Your Role in Treatment

Helping your child become independent and able to care for him- or herself is of paramount importance. A first step to that goal is for you to know your child’s diagnosis and its treatment thoroughly.

Not only must you understand your child’s condition fully, you must educate your child’s other caregivers. Once your child is able to communicate, you must be certain that he or she understands his or her condition and can explain it to someone, if necessary.

It’s also important to know your child’s treatment plan, including:

• The type and severity of bleeding disorder
• The type of product used to treat the bleeding disorder
• How it’s administered
• How much is administered (called the dosage)
• How often it’s administered

Get the Child Involved

A parent’s ultimate goal is to raise a child who is independent, healthy, well-adjusted, and happy. While it may take a bit more work for both the parent and child, a child with a bleeding disorder can be all those things. It is never too early to begin to think of little ways to involve even your very young child in his or her own care. Learning the basics early will help your child be ready to take on full management of his or her care in late adolescence.

Ways to include your child in his or her care:

• Educational events through your Hemophilia Treatment Center (HTC) or local bleeding disorder organization
• Open age-appropriate discussions about your child’s bleeding disorder and treatment plan
• Increasing your child’s responsibility as he or she ages
• Age-appropriate discussions with the medical team

Recognize Early Signs of a Bleed

To treat a bleed promptly, effectively, and thoroughly, you must be able to recognize the early signs of a bleed. By not doing so, you risk lasting joint disease or a worse outcome for your child.

Provide First Aid

It is also important to be able to provide basic first aid to a bleed.

Understanding Treatment Options

The primary approach to the treatment of hemophilia is factor replacement therapy. Your hemophilia care team will teach you—and your child in an age-appropriate way—about the what, when, why, and how of managing hemophilia.

Pain Management

Unfortunately, pain is one of the most common characteristics of a bleeding episode. Fortunately, there are many approaches to help lessen pain. Nonetheless, pain can be a real cause of stress and fatigue for a child with a bleeding disorder, as well as for the caretaker. It’s not unusual for a caretaker to feel helpless. But with an organized strategy to limit and control pain, you and your child can gain confidence in managing a bleeding episode and the pain it causes.

In general, people with hemophilia experience two types of pain:

• Acute pain: short-term pain, which usually results from bleeding into joints or muscles or is the aftereffect of surgery
• Chronic or persistent pain: long-term pain, which usually is due to arthritis that is caused by damage to a joint from repeated bleeding episodes

Often people may be reluctant to let others know that they are in pain. And, of course, babies and very young children may not have the verbal skills to communicate their discomfort. Here are some common pain signs that you can look for:

• Mood changes
• Reluctance to communicate and interact with others
• Increased irritability
• Inability to concentrate
• Difficulty sleeping
• Decreased interest in favorite activities
• Lack of appetite

If allowed to go untreated or inadequately treated, pain can lead to more serious consequences for patients, parents, and other caregivers. It can have an emotional, financial, academic, and spiritual impact on everyone involved. Pain can cause children to miss school, and in later years, it can affect educational and career choices. Pain can necessitate that a parent miss work caring for a child who suffers from acute or chronic pain. Both may miss out on family or social activities. It can lead the caregiver to feelings of helplessness and hopelessness, and can cause the patient and other caregivers to have these feelings as well.

Assessing a person’s level of pain can be difficult because pain is always subjective and is also often fraught with emotion, fear, and anxiety. There are no objective diagnostic tests to measure a person’s level of pain. Often health professionals will use other methods to help patients describe how much pain they have. One commonly used tool is a visual pain scale, of which there are several. In one type, patients are asked to rate their pain on a scale 1 to 10, with 1 being relatively no pain and 10 being the worst pain. For younger children, health professionals might use pictures of different faces from a relaxed face scaled up to a picture of a face of a person crying and have the child point at how he or she is or has felt.

The primary goals of pain control are to relieve suffering and to maintain daily function. Pain can be managed through three main types of care: physical, pharmacologic (with medications), and psychological.

The physical management of pain can include:

• Immobilization of a limb (using a splint, crutches, pillows, etc)
• R.I.C.E. (Rest, Ice, Compress, Elevate)
  • Resting in a comfortable position
  • Ice
  • Compression bandages
  • Elevation of the affected area
• Physical therapy can also be used to manage pain and return limbs to functionality after a bleed and reduce the risk of atrophy and loss of joint motion

The pharmacologic management of pain includes a range of options from over-the-counter products like acetaminophen (Tylenol^®) to narcotics, which must be prescribed and monitored by a doctor. Because bleeding disorders can lead to chronic pain, doctors closely monitor the use of narcotics to reduce the risk of addiction.

The psychological management of pain can be categorized under complementary and alternative medicine (CAM) approaches. In general, these practices have been considered outside of mainstream medicine. This view is gradually changing as more research is performed and more scientific evidence of the effectiveness of some CAM methods is being proven.

As a caregiver, you can help your child—or any person with a bleeding disorder—take control of pain through a variety of techniques and activities. Even simple measures such as distraction, deep breathing, or guided imagery can be helpful. Helping your child learn ways to control the pain rather than being too afraid to do anything or believing that nothing can be done to lessen the pain will improve your child’s—and his or her caregiver’s—quality of life. Keeping your child active and involved, attending school and other activities, is strongly encouraged.

Complementary and alternative approaches include a wide range of techniques and practices. Some of these are well known; others are more obscure. These approaches can be divided into five main categories. (See Table Main Types of Complementary and Alternative Medicine below.)

Remember: check with your hemophilia health care provider before beginning any alternative therapy. This is especially important when considering taking any herbal remedy. Many of them can be toxic, can adversely interact with other medications, and some can directly affect the ability of the blood to clot.

Drugs to Avoid

Some painkillers (called analgesics) can aggravate bleeding. These include acetylsalicylic acid (aspirin), or other nonsteroidal anti-inflammatory drugs (eg, ibuprofen). The over-the-counter analgesic acetaminophen (Tylenol^®) (called paracetamol outside the United States) and the prescription narcotic codeine are safe alternative analgesics.

As with any herbal supplement or remedy, the influence of recreational and illicit drugs on a bleeding disorder and its treatment is largely unknown. They could be harmful and should be avoided.

What Is Home Therapy?

Treating bleeds at home without a health care provider present is called home therapy. Because clotting factor (called factor) has to be given intravenously, family members will need to learn how to:

• Assemble the needed supplies to administer the clotting factor
• Determine dosage
• Mix the clotting factor
• Draw the factor into a syringe
• Start an intravenous line for the infusion
• Administer the factor slowly
• Dispose of used needles and supplies
• Record the symptoms of the bleeding episode and the treatment given
• Know the signs of a serious bleeding episode and when to contact a health care provider, if needed
• Provide calming support and manage pain if needed

Is Home Therapy Right for Our Family?

Your Hemophilia Treatment Center (HTC) will help you decide if home therapy is appropriate for you and your family. Home therapy is a major responsibility. It requires a good deal of knowledge about identifying and assessing bleeding episodes. It also requires skills to do the infusion.

If your child is very young, he or she may not be ready or able to cooperate with home therapy. Home therapy may also not be appropriate if your child has mild or moderate hemophilia and bleeds only a few times a year. If the bleeding episodes are infrequent, maintaining the skills for safe home therapy is difficult.

Your HTC health care team will train you to identify bleeding episodes as soon as they occur and give you a plan of action if a bleed starts.

The HTC team will teach you everything you need to know to maintain your child’s treatment plan. They will work with you so you learn how to mix a clotting factor and know how much to give. They will teach you how to choose the best vein for the infusion. If your child has a port, your health care provider will show you how to care for it.

The HTC health care team will show you how to keep treatment logs, which will allow them and you to follow the progress and effectiveness of your child’s treatment plan. These treatment logs are also used to determine when and how often you need to order the clotting factor and other supplies.

Home Therapy Safety Guidelines

Just as in a doctor’s office, hospital, or Hemophilia Treatment Center (HTC), safety during home therapy is paramount.

Safety procedures you should follow when administering treatments at home:

• Clean your treatment work area before and after treatment
• If you spill blood or clotting factor on the work area, clean the surface with chlorine bleach
• Use alcohol-based cleanser or wash your hands before and after treatment
  • Make sure you use soap and warm water
  • Wash your arms up to the elbows and rinse properly
• Observing the universal precautions is recommended for all persons handling clotting factor infusion equipment. These are:
  • Gloves should be worn by persons who prepare or infuse blood products and during disposal of infusion equipment and waste
  • A needle that has broken the skin should not be reused
  • Used needles should never be recapped
  • Used needles should be placed in a sharps container in a location out of the reach of young children
  • Lock or seal the container lid closed when it is full
    • The regulations and laws for disposing of sharps containers vary from state to state and even county to county. Some ways to dispose of sharps containers may be through a mail program or drop off at a hospital, pharmacy, or other designed location. Contact your state health department for your local guidelines.