Last updated on January 11, 2022
Content By: Dr. Nikita Toshi BDS, Assistant Manager (Medical Review), Dr. Ritu Budania MBBS, MD (Pharmacology) Head, Medical Affairs &
Last updated on January 11, 2022
Blood is often seen as a crimson saviour of our bodies. It is a transporter of all the oxygen and nutrients our organs need. As a liquid vessel that travels throughout our body in the crevices of all the organs and veins, it carries out other important functions as well.
One of the main functions is the role of lifeguard that the blood assumes – as soon as it detects any injuries or wounds in your body, it gathers its task force of proteins and platelets and forms a secure gel-like seal called blood clots.
This process of clotting is called blood-coagulation. However, sometimes due to genetic reasons, deficiencies, consequences of medications, our blood may not be able to carry out this protective function efficiently. This leads to some of the types of bleeding disorders that slow down or interfere with the mechanism of clotting, which may in turn lead to excessive bleeding and blood loss.
The main step from your end to manage different types of bleeding disorders is to learn as much about them as possible. Throughout this article, explore questions like the bleeding disorder meaning, causes, symptoms to look for and treatment. You even get to connect with some real stories of people just like you to help you realize that you have all it takes to gain control over this condition!
Blood is often seen as a crimson saviour of our bodies. It is a transporter of all the oxygen and nutrients our organs need. As a liquid vessel that travels throughout our body in the crevices of all the organs and veins, it carries out other important functions as well.
One of the main functions is the role of lifeguard that the blood assumes – as soon as it detects any injuries or wounds in your body, it gathers its task force of proteins and platelets and forms a secure gel-like seal called blood clots.
This process of clotting is called blood-coagulation. However, sometimes due to genetic reasons, deficiencies, consequences of medications, our blood may not be able to carry out this protective function efficiently. This leads to some of the types of bleeding disorders that slow down or interfere with the mechanism of clotting, which may in turn lead to excessive bleeding and blood loss.
The main step from your end to manage different types of bleeding disorders is to learn as much about them as possible. Throughout this article, explore questions like the bleeding disorder meaning, causes, symptoms to look for and treatment. You even get to connect with some real stories of people just like you to help you realize that you have all it takes to gain control over this condition!
Written by
BDS, Assistant Manager (Medical Review)
Reviewed by
MBBS, MD (Pharmacology) Head, Medical Affairs
Blood is a protective fluid. Throughout our body, it carries everything we need to live – oxygen and nutrients. As a part of this process, it acts as a healing agent as well. The moment blood detects any injury or invasion of foreign substances, it gets to work and forms clots to protect our body. This process is called coagulation.
While this is a protective mechanism, sometimes the clots can be wrongly formed, indicative of Coagulopathy. Therefore, coagulopathy or bleeding disorder means that your body undergoes a condition where it experiences an abnormal amount of bleeding, due to a problem with your clotting system.
This bleeding and clotting disorder is of various types – predominantly, Haemophilia, Von Willebrand disease and platelet disorders. Depending on the types of bleeding disorders, the severity and treatment of this condition can vary. However, excessive bleeding may lead to concern of blood loss, especially from vital organs like the heart and the brain.
The statistics of those with this condition varies based on the type of bleeding disorder, however, in terms of Hemophilia, the USA and Brazil report the highest case of around 4 cases every 1,00,000 population. In comparison, India reports only 0.9 cases for every 1,00,000 population, which some studies find to be an underrepresentation owing to mistreatments, undiagnosed cases or lack of awareness. A study into one of the major types of a bleeding disorder, Haemophilia, shows that it is more common among men than women worldwide.
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Blood clots are our body’s way of plugging blood flow to stop excessive blood loss from injury or harm. The process of blood clotting, called Haemostasis, is a team effort by several clotting factors (proteins) and platelets in the blood. There are more than 20 clotting factors and every type of bleeding disorder is caused by the deficiency or abnormality of a specific clotting factor.
Here are some of the most common types of bleeding and clotting disorders in India:
The condition caused by factor VIII deficiency (8) or protein is called Haemophilia A, often called Classic Haemophilia. This is the most common inherited bleeding disorder among the two Haemophilia types, as it is 4 times more common than Haemophilia B across most ethnic groups.
In this haemophilia type, people tend to bleed longer and more profusely than others, especially during minor injuries like cuts, dental procedures, periods, etc. This is due to clotting FVIII deficiency (factor 8). Haemophilia A causes can be traced to the level of FVIII in blood. Our blood needs 50% – 150% of Factor VIII to form a clot, anything below that range (1% – 49%) is an indication of Haemophilia type A.
Haemophilia B is a genetically inherited condition commonly called Christmas Disease. Just like type A, this is also one of the most common inherited bleeding disorders. Haemophilia B is caused by a deficiency in FIX (factor 9), where about ⅓ of the cases are due to a change in the gene over the person’s lifetime.
Similar to type A, anyone diagnosed with type B haemophilia tends to bleed excessively either internally or externally from their cuts and injuries. For a clot to form, around 50% – 150% Factor IX is required in the blood. Anything below that level (0% – 49%), is considered a deficiency of Factor IX, denoting the presence of Haemophilia type B.
The clotting process requires the joint effort of several proteins in the blood. The absence, deficiency or even malfunction of the Von Willebrand Factor (VWF) in the blood leads to improper clotting of blood or profuse bleeding. The VWF binds to protein VIII to form a clot, so even in the case of factor VIII deficiency, Von Willebrand Disease can emerge.
There are a few Von Willebrand Disease types based on the severity of disease and inheritance factors:
Type 1 VWD – Those containing 20% – 50% of the normal requirement of VWF for the blood to clot. VWD type 1 is the mildest form of the disorder and accounts for approximately 70% – 80 of cases.
Type 2 VWD – People with this Von Willebrand Disease type have normal levels of VWF in the blood but it does not function effectively. VWD type 2 is further subdivided depending upon the specific, underlying defect involving VWF. These subtypes are known as VWD type 2a, 2b, 2m and 2n. Type 2 VWD accounts for 20% of cases.
Type 3 VWD – Those with type 3 VWD have dangerously low levels to no amount of VWD in their blood. They might also have a factor VIII deficiency which might cause continuous bleeding in their muscles and joints. It can lead to severe menstrual bleeding in females and postpartum haemorrhage after childbirth. This accounts for 5% of the cases diagnosed.
Acquired VWD – This is usually the consequence of medications, being diagnosed with autoimmune disorders, lupus, cancer or heart disease among adults.
The following factor deficiencies are considered a rare inherited fibrinogen deficiency that has emerged only in the last 60 – 70 years:
Factor I – Factor I is also called fibrinogen deficiency, it is mostly found in newborns after circumcision when they bleed from the umbilical cord.
Factor II – Factor II is also called prothrombin deficiency. Though it is common among males and females, it is still a rare condition.
Factor V – Factor V is also called parahemophilia. Factor V deficiency is mainly marked by nose bleeds. Factor V deficiency is also denoted by excessive period bleeding and bruising.
Factor VII – Also called Alexander disease or proconvertin deficiency is usually identified at birth itself. It is characterised by bleeding from the central nervous system of the newborn.
Factor X – Factor X is also called Stuart-Prower factor deficiency. It has mild to severe symptoms characterised by intramuscular bleeding.
Factor XII – Factor XII is another rare type of bleeding disorder, where symptoms of bleeding are unusual. The patient usually has slower healing after a wound or injury but does not have typical bleeding disorder symptoms.
Platelets are special cells in the blood that act with red and white blood cells to form clots, among its other functions. Thrombasthenia is the condition where the platelet function is impaired, hence our body experiences abnormal bleeding.
Thrombasthenia is a rare, inherited clotting disorder that can be traced through genetic studies.
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When you think of a bleeding disorder characterised by excessive bleeding, you would imagine that the symptoms would be explicit and visible but not all the symptoms are such. The bleeding disorder symptom depends on the type of bleeding disorder as well. It should also be noted that not all patients with coagulopathy will experience all the bleeding disorder symptoms.
Frequent and easy bruising – Bruising can arise due to the inability of the body to form clots. The excessive bleeding from an injury is due to a deficiency of any of the proteins in the blood required for clotting. Unexplained bluish patches (bruises) on skin that may be painless or sometimes painful should not be ignored.
Haemorrhage into the joints – Also called hemarthrosis, it denotes bleeding in the joints like elbows, ankles and knees. Though this can happen after an injury, this is also typical of any type of bleeding disorder, specifically haemophilia symptoms, where the patient bleeds unprovoked.
Lots of blood loss from small cuts and wounds – The excessive bleeding from an injury is due to a deficiency of factors in blood like factor V deficiency, deficiency of platelets or other reasons which delay or prevent your body from clotting to lock in the blood.
Heavy menstrual bleeding – A significant symptom of bleeding disorders in females is prolonged menstrual bleeding. This is found to be caused by any of the Von Willebrand disease types, which causes impairment in blood clotting.
Symptoms of coagulopathy may be hard to diagnose, especially when it comes to bleeding disorders in females. This is because excessive bleeding may often be dismissed as normal.
However, here are some of the instances where bleeding disorder symptoms cannot be overlooked and may need immediate medical aid:
Heavy menstrual bleeding – Especially when the flow is longer or heavier than normal.
Longer bleeding after minor cuts – After a cut or injury, the blood usually clots or dries up. If the bleeding occurs longer than usual, visit the doctor.
Frequent and profuse nose bleeds – Nosebleeds can be caused by heat or injury to the nasal passage. However, if the bleeding lasts longer than normal and is triggered at random times, see the doctor.
Swollen and painful joints – Especially when your elbows or knees hurt when pressed. This could be internal bleeding.
Any signs of internal bleeding in the brain – Such as a throbbing headache, dizziness, blurred vision, loss of consciousness, slurred speech, sudden vision disturbances, etc.
Any injury with prolonged bleeding – If you are bleeding excessively from your wound, chances are, your body is facing difficulties forming a clot, which symbolises the presence of a bleeding disorder.
Another reason to be well-read about the different kinds of bleeding disorders is the possibilities of consequences it could lead to:
Bleeding in the brain
Bleeding in the joints
Internal bleeding in organs such as the intestine, stomach, throat, etc.
Damage to the joints from constant bleeding
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It is disheartening to see your loved ones get injured in the first place but when it is followed by excessive and uncontrolled bleeding, it could be harder to manage. This is precisely why understanding the causes of bleeding disorders will be an important step to acting proactively to find effective treatments for the same.
Here are some of the causes of bleeding disorders to check yourself for:
Genetic Predisposition – Having a parent or close family member suffering from a bleeding disorder increases the risk.
Clotting issues – This is one of the main causes of bleeding disorders. Coagulation factors are proteins in the blood that work towards forming a clot. The clot is a protective mechanism that seals off any injured vessel or area, to prevent excessive bleeding. A deficiency or abnormality in one or more clotting factors is mostly hereditary but can also be acquired in later stages in some cases.
Low RBC count – Red Blood Cells are one of the most integral components of blood. It is responsible for transporting oxygen to the rest of your body. A low RBC count can cause anaemia, which may lead to weakness, fatigue, brittle skin and a longer time to form clots.
Deficiency of vitamin K – Vitamin K is important for coagulation or the process of clot formation. A deficiency of vitamin K causes your body to not have sufficient proteins to form clots when you are injured. This may lead to excessive bleeding and weaker bones. Therefore, to manage the consequences that a deficiency of vitamin K causes, it is advisable to take medical help, especially in the form of vitamin K shots. Along with medication, including green leafy vegetables could help overcome the effects that deficiency of vitamin K causes.
Effects from certain medications such as anticoagulants – Anticoagulants are medications used to stop or slow down the process of clot formation. This is especially prescribed for those with complications from clotting disorders such as strokes, deep vein thrombosis, pulmonary embolism, etc.
However, the side effects of such anticoagulant medicines as Apixaban, Edoxaban, etc, is that it could lead to the inefficiency of our body to form clots in the long run. This is one of the leading causes of haemophilia A as well, as these medicines could lengthen the time it takes for clots to form.
Effect of liver disease – The liver is closely linked to blood clots. This is because the liver is the powerhouse of most of the factors that are responsible for clot formation as well as inhibition. The same is also one of the causes of platelet deficiency. Therefore, a bleeding disorder means that there could be a link to liver health as well, as liver damage mostly leads to problems with coagulation.
A Low number of platelets – A condition called thrombocytopenia.
Thrombocytopenia or platelet deficiency is caused by the following:
Cancer - especially leukaemia
Viral infections such as Dengue, Hepatitis C or HIV
Chemotherapy or radiation therapy
Trapped platelets in the spleen (due to any disorder of spleen)
Alcohol addiction
Autoimmune disorders
Bone marrow disorders
Idiopathic Thrombocytopenia (Low count of platelets due to no specific reason)
Many symptoms of bleeding and clotting disorders can be helpful to diagnose the condition at birth itself, although difficult to identify. This is possible through a genetic study. If the mother is a carrier of a recessive gene of haemophilia, the child could be diagnosed with any type of bleeding disorder even before birth and seek Haemophilia A treatment.
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Like any disorder, the bleeding disorder treatment is very much doable. With the right treatment, you can lead a relatively healthy lifestyle as well. Understanding the various bleeding disorder treatment options can help you make informed decisions before the procedure and even help you mentally prepare for them. Please keep in mind that the treatment depends on the type of bleeding disorder the patient is diagnosed with.
Patient history as well as a physical examination is a routine first step in the treatment process. The doctor gathers patient information regarding any family history of bleeding or related disorders. The sex of the patient is an important factor to consider as well, as women may notice and report more excessive bleeding than men.
A physical exam is done to identify any bruises or injuries. They also see if any other treatments and medicines have been used in place to treat the excessive bleeding.
Some of the questions you may be asked are:
Do you experience any unusual bleeding or bruises in any part of your body?
Does anyone in your family experience the same?
Has any part of your body, especially your joints felt sore or in pain?
How is your menstrual flow?
Do you experience any excessive bleeding post giving birth?
Does your head or vision feel fine?
Have you experienced any nosebleeds recently?
The complete blood count (CBC) test measures and understands the components that make up the blood – RBCs, WBCs and platelets. This is ideal for the diagnosis of bleeding disorders due to low platelet counts.
Platelets in our blood stick or clump together to form blood clots. The Platelet Aggregation test evaluates how well the platelets in our blood can coagulate. This is a test that can help determine the causes of platelet deficiency.
Also called Coagulation Factor Test. It tests the blood’s ability to clot, concerning the function of each of the factors (factor I, II, III, V, etc) efficiency in the clotting process. This test can help understand what type of bleeding disorder is present in the patient.
Some bleeding disorders, especially haemophilia, can be detected in the parents’ genes. The haemophilia gene is present in the X chromosome, which the mother could carry as a dominant or recessive gene. Genetic testing would help identify if the haemophilia gene is present in the child, to start the bleeding disorder treatment early.
Iron supplements – As bleeding disorders lead to excessive blood loss, iron supplements help pump more haemoglobin to the RBCs and increase their count. This helps in preventing anaemia and forming clots as well.
Blood transfusions – To compensate for the blood loss, an ideal blood type is transfused into the patient. Sometimes, just the blood proteins such as factor V or VIII are transfused to stimulate blood clotting in the patient. It is advisable to be informed about blood transfusions and initial requirements.
Plasma transfusions – Also called convalescent plasma therapy, this procedure is done to correct any plasma deficiencies in the blood so the blood clotting can be faster.
Factor replacement therapy – Just like the blood and plasma transfusion, certain deficient factors or clotting proteins in the blood (such as FVIII for Haemophilia A and FIX for type B) can be transfused to the patient to treat the deficiency.
Topical products – Also called hemostats, they are used post-surgery to stop excessive bleeding. It is also used as one of the haemophilia A treatments locally, to slow down the blood flow. Some of the topical products are collagens, gelatin sponges, thrombin preparations and sealants.
Nasal sprays – These are specially used for children to produce a homeostasis effect (to restore the deficiency factors in the blood). This is ideally used as a home medicine for sudden bleedings, once medically prescribed.
Surgery or immobilization – This is carefully carried out for those experiencing bleeding in their joints. This is only taken as a last resort to prevent total damage to the joints caused by continual bleeding.
The kind of treatment you or your child receives depends on the type of bleeding disorder diagnosed. Other factors that the medical professional might consider before deciding on the treatment plan for the child are:
Age of the child
The intensity of the condition – how normal is the clotting factor levels and how severe is the bleeding disorder condition?
Tolerance of the child to certain medical procedures and therapies
Prognosis – what will the condition develop into throughout the child’s lifespan?
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It is possible to live a relatively healthy and fulfilling life even if you are diagnosed with a bleeding disorder. This balance is within your control and all you need to do is supplement your medicines with some doable life changes.
Ongoing therapy – As soon as you get diagnosed with any specific type of bleeding disorder, your doctor or physician would prescribe an ongoing therapy to manage the condition and bring it under control. You must follow it well. It might help you to make medicine schedules, keep track of doctor appointments and even prepare diet charts, so you stay in full control of the condition.
Eating a balanced diet – Although there is no specific diet prescribed for those diagnosed with a bleeding disorder, you can make dietary changes to manage the complications from it. A typical symptom is weak and sore joints. Thus, eating Vitamin K, C rich foods and other heart healthy foods can help strengthen your joints and muscles as well. To compensate for the blood loss, you can also include iron-rich foods like dry fruits, peas, spinach, beans, etc.
Regular physiotherapy – For the same reason as a balanced diet, some benefits of exercise or physiotherapy can help strengthen your body to cope with excessive bleeding. Through this, you can lessen the damage to your joints and bones as well.
Prioritising your mental health – Having a bleeding disorder might make you tread a lot of activities you enjoy with care. This could be frustrating and often concerning to deal with. This is exactly why you need to improve mental and emotional healthy by practicing emotional first aid as thoroughly as your physical well-being. Some of the ways to do that is:
Joining a support group with people who are diagnosed with/have been through bleeding disorders.
Taking frequent therapy sessions to deal with the stress of being diagnosed with the disorder.
Volunteer with the elderly or the needy to boost your wellness and gain the confidence to handle your diagnosis as well.
Journal your day - how you felt, how you dealt with difficult situations, etc.
It could feel isolating to experience a bleeding disorder and to deal with it on a day-to-day basis. However, there is strength in the sense of knowing you’re not alone.
There are thousands, if not millions just like you to understand what you go through. Here are a few excerpts narrating real people to tell you their story of living with a bleeding disorder:
What Keerthi believes has helped Nikhil cope with his condition as a child:
Being in a situation where she feels helpless, here are the positives she was able to see:
Being diagnosed for haemophilia A across 4 generations, here is how Sherin finds her way to leading a happy life:
It is possible to balance a healthy lifestyle with any type of bleeding disorder you are diagnosed with. Being aware, proactive and following your medical advice carefully can help bring great control over your condition. It is also important to seek out support groups and find as many ways to stay engaged and informed about your lifestyle with a bleeding disorder.
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Although bleeding disorders in India is not as prevalent as it is in the US or Europe, the most common types of bleeding disorders found here in India are:
Apart from expert medical diagnosis, look out for any of the following symptoms:
Although you can watch for the symptoms of a bleeding disorder, it is advisable to take expert medical advice. A doctor would be able to identify the cause, intensity, course of action and treatment plans for your disorder.
Haemophilia and other bleeding disorders are manageable with the right treatment and precautions. Life-threatening factors include – internal bleeding in the skull, liver failure and blockage of airways in the internal organs. If not diagnosed and treated timely, these can lead to life-threatening complications.
A study reveals that although both of these types of haemophilia are manageable, type B is clinically not as severe as type A haemophilia. Moreover, there is a greater prevalence of those diagnosed with type A than B.
Although not completely possible, early detection can help you take precautionary steps to gain control of the disorder. Treatments such as factor replacement therapies and blood transfusion can help give your blood a healthier pool of proteins to form clots.
Bleeding disorders are diagnosed through some of the following tests:
Inherited Bleeding Disorders may not be completely curable by treatments. However, treatments, medications and lifestyle changes can drastically bring down the threat of the disease and make it more manageable to live with.
Yes, in India, people with blood disorders such as thalassemia, hemophilia and sickle cell disease are recognized as ‘persons with disabilities under the Rights of Persons with Disabilities (RPwD) Act, 2016.
Although some bleeding disorders may be lifelong, treatments can decrease the severity of the bleeding disorder over the years. There have also been recent developments in research to genetic studies that offer sustained correction of Haemophilia.
Internal bleeding in the joints or to vital organs can be painful. External bleeding left untreated can also cause pain to the muscles, bones and joints. However, this pain also acts as an early warning sign to get diagnosed and seek medical help right away.
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