What are treatments for hemophilia?

he mainstay of treatment is replacement of the blood clotting factors. Clotting factor concentrates can be purified from human donor blood or made in the laboratory using methods that do not use donor blood. This type of therapy is known as replacement therapy. Clotting factor replacement therapy is carried out by infusing the clotting factor concentrates into a vein, much like a blood transfusion. This type of therapy can be administered at home with proper instruction and training.

Depending upon the severity of the condition, replacement therapy may be carried out on an as-needed basis (called demand therapy) or on a regular basis to prevent bleeding episodes (known as prophylactic therapy).

People who have mild cases of hemophilia A are sometimes treated with the drug desmopressin, also known as DDAVP. This drug stimulates release of more clotting factor by the body. It is administered either slowly through the intravenous route (IV) or occasionally, in nasal spray form.

Pain relievers may be prescribed for symptom relief, but pain relievers other than aspirin or non-steroidal anti-inflammatory medications (such as naproxen, ibuprofen) must be used, since these types of drugs further inhibit the blood's ability to clot. Acetaminophen (Tylenol and others) is often given for pain relief

What are the signs and symptoms of hemophilia?

Hemophilia can vary in its severity, depending upon the particular type of mutation (genetic defect). The degree of symptoms depends upon the levels of the affected clotting factor. Severe disease is defined as <1% factor activity, 1% to 5% factor activity is moderate disease, and greater than 5% factor activity constitutes mild disease. The extent of bleeding is dependent upon the severity (the amount of factor activity) and is similar for hemophilia A and B.

With severe hemophilia (A or B), bleeding begins at an early age and may occur spontaneously. Those with mild hemophilia may only bleed excessively in response to injury or trauma. Female carriers of hemophilia have variable degrees of factor activity; some may have near normal levels and do not show any bleeding tendencies, while some may have less than the predicted 50% reduction and may bleed more often than non-carrier females.

In severe hemophilia, bleeding episodes usually begin within the first 2 years of life. Heavy bleeding after circumcision in males is sometimes the first sign of the condition. Symptoms may develop later in those with moderate or mild disease. The bleeding of hemophilia can occur anywhere in the body. Common sites for bleeding are the joints, muscles, and gastrointestinal tract. Specific sites and types of bleeding are discussed below.

• Hemarthrosis (bleeding into the joints) is characteristic of hemophilia. The knees and ankles are most often affected. The bleeding causes distension of the joint spaces, significant pain, and over time, can be disfiguring. Over time, joint destruction occurs, and joint replacement surgeries can be required.
  
• Bleeding into the muscles may occur with hematoma formation (compartment syndrome).
  
• Bleeding from the mouth or nosebleeds may occur. Bleeding after dental procedures is common, and oozing of blood from the gums may occur in young children when new teeth are erupting.
  
• Bleeding from the gastrointestinal tract can lead to blood in the stool.
  
• Bleeding from the urinary tract can lead to blood in the urine(hematuria).
  
• Intracranial hemorrhage (bleeding into the brain or skull) can lead to symptoms such as nausea, vomiting, and/or lethargy.
  
• Increased bleeding after surgery or trauma is characteristic of hemophilia.

What causes hemophilia?

As mentioned above, hemophilia is caused by a genetic mutation. The mutations involve genes that code for proteins that are essential in the blood clotting process. The bleeding symptoms arise because blood clotting is impaired.

The process of blood clotting involves a series of complex mechanisms, usually involving 13 different proteins classically termed I through XIII and written with Roman numerals. If the lining of the blood vessels becomes damaged, platelets are recruited to the injured area to form an initial plug. These activated platelets release chemicals that start the clotting cascade, activating a series of 13 proteins known as clotting factors. Ultimately, fibrin is formed, the protein that crosslinks with itself to form a mesh that makes up the final blood clot. The protein involved with hemophilia A is factor VIII (factor 8) and with hemophilia B is factor IX (factor 9).

What is haemophilia?

Haemophilia   is from the Greek haima  'blood' and philia  'love'  is a group of hereditary genetic disorders that impair the body's ability to control blood clotting or coagulation, which is used to stop bleeding when ablood vessel is broken. Haemophilia A (clotting factor VIII deficiency) is the most common form of the disorder, occurring at about 1 in 5,000–10,000 male births.Haemophilia B (factor IX deficiency) occurs at about 1 in about 20,000–34,000 male births.

Like most recessive sex-linked, X chromosome disorders, haemophilia is more likely to occur in males than females. This is because females have two X chromosomes while males have only one, so the defective gene is guaranteed to manifest in any male who carries it. Because females have two X chromosomes and haemophilia is rare, the chance of a female having two defective copies of the gene is very low, so females are almost exclusively asymptomatic carriers of the disorder. Female carriers can inherit the defective gene from either their mother or father, or it may be a new mutation. Only under rare circumstances do females actually have haemophilia.

Haemophilia lowers blood plasma clotting factor levels of the coagulation factors needed for a normal clotting process. Thus when a blood vessel is injured, a temporary scab does form, but the missing coagulation factors prevent fibrin formation, which is necessary to maintain the blood clot. A haemophiliac does not bleed more intensely than a person without it, but can bleed for a much longer time. In severe haemophiliacs even a minor injury can result in blood loss lasting days or weeks, or even never healing completely. In areas such as the brain or inside joints, this can be fatal or permanently debilitating.
Deficiency in coagulation factor VIII is the most common cause of haemophilia.

viral skin diseas

Shingles: An acute infection caused by the herpes zoster virus, the same virus as causes chickenpox. Shingles is most common after the age of 50 and the risk rises with advancing age. Shingles occurs because of exposure to chickenpox or reactivation of the herpes zoster virus. The virus remains latent (dormant) in nerve roots for many years following chickenpox.

Shingles is an extraordinarily painful condition that involves inflammation of sensory nerves. It causes numbness, itching or pain followed by the appearance of clusters of littles blisters in a strip pattern on one side of the body. The pain can persist for weeks, months or years after the rash heals and is then known as post-herpetic neuralgia.

People with shingles are contagious to persons who have not had chickenpox and can catch chickenpox from close contact with a person who has shingles. Treatment includes antiviral medication and pain medication.

The term shingles has nothing to do with a shingle on a roof or the small signboard outside the office of a doctor but is derived from the Latin cingulum meaning girdle, the idea being that shingles often girdles part of the body.

bacterial skin diseas

Rocky Mountain spotted fever (RMSF): An acute febrile (feverish) disease initially recognized in the Rocky Mountain states, caused by Rickettsia rickettsii transmitted by hard-shelled (ixodid) ticks. Occurs only in the Western Hemisphere. Anyone frequenting tick-infested areas is at risk for RMSF.

The onset of symptoms is abrupt with headache, high fever, chills,muscle pain. and then a rash. The rickettsiae grow within damaged cells lining blood vessels which may become blocked by clots. Blood vessel inflammation (vasculitis) is widespread.

Early recognition of RMSF and prompt antibiotic treatment is important in reducing mortality.

The first person to describe the disease was an ear, nose and throat specialist, Edward Ernest Maxey. Maxey reported the disease in 1899. Seven years later, a pathologist named Howard Taylor Ricketts showed that it was transmitted by a tick bite. The agent that causes the disease was named for him -- Rickettsia rickettsii.

Rocky Mountain spotted fever is also called spotted fever, tick fever, and tick typhus.

Allergic skin disorder

Eczema: A particular type of inflammatory reaction of the skin in which there are typically vesicles (tiny blister-like raised areas) in the first stage followed by erythema (reddening), edema (swelling), papules (bumps), and crusting of the skin followed, finally, by lichenification (thickening) and scaling of the skin. Eczema characteristically causes itching and burning of the skin.

Eczema, which is also called atopic dermatitis, is a very common skin problem. It may start in infancy, later in childhood, or in adulthood. Once it gets underway, it tends not to go quickly away.

There are numerous types of eczema, including:

·                       Atopic dermatitis -- a chronic skin disease characterized by itchy, inflamed skin

·                       Contact eczema -- a localized reaction that includes redness, itching, and burning where the skin has come into contact with an allergen (an allergy-causing substance) or with an irritant such as an acid, a cleaning agent, or other chemical

·                       Allergic contact eczema -- a red, itchy, weepy reaction where the skin has come into contact with a substance that the immune system recognizes as foreign, such as poison ivy or certain preservatives in creams and lotions

·                       Seborrheic eczema -- a form of skin inflammation of unknown cause that presents as yellowish, oily, scaly patches of skin on the scalp, face, and occasionally other parts of the body

·                       Nummular eczema -- coin-shaped patches of irritated skin - most commonly on the arms, back, buttocks, and lower legs - that may be crusted, scaling, and extremely itchy

·                       Neurodermatitis -- scaly patches of skin on the head, lower legs, wrists, or forearms caused by a localized itch (such as an insect bite) that becomes intensely irritated when scratched

·                       Stasis dermatitis -- a skin irritation on the lower legs, generally related to circulatory problems

Dyshidrotic eczema -- irritation of the skin on the palms of hands and soles of the feet characterized by clear, deep blisters that itch and burn.

MEDICINE FOR ALLERGY 

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Medicine

Medicine is the science and art of healing. It encompasses a variety of health carepractices evolved to maintain and restore health by the prevention and treatment of  llness.

Contemporary medicine applies health science, biomedical research, and medical technology to diagnose and treat injury and disease, typically through medication orsurgery, but also through therapies as diverse as psychotherapy, external splints & traction, prostheses, biologics, ionizing radiation and others. The word medicine is derived from the Latin ars medicina, meaning the art of healing.

Though medical technology and clinical expertise are pivotal to contemporary medicine, successful face-to-face relief of actual suffering continues to require the application of ordinary human feeling and compassion, known in English as bedside manner.