Family Planning
Many families now consider how to best plan for their pregnancies, their deliveries, and their families when there is a known family history or suspected family history of Hemophilia. The life expectancy and quality of life amongst those living with Hemophilia have dramatically improved. For families where the risk to have a child with Hemophilia or for biological female who may be symptomatic carriers, consideration for how they would manage a conception, a pregnancy or a delivery may be different to those who do not have such a history. This section is intended to provide a broad overview of the reproductive options that exist for individuals with or at risk for Hemophilia. We encourage you to have a more detailed discussion with your healthcare providers who can refer you to a high-risk OB/GYN (Maternal-Fetal Medicine) who specializes in blood disorders, a Genetic Counselor who has a particular interest in blood disorders, or a Reproductive Endocrinologist.
Just as every individual makes their own decisions, so much does each family as they consider what is in their best interests as it relates to their reproductive options. Open and respectful conversations with partners, and professionals often lend an opportunity for you to explore what is important for you in wanting information and what you would do with that information once it is obtained.
For example, patients may choose to learn whether their baby has Hemophilia because it will help them not only prepare for that child’s birth but also allow for a safer delivery. For others, perhaps the knowledge will put their minds at ease, regardless of the result, removing the question and allowing them to enjoy their pregnancy. For others, maybe a diagnosis of an affected child would prevent them from continuing their pregnancy and they would choose termination. Perhaps an individual would choose to use an egg donor or a sperm donor to remove the risk of having an affected child altogether. And, finally, some individuals will choose adoption or child-free families. This is not an all-inclusive list but offers some examples to start with. There are no “correct” answers but talking about what is important will allow you to best assess what will be the right decision for you.
Preconception
Ideally, planning for pregnancy starts before conception. It is a good idea to have a preconception care checkup to ensure your own health status before you get pregnant. The goal of this checkup is to help increase your chances of having a healthy pregnancy and a healthy baby.
During this visit, your provider will ask about your medical and family history, your diet and lifestyle, medications you take, and any other past pregnancies.
Your provider will also encourage you to maintain good general health. The best way to ensure good health is to follow some simple guidelines. These include:
- Eat a balanced and healthy diet that includes fresh fruits and vegetables, especially dark leafy greens.
- Get plenty of rest.
- Participate in regular physical activity.
- Manage stress.
- Adopt a smoke-free, drug-free lifestyle and keep alcohol use to a minimum.
- Avoid taking certain medications.
Your provider may also recommend that if you have not already been vaccinated you should be immunized against hepatitis A and hepatitis B. This is in case you might need a transfusion during delivery.
What to Expect BEFORE Pregnancy
If you, the father of your baby, or any close family member have a history of an inherited bleeding disorder, you may have a higher chance of having a baby with a disorder, too. When considering a pregnancy, you will want to discuss this with your provider. As suggested, it may be recommended that you speak with a Genetic Counselor to discuss inheritable conditions. This helps you to discuss what preconception or prenatal options might be best for you and your pregnancy, as well as discuss the other aspects of pregnancy management.
What you need to do BEFORE you become pregnant
When considering a pregnancy, the best preparation starts with getting the right advice and assistance before you become pregnant. The treatment that your providers recommend will depend on your bleeding disorder, your overall health, your partner’s health, and your pregnancy.
First, seek out specialized, integrated, and comprehensive care. Your HTC or hematologist should be able to connect you with a Maternal-Fetal Medicine (MFM) physician or an OB/GYN who specializes in high-risk pregnancies to create a pregnancy management plan. When meeting with your MFM or OB/GYN, here are some questions you will want to ask:
- Find out more about any potential risks you may have of bleeding and/or clotting during and after delivery.
- Find out about any potential risks of bleeding complications to the fetus during birth, and what types of monitoring techniques and deliveries should be used to avoid bleeding.
- Meet and consult with a genetic counselor to find out more about the inheritance of your disorder and what prenatal diagnostic options are available.
- Ask about your options for pain management during labor.
- Find out when and if a caesarean section (also called a C-section) is advised during delivery. If it is, you may require treatment to raise factor levels prior to your procedure.
- Learn more about whether it will be necessary for you to receive treatment to prevent postpartum hemorrhage after delivery and what that treatment will be.
- Seek additional counseling during your pregnancy to receive information on lifestyle advice related to your disorder beyond your pregnancy and additional information that may help you and your child.
Pregnancy and Delivery
What to expect DURING pregnancy
Your care will be carefully managed in conjunction with other specialists including a hematologist. During your pregnancy your clotting factor levels will be checked. If your clotting factor levels are low before an invasive test or before delivery, you may need treatment with clotting factors. Sometimes a medication called Desmopressin is given for this purpose. Additionally, women with von Willebrand disease (VWD) have a greater risk of bleeding during pregnancy. It is important to notify your doctor of any signs of bleeding while pregnant. During this time, you also will be referred to see an anesthesiologist to discuss your options for pain relief during labor, including an epidural.
What to expect DURING labor and delivery
Many measures will be taken to reduce the risk of bleeding to you and your baby. If your baby is at risk of bleeding, there are certain procedures that are generally avoided during labor and delivery. These procedures include an internal fetal heart rate monitor and delivery by vacuum extraction or forceps. If your baby is at risk of severe bleeding, your doctor will likely recommend delivery by cesarean and in a specialized center.
Creating a Birth Plan
A birth plan is a document that lets your medical team know your preferences for things such as how to manage labor pain. Keep in mind that you can’t control every aspect of labor and delivery, and you’ll need to stay flexible in case something comes up that requires your birth team to not follow the plan. However, a printed document gives you a place to make your wishes clear. It can also help you make sure that all procedures and medications are clearly explained to you before being given to you and/or your baby. Most hospitals provide a birth plan worksheet or brochure to explain their policies and to let you know what your options might be. That information can help you and your provider discuss your labor and delivery preferences and options.
Some items to consider when creating your birth plan are:
During Labor:
☐ How will the fetus be monitored?
☐ What type of pain medication is safe for my baby and me?
☐ Will I be able to have an epidural?
☐ In what circumstances will I need to have a cesarean delivery?
After Delivery:
☐ How will the umbilical cord be handled?
☐ Should I bank by baby’s cord blood?
☐ If my baby is a boy, circumcision should be delayed until the status is known.
The management of childbirth will depend on your needs and your potentially affected infant at the time of delivery. If you or your baby are at risk for severe bleeding, it is recommended that you seek prenatal care and delivery in a state-of-the-art facility where, in addition to specialists in high-risk obstetrics, there is a hematologist with expertise in hemostasis.
Many hospitals as well as HTCs offer specialized care for pregnancies involving bleeding disorders, and have the resources required to address serious bleeding problems if needed (i.e., laboratory, blood bank, transfusion services, and pharmacy).
Pain Relief During Labor
An epidural anesthetic provides pain relief during labor. Since bleeding can occur in the epidural space, anesthesiologists usually refrain from giving a female with a bleeding disorder an epidural or spinal anesthetic unless her factor levels are continuously higher than 50 percent of normal. If you need pain relief while in labor, and do not have levels that are continuously higher than 50 percent of mild, narcotic pain relievers can be used instead. If you require a cesarean delivery, a general anesthetic can also be used instead of a spinal.
Postpartum
What to expect AFTER delivery
If your clotting factors are low around the time of delivery, you may need treatment with clotting factors for a few days following delivery to reduce your risk of bleeding. A cord blood sample will be taken from the umbilical cord after delivery to check if the baby is affected or not. It may be recommended that your baby receives Vitamin K by mouth rather than injection. And, if you are planning on having your baby boy circumcised, this should be delayed until the clotting status of your baby is known. Last, some babies may need a special scan of their brain to ensure that there is no bleeding inside of the baby’s head after delivery.
Abnormal bleeding AFTER delivery
Excessive bleeding from the uterus, or postpartum hemorrhage, is a concern for many females who have a bleeding disorder. It can occur soon after delivery but may occur as late as 1 month afterward. Females with bleeding disorders are particularly vulnerable to delayed postpartum hemorrhage, the type that occurs more than 24 hours after childbirth.
Blood loss is considered not normal or excessive if one of the following occurs within 24 hours of delivery:
- More than 2 pints of blood are lost
- You experience symptoms along with the blood loss of low blood pressure, rapid heart rate, dizziness, light-headedness, fatigue, and weakness
The most common cause of excessive bleeding at delivery is when the uterus does not contract as it should. Other times, excessive bleeding can also result from incisions or tears.
Preventing postpartum hemorrhage
One of the most important things you can do to decrease the risks of postpartum hemorrhage is to make sure that a knowledgeable physician or hematologist is caring for you. Before going into labor, your physician should take steps to prevent or to prepare for excessive bleeding after delivery by ensuring that your factor levels are sufficient. After delivery, your team will monitor you closely to make sure that the uterus has contracted and to assess bleeding.
Treatment for postpartum hemorrhage
If excessive bleeding occurs, your provider will perform measures to address the problem. If the bleeding continues, additional medications, blood products, and even surgery may be required.