Managing medication

The most common preventative antibiotic for CGD is co-trimoxazole (Septrin), which stops serious bacterial infections.

It can be taken in tablet or liquid form. Co-trimoxazole tablets are round and white. Most people take 480mg tablets, but 960mg tablets are available. Liquid medicine is available for adults at a strength of 480mg of co-trimoxazole per 5ml and for children in 240mg per 5ml.

This should be taken EVERY day.

• Take it with/after food and with plenty of water. But don’t worry if you or your child can’t always manage this. Just make sure you or they drink lots during the day to help the kidneys flush the drug out.

• Check you’re not taking too many antibiotics. If you or your child are taking antibiotics like ciprofloxacin or flucloxacillin, it may not be necessary to take co-trimoxazole too. However, you should re-start co-trimoxazole as soon as the  course of antibiotics is finished. Always check with your doctor or nurse before stopping any medication.

• Take antibiotics when it’s convenient. You can take them once a day or divide them into morning and evening doses – whichever is more convenient. For instance, if you are prescribed one tablet twice a day and you take 480mg tablets, you are taking 960mg each day. You can either take the tablets twice a day, or both at the same time once a day.

• Nausea, vomiting, skin rashes and headaches. These are the most common side effects.

• Bone marrow problems. Co-trimoxazole can make some patients’ bone marrow work less effectively. This can lead to anaemia and bruising or bleeding more easily. Tell your doctor if you or your child feels tired than usual or there’s bruising or bleeding. They’ll be able to check for bone marrow problems with a simple blood test.

• Sensitivity to sunlight. Taking co-trimoxazole can make people more sensitive to the sun. Anyone taking it should use SPF25 or higher sun protection cream when they’re in the sun.

Itraconazole (Sporonox) is the most common antifungal medication prescribed for people with CGD.

It comes in capsules or as a liquid. Capsules contain 100mg of itraconazole and the liquid has 100mg in every 10ml.

Sometimes people with CGD struggle to absorb itraconazole through their gut, especially if it’s in capsule form. So they may need to take a higher dose. If a doctor is concerned about this, they may do a blood test to check the level of itraconazole in the person’s system.

It’s easier to absorb liquid itraconazole, but this is more likely to cause diarrhoea and/or abdominal pain in some people. It also tastes very bitter.

• Take capsules with food. This can help them to be absorbed. Taking itraconazole with an acidic drink, such as fruit juice, squash or a fizzy drink can also help. The capsules shouldn’t be split and taken as granules as this prevents the drug from being absorbed properly.

• Take liquid on an empty stomach. Ideally, itraconazole liquid should be taken an hour before or after food. Just before bed can be a good time. It’s best to take it with water or weak squash (not milk, as this counts as food).

Getting children to take bitter-tasting itraconazole can be difficult but if they are prescribed it, they need to. Make any necessary adjustments to our advice to make this happen.

 

• Stomach problems. These can include nausea, indigestion, diarrhoea, abdominal pain and constipation.

• Headaches, dizziness and menstrual problems like bleeding between periods. These are less common side effects.

• Lower liver function. Itraconazole, and occasionally co-trimoxazole, can cause certain liver enzymes to increase. This is rare and reversible. It’s easy for a doctor to monitor this with a simple blood test called a liver function test. Doctors generally do routine liver function tests every six to 12 months for people with CGD. However, this will vary from person to person.

• Diarrhoea. Co-trimoxazole and itraconazole can cause diarrhoea. Itraconazole – especially the liquid form – is the most likely culprit. Tell your doctor if you/your child has diarrhoea that doesn’t go away.

• Allergic reactions. Although it’s rare, some people with CGD do have allergic reactions to their medication, ranging from mild to severe. Signs of a mild reaction can include skin rashes, itching, a high temperature, shivering, a flushed face, dizziness and a headache. If you experience any of these symptoms, report them to your doctor or nurse.

Signs of a severe allergic reaction include chest pain and difficulty breathing. Call for an ambulance straight away if you or your child with CGD has either.

It may be tempting not to take your medicines, especially if you’re feeling OK.  This can be particularly true for young people, who sometimes think that stopping their medication means they’ll be able to forget all about having CGD.

But remember: the times you feel especially well, or the young person you care for feels well, are when medicines are working effectively. Stop taking them and you open yourself to infections.

Despite taking preventative medicines, people with CGD will sometimes get ill with infections.

When that happens, it’s important to get treated quickly with antibiotics or antifungal medicine. You or your child will often have these through an intravenous drip to make sure they work quickly and effectively.

Other treatments for infections include interferon gamma, white cell transfusions and bone marrow transplants.

Women with CGD can become pregnant and have babies without experiencing any adverse health effects. However, it’s best for a woman planning a pregnancy to discuss her plans with her doctor before becoming pregnant. She might need to change to medication that is safe to use during pregnancy and doesn’t harm the baby.

It is important to talk to your medical team – don’t just come off your drugs without medical advice.

 

It’s important that people with CGD keep their vaccinations up-to-date. These include:

• Childhood vaccines. Children with CGD should be fully immunised. The only immunisation adults and children with CGD shouldn’t have is the BCG, which protects against tuberculosis. All others, including for polio, tetanus, measles, mumps and rubella (MMR) and meningitis C, are okay. Children will get their vaccines at school and adults can go to their GP practice nurse. If your child is offered the nasal vaccine please check with your immunology team that this is the correct vaccine for your child.

• Flu vaccine. Flu is a virus, so people with CGD can fight it off as well as anybody else. However, people with CGD can have problems with chest infections associated with flu. So anyone with CGD over six months old should have the seasonal and swine flu vaccines each year.

All vaccines can cause adverse reactions and side effects. Your doctor or nurse will tell you about the possible side effects of each immunisation you have. Common side effects for all vaccines include a sore arm and redness or swelling at the injection site. Talk to your doctor or CGD nurse if you have these. Serious reactions are rare.

Some people with CGD may be interested in having complementary therapies like aromatherapy, reflexology, massage and homeopathy in addition to their conventional treatment.

Treatments which help you relax, such as hypnotherapy and massage, can help with day-to-day pain and bowel problems.

However, complementary therapies aren’t scientifically proven so you should only use them to support the treatment your doctor prescribes.

You shouldn’t have certain therapies if you’re on some medications. Always discuss this with your doctor before having a new therapy.

Remember that just because a medicine is ‘natural’ or ‘herbal’, it doesn’t mean it’s guaranteed to be safe. Always ask your doctor to check the contents of homeopathic/herbal treatments to make sure you can take them. Some won’t be suitable for people with CGD.

• Don’t stop taking your CGD preventative medications (itraconazole and co-trimoxazole).

• Don’t stop any other conventional treatments without consulting your doctor.

• Make sure the person doing your complementary therapy is properly qualified and registered with the appropriate regulatory body.

• Bear in mind that a therapy that works well for one person won’t necessarily suit someone else.