSDSS), says Nidhi was a normal child till she was nine months old. 

    Nidhi had difficulty in crawling, standing and used to take support of her thighs to get up. “We took her to many doctors. No one was able to diagnose her condition. At Nimhans, for the first time, the doctors told us that she has myopathy, a genetic disorder. She was seven when a doctor in Davanagere diagnosed her with Pompe,” he says. 

    She was on ventilator 24X7 for almost a year. Now she is undergoing an enzyme replacement therapy that is helping her recover slowly. 

    Ten-year-old Tejas Udayshankar loves to dance and is one of the brightest kids in his school. But his joints are swollen, back crouched and he looks as small as a two-year-old. The lively boy was diagnosed with MPS very recently. He also underwent a surgery to keep his spine straight. Otherwise, his head would roll back. “He was a healthy baby, weighing 4.7 kg. When he turned four, we realized something was abnormal but no doctor could diagnose what was wrong,” says his father N Udayshankar. Udayshankar is married to his paternal aunt’s daughter. (Consanguine marriage). 

    Another case of consanguine marriage is that of Ramesh M, whose six-year-old son Yatish M is suffering from Gaucher’s. His spleen had become huge and stomach bloated and no one could diagnose the reason. He has hardly grown and looks younger than his younger brother who is four years old. 

    Many such patients gathered at the centre for human genetics to get their children screened. “These group of rare inherited metabolic disorders are usually found among 1 in 1,000 patients but some of them are as rare as one in a million,” explained clinical geneticist at the centre for human genetics, Dr Meenakshi Bhat. 

Source : Time News Network