Hemophilia: Why compliance is Critical to Joint Health

ARTICLE DIRECTORY

2018






why compliance is critical to joint-health

Hemophilia is a rare, genetically linked bleeding disorder in which blood does not clot normally. It is caused by a deficiency in clotting factors. (Usually factor VIII or factor IX.)

Hemophilia is characterized by excessive bleeding episodes, often involving bleeding into joints and soft tissues such as muscles. Over time, repeated hemarthrosis (bleeding into joints), can result in significant joint damage.

Hemophilia Joint Bleeds

Although it can affect any joint in the body, certain joints, are more vulnerable to damage. Ankles are often the first and most severely affected. Elbow and knees are also prone to accumulated damage. Studies show that as many as half of all joint bleeds occur in the knees.

Bleeding into a joint typically begins with a small break or tear in the synovial membrane. (The synovial membrane is a specialized form of connective tissue which lines the inner surface of joints.)

What Causes Joint Bleeds?
Joint bleeds can be caused by trauma, although many have no obvious cause. Even routine activities and or small bumps can cause minute tears in the synovial membrane and or damage small blood vessels, causing blood to fill the joint cavity. Healthy people who do not have hemophilia, rarely notice any difference in their joints from these kinds of micro-injuries.

Tiny Tears. Big Bleeds
In hemophilia patients however, even tiny abrasions and tears can lead to exaggerated bleeding into the synovial spaces of the joint. (The synovial space is formed by a flexible, thick membrane, filled with a fluid that lubricates the joint.)

Because the space is extremely limited, any large buildup of fluid or blood can cause severe pain, swelling, and damage to the joint and surrounding tissues. Over time, repeated bleeding episodes can cause chronic pain, or even deformity in the affected joint. Click here.

Studies have consistently shown that prophylactic (preventative) factor replacement therapy using plasma derived or recombinant clotting factors helps to reduce the chance of damage caused by these kinds of joint bleeds.

Preventative Factor Replacement is key to Joint Health
Like a keystone , joint health is held in place on either side by a combination of preventative clotting factor treatments and personalized monitoring of the individual joints.

Over time, patients and their treatment teams usually come to understand what kinds of activities typically trigger a bleed, the current health of the joint(s) in question, and the effectiveness and timing of factor replacement therapy.

Hemophilia Joint Damage Can Take a Decade or More to Show
According to the Hemophilia Joint Health Score Reliability Study, the degree of joint damage can be difficult to ascertain because it often occurs very slowly. Even imaging studies may only display subtle changes over time.

This is why the Hemophilia Joint Health Score was developed. The score helps providers assess joint health and impairment by measuring range of motion, flexion, gait, and other factors.

Other tests, including imaging studies and joint aspiration (which involves removing synovial fluid from a joint), can also help healthcare providers determine the health of affected joints. If you, or your child has hemophilia, your hemophilia medical care provider may recommend a joint protocol to help you manage bleeding episodes and reduce any potential complications from joint damage.


critical to joint health

Signs of a Joint Bleed
Signs and symptoms of a joint bleed can include:

  • Pain, tingling, heat, or a bubbling sensation within the joint
  • Feeling an "aura" (a difficult-to-explain sensation that some people get with a bleed)
  • Swelling and pain which increase as the bleed progresses
  • There may be no obvious bruising
  • In children, there may be a reluctance to move the joint, place weight on it, or they may hold it in a flexed (bent) position
  • In children and infants, a bleed may cause them to be cranky or cry, even without outwardly visible damage to the joint

Late stage joint symptoms may include difficulty moving, stiffness, and or chronic pain in the joint. Continued swelling, pain, and or heat may also be present.

Hemophilia Joint Bleed Treatment (Follow YOUR Joint Bleed Protocol)
If you suspect that a joint bleed is in progress, you should take appropriate measures, such as using your prescribed clotting factor, per your healthcare provider's recommendations. Typically, this means taking enough of your factor to raise your level to 40% at the first sign of a joint bleed.

If symptoms continue for longer than 24 -48 hours, be sure to follow any specific protocols and directions provided your medical provider or hemophilia treatment center. Click here.

R.I.C.E
Patients are often encouraged to follow the RICE method (rest, ice, compression, and elevation) to reduce swelling. Be sure to avoid using the joint until any swelling has gone down. To help monitor swelling, you can use a soft tape measure; simply measure the largest point of swelling in the affected joint, and then compare it to the non-affected joint on the other side.

You should also report the bleed to your hemophilia care provider or hemophilia treatment center. Be sure to monitor the size, temperature, of affected joints, as well as your pain levels. Once the swelling and pain are over, your hemophilia physician or physical therapist can advise you about resuming activities to help maintain joint strength and flexibility.

Appropriate Exercise Can Improve Joint Health
Additionally, be sure to talk to your hemophilia specialist about regular, appropriate exercise, flexibility, and conditioning routines such as: gentle yoga, swimming and others to help maintain optimal joint health.

You should also discuss ways to achieve and or maintain your ideal weight too, because excess weight places additional strain on vulnerable joints. Make sure to speak to your healthcare provider about taking your factor concentrates before engaging in activities that are likely to trigger a bleed.

You should contact your treatment center or hemophilia doctor if the symptoms do not subside, or you feel that there may be additional issues, such as a bone fracture, bleeding into your hip or abdomen, or others.

ASP Cares Supports People Living with Hemophilia
If you, or someone you care about, has hemophilia or another rare condition, ASP Cares can help. We provide patient-centered, high touch, personalized pharmacy services for patients with rare disorders. Our highly trained pharmacists and clinicians are experts in all areas of hemophilia. Click here for more information.

ASP Cares. Big enough to serve. Small enough to care.

**Disclaimer**
This content does not represent medical and or legal advice. It is not meant to diagnose or treat any condition. It is not intended as a substitute for the advice of a qualified medical provider.


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