Medicines (including inhaled drugs)
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Information for children, teenagers and families

Medicines (including inhaled drugs)

All drugs can cause side effects - and this is usually quite a long list of possibilites. The ones mentioned here are common, but by no means exclusive.

Creon - see also Pancreatic enzyme replacement in the Nutrition section

What does it do?

Creon is taken to replace the enzymes that should be made by the pancreas. The most important enzyme is called lipase. The body must have lipase to help absorb the energy from fat. Fat provides most of the energy we get from food, so if can't absorb this energy we will lose weight. Sometimes not absorbing the fat will cause tummy ache and make poos smellier than usual! Creon micro has 5000 units of creon per scoop. Creon 10,000 has 10,000 units of lipase in each capsule. Creon 25,000 has 25,000 units of lipase in each capsule.

How often should it be taken?

Creon needs to be taken with all food containing fat. You will need to become experts on knowing how much fat is each type of food. The amount needed varies from child to child - discuss the dose with your CF dietician.

How long should it be taken for?

Most (90%) of children will need creon lifelong from birth. A smaller number of children may be 'pancreas sufficient' and not need creon - at least to start with. A proportion of these children may develop a need for creon as they get older. Once a need for creon is establised, it will be permanently.

Common side effects

None.

Vitamins

Examples: dalavit, vitamin E suspension or tablets, vitamin A&D capsules, Aquadek, menadiol (vitamin K).

What do they do?

Vitamins are vital for the body to work properly. They have to be absorbed from the diet - they cannot be made by the body. Children with CF have trouble absorbing the vitamins that need to be absorbed along with fat - these are vitamins A, D, E and K. Low vitamin A can cause night-blindness, low vitamin D is thought to have lots of effects, most notably problems with low bone mineral density, low vitamin E can cause anaemia and nerve problems. Vitamin K is important for blood clotting and bone health.

How often should they be taken?

All vitamins are taken once per day - with fat containing foods and creon to aid absorption.

How long should they be taken for?

Lifelong.

Common side effects

None.

Oral antibiotics

Examples: flucloxacillin, co-amoxyclav (augmentin), cefaclor, azithromycin, ciprofloxacin, doxycycline, cotrimoxazole (septrin)

What do they do?

Oral antibitoics are used to kill bacteria causing chest infections. They can be used for exacerbations and also as preventatives/prophylactics (usually flucloxacillin or azithromycin).

How often should they be taken?

It varies for different antibiotics and whether they are being used to treat a chest infection or as a preventative. Dosing can be only 3 times per week (for preventative azithromycin) to once daily (treatment azithromycin and doxyclycline) to twice daily (ciprofloxacin and cotrimoxazole and preventative flucloxacillin) to three times per day (for augmentin and treatment flucloxacillin). Flucloxacillin is sometimes prescribed as 4 times per day, but most people find that too difficult to fit in (particularly school-aged children)

How long should they be taken for?

Treatment courses are 2 to 4 weeks long. Preventative flucloxacillin is used twice daily for the first 3 years of life. Preventative azithromycin is used in some children with troublesome chest infections - as a long term treatment 3 times per week (Mondays, Wednesdays and Fridays).

Common side effects

Tummy upset. Ciprofloxacin and doxycyline can make the skin very sensitive to sunburn - children need to cover up and use lots of sun block in sunny weather.

Antifungals

itraconazole, voriconazole

What do they do?

Antifungals are used to kill fungi - most usually Aspergillus, and more rarely Scediosporum

How often should they be taken?

Itraconzole and voriconazole are taken twice per day.

How long should they be taken for?

A course of treatment is usually 4 to 12 weeks.

Common side effects

None.

Inhaled antibiotics

colomycin, promixin, colobreathe, tobramycin, tOBI, tobi podhaler, bramitob, aztreonam, cayston

What do they do?

Inhaled antibiotics are used to kill more troublesome bacteria causing airway infections - usually Pseduomonas. Nebulised colomycin, promixin and colobreathe are all forms of colistin. TOBI, bramitob and TOBI podhaler are all forms of tobramycin. Cayston is the trade name for aztreonam.

How often should they be taken?

Colistin and tobramycin are both taken twice daily. Aztreonam is taken 3 times per day. It takes about 10 minutes to nebulsed a dose of these drugs (less with an ineb or eflow). Dry powder forms (suitable for older children who need alternate month treatment) are quicker to use, but don't suit everyone. All inhaled antibiotics should be taken AFTER physiotherapy.

How long should they be taken for?

If they are being used for eradication of a new isolation of Pseudomonas, the usual treatment course is 3 months. If Pseudomonas is recurrent, then treatment can be extended to 12 months. If this keeps happening, treatment becomes permanent - either using colistin on its own, tobramycin on its own, or alternating one month of each. Aztreonam is reserved for particularly troublesome infections.

Common side effects

None. Occasionally inhaled antibiotics can cause wheeze - a test dose needs to be given when these drugs are started for the first time.

Pulmozyme (DNase)

What does it do?

The airway mucus in children with CF is packed with long sticky strands of DNA. This makes the mucus stick to the walls of the airway and harder to cough up. Mucus sticking to the airway is a great place for more bugs to grow in and cause more irritation. DNase is an enzyme that chops up the DNA into small bits, making it much less sticky and so easier cough out. We recommend all children over the age of 6 years are on DNase. Children who have had troublesome chest infetions may get started on DNase from an earlier age.

How often should it be taken?

DNase is taken once per day as a nebulised drug. It takes about 10 minutes to do (less with an ineb or eflow). There should be a gap of a minimum of 1 hour and ideally 2 hours before physiotherapy is carried. The gap can be longer than this - as long as 12 hours. This means that DNase can be taken after chest physiotherapy in the morning, or early afternoon (eg when children get home from school) leaving a 2 hour (or more) gap before evening physio.

How long should it be taken for?

Usually once DNase has been started, it is lifelong.

Common side effects

None.

Nebulised hypertonic saline

What does it do?

Hypertonic saline (usually 6 or 7%, occasionally 3% if higher strength is not tolerated) is an inhaled drug which draws water into the airway and makes it easier to cough out mucus. It is especially helpful in children with very sticky sputum. It can be used in addition to DNase.

How often should it be taken?

Hypertonic saline is usually taken immediately before and during chest physiotherapy.

How long should it be taken for?

Hypertonic saline can be used as a long term therapy or on an as required basis.

Common side effects

Cough and wheeze. Pre-treatment with salbutamol is recommended - your physiotherapist will show you how to do this. A test dose is required when hypertonic saline is first used.

Prednisolone

What does it do?

Prednisolone is steroid. It can reduce the inflammation in the airways, and so can be helpful if chest exacerbations are taking a long time to settle despite intravenous antibiotics. It is also the mainstay of treatment for ABPA (see 'Problems that might happen' for more details on ABPA).

How often should it be taken?

Prednisolone is taken once per day. Make sure you have the soluble tablets. The 'enteric-coated' tablets are poorly absorbed in people with CF.

How long should it be taken for?

Courses can vary from 1 week to 3 months or more.

Common side effects

Weight gain, change in facial appearance (face gets rounder), sometime increased hair growth, stomach irritation (omperazole is recommended with courses of prednisolone for more that 1 week). Longer term use can cause immune suppression, adrenal insufficiency, growth failure, osteporosis and diabetes. So quite a lot a side effects, which is why we try and keep prednisolone use to a minimum.