About one in 5000 people in Germany suffers from a blood clotting disorder. Thereby, the triggers as well as the treatment of clotting disorders are very different.
What is a blood clotting disorder?
Blood clotting disorders are either too weak or too strong clotting (hemostasis) of the blood in the event of injury to one or more blood vessels. Normally, clotting occurs due to an interaction of several factors: on the one hand, a constriction of the blood vessels takes place, which is intended to stem the further inflow of blood that would then leak from the wound. On the other hand, the thrombocytes, i.e. the blood platelets, stick together at the corresponding site and thus ensure rapid wound closure, which is then reinforced by the so-called fibrin threads. These filaments are the product of 12 clotting factors in the blood plasma that are activated in an emergency. If the functionality of these clotting factors is impaired, a blood clotting disorder is present, which can lead to either too much clotting (e.g., thrombosis) or too little (e.g., hemophilia).
Causes
There are several reasons for the occurrence of a blood clotting disorder. In some cases, a lack of platelets ensures that the damaged blood vessels cannot be sufficiently clotted by them and thus the bleeding cannot be stopped properly. This is the case, for example, in the so-called Von Willebrand-Jürgens syndrome. However, even if platelets are present in sufficient quantities, they may lack the ability to stick together. In rare cases, this malfunction can be inherited. Far more frequently, however, it occurs as a side effect of taking certain medications. Another trigger of too weak clotting of the blood can be the dysfunction of the clotting factors. This is the case, for example, in the famous hemophilia. In addition, since the liver is responsible for the formation of most of the 12 clotting factors, diseases of the liver can also be causative for a blood clotting disorder.
Symptoms, complaints, and signs
A blood clotting disorder is primarily manifested by the frequent occurrence of hematomas. If a clotting disorder is present, bruising occurs after even light blows to the skin and usually persists for several weeks. Bleeding after an injury or operation lasts much longer than usual or recurs after the wound has already closed. Furthermore, hemorrhages of various sizes may occur. Small skin bleedings, so-called petechiae, but also extensive wounds and bloody rashes are typical. These symptoms clearly indicate a blood clotting disorder. In most cases, they lead to further physical signs. Thus, bleeding that lasts for a longer period of time becomes noticeable through the symptoms of anemia: The affected person is pale, has sunken eye sockets and is physically and mentally less capable. Headaches, visual disturbances and joint pain may also occur as a result of the disease. Sometimes blood is noticed in the urine or stool. Depending on the degree of deficiency, swelling may occur in the muscles and ankles, usually associated with movement disorders and nerve pain. Gastrointestinal symptoms such as nausea, vomiting and diarrhea are also typical in blood clotting disorders. Likewise, dizziness and poor concentration may occur.
Diagnosis and course
A blood clotting disorder can have dire consequences for the affected person. For example, too much clotting can cause blood clots to form, which can then lead to blockage of vessels in the brain, lungs, or heart. In the worst case, this can lead to a heart attack, pulmonary embolism or stroke. Too weak blood clotting, on the other hand, means great danger for those affected, even in the case of small cuts or unnoticed internal bleeding, because the bleeding is very difficult to stop. Blood coagulation disorders are primarily detected by laboratory tests in which the activity of the blood coagulation is checked, for example by determining the number of platelets. In addition, the number of clotting factors in the blood can also be determined. If a platelet deficiency is the cause of the blood clotting disorder, a bone marrow sample is also taken to investigate the reason for the deficiency.
Complications
A blood clotting disorder can mean both prolonged bleeding and shortened bleeding, both of which can have different complications. Prolonged bleeding in hemophilia causes bleeding in all sorts of places in the body. For example, major blood loss can occur around muscles, causing severe pain. In the abdomen, this can be misleading and inflammation of the appendix (appendicitis) is more likely to be thought of, so surgery that is not needed may be initiated. Severe bleeding can also lead to compression of organs. In the area of muscles and vessels, this can lead to a compartment syndrome, which in the worst case results in the death of the individual muscle groups. In the neck–head area, hemorrhage also typically leads to a narrowing of the airways and the affected person suffers from shortness of breath. Shortened bleeding in the context of thrombophilia allows the risk of thrombosis to increase. Thromboses are predisposed especially in the leg veins. These lead to severe pain and edema in the legs. In addition, ulcers form more easily on the ankles and feet. In the worst cases, the thrombosis breaks loose and is carried with the bloodstream. This can lead toward the lungs and brain, causing pulmonary embolism or even stroke.
When should you go to the doctor?
Bruises after a fall or an impact injury are harmless in most cases and do not require medical treatment. You also don’t need to see a doctor immediately for minor nosebleeds or small wounds. However, if larger bruises form after a harmless collision or minor impact, caution is advised because they could be signs of hemophilia, which can become life-threatening if left untreated. People who suffer from hemophilia have impaired blood clotting and can bleed to death from major injuries or surgeries without medical attention. Just like large bruises, frequent, severe nosebleeds or smaller wounds that keep bleeding can indicate hemophilia. If either sign is present, it is imperative to see a coagulation specialist, because hemophilia requires medication because affected individuals lack a clotting factor that helps wounds heal. To avoid bleeding to death, they must inject themselves intravenously with the missing clotting factor. If a blood clotting disorder is suspected, a blood test should always be performed. People with a tendency to thrombosis and blood clots should also see a doctor.
Treatment and therapy
Treatment of a blood clotting disorder depends entirely on its particular trigger. For example, if there is a lack of platelets, these must be artificially supplied in the form of a platelet concentrate. Since a platelet deficiency can also be triggered in rare cases by an erroneous reaction of the immune system, this must be prevented by taking immunosuppressants such as cortisone. A deficiency in clotting factors can also be remedied by regular administration of a special concentrate. If, on the other hand, external factors such as the use of medications or alcohol abuse are responsible for the disturbed blood clotting, these must of course be discontinued immediately or withdrawal must be started. If, on the other hand, there is excessive clotting with a tendency to form blood clots, the first thing to do is to find the cause of this process. These are often found in other diseases such as cancer or infections or in the after-effects of childbirth or severe blood loss. If these causes are correctly identified and addressed, this also leads in most cases to the normalization of blood clotting and, as a consequence, to the disappearance of the blood clotting disorder.
Outlook and prognosis
In most cases, a blood clotting disorder can be treated relatively well and easily, allowing the affected person to participate in ordinary daily life without complications or limitations. However, the course of the disease also depends on the exact cause, so a general prognosis cannot be given. In the case of erroneous reactions of the immune system, the blood clotting disorder is relatively well alleviated by the administration of suppressants or cortisone.When taking other medications or in the case of severe alcohol dependence, the underlying disease must be treated first and foremost, or the respective medication must be replaced or discontinued. If the cause is identified correctly and at an early stage, the blood clotting disorder can be completely limited in most cases. The affected persons must always inform the treating physician about the blood clotting disorder when it occurs in order to avoid severe bleeding. A healthy lifestyle with lots of sports activities and a healthy diet can prevent possible thrombosis due to this disorder. In the case of cancer as the cause of the blood clotting disorder, it is usually not possible to make a positive prediction about the course of the disease. In this case, the course is highly dependent on the type of cancer and its severity.
Prevention
A blood clotting disorder based on hemophilia cannot, by its very nature, be prevented. If the coagulopathy is based on medication, the coagulopathy can be prevented by stopping the medication. However, this should only be done in consultation with the physician. Blood coagulation disorders that can lead to thrombosis can be prevented in good time with plenty of exercise, sport and a healthy diet. Despite all this: blood clotting disorders and their possible consequences can be dangerous but fortunately they are also well treatable.
Follow-up
Aftercare for a blood clotting disorder is provided by means of preventive measures and medical checkups. The type of aftercare also depends on the nature of the blood clotting disorder. In almost every case, it is necessary to have the flow properties of the blood checked regularly and to keep track of the clotting factors. In people with a strong tendency to clotting, follow-up consists primarily of regular checks of the vessels. Since thromboses occur frequently, they must be detected early. Accordingly, prophylactic administration of blood thinners may be useful. In stressful situations involving little exercise, such as long-distance flights, the administration of blood thinners is recommended. In forms of hemophilia, follow-up care consists of clarifying abnormalities (for example, skin discoloration, blood in the stool or urine) and urgently avoiding injuries. Medication, administration of hormones or donor factors may be necessary for prophylaxis throughout life. If internal bleeding has led to tissue damage, follow-up care consists of appropriate therapy. For affected muscles or bones, this means using physical therapy. Sports can be useful to strengthen the joints, as these are also affected in hemophilia. Endurance sports are suitable, but not contact sports. In the case of damage to the organs, on the other hand, aftercare must be based on the damage in question.
What you can do yourself
Adjustments in everyday life to blood clotting disorders depend on whether there is decreased blood clotting or too much blood clotting. In the case of reduced blood clotting, as is often also produced voluntarily by medication in the case of cardiac arrhythmias or after the implantation of artificial heart valves, there is basically a risk of bleeding that is difficult to stop. This can lead to complications in the case of internal injuries. If the insufficient blood clotting, as in the case of so-called hemophiliacs, is due to hereditary factors, causal therapy is not possible. In cases where the unintentional reduced clotting ability of the blood is due to the use of certain medications or to alcohol abuse, alternative medications or alcohol withdrawal should be considered. An important measure in the case of artificially induced “blood thinning” by taking coagulation inhibitors consists, on the one hand, in necessary increased attention to avoid injuries due to the strong risk of bleeding. In addition, it is advisable to carry a small identification card on which it is noted which anticoagulant drugs are being taken and at what strength. This is particularly important in the event that surgical intervention becomes necessary after an accident. In the opposite case of too much anticoagulant medication, there is always an increased risk of thrombosis, stroke or heart attack. To minimize the risk of thrombosis, self-help measures can have a preventive effect in addition to taking coagulation inhibitors.These are sports activities and healthy diet to avoid vitamin and mineral deficiencies.
