Autosomal recessive polycystic kidney disease (ARPKD) in children and adolescents
This information is for parents of children and adolescents with ARPKD. It explains how ARPKD might affect children over the age of 2 years. If you have a younger child with ARPKD or have been told your baby might have ARPKD, see our information on ARPKD in babies.
Getting new information on ARPKD can cause worry, especially if your child is recently diagnosed or has new symptoms. Please call our helpline on 0300 111 1234 if you’d like to talk to someone about ARPKD.
Contents
Key Facts
- ARPKD is a rare disease that affects a child’s kidneys and liver.
- Signs of ARPKD might be spotted before or after your child is born.
- Doctors can diagnose ARPKD using an ultrasound scan of their kidneys and liver.
- Your child’s treatment team will include multiple specialists and your GP.
- If doctors aren’t sure whether your child has ARPKD or a different condition, they might suggest a blood test to check for the genes that cause ARPKD.
- Most often, ARPKD is caused by an altered gene called PKHD1. Less often it’s caused by genes called DZIP1L or CYS1.
- The symptoms of ARPKD vary greatly from child to child. Some have few or only minor symptoms, while others can be very poorly.
- Effects of ARPKD can include kidney damage, liver damage, blood problems, nutrition and growth problems, a large tummy, and psychological effects.
- Treatments are available to help.
- As your child approaches adulthood, they’ll need to move from children’s to adults’ health services. Their treatment team will help to make this smooth.
- Towards the end of this web page, we give tips on choosing where your child is treated, travel insurance, getting practical and financial help, and coping.
What is ARPKD?
Autosomal recessive polycystic kidney disease (ARPKD) is a rare disease. It affects about 1 in every 20,000 babies born. It affects a child’s kidneys and liver, which can cause health problems.
Signs of ARPKD are sometimes spotted when a baby is growing in the womb. Other times, it’s not diagnosed until after birth or during childhood or adulthood.
ARPKD affects people in different ways:
- Babies diagnosed before or at birth tend to have more severe ARPKD. It often affects their lungs and kidneys.
- Some children have milder ARPKD and don’t get many symptoms until they’re older.
- People whose symptoms begin in adolescence or adulthood tend to have greater liver problems than kidney problems.
ARPKD can’t be cured but there are many treatments to help with symptoms. A range of support will be available to you and your child throughout their life.
Finding out your child has ARPKD
Some parents find out their child has ARPKD before or soon after birth. These babies often have kidney and lung problems.
ARPKD might also be diagnosed during infancy based on a genetic test, even if a baby has no symptoms.
Less commonly, parents don’t find out their child has ARPKD until later in childhood when symptoms start.
The first signs might be:
- weeing much more than usual
- bedwetting at night
- poor weight gain or growth problems
- unusual findings on a blood or urine test
- high blood pressure
- infections of the urinary tract or bile duct
- liver problems
- unusual findings on a kidney or liver scan
If your doctor thinks your child might have ARPKD, they’ll refer your child to hospital for tests. Doctors can usually confirm whether or not a child has ARPKD using an ultrasound scan of their kidneys and liver. An ultrasound uses sound waves to see inside their body.
Some other diseases cause symptoms similar to ARPKD. If doctors aren’t sure whether your child has ARPKD or a different condition, they might offer a different type of scan called magnetic resonance imaging (MRI). Alternatively, they might offer a genetic test to look for genes that cause ARPKD. If you agree, they’ll take a blood sample from your child for this test. We explain the genetics of ARPKD later.
Finding out that your child has ARPKD can be a shock. You may have many questions about ARPKD after the appointment. Ask if you can have another appointment with the doctor to discuss these.
There is a lot of useful information on this web page. You might also find it useful to call our helpline (0300 111 1234) and connect with other parents on our ARPKD Support UK Facebook group.
Your child’s treatment team
As ARPKD can affect different parts of the body, your child’s treatment team will include multiple people. Some of the specialists who might be involved are shown in the diagram below. Other people might be involved too, depending on your child’s needs.
How will ARPKD affect your child?
The symptoms of ARPKD can vary greatly from child to child, and even within the same family. Some children have few or only minor symptoms, while others can be very poorly.
Common problems in children with ARPKD are shown in the picture below.
Kidney problems
ARPKD can cause fluid-filled sacs (cysts) and scar tissue (fibrosis) to form in the kidneys. This can make the kidneys very large. Over time, damage to the kidneys can stop them working properly and cause problems throughout the body.
The severity of kidney problems varies from child to child. Some don’t have kidney problems at all, while others need dialysis or a kidney transplant.
Reduced kidney function
The main jobs (functions) of the kidneys are to:
- filter waste from the blood into urine
- balance the amount of water, salt and minerals in the blood
- tell the body when to make more red blood cells (which carry oxygen)
- help control blood pressure
- keep bones healthy
Many children with ARPKD (but not all) have reduced kidney function. This means their kidneys don’t work as well as they should. Some children are born with low kidney function. In others, kidney function begins to fall after a few years.
In most children with ARPKD, kidney function falls throughout childhood and their kidneys may eventually fail. If your child’s kidneys fail, they’ll need ‘kidney replacement therapy’ to survive.
Kidney replacement therapies work well.
The three options are:
- a kidney transplant
- peritoneal dialysis, which uses fluid placed in your child’s abdomen to clean their blood
- haemodialysis, which uses a machine to filter your child’s blood
You can learn about kidney transplants and dialysis on the NHS website.
By age 10, about 3 in every 10 children with ARPKD reach kidney failure and so need dialysis or a kidney transplant. By age 20, 6 in every 10 people with ARPKD reach kidney failure.
Doctors can usually predict when your child’s kidneys will fail about a year or two before this happens. This means you’ll have time to discuss options for their treatment and prepare. Each option has some risks and benefits — ask your kidney specialist to explain these. Kidney transplant is the best option for most children as donated kidneys usually work very well.
Assessments and treatments
Your child’s kidney specialist will use blood and urine tests to check how well your child’s kidneys are working. The frequency of these checks will depend on your child’s kidney function. Your child will have checkups at least 1 to 3 times a year.
Low kidney function can cause a few problems, such as high blood pressure and low levels of sodium in the blood. Your kidney specialist will check for these and give your child any treatments they need.
If your child is reaching kidney failure, your kidney specialist will explain the options of dialysis and transplant to you. If you decide to opt for a kidney transplant, the first step is to see if you (their mum or dad) or another willing relative is a suitable donor. If not, your child’s transplant team will put them on the waiting list for a kidney. They’ll have dialysis (if needed) while waiting for a transplant.
Enlarged kidneys
The kidneys of children with ARPKD can grow very large due to their kidney cysts. This may cause your child to have a large tummy. This can be most noticeable in young children.
Children don’t usually find large kidneys painful but they may feel full early on in a meal and may have a smaller appetite. This can cause nutrition and growth problems (see below). They may get out of breath more easily as there is less room for their lungs.
Assessments and treatments
If your child has large kidneys, your kidney specialist might suggest your child avoids high-risk and contact sports. This is to reduce the risk of their kidneys being injured. This is a choice for you and your child to make together.
If your child’s kidneys are causing a lot of symptoms, your kidney specialist might recommend one or both kidneys are removed. This surgery is called nephrectomy. If your child has both kidneys removed, they’ll need dialysis followed by a kidney transplant.
Urinary tract infections
The urinary tract includes the kidneys, the tubes to the bladder (ureters), bladder, and the tube you wee through (urethra). Up to half of children with ARPKD get urinary tract infections from time to time. These are more common in girls than boys. Sometimes, an infection can come back after treatment.
If your child develops a urinary tract infection, they’ll need a course of antibiotics.
Signs to watch out for are:
- a high temperature (above 38°C)
- needing to wee more often than usual
- stinging when they wee
- pain in their side
If your child has any of these signs, see a doctor promptly.
Assessments and treatments
If your child has signs of a urinary tract infection, your doctor will ask for a urine sample to do a simple test for an infection (called a dipstick test). They’ll also send urine off to the laboratory to check for bacteria. In the meantime, they’ll prescribe some antibiotics that are likely to work.
Once the test results come back, they’ll change your child’s prescription if needed to different antibiotics.
Weeing much more than usual
If ARPKD is affecting your child’s kidneys, they may produce more urine than usual. This means they’ll need to wee often and might wet the bed at night. Your child is also likely to get thirsty and dehydrated more easily than other children.
Things you can do to help include:
- Don’t show frustration about bedwetting in front of your child.
- Reassure your child if they get upset — bedwetting usually reduces over the years.
- Chat to your GP, school nurse and other parents for tips.
- Don’t reduce your child’s fluid intake to try to solve bedwetting — it’s important they stay hydrated.
- Encourage your child to drink when thirsty (however, if your child has low sodium levels, follow your doctor’s advice on limiting drinks).
- Check your child has plenty of drinks with them if they’re going out for the day.
- Explain to your child’s school that they need regular drinks and toilet breaks.
- Check the colour of your child’s wee. If it’s straw coloured or darker, they need to drink more.
Assessments and treatments
Your kidney specialist can explain the right amount for your child to drink each day. This might change over time depending on your child’s kidney health. They can also give you tips and information on bedwetting. School nurses and school health services may also be of help.
Blood problems
The kidneys help to keep the right amount of fluid, chemicals and blood cells in the blood. 8 They also help to control blood pressure. When the kidneys are damaged by ARPKD and their function falls, some problems with the blood can occur.
Low numbers of red blood cells
Red blood cells in the blood carry oxygen from the lungs to the whole body. The kidneys make a hormone (called erythropoietin) that tells the body when it needs to make more of these cells. ARPKD kidneys might not make enough of this hormone. This can lead to low levels of red blood cells, which is called anaemia.
Signs of anaemia include:
- tiredness and low energy
- getting out of breath easily
- pale skin
Assessments and treatments
Your child’s regular checkups will include a blood test for anaemia.
If your child gets anaemia, iron supplements can help. Another treatment for anaemia is an erythropoietin-stimulating agent. You might hear this called ‘EPO’. This medicine encourages your child’s body to make more red blood cells.
Too much acid in the blood
ARPKD can cause acid made by the body to build up in the blood. This is called metabolic acidosis and can be found using a blood test. It happens because not enough acid is being removed by the kidneys.
Signs of metabolic acidosis include:
- being out of breath
- being weak and tired
- feeling or being sick
- not feeling hungry
This build-up of acid is not good for your child’s kidneys, bones or muscles so treatment is important.
Assessments and treatments
Your kidney specialist can check for metabolic acidosis using a blood test.
If your child has metabolic acidosis, their kidney specialist (or a dietitian) might recommend changes to your child’s diet. Eating more fruits and vegetables can help greatly, as can other diet changes.
If this doesn’t help enough, your kidney specialist might recommend your child takes sodium bicarbonate or another medicine. These can help to balance the acid level of your child’s blood.
Metabolic acidosis is often a sign of falling kidney function. So, your kidney specialist might start discussing dialysis or a transplant with you.
High blood pressure
About three quarters of children with ARPKD have high blood pressure (hypertension). It’s important this is treated because it can put stress on your child’s heart. It can also increase the risk of cardiovascular problems later in life (including stroke and heart failure).
Controlling blood pressure carefully might also help to delay damage to the kidneys. This has been shown in some kidney diseases but not yet in children with ARPKD.
Assessments and treatments
Your child will have regular blood pressure checks. You might also be given a blood pressure monitor to do checks at home.
If your child’s blood pressure is high, medicines can help. Examples are angiotensin-converting enzyme (ACE) inhibitors and angiotensin II receptor blockers (ARBs). High blood pressure caused by ARPKD can be hard to control, so your child might need more than one medicine.
Low sodium levels
About 1 or 2 in every 10 children with ARPKD have low levels of sodium in their blood. The medical term for this is hyponatraemia. It occurs due to changes in the way the kidneys filter the blood and make urine.
Assessments and treatments
If your child has low sodium, it can help to reduce the amount of fluid they drink and change the foods they eat. Your kidney specialist or a dietitian will explain what to do.
Don’t change the amount your child is drinking or their diet unless you’re told to by a specialist who understands ARPKD.
Liver problems
Up to half of all children with ARPKD have some symptoms related to their liver. Liver problems can start any time from birth to adulthood.
The liver has many roles, including:
- making bile for digestion
- turning digested food into energy
- controlling levels of fat, acids and sugar in the blood
- storing iron and vitamins
ARPKD causes scarring to the liver, which is called congenital hepatic fibrosis (CHF). It can also cause liver cysts. These changes don’t usually stop the liver tissue from doing its jobs. However, they can cause other problems, which we explain next.
Enlarged liver
Liver cysts and scar tissue caused by ARPKD can make the liver much larger than normal. This can cause your child to have a large tummy. This can be most noticeable in young children.
Their liver might push on other organs. This may lead your child to:
- have discomfort or back pain
- feel full early in a meal and have less appetite
- have acid reflux (heart burn), where stomach acid leaks into their oesophagus (gullet)
- get out of breath more easily, as there is less room for their lungs
Assessments and treatments
Each year, your liver specialist will do a physical exam to feel the size of your child’s liver. They’ll also do blood tests to check liver function.
By the age of 5, your child should have an ultrasound scan to check for liver problems. Depending on what this shows, your child will have follow-up scans every 1 to 3 years.
It’s uncommon for liver function to become low due to ARPKD. If this happens, your liver specialist might recommend a liver transplant. You can learn about liver transplants on the NHS website.
If your child is not getting enough nutrition, help is on hand. We explain this later.
Widened bile duct
Bile is a fluid that helps digestion. It’s made in the liver and travels through the bile duct to the gut. ARPKD affects the way the bile duct forms in babies, making it wider than usual. This is a lifelong problem that is sometimes called Caroli disease. It can be spotted on a liver scan.
A widened bile duct can reduce the flow of bile to the gut. If this happens, your child might not absorb vitamins from their food well.
A wide bile duct is more likely to get infected with bacteria. This is called cholangitis.
Signs of cholangitis are:
- a high temperature (over 38°C)
- pain in the top right of your child’s tummy (abdomen)
It’s important to see your doctor promptly if your child has these signs, as there is a risk of the infection spreading to the bloodstream. This can cause sepsis, which is dangerous.
Assessments and treatments
If your child has a widened bile duct, their liver specialist will do a liver scan each year to check it. They’ll also do blood tests to check whether your child is getting enough vitamins. If not, vitamin supplements and bile acid supplements can help. Your child should take bile acid supplements when they eat fatty meals. This helps them digest food better and absorb more vitamins.
If your child has signs of cholangitis, their doctor will do a blood test. If the test confirms cholangitis, your child will need antibiotics to treat the infection.
High pressure in the liver vein
High blood pressure in the vein going into the liver is called portal hypertension. It can happen when the liver has a lot of scarring (fibrosis). This scar tissue stops the blood getting into the liver as easily as usual, so the pressure mounts up.
About 4 in every 10 people with ARPKD get portal hypertension. This might start in childhood or adulthood.
Portal hypertension can lead to higher blood pressure in other veins too, causing problems.
These include:
- An enlarged spleen. One of the jobs of the spleen is to make a type of blood cell called a platelet. Platelets cause our blood to clot if we get an injury. When the spleen is enlarged, it might not make enough platelets, which is called thrombocytopenia. This needs treatment otherwise your child is at risk of bleeding.
- Fluid in the abdomen (ascites). Ascites happens when too much fluid collects in the membrane bag that surrounds the organs in the abdomen. This can be uncomfortable for your child and give them a larger belly.
- Swollen blood vessels in the oesophagus (gullet) or gut. If your child gets enlarged blood vessels (varices) in their oesophagus or gut, there is a risk that these will bleed. This is rare but can be dangerous.
Assessments and treatments
All children with ARPKD should have a liver ultrasound scan by the age of 5 years to check for portal hypertension. Your child might have this scan when they’re younger if they have liver symptoms. If portal hypertension is found, your child will have yearly scans. If not, they’ll have scans every 2 to 3 years.
If your child has portal hypertension, they’ll have an endoscopy each year to check for swollen blood vessels in their digestive system. For this procedure, an endoscopist will put a tube containing a small camera down your child’s throat into their stomach. If they see swollen blood vessels, these can be treated with medicines or a small procedure during the endoscopy. You can read about endoscopy on the NHS website.
Your child may have their bottom (rectum) checked for enlarged blood vessels too. This procedure is called proctoscopy. The endoscopist uses a tool with a light in it to do this exam.
If your child has portal hypertension that is causing serious problems, their liver specialist might recommend a liver transplant or other surgery. By age 10, 1 in 10 children with ARPKD need a liver transplant. Sometimes, this is done at the same time as a kidney transplant.
As liver problems tend to worsen gradually, you’ll have time to plan for the liver transplant. With your agreement, your child will be put on the waiting list for a donated liver. Find out about liver transplants on the NHS website.
Liver and spleen problems can also increase the risk of certain infections. Your doctor might recommend that your child has extra vaccinations to protect them from these.
Nutrition and growth problems
About a third of children with ARPKD are short and light for their age. One of the reasons for this is that some children with ARPKD find it hard to eat as much as other children. This can happen if your child has enlarged kidneys or liver. These can push on their stomach, stopping them feeling as hungry and making them feel full quickly.
Enlarged kidneys and liver can also push on the gut, slowing digestion. If your child has a wide bile duct, this can reduce the amount of bile entering the gut, which also affects digestion. These problems can stop your child getting the right amount of nutrients they need to grow. Help is on hand.
Assessments and treatments
Your doctor can check whether your child is getting enough vitamins using a blood test. If any vitamin levels are low, vitamin supplements can help.
Your doctor will measure your child’s weight and height in checkups. If your child is a low weight or height for their age, your doctor might recommend supplemental foods. These are special drinks or pastes containing a lot of calories and nutrients. They are often taken by mouth.
If your child finds it hard to drink or eat these supplements, they might need a feeding tube for a while. This could be a temporary tube put through their nose into their stomach (a nasogastric tube). However, if your child needs extra feeds for more than a few weeks, their doctor might recommend a gastrostomy (or ‘PEG’).
A gastrostomy is a hole made by a surgeon through their skin to their stomach. The surgeon will put a tube through the hole, through which your child can be given supplemental feeds. Your child will have the gastrostomy for as long as they need extra feeds, which could be weeks to years.
A gastrostomy has benefits and risks — ask your doctor to talk you through these. You can learn more on the Great Ormond Street Hospital website.
If eating problems are caused by enlarged kidneys, your kidney specialist might suggest removing one or even both kidneys. If both are removed, your child will need dialysis followed by a kidney transplant.
If your child is not growing as much as usual for their age group, their doctor might suggest growth hormones. These usually work well to boost growth.
Challenges with emotions, behaviour and attention
ARPKD might affect some children’s mental function. Your child’s mental function includes the way they think, learn and socialize. Mental function affects behaviour and attention too. There has not been much research on this topic, so there are few clear facts.
If your child’s mental function is affected, they might find some aspects of school and other areas of life more challenging. For example, they might find it harder to focus their attention or to control their behaviour or emotions at times.
These issues might be more common in children with:
- poor kidney function
- high blood pressure
- poor lung function at birth
The reasons for this are not fully understood but could relate to the way the brain develops (matures) in early childhood.
Assessments and treatments
Once or twice a year, your child’s treatment team should check how well your child is doing socially and behaviourally. For example, they might ask how your child is getting on in school, at home and at clubs. They should also ask about your child’s and family’s quality of life.
If helpful, your doctor can refer your child to a specialist in mental health (psychologist) or the brain and behaviour (neuropsychologist). These specialists can do a more detailed assessment and provide advice and treatments. Talking to your child’s school can also be helpful.
For more information and advice on children’s moods and behaviour, see the Young Minds website.
Why some children have ARPKD
You did not do anything wrong to cause your child to have ARPKD.
ARPKD is caused by a gene alteration. The condition occurs when a child has two copies of a gene that causes ARPKD. Most often the altered gene is PKHD1. Less often children have an altered gene called DZIP1L or CYS1. In most cases, children inherit these altered genes from both parents. Occasionally only one parent has an altered gene, and the second alteration happens by chance.
As PKHD1 is the more common gene linked to ARPKD, we use it to explain ARPKD inheritance below.
PKHD1 gene inheritance
We all have 2 copies of the PKHD1 gene. This gene is for a large protein called fibrocystin that is found on cells that line the kidney and liver ducts. The reason that altered PKHD1 genes cause ARPKD is not fully understood.
A child needs to inherit 2 altered (faulty) PKHD1 genes to get ARPKD:
- 2 altered PKHD1 genes = ARPKD
- 1 altered PKHD1 gene and one normal copy = healthy carrier
- 2 normal copies of the PKHD1 gene = healthy and not a carrier
This pattern of inheritance is called ‘autosomal recessive’. It’s shown in the figure below.
Carriers have no symptoms and usually don’t know they have the gene unless they or someone else in the family has had a baby with ARPKD before. About one in every 70 people in the UK is an ARPKD carrier.
When two carriers have a baby, the chance of the baby having ARPKD is 1 in 4 (25%). There is also a 2 in 4 (50%) chance of the baby being a carrier. This chance is the same for each child the couple has.
Tests for ARPKD in family members
If your child has been diagnosed with ARPKD, doctors may offer tests to:
- you as parents
- any other children you have together
This is to check whether anyone else in the family has ARPKD that needs monitoring and treatment.
Genetic testing
Genetic testing can check for altered genes that cause ARPKD. It can also show whether a person is a carrier. A carrier is a person who does not have ARPKD but can pass on a gene causing the condition to a child.
Knowing you’re a carrier can be useful for planning a family. When 2 carriers want to have a baby together, they might be able to have a special type of in vitro fertilization (IVF) where an embryo without ARPKD is selected. This is called preimplantation genetic testing.
Ultrasound scans
An alternative to having a genetic test for ARPKD is an ultrasound scan to check for signs of the condition in the kidneys and liver.
It’s your choice whether to have a genetic test, scan, or neither, for yourself and your children. Ask your doctor to explain the pros and cons.
For more information, see our web page Genetic counselling and testing in ARPKD.
What to expect as your child nears adulthood
There are a growing number of adults living with ARPKD across the world. The health problems they face depend on how ARPKD is affecting their kidneys and liver. Adults with ARPKD can live full and productive lives.
As your child approaches adulthood, they’ll need to move from children’s to adults’ health services. This usually happens by 18 years old in England, Wales and Northern Ireland and 16 years old in Scotland.
By this age, many children are ready to take more control of their health care, but the change can be unsettling. To help things go smoothly, your child’s treatment team will start chatting with them about the move when they’re about 12–14 years old. This gives plenty of time for your child to:
- learn about (and visit) adult services
- learn how to manage their own health
- plan for their future
- choose when and how they want the move to happen
- decide what wider support they need (for example with housing, education and employment)
They do this with help from you, their healthcare team and others.
After the move to adult services, your child will have extra support while they settle in.
For more information, see ‘Transition to adult services: a guide for parents’ by Together for Short Lives.
Practical Tips
Getting the best care for your child
If your doctor is referring your child to a specialist, you usually have a right to choose the hospital or service they’ll go to. You need to make this choice before the referral is made.
Things to research or ask your doctor are:
- What options are there for where your child could be referred?
- Which places have the most experience in treating children with ARPKD?
- How long will you need to wait for an appointment?
- How far is the hospital or service?
- How easy is it to get to?
Here are some tools to help:
If you want to swap to a different hospital or consultant after referral, the NHS might be able to help. However, this isn’t your legal right. Talk to your current specialist or doctor. Give your reasons for wanting to swap and ask what the process is.
Travel insurance
If you want to go abroad, it’s a good idea to get travel insurance for your family. This may cost you more as your child has a long-term condition. Give yourself plenty of time to look into your options.
For advice and a list of insurance companies used by other people with kidney disease, see Kidney Care UK.
Financial and practical help
If your child is disabled or needs extra care, you might be able to get financial support:
- Disability living allowance: Families of children under 16 who have difficulty walking or who need extra care may be able to claim a disability living allowance. See Gov.uk.
- Carers allowance: If your child is eligible for disability living allowance and you care for them for more than 35 hours a week, you may be able to claim a carer’s allowance. See Gov.uk.
- Other government benefits: Some families might be eligible for additional benefits or help. See the Well Child website.
- Grants from organizations:
- The Well Child website lists some organizations who offer grants (financial help) to families of children with serious conditions. These grants might be for special equipment, therapies and respite care, for example. Respite care is when other people provide some care for your child to give you a break.
- Kidney Care UK also provides grants for families of people on dialysis or who have had a transplant if they can’t pay their bills and other costs.
- For children of school age, the council may provide useful support through school health services and nurses. For example, they may provide support on bed wetting, emotional wellbeing, and behaviour. Contact your school or council to find out more.
Help coping
Finding out that your child has a long-term health condition can be a big shock. Over time, you’ll adapt to your child’s needs as a family and your confidence in managing their condition will grow. However, you might go through periods of stress or where relationships are strained. You might worry how siblings are coping too.
Parents of children with a long-term condition can sometimes feel guilty, exhausted, angry or sad. However, they can also feel deep love, pride and awe at the strength of their child.
Tips for coping include:
- Talk openly with family and friends about how you’re feeling.
- Accept help from friends and family.
- Set aside time to relax, sleep and do things you enjoy.
- Consider respite care if your child needs a lot of care — this allows you to recharge.
- Consider seeing a counsellor to help you identify your feelings and learn how to manage them in practical ways.
- Learn how to recognize stress and trauma, ways to help yourself recover, and when to get expert support (see the MIND and mobilise websites for useful articles).
- If anxiety, depression or other mental health issues are affecting your daily life, talk to your GP.
Our helpline is open for information and support (details below). We also have a Facebook support group for families affected by ARPKD. You may also find it helpful to seek support from organizations providing support to carers.
PKD Charity Helpline
Our confidential helpline is available 9.30am to 5.00pm, Monday to Friday except bank holidays:
Additional charities who provide useful support are listed at the end of this factsheet.
More from the PKD Charity
More information from others
- InfoKID provides information to parents and carers of babies and children with kidney conditions.
- Kidney Care UK provides information, support, and grants to kidney patients and their families.
- The Children’s Liver Disease Foundation provides information and support for families of children with liver diseases.
- Kidney Kids Scotland provides support for families of babies and children with kidney diseases in Scotland.
- Kidney Wales (Aren Cymru) provides support for people with kidney diseases in Wales.
- Children’s Kidney Fund N.I. helps families of children with kidney diseases in practical ways in Northern Ireland.
- Well Child gives seriously ill children the chance to thrive at home.
- The Rainbow Trust supports parents and siblings of children who are seriously ill.
- Together for Short Lives helps families caring for a seriously ill child make the most of every moment together.
- The Genetic Alliance UK provides information and support to people affected by a genetic disorder.
- MIND provides information and support to people with mental health problems.
- Carers UK offers information and support for people acting as carers in the UK, as well as ways to connect with others.
The NHS provides general health information, including on scans, dialysis, transplant, and urinary tract infections.
Disclaimer regarding imagery
All photography appearing on this web page is for illustrative purposes only and is freely available via Pexels. The medical history of persons shown is unknown.
Further information
All of our publications are based on references but these are removed for ease of reading on our webpages. A version of this webpage with references included is available upon request by emailing [email protected]
Authors and contributors
Written by Hannah Bridges, PhD, independent medical writer, HB Health Comms Ltd. Reviewed by Professor Manish Sinha, Consultant Paediatric Nephrologist, Guy’s and St Thomas’ NHS Foundation Trust.
With thanks to all those affected by ARPKD who contributed to this publication.
Ref No: ARPKD.CYP.V1.0
© PKD Charity 2025
First published: May 2025
Due to be medically reviewed: May 2028
Disclaimer: This information is primarily for people in the UK. We have made every effort to ensure that the information we provide is correct and up to date. However, it is not a substitute for professional medical advice or a medical examination. We do not promote or recommend any treatment. We do not accept liability for any errors or omissions. Medical information, the law and government regulations change rapidly, so always consult your GP, pharmacist or other medical professional if you have any concerns or before starting any new treatment.
If you don't have access to a printer and would like a printed version of this information sheet, or any other PKD Charity information, call the PKD Charity Helpline on 0300 111 1234 (weekdays, 9am–5pm) or email [email protected]
The PKD Charity Helpline offers confidential support and information to anyone affected by PKD, including family, friends, carers, newly diagnosed or those who have lived with the condition for many years.
