It is a tribute to human resilience that most youngsters and their families survive adolescence. Being a teenager is difficult enough without the burden of haemophilia. Whilst parents need patience, a sense of humour and a bottomless bank balance, their son needs both the security of home and the space to grow to the independence of adult life.

There is one word which sums up everything families needs to know about adolescence and haemophilia. It is CONTROL. When someone is in control of their health life is normal. When health is compromised and in control of them, life is abnormal. If someone with haemophilia allows his disorder to govern his life he is not in control; his haemophilia is, and his happiness is clouded. Learning to put life first and haemophilia second is the single most important feature of growing up.

To be in control requires discipline. It is useless to simply "forget" haemophilia and not to, for instance, have a dose of factor VIII or IX before an event known to provoke a bleed. It is silly to imagine that a run of bleeds into a major joint will just "go away" without therapy or subsequent damage. It is foolhardy to go off on holiday without packing the equipment needed to treat a bleed, or finding out the location of the nearest haemophilia centre. In all these cases common sense planning ensures that life can run smoothly without the disruption of untreated haemorrhage.

Most children will have learnt to control their haemophilia before puberty. They are in command, knowing exactly when to ask for treatment, judging the dose, mixing their concentrate, increasingly doing their own venepunctures, and clearing up afterwards. And then forgetting about it and getting on with the far more exciting events of life with their friends at home and school.

If this hasn't happened hemophilia can make adolescence very difficult for a while. At some stage there has to be a break. No-one wants haemophilia, but it is there. Coming to terms with it whilst at the same time wanting to slip the net of parental influence, can be very frightening and hurtful. The obvious response is anger, and anger used to be a frequent occurrence in the consulting room before comprehensive care became established and families learnt about letting children taking responsibility for their haemophilia gradually as they grew up.

CAREERS

Choice of career is especially important in these days of high unemployment. Few jobs are closed to those with severe haemophilia; they include work involving particular hazard (for instance the armed forces and emergency services) or the risk of being isolated from special medical help should an emergency arise. With these exceptions and contrary to popular opinion, people with severe haemophilia are able to work manually, and some of them undertake the sort of work that would rapidly floor most doctors. Obviously, whatever career is chosen the man with haemophilia must be ready to compete as an equal to somebody without haemophilia, both when applying for jobs and when working.

There are obvious benefits in becoming as highly qualified as possible before leaving school or university; the choice is so much wider. All schools have easy access to advice about careers, but sometimes this is given rather late in the day. In the context of severe haemophilia it is important that the possibilities are explored sooner rather than later. Early planning helps ensure that any disruption caused by untoward bleeding is catered for, and special help with difficult subjects is provided. Most youngsters need time to develop their own ideas about what they want to do with their lives; early decisions can always be changed later in the light of new interests and work experience. When doubt about choice of career persists a consultation with an educational psychologist may help to point an indecisive teenager in the right direction.

SEXUALITY

The knowledge that haemophilia is inherited and that the abnormal gene can be passed on to children is bound to colour attitudes to sex and parenthood. Young men who have not yet come to terms with their haemophilia feel more "different" than is usual in adolescence, and may find early courtship difficult because of this. Carrier girls are vulnerable because of the possibility of having haemophilic sons. Life is especially hard for them if there is a bad family history, or if Dad's haemophilia is complicated by severe arthritis, hepatitis or HIV infection.

Two aspects of adolescence may need sensitive counseling. Firstly, carrier girls may have low factor VIII or IX levels and these may be linked to heavier periods than usual. When this happens it is very easy to put things right - one of the low dose pills, or regular courses of cyklokapron, are all that is needed. Secondly, young men may have bleeds induced in the heat and passion of the moment. Again, treatment is straightforward, painful bruising being countered by a dose or two of the relevant factor concentrate. One event that is fairly common at this age is a psoas bleed - that needs energetic treatment with replacement therapy for several days, together with bed rest and then physiotherapy. The psoas muscle runs from the side of the backbone and sweeps forwards round the pelvis to emerge in the front of the thigh. If not stopped quickly bleeding into it compresses the major nerve to the quadriceps group of muscles leading to weakness, instability of the knee and a loss of sensation to touch which can extend from groin to foot. Recovery from a poorly treated psoas bleed can take many months.

SPORTS

Keep active : keep well! Physical activity promotes powerful muscles which protect the major joints. Flabby muscles cannot support joints properly and bleeding episodes are more frequent. Obesity adds to the problem by putting more strain on the unstable joints.

Children of all ages should be encouraged to participate in sports. By the time the boy with haemophilia has reached adolescence he should know which activities he enjoys. He should also be learning those activities which result in bleeds and, if these occur during a sport he has set his heart on, whether he can control them with prophylactic factor VIII or IX beforehand. Regular, enjoyable sport results in feelings of well-being and fulfillment, which help counterbalance the unease and loneliness that haemophilia can provoke. Team work is another powerful weapon in dispelling ideas of isolation or handicap.

Recently, on behalf of the World Federation of Hemophilia, I asked colleagues for their views on sports most suited to people with severe haemophilia A or B. All agreed that swimming, table tennis and walking were to be encouraged, and many more sports were recommended. The "top 10" sports are listed in the first table.

In the second table are the least recommended sports, some of which carry a major threat of head or neck injury.

Altogether the questionnaire listed 69 sports. Bungee jumping was not one of them, although I know three people with haemophilia who have tried it. It would terrify me, and is probably the quickest way of finding out if there are weak blood vessels in the brain - prophylaxis is advisable first! Football (soccer) was well down the list; some doctors thinking that at a competitive level the risks of damage to the legs is too high. I do not share this view, preferring to let youngsters make up their own minds. What is essential for football, as for any sport, is that the usual protective clothing is always worn. No self respecting batsman (if there are any left) would dream of facing Merv Hughes without all the gear.

TRAVEL

During the teens most youngsters make their first escape from parents and siblings. Holidays abroad, summer camps and trips with the school all provide the necessary inducements. Haemophilia is not a bar to travel. Again, common sense precautions ensure the trip is a happy one. A holiday letter detailing the medical history and the usual haemophilia treatment should be obtained from the centre which should also provide a clearance note for customs for any equipment, concentrate or desmopressin (DDAVP) carried. A list of centres nearest to the destination, holiday insurance (read the small print!) and, in Europe, Form E111 are the other essentials. And don't forget that lads (and lasses) with the haemophilia gene are just as likely to fall foul of alcohol, drugs and sexually transmitted diseases whilst in Benidorm as anyone else.

Finally, having survived parents, brothers, sisters, schoolmasters doctors, the dreaded nurse, AND adolescence, the average young man with haemophilia wants nothing more than to live his life his way. Great! But one thing to think about please. Others less fortunate than yourself (especially those with haemophilia in developing countries) now need your enthusiasm, your energy and your expertise in living with haemophilia. The various haemophilia societies and the World Federation of Hemophilia will welcome your active participation. So add them to your list. Greenpeace, Save the Whales, Antivivisection… and the Haemophilia Society.

Main lesson for life : maintain your CONTROL.

Published in 1994