UAE: Hospital saves life of 12-year-old boy suffering from rare neurological disease

Doctors at a private hospital in Sharjah saved a 12-year-old boy's life from a rare neurological disorder that left him paralysed and with respiratory failure.

Son of Indian expatriates Praveen and Tanu Lakhwani, Nikhil, complained of persistent leg pain and tiredness in early April. His parents didn't think much of the fatigue as Nikhil had just recovered from an upper respiratory tract infection two weeks ago, and children tend to get tired during the summer months.

However, things quickly went from bad to worse for Nikhil as he complained of persistent pain in his legs. Suspecting an injury, his parents took him to a local Orthopaedician in their neighbourhood, who ruled out any orthopaedic issue after conducting relevant tests.

Beginning of an ordeal

Following this, Nikhil was taken to a specialised centre and was referred to the emergency department at NMC Royal Hospital, Sharjah, for the diagnosis and the appropriate treatment. Once Nikhil was admitted, the on-call paediatrician quickly concluded it to be a neurology case.

Dr Manoj Singh, consultant of pediatric neurology at NMC Royal Hospital Sharjah, said, "Nikhil had no nerve reflex and had muscle power of Grade 2/5 in lower limbs and Grade 4/5 in upper limbs. The clinical picture was typical of severe and rapidly progressing Guillain-Barre Syndrome (GBS)."

After confirming the diagnosis with the help of an MRI and Nerve Conduction Study, he was immediately transferred to the pediatric ICU to initiate treatment as his condition worsened.

What is GBS

Specialist paediatric intensive care Dr Deepika Gandhi, NMC Royal Hospital Sharjah, said, "It is a rare and an uncommon condition. Guillain-Barre Syndrome (GBS) occurs in all age groups. Adults are affected more commonly than children."

She explained, "The overall incidence has been estimated to range from 0.4 to 2.4 cases per 100,000 per year. We diagnose less than 10 every year. With the widespread eradication of polio, GBS is the most common cause of acute and subacute flaccid paralysis in infants and children."

Dr Gandhi said, "Like what we saw in Nikhil's case, it is frequently preceded by an unspecified infection. This disease is also called ascending paralysis. It starts with the toes and ascends upwards, while the recovery happens in the reverse direction. Breathing improves first, and the last would be the legs. The time factor is critical as the disease progresses fast; hence an early diagnosis and prompt care can save a life and minimise the impact of the disease."

However, IVIG (Immunoglobulin) and plasma exchange have significantly changed the course of the illness - the treatment protocol also chosen for Nikhil.

After hospitalisation and despite treatment, Nikhil continued to deteriorate. He had become wholly paralysed (Power 1:5 in all four limbs) and also developed respiratory failure.

Initially, his disease progression seemed to slow down, and he was transferred to a ward after five days of PICU support. However, his muscle weakness gradually increased with an inability to swallow, and he needed to be put on a ventilator for respiratory failure.

Nikhil's mother, Tanu, said, "We were explained that our son's paralysis involved his breathing muscles and the muscles of his throat. He could not swallow and was choking on his own saliva."

She said, "The doctor told us what lies ahead for us in the coming days. She said the disease was progressive, and the debilitating muscle strength would take a while to reverse. It will become severe first, then plateau; after that, he shall start improving. It may be weeks before he starts walking again, even if he gets discharged. It will be a few months before he resumes his normal functions with the same ease as before."

"With the progression of the disease, despite the treatment, Nikhil started having many medical problems. He developed kidney stones because of prolonged muscle paralysis leading to bone demineralisation, developed muscle pain and leg pain, and suffered from irregular heart rate with BP fluctuations. In addition to the muscle weakness, GBS affected many other organs.", said Dr Gandhi.

Nikhil's mother Tanu recalls, "It was so heartbreaking to see him always in pain, wherever one touches him. The doctor explained that it is not neurological and not real, but he is feeling the pain as his nerves are behaving abnormally. All this was tormenting as our child was normal before this disease took over."

The path to recovery

After receiving treatment from a team of doctors, including a paediatric nephrologist, paediatric cardiologist, paediatric urologist, a paediatric endocrinologist and paediatric neurologist, Nikhil finally began showing signs of improvement.

"As his muscle power started improving, he was removed from the ventilator," said Dr Gandhi. They led the team of paediatric superspecialists in their battle to bring Nikhil back to the path of recovery. The hospital has recently inaugurated its all-new wing of paediatric super specialities with neonatal and paediatric ICUs under one roof for better care pathways.

The parents were relieved that Nikhil was off the ventilator and was showing improvement, yet they were apprehensive about the timeline of his full recovery. With some hard work by his physiotherapists on his muscle strength and breathing, Nikhil started to take small steps with support showing his team of caregivers and family the initial signs of recovery.

On May 10, after spending 35 days in the hospital, the time came when all super paediatric specialists taking care of Nikhil agreed to discharge him with the advice to continue with his physiotherapy sessions. After three weeks of returning home, Nikhil walked unaided for the first time. And, within three months of recovery, he can run, jump, cycle, and climb quickly.

Nikhil shared with his mother that he remembered everything about his hospitalisation except the deep sedation period in which he was on a ventilator.

"As a parent, our mind is so sensitive regarding our children, and it's tough to sometimes remain rational in such situations. Nikhils recovery is a good testimonial of reference for anyone who gives up hope in despair. He started out with ascending paralysis to trekking mountains with me. It is a story of hope and perseverance," said Nikhil's father - Praveen Lakhwani.

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