DRUGS & CONDITIONS

WHAT IS HEMOPHILIA?

Hemophilia is a genetic disorder of the blood clotting system. Over 50 substances are needed for blood to clot. If one of the substances is missing or if the amount of the substance is too low, the person may bleed spontaneously or bleed excessively when trauma occurs.

There are different types of hemophilia, depending on which factor is missing or too low. Problems with Factor VIII are most common, affecting 90% of patients. Problems with Factor IX affect 9% of patients with hemophilia. Problems also occur with factors XI, X, VII and V but these cases are rare. All of the rare cases combined account for only 1% of patients with hemophilia.

Hemophilia A was the first recognized form of hemophilia. Hemophilia A is also known as Factor VIII Deficiency and Classical Hemophilia. Patients with Hemophilia A do not have enough Factor VIII.

Hemophilia B is also known as Factor IX Deficiency. Another name for Hemophilia B is Christmas Disease, named for the patient in whom the problem was first defined. Patients with Hemophilia B do not have enough Factor IX.

All types of hemophilia are present in either a severe, moderate or mild form. Patients with severe hemophilia have less than 1% of the relevant factor. Patients with moderate hemophilia have between 1% and 5% of the factor. Patients with mild hemophilia have between 5% and 40% of the factor.

About 75% of patients with hemophilia have the severe form of the disorder. These patients have excessive bleeding after dental work, surgery and trauma. Patients with severe hemophilia have spontaneous bleeding or bleeding when no noticeable trauma has occurred. The average number of bleeds per year is 30. Spontaneous bleeding usually affects major joints such as the knees.

Patients with moderate hemophilia account for 20% of all cases. These patients are at high risk for excessive bleeding after surgery or trauma. Spontaneous bleeding rarely occurs in patients with moderate hemophilia.

Mild hemophilia accounts for about 5% of all hemophilia cases. These patients have excessive bleeding with severe trauma or surgery but may not experience any other problems or symptoms of the disorder. Many patients with mild hemophilia do not realize they have the disorder until bleeding occurs due to a major event such as an accident or surgery.

All forms and severities of hemophilia can be treated. Proper treatment reduces the risk of serious complications and allows patients to lead a full and productive life.


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

WHO GETS HEMOPHILIA?

Hemophilia occurs when the gene to produce clotting factor does not work correctly. Without the proper amount of clotting factor, bleeding occurs easily.

Hemophilia is an inherited disorder in about two thirds of the cases. About one third of the cases of hemophilia occur due to spontaneous changes in the gene or mutations. The presence of a mutated gene may not be noted until a woman with no family history of hemophilia has a son with the disorder.

The gene for hemophilia is carried on the X chromosome. The gene for hemophilia is also recessive. This is why hemophilia is referred to as an X-linked recessive disorder. If there is not a normal gene present to offset the defective, recessive gene, the disorder will be present. Whether or not a child will have hemophilia or be a carrier for the disorder depends on the status of the mother and of the father. The figures below show how this type of disorder is inherited.

Males have an X chromosome and an Y chromosome. The X chromosome comes from the mother and the Y chromosome comes from the father. If the mother has a defective gene, the son's chance of having hemophilia is 50%, depending on which X chromosome is inherited. A son cannot inherit the disorder from his father, even if the father has hemophilia.

Females have two X chromosomes. One X chromosome comes from the mother and one comes from the father. All daughters of men with hemophilia will be carriers. Carriers rarely have the disorder but are able to pass the defective gene to their offspring. The daughter also has a 50% chance of becoming a carrier if the mother is a carrier, depending on which X chromosome is inherited.

In most cases, one of the daughter's two X chromosomes is normal. Most females with a gene for hemophilia do not have symptoms of the disorder because a normal gene offsets any problems caused by the one that is defective. In some cases, however, the normal gene cannot offset the problem completely and the female will have low factor levels. When factor levels are low, the female can show symptoms of hemophilia such as excessive menstrual bleeding and excessive bleeding after childbirth, surgery and dental work.

Hemophilia occurs in about 1 of every 7,500 males. Of these, about 90% of cases are Factor VIII Deficiency (Hemophilia A) and 9% are Factor IX Deficiency (Hemophilia B). About 1% of hemophilia cases is due to deficiencies of Factor XI, X, VII or V. The pattern of inheritance for these factor deficiencies varies.


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

WHAT IS THE CAUSE OF HEMOPHILIA?

Hemophilia occurs when one of the factors needed for blood to clot is missing or the amount present is too low for normal clotting to occur. Factor VIII is missing or too low in about 90% of hemophilia cases. Factor IX is deficient in about 9% of cases. Factor XI, X, VII or V is missing or too low in the remaining 1% of hemophilia cases.

The process of blood clotting starts the moment damage to the blood vessel wall occurs. Platelets begin collecting at the damaged site. As more platelets collect, a plug forms in the hole to stop bleeding. Although the platelet plug can hold for a while, without a "glue" to permanently hold the platelets together, the plug breaks apart and bleeding begins again. The "glue" that holds the plug together is called fibrin.

Fibrin is the end product of two blood clotting pathways. Each pathway proceeds in a cascade or stepwise fashion with the product in one step acting as the trigger for the next step.

The first pathway (extrinsic system) is triggered when damage causes a substance called tissue thromboplastin to be released from the tissue. The second pathway (intrinsic system) is triggered when the blood comes into contact with the blood vessel wall. The steps that lead to the formation of fibrin requires factors VIII and IX.

The body produces the different clotting factors based on the instructions from a gene. In hemophilia, the gene that causes factor to be produced is defective. In most cases of hemophilia, the defective gene is passed from parent to child. In cases where there is no family history of the disorder, hemophilia occurs due to a change or mutation of the gene. In such cases, the mutation may only be discovered after the birth of a son with hemophilia.


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

WHAT ARE THE SYMPTOMS?

Hemophilia is a disorder of the blood clotting system. Because blood does not clot when it should, patients experience frequent and excessive bleeding. The degree to which bleeding occurs depends in part on the severity of the disorder. Patients can have severe, moderate or mild hemophilia.

A hallmark of severe hemophilia is spontaneous bleeding. In these cases, bleeding occurs without any recognizable trauma. Bleeding can occur in any part of the body. However, spontaneous bleeds are unusual in the joints of the fingers, wrists, feet and spine. Spontaneous bleeds are most common in the knees, ankles, elbows and shoulders. Bleeding into the joints is called hemarthrosis.

Hemarthrosis usually begins after the child begins to walk. As the bleeding begins, the person may experience a warmth or tingling in the joint. As bleeding progresses, there is usually a feeling of stiffness, fullness, and pain. The joint swells and may be warm and tender. Bleeding into the joint limits the ability to move the joint. If not treated, hemarthrosis can cause chronic joint problems.

Hemophilia can cause bleeding from circumcision. Bruising often occurs after injections in infants. As the child becomes more active, excessive bruising occurs. Excessive bleeding also occurs when teeth are pulled or other trauma occurs.

Symptoms of moderate hemophilia are the same as those for severe hemophilia with one exception. Spontaneous bleeding is rare in moderate hemophilia. When bleeding occurs, the person is usually able to identify some trauma that caused the event.

Patients with mild hemophilia have excessive bleeding with trauma or surgery. However, these patients may not experience any other problems or symptoms of the disorder. Many patients with mild hemophilia may not realize they have the disorder until bleeding occurs due to a major event such as an accident or surgery.

The difference between symptoms for Factor VIII Deficiency (Hemophilia A) and Factor IX Deficiency (Hemophilia B) is the degree of severity. Symptoms are often milder for Factor IX Deficiency, due in part to the severity of the disorder. Severe Factor IX Deficiency is less common. Many patients with Factor IX deficiency do not have symptoms until stressed by surgery or trauma.

The goal of treatment for hemophilia is to prevent and/or reduce the frequency of symptoms. Increasing factor levels to at least 2% of normal can cause symptoms of severe hemophilia to improve to the level of moderate or even mild hemophilia. This can be achieved with regular preventive infusions of factor.


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

HOW IS HEMOPHILIA DIAGNOSED?

The diagnosis of hemophilia is made through a series of tests on a sample of the patient's blood. The pattern of positive and negative results from different tests will diagnose the presence of hemophilia as well as the type. The tests listed below are used to diagnose hemophilia.

Platelet count: This test counts the number of blood platelets. This test is normal for patients with hemophilia.

Activated partial thromboplastin time (APTT): This test diagnoses problems with Factor VIII and Factor IX. Almost 100% of patients with severe and moderate Factor VIII deficiency can be diagnosed with an APTT. Diagnosis of mild cases varies depending on the type of materials used in testing the blood sample. Most carriers cannot be diagnosed with an APTT. The test measures the length of time that it takes for a blood clot to form. The activated partial thromboplastin time is longer than normal for patients with Factor VIII Deficiency (Hemophilia A) and Factor IX Deficiency (Hemophilia B).

Factor assay: This is the most exact test to diagnose the type of hemophilia. A factor assay can distinguish between a Factor VIII deficiency and a Factor IX deficiency. Adding normal plasma to the patient's plasma will correct the abnormal APTT test result for Factor IX deficiency. Adding normal plasma to the patient's plasma will correct the abnormal APTT test result for Factor VIII deficiency.


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

WHAT CAN I EXPECT OVER TIME WITH HEMOPHILIA?

What you can expect over time with hemophilia depends on many factors. The severity of hemophilia, treatment, and current health status can affect the future for a patient with this disorder.

Patients with mild hemophilia are less likely to have serious problems related to the disorder. However, severe hemophilia can be improved to the level of moderate or even mild hemophilia. Increasing factor levels to at least 2% of normal can significantly improve severe hemophilia.

If proper treatment is not used, hemophilia can cause chronic problems such as arthritis. However, research has shown that patients who use preventative treatment have fewer problems related to hemophilia. Starting treatment before chronic problems can begin lessens the risks of crippling and bleeding into the joints and muscles.

Many patients with hemophilia fear bleeding that might occur with dental care. These patients often fail to take care of their gums and teeth. As a result, they have more dental problems, which can create other problems. Taking care of the gums and teeth can reduce the risk of future dental problems and the risks and pain they can cause.

Many patients who received blood products before 1985 were infected with the AIDS and hepatitis viruses. These diseases do not have a cure at this time and can affect the patient's life course. Since 1985, the safety and purity of products for treating hemophilia has significantly improved. For this reason, patients without other blood-borne diseases can have a normal life expectancy.

Hemophilia is a chronic disorder. This means that there is no cure and patients must deal with it throughout their lives.

Hemophilia can be in control of the patient's life or the patient can be in control of the hemophilia. Current treatment approaches allow patients to lead full and meaningful lives. Hemophilia treatment centers are designed to help patients with hemophilia address issues in all aspects of life.

Using services such as counseling to help manage problems related to hemophilia is not a sign of weakness. Following your doctor's advice and using needed services is a sign that you intend to control the hemophilia and its impact.


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

WHAT IS THE TREATMENT OF HEMOPHILIA?

Hemophilia is treated by providing the missing or low factor. The treatment schedule depends in part on the severity of the disorder and the age of the patient.

Prophylactic treatment, or treatment to prevent problems, is often used for children and some adults. Usually, patients with Hemophilia A receive prophylactic treatment with Factor VIII three or four times each week. Patients with Hemophilia B usually receive Factor IX two or three times each week. Patients who are not receiving prophylactic treatment are given factor on an as needed basis.

Factor products are labeled in terms of international units. "Unit" refers to a standard in which 1 IU is the amount of factor in 1 milliliter of fresh pooled plasma. Patients use a prescribed number of units of factor per kilogram of their body weight.

There are many factor replacement products. The products differ based on the purity and the source of factor. Purified products were first available in the 1960's. Partially purified products became available as concentrates for reconstitution in the 1970's. Recombinant products became available in the early 1990's.

Since 1985, the safety and purity of products for treating hemophilia has significantly improved. For this reason, patients without other blood-borne illnesses can have a normal life expectancy. Treatment that is begun and maintained appropriately can also reduce the risk of other problems associated with the disorder and allow for a higher quality of life.

Treatment Products

Factor concentrates are made by pooling thousands of units of plasma. The process begins by freezing bags of plasma. When the plasma is thawed, factor remains as a solid. The solid substance is separated and then pooled. Products then go through a purification process to reduce the risk of transmitting a virus. The method used for purification varies by manufacturer. These products provide the least expensive form of treatment. However, many patients believe the risk of a viral infection is still high with factor concentrates. These patients often choose one of the other forms of hemophilia treatment products.

Highly purified monoclonal plasma derived factors provide ultra-pure concentrates of factor. Like factor concentrates, these products are produced from human plasma. However, to produce these products, the plasma goes through a complex purification process. After this purification step is complete, the product goes through another purification process to further reduce the risk of transmitting a virus. Highly purified products are more expensive than factor concentrates.

Recombinant factor is produced by using production sources such as Chinese hamster ovary cells or baby hamster kidney cells. The gene for producing factor is inserted into these cells and the factor is manufactured within the cells. Factor is obtained in cell cultures and then purified. The product is provided as a powder. The powder is mixed with a special liquid just before it is used. The risk of viral infection with these products is theoretical, due to the manufacturing process. However, recombinant factor products are the most expensive factor product.

Porcine factor products do not transmit human viruses and are screened for any porcine viruses. These products may need to be used by patients who have inhibitors to human factor products.


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

MONITORING HEMOPHILIA: WHEN SHOULD I CALL THE DOCTOR?

Hemophilia can be challenging for both patients and their families. Many problems, however, can be resolved or at least improved with the help of a healthcare professional. Generally speaking, it's a good idea to talk to a nurse or doctor about any aspect of hemophilia that causes unusual discomfort or concern. Many times, a simple suggestion can help make patients more comfortable. Other times, prompt medical attention is needed. Whenever a bleed is suspected, infuse factor as soon as possible, even before seeking medical attention.

Seek medical advice when patients experience any of these problems:

Muscle Pain

Muscle pain, swelling, warmth or tightness can be evidence of a muscle bleed. If swelling puts pressure on a nerve or blood vessel, symptoms may also include tingling, weakness or muscle spasms. Muscle bleeds are more likely in the calf, thigh, groin and forearm muscles. Muscle bleeds usually occur as the result of major or minor trauma.

After major trauma, factor should be administered right away. The muscle should be immobilized. Ice should be applied, but not directly to the skin. Ice packs should be wrapped in a towel to prevent ice burns. Large muscle bleeds resolve slowly. In these cases, factor may need to be infused over a period of several days. Bleeds from minor traumas are more common and resolve more quickly.

Head or Neck Trauma

Trauma to the head or neck presents special risks for people with hemophilia. If not properly treated, serious problems may result. Head or neck trauma should always be regarded as life threatening and the injured person should be taken to the emergency room. Factor should be infused as soon as possible.

Bleeding in the brain may produce headache, confusion, slurred speech, weakness, nausea or vomiting. Symptoms, however, do not always appear at once. Untreated intracranial bleeding can result in death or permanent disability in mental function, physical movement or seizure disorder. Bleeding in the neck should be suspected if the patient experiences swelling, shortness or breath or difficulty swallowing.

Abdominal Trauma

A heavy blow to the abdomen can be serious for a person with hemophilia. Internal bleeding should be suspected if the patient is experiencing pain, lightheadedness or fainting. Following abdominal trauma, factor should be infused ASAP and the patient should immediately be seen in a hospital emergency room.

Joint Pain

Joint pain could be a symptom of hemarthrosis--bleeding into a joint. If the joint is painful, tingling, swollen or hard to move, hemarthrosis is likely. Some patients also experience a sense of anxiety or restlessness. The more bleeding that occurs, the greater the pain is likely to become. Joints most prone to bleeding are the knees, ankles, elbows, hips and shoulders.

Bleeding into a joint is treated with factor, rest and pain medication, if needed. These treatments should be given promptly. If hemarthrosis happens often, serious problems can result. Preventative measures should be taken. Repeated problems should be evaluated by a doctor in light of how often factor is given.

Depression

Depression is a common in patients with chronic illness. Both adults and children may get depressed. People with a chronic illness like hemophilia have a 25-33% chance of developing depression.

Symptoms usually include a loss of interest in daily activities and/or feeling great sadness. Other symptoms might include:

The good news is that depression is a very treatable illness. Over 80 percent of depressed people improve when treated. Depression may be treated with medication, psychotherapy, or both.


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

MINOR BLEEDS IN HEMOPHILIA

Bleeding occurs in hemophilia because of poor clot formation. Although large volumes of blood are not lost quickly, prolonged oozing can lead to the loss of a large volume of blood over time. Therefore, patients with hemophilia should take steps to stop seemingly minor bleeding.

Bruises

A hematoma or bruise occurs when blood loss is confined to a small, enclosed area. Bruises are common in patients with hemophilia. Bruises are usually small and tend to clear up without treatment.

Cuts

The type of treatment needed for a cut depends on the severity and location of the cut. In general, minor cuts can be treated by applying pressure to the site. Applying ice packs to the site may also be helpful. In some cases, agents such as Gelform or Fibrin Foam may be applied to the cut to help stop bleeding.

Ice packs should not be applied directly to the skin. Packs should be wrapped in a towel to prevent ice burns.

Nose Bleeds

Nose bleeds can be triggered by many things including injury. However, most nose bleeds are due to dry or irritated tissue.

Coating the inside of the nose with a thin layer of petroleum jelly can help reduce nose bleeds in patients who live or work in dry environments. Using humidifiers may also help reduce irritation, especially during winter months.

Nose bleeds can usually be treated by keeping the head upright and applying pressure to the nostrils. Applying an ice pack to the side of the nose may also be helpful.

Children often swallow blood from nose bleeds. Blood that has been swallowed may cause black, tarry stools.

Dental Disease

Patients with all levels of severity of hemophilia can experience excessive bleeding with dental work. Dentists who are knowledgeable about hemophilia often provide the best care for patients with this disorder.

The two most common dental diseases are dental caries or cavities and gingivitis/periodontitis or gum disease. Both of these diseases can be prevented through good dental hygiene. Many patients do not take care of their teeth and gums because of fear that they may cause a bleed. However, not practicing good dental hygiene increases the risk of a bleed.

When there is bleeding in the mouth, the diet should consist of cold or chilled and soft foods. Straws should not be used. Sucking on the straw can cause any clot that forms to come loose.

Hematuria

Hematuria refers to blood in the urine. Some patients can have bleeding in the urinary tract but do not have any symptoms such as pain. Other patients may have mild or severe pain.

Hematuria can cause the urine to become red and cloudy. In some cases, blood in the urine may only be detected with a routine urine test.

Hematuria increases the risk of clots forming in the urinary tract. Treatment for hematuria includes fluids and rest. Fluids help prevent clots from forming. If factor is infused, patients may be advised to drink fluids and void frequently to further reduce the risk of clots.


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

IV THERAPY AND RESERVOIRS FOR HEMOPHILIA

Factor must be infused into a vein to treat hemophilia. For patients who infuse factor only when bleeds occur, accessing a vein may not be a problem. For patients who infuse factor on a regular basis, accessing a vein can pose challenges. To reduce these challenges, many patients with hemophilia use an implanted reservoir, also called a port, when frequent infusion is needed.

Doctors insert the reservoir during minor surgery in which the patient is under anesthesia. The reservoir is placed just below the collar bone under the skin. A tube is then attached to the reservoir and then sewn into the subclavian vein. The reservoir remains in place permanently unless a problem occurs.

The access needle is called a Huber needle. The needle is inserted through the skin and into the reservoir. The L-shape allows part of the needle to stay in the reservoir and part of the needle to lay flat against the skin. Factor is injected through the Huber needle into the reservoir.

Patients who use an implanted reservoir report very few problems. Infection is a concern and occurs in 10-15%. Other common problems are growth and access. Some children need a larger reservoir implanted when they grow. Most patients need someone to change the Huber needle due to the location of the reservoir. Patients are usually not able to see the reservoir site although some patients can change the needle by looking in a mirror. Bleeding at the reservoir site can occur, and occasionally a blood clot can block the tip of the catheter tubing. This can prevent infusions through, or blood draws from, the reservoir.


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

SAFETY GUIDELINES FOR HEMOPHILIA

Using the guidelines listed below can help you improve safety.

Personal Safety

Health Safety

Factor Safety

General Safety


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

EMERGENCY MANAGEMENT FOR HEMOPHILIA

Trauma can present special risks and problems for patients with hemophilia. The most dangerous traumas are those to the head, neck and abdomen.

Bleeding in the head, or intracranial bleeding, can occur as a result of trauma. In patients with severe hemophilia, these bleeds can also occur suddenly, without an apparent cause. Intracranial bleeding accounts for about 24% of all deaths. If not properly treated, intracranial bleeding can cause seizure disorders and permanent problems with mental function and with movement.

Trauma to the head does not always cause immediate symptoms. However, head injuries should always be treated as though they were life-threatening. When a head injury occurs, factor is usually infused as soon as possible. Doctors advise that head injury severe enough to cause stunning or loss of consciousness should be regarded as serious. In such cases, patients should infuse factor immediately. If the patient remains unconscious and unable to infuse factor, someone who knows the proper way to infuse factor should do so. In both circumstances, the patient should be seen in an emergency room. If possible, it is wise for the patient or their family to bring their own factor to the hospital.

Trauma to the neck can quickly lead to serious problems. Doctors advise patients to treat trauma to the neck as though it were life-threatening. As with head trauma, patients should infuse factor immediately and then go to the emergency room.

Trauma to the abdomen is often a result of a heavy blow. In many cases, the extent of injury in the abdomen may not be known until symptoms appear. It is usually best for patients to assume that serious injury has occurred and seek proper medical attention.

Trauma is never planned but steps can be taken to reduce its impact.

If trauma should occur:

Warning signs of bleeding in the brain

Warning signs of bleeding in the neck

Warning signs of bleeding in the abdomen


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

IMPACT OF HEMOPHILIA: A HOLISTIC VIEW

A holistic view of health states that health consists of six areas: physical, emotional, social, spiritual, occupational and intellectual. Each area of health affects all other areas.

Chronic disorders can place heavy burdens on all areas of health. Hemophilia may place more burdens than most disorders because the impact of hemophilia usually begins at birth.

In most cases, hemophilia is an inherited disorder. In some cases, guilt and/or blame may be placed on a parent, usually the mother, when a child is born with the disorder. Such views may be self-imposed or come from others. These views can lead to more stress and problems for the parent and for relationships.

As the child grows, parents may feel undue stress from fear about the child's safety. Fear can cause parents to guard the child too closely. Without enough freedom, a child can miss aspects of life that are important for growth in other areas. Many people are afraid of things they do not understand. Because hemophilia is a disorder of bleeding, patients and even family members must sometimes endure the actions that come from others' lack of knowledge about the disorder. These actions may be as simple as not asking a child to play, but they are still painful.

The intensity of treatment that is needed for hemophilia depends on the severity of the disorder. Some families may feel the need to live close to a treatment center that offers special services for patients with hemophilia. Choices about where to live can then affect career choices.

The costs for treating hemophilia are very high. Health insurance coverage is a must for most families. Types of health coverage that may be offered can affect the job choices for the parents and later, for the working patient.

As difficult as hemophilia can be, the impact of hemophilia does not always have to be bad. Families can become stronger as they unite for a common cause - the welfare of the child. A child can learn to value himself more fully as he learns to see himself as a person with a disorder first and the disorder second. Families and patients can choose to see the value of a job or housing choice that might not be possible with other choices. People can also choose to realize the value of help from others.

Hemophilia Treatment Centers offer a variety of services for patients with hemophilia and for family members (Please see Coping With -Foundations for more information). Use of services can stop many problems before they occur. Use of services when problems begin can help prevent them from getting worse.

In the past, hemophilia was a disorder that controlled every aspect of life, often in a harsh way. Now, modern treatments have opened the door for patients with hemophilia and family members to live more fully. Patients and family members can take control of all areas of their health.


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

HEPATITIS AND HEMOPHILIA

Hepatitis is an inflammation of the liver. Of the viruses that cause hepatitis, Hepatitis A (HAV), Hepatitis B (HBV) and Hepatitis C (HCV) are most common.

Hepatitis A

Patients who are infected with the Hepatitis A virus can transmit it to others for several weeks.

Hepatitis A causes flu-like symptoms during the acute or early phase of the disease. Patients may also develop a yellow color in the skin and eyes, dark yellow urine and light-colored stools. Some patients do not have any symptoms.

Hepatitis A is diagnosed with a blood test that determines the presence of antibodies to the virus. The body forms antibodies to destroy foreign substances in the body. Hepatitis A antibodies appear about four weeks after infection.

Most patients do not require treatment for Hepatitis A. This disease usually does not become a chronic or long-term condition nor do chronic problems occur as a result of the infection.

Hepatitis A can be prevented with vaccination, which is about 90% effective. Children can get the vaccine after they turn 2 years old. Children aged 2 to 18 will need two shots, given over the course of a year. Adults get one shot.

Hepatitis B

Patients infected with the Hepatitis B virus can sexually transmit the virus to others. Mothers can transmit the virus to newborns, who are exposed to blood and vaginal fluids during delivery and to infants who breastfeed. Some patients with hemophilia who used blood products before 1985 became infected with Hepatitis B.

Some patients have flu-like symptoms when infected with Hepatitis B. Patients may also develop a yellow color in the skin and eyes, dark yellow urine and light-colored stools. However, most patients do not have any symptoms.

Almost 90% of patients recover from Hepatitis B without medical treatment. Many of these patients develop antibodies to the virus, which makes them immune to future infections. Some patients become carriers of the disease. Carriers do not have symptoms but can transmit the virus to other people. Some patients with Hepatitis B may become chronically infected and need treatment.

Treatment for hepatitis B may involve a drug called interferon. It is given through shots. Most people are treated with interferon for 4 months. Lamivudine is another drug used to treat hepatitis B. Lamivudine is taken by mouth once a day. Treatment is usually for one year. Sometimes lamivudine is combined with interferon. Over time, hepatits B may cause the liver to stop working. If this happens, the patient will need a liver transplant.

Hepatitis B can be prevented with a vaccination. Infants get the first shot within 12 hours after birth. They get the second shot at age 1 to 2 months and the third shot between ages 6 and 18 months. Older children and adults can get the vaccine, too, with three shots given over 6 months.

Hepatitis C

Hepatitis C is the most common form of hepatitis in patients with hemophilia. Most of these patients were infected with blood products used before 1985. There is evidence that Hepatitis C virus can be transmitted sexually but this is rare.

There are usually no or only minor symptoms of Hepatitis C. However, patients may have significant liver damage within 10 years. Patients may progress to having cirrhosis within 20 years. Cirrhosis is the scarring of tissue in the liver that keeps the liver from working properly.

Hepatitis C is diagnosed with blood tests. One test can determine the presence of Hepatitis C antibodies. Another test can determine the presence of the virus itself.

Hepatitis C is treated with a drug called peginterferon alone or in combination with another drug called ribavirin. Over time, hepatitis C may cause the liver to stop working. If this happens, the patient will need a liver transplant.

Secondary infection with hepatitis A can be life threatening. All people with hepatitis C should receive the hepatitis A vaccine if they are hepatitis A negative.

Risk of hepatitis infection

Since 1985, improved screening techniques have greatly reduced the risk of hepatitis for patients with hemophilia. Improved manufacturing of products has further reduced the risk.


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

HIV/AIDS AND HEMOPHILIA

In 1981, health officials noted a high number of cases of rare diseases in a group of homosexual men. In 1982, officials noted these diseases in heterosexual males with hemophilia. Intense research led to the discovery in 1983 of the virus that is now known as HIV.

HIV stands for "Human Immunodeficiency Virus". This is the virus that causes Acquired Immune Deficiency Syndrome (AIDS).

By 1985, health officials had learned that HIV was spread through blood and blood products. Screening tests were developed and were used to stop blood donated by HIV-infected persons from being added to the nation's supply. The existing blood supply also went through intense screening to remove any infected products that were already in the system.

By the time screening began, however, almost half of the patients with hemophilia had been infected with HIV. Today, about 10–15% of patients are living with HIV and AIDS. This percentage is going down as older persons die and the number of persons born since 1985 goes up.

Being HIV-infected is not the same thing as having AIDS. Patients who are HIV-infected simply have the virus. These patients have tested positive for antibodies to the virus. The body forms antibodies to destroy foreign matter such as viruses in the body. Over time, HIV damages the immune system.

During the early stage of HIV infection, patients often have flu-like symptoms. In most cases, these symptoms go away and patients may be symptom-free for a long time. As the immune system gets weaker, other symptoms occur, including night sweats, fatigue, sore throat, mouth infections, severe diarrhea and loss of body weight.

AIDS is the advanced and final stage of HIV infection. By this stage, the immune system is damaged to the point that the body can no longer fight off infections. "Opportunistic" infections occur. These infections are caused by germs that only thrive when a weakened immune system provides the opportunity. Rare cancers may also develop.

The length of time needed for AIDS to develop varies from person to person. The goal of treatment for HIV is to lengthen the time between initial infection and AIDS. With increased research and improved treatments, this time frame is growing.

Patients with hemophilia who are also HIV-positive tend to have a chronic form of hepatitis. As treatment for HIV has improved, concern has become more focused on infection with hepatitis viruses.

Improved screening techniques have greatly reduced the risk of transmitting viruses via blood and blood products to patients with hemophilia. Improved manufacturing has also increased the safety and purity of products for treating hemophilia, reducing the risk of transmitting viruses via blood and blood products even further.


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

MUSCLE PROBLEMS IN HEMOPHILIA

Patients with severe hemophilia can have sudden bleeds into the muscle tissue. All patients with hemophilia can have muscle bleeds due to severe trauma. Bleeding is most likely to occur in the calf, thigh, groin and forearm muscles.

Symptoms of a bleed may vary somewhat, depending on which muscle is affected. Bleeds in all muscles usually cause pain, swelling or tightness of the muscle, and sometimes warm to touch. If the swelling presses against or increases pressure around a major nerve or blood vessel, tingling sensations, weakness or extreme muscle spasm can occur.

Without proper treatment, muscle bleeds can lead to paralysis of the affected region. Hands, feet and even whole limbs can also become frozen into unusual positions.

Treatment usually consists of infusing factor as soon as possible after major trauma. Rapid treatment is particularly important for bleeding in the thigh muscles. Factor may need to be infused several days because large muscle bleeds tend to clear up slowly.

Treatment with factor should be started at the first sign of muscle pain. Patients do not need to wait for swelling to begin. The affected area should be kept immobile. If possible, ice should also be applied to the area.

Small bleeds into the muscles are more common than large bleeds. Small bleeds may occur as a result of minor trauma for patients with severe and moderate hemophilia. With treatment, small bleeds tend to clear up quickly. In many cases, small bleeds may clear up without treatment.

It is always best to prevent bleeds into the muscles whenever possible. Bleeds can often be prevented by following general safety tips.

Safety Tips


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

KEEPING TRACK OF FACTOR

Hemophilia is treated by giving the missing or low factor. All patients with hemophilia should keep a written record of their use of factor. This record is often called a Factor Log.

Patients are sometimes annoyed because they must keep a Factor Log. This may be especially true for patients who infuse factor every two or three days. Remembering the medical importance of the log can help reduce frustration.

The format of the Factor Log may vary from person to person. However, all logs should contain certain information. Patients should always record the following:

When factor is given is very important. Factor levels must also be low enough to prevent side effects. Doctors know how long factor should be effective after it has been given. If problems such as bleeding or side effects occur, doctors will want to review when factor has been used. This information will help doctors determine if factor is being given too often or not often enough or if other problems may be occurring.

Some patients use factor on a regular basis to prevent problems from occurring. These patients may use more factor if a bleed occurs. Other patients use factor only when it is needed to treat a bleed. Doctors use information about why factor was used to determine if bleeding problems are occurring too often given the treatment plan.

Repeated bleeds at one site can cause other serious problems. Information about where bleeding is occurring helps doctors determine if other tests and/or treatments are needed.

Recording the Lot Number and the Expiration Date of the factor is a safety measure. For instance, if an illness occurs with using factor, it is essential to know what batches of factor are implicated. The simple act of recording the expiration date may prevent patients from mistakenly using old factor.

Factor dose, reason factor was used, lot number and expiration date may seldom change. However, this information is still important for each infusion. Using a Factor Log format that allows the last information to be dittoed (") can eliminate the need to rewrite this information. Likewise, any other information that remains the same from one infusion to the next can also be dittoed. When using this method for recording information, it is important that patients make sure that the information that is being dittoed is still correct.

A sample of a Factor Log format that allows information to be dittoed appears below. Patients can get a blank copy of this form for personal use by clicking on the reference.

Sample Factor Log
Date Time Dose Reason Site of Bleed Factor Lot Number Factor Expiration Date
Prevention Symptoms
1-10-00 8:00 am 250 u X   N/A bax555 12-1-00
1-12-00 8:00 am 250 u X   N/A " "
1-13-00 2:30 pm 250 u   X R knee " "

Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

FACTOR LOG

Download the factor log

Name:        Current Weight:       
Date -        Time -       Product Infused:       
# Units Infused:       
Lot #:       
Exp Date:      
Reason for Infusion:
Immune Tolerance       Prophylactic 
Other                        Bleed
Infused By:    Self             Parent/caregiver
Nurse         Other
Date -      
Time -      
Product Infused:       
# Units Infused:       
Lot #:       
Exp Date:      
Reason for Infusion:
Immune Tolerance       Prophylactic 
Other                        Bleed
Infused By:    Self             Parent/caregiver
Nurse         Other
Date -      
Time -      
Product Infused:       
# Units Infused:       
Lot #:       
Exp Date:      
Reason for Infusion:
Immune Tolerance       Prophylactic 
Other                        Bleed
Infused By:    Self             Parent/caregiver
Nurse         Other
Date -      
Time -      
Product Infused:       
# Units Infused:       
Lot #:       
Exp Date:      
Reason for Infusion:
Immune Tolerance       Prophylactic 
Other                        Bleed
Infused By:    Self             Parent/caregiver
Nurse         Other
Date -      
Time -      
Product Infused:       
# Units Infused:       
Lot #:       
Exp Date:      
Reason for Infusion:
Immune Tolerance       Prophylactic 
Other                        Bleed
Infused By:    Self             Parent/caregiver
Nurse         Other
Date -      
Time -      
Product Infused:       
# Units Infused:       
Lot #:       
Exp Date:      
Reason for Infusion:
Immune Tolerance       Prophylactic 
Other                        Bleed
Infused By:    Self             Parent/caregiver
Nurse         Other
Date -      
Time -      
Product Infused:       
# Units Infused:       
Lot #:       
Exp Date:      
Reason for Infusion:
Immune Tolerance       Prophylactic 
Other                        Bleed
Infused By:    Self             Parent/caregiver
Nurse         Other
Date -      
Time -      
Product Infused:       
# Units Infused:       
Lot #:       
Exp Date:      
Reason for Infusion:
Immune Tolerance       Prophylactic 
Other                        Bleed
Infused By:    Self             Parent/caregiver
Nurse         Other
Date -      
Time -      
Product Infused:       
# Units Infused:       
Lot #:       
Exp Date:      
Reason for Infusion:
Immune Tolerance       Prophylactic 
Other                        Bleed
Infused By:    Self             Parent/caregiver
Nurse         Other
Date -      
Time -      
Product Infused:       
# Units Infused:       
Lot #:       
Exp Date:      
Reason for Infusion:
Immune Tolerance       Prophylactic 
Other                        Bleed
Infused By:    Self             Parent/caregiver
Nurse         Other
Date -      
Time -      
Product Infused:       
# Units Infused:       
Lot #:       
Exp Date:      
Reason for Infusion:
Immune Tolerance       Prophylactic 
Other                        Bleed
Infused By:    Self             Parent/caregiver
Nurse         Other
Date -      
Time -      
Product Infused:       
# Units Infused:       
Lot #:       
Exp Date:      
Reason for Infusion:
Immune Tolerance       Prophylactic 
Other                        Bleed
Infused By:    Self             Parent/caregiver
Nurse         Other


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

INHIBITORS AND HEMOPHILIA

An antibody is a substance produced by the immune system to protect the body from foreign substances that enter the body. In hemophilia, an antibody can be formed to destroy the factor. This antibody is called an inhibitor. Inhibitors do not cause more bleeds. However, inhibitors make it more difficult for factor to work.

Doctors may suspect that an inhibitor has developed when the patient no longer responds to the dose of factor that has been working. Doctors may also find inhibitors through routine annual testing.

The Bethesda inhibitor assay is a test to measure inhibitors. The amount of inhibitor is reported as the number of Bethesda units or Bethesda titer. When more inhibitor is present, the Bethesda titer will be higher.

Inhibitors are labeled as either "high responding" or "low responding". A high responding inhibitor reacts very quickly when factor is present. The Bethesda titer also goes very high and can take months to drop to a low level again. The response of a low responding inhibitor is much slower and Bethesda titer will be much lower.

Inhibitors can develop at any time. However, the risk of developing an inhibitor is greatest during childhood. Between 20-35% of patients with Hemophilia A develop an inhibitor compared to less than 5% of patients with Hemophilia B. Inhibitors in patients with Hemophilia B can be associated with anaphylaxis to factor IX concentrates. For both types of hemophilia, inhibitors usually develop in patients with the severe form of the disorder. Inhibitors can develop in patients with moderate hemophilia but are rare in patients with mild hemophilia.

Treatment for Inhibitors

The presence of inhibitors can make managing the bleeding associated with hemophilia much more difficult. For this reason, treatment to eliminate inhibitors may be used. Treatment to stop bleeding when inhibitors are present includes several different products.

Immune tolerance therapy is most effective if started when Bethesda titers are less than 10 BU. This treatment uses large amounts of factor to train the immune system to accept the factor and eliminate the inhibitor.

Prothrombin complex concentrates (PCCs) and activated prothrombin complex concentrates (aPCCs) use other clotting factors to bypass the need for factor VIII or factor IX. The effectiveness of these products varies from patient to patient. This treatment is short-acting, which means the effects only last for a short time. Using this treatment can occasionally cause clots in the blood vessels.

Activated prothrombin complex concentrates may be used to treat bleeding when inhibitors to factor VIII are present.

Recombinant factor VIIa is a treatment for bleeding that has shown very good results. However, this treatment is very expensive and the effect only lasts about two hours, which requires several IV doses given three hours apart to stop bleeding.

Porcine factor VIII has been another option for patients with FVIII inhibitors. The similarity of factor VIII from pigs to that of factor VIII from humans lets it work in humans. In about 25% of patients, the FVIII inhibitor also affects the porcine factor VIII. The differences between the two factors keep the inhibitors from attacking the porcine factor VIII. Some patients can develop allergic side effects to porcine factor VIII. Presently, porcine factor VIII is not commercially available, but can be obtained on a compassionate basis.


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

HERBAL-DRUG INTERACTIONS

 Herbal  Drug  Effect
Echinacea Anabolic steroids, amiodarone, methotrexate, ketoconazole Toxic effects on liver
Feverfew NSAIDs
Blood thinning agents
Decreased effect of feverfew
Increased risk for bleeding
Garlic Aspirin, blood thinning agents Affects blood clotting
Ginkgo biloba Anticoagulants, aspirin, NSAIDs
Anti-seizure medicines, tricyclic antidepressants
Increased risk of bleeding
Increased risk of seizures
Ginseng MAO inhibitors
Corticosteroids
Warfarin
Headache, tremors, mania
May increase toxic effects of steroids
Affects blood clotting
Siberian ginseng Digoxin Elevated blood levels of digoxin
Kava Benzodiazepines Sedation, dizziness, confusion
Ma huang (ephedra) Antidepressants, stimulants Increased effect of ephedra, seizures
St. John's wort Piroxicam, tetracyclines
Antidepressants, stimulants
Theophylline
Increased sensitivity to light
Increased toxic effects
Decreased theophylline levels in the blood
Saw palmetto Estrogen Increased effect of saw palmetto
Valerian Sleeping pills, anti-anxiety medications, muscle relaxants Sedation, confusion, dizziness

Herbs That May Affect Blood Clotting
Angelica Clove Licorice Tumeric
Anise Danshen Meadowsweet Wild carrot
Arnica Fenugeek Prickly Ash Wild lettuce
Asafoetida Garlic Onion Willow
Bogbean Ginger Papain Boldo
Ginkgo Passionflower Capsicum Ginseng (Panax)
Poplar Celery Horse chestnut Quassia
Chamomile Horseradish Red Clover  

Source: Natural Medicines Comprehensive Database


Last Modified Date: September 10, 2009 © Accordant Health Services, a CVS/Caremark company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

HEMOPHILIA AND GOOD NUTRITION

Hemophilia patients can boost their overall health by eating a highly nutritious, well-balanced diet. In general, a healthy diet is one that focuses on fruits, vegetables and whole grains and limits fat and cholesterol. A well-balanced diet can provide all of the nutrients hemophilia patients need.

Certain nutrients are of special interest to people with hemophilia. Patients can choose foods that ensure a good supply of:

B Vitamins.

The eight B vitamins are B1 (thiamin), B-2 (riboflavin), B-3 (niacin), B-5 (pantothenic acid), B-6 (pyridoxine), B-7 (biotin), B-12 (cobalamin) and folic acid. B vitamins help convert blood sugar into energy and help the body fight disease. They benefit the skin and eyes, fight depression, and help form red blood cells.

Vitamin B-12 is found only in animal products. Egg yolks, meats, poultry, fish, fermented cheeses and dry milk are good sources. Some sources for the other B vitamins include bananas, lobster, brewer's yeast, peas, corn, peanut butter, whole grains, orange juice and soybeans.

Vitamin K

Vitamin K helps blood to clot properly. It helps prevent excessive bleeding. It also plays a role in bone health. Sources include canola, soybean and olive oils, beef liver, bran, green tea, turnip greens, broccoli, spinach, cabbage, asparagus and dark green lettuce.

Iron

Iron forms part of the oxygen-carrying protein, hemoglobin in the red blood cells. It also makes up part of some enzymes. We get iron from eating grain products, meat, poultry, fish, vegetables, legumes, nuts, and soy.


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

IMMUNIZATIONS

Hemophilia and Flu Shots

Flu shots are generally recommended for people with hemophilia. This immunization is especially important for people with compromised immune systems, such as those with HIV/AIDS. The nasal-spray flu vaccine contains weakened live viruses and should not be used by a person with hemophilia and HIV/AIDS. However, the flu shot does not contain live viruses so you cannot get the flu from the flu shot. Flu shots should be given under the skin (subcutaneously) rather than into the muscle. If this is not possible, people with hemophilia should be infused with factor before the shot to prevent a bleed in the muscle. After the shot, the area should be iced. Talk with your doctor about the best way to get a flu shot.

The Influenza Vaccine

Influenza ("the flu") is a highly contagious respiratory tract infection caused by a virus. Symptoms can include fever, sore throat, cough, runny nose, headache, chills, tiredness, and muscle aches. In children, the flu can cause high fever and seizures. Each year about 200,000 people in the U.S. are hospitalized because of the flu and about 36,000 people die.

The best protection against getting the flu is vaccination. The vaccine causes the body to produce antibodies that protect you when you are exposed to flu viruses during flu season. The "flu shot" is generally recommended for people with a chronic health condition. The "flu shot" contains a killed virus that is given with a needle. The nasal-spray flu vaccine contains a weakened live virus and is only approved for healthy people between the ages of 5 and 49 years.

You should get a flu shot every year. The vaccine typically protects against three types of virus that scientists expect to be causing the most severe flu worldwide. Because the expected viruses change from year to year, the vaccine changes as well.

The best time to receive the vaccine is before the flu is likely to occur. Doctors recommend being immunized in October or November. Protection begins about two weeks later, after the body's immune system has had time to make antibodies against the flu viruses. A flu shot doesn't absolutely guarantee that you won't get the flu but if you've had a flu shot and still get the flu, it will usually be a much milder case than if you had not gotten the flu shot. Even if you have not received a flu shot early in the season, you should still consider getting one. Talk with your doctor before getting a flu shot.

Certain people should make sure to get this vaccination. The Centers for Disease Control & Prevention recommends vaccination for the following groups of people:

There are a few people who should not get a flu shot. These include people who:

The flu shot may cause some people to experience a sore arm. Severe reactions are rare and the risk for a reaction is much, much less than the risk of severe complications from the flu!

For more information about getting a flu shot for this year's flu season and the flu vaccine shortage, visit the Centers for Disease Control and Prevention Web site at www.cdc.gov/flu.

The Pneumonia Vaccine

The pneumonia vaccine prevents infection by the Streptococcus pneumoniae bacteria. These bacteria are also called pneumococci. Pneumococcal diseases kill at least 40,000 Americans each year. Children under the age of 2 and adults over 65 are most vulnerable to pneumococcal bacteria. When the infection is in the bloodstream, it is called bacteremia. When they invade the tissues and fluids surrounding the brain and spinal cord, the result is meningitis. Pneumococcus bacteria are also responsible for many ear infections and sinus infections. In the lungs, these bacteria cause pneumonia. Pneumococcal pneumonia is a common complication of the flu. About 500,000 cases of pneumonia occur each year in the United States.

The best way to protect yourself from pneumococcus bacteria is to receive the pneumonia vaccine. The pneumonia vaccine provides protection from the most common strains of pneumococcal bacteria. The pneumonia vaccine can be given at the same time as a flu shot with no risk of increasing side effects. The pneumonia vaccine uses inactive bacteria to help the immune system recognize and fight off any live bacteria it may encounter in the future.

The pneumonia vaccine is recommended for these groups of people:

The National Immunization Program recommends that children younger than 23 months of age be vaccinated with the pneumococcal conjugate vaccine.

You should talk to your doctor about vaccination if you are pregnant. The safety of the pneumonia vaccine during pregnancy has not been evaluated.

Questions and Answers about Flu and Pneumonia Immunizations

Q. Are flu and pneumonia immunizations really all that important?

A. Yes, these immunizations really are very important, especially if you are 50 years of age or older, or have a chronic health condition. Together, flu and pneumonia are the seventh leading cause of death in the United States and the fifth leading cause in people 65 and over. Talk with your doctor before getting a flu or pneumonia shot.

Q. How contagious is the flu?

A. The influenza virus is highly contagious. It is easily spread from one person to another by coughing and sneezing. A person who has "caught" the flu usually begins feeling sick between one and five days after being exposed. An infected person is contagious from one day before any symptoms appear and continues to be contagious for up to a week. It is also possible for some people to be infected with the flu but experience no symptoms. Those without symptoms can still pass the virus on to others.

Q. What kind of complications can be caused by the flu?

A. Pneumonia, sinus infections, ear infections, and worsening of chronic medical conditions are some of the complications that can occur as a result of the flu. Even death can occur in very severe cases.

Q. If I get the flu, will I need an antibiotic?

A. No. There are antiviral drugs that may be effective and are approved for influenza. You should talk with your doctor about this issue.

Q. How many doses of pneumonia vaccine are needed?

A. For people with chronic health conditions or who are over the age of 65, a second dose of pneumonia vaccine is advised if it has been five or more years since the last dose. A second dose is also specifically recommended for those who:

Children ten years old and younger may get a second dose three years after the first dose. Children older than ten should get a second dose five years after the first dose.

Q. I still have questions about these immunizations. Where can I get more information?

A. There are many sources of information. You can start by asking your doctor or your Accordant nurse for more information. You can also visit your health plan's Web site. You can always find up-to-date, reliable information on the Centers for Disease Control National Immunization Program Web site (http://www.cdc.gov/vaccines/) and at the Immunization Action Coalition Web site (http://www.immunize.org/). You can also call the National Immunization Information Hotline at 1-800-232-2522 (English) or 1-800-232-0233 (Spanish).

References

World Federation of Hemophilia. Guidelines for the management of hemophilia. Available at: http://www.wfh.org/2/docs/Publications/Diagnosis_and_Treatment/Gudelines_Mng_Hemophilia.pdf  Accessed August 22, 2007.

National Hemophilia Foundation. Baby and toddler tips. Available at: http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=194&contentid=66  Accessed August 22, 2007.

Centers for Disease Control and Prevention. Key facts about influenza and the influenza vaccine. Available at: http://www.cdc.gov/flu/keyfacts.htm. Accessed August 28, 2006.

Centers for Disease Control and Prevention. Inactivated influenza vaccine 2006-2007: What you need to know. Available at: http://www.cdc.gov/vaccines/pubs/vis/downloads/vis-flu.pdf  Accessed August 28, 2006.

National Institute of Allergy and Infectious Diseases. Pneumococcal Pneumonia. Available at: http://www.niaid.nih.gov/factsheets/pneumonia.htm  Accessed: October 14, 2005.

Centers for Disease Control and Prevention. Pneumococcal polysaccharide vaccine: what you need to know. Available at: http://www.cdc.gov/vaccines/pubs/vis/downloads/vis-ppv.pdf Accessed August 28, 2006.

Morbidity and Mortality Weekly Report. Prevention and Control of influenza. Available at: http://www.cdc.gov/mmwr/preview/mmwrhtml/rr5103a1.htm  Accessed August 28, 2006.

MedlinePlus. Pneumococcal polysaccharide vaccine. Available at: http://www.nlm.nih.gov/medlineplus/ency/article/002029.htm  Accessed October 2, 2006.


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

EXERCISE AND HEMOPHILIA

Is your weight within a healthy range for your height and build? If not, you should know that being overweight or underweight can have a negative impact on your health.

The American Heart Association says that even 10-15 pounds of extra weight can cause problems. Excess pounds are associated with many health problems. In fact, according to the National Institutes of Health, the more extra weight our bodies bear, the more likely we are to have health problems. Being underweight carries a different set of risks. Weighing too little makes it harder for the body to fight infection. Being underweight can also mean we are not getting enough of the nutrients we need to sustain good health.

Weight Control and Chronic Conditions

If you have a chronic condition, it is very important to pay attention to your weight. Many chronic conditions cause people to gain weight because reduced mobility or fatigue slows their ability to burn calories. Other people lose weight because illness curbs their appetite or affects their ability to absorb nutrients. Weight gain or loss can also be due to medicines.

Maintaining a normal weight may be more difficult with a chronic condition, but it is important. A normal weight reduces the risk of getting preventable illnesses that would complicate your chronic condition. A normal weight also enhances the body's ability to cope with an ongoing illness. Amy Martin, a registered dietitian, explained, "Your body is already undergoing stress by having that chronic disease. So by not taking care of it through exercise and nutrition, you're adding to the damage. You're making it extra hard for your body to deal with the disease."

How Nutrition and Exercise Work Together

When we take in more calories than we use up, we gain weight. When we use more calories than we take in, we lose weight. Weight control is all about balancing calorie use with calorie intake. To lose one pound, we must burn 3,500 calories. A person who eats 250 fewer calories per day will lose one pound over a two-week period. Losing weight can be a slow, difficult process if we rely on diet alone. Weight loss gets easier and happens faster, however, when we add exercise. The rate of weight loss can double by combining diet and exercise. A person who eats 250 fewer daily calories and takes a brisk 30-minute walk four days a week should lose a pound a week. There is more good news: exercise raises the metabolism. This means that people who exercise regularly keep burning extra calories for several hours after they stop exercising.

Exercise has many other health benefits. It helps reduce the stress and depression that often plague people with chronic conditions. It boosts cardiovascular health, helps reduce blood pressure, benefits the digestive tract and strengthens bones. Exercise causes the muscles around joints to get stronger. This reduces strain on the joints and helps protect them. Other benefits include sleeping better at night, less fatigue and more energy.

Clearly, patients who can exercise, should. Patients need as much appropriate exercise as possible. But not everyone can exercise for long periods of time. Some patients really experience fatigue with exertion. These patients can consider sporadic exercise throughout the day. Routine activities like climbing stairs, gardening or cleaning the house may be better ways for some patients to burn calories. All physical activity burns calories, so anytime we are moving it "counts."

The important thing to remember is that both good nutrition and regular exercise are needed for optimal weight control. One without the other is not nearly as effective. The National Institutes of Health says, "Research consistently shows that regular physical activity, combined with healthy eating habits, is the most efficient and healthful way to control your weight."

If you have a chronic illness, talk with your doctor before changing your diet or starting an exercise program.


Last Modified Date: September 10, 2007 © Accordant Health Services, a Caremark company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

HEMOPHILIA AND STRESS

Human beings experience stress as a response to change. While everyone has stress, individuals and families dealing with hemophilia often have more stress than other people do. Chronic illnesses like hemophilia may require people to make changes in many areas of their lives.

Hemophilia has the potential to create stress in some or all of these ways:

Coping with Stress

It is very important for hemophilia patients and parents to learn how to cope with stress. Young patients must understand the importance of taking good care of themselves. Parents can learn ways to reduce family stress and teach these methods to their children. Good nutrition, sufficient sleep and regular exercise all help buffer hemophilia patients and parents against stress.

Experts say that the key to managing stress is how we think about the things that cause us stress. Patients and parents are encouraged to distinguish between things that can and cannot be changed. When things can't be changed, thinking must change. Many people have learned to combat stress by mastering relaxation methods. Books and tapes, support groups, counseling, exercise, humor, breathing techniques and sometimes medication can help. In addition, families can make choices that help reduce some of life's routine stresses. This starts by identifying stressors and then taking steps to reduce as much stress as possible.


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

BLOOD PRESSURE AND HEMOPHILIA: QUESTIONS AND ANSWERS

Q. What is blood pressure?

A. Every beat of your heart forces oxygen-rich blood into the aorta, the largest artery in your body. When the heart contracts, the blood in the aorta is at its maximum pressure. Between heartbeats, blood is at its minimum pressure. A blood pressure reading records these two pressures, which are displayed as a fraction. The top number is the maximum pressure (systolic); the bottom number is the minimum pressure (diastolic).

Blood pressure can vary from one moment to the next. Emotions, posture, physical activity, medications, sleep, and other things can cause blood pressure to change.

Q. How can I find out what my blood pressure is?

A. Blood pressure is measured with a sphygmomanometer. This medical instrument has an inflatable cuff that is placed around the upper arm. The upper arm is the best place to take a blood pressure reading because it is approximately the same elevation as the aorta. Blood pressure is expressed as "millimeters of mercury" (mm Hg). This is a measurement of how high mercury rises in a tube under the force exerted by the blood pressure.

Your doctor's office probably checks your blood pressure at each visit. Between visits you can use blood pressure machines available for customers at many pharmacies. You can also purchase a sphygmomanometer for home use.

Q. What do the blood pressure numbers mean?

A. Both of the numbers in a blood pressure reading are important indicators of heart and blood vessel health. Blood pressure of less than 120/80 is considered a normal reading for adults. If you have diabetes, a normal blood pressure goal is 130/80. Values of 120–139/80–89 are considered "prehypertensive." These people are at higher risk for developing hypertension and need to monitor their blood pressure more closely. A blood pressure equal to or greater than 140/90 is considered high. Blood pressure that is lower than your normal value can result from dehydration, internal bleeding, blood loss, or shock from heart failure.

High blood pressure makes the heart work harder and weakens it over time. It also increases the chances of heart attack, stroke, kidney failure, or congestive heart failure. Elevated blood pressure usually has no symptoms. Readings in the "high" range (140/90 or greater) could signal current or potential problems. Monitor your blood pressure and tell your doctor if your blood pressure readings are 140/90 or higher.

Q. Can stress affect my blood pressure?

A. Stress is a factor for high blood pressure. Our minds and bodies respond to stress in much the same way that we respond to physical dangers. Stress stimulates the release of the same powerful hormones that enable a person to either flee to safety or defend himself in a crisis. This is the classic "fight or flight" response. A chronic illness like hemophilia can create not only physical stress, but also financial, social, emotional, and occupational stresses. Any of these or any combination of these stresses can indeed affect blood pressure.

You may not be able to eliminate all of the stress that accompanies hemophilia. What can change, however, is the way you think about stressful events and circumstances. This is the basic idea behind many of the complementary and alternative treatments categorized as "body/mind medicine." (See the "Pain, Stress and Mood" and the "Complementary and Alternative Medicine" sections of the Accordant Library for information on managing stress.)

Q. Why is it important for patients with hemophilia to monitor blood pressure?

A. In hemophilia, blood pressure changes can accompany medications taken to address certain symptoms. Circulatory disease has been reported as the second most common cause of death in persons with hemophilia in the United States. High blood pressure and other factors may increase your risk of ischemic heart disease.

If you have hemophilia, it is important to pay close attention to your blood pressure. Your doctor may ask you to monitor your blood pressure and to report readings that exceed a specific range. Always follow your doctor's advice concerning blood pressure.


References

American Heart Association. Blood Pressure. Available at: http://www.americanheart.org/presenter.jhtml?identifier=4473 Accessed December 13, 2005.

American Heart Association. Your High Blood Pressure Questions Answered—Low Blood Pressure. Available at: http://www.americanheart.org/presenter.jhtml?identifier=3034848  Accessed December 13, 2005.

American Heart Association. Am I at Risk? Available at: http://www.americanheart.org/presenter.jhtml?identifier=2142  Accessed December 13, 2005.

Kulkarni R, Soucie JM, Evatt BL. Prevalence and risk factors for heart disease among males with hemophilia [abstract]. Am J Hematol. 2005;79(1):36–42.


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

HEMOPHILIA CHAPTERS

 Chapter Name Location Information
National Hemophilia Foundation (NHF)  
www.hemophilia.org  
National Headquarters   National Hemophilia Foundation 116 West 32nd Street, 11th FL New York, NY 10001   Phone: (212) 328-3700 Fax: (212) 328-3777
ALABAMA  
Hemophilia and Bleeding Disorders of Alabama (HBDA)   www.hbda.us   151 Market Place Montgomery, AL 36117   Phone: 334-277-9446 Fax: 334-272-9167
ARIZONA  
Arizona Hemophilia Association  
www.hemophiliaz.org
 
818 East Osborn Rd., # 105 Phoenix, AZ 85014   Phone: (602) 955-3947 Toll Free: (888) 754-7017 Fax: (602) 955-1962
CALIFORNIA  
Central California Hemophilia Foundation (CCHF)  
http://www.cchfsac.org
 
PO Box 163689 Sacramento, CA 95816   Phone: 916-296-9066 Fax: 916.489.1569
Hemophilia Association of San Diego County (HASDC)  
www.hasdc.org
   
3570 Camino Del Rio, North Suite #108 San Diego, CA 92108   Phone: (619) 325-3570 Fax: (619) 325-4350 E-mail: info@hasdc.org  
Hemophilia Foundation of Northern California  
www.hemofoundation.org
 
6400 Hollis Street Suite 6 Emeryville, CA 94608   Phone: (510) 658-3324 Toll Free: (888) 749-4362 Fax: (510) 658-6111
Hemophilia Foundation of Southern California (HFSC)  
www.hemosocal.org
 
6720 Melrose Ave. Hollywood, CA 90038   Phone: (323) 525-0440 Toll Free: (888) 371-4123 (CA only) Fax: (323) 525-0445 E-mail: ofcmgr@hemosocal.org commadv@hemosocal.org
COLORADO  
Hemophilia Society of Colorado (HSC)  
www.cohemo.org
     
P.O. Box 4943 Englewood, CO 80155-4943   Phone: (888) 722-0233 or (888) 687-CLOT Fax: (303) 629-7035 E-mail: hsc@cohemo.org
CONNECTICUT  
* New England Hemophilia Association (NEHA)  
www.newenglandhemophilia.org
  Serve: MA, CT, RI, VT, NH and ME  
347 Washington Street, Suite 402 Dedham, MA 02026-1862   Phone: (781) 326-7645 Fax: (781) 329-5122 E-mail: info@newenglandhemophilia.org
DELAWARE
* Delaware Valley Chapter (DVC)  
www.hemophiliasupport.org   Serve: DA, PA
222 South Easton Road, Suite #122 Glenside, PA 19038   Phone: (215) 885-6500 Fax: (215) 885-6074 E-mail: hemophilia@navpoint.com  
DISTRICT OF COLUMBIA
Hemophilia Association of the Capital Area (HACA)  
www.hacacares.org
  Serve: northern Virginia, Washington, DC, and Maryland counties (Montgomery and Prince Georges).
10560 Main St. #PH-4 Fairfax, VA 22030   Phone: 703-352-7641 Fax: (703) 352-2145 E-mail: hacacares@aol.com
FLORIDA  
Florida Hemophilia Association  
www.floridahemophilia.org
 
18001 Old Cutler Road, # 501 Palmetto Bay, FL 33157   Toll Free: (888) 880-8330 E-mail: info@floridahemophilia.org
Hemophilia Foundation of Greater Florida, Inc (HFGF)  
www.hemophiliaflorida.org
1350 North Orange Avenue Suite 227 Winter Park, FL 32789   Toll Free: (800) 293-6527 E-mail: Hemofoundation@earthlink.net
GEORGIA  
Hemophilia of Georgia, Inc. (HoG)  
www.hog.org
 
8800 Roswell Road Suite 170 Atlanta, GA 30350   Phone: (770) 518-8272 Fax: (770) 518-3310 E-mail: mail@hog.org
IDAHO  
Hemophilia Foundation of Idaho  
http://idahoblood.org
or
http://blooddrop.org
1220 S. Vista Ave., Suite F Boise, ID 83705   E-mail: idahoblood@velocitus.net or info@idahoblood.org
ILLINOIS  
The Hemophilia Foundation of Illinois (HFI)  
www.hfi-il.org
332 S. Michigan Avenue, #1135 Chicago, IL 60604-4305 Phone: 312.427.1495
Fax: 312.427.1602
E-mail: info@hfi-il.org
INDIANA  
Hemophilia of Indiana, Inc. (HOII)  
www.hemophiliaofindiana.org
 
5170 E. 65th St., Suite 106 Indianapolis, IN 46220    Phone: (317) 570-0039 Toll Free: (800) 241-2873 Fax: (317) 570-0058
KANSAS  
Midwest Hemophilia Association (MHA)  
www.midwest-hemophilia.org
  Serve: KS, MO
PO Box 412866 Kansas City, MO 64141 800-431-7960
KENTUCKY  
Kentucky Hemophilia Foundation, Inc. (KHF)  
www.kyhemo.org
 
1850 Taylor Ave. Suite #2 Louisville, KY 40213   Phone: (502) 456-3233 Toll Free: (800) 582-2873 Fax: (502) 456-3234 E-mail: info@kyhemo.org  
LOUISIANA  
Louisiana Hemophilia Foundation (LHF)  
www.louisianahemophilia.org
 
3636 South Sherwood Forest Blvd. Suite 450 Baton Rouge, LA 70816-2285   Phone: (225) 291-1675 Fax: (225) 291-1679 E-mail: lahemophilia@hipoint.net
MAINE  
* New England Hemophilia Association (NEHA)  
www.newenglandhemophilia.org
  Serve: MA, CT, RI, VT, NH and ME
For details see Connecticut  
MARYLAND  
Hemophilia Association of the Capital Area (HACA)  
www.hacacares.org
  Serve: northern Virginia, Washington, DC, and Maryland counties (Montgomery and Prince Georges).
For details see District of Columbia  
MASSACHUSETTS  
* New England Hemophilia Association (NEHA)  
www.newenglandhemophilia.org
  Serve: MA, CT, RI, VT, NH and ME
For details see Connecticut  
MICHIGAN  
Hemophilia Foundation of Michigan (HFM)  
www.hfmich.org
 
1921 West Michigan Avenue Ypsilanti, MI 48197   Phone: 734-544-0015 Toll Free: (800)-482 -3041 Fax: 734-544-0095
MINNESOTA  
Hemophilia Foundation of Minnesota/Dakotas (HFMD)  
www.hfmd.org
  Serve: MN, ND, SD  
750 South Plaza Drive # 207 Mendota Heights, MN 55120   Phone: (651) 406-8655 Toll Free: (800) 994-4363 Fax: (651) 406-8656 E-mail: hemophiliafound@visi.com
MISSOURI  
Midwest Hemophilia Association  
www.midwest-hemophilia.org
  Serve: KS, MO
For details see Kansas
MONTANA  
Rocky Mountain Hemophilia and Bleeding Disorders Association (RMHBDA)  
www.rockymountainhemophilia.org
 
2100 Fairway Dr., Suite 107 Bozeman, MT 59715
OR 8278 Chapman Road Bozeman, MT 59718   Phone: (406) 586-8674 Fax: (406) 388-5650
NEBRASKA  
Nebraska Chapter of the National Hemophilia Foundation  
www.nebraskanhf.org
 
215 Centennial Mall South Suite 512 Lincoln, NE 68508   Phone: (402) (742-5663) Fax: (402) 742-5677 E-mail: Office@nebraskanhf.org
NEVADA  
Hemophilia Foundation of Nevada (HFNV)  
www.hfnv.org
 
1850 Whitney Mesa, Suite 150 Henderson, NV 89014   Phone: (702) 564-4368 Fax: (702) 739-3200 E-mail: info@hfnv.org
NEW HAMPSHIRE  
* New England Hemophilia Association (NEHA)  
www.newenglandhemophilia.org
  Serve: MA, CT, RI, VT, NH and ME
For details see Connecticut  
NEW MEXICO  
Sangre de Oro, Inc., Hemophilia Foundation of New Mexico PO Box 51494 Albuquerque, NM 87181-1494   Phone: (505) 341-9321 Toll free: (866) 341-9321 Fax: (505) 292-5818 E-mail: sangredeoro@comcast.net
NEW YORK  
Bleeding Disorder Association of Northeastern New York (BDANENY)  
www.bdaneny.org
 
61 Main Street Petersburg, NY 12138   Phone: (518) 782-9787 Fax: (518) 356-5612 E-mail: bdaneny@bdaneny.org
Hemophilia Center of Western New York, Inc.  
www.hemophiliawny.com
 
936 Delaware Avenue Buffalo, NY 14209   Phone: (716) 896-2470 Fax: (716) 898-5537 E-mail: hemoctr@pce.net
Mary M. Gooley Hemophilia Center  
http://www.hemocenter.org
 
1415 Portland Avenue # 500 Rochester, NY 14621   Phone: (585) 922-5700  Fax: (585) 922-5775 Email: robert.fox@rochestergeneral.org
New York City Hemophilia Chapter (NYHC)  
www.nyhemophilia.org   Serve: Long Island, Staten Island, Westchester, Queens and Brooklyn
358 Fifth Ave, Suite 401 New York, NY 10001   Phone & Fax: (212) 877-0280 Email: melissa.penn@nyhemophilia.org
NORTH CAROLINA
Hemophilia of North Carolina (HNC)  
http://www.hemophilia-nc.org
260 Town Hall Drive, Suite A Morrisville, NC 27560   Phone: 919-319-0014 Toll Free: (800) 990-5557 Fax: 919-319-0016 E-mail: info@hemophilia-nc.org
NORTH DAKOTA
Hemophilia Foundation of Minnesota/Dakotas (HFMD)  
www.hfmd.org
  Serve: MN, ND, SD
For details see Minnesota
OHIO  
Central Ohio Chapter of NHF  
www.nhfcentralohio.org
834 West Third Avenue, #A Columbus, OH 43212   Phone: 614-429-2120 Fax: 614-429-2150
Tri State Bleeding Disorders Foundation, A Chapter of NHF (TSBDF)  
www.tsbdf.com
   
635 W. Seventh Street, # 407 Cincinnati, OH 45203   Phone: (513) 961-4366 Fax: (513) 961-1740 E-mail: hemophilia@fuse.net
Northern Ohio Hemophilia Foundation (NOHF)  
www.nohf.org
 
One Independence Place 4807 Rockside Road, # 380 Cleveland, OH 44131   Phone: (216) 834 - 0051 Toll Free: (800) 554 -4366 Fax: (216) 834 - 0055
Northwest Ohio Hemophilia Foundation  
www.nwohemophilia.org
 
P.O. Box 12606 Toledo, OH 43606   Phone: (419) 291-5882 Fax: (419) 479-3269
Southwestern Ohio Hemophilia Foundation  
www.swohiohemophilia.org
3131 South Dixie Dr., # 103 Moraine, OH 45439   Phone: 937-298-8000 Fax: 937-298-8080 E-mail: info@swohiohemophilia.org
OKLAHOMA  
Oklahoma Hemophilia Foundation (OHF)  
www.okhemophilia.org
 
PO Box 30242 Edmond, OK 73003
OREGON  
Hemophilia Foundation of Oregon (HFO)  
www.hfo.info
 
5319 SW Westgate Drive, Suite 126 Portland, OR 97221   Phone: 503-297-7207 Fax: 503-297-0127
PENNSYLVANIA  
* Delaware Valley Chapter (DVC)  
www.hemophiliasupport.org   Serve: DE, PA
For details see Delaware
Western Pennsylvania Chapter of NHF  
www.westpennhemophilia.org
 
20411 Rt. 19, Unit 14 Cranberry Twp., PA 16066   Phone: 724-741-6160 Fax: 724-741-6167 E-mail: wpcnhf@earthlink.net
RHODE ISLAND  
* New England Hemophilia Association (NEHA)  
www.newenglandhemophilia.org
  Serve: MA, CT, RI, VT, NH and ME
For details see Connecticut  
SOUTH CAROLINA  
Hemophilia of South Carolina PO Box 2386 Irmo, SC 29063   Phone: (888) 829-4849 Fax: (888) 829-4849 E-mail: ofcmgr@hemosocal.org
SOUTH DAKOTA  
Hemophilia Foundation of Minnesota/Dakotas (HFMD)  
www.hfmd.org
  Serve: MN, ND, SD
For details see Minnesota
TENNESSEE  
Tennessee Hemophilia and Bleeding Disorders Foundation (THBDF)  
www.thbdf.org
203 Jefferson Street Smyrna, TN 37167   Phone: (615) 220-4868 Fax: (615) 220-4889 E-mail: mail@thbdf.org
TEXAS  
* Lone Star Chapter of NHF  
www.lonestarhemophilia.org  
10500 Northwest Freeway, # 226 Houston, TX 77092   Phone: 713-686-6100 Fax: 713-686-6102
Texas Central Hemophilia Association, Inc. (TexCen)  
www.texcen.org
 
3530 Forest Lane, Suite 311 OR 12700 Hillcrest Rd., Suite 191 Dallas, TX 75230   Fax: 972-386-4211 E-mail: mail@texcen.org
UTAH  
Utah Hemophilia Foundation (UHF)  
www.hemophiliautah.org
772 East 3300 South, #210 Salt Lake City, UT 84106   Phone: (801) 484-0325 Fax: (801) 484-4177
VERMONT  
* New England Hemophilia Association (NEHA)  
www.newenglandhemophilia.org
  Serve: MA, CT, RI, VT, NH and ME
For details see Connecticut  
VIRGINIA  
Hemophilia Association of the Capital Area (HACA)  
www.hacacares.org
  Serve: northern Virginia, Washington, DC, and Maryland counties (Montgomery and Prince Georges).
For details see District of Columbia  
Virginia Hemophilia Foundation  
www.vahemophilia.org    
1505 Leewal Ct. Richmond, VA 23238   Phone: (804) 740-8643 Toll Free: (800) 266-8438 Fax: (804) 740-8643 E-mail: vahemophiliaed@verizon.net
WASHINGTON  
Bleeding Disorders Foundation of Washington (BDFWA)  
www.bdfwa.org
9659 Firdale Avenue Edmunds, WA 98020   Phone: (206) 533-1660 Fax: (206) 533-1686
WISCONSIN  
Great Lakes Hemophilia Foundation (GLHF)  
www.glhf.org
 
638 N. 18th Street, Suite 108 Milwaukee, WI 53233   Phone: (414) 257-0200 Toll Free (888) 797-4543 Fax: (414) 257-1225 E-mail: glhf@execp.com
WYOMING  
Rocky Mountain Hemophilia and Bleeding Disorders Association  
www.rockymountainhemophilia.org
  Serve: MO & WY
For details see Montana  
DRUGS & CONDITIONS

SUPPORT FOUNDATIONS FOR HEMOPHILIA

The World Federation of Hemophilia is an international, not-for-profit organization located in Montréal, Canada. The organization was founded in 1963 by Frank Schnabel. The World Federation of Hemophilia is dedicated to introducing, improving and maintaining care for persons with hemophilia and related disorders. Staff and volunteers help member organizations, healthcare providers and governments deliver quality care. The organization adapts its work to fit the needs of each country and helps develop hemophilia services, provides workshops, trains fellows, provides education, fund raises and works to ensure that at least $8.00 of hemophilia care is provided from every dollar received. You can contact the organization at:

World Federation of Hemophilia
1425 René Lévesque Boulevard West, Suite 1010
Montréal, Quebec
Canada, H3G 1T7
Tel: (514) 875-7944
Fax: (514) 875-8916
Email: wfh@wfh.org
Website: http://www.wfh.org/

The National Hemophilia Foundation is based in the United States. This organization is committed to finding the cures for hemophilia and other inherited bleeding disorders and to prevent and treat the complications of these disorders. Through its national staff and network of community chapters, the organization provides education, advocacy and research. The National Hemophilia Foundation maintains HANDI, an information clearinghouse on hemophilia and related disorders. You can contact the organization at:

National Hemophilia Foundation
116 West 32nd Street, 11th Floor
New York, NY 10001
Phone: (212) 328-3700
Fax: (212) 328-3777
HANDI Phone: (800) 42-HANDI
HANDI Fax: (212) 328-3799
Email Webmaster: webmaster@hemophilia.org
Email HANDI: handi@hemophilia.org
Website: http://www.hemophilia.org/

Local Hemophilia Foundations
Many states have local chapters loosely affiliated with the NHF. These chapters are advocates for patients and their families with bleeding disorders and can be a good source for local information and support. Many have social activities, which provide a forum for individuals to meet one another and share experiences. They also have educational seminars. Local chapters can be located on-line.


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

HEMOPHILIA: TELLING YOUR FAMILY

Your child has been diagnosed with hemophilia. Or perhaps you've been diagnosed with this disease yourself. Now its time to tell your family. How will they react? Adults and children react to the news of a family member's chronic illness in many different ways. However, it is likely that each person's reaction will change over time, and will eventually include, to some extent, each of these feelings:

Denial: "No, it can't be true!"
Anger: "Why you? Why our family?"
Bargaining: "I'll do anything, if only this can be changed. Anything!"
Depression: "I feel hopeless. I'll never feel joy again."
Acceptance: "This is happening and we will cope with it."

Taken together, these feelings comprise what is known as the "grief cycle." The grief cycle is the normal emotional process all people go through when confronted with a significant loss. Do you recognize these feelings? Chances are, as the parent or the patient, you can place yourself somewhere on the chart above right now. That's because you are in the process of grieving your own or your child's illness.

There is no "right" or "wrong" way to grieve the loss of one's own health or that of a family member. The grief cycle has been likened to a roller coaster or a slippery slope. It's unpredictable! The phases of the grief cycle may occur in any order and can last for varying amounts of time. The phases usually also repeat. It is even possible to experience all of these feelings in the same day!

Depression is the turning point in the grief cycle. It is the classic sign of grief. Depression embraces the reality of the diagnosis. Once the diagnosis is fully grieved it can be fully accepted. That's why even though depression feels bad, it's actually a good thing. Depression is the gateway to acceptance. With acceptance, comes the energy to deal with the challenges of the illness.

It is possible to get "stuck" in any phase of the grief cycle. Prolonged anger, depression or denial aren't healthy because they keep the grieving person from arriving at the acceptance phase. In the context of a family, this can affect everyone. Serious ongoing depression, denial or anger on the part of any family member may require a therapist's help.

Telling Your Family

If you are married, your spouse probably already knows the diagnosis. If not, he or she should be the first to know. Together, you can tell the rest of your family. Here are a few tips:

Timing

Don't share this news at a time when family members are hungry, tired, or upset about some other issue. Choose a time when everyone in the immediate family can be present. Take the phone off the hook.

Detach

Prepare for this discussion by "detaching"-- stepping back emotionally from the situation. Let go of your expectations about how you would like your family to respond. Give your family the freedom to feel and say whatever they need to. You may witness great emotion or no emotion at all--but don't take it personally. People handle the news of a chronic illness in different ways. Understanding and support develop over time.

Telling Children

Use language that is appropriate to the child's age. Keep the explanation simple. Make sure children know that they can't "catch" hemophilia the way they can catch a cold. For school aged children, write the words "hemophilia" on a piece of paper so they can begin to recognize the name of the disease. Young children sometimes tend to blame themselves for things they cannot understand, so it is important to tell them that hemophilia is not their fault.

Telling Teenagers

On the surface teenagers may appear "cool" or may not seem to react at all. Inside, however, they may feel worried, afraid, embarrassed or have other emotions they don't want to talk about. They may have many questions they aren't ready to ask. Stay positive and open. Leave written materials about hemophilia on the coffee table where they can access them casually later.

Telling Parents, In-laws, Adult Children and Close Relatives

Parents, in-laws, siblings, grown children or other close relatives may respond in a variety of ways. Younger adult relatives, especially, may assume a caretaking role. They may want to step in and help or even manage the situation. Parents and in-laws who are elderly or in poor health themselves may feel panicked or burdened. They may feel that they are expected to provide support they are not capable of providing. The amount of help and support that will be appreciated and appropriate depends upon your relationship with the relative, your actual needs, and their actual ability to be supportive. Ongoing discussions can help set boundaries that everyone can be comfortable with.


Last Modified Date: July 31, 2009 © Accordant, A CVS Caremark Company. All rights reserved.

This article has been reviewed for accuracy by a member of the Accordant Health Services Medical Advisory Team.

This information is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting with a qualified healthcare provider. Please consult your healthcare provider with any questions or concerns you may have regarding your condition. Use of this online service is subject to the disclaimer and the terms and conditions.

DRUGS & CONDITIONS

ERIC DELSON MEMORIAL SCHOLARSHIP

Supporting and Encouraging Education For Students With Bleeding Disorders

The Eric Delson Memorial Scholarship is intended for college, vocational or private school students that are clinically diagnosed with hemophilia or von Willebrand disease. Three renewable $2,500 scholarships are awarded each year for new or current college or vocational-technical school students, and one renewable $1,500 scholarship is awarded to a student attending private school, grades 7-12.

This scholarship program is administered by Scholarship Management Services, a department of Scholarship America. Scholarship Management Services is the nation’s largest designer and manager of scholarship and tuition reimbursement programs for corporations, foundations, associations and individuals. Awards are granted without regard to race, color, creed, religion, sexual orientation, age, gender, or disability.

Students interested in applying must complete a college or private school application and send it with a current transcript of grades to Scholarship America, postmarked no later than July 1.

The Eric Delson Memorial Scholarship Program
Scholarship Management Services
One Scholarship Way
Saint Peter, Minnesota 56082

Who is Eric Delson?

The Eric Delson Memorial Scholarship was established in 1993 in honor of Eric Delson, former Vice President of Marketing and Clinical Services. As a person with hemophilia, Eric did not allow challenges to deter him from any goals he set out to achieve. Although limitations were well-known to Eric, these same limitations were the fuel that drove him to enhance the lives of others who were living with hemophilia. Through his example of leadership, commitment, determination and compassion, Eric’s positive influence touched many who had the privilege of knowing him and others who did not.