Saturday 21 September 2019

Lupus: What to do when the body attacks itself

Jessica Doran knew something wasn't right, but it took weeks of tests and agonising pain before she was finally diagnosed with lupus

Jessica Doran endured weeks of suffering before finally getting a diagnosis. Photo: Dave Meehan.
Jessica Doran endured weeks of suffering before finally getting a diagnosis. Photo: Dave Meehan.
Jessica Doran has grown closer to her parents, as a result of her condition

Shane Cochrane

IT SHOULD have been an enjoyable night out with friends to celebrate her 17th birthday. But during the meal, Jessica Doran began to feel unwell. "I started getting cramps in my legs," she says. "I'd had them before, but this was really bad. And then the pain started in my hands too.

"I tried to get out of my seat to go to the bathroom, but I couldn't. I started panicking. I couldn't stand up."

Jessica's mother took her home. When morning brought no relief, she took her to A&E.

"They did a few blood tests, but they couldn't find anything. And when they examined me, there was no inflammation."

Jessica's GP referred her to a rheumatologist. There was a six-month wait at the local hospital. Not wanting their daughter to endure another day of this, Jessica's parents opted to go private. This still meant a wait of a few weeks.

"By the time I got to see the specialist, my symptoms were at their worst.

"I'd gotten so bad, I couldn't get up out of my bed without crying," she says.

"On the morning of the appointment, my mam had to put me in the bath. She had to actually get me into the bath – and hold me up. She dressed me. She linked me to the car and fastened me in.

"I couldn't do anything without being in really bad pain." Strange as it may seem, this was a good thing; the rheumatologist couldn't miss these symptoms.

"She was the first doctor to actually see that there was something wrong. That day, all my joints were really swollen."

Jessica was subjected to a number of tests and examinations. But she also took the opportunity to voice her own suspicions as to what was wrong.

"I wanted to know what was wrong with me, so I had done some research. I'd found an article about a girl with lupus, so I asked the rheumatologist, 'could it be this?'"

Lupus is an autoimmune condition. Normally, when the body's immune system detects a virus or bacteria, it releases antibodies to kill the infection.

But lupus causes the immune system to release antibodies that attack healthy cells. The body – quite literally – attacks itself.

There are a number of different types of lupus, but when we talk about lupus we generally mean systemic lupus erythematosus, a form of the condition that can affect any part of the body.

While the nature and severity of the symptoms will vary, most lupus sufferers will normally experience joint pains, skin rashes and fatigue. Fevers, high blood pressure, headaches and migraines are also common.

Because many of these symptoms are indicative of other, much more common, conditions, and because existing blood tests can't provide a definitive result, lupus is very hard to diagnose.

Jessica's rheumatologist ordered a blood test for lupus.

It came back negative. But Jessica did have lupus, and she would have to endure weeks of agony and sickness before it would be diagnosed.

The rheumatologist put her on steroids "just to get me through". But, as her symptoms worsened, higher and higher doses were needed.

"It was really bad. I live five minutes from the school, but my parents had to drive me there and pick me up again. My friends had to carry my schoolbag.

"I barely got through my Leaving Cert. If an exam had lots of writing, I'd have to give up half-way through. The pain in my hands would get too bad."

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Jessica Doran has grown closer to her parents, as a result of her condition

 

The rate of Jessica's deterioration increased. "At the same time every day, I'd get the shakes. And they weren't normal, like you'd get when you're cold. I'd be shaking for an hour, and I'd be freezing – but my temperature would be sky high. I'd have headaches. And my joints ... I could barely move."

Jessica's rheumatologist decided to try Methotrexate, a drug used to treat the pain and swelling of rheumatoid arthritis.

Because Methotrexate works by suppressing the immune system and leaves the body less able to fight infections, Jessica was to have flu and pneumonia vaccinations.

But within 24 hours of receiving the pneumonia jab, Jessica's temperature rocketed and she started shaking uncontrollably.

"When I got to A&E, the rash had got really bad. It had been on my arms, but now it was on my legs and on my face.

"The doctors were treating me for meningitis. All the other tests said that it wasn't meningitis, but the doctors were shocked at just how bad I was, so they kept me in for more tests."

One of the tests was a skin biopsy to determine the nature of the rash. It was this biopsy that finally told the doctors what Jessica had long suspected: she had lupus.

She was immediately given Plaquenil, an anti-malarial drug used to treat lupus symptoms.

But Jessica's ordeal was far from over. Her body was struggling to cope with being so inflamed for so long.

"I started getting this really bad headache. I had a really high temperature. It was so bad I couldn't open my eyes, and any noise at all was really painful.

"They gave me sleeping tablets and painkillers and stuff. The next day I was really bad. I had a sky-high temperature and they were trying to cool my body down. I was in so much pain, I was shouting and screaming. I couldn't cope with the pain.

"The nurses had to keep pushing the emergency button to get the doctors. I was really scared, and my mam was really upset."

But, slowly, the medication began to take effect; Jessica's temperature dropped, as did her inflammation levels. She was kept in hospital while they treated her with Methotrexate.

"I started recovering. They also put me on a high dose of steroids, and in a couple of days I could actually start moving again. It was amazing. I hadn't had that kind of mobility in months.

"I felt like a new person. I could actually get up out of the bed and walk. I could do simple things again. It was such a good feeling."

On its own, lupus is an extremely debilitating condition. But it also brings with it a number of medical complications. It causes inflammation of the heart and arteries, which greatly increases the risk of developing cardiovascular disease. It can cause a kidney disease called lupus nephritis. And about one-third of lupus sufferers will have another autoimmune condition.

Not so long ago, one of these complications would have killed someone with lupus within 10 years of developing the condition. That lupus sufferers now have a "normal" life expectancy is due, for the most part, to drugs.

Anti-malarial drugs are used to treat the main symptoms of lupus, the skin rashes, joint and muscle pains, and fatigue, and are regarded as the best method of long-term lupus management, while immunosuppresants are used to treat severe cases of lupus.

Non-steroidal anti-inflammatory drugs and steroids are also widely used.

But despite their efficacy at treating the symptoms, these drugs can't cure lupus, and they can have some serious side effects.

Anti-malarial drugs can cause headaches, rashes and diarrhoea, and, in a small number of cases, can damage the patient's eyesight.

Immunosuppresants can cause convulsions, liver damage and, because they weaken the immune system, susceptibility to infections.

However, researchers at Northwestern University are in the early stages of developing a non-toxic therapy for lupus. The therapy utilises peptides – small bits of protein found in the body – and has been successfully tested on blood samples taken from lupus sufferers.

"We found that the peptides could not only generate regulatory T cells, but also that they could block and reduce antibody production," says Dr Syamal Datta, who is leading the research.

Regulatory T cells are a type of white blood cell that help keep the immune system in check, and help prevent autoimmune diseases.

"This approach shows that the peptides have the potential to work like a vaccine in the human body, to boost the regulatory immune system of those with lupus, fight autoimmune antibodies and keep the disease in remission," he says.

The therapy's efficacy is second only to its apparent lack of side effects.

"We have not seen any side effects in our extensive pre-clinical studies," says Dr Datta. "The peptides are endogenous, being part of the body's own proteins that are used to educate the developing immune system."

However, a vaccine for lupus may be some way off yet. Dr Datta's therapy requires clinical trials in humans – trials that require the resources of a major pharmaceutical or biotechnology company.

"Despite a great deal of effort, we have not yet heard from any companies with an interest in sponsoring a clinical trial. It may take some time – maybe years – and millions of dollars to develop a drug."

Until then, to keep their symptoms under control, lupus sufferers will have to endure the risks of their medication, and the limits it can place on their lives.

"I really wanted to be a nurse," says Jessica. "That was my dream. I talked to my doctors, but they said it wouldn't be the best option, given the medication I'm on. I'm not supposed to be in hospitals. I can't be around sick people."

But some good has come out of Jessica's experience.

"While I had a relationship with my mam and dad before, I wasn't really close to them. When I was sick, however, I depended on them and I discovered just how much they are there for me. I respect them more, and we have a really close relationship now."

Lupus Group Ireland provides support for those living with lupus. Details available at www.lupusgroupireland.com or telephone 089-424 1203.

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