Resources

Emergency Care for ER Staff

Individuals with bleeding disorders who present to an emergency room (ER) for care should receive appropriate, expeditious care management. Do not keep these patients waiting. A patient with a bleeding disorder could be experiencing a life-threatening bleed which may not be obvious at first glance.

Many patients and their caregivers are very knowledgeable about their disease – be sure to have an open dialogue with them. They may have brought factor replacement medication with them, or may have infused factor prior to arrival in the ER. If the patient has not contacted his or her hematologist/hemophilia treatment center, be sure to do so immediately. The appropriate plan of care should be coordinated with this physician.

Upon discharge from the hospital, your patient will need a log showing infusions given while in your care. Be sure you have documented all infusions administered so they have the information for their records.

The most common bleeding disorders are hemophilia A, hemophilia B, and von Willebrand Disease.

Hemophilia is a hereditary, lifelong blood disorder – often diagnosed at birth – in which the blood clots more slowly than normal. Normally, when an injury occurs that causes bleeding, our bodies respond by clotting (coagulating) the blood so the bleeding slows and eventually stops. People with hemophilia have a deficiency of a blood protein called clotting factor that causes fibrin clots to not form correctly. A deficiency of clotting factor VIII (eight) is called hemophilia A, and a deficiency of clotting factor IX (nine) is called hemophilia B. People with hemophilia do not bleed faster than other people; they just bleed longer.

Von Willebrand Disease (VWD) is a hereditary bleeding disorder characterized most often by bleeding from the skin and mucous membranes (e.g., mouth, nose, throat, gastrointestinal tract) affecting both males and females equally. This bleeding disorder is the most common inherited bleeding disorder. The most common symptoms of von Willebrand Disease are easy bruising, prolonged nosebleeds, heavy or prolonged menstrual periods (lasting more than 7 days), and prolonged bleeding following injury, surgery, dental procedures, or childbirth. Gastrointestinal bleeding can also occur.

Recommended Bleeding Disorder Treatments

Treatment of hemophilia A

1. Recombinant factor VIII concentrates
2. Plasma-derived factor VIII concentrates
3. Cryoprecipitate not recommended
4. Treatment of mild hemophilia A: Desmopressin nasal spray or injection (Stimate® or DDAVP® injection). If not responsive to desmopressin, factor VIII concentrate is recommended.

When bleeding is severe, the appropriate dose of factor VIII is 50 units/kg. This should result in a factor VIII level of 80%–100%.

Treatment of hemophilia B

1. Recombinant factor IX concentrate
2. Plasma-derived factor IX concentrates

Treatment of hemophilia A and B with inhibitors

1. FEIBA, anti-inhibitor coagulant complex
2. NovoSeven® RT, coagulation factor VIIa

Dosing is best determined through immediate discussion with patient’s hematologist. NovoSeven® RT is the preferred agent for patients with Factor IX and a history of inhibitors.

Treatment of von Willebrand (VWD) Disease

See medication package insert for dosing recommendations.

Type 1: Treat with the synthetic intravenous desmopressin or Stimate 1.5 mg/mL nasal spray for bleedingType 2A, 2M and 2N

Type 2A, 2M and 2N: Consider treating with desmopressin (see Type 1 above) if patient previously showed a response to a desmopressin trial (per patient history). If patient has already treated with desmopressin or Stimate prior to ER visit, then a product containing von Willebrand factor (vWF) such as Alphanate®, Humate-P®, Koate®*, Vonvendi® and Wilate® should be considered.

Type 2B and type 3 – and type 1, 2A, 2M and 2N nonresponsive to desmopressin:

Treat with a product that contains the vWF factor protein such as Alphanate®, Humate-P®, Koate®*, Vonvendi® and Wilate®.

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For more in-depth information on treatment of a bleeding disorder,

download Accredo’s ER Guide to Bleeding Disorders (pdf, 404KB)

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Coaches and educators

Understanding that you will be caring for a child with a bleeding disorder may cause anxiety, fear and uncertainty. As you know, every child needs education, social interaction with peers, exercise, and healthy outlets for stress. A child with a bleeding disorder is no different.

To be fully prepared for care of this child, it would be helpful to meet with parents of the child with a bleeding disorder prior to the start of the school year or sports season. The following items can help guide that discussion:

• Contact information – general contact needs as well as emergency situations
• How to recognize and report a bleeding episode or injury
• Actions to take in the event of bleeds or injuries
• Privacy issues
• Role of school nurse or point person if there is no school nurse
• Storage of factor and supplies at the school
• Absences
• Physical education, sports, and class trips

As a coach or educator, it is important to focus on what the child with a bleeding disorder can do rather than focus on any limitations.

What is hemophilia

Hemophilia is a hereditary, lifelong blood disorder in which the blood clots more slowly than normal. Normally, when an injury occurs that causes bleeding, our bodies respond by clotting (coagulating) the blood so the bleeding slows down and eventually stops. People with hemophilia have a deficiency of a blood protein called clotting factor, and their blood does not clot fast enough.

RICE for treating injuries

If a child has a bleed while in your care, follow the care plan set with the parents. If you are waiting for a parent or healthcare professional to arrive and administer factor when a bleed occurs, you can follow the RICE (Rest, Ice, Compression, Elevate) plan for temporary relief.

R = REST	Stop the activity, infuse clotting factor or medication and protect the body part. To rest the injured area, a sling, splint, crutches or a wheelchair may be needed for several days to several weeks after an injury.
I = ICE	Ice or cold packs are useful in slowing the bleeding, reducing inflammation and reducing pain. Heat should not be applied to an area that is bleeding, hot or swollen.
C = COMPRESSION	Compression with soft elastic supports helps reduce swelling and pain.
E = ELEVATION	Elevation above the level of the heart reduces swelling.

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For a detailed guide to aid in caring for a child with a bleeding disorder,

download Accredo’s Coach and Educator Guide (pdf, 4,502 KB)

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Prescribers

Accredo has the experience you need for your bleeding disorder patients. We provide in-home nursing staff to administer infusions where available by law, help your patients determine their insurance coverage and payment options and work with you to provide patients with information, disease management, and treatment options with the assistance of Accredo’s specialty-trained pharmacists.

How we serve your patients

Delivery to the place of administration.
We’ll ship many medications right to the patient’s home, doctor’s office or clinical setting where the medication will be professionally administered*. Shipping is at no additional cost to the patient. Accredo dispenses from three centralized locations and more than twenty-five branch locations.

Clinical education and support.
Our bleeding disorder-trained pharmacists and registered nurses educate new patients on how to use, store and administer their medication, as well as help set reasonable expectations for their therapy.

Patient-specific billing program.
Patients can authorize us to ship their medication to their home, doctor’s office or clinic but to send the bill directly to the insurance provider*. We have a personalized program that helps your patients make the most informed decisions concerning their insurance coverage.

Adherence checks.
Through education, personal support and regular contact, we encourage our patients to adhere to their treatment regimen.

Home infusion training.
If a medication needs to be infused and you order the training, then Accredo will teach the patient how to administer the therapy to help him or her overcome this hurdle.

In-home infusion.
If you decide a patient would benefit from home infusions, then Accredo will coordinate the details. Accredo has its own nursing staff across the country. We’ll research the patient’s benefits, provide the medication and arrange for a nurse to make a home/school visit in accordance with the doctor’s plan of treatment.

Local nursing support.
With more than 30 branch and nursing locations and over 550 field nurses, 63% with pediatric experience, we can provide clinical support and intervention throughout the country.

Bilingual support.
Having a bilingual clinical care team allows us to provide support to our Spanish-speaking patients without fear of improper medical treatment due to language barriers. We also provide third-party interpreters for other languages, when needed.

Care training.
With everything from in-school training, educational programs for children and families, and one-on-one care training, to group presentations at association meetings – we arm our patients’ caregivers with the tools and insight to feel confident they are properly caring for the bleeding disorder patient in their lives. As an added benefit, we offer a variety of material for quick reference in caring for a bleeding disorder patient.