Boy, 12, struck down by rare Moya Moya disease

Doctors fear that if Stephen Hunte succumbs to a third stroke, it would mean death for the twelve-year-old. Stephen is a victim of the rare disease called Moya Moya — a disease which causes blood vessels leading to the brain to narrow and close, and is in dire need of surgery which costs an estimated US$60,000. His is the only known surviving case in the Western Hemisphere. Cases of the disease were first found in Japan in the 1960s and later in the United States, Europe, Australia and Africa. Moya Moya disease, which primarily affects children, adolescents and young adults, is accompanied by muscular weakness or paralysis affecting one side of the body, memory loss, involuntary movements and vision problems. Its cause is unknown and no cure has been found. Stephen experiences all of the symptoms. He has lost his vision, he is paralysed on the right side and needs assistance in walking, and has severe memory loss. “He doh remember my name, his name, anybody’s name. He says ‘yes’, ‘no’ and if he wants something he would say ‘umm’ and point,” said his mother Gloria Maharaj, 39, a single parent of two who resides in Kelly Village, Caroni.

The once very active, lover of cricket and “good eater,” Gloria said, “hasn’t seen the sunlight in months.” Stephen was admitted to hospital on two occasions. In the first instance, he spent three weeks at the Eric Williams Medical Sciences Complex (EWMSC) after experiencing a cerebrovascular accident (stroke) in mid March. (Stephen is a standard five student of Fatima RC Primary School. He was not able to sit his SEA exams.) Three days later, he was again admitted to hospital as a result of having a second stroke. He was released after five weeks. “The Ambulance brought my son home in a stretcher. They said they could do nothing for him,” lamented Gloria. Currently, he is awaiting surgery, but needs funds for securing a plane ticket to the US as well as funds to cover the cost of the surgery. One of three surgeries Stephen must undergo includes Intracranial-Extracranial Bypass Graft which involves the connection of normal blood vessels in the scalp to blood vessels on the surface of the brain in an effort to increase the blood flow to the brain. Dr Victor Terapelli, paediatrician at EWMSC and one of a team of doctors who attended to Stephen said that his illness was confirmed as Moya Moya by Dr Rasheed Adams. “He needs bypass surgery. If the blood vessels in the brain keep on haemorrhaging, it can damage the brain. The surgery can stop it at some stage.” Dr Adams said that while there are doctors in Trinidad who have been trained to perform the operation, “we do not have the neurosurgical equipment and an ICU for that.” “We treated him for his stroke. His initial stroke was without bleeding, his blood supply was cut-off and the second one, there was bleeding. He also suffered seizures and fits and we treated him for that,” said Dr Adams.

Since local doctors have been unable to perform the operation, surgeons were sought in the US. Stephen’s father, Leo Stoute, who resides in Brooklyn, New York has been able to make successful contact with neurosurgeon Dr Christopher S Ogilvy of the Massachusetts General Hospital, Boston who is willing to do the operation. A date for Stephen’s evaluation in the US was set for August 1, 2003, but due to a delay in acquiring necessary documentation to present to the US Embassy for a visa and lack of funds, a date has to be rescheduled. “We are waiting on a letter from Stephen’s insurance company, Aetna US Healthcare Insurance, to let us know how much we can secure for the operation,” Gloria told Sunday Newsday. “Dr Ogilvy said he (Stephen) could hold out a bit but depending on how severe the haemorrhaging to the brain.” Gloria was alerted of signs that her son was ill when he complained of severe headaches. “Before his first stroke he complained three times of the headaches at night. I gave him Panadol and soak his head thinking is a normal something. Most of the headaches, though, he experienced at school. But it was a day while playing cricket the headache started, then his neck started to pain him, so I took him to Mt Hope Paediatrics. They said it was exertion,” she said. When the headaches continued she took Stephen to a private medical practitioner, later the same day, who “treated him for food poisoning.” “The next day the headaches were more severe and I took him to the hospital. They said it was a muscular something because of the neck pain... I took him by another private doctor who said it was stress... But it was two days later when he (Stephen) said  ‘mummy meh hand’, he couldn’t stand, he couldn’t move his right hand and foot and then we took him back to the hospital where they warded him,” said Gloria.

A CT scan and an MRI showed Stephen suffered vasculitis of cerebral vessels consistent with Moya Moya pattern. His condition improved and Stephen was released from hospital. “Two days later he told me he was going for a walk, but then he met some friends and played cricket. I noticed when he came home his eyes were red... By 2.45 the next morning he started screaming ‘ow, ow mummy my head!’ and we took him back to the hospital. They took another CT scan which showed a swelling to the brain. He had gone back to square one — he couldn’t eat or walk. A third CT scan showed a frontal haemorrhage extending into the ventricles. “By that time I had left my job and I was staying with him at the hospital,” said a teary-eyed Gloria. Gloria used to fill a hundred 100lbs flour bags a day with manure, for 50 cents per bag. But her job as cleaner at MTS PowerGen, Pt Lisas was cut short when Stephen fell ill. “All he did was sleep. I prayed and read the Bible...” She recalled that Dr Nunez “put me to sit and he said ‘mums, your child is suffering with a disease called Moya Moya. To understand what it is go on the Internet.’ He said my child needs surgery and that no doctors could handle him. He said I have to look for one abroad and that I would get trouble in doing so seeing how rare his case is.

“That day I cried from Mt Hope to Port-of-Spain; when you hear no cure yuh think that is the end of your child... If it wasn’t for God, St Ann’s pick me up already!” Gloria exclaimed. She wrote to the Ministry of Health for assistance. The Welfare Office responded. One of Stephen’s Stardard five classmates came to his assistance and is helping out with foodstuff. “So I am asking for whatever help I could get...” She ensures that Stephen gets some therapy by “rubbing him with sweet oil and giving him exercise. If I don’t do that his hands will get stiff. My advice to parents — they need to be careful that when children complain of pain whether it is their belly or head to check it. My child is a victim of this rare disease and I am forced to take him out of the country. It’s a pity I cannot find a doctor in my own country to see about my son.”