Hematomas and uncontrolled bleeding, sometimes without an apparent cause, are typical signs of reduced blood clotting capacity. Here’s how to recognize dangerous situations.
The main feature of hemophilia is the long blood clotting time, on which abnormal bleeding depends on variable severity. In fact, it goes from an almost trivial tendency to the appearance of bruises or to a difficulty in healing more or less deep cuts, until, in the most serious cases, to bleeding to damage the internal organs or even extremely life-threatening cerebral hemorrhages.
Recognizing the symptoms, diagnosing the disease early and setting up an adequate therapy allows to avoid even serious damage and long-term complications highly disabling to the locomotor system.
The extent of the symptoms of hemophilia very often depends on the level of severity of the disease: both type A and type B haemophilia can occur in mild, moderate or severe form.
About 60% of haemophiliacs are affected by the severe form in which symptoms usually appear around 6-9 months of age, when babies begin to sit alone, crawl or take their first steps.
It is not possible to “predict” how many bleedings may occur over the life of a hemophiliac individual, however there are some joints that can easily be the target of bleeding such as knee, hip, elbow and ankle.
When blood flows too much
The extremely slow way in which blood coming out of a cut or wound coagulates can give rise to suspicion of a coagulation defect.
In most hemophiliacs, scratches and small wounds do not create major problems, but injuries to the small vessels of the joints and muscles may not be visible and continue to bleed for a long time “internally”.
In children with severe forms, small subcutaneous hemorrhages may occur, which manifest as bruising, especially in those parts of the body from which parents commonly lift or support them.
Other manifestations that must arouse attention are, for example, difficulty in moving the limbs, which can be an expression of the pain that the child feels due to bleeding, or crying in the presence of swelling.
Severe and less severe forms
In severe forms, even very minor traumas can cause bleeding, and these can involve internal organs or arise spontaneously.
Symptomatic bleeding of hemophilia are nosebleeds (from the nose, most often associated with hemophilia type B), gingivorrhagia (from the gums), gastrointestinal and urinary bleeding, the presence of blood in the urine (hematuria) or feces, and intracranial hemorrhages.
In haemophilia patients, the probability of major bleeding following tooth extractions or surgical operations is high, especially if the deficient coagulation factor is not administered beforehand.
A trauma to joints and muscles
In individuals with hemophilia, joint health is particularly at risk: microtraumas can trigger joint hemorrhages, which are called hemarthrosis, and these, if repeated, can lead to the degeneration of the joint itself with consequent stiffening and loss of functionality (hemophilic arthropathy).
The most frequently affected sites are the knee and ankle, but elbow, shoulder and hip may also be involved.
Hemarthroses initially cause pain and swelling which, if not properly and promptly treated, degenerate into large swellings with consequent difficulty in movement.
Like hemarthrosis, muscle hematomas, if left untreated, can cause functional damage.
From symptoms to diagnosis
Hemophilia is a hereditary disease and, therefore, an accurate family history, especially in the presence of the symptoms described, is indispensable.
However, in order to have a correct diagnosis of hemophilia, familiarity and symptoms alone are not enough, but specific laboratory and genetic analyzes are needed, which not only confirm or not the disease and the type but, above all, allow to evaluate the level of severity that is the key factor to be able to correctly set up a therapeutic plan.
Joycelyn Elders is the author and creator of EmpowerEssence, a health and wellness blog. Elders is a respected public health advocate and pediatrician dedicated to promoting general health and well-being.
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