Bumps and scrapes are a part of every child's life. For most kids, a tum­ble off a bike or a stray kick in a soc­cer game means a tem­po­rary bruise or a heal­ing scab. For chil­dren with haemophil­ia, these nor­mal trau­mas of child­hood are rea­son for ex­tra con­cern. Haemophil­ia is a rare bleed­ing dis­or­der that pre­vents the blood from clot­ting prop­er­ly, so a per­son who has it bleeds more than some­one with­out haemophil­ia does. It's a ge­net­ic dis­or­der, which means it's the re­sult of a change in genes that was ei­ther in­her­it­ed (passed on from par­ent to child) or oc­curred dur­ing de­vel­op­ment in the womb. Haemophil­ia af­fects most­ly boys-about 1 in every 5,000–10,000 is born with it. Girls are more rarely af­fect­ed. A male can't pass the gene for haemophil­ia to his sons, though all his daugh­ters will be car­ri­ers of the dis­ease gene. Each male child of a fe­male car­ri­er has a 50 per cent chance of hav­ing haemophil­ia.

About haemophil­ia

There are two ma­jor kinds of haemophil­ia, haemophil­ia A and haemophil­ia B. About 80 per cent of cas­es are haemophil­ia A. Haemophil­ia is clas­si­fied as mild, mod­er­ate, or se­vere, based on the amount of the clot­ting fac­tor in the per­son's blood. In gen­er­al, a per­son with milder haemophil­ia may on­ly bleed ex­ces­sive­ly once in a while, where­as se­vere haemophil­ia puts some­one at risk for hav­ing bleed­ing prob­lems much more of­ten.

Signs and symp­toms

Signs and symp­toms of haemophil­ia vary, de­pend­ing on sever­i­ty of the fac­tor de­fi­cien­cy and the lo­ca­tion of the bleed­ing. Few ba­bies are di­ag­nosed with haemophil­ia with­in the first six months of life, be­cause they're un­like­ly to sus­tain an in­jury that would lead to bleed­ing. Once ba­bies with haemophil­ia be­gin crawl­ing and cruis­ing, par­ents may no­tice raised bruis­es on the stom­ach, chest, but­tocks, and back. Some­times, be­cause bruis­es ap­pear in un­like­ly places, par­ents may be sus­pect­ed of child abuse be­fore their child is di­ag­nosed with haemophil­ia.

The ba­by may al­so be fussy and may not want to reach for a cup, walk, or crawl. Oth­er symp­toms in­clude:

• pro­longed nose­bleeds

• ex­ces­sive bleed­ing from bit­ing down on the lips or tongue

• ex­ces­sive bleed­ing fol­low­ing a tooth ex­trac­tion or loss of a tooth

• ex­ces­sive bleed­ing fol­low­ing surgery

• blood in the urine (called hema­turia)

The most com­mon type of bleed­ing in haemophil­ia in­volves mus­cles and joints. A child with haemophil­ia will usu­al­ly refuse to move the af­fect­ed joint or mus­cle be­cause of pain and swelling. Re­cur­rent joint bleed­ing can al­so lead to chron­ic dam­age.

Di­ag­no­sis

Your doc­tor may sus­pect your child has haemophil­ia if there's a pat­tern of bruis­ing and bleed­ing, par­tic­u­lar­ly if this in­cludes bleed­ing in­to the joint. Di­ag­nos­ing the con­di­tion re­quires a set of blood tests, in­clud­ing a com­plete blood count (CBC), pro­throm­bin time (PT), ac­ti­vat­ed par­tial throm­bo­plas­tin time (PTT), fac­tor VI­II lev­el, and fac­tor IX lev­el.

Treat­ment

Haemophil­ia is a life­long con­di­tion with no cure (oth­er than liv­er trans­plan­ta­tion, a pro­ce­dure that can some­times cause health prob­lems more se­ri­ous than haemophil­ia it­self), how­ev­er, it can be suc­cess­ful­ly man­aged with clot­ting fac­tor re­place­ment ther­a­py-pe­ri­od­ic in­fu­sions of the de­fi­cient clot­ting fac­tor in­to the child's blood­stream.

Pre­vent­ing prob­lems

Par­ents can help kids with haemophil­ia pre­vent prob­lems by en­cour­ag­ing healthy be­hav­iors. For ex­am­ple, ex­er­cise can strength­en mus­cles and help de­crease bleed­ing from in­juries. Swim­ming is strong­ly en­cour­aged be­cause it ex­er­cis­es all the mus­cle groups with­out putting stress on the joints. The child's weight should al­so be man­aged prop­er­ly, be­cause ex­cess weight can cause strain in re­gions of the body and in­crease bleed­ing risks. Al­though each stage of de­vel­op­ment comes with its own set of is­sues, ex­perts say the tod­dler and teenage years can be the most chal­leng­ing for kids with haemophil­ia. Both phas­es nat­u­ral­ly in­volve a child's quest for in­de­pen­dence.

When to call the doc­tor

Cer­tain bleeds re­quire med­ical at­ten­tion, in­clud­ing those in­juries af­fect­ing:

• the cen­tral ner­vous sys­tem - any sus­pect­ed trau­ma to the head, neck, or back

• the face, in­clud­ing the eyes and ears

• the throat or an­oth­er por­tion of the air­way

• the gas­troin­testi­nal tract (which might pro­duce signs such as bright red or black blood in the stool)

• the kid­neys and uri­nary tract (if you find blood in the urine, this may re­quire treat­ment and bed rest)

• the il­iop­soas mus­cle in the trunk (which might pro­duce signs that mim­ic a hip or ab­dom­i­nal bleed, in­clud­ing low­er ab­dom­i­nal/groin or up­per thigh pain, an in­abil­i­ty to raise the leg on the af­fect­ed side)

• the gen­i­tal area

• the hips or shoul­ders (these can be com­pli­cat­ed bleeds be­cause they in­volve the ro­ta­tor joints)

• large mus­cle com­part­ments, such as the thighs.

(Kid­sHealth)