For two-three months before the monsoon,when the weather is hot and dry,when fruits are ripening on the mango and litchi trees,and when villages are preparing for the coming showerslayering roofs of their thatched huts with more bricks and strawparents in north Bihars Muzaffarpur district wake up each morning with dread.
Every year,around this time,900 and 1,200 children between the ages of two and five are afflicted with a mystery illness. It starts with lethargy and a light fever,but shows the first signs of worsening towards the morning. Many of the children are dead before they can reach a hospital.
The district saw 44 deaths in 2011,121 in 2012 and 39 this year. Bihar Health Secretary Sanjay Kumar admits they dont know the reason for this decline. The cases have dropped this year simply because God has been kind, he says. We dont know what we have done right. Right now,we are all groping in the dark.
The latest to draw a blank is a team from the Centers for Disease Control (CDC) in Atlanta,US,which was roped in by the National Centre for Disease Control (NCDC) to test samples from this year. A report submitted to the state and Centre by the NCDC in the first week of September said the results have been negative for several known viruses,including Japanese Encephalitis (JE),enteroviruses,West Nile virus,dengue,Chandipura and Nipah.
In fact,after testing blood samples,rectal swabs and cereobrospinal fluids of patients,as well as brain tissues collected from deceased patients,researchers have not been able to isolate a single strand of a virus. The question now staring them in the face is: is it even a virus?
The children here started falling ill many years ago,according to villagers and local doctors. As the cases subsided with the monsoon,villagers called the disease a curse of the rain gods,while many parents referred it to as chamki. Doctors called it variously a mystery disease,monsoon fever,heat stroke and encephalitis.
It was only five years ago that the administration took note. As soon as active surveillance programmes were launched in 2010,and medical experts started coming from across the country and the world,the alarming extent of the disease was obvious. If the Muzaffarpur district saw 120 cases and 44 deaths in 2011,the six districts adjoining itincluding East and West Champaran,Sitamarhi,Samastipur,Gopalganj and Siwantogether saw 944 cases and 204 deaths. In 2012,this jumped to 1,225 cases and 420 deaths overall,and 334 cases and 121 deaths in Muzaffarpur. In 2013,not just Muzaffapur,but the region too has seen a decline,to 170 cases and 60 deaths.
An afflicted child starts showing symptoms towards the evening,becoming lethargic and quiet. Towards night,the child falls into a seemingly calm sleep,though with the beginnings of a light fever that most parentsusually tired after a long day in the fields,fail to notice. Barely a few hours after daybreak,the victims wake up convulsing,their eyeballs still and their limbs rigid. This is followed by vomiting and then frothing at the mouth. As their fever mounts,children faint and many perish on their way to the districts three main hospitals.
But what exactly is killing the children?
I want bhaat (rice). I am tired. I dont go to school. My hands are loose. I had two brothers. I want the jhadu (broom). My brothers are dead.
Mohammad Sarfuddins neighbours in Musachat village call this nonsense,saying the 10-year-old has been talking like this since he had convulsions two years ago. He was in hospital for two months and now,his speech is slurred,he cannot move his right hand,he needs help standing up,and collapses after walking just a few metres.
Sarfuddin is a recorded case of Muzaffarpurs mystery disease,and health workers say he must have physiotherapy and speech therapy. They promise his mother repeatedly that treatment will be free.
Shakina Khatoon does not believe them. She says she had two other sons before Sarfuddin,both of whom died after similar convulsions. However,she has no treatment records to prove her claims. While she wants to do her best for Sarfuddin,she is not sure if she can convince her husband,a rickshaw puller,to take a day off and make the trip to town.
When experts first started trying to join the dots,the confidence that it had to be JE was palpable. The disease was thought to infect hundreds of children in the belt around Gorakhpur in eastern Uttar Pradesh annually,and though the region is 250 km away,the symptoms were similar. JE is also known to cause high fever,convulsions and death,as well as leave some survivors with altered brain function,loss of speech or hearing,and compromised limb functionsimilar to Sarfuddin.
When the first locally tested samples tested negative for the JE virus,tests were repeated,by multiple agencies last year and again this yearincluding the National Institute of Virology in Pune,the NCDC in Delhi,units of the Indian Council of Medical Research such as Patnas Rajiv Memorial Research Institute (RMRI),Delhis Safdarjung Hospital and Lady Hardinge Medical College,and a team from the CDC,Atlanta. However,all ruled out JE.
Since the disease was not found in patients,we decided to look for it in the vectorsmosquitoes which carry the JE virus from hosts such as pigs,water birds or other livestock to humans, says Dr Pradip Das,director of RMRI. However,no known vector of JE was found on the scene. Now we have eliminated the theory of JE completely, Dr Das says.
The RMRI and NCDC conducted similar tests,but to no avail.
Bablu (5) got the chamki this year. His story is like many of his friends,but what has investigators excited is that Bablu,who lives in village Chainpur of Meenapur block,has no residual symptomshe walks and talks normally,with no memory loss,and has returned to school.
Says father Subhash Prasad,Within half an hour of his first chamki,I put him on my motorcycle and rushed him to hospital. He was unconscious for 72 hours,but he woke up and asked me about Ajay Devgn. He had just seen a film of his the night before he fell ill.
However,after he was discharged from hospital,the parents had a scare. Bablu stopped talking and did not speak properly the first 15 days. He wouldnt eat and resisted attempts to make him sit up or stand and insisted on lying down.
He would say silly things,stare at the fields and refuse to go out to play with the boys. Then one day he woke up and said he was hungry, says the mother,smiling. Recovery was fast after that. Soon Bablu was walking and,within a week,he was back in school.
For scientists,the complete recovery of children like Bablu is another piece in the Muzaffarpur puzzle,as it distinguishes the disease from other similar encephalitis outbreaks,in particular JE. Many are already saying that the excessive focus on JE ended up wasting precious years.
According to Dr P K Kushwaha from BRD Medical College in Gorakhpur,who was invited to study the Muzaffarpur belt last year,In Gorakhpur,cases were characterised by involvement of the kidneys,liver,heart and brain,with several cases of compromised brain function,hearing or sight,and paralysis of the limbs. The Muzaffarpur cases have no involvement of any other organ except the brain and,if cured,show fast recovery,with no reports of residual brain deficits,though there may be isolated incidents.
Admits Dr Das: There are several characteristics which make Muzaffarpurs disease different. JE is a post-monsoon disease,while all the cases here are seen before the rains. Here,in case of fever,sponging with cold water and removing all clothes helps,unlike in JE where patients are advised to cover up. In JE,affected children are up to 15 years of age,while here its kids till the age of five-six who bear the brunt.
There are other differences: in Gorakhpur,deaths occur within 48-72 hours of convulsions,while in Muzaffarpur,mortality strikes within a few hours of the first convulsions.
While the treatment is symptomatic,with doctors managing convulsions with anti-convulsant drugs like Diazepam,and fever with paracetamol,in Gorakhpur,other symptoms like internal bleeding,respiratory failure,kidney failure and compromised heart functions assume more importance,Dr Kushwaha adds.
Banarsi Devis grandson Mintu Kumar,5,from Kirti Bharav village had gone to the fields for his morning rituals. He had spent the previous evening playing with friends,despite having fever for the past four-five days. He had so much energy even with the fever. We kept telling him to stay at home and get some rest but he insisted he wanted to play. Could that have led to his disease? she wonders. He had also stopped having any food for the past couple of days,with his grandmother and mother having a hard time trying to make him have some milk at least.
That day,soon after he woke up around 6.45 am,he had a headache and then a vomiting fit. His parents rushed him to hospital,but Mintu passed away the same day. His first convulsions were to be his last. The nausea,the headache and the convulsionscould it all have been neurological symptoms of the mystery disease?
The CDC-NCDC team that went to Muzaffarpur this year included one of the worlds only neurologist-epidemiologists,Dr James J Sejvar,from Atlanta. He was called in as clinicians started looking for more patterns for clues,and noticed increasing neurological symptomsnausea,seizures,unconsciousness and,in a few,altered brain function. Data from this year shows that 98 per cent of the patients in Muzaffarpur reported generalised seizures and 85 per cent had loss of consciousness,with 40 per cent having documented fever.
More specialists were brought in and new investigations to test brain functions were introduced this year. Samples of cerebrospinal fluidthe fluid circulating in the spinal cordwere collected through lumbar puncture and CT scans were done on all children,funded by the state government.
Other national agencies in previous years had collected their own samples,including two samples of brain tissue from deceased patients taken by the NIV in Pune last year. The results were baffling. All cerebrospinal samples were found to be clear,while the brain tissue held no answers either.
The CDC Atlanta examined MRIs of 10 children and reported no signs of inflammation in the brain tissue,according to preliminary findings published in an NCDC quarterly newsletter.
In fact,there were no signs of any infection at all in the CSF. This throws up a huge question. We dont know if any pathogen is involved at all. That could mean our search for viruses as possible causative factors has been in the wrong direction, Dr Das says.
Sarita,6,loves playing among the litchi fields. Her home in Chainpur village is down a narrow lane through them. She has even given names to the bats that hang from the mango trees nearby. Ask her if she might have eaten some fallen litchis half-eaten by bats or other birds and she laughs.
We have a game where the person who gathers the maximum litchis wins. Of couse I may have eaten fallen litchis,but how can litchis make me sick? They make my cheeks red.
Her rickshaw puller father Lakshman Rai also dismisses the litchi theory. I grew up in these fields as did my brothers,father and uncle. How can the fields be blamed? Sarita says they are her home. The trees dont have any fruits right now as the litchi season is over.
Sarita adds: I am famous now in school because I survived the disease. In fact,my teacher made me stand in front of the whole class and tell them about the hospital.
It was in history that experts found clues to suspect litchis. Looking at other Acute Encephalitic Syndrome-like outbreaks in the country,they found that Siliguri had seen a similar situation in 1996,which was eventually traced to imported raspberries. Putting two and two together,they said the cause of Muzaffarpurs mystery disease may be its litchis.
Muzaffarpur is famous for its litchis. The disease is seasonal,coming with the onset of the fruiting season. So our teams decided it was a viable theory. We noticed there were many bats also which ate the litchis,and the half-eaten fruits would fall and be grabbed by the children, says Dr Ram Singh,the NCDC Patna in-charge.
But this also led to inconclusive findings. Liver function tests and kidney function tests on patients to test for toxins have all been negative,with no evidence of absorption of any poisonous agent from fruit or otherwise, Dr Singh says.
Scientists then turned to the environment for clues,looking for triggers that came annually. Heat was picked as one such factor. Doctors at the Sri Krishna Medical College (SKMC) in Muzaffarpur which has treated the second highest number of cases this year here have published two articlesnotably the only peer-reviewed medical publications from the area so farsaying that high temperatures and peak humidity could be leading to heat strokes,which if untreated for long periods may be contributing to the exaggerated symptoms.
In India,since the JE virus is the most recognised common cause of encephalitis,especially in an outbreak,physicians and public health and administrative officials have a tendency to attribute all the epidemics of febrile convulsive disorder in children to this virus, the authors state in the July issue of the Annals of Tropical Medicine and Public Health.
Noting that no infective organism or its antigen or antibody was found in any of the samples tested,the authors say,overall picture of these cases was suggestive of heat stroke.
Other clinicians however discount this theory,stating that episodes of heat strokes cannot be seen in the wee hours of the morningthe time when most children take a turn for the worse.
The topography of the area has also been considered as a possible clue. According to Civil Surgeon,Muzaffarpur district,Dr Gyan Bhushan,In 1934,Muzaffarpur suffered an earthquake which destroyed most of the district,and left it shaped like a bowl. This,many experts have said,has led to collection of water,slush and other infectious agents in this land. This is also a theory.
Mohammad Sarfuddins parents had taken him to Kejriwal Hospital,a trust-run hospital that has received the highest number of cases this year,after trying out a local fakir as soon as he got his first chamki. Many others choose to first try local nuskhas (concoctions) despite repeated awareness campaigns by the administration this year. The disease is characterised by a short gap of four-five hours between the first convulsions and death.
With scientists struggling for answers,last year,the Bihar health authorities finally decided to broaden the nomenclature of Muzaffarpurs illness and term it AES,a term covering over 300 viruses,bacteria,protozoa,other pathogens,toxins,environmental and climatic factors and topographypractically anything and everythingas causative factors. Isolation wards were set up,despite doctors saying the disease was not communicable. Dr Bhushan says the isolation is meant to provide the patients concentrated better care.
The CDC team has given the Muzaffarpur illness an even broader nameAcute Neurological Syndrome. We are trying to capture all possibilities since there was definitive brain involvement. Many patients did not have fever so we are not sure of an infectious agent or any pathogen, a member of the CDC team visiting Patna said.
The search for clues continues. NIMHANS Bangalore which has elaborate paraphernalia for brain cell microbiological testing will be sent samples over the next two months to test for 22 pathogens associated with AES. NCDC scienticists,meanwhile,are planning to travel to CDCs Atlanta headquarters with cerebrospinal fluids from patients by the end of the year to test for new pathogens.
The National Institute of Occupational Health in Ahmedabad is already conducting tests to study the levels of an enzyme known as cholinesterase that is necessary to return neurons to their resting phases after activation.
One aspect that has baffled investigators is how entire villages acquire immunity after one-two children nearby have contracted the disease. The Meenapur block is one such area which has acquired a seeming immunity but where some villages continue throwing up cases. After contributing to the highest toll last year,the block has seen numbers go down this year.
According to Dr Bhushan,There is no clustering of cases from one area,yet after one episode of a few children in the area,in the next few years,these areas have been seen to report a fall in cases. Genetic factors were considered and then ruled out since all the children in a particular family never got the disease,and all patients are not natives.
But there is one one common factor that binds all the children. Crowded into the three main hospitals of one of Indias most backward regions,they all show low sugar levels (hypoglycemia) and elevated sodium levels (hyponatremia).
Investigators say these blood indicators are a no-brainerthe children are all malnutritioned.
with inputs from Santosh Singh