It’s Friday. Luke and Elsie are home from school early and having a snack with their mum.
‘What are we going to do this afternoon, mum?’ says Luke.
‘I’ve got to go to the chemist first to pick up my medicine then we can do whatever you want to do.’ replied their mum.
‘Why do you need medicine?’ Elsie asks.
There are many cells in your liver. Tap or click on one of them, you'll be able to see how it works.
The cholesterol in your blood that harms your arteries, is called LDL. Click for more information.
At the core of your cells you'll find DNA. DNA contains all the information about your body. Every human being has little mistakes in the DNA. People with FH have a mistake that makes them have less fishing rods.
This is your liver. Your liver is like a small factory. It takes cholesterol out of your blood.
Cholesterol is fat that your body needs as a building material for cells and hormones. Cholesterol is found in the blood as LDL and HDL.
HDL (good cholesterol) is like a vacuum cleaner: it helps remove cholesterol from blood vessels. This is how the cholesterol level in your blood vessels is controlled. LDL (bad cholesterol) is the type that sticks to your arteries and gets in and furs them up. When you get older, this might cause a heart attack or stroke.
At the core of your cells you'll find DNA. It determines whether you have FH or not. Click for more information.
healthy coronary artery
coronary artery from a smoker
Slide up the yellow arrow to peak inside
Inside your Body
The harmful chemicals in cigarette smoke pass through your lungs and into your arteries, just like oxygen does, but the difference is that these harmful chemicals make the artery walls less smooth and so the LDL sticks more easily to them, and then gets into the vessels. Over time this can build up and eventually block the artery.
Not only is smoking bad for your lungs but also for your heart and your arteries too. Smoking makes your artery walls rougher but did you know that some vitamins found in fruit and vegetables help them stay smooth, so the cholesterol finds it hard to stick?
'I have enough fishing rods, don’t I, mum?' says Luke. Mum smiles. 'We’ll get the doctor to check that you both do.' 'How?' says Luke.
'It’s easy, they just take a little bit of blood from your arm or finger and give it to a laboratory where they’ll be able to see how much cholesterol is in it. Sometimes the lab will check your DNA too and your mum and dad’s.' 'What’s DNA?' Elsie asks.
'It's what makes you who you are and is made up of parts called genes. They decide whether you are a boy or a girl, what colour your hair is and... the number of fishing rods in your liver. You’ve inherited half of your DNA genes from me and half from dad. Now, your grandma has a mistake in the gene that makes her fishing rods so she doesn't have enough and I got that same gene from her. People with a mistake in this gene have a condition that is called familial hypercholesterolaemia or inherited high cholesterol but we call it FH, which is much easier to remember and say.'
Since the discovery of FH, doctors and researchers have tried very hard to find people in the general population who have FH, so they can be given statins to lower their cholesterol and to prevent them getting early heart disease. In most countries in the world we think that about 1 in 250 people have Familial Hypercholesterolemia, so we think that in the UK there are about 200,000 people who have FH. However only about 20,000 of them have been found so far, and the rest are not being treated and are at high risk of early heart disease. Therefore, letting your relatives know that they may have FH will mean they can go to their GP and ask to be tested. Once the precise change in the DNA that is causing FH in a family has been found, it can easily be tested for in relatives. This can be done on a small sample of blood or even saliva! Children should be tested before they are 10 years old, but they can be tested much earlier if the parents ask for this. Children with FH should be advised to eat a healthy diet and get lots of exercise, and should be told that smoking is very very bad for them!
Most people with FH don’t know that they have FH!
More than a hundred years ago, a Russian military doctor called Nicolay Anichkov showed in rabbits that eating a diet containing a lot of fat causes more heart disease. This was the start of research into the health problems caused by too much fat in blood. In 1985, two American scientist called Mike Brown and Joe Goldstein were given the Nobel prize for their discovery of the cause of the genetic disease of Familial Hypercholesterolemia. They first discovered the "fishing rods" on the liver that take cholesterol out of the blood. As a result, they could also look for a drug that could help increase the number of fishing rods in the liver. They found the first statin drug called lovastatin, a medicine that helps to get cholesterol out of the blood. Later, better statins were invented. Almost all people with FH now take stains to keep them healthy.
Discovery of Cholesterol / Familial Hypercholesterolemia (FH)
Luke and Elsie go to the hospital with mum and dad. In the corridors they see lots of doctors and nurses. As they get in the elevator an old man in a bed is pushed in by a porter.
'What are you two kids doing here?' he asks smiling. Elsie tells him that they are going to have a blood test to see if they have enough fishing rods. The old man’s eyes twinkle. 'Well, I only go fishing on Sundays, so if you don’t have enough, you can borrow some of mine’ he looks a bit fierce 'but don’t lose them.’ Luke and Elsie shake their heads seriously then the old man laughs. ‘Only joking,’ When he gets out they wave him goodbye.
Luke puts on a brave face when the doctor takes his blood. It’s alright though, and he hardly feels the little scratch the needle makes.
Afterwards mum says that as it’s a special day they can have a pizza and then go to see a film.
Doctors can help you stay healthy with FH. But there’s a lot you can do too. Taking your medicines every day, eating healthily and keeping fit.
From the age of 8-10 years, children with FH may be given medicines to lower their LDL cholesterol. Usually it’s a little pill you take every day called a statin. Just like cleaning your teeth to keep them clean and healthy, you take your pill to keep your blood vessels clean and healthy.
Wat doet stetine?
Eten > mond > darm > vet in bloed > hengels
pillen zijn schoonmakers van bloedvaten (tandpasta?) Zonder pillen slibt je bloedvat dicht.
Stay healthy with FH
Luke, however, didn ‘t have enough fishing rods so he’ll have to take a pill, just like his mum. The doctor explains more about FH and how eating and drinking healthily and keeping fit is really imortant too. Luke finds it really interesting but is worried he won’t remember it all but his mum says that he can always ask her. The doctor makes him feel that although he has FH, he can still have a normal life, like his friends.
A few weeks later they all go back to another doctor for the result of the blood tests. He tells Elsie that she has a normal amount of fat in her blood so she has enough fishing rods and doesn’t need any medicine.
This is very rare and happens when a father and mother both pass on FH genes to their child. That is called homozygous FH. A child with this has hardly any fishing rods. Taking a statin may help and there are new medicines being found that may also help. There is also a special machine that takes the fat out of the blood just like the fishing rods.
The doctor laughs, 'Of course you can, but only now and then, just not every day, and make sure you keep fit. Swimming, running or any other sport is good. Not just for you, Luke, because what’s healthy for people with FH is healthy for everybody else too.'
Eating and being active
By eating a healthy diet, the LDL-cholesterol (a fatty material in your blood, that isn't good for you if there’s too much of it) can be lowered by 10 to 15%. By being active you are raising the HDL-cholesterol (the fatty material in your blood that helps to keep the arteries clear). This is the way the cholesterol levels in your blood are lowered.
Older children (from the age of 8) can take a medicine. This makes sure that you have the right levels of cholesterol in your blood and will be able live a long and healthy life.
The doctor asks Luke and Elsie what they’re doing this weekend. They tell him they’re going swimming.
'And then we’ll have some chips.' Luke suddenly realises what he’s said and says unhappily that he can’t do that anymore, can he?
Colour in the picture
Find out about FH
What do you eat?
Draw your own family tree
Create an outdoor play garland
8-11 years old
What does the doctor measure?
6-8 years old
Info sheets and things to do.
What do you know about FH ?
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More information and links:
Telephone number: 0345 4505988
Production: LVB en De Hart&Vaatgroep
Story: Jessica Langenhoff
Animations: René Overhorst
Illustrations: Hanneke van der Meer
English Adaptation: Prof Steve Humphries, Uma Ramaswami, Phil Rowlands
Thanks to everyone, who has contributed to this booklet, especially the children, parents and FH experts Dr. Bert Wiegman, Dr. Petr Jira, Professor Steve Humphries and Uma Ramaswami.
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Here are Luke, Elsie and William. They all look the same, just ordinary children. But two of them are extraordinary. They have FH and take medication. Click the hands to find out what their medicine is and how it works.
William has got FH. The statins work well but his cholesterol is still higher than the doctor would like, even though he is active, plays lots of sport and has a healthy diet. So he is given another tablet to take. This medicine is called Ezetimibe and it stops cholesterol from food being taken into the body in the intestine.
After a few weeks of being given this new medicine, William has a lot less cholesterol in his blood, and the doctor is happy with the results. So is William!
Luke has got FH. Every day he takes medication, a little pill. He eats a healthy diet, and is very active. Inside his pill is a substance that is called 'statin'. It lowers the amount of cholesterol in your blood.
There are different kinds of statins and you and your doctor can choose the 'one' that suits you best. If a medicine works very well for your father or mother, it mostly works really well for you too.
Taking a statin is like using toothpaste to clean your teeth. Take a little pill each day and your arteries are kept clean. After a few weeks of taking the pill Luke has got a lot less cholesterol in his blood.
Elsie hasn't got FH. Therefore she doesn't need to take any medicines. But just like everyone else who doesn’t have FH, it is important that Elsie eats a healthy diet and keeps active and fit.
Elsie: no FH
William: FH+Statins+ Ezetimib
FH and Medicine
A Story of Fishing and Cholesterol
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'Okay, do you know where your liver is?’ Luke and Elsie smile and tap their tummies. ‘Smartypants,’ says Mum. 'Well, the liver is like a little factory for your body which makes cholesterol. Our body needs some cholesterol, but not too much. Cholesterol travels around our bodies in the bloodstream so it can reach the body parts that need it. These body parts use things like little fishing rods to catch the cholesterol from the bloodstream.'
'If there is too much cholesterol in our blood, the liver uses its fishing rods to fish out any extra cholesterol and gets rid of it. If you have the right number of fishing rods then it’s all good, but if you don’t have enough, like me, too much cholesterol stays in the blood. Over time this can block up the blood vessels. The medicine I take stops this from happening.'
'How?', Luke and Elsie ask.
'It stops the liver making and sending out too much cholesterol.' Mum laughs. 'Which means I stay healthy and we can still have lots of fun doing things.'