PUSHING YOUR LIMITS AND MAKING CHOICES
As a teenager, your life is probably rapidly changing in many ways. You’re looking and feeling differently, becoming more independent, making new friends, pushing your curfew… As hard as it may be sometimes, you also need to think seriously about the choices you make for your health. Your parents won’t always be there the moment you need them – and as you continue to grow, so will your responsibilities.
At this age, it’s natural that you and your friends want to test your limits by trying new things. But as a teen with hemophilia, you will have to carefully consider your behaviour and the activities you participate in. Is what you are about to do too risky? It may be hard to not be carefree like your friends, and do all the things you want. But always think: is it worth it? Is this behaviour putting my health at risk? Your health is the most important thing: and good friends will be the ones who understand the compromises you may have to make to put your health first.
TAKING CHARGE OF YOUR HEALTH
The biggest step towards your independence is learning to manage your treatment. If you take clotting factor therapy and haven't learned how to infuse it on your own, now is a good time to start learning. Taking on this responsibility can help increase your confidence and independence. Here are some other important ways you can start taking charge of your hemophilia, and your health:
➥ If you're on prophylaxis, stick to your treatment routine
➥ Before playing a sport or doing something active that may cause a bleed, infuse clotting factor
➥ If you have a bleed, treat it immediately
➥ When you’re away from home – maybe on a school trip or a sleepover – take your supplies with you
➥ Program important numbers into your cell phone—including your clinic's number and nearest emergency room
Being prepared and using common sense are how you can demonstrate to your parents that you can enjoy more freedom and manage your hemophilia. Go Back
STAYING ACTIVE
It’s natural to want to do what your friends are doing. A lot of them might join organized sports teams and you can too—just make sure to do a couple of things before signing up:
➥ Think about the risk of the potential injuries with the sport. Is there a lot of body contact? Is there a high potential for a fall or injury? Remember that you might need extra infusions and have to deal with pain if you get hurt. Could there be another sport that you’ll find fun that’s less risky?
➥ Then, speak to your parents and nurse or doctor to see if they recommend any changes to your treatment, such as starting prophylactic treatment or make a change in the regimen you are already on. It’s important to discuss the risks and benefits with your hemophilia team members, especially the physiotherapist. The best choices are made when you have the most information.
TALKING TO YOUR PARENTS
You’ve got so much racing through your mind: school, sports, peer pressure, hobbies, hormones, and the list goes on. Every teenager wants more independence and time away from their parents – but keep in mind that your parents have a hard time letting go. They love you and want to protect you, and it can be hard for them to adjust to your increased independence. So, if they’re overprotective now and then, cut them some slack!
You can help your parents get used to your growing independence by assuring them that you are thinking about your health and include them when making activity choices. Seeing that you are asking for more responsibility in managing your own care and making responsible choices will help them let go, while still being there whenever you need them.
Of course, talking to your parents isn't always that easy, so if you need someone to talk to or answer your questions (even the embarrassing ones!) don't forget that the HTC nurses are more than happy to offer you advice, tips, guidance, and the support you need as you grow more independent. You may also find support from others in the hemophilia community. Go Back
BEING MORE IN CHARGE
If you’re moving away from home, you’ll soon get used to a new set of responsibilities, like buying groceries (and cooking!), doing laundry, and paying bills. Along with these ‘grown up’ chores, come new responsibilities in managing your treatment and your health. This includes:
➥ Communicating directly with your team at the HTC
➥ Making and attending appointments
➥ Ordering factor treatment, if you’re on it
➥ Documenting and submitting treatment logs
As you get used to being on your own, you’ll start to find your groove and these things will become easier. In these early days, don’t hesitate to reach out to your parents for support, guidance and a pep-talk or two. And remember, to always keep your emergency contacts stored into your cellphone. If you don't already wear one, consider a MedicAlert bracelet to notify others of your condition in case you are unable to.
YOUR RELATIONSHIP WITH YOUR PARENTS/GUARDIANS
Your relationship with your parents/guardians is probably starting to change: they will need to start stepping back and letting you make your own decisions. Keep in mind that this can be difficult for them. They may be adapting to a sense of loss as they let you go off on your own. It’s natural for them to worry and check in often, which you may interpret as them ‘interfering.’ Try to be patient with them. They probably just need time to see that you can manage everything on your own – laundry, cooking, school and your health. Go Back
CHOOSING YOUR CAREER
What do I want to do with my life? That’s probably a question you’re asking yourself right about now – or at least thinking about. Most careers are totally fine for people living with hemophilia, and you have many paths to choose from. But there are a few jobs that may not be ideal because of a high risk of injury. Other jobs may be risky because they take you far away from where treatment is readily available, like if they require travel to remote destinations. Don’t let this deter you from setting off on your career path. Think about what you enjoy, what you could see yourself doing for a long time, and any potential risks of that path.
RELATIONSHIPS AND SEXUALITY
You’ve met a great girl or guy – or new friends – and things are going well. Do I need to tell him or her about my hemophilia? How will they react? When is a good time, and how do I start the conversation? It’s your choice who you tell and when. To help you decide who to confide in, think about:
➥ Put yourself in their shoes—would you want to know if you were them?
➥ Is it a friend you see during sports activities where your condition could come up?
➥ If you’re dating someone, could it be something long-term?
➥ Do you think it would really change the way they view you?
➥ Are you prepared to answer their questions about your condition?
➥ Because the hemophilia gene can be passed on to your children, how might this affect your relationship or your sex life? Learn more about things to consider when starting a family
There are no easy answers. Disclosing your hemophilia to those close to you can be very hard. Many people aren’t informed about the disease: it may be as simple as educating them and dispelling myths.
If you need someone else to talk to about some of these issues, consider talking to someone else living with hemophilia who may have gone through the same issues. If you know any peers through the Canadian Hemophilia Society, reach out to them – or contact the organization to help you find someone to talk to. Go Back
In two-thirds of the cases of hemophilia, the condition is inherited. But in about a third of cases, however, there is no family history. People with mild hemophilia are sometimes not diagnosed until they reach adulthood, perhaps after a prolonged bleed following surgery. Some women may only be identified as carriers after they have a child with hemophilia. For anyone affected by hemophilia, issues of family and children are often a large concern as they settle down.
PROPHYLAXIS INFUSION THERAPY
In recent years, many people with hemophilia, especially those with severe hemophilia, have begun to receive factor treatment as prophylaxis – to help prevent bleeding, rather than treating it afterwards. Prophylactic factor infusions are given just before an activity, or on a regular schedule (usually every 2 or 3 days) to maintain consistent clotting factor levels in the blood. Prophylaxis infusion therapy can result in fewer bleeds and help prevent joint damage. Go Back
PLANNING A FAMILY
If you or your partner have hemophilia or are a carrier, there are important issues to consider when planning a family. Because the condition is an inherited genetic disorder, there is a chance that you could pass it on to your children. The gene mutation that causes hemophilia can be passed on through either the father or the mother:
➥ A father with hemophilia will have boys without hemophilia and girls who will be carriers.
➥ A mother who carries the gene for hemophilia has a 50/50 chance of having a boy with hemophilia and a 50/50 chance of having a girl who is a carrier, with each pregnancy. Learn more about how hemophilia is inherited
THE ROLE OF GENETIC COUNSELLING
If you’re thinking about starting a family with your partner, you may want to consult a genetic counsellor. A genetic counsellor is someone who is trained to educate and guide people about the risks of passing on conditions to their children. Some information they may discuss with you:
It is very important that a carrier mother's doctor knows during her pregnancy that she is a carrier. This way, she can be provided the best care possible during pregnancy and delivery. Factor levels may need to be tested during pregnancy (usually at 28 and 34 weeks) so that precautions can be taken to reduce the risk of excessive bleeding.
The sex of the baby should also be identified in advance, at least to the doctor. If it is a boy that might have hemophilia, a plan can be developed in case of complications. Go Back
Women who are carriers of hemophilia have one ‘normal’ X chromosome and one ‘abnormal’ X chromosome. The normal X chromosome produces enough clotting factor to protect carriers from the most severe form of hemophilia. But – clotting factor levels vary widely in women who are carriers. Learn more about what it means to be a carrier
Many carriers have a clotting level between 30% and 70% of normal and do not usually suffer from severe bleeding, although they may suffer from the most common symptom – heavy menstrual bleeding. However, some carriers have less than 30% of the normal level of factor VIII or IX. These women are considered to have mild hemophilia. Up to 60% of carriers are estimated to have bleeding problems.
WHEN TO SEE YOUR DOCTOR: THE SYMPTOMS OF ABNORMAL BLEEDING
Because hemophilia is thought of as a ‘male’ disease, many women who notice the symptoms of a bleeding disorder go undiagnosed. The most common symptoms of a bleeding disorder in women include:
Heavy or prolonged menstrual bleeding (menorrhagia)
Abnormal bleeding after childbirth
Easy bruising
Frequent and prolonged nosebleeds
Prolonged bleeding after dental procedures
Abnormal bleeding after surgery or trauma
MORE ON MENORRHAGIA
Heavy, prolonged bleeding is the most common symptom of a bleeding disorder in women. When this happens throughout a woman’s period, it’s called menorrhagia. Many women don’t realize that menorrhagia could be the sign of a bleeding disorder – and don’t see their doctor. It’s common for women to think, “I always have heavy periods” or “All the women in my family bleed a lot.”
Menorrhagia can have significant effects on a woman’s life, including chronic fatigue, embarrassment from ‘bleeding through’, missed work, severe pain during a period (dysmenorrhea), and mood changes. An even more serious consequence is that many women have had hysterectomies because of an undiagnosed and untreated bleeding disorder.
MONITORING GIRLS DURING PUBERTY
Menstrual bleeding can be especially heavy at the time of a girl's first period. When there is a family history of a bleeding disorder, a girl should be closely followed through puberty. The medical team should include:
➥ Gynecologist;
➥ Hematologist with experience in treating bleeding disorders; and
➥ Family physician or pediatrician.
PREGNANCY AND CHILDBIRTH
Most women have few bleeding problems during pregnancy, or during childbirth. But it still is very important for the woman’s healthcare providers to be aware of her carrier status so that plans can be made for a safe delivery. This includes ensuring special safety measures are in place during childbirth to avoid injury to the mother, and child. As well, clotting factors may have to be monitored during pregnancy, especially as the date of delivery approaches.
PRECAUTIONS FOR WOMEN
Women with a bleeding disorder need to follow some precautions to help prevent excessive bleeding. For example, she should never take a drug that can affect the platelets (clotting proteins) in the blood. These include:
➥ Aspirin® (ASA)
➥ Drugs containing Aspirin® (e.g., Midol®, Alka-Seltzer®)
➥ Non-steroidal anti-inflammatory drugs (e.g., Advil®, Aleve®)
Often, these drugs are used to treat menstrual cramps, but can actually make bleeding worse. Always speak to your doctor or pharmacist about a drug you are planning to take to ensure if it safe for you to do so.
UNDERSTANDING AGE-RELATED ISSUES
As we get older, we’re bound to encounter changes in our health that come with age. The difference for someone with a bleeding disorder is that they may be at higher risk for certain age-related issues. Also, these secondary conditions need to be managed carefully, as they may cause or worsen other problems related to hemophilia.
In the past, little was known about the effects of age-related conditions on those within the community. Now, we understand that many of the health concerns the rest of the population faces as they get older may also affect people with hemophilia.
As you age, you should be aware of your risk for the following conditions and the challenges with treating them in the presence of hemophilia.
HIGH BLOOD PRESSURE
People with hemophilia are twice as likely to have abnormally high blood pressure (or hypertension). Because this condition can increase your risk of bleeding regardless of the presence of hemophilia, you should have your blood pressure checked often and – if you have hypertension – make sure you treat both your hemophilia and your elevated blood pressure.
HEART DISEASE
Managing hemophilia over the years can increase your risk for heart disease as you age, since certain factors – like infusion of factor concentrates and HIV infection – may make it more likely. In the event of a heart attack, make sure the treating physician is aware of your bleeding disorder so he or she can take necessary precautions to treat your heart without causing added hemophilia-related complications.
HIGH CHOLESTEROL
Cholesterol levels have actually been shown to be lower in people with hemophilia compared to the general population. However, because of the potential increased risk of cardiovascular (heart) disease in hemophilia, cholesterol levels should be measured regularly to check for this issue, which is also called hypercholesterolemia.
DIABETES
It is not known for sure whether the risk of diabetes is higher among people with hemophilia compared to those without it. As you get older – especially if you’re overweight – your blood sugar levels should be checked once each year to test for diabetes.
OSTEOPOROSIS
People with osteoporosis have low bone mass and more fragile bones, leading to an increased risk of fracture. Low bone mineral density is common in people with hemophilia, putting you at risk for osteoporosis. To help prevent this condition, talk to your doctor about suitable sports and other weight-bearing activities that can help you maintain your bone health. Learn more about joint disease
KIDNEY DISEASE
Though everyone over the age of 60 may be at risk for kidney disease (also called renal disease), people with hemophilia may have additional risk factors, including HIV infection, factor inhibitors, and bleeding in their kidneys. If you have high blood pressure or diabetes, this also increases your risk of developing kidney disease. To help keep your kidneys working well, maintain a healthy weight and avoid smoking. You may also want to talk to your doctor about having a regular kidney health check as you age.
OBESITY
Obesity is on the rise in the general population, but in people with hemophilia, it has been associated with additional health concerns, including: increased joint pain, increased risk of developing target joints, and an even higher risk of diabetes and heart disease. Regular physical activity is important. If your hemophilia restricts certain activities, talk to a physiotherapist about other options to help you stay active. Learn more about fitness & exercise
SEXUAL DYSFUNCTION
Age-related issues like high blood pressure and diabetes can affect anyone’s sexual health. But people with hemophilia may have added issues that can cause concerns in the bedroom. For example, complications of hemophilia can lead to sexual dysfunction including lack of libido and impotence, as can chronic infections with hepatitis C or HIV. Some men take oral medications for erectile dysfunction (ED), but beware that these ED treatments may cause nosebleeds in people with hemophilia.
ADVICE ON AGING WITH HEMOPHILIA
While certain health concerns are more likely as you age – and, in some cases, even more likely if you have hemophilia – there are things you can do to take care of yourself as you get older.
Start with these 10 tips for making the most out of your later years with hemophilia:
An initiative by the ADVANCE Working Group is underway to identify and research key issues in the aging hemophilia population. For more information about this research, visit advance-haemophilia.eu
ADVANCE=Age-related DeVelopmentsANd ComorbiditiEs in hemophilia.