Hemophilia is a rare disorder in which the blood doesn't clot in the typical way because it doesn't have enough blood-clotting proteins (clotting factors). If you have hemophilia, you might bleed for a longer time after an injury than you would if your blood clotted properly.
Small cuts usually aren't much of a problem. If you have a severe form of the condition, the main concern is bleeding inside your body, especially in your knees, ankles and elbows. Internal bleeding can damage your organs and tissues and be life-threatening.
Hemophilia is almost always a genetic disorder. Treatment includes regular replacement of the specific clotting factor that is reduced. Newer therapies that don't contain clotting factors also are being used.
Signs and symptoms of hemophilia vary, depending on your level of clotting factors. If your clotting-factor level is mildly reduced, you might bleed only after surgery or trauma. If your deficiency is severe, you can bleed easily for seemingly no reason.
SIGNS AND SYMPTOMS OF SPONTANEOUS BLEEDING INCLUDE:
- Unexplained and excessive bleeding from cuts or injuries, or after surgery or dental work
- Many large or deep bruises
- Unusual bleeding after vaccinations
- Pain, swelling or tightness in your joints
- Blood in your urine or stool
- Nosebleeds without a known cause
- In infants, unexplained irritability.
- Bleeding into the brain
A simple bump on the head can cause bleeding into the brain for some people who have severe hemophilia. This rarely happens, but it's one of the most serious complications that can occur.
SIGNS AND SYMPTOMS INCLUDE:
- Painful, prolonged headache
- Repeated vomiting
- Sleepiness or lethargy
- Double vision
- Sudden weakness or clumsiness
- Convulsions or seizures.
Severe cases of hemophilia usually are diagnosed within the first year of life. Mild forms might not be apparent until adulthood. Some people learn they have hemophilia after they bleed excessively during a surgical procedure.
Clotting-factor tests can reveal a clotting-factor deficiency and determine how severe the hemophilia is.
For people with a family history of hemophilia, genetic testing might be used to identify carriers to make informed decisions about becoming pregnant.
It's also possible to determine during pregnancy if the fetus is affected by hemophilia. However, the testing poses some risks to the fetus.
Hemophilia is a bleeding disorder that slows the blood clotting process. People with this condition experience prolonged bleeding or oozing following an injury, surgery, or having a tooth pulled. In severe cases of hemophilia, continuous bleeding occurs after minor trauma or even when there is no obvious injury (sometimes called spontaneous bleeding). Serious complications can result from bleeding into the joints, muscles, brain, or other internal organs. Milder forms of hemophilia do not necessarily involve spontaneous bleeding, and the condition may not become apparent until abnormal bleeding occurs following surgery or a serious injury.
The major types of this condition are hemophilia A (also known as classic hemophilia or factor VIII deficiency) and hemophilia B (also known as Christmas disease or factor IX deficiency). Although the two types have very similar signs and symptoms, they are caused by variants (also known as mutations) in different genes. People with an unusual form of hemophilia B, known as hemophilia B Leyden, experience episodes of excessive bleeding in childhood but have few bleeding problems after puberty.
The two major forms of hemophilia occur much more commonly in males than in females. Hemophilia A is the most common type of the condition; 1 in 4,000 to 1 in 5,000 males worldwide are born with this disorder. Hemophilia B occurs in approximately 1 in 20,000 newborn males worldwide.
Variants in the F8 gene cause hemophilia A, while variants in the F9 gene cause hemophilia B. The F8 gene provides instructions for making a protein called coagulation factor VIII. A related protein, coagulation factor IX, is produced from the F9 gene. Coagulation factors are proteins that work together in the blood clotting process. After an injury, blood clots protect the body by sealing off damaged blood vessels and preventing excessive blood loss.
Variants in the F8 or F9 gene lead to the production of an abnormal version of coagulation factor VIII or coagulation factor IX, or reduce the amount of one of these proteins. The altered or missing protein cannot participate effectively in the blood clotting process. As a result, blood clots cannot form properly in response to injury. These problems with blood clotting lead to continuous bleeding that can be difficult to control. The variants that cause severe hemophilia almost completely eliminate the activity of coagulation factor VIII or coagulation factor IX. The variants involved in mild and moderate hemophilia reduce but do not eliminate the activity of one of these proteins.
Another form of the disorder, known as acquired hemophilia, is not caused by inherited gene variants. This rare condition is characterized by abnormal bleeding into the skin, muscles, or other soft tissues, usually beginning in adulthood. Acquired hemophilia results when the body makes specialized proteins called autoantibodies that attack and disable coagulation factor VIII. The production of autoantibodies is sometimes associated with pregnancy, immune system disorders, cancer, or allergic reactions to certain drugs. In about half of cases, the cause of acquired hemophilia is unknown.
Hemophilia A and hemophilia B are inherited in an X-linked recessive pattern. The genes associated with these conditions are located on the X chromosome, which is one of the two sex chromosomes. In males (who have only one X chromosome), one altered copy of the gene in each cell is sufficient to cause the condition. A characteristic of X-linked inheritance is that fathers cannot pass X-linked traits to their sons.
In females (who have two X chromosomes), a variant would usually have to occur in both copies of the gene to cause the disorder. However, in some instances, one altered copy of the F8 or F9 gene is sufficient, because the X chromosome with the normal copy of the gene is turned off through a process called X-inactivation. X-inactivation occurs early in embryonic development in females. Through this process, one of the two X chromosomes is permanently turned off (inactivated) in somatic cells (cells other than egg and sperm cells). X-inactivation ensures that females, like males, have only one active copy of the X chromosome in each body cell.
Usually X-inactivation occurs randomly, such that each X chromosome is active in about half of the body cells. Sometimes X-inactivation is not random, and one X chromosome is active in more than half of cells. When X-inactivation does not occur randomly, it is called skewed X-inactivation.
In many females with a variant in one copy of the F8 or F9 gene, X-inactivation is random and the chromosome with the normal copy of the gene is turned off in about half of cells. These individuals have about half the usual amount of coagulation factor VIII or coagulation factor IX, which is generally enough for normal clotting. However, in some females with an F8 or F9 gene variant, X-inactivation is skewed, and the chromosome with the normal copy of the gene is turned off in more than half of cells. These individuals can have less coagulation factor VIII or coagulation factor IX than usual and are at risk of abnormal bleeding.
LIFESTYLE AND HOME REMEDIES: -
To avoid excessive bleeding and protect your joints:
Exercise regularly. Activities such as swimming, bicycle riding and walking can build muscles while protecting joints. Contact sports — such as football, hockey or wrestling — are not safe for people with hemophilia.
Avoid certain pain medications. Drugs that can make bleeding worse include aspirin and ibuprofen (Advil, Motrin IB, others). Instead, use acetaminophen (Tylenol, others), which is a safer alternative for mild pain relief.
Blood-thinning medications should be avoided. Medications that prevent blood from clotting include heparin, warfarin (Jantoven), clopidogrel (Plavix), prasugrel (Effient), ticagrelor (Brilinta), rivaroxaban (Xarelto), apixaban (Eliquis), edoxaban (Savaysa) and dabigatran (Pradaxa).
Practice good dental hygiene. The goal is to prevent tooth and gum disease, which can lead to excessive bleeding.
Get vaccinations. People with hemophilia should receive recommended vaccinations at the appropriate ages, as well as hepatitis A and B. Requesting use of the smallest gauge needle and having pressure or ice applied for 3 to 5 minutes after the injection can reduce the risk of bleeding.
Protect your child from injuries that could cause bleeding. Kneepads, elbow pads, helmets and safety belts all help prevent injuries from falls and other accidents. Keep your home free of furniture with sharp corners.
To help you and your child cope with hemophilia:
A medical alert bracelet can be helpful. This lets medical personnel know that you or your child has hemophilia, and the type of clotting factor that's best in case of an emergency.
Talking with a counselor. Striking the right balance between keeping your child safe and encouraging as much activity as possible can be tricky. A social worker or therapist with knowledge of hemophilia can help identify the least amount of limitations your child needs.
Let people know. Be sure to tell anyone who will be taking care of your child — babysitters, child care workers, relatives, friends and teachers — about your child's condition. If your child plays noncontact sports, be sure to tell coaches, too.
HAEMOPHILIA AND EFFECTIVENESS OF HOMOEOPATHY ON IT: -
#) Haemophilia is one of the most important blood disorders prevailing in our society.
#) It is a hereditary Disorder of coagulation of blood due to deficiency of Antihaemophilic Globulin in blood resulting in haemmorrhagic tendency.
#) Haemophilia A is due to disorder in level of Factor 8.
#) Haemophilia B is due to deficiency of Factor 9.
#) The persons suffering from the disease experience it's manifestation in Infancy.
#) Males are usually affected (There are many Homoeopathic medicines for treatment of male diseases
#) There is excessive bleeding from nose, mouth, alimentary tract, urinary tract etc.
#) Specific factor 8 assay can confirm its diagnosis.
#) Homoeopathy has shown immense help in treating Haemophilic patients.
#)There are various Homoeopathic medicines which have been useful in controlling bleeding tendencies in Haemophilia.
HOMOEOPATHIC MEDICINES FOR HAEMOPHILIA: -
Certain studies have shown efficacy of homoeopathy for the management of Haemophilia. There are various Homeopathic remedies, which prove to be useful in controlling the bleeding tendencies in Haemophilia. These homeopathic medicines have an affinity to act on the blood vessels, capillaries, and helps in accelerating the healing mechanism.
Homeopathy in the treatment of Haemophilia can be given as a supportive line of treatment especially in long standing cases.
The medicines that can be thought of use are:-