Complications of having a Bleeding Disorder

 

Complications are problems that develop directly or indirectly by a disease, disorder, or condition. Some of these complications can be prevented, so it is important to educate yourself and follow your doctor’s recommendations for optimal treatment of bleeds.

Joint Damage

The most common complication of hemophilia is joint disease. This occurs when there is repeated bleeding into the joints. Joint bleeds cause pain and discomfort that can lead to chronic swelling and deformity. Many people with hemophilia suffer from arthritis and, in severe instances, may require joint replacement surgery.  

Inhibitors

Approximately 30% of people with hemophilia A and approximately 2-3% of people with hemophilia B develop inhibitors. Inhibitors are antibodies that the body develops because it sees the infused factor as a foreign substance that needs to be destroyed. Antibodies are proteins that eat up the activated factor before it has time to stop the bleeding.  

Viral Infection

Factor products today are considered theoretically safe. However, there is always a remote risk of a new virus being introduced into the blood supply and therefore, into the plasma derived factor concentrates. The manufacturing processes currently used are sophisticated and likely to eliminate known and new viruses, so the risk is very low. 

Emotional and Psychological

Feelings are normal and everyone experiences positive and negative feelings at different times in their lives. Being diagnosed with a chronic condition or learning that your child has a chronic condition may make a person feel stressed, sad, and sometimes depressed. It is important to know that these feelings are normal, and it is also critical to know when to seek help if you feel overwhelmed with emotions.

      Joint Damage

The most common complication of hemophilia is joint disease.  A joint is an area where two bones come together. People with hemophilia can bleed into the joint space after an injury or, at times, without obvious cause.  The pressure of blood filling the joint cavity causes significant pain and can lead to chronic swelling and deformity.  Joint damage can occur after repeated bleeding into the same joint or after one serious joint bleed.  The joints typically affected include the elbows, ankles and knees.

How does joint damage occur?

Joint damage in people with a bleeding disorder is similar to joint damage of a person with arthritis.  The damage occurs in the synovium and the cartilage around the bones.

The synovium is a lining that lubricates and feeds the joint; it also removes fluid and debris from the joint.  There are blood vessels in the synovium and that is why bleeding into the joints is common in people with a bleeding disorder.  One of the synovium’s functions is to remove fluid from the joint. When there is blood in the joint, the synovium absorbs it.  Blood has iron and it is believed that the iron in the blood causes the the lining to get thicker.  As the synovium gets thicker, it contains more blood vessels and therefore subsequent bleeding is more likely.

Joints have two types of cartilage, cartilage around the ends of the bones and cartilage to absorb shock.   The cartilage affected when bleeding occurs is the cartilage around the bone area.  This cartilage is a smooth surface on the ends of the bones and allows the two bones connecting in that joint to move without friction on each other.  When bleeding occurs, enzymes from the swollen synovium destroy the cartilage that covers the ends of the bones.   As the cartilage erodes and becomes pitted, the rubbing of bone on bone is very painful.   The cartilage that absorbs shock in a joint is usually not affected by bleeding, but rather by injuries in sports or other physical activities.

As described, joint bleeding can become more likely even after one joint bleed.  Joints that bleed often are usually referred to as “target joints”.  As joint damage progresses, movement may become restricted in that joint.

What are the symptoms of a joint bleed?

  • Warmth

  • Swelling

  • Tingling inside the joint

  • Discomfort

  • Pain

  • Loss of motion

  • Stiffness

Small children may not be able to describe the symptoms above.  Below are some signs to watch for in small children:

  • Irritability

  • Crying

  • Favoring a limb – a baby may hold bottle with opposite hand than usual, toddler may use opposite hand to eat

  • Refusing to walk – child may not want to move or may walk trying to avoid bearing weight on the affected leg

What should be done when a joint bleed is suspected?

It is very important not to ignore the signs of a joint bleed.  Early treatment with factor concentrate is crucial to reduce the risk of joint damage.  If you suspect a joint bleed, call your doctor immediately to avoid complications.

In addition to factor treatment, the following can help a bleeding joint feel better and minimize damage:

  • Apply ice to the area

  • Rest the joint

  • Elevate the limb

What is the treatment for damaged joints?

Over time, joints can become severely damaged and the person suffers from acute pain and restricted range of motion in that joint.  Surgery can be effective in managing pain and improving movement of the joint.  It is important to note that a hemotologist must be involved in the planning of all invasive procedures to ensure proper clotting levels during and after surgery. The following are common surgical procedures used to aleviate pain and improve function of damaged joints:

Synovectomy

A syncovectomy is the removal of the synovium.  Removing the synovium stops the bleeding cycle caused by the thick synovium.  The procedure doesn’t make the joint “like new”, but it helps aleviate pain and improve function of the joint. There are three synovectomy techniques used:

  • Radioactive – A radioactive fluid is injected into the joint, which reduces the swelling in the synovial membrane.

  • Arthroscopic  – The synovium is removed through surgical incisions in the area.  A small camera is inserted into the joint to help guide the removal of the synovium.

  • Open – The joint is opened surgically and the synovium removed.

Joint replacement

Joint replacement is a surgical procedure that helps people who suffer from chronic pain that interferes with their daily activities.  During this surgical procedure, the damaged joint and bone are removed and replaced with plastic and metal components. Joint replacement surgery is more common on knee and hip joints than on elbows, shoulders and ankles.  After surgery and physiotherapy, most people are left with a pain-free joint and improved range of motion.  Replacement of the artificial joint is sometimes necessary as it can wear out or become loose.  Ninety percent of hip and knee replacements should last 10 years.

     Viral Infection

In the 1980’s, approximately 90% of people with severe hemophilia were infected with the HIV virus and almost all patients with hemophilia who used factor products before 1988 were infected with Hepatitis C (HCV).  Blood-borne infections were a major complication of treatment for people living with hemophilia.  At the time, factor products were manufactured using large plasma donor pools and the process lacked specific tests for infectious agents.  This tragedy resulted in the loss of many lives and brought to the forefront the need to change the manufacturing and screening processes.

Today, factor products today are considered theoretically safe.  Scientists have developed techniques to manufacture clotting factor from animal cells.  Clotting factor created from animal cells is called recombinant factor.  Recombinant factor concentrates have never been known to transmit pathogens of any kind.  Plasma-derived products continue to play an important role in the treatment of bleeding disorders.  With testing and viral inactivation processes available today, plasma-derived products are considered very safe.

      Emotional and Psychological

Feelings are normal and everyone experiences positive and negative feelings at different times in their lives. Being diagnosed with a chronic condition or learning that your child has a chronic condition may make a person feel stressed, sad, and sometimes depressed. It is important to know that these feelings are normal, and it is also critical to know when to seek help if you feel overwhelmed with emotions.

Parents

Hearing that your new baby has a chronic condition can be very stressful. Parents of children recently diagnosed with hemophilia often experience a period of shock, and they may feel stressed and emotional about their child’s condition. It is important to remember that the bleeding disorder is only one part of your child’s life. The bleeding disorder should not define his or her identity. Many parents expect to have a perfect baby, but the truth is that no one is perfect. Almost every child will experience some sort of medical concern during their life, whether it is an eyesight problem, allergies, asthma, kidney problems, etc. Every child is special in his or her own way. Children with a bleeding disorder have the power to lead successful and fulfilling lives.

 

Some parents go through the following emotions as they confront the diagnosis of a bleeding disorder:

Denial

Some parents experience a period of denial because it is too hard to face the reality of the diagnosis. Denial is a coping mechanism, and it will not make the situation go away. It is important for denial to only last a short period of time, if it exists at all, so that parents can move into an acceptance period. Being in denial for a long time can ultimately affect the child’s well-being. A parent in denial may miss the signs of a bleed or may make comments that make the child feel that hemophilia is bad, and therefore the child is bad.

Withdrawal

Some parents go through a period of withdrawal where they become emotionally distant from the child and/or from other people. Since bleeding disorders are genetic, parents may feel guilty that they “gave this” to their child and subconsciously may detach from the child. It is important to remember that there is nothing the parent did wrong to give a bleeding disorder to their child. Passing a gene for a bleeding disorder is no different than passing a gene for blue or brown eyes.

If a parent goes through withdrawal for a long time, they may miss out on wonderful childhood experiences. Every parent must remember to enjoy their child each and every day, whether the child has a medical diagnosis or not. Other parents withdraw from social situations and family and friends. They may feel that no one understands what they are going through, and therefore prefer to spend time alone with just the immediate family. Isolating yourself and the family is not healthy. Seeking help and building a support network are not easy to do sometimes, but they are important steps in dealing with and accepting the diagnosis of a bleeding disorder. It is recommended for parents to seek support as they adjust to having a child with hemophilia.

There are resources that can help parents get through this difficult adjustment period:

  • HTC: Treatment centers often have a social worker as part of their staff. These specialists can help parents as they adjust to the diagnosis of a child by providing resources and guidance to help with the financial, emotional, and social stress associated with a new diagnosis.

  • Parent Support Groups: There are private groups on social media that consist of parents of children with hemophilia. Parents in these groups can discuss how they are feeling, experiences they have had, lessons learned, etc. These groups are a great way to get educated and get support from others that understand what you are going through. As with any social media, it is important to know how the group is structured, who the members are, what the privacy policies are, and how posts are monitored, in order to ensure a healthy exchange of information.

  • Local Hemophilia Community: There are local non-profit organizations that serve people affected with bleeding disorders around the country. These organizations provide educational conferences as well as social events that bring individuals with bleeding disorders and their families together a few times per year. 

Children

Children process emotions and feelings differently as they grow developmentally.  A child who is 3 years old asks very different questions about hemophilia than a 9-year-old or a 14-year-old child.

Preschoolers

Children in this age group are egocentric. They believe everything happens because of them and that they can make things different if they just wish hard enough. A child in this age group needs to be reminded that factor will stop the bleeding so that he can go and play and have fun. It is important to not treat factor as a punishment. Parents should avoid saying things like, “If you keep doing that, I’ll have to give you factor.” This kind of phrase has a negative, almost punishment-like approach, and needs to be avoided.

School Age Children

Children in this age group are capable of seeing the cause and effect of situations on their own. In other words, they can understand that certain activities may lead to a bleed, and therefore, to factor treatment. At this stage, children start to think about others’ feelings and may start worrying about their parents. Some children go through periods when they don’t want to tell their parents about a bleed or pain because they don’t want their parents to feel hurt. It is important for parents to reassure the child that the bleed needs to be reported and that together they will take the proper steps to ensure it heals quickly. Parents must not overreact when a child reports a bleed, and they should treat the issue calmly and supportively.

Teenagers

By the time your child reaches adolescence, he will likely know as much about hemophilia as you do. Adolescents worry about what others think, so having hemophilia may make them feel different. It is important for your child to know other teenagers with a bleeding disorder so they know they are not alone. As a parent, you can help your teenager by being a good listener. It is important for parents to not try to fix their problems or tell the young adult how they should feel. Parents need to listen, and be sympathetic and supportive.

Cited from www.hemphiliafed.org 

 
 
 

About us:

Tennessee Hemophilia & Bleeding Disorders Foundation is a non-profit 501(c)(3) organization.  The foundation was founded in 1970.

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