Babies are fun! We know that because Adam, our first grandchild, is busy giving us a refresher course in small people.

When a baby with haemophilia is born he is just as much fun as any other baby. Nothing in his childhood needs to be different.

It is terribly important to realise this. The "START-LINE" for anybody must be normality. This is true of any disorder because if the normal things are not recognised and nurtured they will not thrive. When this happens the disorder can eventually take precedence over everything else, smothering the child and his family and eventually leading to the self recognition of haemophilia as a handicap. A diagnosis of haemophilia is not a handicap; it can only become one if the family or their medical advisers allow it to.

PREGNANCY, BIRTH AND DIAGNOSIS

When there is a family history and the prospective mother knows from this that she may be a carrier, she and her partner should talk to doctors with experience of up-to-date haemophilia care. Any decision about having a child who may have haemophilia always lies with the couple themselves, but doctors can help in the diagnosis of carriership and sometimes, if the couple want it, the diagnosis of haemophilia in the womb. If a twin pregnancy is diagnosed what are the chances of both babies being affected? Identical twin boys will of course both have haemophilia if they have inherited the gene. Non-identical twin boys may or may not both have the condition, it depends on which of the mother's X chromosomes each have inherited.

When a couple decide that they want pregnancy to go to term, arrangements can often be made for diagnosis to be carried out on a specimen of blood taken from the umbilical cord immediately after birth. It is important that good facilities are available both within the maternity hospital for taking the specimens, and within the nearest Haemophilia Centre so that the specimens can be tested very quickly. Any delay may give a false result.

Haemophilic babies are born perfectly normally and are not in any danger during normal vaginal delivery. Caesarian section is only indicated when there are particular difficulties during the delivery, for instance a potentially difficult forceps procedure. On the sixth day of life all babies have blood taken from a heel for the Guthrie test for phenylketonuria. The skin prick is small, and I have never known problems even when the infant has severe haemophilia.

When there is a family history the parents' attitudes to a new born baby with haemophilia will obviously be coloured by the family experiences. If these have been bad with, for instance, particularly nasty bleeds or experiences of surgery going wrong, or premature death of relatives, then the couple will be more fearful for their child than they would be in a family where all the experience is good. Research and development in haemophilia is so rapid that it is very important for couples in this position to get up to date information before their child is born, so they can act on fact and not fantasy. Old medical textbooks from the local library are NOT recommended.

When there is no family history the diagnosis of haemophilia will not be made at birth. It will probably follow either the appearance of bruising when the baby is around one year old and beginning to be more mobile, or perhaps prolonged bleeding following injury in the mouth. Typically, the delicate piece of tissue between the top lip and the gum is injured. In normal children bleeding quickly stops. In a child with severe haemophilia it commonly restarts again because healing is easily disturbed by movement during eating or chattering.

Unfortunately, because haemophilia is so rare (around 1 in 10,000 boys), its diagnosis may be delayed for some time and doctors may look for other causes of bruising including, in some cases, non-accidental injury. This is awful for any parent, but unfortunately reflects our society and the fact that babies who have been injured intentionally form a normal part of the life of any children's department. Clotting tests soon lead to the right diagnosis and referral to the nearest Haemophilia Centre.

Families who have had other children before the son with haemophilia already have experience of the normal growth and development of children, with all the successes and setbacks usual as kids grow up. They can therefore tell the difference between the trials and tribulations of everyday life and the problems caused by haemophilia. The couple with a first born baby with haemophilia have a lot to learn, and life can be especially difficult if they have been blessed with a hyperactive youngster whose only intention is to take command of the family, day and night.

THINGS THAT NEED DOING ONCE YOU ARE HOME WITH YOUR BABY

First and foremost is the need to have a good family doctor and to have access to a good Haemophilia Centre. The family doctor does not need to be expert in the condition but he or she gives all the usual support needed by a young couple and their children. The Haemophilia Centre staff work with the family doctor whilst providing the specialist care for the haemophilia. You should also join the Haemophilia Society.

Make sure that you understand your son's diagnosis and are given a "Special Medical Card for Haemosrhagic States", which should be filled in fully. In particular, it is important to know his factor VIII or IX level because on this depends the specific treatment of his disorder. For instance, if he has a factor VIII level of over 5% he may be able to be treated with desmopressin rather than with a blood product. Throughout his childhood the doctors will routinely test him for inhibitors to factor VIII or IX; the discovery of an inhibitor can mean that a change in treatment is needed.

Like any child, the boy with haemophilia needs to grow up in a secure environment. Injuries are more likely to occur in the cramped, crowded conditions of inner cities with steep stairs and access only to busy streets, than in more spacious houses with gardens. Help can often be given in re-housing by staff of the Haemophilia Centre. Every family with a child with haemophilia should have a telephone in the house, and again Haemophilia Centre staff can often help persuade local authorities to provide telephones for families who could not otherwise afford them.

All parents need to escape the demands of their offspring at times, and the parents of a haemophilic child are no exception. Babysitters can be armed with knowledge of where the couple are, and if they wish the parents can nowadays carry a pager with them whilst they go to the cinema or local pub.

One of the family doctor's roles is to ensure that all children should be immunised against the full range of infectious diseases. When injection is needed, that too is perfectly safe in haemophilia because the volume of the injection is small. My advice is to keep pressure over the injection site for four or five minutes to prevent any untoward bruising. In the case of BCG, for the prevention of tuberculosis, there is no need to put pressure on. In addition to the usual immunisations in childhood all children with haemophilia should be vaccinated against hepatitis B. There is also a new vaccine against hepatitis A and this will soon probably also be recommended to families. No vaccine yet exists for hepatitis C.

Other than the injections needed for immunisation, all other intramuscular injections are banned in haemophilia. Medicines are given by vein instead. Remember that no child should be given aspirin nowadays. This is especially important in haemophilia because the drug interferes with the stickiness of the blood platelets, and adds to problems with bleeding.

It does not matter how babies with haemophilia are fed, although my general advice to all mothers is always to try to breast feed before turning to the bottle. Weaning is normal. Nor is there any reason for handling babies with haemophilia differently than any other baby. Cots do not need to be specially padded and the same care that parents take in choosing any toy applies to those chosen for the baby with haemophilia. He should be cuddled and played with, and swung and enjoyed by all the family, relatives and friends just like any other child. There is no need to make any alterations to pushchairs or prams. He can have a baby bouncer and a baby walker, but make sure the walker cannot tip up easily as he becomes more adventurous. If you live in a house with stairs, have a gate fitted at the top of them. In the car make sure that he has a proper baby seat securely fastened in the back. Never travel with him in a front seat.

When I was young clothing for babies was dreadful. Everybody, whether boy or girl, seemed to be attired in white dresses with plenty of lace. This may or may not have something to do with some of the stones we hear about certain sectors of the British establishment! Nowadays clothes for youngsters are super, bright and colourful, warm in winter and cool in summer. That's fine but make sure that, whatever its colour, his footwear is of good quality as he grows up.

SOME COMMON QUESTIONS

Three particular questions that are sometimes raised during infancy concern circumcision, teething, and anal fissures.

Circumcision should not be performed unless there is a real necessity. In medical terms this means good evidence that a tight foreskin obstructs the flow of urine or sometimes becomes inflamed, rather than that the foreskin doesn't retract; that is usual in many boys until they are around seven years old. If there is a medical reason, or there are irrevocable religious reasons, then the procedure must be carried out very carefully in an operating theatre with full haemophilia protection. This will mean the baby and his mother staying in hospital for a few days.

Babies with even severe haemophilia have no more than the usual trouble when they teethe. Later, when they start to lose their baby teeth all is normal too; the teeth are pushed out very slowly with only a minimal amount of bleeding that does not require treatment.

A lot of babies develop small tears around the anus after passing hard stools A tear like this is called an 'anal fissure' and it distresses the baby because it is painful during defecation. In haemophilia it can also lead to a little more bleeding than usual. Fissures heal up easily if stools are softened by, for instance, adding more sugar or fruit juice to the diet of a baby being artificially fed. Sometimes a little local anaesthetic cream can help. I have never known a baby with haemophilia and an anal fissure require specific haemophilia treatment.

FOLLOW UP

Other than the need for regular follow-up at the Haemophilia Centre, most families need to spend very little time visiting a hospital in the early years. When they do, it is extremely important that fathers make every effort to get along as well. In our society it is the mothers who are often expected to take their children to hospital for follow-up and to cater for their bleeding episodes. In the old days this attitude used to lead to haemophilic children growing up without real recognition of their dads and with over-dependence on the love and care of their mothers.

Main lesson of the day: ENJOY HIM!

Published in 1994