Managing Delivery In Women With Vwd
In patients with low VWF or type 1 VWD, VWF and FVIII levels increase during pregnancy, especially in the third trimester. At the time of delivery, these levels can be 2 to 3 times higher compared with baseline levels. However, some women, even with mild-type 1 VWD, do not show this increase. In type 2 VWD patients, VWF:Ag and FVIII levels also increase during pregnancy, but functional VWF remains low. Levels of VWF and FVIII in VWD type 3 remain low throughout pregnancy and the women should therefore always be treated with factor concentrate at delivery. Because of the unpredictable increase of levels during pregnancy, it is necessary to measure VWF:Ag, VWF:RCo , and FVIII between 30 and 34 weeks of pregnancy. To prevent bleeding during delivery and postpartum, current guidelines for women with VWD state that if the functional levels of VWF and FVIII are < 50 IU/dL during the third trimester, then desmopressin or IV VWF/FVIII concentrate should be administered during vaginal and caesarean delivery. Desmopressin can only be administered in women with a previously demonstrated good response to FVIII and VWF and should be used with caution as was discussed in Managing the delivery in carriers of hemophilia.
How Is Hemophilia Treated
Hemophilia treatment centers can provide full care to kids with hemophilia. Check the CDC’s website to find locations throughout the U.S.
The medical care team for kids with hemophilia usually includes:
- a hematologist
- a nurse who specializes in conditions related to bleeding
- physical therapists to help with strengthening and stretching and joint health
- social workers and/or psychologists to help kids and parents cope with hemophilia
The main treatment for hemophilia is factor replacement therapy. This gives the body the clotting factor it needs. It is given intravenously . A person may get factor replacement on a regular schedule or as a treatment for bleeding. Several products are now available. Talk about treatment options with the hemophilia care team.
Factor replacement can be done:
- at a hospital or hemophilia treatment center
- at home by a nurse
- at home by the person with hemophilia or someone else, after being trained
Doctors also can give medicines to help control bleeding during procedures and surgeries.
Some people with hemophilia develop inhibitors . Their bodies see the new clotting factor as an invader and develop antibodies that block its clotting action. This can make the hemophilia hard to treat, and requires different factor replacement therapy.
Families With A History Of Hemophilia
Any family history of bleeding, such as following surgery or injury, or unexplained deaths among brothers, sisters, or other male relatives such as maternal uncles, grandfathers, or cousins should be discussed with a doctor to see if hemophilia was a cause. A doctor often will get a thorough family history to find out if a bleeding disorder exists in the family.
Many people who have or have had family members with hemophilia will ask that their baby boys get tested soon after birth. In the best of cases, testing for hemophilia is planned before the babys delivery so that a sample of blood can be drawn from the umbilical cord immediately after birth and tested to determine the level of the clotting factors. Umbilical cord blood testing is better at finding low levels of factor VIII than it is at finding low levels of factor IX . This is because factor IX levels take more time to develop and are not at a normal level until a baby is at least 6 months of age. Therefore, a mildly low level of factor IX at birth does not necessarily mean that the baby has hemophilia B. A repeat test when the baby is older might be needed in some cases. Learn more about the inheritance pattern for hemophilia.
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When Should I Seek Immediate Care Or Call 911
- Your child has a head injury or a seizure.
- Your child has bleeding from a injury to his throat, neck, or eyes.
- Your child has a bleeding episode that cannot be controlled.
- Your child has chest pain or trouble breathing.
- Your child has many large bruises on his body, or swelling in his joints.
- Your child has joint pain that lasts longer than 3 days.
- Your child has severe hemophilia and has pain in the lower part of his stomach, groin, or lower back.
- Your child is vomiting blood or has blood in his bowel movement.
What Are The Symptoms Of Hemophilia
The most common symptom of this disorder is heavy, uncontrollable bleeding.
The severity of hemophilia depends on the amount of clotting factors in the blood. Those affected with hemophilia that have levels greater than 5% most often have bleeding only with major surgeries or tooth extractions. These children may not even be diagnosed until bleeding complications from a surgery occur.
Severe hemophilia is when the factor VIII or IX is less than 1%. Bleeding can occur in these children, even with the minimal activities of daily life. Bleeding may also occur from no known injury. Bleeding most often occurs in the joints and in the head.
Your child’s symptoms may also include:
The symptoms of hemophilia may look like other problems. Always check with your child’s doctor for a diagnosis.
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The Genetics Of Hemophilia
Hemophilia A is an inheritable disease, meaning it is passed down from parents to children. The X and Y chromosomes are called sex chromosomes. The gene for hemophilia is carried on the X chromosome. Hemophilia is inherited in an X-linked recessive manner. Females inherit two X chromosomes, one from their mother and one from their father . Males inherit an X chromosome from their mother and a Y chromosome from their father . That means if a son inherits an X chromosome carrying hemophilia from his mother, he will have hemophilia. It also means that fathers cannot pass hemophilia on to their sons.
But because daughters have two X chromosomes, even if they inherit the hemophilia gene from their mother, most likely they will inherit a healthy X chromosome from their father and not have hemophilia. A daughter who inherits an X chromosome that contains the gene for hemophilia is called a carrier. She can pass the gene on to her children. Many women who carry the hemophilia gene also have low factor expression, which can result in heavy menstrual bleeding, easy bruising, and joint bleeds. Some women who have the hemophilia gene have factor expression low enough to be diagnosed with hemophilia.
For a female carrier, there are four possible outcomes for each pregnancy:1. A girl who is not a carrier2. A girl who is a carrier3. A boy without hemophilia4. A boy with hemophilia
Hemostatic Changes In Healthy Women During Pregnancy And Delivery
During pregnancy, the hemostatic balance is changing toward a procoagulant state in order to be prepared for blood loss during delivery and the postpartum period. This procoagulant state is caused by a physiological increase of many coagulation factors, including factor VIII and von Willebrand factor with a peak shortly before giving birth. The procoagulant state is also characterized by a decrease of anticoagulant proteins and reduced fibrinolysis. Twelve weeks postpartum, these changes have returned to normal. The physiological increase in FVIII, VWF, and, to a lesser extent, FIX during pregnancy are relevant for the management of pregnant carriers of hemophilia and women with VWD.
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How Is Hemophilia A Treated
Most treatments for hemophilia A focus on replacing the missing protein, FVIII , so a person can form a clot, and so reduce or eliminate the bleeds associated with the disorder. Treatments that work to prevent bleeding through new mechanisms have recently come to the market or are in clinical trials. People with hemophilia A have several different medication options for treatment.
The main medication to treat hemophilia A is concentrated FVIII product, called clotting factor or simply factor. There are two types of clotting factor: plasma-derived and recombinant. Plasma-derived factor is made from human plasma. Recombinant factor products are developed in a lab through the use of DNA technology. While plasma-derived FVIII products are still available, approximately 75% of the hemophilia community takes a recombinant FVIII product.
These factor therapies are injected into a vein in the arm or hand, or through a port in the chest. The NHF’s Medical and Scientific Advisory Council encourages the use of recombinant clotting factor products over plasma-derived because they are safer from blood-borne viruses and diseases.
What Is Hemophilia B
If your child has hemophilia, their blood doesn’t clot like it should. If they have a cut, scrape, or other injury, they will bleed longer than other people do. The bleeding can happen both on the surface or inside their body.
It can be serious. With the right treatment, though, and by avoiding certain risks, your child can have an active life.
People with hemophilia donât have enough of a protein that helps blood clot. There are two main types of the condition: A and B. Your child’s type depends on which protein, or clotting factor, they lack.
There are 13 of these clotting factors in our blood. With hemophilia B, you don’t have enough factor IX. More than half the people with it have a severe case they have almost no factor IX in their blood.
Diagnosis usually comes in early childhood. Only about 2 in 10 people who have hemophilia have this type. It happens more in boys than girls.
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How Is Hemophilia Diagnosed
Your doctor will perform a physical examination to rule out other conditions. If you have symptoms of hemophilia, the provider will ask about your familys medical history since this disorder tends to run in families. In children with severe hemophilia, the diagnosis is usually made in infancy. This might happen at the time of circumcision or when the toddler starts to walk and develops excessive bleeding or bruising with minor trauma.
Blood tests are then performed to determine how much factor VIII or factor IX is present. These tests will show which type of hemophilia you have and whether it is mild, moderate, or severe, depending on the level of clotting factor in the blood:
- People who have 5%-30% of the normal amount of clotting factors in their blood have mild hemophilia.
- People with 1%-5% of the normal level of clotting factors have moderate hemophilia.
- People with less than 1% of the normal clotting factors have severe hemophilia.
Your doctor may request that other family members have a Factor VIII level drawn to determine if they are affected. In some cases genetic testing may be necessary.
What Is Our Approach In A Hemophilia Carrier During Delivery
Based on the observed high prevalence of PPH, the Dutch guideline was recently updated with the aim of lowering the risk of bleeding by targeting higher factor levels at delivery. Therefore, the cutoff value of FVIII or FIX, measured in the third trimester for treatment with factor concentrates, was increased from 50 IU/dL to 80 IU/dL. In addition, the targeted peak level at delivery was increased from 100 to 150 IU/dL. After initial treatment, trough levels are kept > 50 IU/dL for 3 days and for 5 days . In addition, after delivery, we give 1 g of oral tranexamic acid 3 to 4 times daily for 7 days. Because our guideline differs from other international guidelines, outcome data will be prospectively collected and evaluated .
Are There Home Treatments For Bleeds In People With Hemophilia
In most cases a child with severe hemophilia is managed at home by his parents, with the administration of factor on a prophylactic schedule. As the child gets older, he is taught how to self-administer his factor on schedule or when an acute bleed occurs. At the time of a joint bleed, you can treat the pain using the RICE protocol.
Pain medications are limited in hemophilia because common pain relievers such as aspirin, ibuprofen and naproxen can aggravate bleeding. If you or your child is suffering from pain related to an injury, you can use acetaminophen .
Longitudinal Multidisciplinary Care Coordinated By A Hemophilia Treatmentcenter
The care of a newborn with hemophilia begins at the diagnosis of pregnancy or, in somecases, before conception, and continues through delivery and the neonatal period. Manypractitioners will be involved: pediatric hematologists, adult hematologists, hemophilianurses, obstetricians and maternal-fetal specialists, obstetrics nurses, geneticcounsellors, neonatologists, pediatricians, radiologists, and experts in blood banking andlaboratory medicine. Parents may wish for care to be provided by midwives or doulas thereis no reason to exclude these practitioners, but they should not be considered substitutesfor medical professionals with expertise in high-risk obstetrics, neonatology, andhemophilia care. The parents of the newborn are essential members of this team, and theirpreferences should be solicited and considered throughout.
Ensuring this multidisciplinary care should be the guiding principle, and the HemophiliaTreatment Center has a central role in coordinating care during pregnancy, delivery,and the newborn period. Care at an HTC reduces mortality and improves outcomes in patientswith hemophilia,, although this has not been specifically demonstrated for newborns or for hemophiliacarriers.
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Families With No Previous History Of Hemophilia
About one-third of babies who are diagnosed with hemophilia have no other family members with the disorder. A doctor might check for hemophilia in a newborn if:
- Bleeding after circumcision of the penis goes on for a long time.
- Bleeding goes on for a long time after drawing blood and heel sticks .
- Bleeding in the head after a difficult delivery or after using special devices or instruments to help deliver the baby .
- Unusual raised bruises or large numbers of bruises. If a child is not diagnosed with hemophilia during the newborn period, the family might notice unusual bruising once the child begins standing or crawling.
Those with severe hemophilia can have serious bleeding problems right away. Thus, they often are diagnosed during the first year of life. People with milder forms of hemophilia might not be diagnosed until later in life.
What Are The Signs And Symptoms Of Hemophilia
Bleeding can occur anywhere in your child’s body. It may happen in places you can see, such as the nose or mouth. It can also happen inside his body and may cause pain, swelling, and bruising. He may have any of the following signs and symptoms:
- Frequent bruising
- Frequent bleeding from the nose or gums
- Pain and swelling in joints or muscles
- Bleeding that lasts a long time
- Bowel movements that are black
- Urine that is pink or red
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