Great Ormond Street Hospital for Children NHS Foundation Trust: Information forchildren & young people

Cystic fibrosis medicines This information sheet from Great Ormond Street Hospital explains about the medicines you may have to take if you have cystic fibrosis (CF) and why they are important. Having CF means taking lots of different medicines. Part of growing up and becoming more independent is managing your medicines, knowing what they are for and when you need to take them. The tips in this information sheet will hopefully help you fit in your medication around your busy lifestyle and also give you a bit more information about looking after your medication. If you are having problems with your medication or, have any questions, please talk to us. Remember, we are here to help you.

Your medicines are important

Common medicines

With some medicines, you will notice immediately that they make you feel better – with others this might not be so obvious. However, missing or not taking your medicine can put your health at risk even if you don’t immediately notice any difference in how you feel.


The dose prescribed by your doctor may be different from the dose quoted in the patient information leaflet in the package so it is important that you always follow the directions on the label put on by pharmacy or the instructions your doctor/ pharmacist has given as the dose has been checked and personalised to your specific needs. Medicines have two names: a generic name and a brand name. For example, ibuprofen is a generic name and Nurofen® is a brand name. You may also hear medicines referred to as ‘proprietary’. This is the same as a brand name. Medicines sometimes have different names outside the UK – they may be known by their generic name or the manufacturer may use a different brand name. For example, the generic medicine paracetamol is known as acetaminophen in the USA.

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As the mucus your lungs produce is much thicker than usual, it can be more difficult to clear. This means that bugs you have breathed in from the environment can hang around in your lungs and cause infection. We sometimes give antibiotics to CF patients regularly (prophylactic) because we want to reduce the chances of the bugs causing an infection. These antibiotics are often given by mouth or inhaled via a nebuliser up to twice a day. It is very important that you take your antibiotics regularly as not taking them may lead to infection. Common names of antibiotics you might have include: „„ co-amoxiclav (Augmentin®) „„ azithromycin „„ ciprofloxacin „„ flucloxacillin „„ colistimethate sodium (Colomycin®, Promixin® and Colobreathe®) „„ tobramycin (Bramitob® and Tobi®) Occasionally you may need to come into hospital for an intensive course of intravenous antibiotics. This involves giving antibiotics through a vein directly into the blood stream and is a more aggressive treatment. This is given to try to get rid of the bugs which cause severe infection in your lungs. This admission is usually for 10 to 14 days but sometimes can be for longer.

© GOSH NHS Foundation Trust May 2015

The common antibiotics we use intravenously are called ceftazidime and tobramycin unless there is a reason why we cannot use these. We will look at what bugs have grown in your sputum samples or cough swabs in the past and tailor your treatment accordingly.

Mucolytics These are medicines that reduce the stickiness and/or thickness of the mucus in your lungs making it easier to clear. You normally have these once or twice a day inhaled via a nebuliser. Common names of mucolytics you might have include: „„ hypertonic sodium chloride/saline or sodium chloride 3%, 6% or 7% (MucoClear® or Nebusal®) „„ dornase alfa (Pulmosyme® or DNase®)

Enzyme replacement We all have an organ in our body called the pancreas. Along with other functions, it produces enzymes that help to digest food, in particular fat. In most CF patients (but not all), the pancreas does not produce these enzymes and they need to be replaced by a supplement which makes up for the enzyme your pancreas is not making. The enzyme replacement medication contains three types of enzyme (protease, lipase and amylase), which digest the food you eat so your body can use it to stay healthy. The amount of replacement you take will depend on what and how much you eat. Enzymes should be taken with meals and snacks. Your CF dietician will discuss this with you. If you do not take your enzymes, you will probably have tummy pain, pale, runny, smelly and fatty poo and problems with your weight. Common names of enzymes you may have had are: „„ Pancreatin (Creon® or Creon Micro®)

Anti-reflux medication Reflux occurs when stomach acid flows back up the food pipe (oesophagus) causing heart burn or indigestion. Anti-reflux or acid suppression medication reduces the amount of acid your stomach produces, which in turn reduces the symptoms of reflux. Sometimes, we use these medications

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to help more of your enzyme replacement medicines reach the right part of your digestive system. Stomach acid can destroy the enzyme replacement you take, so reducing the amount of stomach acid with theses medicines lets more of it be absorbed. Ideally, you should take antireflux medication about half an hour to an hour before food. Common names of antireflux medications include: „„ lansoprazole (Zoton®) „„ omeprazole (Losec®) „„ ranitidine

Vitamin supplements Vitamin supplements are very important for you. We know that the absorption of fat from your food is reduced in CF. This means that the absorption of fat-soluble vitamins such as vitamins A, D, E and K which you would have normally get from your diet are reduced as well. Having low vitamin levels can put your health at risk. You should take your vitamin supplements with a meal along with your enzyme replacement to help absorb them into your system. Common names of vitamin supplements include: „„ Abidec® and Dalivit® „„ Multivitamin BPC „„ Alpha tocopheryl „„ Vitamin E „„ Calcichew D3® and Calcichew D3 Forte® „„ Menadiol sodium phosphate „„ Phytomenadione

Bile acid supplements Bile is made in the liver and flows through a tube (the bile duct) to the intestines, where it helps to break down fat in food. In CF, the bile duct sometimes becomes blocked so the bile cannot reach the food very well. The build-up of bile can also damage the liver. If you have signs that this may be happening or if you have CF-related liver disease, you will be prescribed a medicine called ursodeoxycholic acid which will improve the flow of bile. Common names for ursodeoxycholic acid include: „„ Ursofalk® „„ Destolit® „„ Ursogal® © GOSH NHS Foundation Trust May 2015

Inhalers We sometimes use inhalers to deliver certain medications directly into your lungs where they need to work. There are many different types of Inhalers (Easi-Breathe®, Accuhaler®, Turbohaler® and Twisthaler® amongst others). Generally the medicines we give via inhalers can be divided two types Relievers - The reliever inhaler is usually blue in colour. The medications in these inhalers work quickly and for a short period of time and are often used to relieve breathlessness or to prevent you from getting breathless from doing physiotherapy or your nebuliser treatment. You should use the reliever inhaler twice a day before physiotherapy/nebulised treatments but you can use it more often if needed. Common names for reliever inhalers include „„ Salbutamol (Ventolin®) (Salamol®) – blue „„ Terbutaline (Bricanyl®) Steroid inhalers – These are used to help reduce the inflammation in the lungs. The inhalers are usually brown, purple or orange in colour and you should use it twice a day. A common side effect associated with steroid inhalers is that they can cause a fungal infection in your mouth. An easy way to prevent this is to rinse your mouth and/ or gargle with some water every time you use the inhaler. This also applies to using combination inhalers as well. Common steroid inhalers are „„ Beclometasone (Clenil Modulite®) - Brown „„ FlutIcasone (Flixotide®) - Orange „„ Some inhalers we use combine steroid with a long acting reliever medication. The common ones we use are • Seretide® (Purple) • Symbicort® (white) Your pharmacist, doctor, nurse or physiotherapist will show you how to use the inhaler. You could also watch our video on using an inhaler available online at medicines-information/how-to-help-yourchild-use-an-inhaler/video-how-to-helpyour-child-use-an-inhaler or on the GOSH YouTube™ channel. You may also find using a spacer (Aerochamber®) or a volumatic device may help improve your technique when using inhalers. These are plastic chambers where the inhaler fits in one Sheet 3 of 5

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end and you form a tight seal, with your lips around the mouth piece or use a mask around your mouth and nose, at the other end. The medication is deposited into the chamber by depressing the canister in the inhaler. Please note: some types of inhalers do not fit a spacer device or volumatic.

Subcutaneous insulin injections Sometimes people with CF develop a type of diabetes and we use insulin to help control your blood glucose levels if your body is not able to do so itself. Insulin is a hormone that your pancreas produces in response to eating food. It allows your body either to use sugar (glucose), from carbohydrates in the food that you eat, for energy or to store glucose for future use. Insulin helps keeps your blood glucose level from getting too high or too low. It is given via a subcutaneous injection, which is an injection just under the skin. Insulin comes as a prefilled disposable pen (FlexPen®) or penfill cartridge, where the cartridge can be replaced when finished. Unused Insulin pens and cartridges should be stored in the fridge. You should store insulin pens and cartridges in the fridge until you start to use them. Once you start an insulin pen do not store it in the fridge and throw it away within 30 days. Write the date you started using the pen on the label to remind you. There are three types of insulin – each works at a different speed and lasts a different time. The diabetes team will tell you which one(s) to use, how much and how often. Rapid Insulin (acts within minutes-hours) „„ Insulin aspart (Novorapid®) Intermediate acting insulin (acts over 8 to 10 hours) „„ Isophane Insulin (Insulatard®)(Humulin I®) Long acting insulin (acts over 12 to 24 hours) „„ Insulin detemir (Levemir®) „„ Insulin glargine (Lantus®)

© GOSH NHS Foundation Trust May 2015

Taking your medicines „„ The best way to remember to take you medication is make them a routine part of your daily life like brushing your teeth, having a shower, watching a specific TV program every evening. „„ For those medicines that can be taken with food or are not affected by food, plan on taking them around/with your meal „„ Take your morning medication as soon as you wake up. Keep a glass of water on your bedside table. „„ There are many other ways to help you remember to take your medication • Get up early enough in the morning to give yourself time and privacy to take your treatments so that your routine has minimum impact on your daily life outside of your home. • Setting multiple alarms on your phone to remind you to take your medication • Using apps on your phone that send you alerts reminding you to take your medication • Using appliances such as dosette or pill boxes. This lets you see if you have taken your medication. As you have to take the medication out of their packaging and put them in the individual chambers, this is a very good way of learning the characteristics of the tablet/capsules such as shape, size and colour to help you identify each one. These dosette or pill boxes are widely available in many pharmacies

Will the medicines have any side effects? You have to take medication to make you feel better but unfortunately they do sometimes have side effects. All medications should come with a patient information leaflet inside the package which will outline all side effects ever seen for the medication. Most side effects are identified when the medicine is being tested, and all of these are included in the patient information leaflet. This can make for worrying reading, but remember that some of the reported side effects are extremely rare. You are unlikely to experience any of them but it is important to be aware of them. If you do experience any of the side effects or have any questions or concerns, please speak to the CF team or your family doctor (GP).

Storing your medicines „„ Keep the medicines in a cupboard (preferably one with a lock) away from heat and direct sunlight if they do not need to be refrigerated. „„ Do not put the medicines in the fridge or freezer unless it specifically says to do so on the packaging or the label put on by the pharmacy „„ Make sure that other young children cannot see or reach the medicine. „„ Keep the medicine in its original container. If you are using a dosette or pill box, only put in one week’s worth of medicines at a time.

• Friends and family can be a great help in helping to remind you about taking your medication. • Keep a diary or planner and tick off each dose as you have taken it.

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© GOSH NHS Foundation Trust May 2015

General advice about medicines „„ Some medicines should be taken with food or milk. Other medicines work best on an empty stomach. There are a few medicines that should not be taken with certain foods, juices or milk. This should be shown on the label put on by the pharmacy on the box or package. If you are not sure which food and drink you should have with which medicine, speak with your pharmacist. „„ If you are not sure that the medicine is working, speak to someone in the CF team. Do not take more than you have been instructed to take, as this may do you harm rather than good.

„„ Make sure you always have enough medicines at home. Do not wait for them to run out before you order more. Make sure you order your medication at least two weeks before they are about to run out. You may be able to do this online if your GP offers this service – set a diary reminder on your phone if that helps. „„ Make sure that medicine you have at home has not reached the ‘best before’ or ‘use by’ date on the packaging. If any medicines have passed this date, take them to your pharmacist for disposal – do not flush them down the toilet or throw them away in your household rubbish. „„ If you are thinking of taking any other medicines or herbal supplements that are not prescribed by us, please check with us or your GP before you start them as we want to make sure that they are safe for you to take and do to react/interfere with your other medicines.

Remember that we are here to help you if you have any questions about your medicines so please call us on 020 7405 9200 Ext. 1119 to speak to one of us in the CF team.

Compiled by the Cystic Fibrosis Unit in collaboration with the Child and Family Information Group Great Ormond Street Hospital for Children NHS Foundation Trust, Great Ormond Street, London WC1N 3JH Sheet 5 of 5

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© GOSH NHS Foundation Trust May 2015