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Painful crisis: Dr Julie Makani’s fight against sickle cell disease

Sunday November 06 2011
CELL

Dr Julie Makani

Dr Julie Makani, 41, from Tanzania, recently received the Royal Society Pfizer Award from the Royal Society, at a ceremony at the Royal Society in London.

Dr Makani is currently working on intervention studies on sickle cell disease through a Wellcome Trust-funded 5-year project on the role of hydroxyurea in the treatment of the disease.

Hydroxyurea is the only drug licensed for treatment of health complications caused by sickle cell.

In studies conducted in the developed world, the use of hydroxyurea reduces the likelihood of what is referred to as a “painful crisis,” — blood transfusion, hospitalisation and death.

(A painful crisis is when red blood cells get stuck in small blood vessels in any part of the body, and cause the sufferer extreme pain.) However, there are no concrete studies done in Africa to determine the best way to use this drug on the continent.

As well as lecturing at the University of Dar es Salaam, Dr Makani is also a consultant physician for haematology and blood transfusion at the Muhimbili National and Teaching Hospital. She is a visiting clinical research fellow, Nuffield Department of Clinical Medicine, University of Oxford as well as principal investigator, sickle cell disease study for the Muhimbili Wellcome Programme.

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The Royal Society Pfizer Award was unexpected. “When I received the e-mail, I was shocked but excited. It means a lot to me and the programme.

A lot of people have been working under difficult circumstances to provide care to patients as well as training research personnel. The award sends a positive message to African scientists that it is possible to conduct quality research in a busy public health institution like Muhimbili.

That it is possible to do clinical research that contributes to an area like genetics and also benefit our communities,” said Dr Makani.
Part of the money she has received will be used to place two of her colleagues in a long-distance post-graduate training programme on haemoglobinopathies at the University College, London.

Dr Makani first learnt of sickle cell in her teens when her cousin Mary Ngelela, who often visited their home, came around with her four brothers.

Three of the young men had sickle cell, the youngest in his teens. One of them, Richard, had late onset sicke cell and suffered his first painful crisis while visiting Dr Makani’s home.

“I remember coming down the stairs and seeing Richard in severe back pain.

To ease the pain, he positioned himself such that his lower body was on the chair while his upper body dangled down. It was traumatic to see my cousin who was only a few years older than me in such pain,” Dr Makani said.

Richard was rushed to hospital and diagnosed with sicke cell disease.

The severe pain he was suffering from is part of the spectrum of debilitating painful episodes that people with sicke cell suffer throughout their lives.

This personal encounter with the disease was instrumental in shaping Dr Makani’s decision to dedicate her research career to studying this disorder.

Educational background

Dr Makani attended St Constantine’s Primary school in Arusha, Tanzania, which had a unique philosophy that kindled her interest in science. “There was nothing like a stupid question.

The teachers encouraged us to examine known facts and learn more through reading.

The teachers who inspired me were those who said ‘That’s a good question but I don’t know the answer. Let me read and find out,’” Dr Makani said.

After completing her A-levels, Julie spent a year in national service and later started her medical degree at the Muhimbili University.

In her fourth year, she did an in elective in the UK. She was impressed by the way the medical students learned and decided this was where she would do her post-graduate studies.

She graduated from medical school in 1994, immediately got married and while finishing her internship at the Muhimbili Hospital, had her first baby.

“A friend once advised me that if I wanted to pursue a demanding career, the kind of person I married would have a huge bearing on my success and that is why I accepted the marriage proposal from my boyfriend.

I knew that I wanted both a career and a family life and he understood,” Dr Makani said.

In 1997, Dr Makani got a Commonwealth scholarship for a post-graduate diploma in internal medicine at the Hammersmith Hospital, Royal Postgraduate Medical School, University of London. It was a steep learning curve, both academically and socially. While there she sat for the Part 1 examination to become a member of the Royal College of Physicians of the UK.

“It was the first time in my life that I failed a significant exam. But I recovered from the shock, studied harder and passed it on the second sitting,” Dr Makani said.
Four days after the birth of her second child, Dr Makani sat an interview for a six-month job at the Hammersmith Hospital, which for foreign doctors was considered near impossible. “This time, I left my two children with my husband in Tanzania.

It was hard leaving them especially since the youngest was only four months old, but I focused on my work and learned to deal with the difficulties,” she said. While in the UK, Dr Makani developed a proposal to study cerebral malaria back at Muhimbili, with the support of Oxford University.

In the meantime, her cousin Richard, who continued to suffer a mild form of sickle cell, had managed to complete seven years of medical training in Russia.

He had returned to Tanzania, finished his internship and was working as a doctor. Infortunately he suffered a stroke as a result of sicke cell disease and died suddenly.

Richard’s older brother who also had the disease died soon after, leaving behind two young children. Three of Dr Makani’s cousins had now died from complications related to sickle cell disease.

Personal loss

In 2003, Dr Makani was awarded a PhD training fellowship by the Wellcome Trust to study sickle cell in Tanzania. “Although I was excited by the opportunity of starting research on sickle cell, I remember my father saying, ‘I wish you had started working on this disease earlier, perhaps you could have saved your cousins’ lives.’

It had been a difficult time for us as a family,” Dr Makani said.

When she started her research, whose aim was to look into the causes of illness and death in individuals with SCD in Tanzania, she was struck by how common the disease was in the community.

“We had expected to recruit 400 SCD sufferers in the first year but we recruited 1,000,” Dr Makani said.

Family

Closer home, her cousin Mary Ngelela noticed that her deceased brother’s children were exhibiting symptoms similar to the ones she had observed in her brother.

“When Mary brought her nephews to us for testing, it was devastating to discover that both boys had SCD.

It became obvious then that a support structure was clearly needed for families dealing with the repercussions of this condition,” Dr Makani said. She co-founded the Sickle Cell Foundation of Tanzania to help raise awareness of the disease.

The foundation works closely with the Ministry of Health and Social Welfare to develop a national programme for the management of the disease in Tanzania.

Dr Makani is now working towards intervention studies.

Although she has a busy working life, Dr Makani says a balance is possible. “Being a mother, wife and having a career are all integral parts of life. Women should not feel that one should take precedence over the others,” Dr Makani says.

She spends her free time with her three daughters, “doing whatever it is they want to do.”

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