Children and PH

Unfortunately, there is no minimum age for PH sufferers. Not only is it seen in adults of all ages, sizes and backgrounds but it can be found in the newborn, young children, teenagers and in early adulthood as well. In children however, the prognosis, treatments, investigations and overall needs can differ greatly from adults.

However, perhaps one of the most important things to bear in mind in children with PH is the need for the parents to be the child’s advocate, spokesperson and carer.

The symptoms of PH for children are similar to that of an adult, though children are more likely to experience tiredness, dizziness and breathlessness and for many fainting is common (see Summary). They can fail to put on weight like a normal child.  Children may also experience other unique problems such as slowed growth.

Tests carried out look for long standing blood clots in the lungs (which can often be seen in adults with PH) are rarely required in childhood. Children tend to be diagnosed earlier than adults, but just like adults they are often misdiagnosed several times before a correct diagnosis is made. The commonest misdiagnosis is asthma.

Severe, sustained PH is potentially fatal in any patient. Untreated PH in children worsens more quickly than the same condition in adults. Children also change more quickly than adults and the doctors have to watch them more closely and be ready to change the treatment if the child is not doing well. However, with treatment, children appear to have an overall better prognosis than adults. In the past decade or so, we have seen that a more positive and aggressive approach to the overall management of pulmonary hypertension in children has evolved. Recent advances in genetics and newer understandings in cell biology are providing fresh insights into the nature of this disease and new therapies offer an improved quality of life and increased survival in both adults and children.

Children are responsive to the same treatments as adults. Treatment for PH is lifelong. The treatment used has to be individualised and adjusted according to how the child is responding. Children need close monitoring by a highly experienced PH team to ensure a satisfactory and sustained response to treatment. Optimising management of the condition markedly improves quality of life and survival. Lung transplant is a treatment option considered in children with severe PH who are deteriorating despite maximal medical treatment.

In most cases, how your child is feeling is the best indicator of how well their body is coping with PH. A child with PH can still go to school, dance, play sports, ride a bike and unfortunately be just a naughty and infuriating as any other child! Just like other children (and adults for that matter), a child with PH can help themselves by eating a healthy and balanced diet and having good rest periods. It is really important to have a greater degree of caution in allowing other non-PH doctors prescribe medication for a child with PH – if in doubt always ask your PH team. Make sure your child’s dentist knows that they have PH. Never allow anyone to give your child a general anaesthetic without telling the PH team so that they can get in contact with the anaesthetist.

Herbal medicines should be used with caution as we understand little about them, and some have actually been shown to be harmful. Additionally, many children with PH will be taking warfarin or another drug to help “thin the blood” and these types of drugs can interact with a number of common prescription medications and over-the-counter medications. Again, if in doubt, ask your PH team!

Keeping it normal!

When a child has PH it can affect the whole family. The child, mum and dad, grandparents and other siblings may understandably all feel fed up and worried by the disease at times.  It may be hard, but you should try to treat any child with PH as normally as possible. A child with PH will be happier if the rules stay the same and life carries on much the same as before. They will feel safer and just as loved as the rest as the family if they are treated just like the rest of the family. Children that are treated differently behave differently and a child with PH is only “different” in that they have an illness

As with any sick child, behavioural problems can crop up from time to time.  Deal with them as soon as possible. For example, children when leaving hospital can be more demanding than usual. This can be a common reaction (it can even be seen in adults!), so you should be ready for it. Children just out of hospital can be clingier or may revert to earlier behaviours such as bedwetting. Again, just ask you PH team for help or advice.

Try hard not to forget other children in the family, they may feel upset at the extra attention that their brother or sister is being given. Make sure that friends and family are aware of the need not to pay less attention to your other children and ask that they treat them as equal.

Can you get extra help?

You need to make sure that you have contact with a social worker (ask your PH team if you haven’t already been introduced to one). You may be entitled to benefits that will help deal with any potential extra burdens that you might experience.  A child with PH will probably still be able to attend normal schooling but you may need help in dealing with worries from the school staff about PH.

Holidays are important part of everyone’s lives and while it may take a little more planning with a child with PH (for example you may need to sort out oxygen therapy), it is a great opportunity for the whole family to get away from the normal stresses and strains of everyday life. Holidays can be expensive, but don’t allow this to stop you. Look for help and don’t be afraid to ask, the PHA-UK is normally able to offer assistance.

Summary

Some children with PH have relatives with the disease (although this is extremely rare), but it can develop in a child of any age, boy or girl. Babies can even be born with PH (this is usually because they don't get enough oxygen around the time they are born). In older children, we don't really know why PH affects some but not others. There might be a genetic reason inherited from a parent and / or exposure to something else in the environment. Sometimes a child's PH is caused by another disease. However it arises, if your child has PH, it is not your fault. You didn’t do anything wrong. Unfortunately, it just happens.

Children with untreated PH are often tired a lot, gasp for breath when they exercise, and their lips and fingernails might have a bluish tint. Because children run around more than adults, and their blood vessels relax and constrict more easily, children are more likely than adults to get dizzy and faint. In the school playground, a child with PH might have a hard time keeping up with the others. Unfortunately, if PH in children is not treated, it seems to worsen more quickly than it does in adults. But with treatment, young children do better than adults.

Keep in close contact with your child’s PH team. Ask what you should do if a problem arises out of normal hours, for example at night or at weekends.  Try and make sure your own GP is as up to date as possible with PH and more importantly your child’s treatment regime.