Signs and symptoms of hemophilia
The symptoms of hemophilia depend on the severity of the condition, but the chief indication is prolonged bleeding.
The bleeding may occur spontaneously, so we observe
- sudden nosebleeds
- bleeding gums
- bleeding within joints and muscles
The bleeding may occur after a medical or dental intervention, such as a tooth extraction.
A) Mild hemophilia
Kids born with mild hemophilia may not have any signs for years.
Mild hemophilia usually becomes manifest after a wound, or surgery, or a dental procedure such as having a tooth removed.
B) Moderate hemophilia
Kids with moderate hemophilia are affected like those with mild hemophilia, but they bruise easily.
They may have manifestations of internal bleeding around their joints, especially if they have a blow or a fall that hits their joints (joint bleed).
The manifestations usually start with a tingling feeling of irritability and mild pain in the involved joint, especially the ankles, knees, and elbows. Shoulder, wrist, and hip joints can be affected.
If we ignore joint bleed, it can lead to:
- more severe joint pain
- stiffness
- the bleeding area becomes warm, swollen, and tender
C) Severe hemophilia
The manifestations of severe hemophilia are related to the moderate form of hemophilia. However, joint bleeding is more severe.
Kids with severe hemophilia have spontaneous bleeding.
Bleeding starts without a definite cause and may develop:
- nosebleeds
- bleeding gums
- joint bleeds
- muscle bleeds
Without adequate therapy, individuals with severe hemophilia may develop:
- joint deformity, which may need joint replacement surgery
- soft tissue bleeding
- severe internal bleeding
Bleeding inside the skull, identified as a brain or subarachnoid hemorrhage.
However, spontaneous bleeding inside the skull is rare and induced by a head injury.
Bleeding in the skull is a life-threatening condition that requires urgent intervention.
The symptoms of a brain hemorrhage include:
- neck stiffness
- severe headache
- projectile vomiting
- blurring vision
- an altered mental state such as confusion
- difficulty speaking, such as slurred speech
- loss of coordination of the facial muscles
Females carriers produce adequate clotting factors from their one normal gene helping them to limit bleeding disorder, but some may manifest as mild hemophilia manifestations.
Complication
Complications are frequent with severe and moderate hemophilia and may result from the disease itself or its therapy:
- Internal bleeding leads to swelling, numbness, or pain of a limb.
- Joint damage from hemarthrosis (hemophilic arthropathy) leads to a painful and deformed joint. Debilitating arthritis may develop.
- Infections occur as a result of using blood transfusions as treatment.
- Intracranial hemorrhage is a life-threatening condition caused by the increase of pressure inside the skull. It can create a loss of consciousness, brain damage, and even death.
- Adverse reactions to clotting factor treatment include the improvement of an immune inhibitor, which opposes factor replacement intervention.
Hemophilic arthropathy is a mix of chronic proliferative synovitis and cartilage damage. The untreated intra-articular bleed may cause apoptosis of chondrocytes and influence the construction of proteoglycans. Iron deposition in the synovium may induce an inflammatory response stimulating the immune system and stimulating angiogenesis, followed by cartilage and bone destruction.
