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Your Life: Dear Miriam - Condemned by their genes to a life of pain; HEALTH.


Thousands of sufferers have an appalling illness that's likely to kill them in their 40s - yet few people have heard of it.

Sickle cell disease (SCD) gets little media coverage, even though Britain has 12,000 sufferers and 250,000 carriers.

So, as this is Sickle Cell Awareness month, I want to increase the profile of this painful and debilitating lifelong illness.

If you've just had a baby with SCD, carry the sickle cell gene or you have the disease yourself, here are the best ways to deal with it.

What is it?

It can't be caught and people with SCD have inherited an abnormal form of the red blood pigment (haemoglobin) in their blood cells. Haemoglobin carries oxygen round the body.

Normal red blood cells can flex but in people with SCD the blood cells are rigid and sickle or C-shaped.

As a result, they can't squeeze through small blood vessels which get blocked.

This stops oxygen from getting through to where it's needed, leading to severe pain.

Sufferers are often well but their lives are marred by painful attacks, called crises, and the risk of other complications, including chest infections, organ damage, strokes, gallstones and bone infections.

Life expectancy is around 42 years for men and 48 for women but scientific research is ongoing to try to increase this.

Who gets it?

It's most common in people (or their descendants) from sub-Saharan Africa, Asia, India, Caribbean and the eastern Mediterranean.

We all have two genes for haemoglobin - one inherited from each parent.

If one parent carries the sickle cell gene, a child could inherit it and become a carrier.

Carriers are usually healthy with normal life expectancy.

But if both parents carry the gene, there's a one in four chance a child could inherit a sickle gene from each of them and have SCD.

How do you know you're a carrier?

If you suspect you may be a carrier, ask your GP to refer you for a genetic test.

How it's treated...

From drugs to surgery

Pain killers

A crisis can last from a few hours to several weeks. For milder crises, non-steroidal anti-inflammatory drugs (such as diclofenac or naproxen) may be enough. However, in the most severe crises, sufferers need hospital treatment with intravenous morphine or pethidine, and possibly antibiotics if there's an infection.

In acute chest syndrome - severe chest pain, fever and breathing difficulties - oxygen therapy is essential and the sufferer may need a blood transfusion.

Folic acid

Children born with sickle cell disease need to take folic acid daily for life to help produce red blood cells quickly.


From birth to five years old, they'll also have to take penicillin daily, due to the immature immune system which makes them more prone to early childhood illnesses.


This drug helps decrease the number and severity of attacks by boosting production of foetal haemoglobin.

Bone-marrow transplants

Bone-marrow transplants have proven effective but a shortage of suitable donors means getting the right match can be difficult.

Iron-removal drugs

After repeated transfusions, the body can become overloaded with iron, which is deposited in the heart muscle causing heart problems. Taking drugs such as Exjade regularly will help remove it.



There are ongoing studies into the usefulness of omega-3s, magnesium, zinc, African yams, certain herbs and natural antioxidants such as vitamin C.

Scientists are also investigating ways of replacing sickle genes with normal genes, using gene therapy.


(1) Drink plenty of water - even mild dehydration can trigger a sickle crisis.

(2) Eat five to 10 portions of fruit and veg a day. Fruit and veg contain powerful antioxidants, helping to bolster immunity against infections like colds and flu which can trigger crises. Particularly potent are dark leafy greens such as spinach and dark red and black fruits.

(3) Don't scrimp on portions - recent research shows that people with SCD need 20 per cent more calories a day than normal children to replace red blood cells that are destroyed.

(4) Keep your weight under control - obesity can promote early onset of hip bone disease. So keep snacks healthy - such as nuts, yoghurt and fruit bars.

(5) Eat plenty of fibre - medication can cause constipation so it's important to counteract that with fibre. Good sources include whole grains - wholemeal bread, brown rice and pasta, plus fruit and vegetables.

(6) Take it easy - stress, fatigue and overexertion can trigger crises. Stay active but avoid exercise that needs lots of stamina like long-distance running. Try walking, golf or cricket.

I wake up every morning in pain

Sally-Ann Ephson, 41, has sickle cell anaemia. She's single with a 19-year-old son, Donovan, and lives in London

"I had my first sickle cell crisis when I was six months old. Back then my parents didn't know much about the condition and I had a difficult childhood.

My family is from Ghana, west Africa, and back in the 60s and 70s none of the teachers had any idea about the disease.

I spent a lot of my early years in hospital, but it calmed down in my 20s and 30s. My condition is severe.

During a crisis the pain is excruciating - I'd rather give birth. But even when I'm not having a crisis, I wake up every day in pain.

I've also had countless blood transfusions - on average one every six to eight weeks for the past 10 years. Consequently I have too much iron in my blood, and I need to have a drug injected into me for nine to 10 hours a day.

It makes me feel very lethargic. There's another drug called Exjade, which could make my life much better, but my primary care trust has turned down my application.

It's been stressful, which doesn't help as stress can trigger a crisis.

But I've had tremendous support from family, friends and the Sickle Cell Society.

Now I'm studying for a diploma in sickle cell management and would like to teach others about the condition.

I've had a hard life, but maybe I can help change things for people with sickle cell in the future."

Additional research: CHRISTINE M ORGAN


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Title Annotation:Features
Publication:The Mirror (London, England)
Date:Jul 21, 2008
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