Rare GBS disease: Early detection, proper treatment matter

Fatema Sharmin thought it was just a common joint pain and stiffness.

So, she started taking arthritis pills. But the incredible pain would not subside even after two days. She now started feeling as if her legs and hands were becoming like bricks – unfeeling, heavy and cold. She was taken to Lakshmipur General Hospital.

The doctor there asked Fatema if she had fever or diarrhoea recently. She said "yes" – Fatema had both around 10 days ago and the 22-year-old housewife assumed food poisoning. Doctors at the southern Bangladesh district hospital referred her to National Institute of Neurosciences and Hospital in capital Dhaka.

Fatema was brought to the neuroscience hospital that night and doctors conducted several tests the next morning. She was diagnosed with Guillain-Barré syndrome (GBS) – a rare disease that attacks the nervous system. Fatema, like many others, never heard of it.

The rare prevalence of GBS caused by bacterial infection is much higher in Bangladesh – almost double the global prevalence – and death from the disease is about five times higher.

Doctors wonder why it is so – unclean living and diarrhoea may be some of the causes.

So, when Fatema was diagnosed with the disease doctors said her treatment would cost around Tk10 lakh, but even then nobody could guarantee that she would be able to walk again.

"I got frightened seriously thanks to the series of diagnoses early in the morning, then a hard-to-spell rare disease and now the treatment cost. I was thinking about my son who was just four at that time," said Fatema.

She developed a severe breathing issue as soon as doctors administered her the first dose of the medication. She was rushed to the intensive care unit and put on a respiratory support machine for seven days where she received the remaining doses of the drug. With some physiotherapies prescribed, the hospital then discharged the patient.

"It took me nearly seven months to return to normal life. Now I am quite okay, but I still feel pain in the winter," added Fatema.

She thanks the doctors for detecting the disease early and proper treatment that she says gave her a new life.

"If I had any knowledge about the disease, I would go to doctors on the very first day and that might have lessened the sufferings I had to endure," she noted.

Higher prevalence

Although GBS cases hover around 2-3 per 100,000 in other countries, the rate is more than 5 in Bangladesh, said doctors. Bangladesh registers at least 700 GBS cases a year while 70% of the cases are from rural areas.

The disease is caused by a bacterial infection called Campylobacter jejuni. GBS symptoms develop within two to three weeks of infection – patients start to lose the sensational feeling and reflex rapidly and limbs at some point become totally numb. Many lose the ability to move, breathe and die eventually.

In developed countries, the GBS mortality rate is 3%-5% while the death rate is 14%-19% in Bangladesh.

The International Centre for Diarrhoeal Disease Research, Bangladesh (icddr,b) – the inventor of oral saline – has been conducting research since 2006 to find out the intensity of GBS in Bangladesh.

Dr Zhahirul Islam, a key scientist at the icddr,b, said, "GBS is quite frequent in Bangladesh. The infection rate among our children is four times higher than in other countries."

The scientist noted that though there has been a higher GBS severity, most of the patients recover from the disease.

He attributed the higher GBS mortality in Bangladesh to fragile medical infrastructure, saying two out of every 10 patients require mechanical ventilators for respiratory support.

"Recovery after lengthy treatments leaves huge medical bills that put many low-income families in serious financial trouble," he added.

Environmentally exposed

Dr Zhahirul Islam said those who have diarrhoea more often are exposed to GBS risks since they found 50% of GBS patients have a history of the water-borne disease.

He said the remaining cases are due to respiratory problems and other unknown reasons.

The scientist said western researchers thought genetic issues are responsible for higher GBS prevalence in Bangladesh, but a number of icddr,b research shows Bangladesh is not genetically but environmentally exposed to the disease.

"We do not maintain proper hygiene. Many rural people still drink surface water. Our hypothesis is that since people in southern Bangladesh are more exposed to poultry waste and have less access to potable water, the GBS cases are more common in the region," he added.

According to icddr,b findings, GBS prevalence in northern and central Bangladesh with a better access to safe potable water is low compared to the south. The centre also found there is a seasonal fluctuation in GBS frequency as most cases are recorded in Bangladesh in the summer and the lowest in the winter.

Scientists believe this is because summer has a high humidity when numerous viral and bacterial infections are more prevalent and diarrhoea also increases.

Despite Bangladesh's remarkable achievement in safe water and sanitation, as many as 6.8 crore people still lack access to safely managed drinking water, while 10.3 crore are deprived of safely managed sanitation facilities, according to a report by the World Health Organization (WHO) and Unicef this year.

80% patients can recover if GBS detected early

Mohammad Uzair Abdullah, a private firm employee in Dhaka, rushed to the hospital as soon as he found his one and a half years old boy was suddenly unable to stand at all. Doctors diagnosed the kid with GBS and started treatment.

After administering 10 injections in five days at a cost of Tk1.30 lakh, the kid was discharged. Then continuing physiotherapy for months, the child was able to walk again.

"My boy is completely okay now. He is taking calcium supplements prescribed by the doctors," said the father.

Neuroscience hospital physician Jahirul Hoque Chowdhury said the recovery rate of their patients is around 80% if the disease is detected at an early stage and patients get proper treatment.

A dirty combination plus jinn

Dr Jahirul Hoque said unhealthy living and a lack of health awareness is a dirty combination in Bangladesh that contributes to higher prevalence of diarrhoea and other infections.

"Since GBS is caused by a gastroenterological infection, we have a higher rate," he noted.

The doctor said rural parents often come to doctors late as they believe prejudices like possession by a "jinn" (an evil spirit) has resulted in the paralysis of their children.

Uzair Abdullah said a kid who was in the hospital bed next to his son had his lungs infected – a tertiary stage of GBS.

"His illiterate parents initially assumed the kid came across a jinn and went to an exorcist. They came to the hospital at the last stage as the health of the boy kept deteriorating," he added.

Besides, an inadequate detection facility and treatment in rural Bangladesh also contributes to the problem.

Doctors call for maintaining hygiene

Dr Jahirul Hoque said although there are no confirmed preventive measures for GBS, maintaining health hygiene and cleanliness is a must to reduce the risks.

Scientist Dr Zhahirul Islam also said people working at poultry farms must maintain hygiene. Rural people should boil surface water and then drink it.

Both of them said many GBS patients come to hospitals late, while early detection could have saved many of them. They emphasised raising mass awareness about the disease.

Efforts on to develop low-cost treatment

Doctors say as many as 86% of the patients in Bangladesh cannot afford the medical bills of GBS.

Currently, there are two established treatment methods available in Bangladesh – intravenous immunoglobulin (IVIG) and plasmapheresis. But both the methods are costly and limited medical facilities in Dhaka offer the treatments.

According to scientist Dr Zhahirul Islam, the IVIG is a common method and can cost up to Tk10-15 lakh. The second method, plasmapheresis – available only at two to three public hospitals in Dhaka – is taken by only 3% of the patients which costs them more than Tk5 lakh each.

The treatments also require ICU support and physiotherapy for patients adding to the medical bills.

A group of doctors from the Bangladesh GBS Group – icddr,b, neuroscience hospital and Dhaka Medical College Hospital – have been working to develop a new and low-cost treatment strategy. They group is hopeful about the project as they say it would benefit the low-income people.