Lupus – affecting children and teenagers
Reproduced from Issue 137 of Arthritis Today
Lupus is a severe and debilitating rheumatic disease affecting the joints and many internal organs. Some 20 per cent of patients develop lupus as children. Here, one teenager tells her story, and we report on how arc research will find out more about lupus in children.
Natalie Kirkham with her younger sister
A couple of weeks before Christmas 2005 14-year-old Natalie Kirkham started feeling unwell, developing a sore throat and breathing difficulties
The Liverpool teenager had barely had a day of illness in the past, so at first her family were not unduly worried. However, what followed was a protracted nightmare which resulted in the teenager losing three stone in weight, having a litre of fluid drained from her lung, a chest drain in place for five days and spending eight weeks in hospital.
After the initial sore throat Natalie came out in a rash all over her body and started to feel extremely unwell. She was given antibiotics for a suspected chest infection; glandular fever and even chronic fatigue syndrome were other possibilities. She went to and fro to her GP surgery with her worried mum Shelley, who also took her to A & E several times. By this time she had also developed high temperatures, had lost a lot of weight, aching muscles and could barely get up from the couch. Blood tests were inconclusive.
Finally, by March 2006, back in hospital again, a physician at long last realised that either juvenile idiopathic arthritis or juvenile lupus could be the cause of Natalie’s health problems. But it still took several more days to admit her with severe lupus. She then spent eight weeks in hospital undergoing further tests and treatment.
“It was a terrible time, no-one knew what was wrong with her and she was very, very poorly,” says mum Michelle. “To go from being such a healthy girl to someone who was so ill was very distressing.”
Natalie was immediately put on steroids and had a biopsy to check her kidney function. Then the fluid was drained from her lung. She was put on powerful drugs; cyclophosphamide, prednisolone and hydroxychoroquine which slowly brought the disease under control and reduced her high blood pressure.
“It is a life-long and life-threatening disease but is often not diagnosed early because its symptoms mimic those of other diseases.”
Juvenile lupus is an autoimmune disease that can affect the joints, skin, muscles, blood vessels and internal organs and can have a huge impact on a child’s health as well as growth, psychological well-being and learning through into adulthood. It is a life-long and life-threatening disease but is often not diagnosed early because its symptoms mimic those of other diseases. Some 20 per cent of people develop it in childhood.
Although lupus can occur at any age and affects many adults, particularly women, it is often more severe in children.
Astonishingly, given the severity of Natalie’s lupus at the onset, her condition is now stable, although as lupus is not a curable disease, but comes in flares, she will continue to be seen by her rheumatologist on a regular basis. Despite missing six months of school she managed to catch up and has recently taken her GCSEs, and now has ambitions to be a children’s nurse. She joined the gym on her recent 16th birthday.
Adds her mum: “Natalie is a bright girl and has a very positive attitude. She has picked up with her school work and her friends. Some nights she still gets tired but the lupus doesn’t really stop her doing anything. She has a check up at Alder Hey every three months and the doctors are delighted with how well she has done.”
Neither Mrs Kirkham, nor Natalie, nor anyone they know had ever heard of lupus, let alone childhood lupus, and both are keen that more people should be aware of the condition.
Now a Liverpool medic is hoping to find out more about the development of the rare but serious disease that can have a devastating effect on young sufferers - in order to come up with better treatment.
Dr Zoe McLaren: new lupus research
Dr Zoë McLaren, a specialist registrar in rheumatology in Liverpool, has been awarded a one-year Barbara Ansell Fellowship of £53,500 by arc to investigate the cause of childhood lupus. The study will take place at the Royal Liverpool Children’s Hospital, Alder Hey, and the Institute of Child Health, University of Liverpool.
Better treatment is needed
“Treatment is available for the condition but there is no cure, and we don’t fully understand what causes lupus,” explained Dr McLaren. “A number of factors are important – genetics, infections, hormones and environmental influences. We know a little from studies in adults but the disease in children appears to be different in many ways.
“Natalie’s story could have been dramatically different. The survival rates for childhood lupus have improved with advances in treatment since the turn of the millennium. And ten-year-survival rates are now close to ninety per cent. However, it is still a very serious illness, and Natalie’s condition is only ‘under control’ because of the strong drugs which suppress her immune system. She will need to continue taking these medications for the rest of her life and will continue to be under the care of rheumatologists in order to monitor her disease and the complications of her treatment.
“By examining the differences between adults and children we hope to understand more about the abnormalities of the immune system in this serious condition in order to help young people like Natalie.”
Lupus is an autoimmune disease, which means the immune system, especially white blood cells, is affected and ends up attacking the body’s own cells, causing disease and organ damage.
Dr McLaren will be studying the role of important white blood cells called neutrophils to find out what part they play in the development of lupus. Her research is part of a larger study being conducted by her supervisor Dr Michael Beresford who is setting up a nationwide database of childhood lupus patients, who will provide samples of DNA to be used for research purposes.
“I am very excited that arc has given me the opportunity to study this serious disease affecting children like Natalie,” she added. “So little is known about lupus in children and research into the disease is desperately needed. This study provides a unique opportunity to understand how the developing immune system affects lupus and how this compares to disease in adults.”
To read the arc information booklet on lupus please click here.
