How to handle bleeding in people with hemophilia

PIC: Peter-Nangulu (left), a hemophilic patient with the executive director, HFU, Agnes Kisakye during blood donation at City Square recently. (Credit: Vivian Agaba)

HEALTH

KAMPALA - On Tuesday, Uganda joined the rest of the world to commemorate World Hemophilia Day under the theme, Sharing knowledge.

Medical experts give tips on how to handle bleeds and injuries in a person with hemophilia.

What is Hemophilia?

Dr Philip Kasirye, a paediatrician and fellow-in-training hematologist/oncologist at Mulago Hospital defines hemophilia as an incurable, hereditary (genetic) bleeding disorder in which the affected person's blood does not clot normally because it lacks sufficient blood-clotting proteins.

Hemophilia is caused by a mutation, which is a change in the genetic material on the X-chromosome, a thread-like structure inside human cells that contains genetic information that is passed down through families.

Kasirye says there are two main types of hemophilia; Hemophilia A, which is as a result of Factor VIII deficiency and majority of patients fall under this category, accounting for between 75-80% cases and Hemophilia B, which is due to factor IX deficiency, with a 10-15% of patients.

 "The hemophilic individual either totally lacks or has reduced amount of factors, especially factor 8 and 9, which are the commonest. This means they do not adequately form fibres and therefore, cannot form a strong clot to control the bleeding," he explains.

Generally, the disease presents more in boys or males as compared to their female counter parts.
This is because the genes for the disease are carried on an X chromosome, given the Y chromosome in men is dormant. Females have two copies of the X chromosomes, while males have one X and one Y chromosomes
For females, the extra X chromosome in the mother is protective if it does not carry the hemophilia gene hence they are largely carriers, this means carriers pass the gene to their male children.

Signs and symptoms

Dr Henry Ddungu, a consultant, hematologist at the Uganda Cancer Institute, Mulago Hospital says the common signs one has hemophilia include; prolonged bleeding than usual, for instance after  a cut, removing a tooth or after surgery such as circumcision or after an accident.

It is also characterised by bleeding, especially into the joints and muscles, spontaneous bleeding (sudden bleeding inside the body for no clear reason).

The experts say a person with hemophilia may bleed in different parts of the body, either internally or externally. Here are the tips on how to handle the bleeds.

Head Injury

Because of danger of brain hemorrhage, all head injuries are considered serious (that is, falls from a height of two feet or more in which the head hits a hard surface or object). If your child receives a significant head injury, he should be infused with factor immediately. If any of the symptoms such as headaches, unusual sleepiness, irritability, dizziness, confusion, nausea and vomiting, rush the child to a health facility. 

Abdominal, stomach and intestinal bleeding

Symptoms of abdominal, stomach and intestinal bleeding include; coughing or vomiting up blood or ‘coffee brown material, bloody, black or tarry stools.

Kidney and bladder bleeding, symptoms of kidney and bladder bleeds include red or reddish-brown urine, back pain and frequent or painful urination.

Kasirye adds that if a child bleeds internally, especially in the vital organs such as the brain, lungs, abdomen, kidneys, it can be life threatening or lead to death.

"If a boy child complains of severe abdominal pain, severe headaches has convulsions or fits, the child has difficulties in breathing, severe vomiting, rush him to the hospital. This is because it could be that the child is bleeding internally in the brain, lungs or abdomen and delays in seeking medical care could be life-threatening or lead to death," Kasirye advises.

Eye bleeding

The experts say if your child has an injury to his eye or if you notice that his eye is swollen or discoloured, take him to the doctor or heamophilia unit at Mulago Hospital immediately, untreated eye bleeds can lead to blindness.

Bruises

Ddungu says as children learn to crawl, walk and run, it is natural for them to fall and get some lumps and bruises.

He says if your child has pain and you feel a hard lump developing underneath the bruises or if you have any other concerns, contact a doctor at the hemophilia unit for advice.

Joint bleeding

Early signs of a joint bleed include limping and a reluctance to use the limb, for instance, switching hands for eating to avoid using the painful joint), there may be complaints of a bubbling or tingling feeling inside the joint and a swelling feels spongy. Later, the child may start holding the limb in a bent position to ease pain. 

General safety tips

Agnes Mavis Kisakye, the executive director of Hemophilia Foundation of Uganda (HFU), an organisation that focuses on raising awareness on the disease, care says it is important to treat bleeding quickly.

"When you stop bleeding quickly, you have less pain and less damage to the joints, muscles and organs," she says.

Kisakye advises against injecting a hemophilic person into the muscles, a muscle injection could cause painful bleeding.

Take care of your teeth and to prevent problems, it is important for the patient to follow the advice of the dentist. Dental injection and surgery can cause major bleeding.

Kasirye says it is important for the care takers to learn about basic first aid. He explains that quick aid helps manage bleeding.