Frequently relapsing nephrotic syndrome: causes
Nephrotic syndrome happens in people of all ages. Steroid-sensitive nephrotic syndrome (SSNS) usually starts when a child is between 2 and 5 years old.
Many cases of nephrotic syndrome – including those that relapse often – are linked to the immune system, which normally protects the body against disease and infection.
Frequently relapsing nephrotic syndrome: tests
Your child will have had tests to diagnose nephrotic syndrome, including urine tests, blood tests and, sometimes, imaging tests (scans). Your child will not usually need further tests for relapses.
A kidney biopsy is not usually needed, but further into your child’s treatment your doctor may recommend one to find out how much damage there is in his or her kidneys. This is especially important as some medicines that are used to treat frequently relapsing nephrotic syndrome may harm the kidneys.
A tiny piece of a kidney is removed from the body with a needle and examined under microscopes. Special medicines are used so your child will not feel any pain or can sleep through the procedure.
Frequently relapsing nephrotic syndrome: treatment
It is important that you continue giving the medicines to your child as your doctor has told you, even if your child is getting better.
Referral to specialist services
Children with frequently relapsing nephrotic syndrome may be referred to a paediatric renal unit (a specialised unit that treats babies, children and young people with kidney conditions). They are looked after by a paediatric nephrologist, a doctor who treats children with kidney problems.
Hospital visits
A team of healthcare professionals may:
- check your child’s kidney function (how well his or her kidneys are working)
- measure how much your child is drinking and how much urine he or she is passing
- test your child’s urine with a dipstick
- check your child’s blood pressure.
Healthcare team
Your child’s healthcare team may include a:
- paediatrician – a doctor who treats babies, children and young people
- paediatric nephrologist – a doctor who treats babies, children and young people with kidney problems
- renal nurse – a nurse who cares for children with kidney problems
- paediatric renal dietician – a healthcare professional who advises what your child should eat and drink during different stages of a kidney condition
- renal social worker – a healthcare professional who supports you and your family, especially with any concerns about money, travel and housing related to looking after your child with kidney disease
- renal psychologist – a healthcare professional who supports your child and family, especially with emotional stresses and strains from having or look after a child with kidney disease
- play specialist – a professional who uses dolls and other toys to help your child prepare for procedures, such as blood tests and dialysis
- renal pharmacist – some hospitals have a pharmacist who specialises in medicines for children and/or adults with kidney problems.
Steroids
If your child has frequent relapses, your doctor may prescribe a small dose of the steroid prednisolone, to be taken once a day. Steroids suppress the immune system, or make it less active.
Prednisolone is available as tablets. Children who are unable to take medicines by mouth may be given a similar medicine called methylprednisolone, which is injected using a needle.
Immunosuppressants
If your child continues to have relapses, your doctor may prescribe other medicines known as immunosuppressants. There are different types of immunosuppressants, and your child may need to take more than one of these to try to treat the symptoms and prevent relapses.
Your doctor will work out the best medicines for your child. They may include:
- levamisole
- mycophenolate mofetil – also called MMF
- cyclophosphamide
- ciclosporin
- tacrolimus
Medicines that are more rarely used are:
- rituximab
- vincristine
- chlorambucil.
Side-effects of immunosuppressants
Immunosuppressants depress the immune system.
We use medicines to help children get better, but sometimes they have effects we do not want – side-effects.
Contact your doctor straight away if your child has any of the following while on steroid medicines:
- fever (temperature above 38°C), with a sore throat or a cough, for more than 12 hours
- a rash or severe bruising
- bad stomach pain or repeated vomiting – sometimes steroids can cause ulcers in the stomach.
Other side-effects
Other side-effects that affect some children are below – speak with your doctor or nurse if you are concerned or need more support:
- nausea, vomiting, stomach pain and/or indigestion (heartburn) due to irritation to the lining of the stomach – your doctor may give your child some medicine to treat this
- behavioural problems – you may find that your child is more likely to have tantrums, be moody, feel depressed, have difficulty sleeping or have nightmares
- weight gain and larger appetite – you can help by making sure your child has lots of physical activity and eats fruits, vegetables and low-calorie food, rather than food that is high in calories (such as cakes, biscuits, sweets and crisps), and by reducing the portion size
- high blood pressure (hypertension) – your child’s blood pressure will be regularly checked, and if it is too high, will need to be controlled by eating a diet with no-added salt.
There may, sometimes, be other side-effects that are not listed above. If you notice anything unusual and are concerned, contact your doctor.
Side effects with high doses or long courses
When steroids are needed over a long time, or in high doses, they may have other side-effects. If you have any concerns, talk to your doctor.
- All steroid medicines, including prednisolone, may affect the adrenal glands so that they produce less of a hormone called cortisol when the body is stressed (e.g. during illness or injury). This means that your child may have more difficulty fighting off an infection, or may recover less quickly from injury or after surgery. If your child is unwell and you are worried about an infection, contact your doctor straight away.
- Steroids can slow growth and affect puberty. They can also cause growth of body hair and irregular periods in girls. Your doctor will check your child’s growth and development.
- Your child’s skin may become thinner, and heal more slowly than usual. Acne (spots) may become worse, or your child may develop mouth ulcers or thrush (candidiasis).
- Your child may develop problems with their hip bones, or their bones may become weaker (osteoporosis). The muscles around their hips and shoulders may also become weaker. If your child has any difficulty walking or moving around, contact your doctor.
- Occasionally, steroids cause diabetes. If your child seems more thirsty than normal, needs to pass urine (wee) often, or starts wetting the bed at night, contact your doctor.
Risk of infection
Because these medicines work on the immune system, your child is more likely to get infections, or have more difficulty fighting off an infection. He or she may also recover less quickly from injury or after surgery.
Contact your doctor straight away if your child:
- has been in contact with somebody with measles or chicken pox (and has not had chicken pox before)
- are unwell and you are worried about an infection.
Risk for the kidney
Although we use these medicines to try to treat nephrotic syndrome, some, especially tacrolimus and ciclosporin, may be harmful to the kidney. Your child will have regular blood tests to check the levels of these medicines to try to avoid this. If they are on these medicines for a long time (more than 2 years), your doctor may do a kidney biopsy to check for kidney damage.
Vaccines
It is important that your child has the vaccines (immunisations) that he or she needs to reduce the risk of some diseases. Speak with your doctor about these – some vaccines are not safe to give while a child is taking steroids or immunosuppressants.
It is important that your child has the flu vaccine each year.
Other treatments
Some children need to take antibiotic medicines to help prevent or treat infections, or diuretic medicines to reduce swelling in their body. Most children need to eat a no-added salt diet to control their blood pressure.
Frequently relapsing nephrotic syndrome: about the future
Most children with frequently relapsing nephrotic syndrome, especially if it can be treated with a low dose of steroids, will have no long-term problems with their kidneys.
Children can usually do the things that other children their age do, including during relapses. After a hospital stay, they should be able to continue going to school or nursery. They can play with other children and stay active. If your child is in contact with measles or chicken pox, tell your hospital straight away.
Future relapses
In most children, there are fewer or no relapses as they get older. Over 8 in 10 children with nephrotic syndrome will not have relapses 10 years after the first episode. Some children will continue to have relapses into their adulthood.
Long-term problems
If your child needs to take immunosuppressants, he or she will have regular check ups and blood tests to monitor for side effects. With some immunosuppressants there may be a risk to kidney function or the blood count and your child will need regular blood tests to check for any abnormalities. Depending on the type of immunosuppressant, the drug levels may also be monitored. Occasionally there may be a risk of hypertension (high blood pressure) and your child’s blood pressure will need to be monitored.
Some children develop chronic kidney disease (CKD) – the kidneys stop working as well as they should – this happens slowly, often over many years. These children may need further care. They will need to go back to the hospital or clinic for regular follow-up appointments.
If your child has CKD, you will learn more over time about how to help manage the condition and what to expect.
Getting support
This can be a difficult and stressful experience for your child and the whole family, including other children.
If you have any concerns or need additional support, speak with your doctor or nurse.