As baby suffering from rare disorder whose treatment costs Rs 16 crore enters critical stage, parents and experts urge state to help
Teera's parents Mihir and Priyanka Kamat have learnt to give her nebulization and chest therapy at home
The condition of baby Teera Kamat, who was diagnosed as a Spinal Muscular Atrophy (SMA) type 1 child, has worsened. mid-day had reported about her on December 11, in 'Baby with genetic disorder needs Rs 16 crore therapy to live.' On December 14 she had to be rushed to the emergency room for a lifesaving procedure, as she was choking. Her father Mihir has appealed to the government to work with pharma and insurance companies to make lifesaving drugs for the ailment available in reasonable time at affordable costs.
Teera urgently needs the imported gene therapy
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Her distraught father, Mihir, said, "The doctors have now said her swallowing muscles are weakened further and next two weeks will be crucial, or else they will have to make her undergo a surgical procedure to insert a paediatric G-tube (Gastrotomy tube). Teera urgently needs the imported gene therapy, any further delay will be too late."
Mihir and Priyanka Kamat
Mihir said, "As soon as Teera hit the four-month milestone, we started seeing her SMA symptoms become more severe. Due to the onset of winter, she continues to have a cold which causes a cough build up in her throat and lungs. Normally, the body will try to clear the mucus by inducing coughing. But because of her chest and general muscle weakness, she is unable to cough on her own and clear her throat. On December 14 she had to be rushed to a nearby hospital for mucus suctioning as she was breathless and had a panic episode."
Sarfraz Alam, Imad's father
He said he is in constant touch with SMA parents at CureSMA India and FSMA India, who have been extremely helpful in guiding him and his wife. "These parents warned us that she will need constant suctioning as her condition progresses. We have now purchased a home suctioning device and requested a family friend who is a doctor, to show us how to do it at home. We are now regularly giving her nebulization and suctioning her airway so she can breathe. At night we constantly monitor her breathing sounds, and pray that she sleeps well through the night," Mihir said.
"Her lung are the most critical part of the body, most affected by SMA. Any reflux could enter the lungs and cause pneumonia, which is fatal for SMA kids. Teera also needs to be kept infection free. If she has infection or antibodies, we are worried she might not qualify for the gene replacement treatment programme," said Mihir.
There are four tools in her respiratory care arsenal - Her nebulizer, pulse oximeter, humidifier and home suctioning machine. Nebulizer converts saline solution to vapour and opens her airways and loosens mucus. "The humidifier makes sure air is moist which reduces the chances of cough accumulation. She needs to be suctioned every 3-4 hours depending on the weather and her cough build up. If she has breathless episodes, the pulse oximeter tells us if she is ok or we have to pick her up and run to the emergency room," Mihir added.
Four month Baby Teera SMA type 1 undergoes Leg Physio at home
Team of specialists for Teera
Teera has a team of specialist doctors who are working to make sure muscles and nerves in every part of her body are in working condition. Her treating physician is a paediatric neurologist at Hinduja Hospital. "We are consulting a known physiotherapist. Due to COVID-19 and her young age, most of the exercises are done at home, and we visit the doctor every two weeks to evaluate progress. Teera has seen a feeding therapist at SDLC (Speech Dysphagia Language Clinic), who helped us evaluate her sucking power, and then adjusted her feeding mechanisms and nutrition accordingly," said Mihir.
On December 17, the Kamats met a Paediatric Gastroenterologist at SRCC hospital who advised a barium swallow test with X-ray for her food reflux issues. He confirmed the X-ray shows severe stomach reflux that would cause liquids to climb up into her nose, which does not happen normally but will happen because the muscles in her throat are weak. "We have been advised to transition her - a four-month-old - to semi solids and then solids to prevent the reflux, and prescribed antacids to help with the existing reflux issues. If the reflux issues are not resolved in two weeks, she will need surgery to place a gastrotomy tube directly in her stomach," explained the father.
Four month baby Teera undergoes Nebulization at home
"Every single day, we live in fear that one night we will fall asleep and she will not breathe. We are afraid she will be weak because we cannot feed her. We are scared that if we suction her too much she might have a throat injury. She may get an infection and get disqualified from the treatment. She will have to get a surgery done at such a young age," said a worried Mihir.
"As parents, we would love to spend some quality time with her. Most of that time is getting spent travelling visiting her doctors, just trying to keep her alive and active. The other half is spent in raising funds to the tune of Rs 16 crore for her treatment. We have currently managed to raise only 17 percent of the funds (over Rs 2.70 crore), and are very, very far from our goal. The link to our fundraiser is https://www.impactguru.com/fundraiser/donate-to-teera," said Mihir.
Government needs to support
"We need help fast. Without any affordable treatment options available to SMA warriors, especially type 1 and 2 babies in India, this is a battle that is going to get harder. There is so much red tape and paperwork to import such treatments to India, and the drugs prices are unreasonably high when imported. We have appealed before, we need the government to work with pharma and insurance companies to make these lifesaving drugs available in reasonable time at affordable costs. These kids are the bright future of India, and we are letting them die just because we cannot agree on an effective policy to support our SMA champions," Mihir said.
"We keep hearing about kids, many under two years of age, all around India passing away because they could not get the required care in time. This disease has not one, but three treatment options available in the world, but no one in India has access to either. Isn't something wrong with that?" he asked.
Another SMA child
Sarfraz Alam, 37, father of late Mohammed Imad Sarfaraz, a six-month-old SMA type 1, from Kausa, Mumbra, has shared his side of the story. "Right to Life is a fundamental right for everyone, then why are SMA infants and patients deprived of it? The government and stake holders should act fast before it is too late," said Sarfraz.
He said, "With the effort of Cure SMA India foundation, on compassionate grounds my child name was picked up in the global lottery (by an international pharma company where selected registered patients get the drug free of cost), but due to the lengthy process and numerous approvals, he was deprived of the same, and breathed his last."
"On May 24, almighty Allah blessed us with our second child, Mohammad Imad Sarfaraz. He was normal at birth and weighed around 2.75 kilograms. On July 23, we visited our home town in Uttar Pradesh with Imad, who had turned two months old, then. My mother and elders in the house, observed that Imad had issues with breathing and was unable to control his torso and had little movement in his lower limbs. They advised me to get him examined by a child specialist," said Sarfraz.
A reputed child specialist advised Sarfraz to consult a paediatric neurologist in Thane and after few tests on September 27, he learnt his son was diagnosed an SMA type 1 case. "We spent next few days on the Internet to understand the ailment and its consequences, which only upset us further, as there was no cure for SMA. Subsequently we got registered with CureSMA India but witnessed Imad sinking slowly, with no cure or medicines," said Sarfraz.
"After November 5 it was the most testing period for us, as Imad, who weighed 6.8 kilos at six months, lost almost 1.3 kilos within 12 days as he developed fever and cough. The paediatrician was also concerned. Imad was having a difficulty in swallowing. Though the fever subsided, we still were unsure about the risk and had to take Imad to AIIMS, Delhi for further treatment. We reached Delhi on November 28, but before we could take him to AIIMS emergency room, Imad was not with us anymore,' said Sarfraz.
SMA Type
SMA is a progressive, rare genetic disease that is caused by missing genes or those that don't work properly, which might be survival motor neuron 1 (SMN1). This gene is basically the blueprint responsible for making a protein required for the survival of the spinal nerve cells. Without this SMN gene the cells in the body start to die and motor neuron cells become weaker thus causing muscle weakness in every part of the body below the neck.
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