Taking your Care Back

1. Taking your Care Back A Trip to the Emergency Department

2. Introduction In a recent paper sent out to Emergency Room Doctors it states the following: “…Children with bleeding disorders confer different challenges to the emergency physician and their staff… Patients and family members (of bleeding disorder patients) are often very well educated in the disease and its management, which can significantly reduce morbidity and mortality.” So what can we do to take control of that care?

3. Obtain and Wear Medical ID such as Medic Alert This is critical. It is the first step to identifying your bleeding disorder. Such identification may save your life if you are injured and unable to speak for yourself!!! More information can be found at: medicalert.org Patientservicesinc.org

4. Be Knowledgeable!! • Take control of your bleeding disorder. Know more about your personal care than anyone. • If you want to receive effective treatment, you must be able to provide accurate, up-to-date information about you and your condition. Remember– these disorders are rare. Many of the healthcare workers you meet will have little or no experience treating a person like you. You may know the signs and the seriousnessof a bleed but you cannot assume the healthcare worker has the same understanding.

5. Be familiar with your treatment product • Which medication works best for you? • Dosage for major and minor bleeds • Maximum dosage (if there is one) • And how you respond to the medication • Know how to prepare the product The correct calculation is 25iu per kilo for mild bleeds and 50iu per kilo for severe bleeds. If you are visiting the ER, chances are you will need a severe dose. (pounds / 2.2 = your kilos) Clotting factor is always the best option to use if it is available. Blood products such as fresh frozen plasma or cryoprecipitate should be used only if the specific factor concentrates are unavailable!!!

6. Know where you are bleeding and what constitutes an immediate response with Factor!! True emergencies due to bleeding complications of hemophilia (Major Bleeding) include: • Head Trauma • Throat/Neck (possible airway restriction) • Abdominal Bleeding • Bleeding into a closed space causing compartment syndrome • Post-traumatic or Post-surgical bleeding Compartment syndrome is the compression of nerves, blood vessels, and muscle inside a closed space (compartment) within the body. This leads to tissue death from lack of oxygenation; the blood vessels being compressed by the raised pressure within the compartment. Compartment syndrome most often involves the forearm and lower leg

7. Know who your hemophilia provider is! You should always know the name and contact information for your Hematologist. An alternative would be to have the contact information for the HTC that you visit as they will have the most information about treating you specifically!!

8. Infuse First If you feel that the emergency is not life threatening and you feel that you will be able to access a vein quickly, infuse before you go to the ER. Be sure to have the information about how much of what product you used so that you can let the treating physician know. If you are unable to infuse first take your factor with you if you can. In the paper sent to the ER it states: “The patient’s supplies should be preferentially utilized; doses to be given will generally be specified by the patient who brings his own supplies. For patients who do not bring their own supplies, and for whom there is no medical information available, but who are very clear about the dose and type of product that they use, it is recommended that their or their families recommendations be followed immediately. “

9. At the ER What to Expect?

10. Who will you see? • Receptionist/Registration Clerk • Triage Nurse • ER Nurse • Physician

11. Saying the Right Things! • You will definitely need to repeat yourself to everyone you talk to. No matter if you told it to the last person you talked to, make sure that you stress that you have a bleeding disorder and that you need to have clotting factor ASAP. • If you find you are not getting prompt treatment, approach the triage nurse again and calmly repeat your problem. Make sure that the Registration and Triage Nurse Highlight that you have a bleeding disorder on your chart so that it is prevalent to everyone who sees it!!! Do Say: “I have hemophilia and I am bleeding into my calf muscle. I need to be seen and receive treatment right away.” Don’t Say: Hey, I’m a hemophiliac here and I need to see a doctor right now. You cant keep making me wait.”

12. Talking to the ER Staff When you are talking to the ER Staff be sure that you: • Speak up and say exactly what your concerns are. You need to be precise about your condition and your concerns. Do Say: “I have severe hemophilia factor VIII deficiency, I fell and hit my head. I could be bleeding into my head and will continue to bleed until I receive treatment. I need to be seen and treated quickly with factor VIII” Don’t Say: “I have fallen and hit my head” If you feel that you condition is potentially life threatening….SAY SO. Otherwise you may be sent to the waiting room to wait.

13. Triage Nurse Be sure to give the triage nurse all the pertinent information with regard to your bleeding disorder such as: • Type of bleeding disorder • Severity • Type of bleed you are experiencing • Current factor product and dosage • Presence of an inhibitor • Other medications • Other complications

14. ER Nurse • Make sure that they know what bleeding disorder that you have, the type of bleed that you have, what product that you use, and if you have an inhibitor. • State any concerns you have about your condition and treatment, such as the need for prompt infusion, and the easiest veins to access. • Know how to calculate your factor dosage. Offer to prepare the factor for the nurse. Don’t mix the factor without consent as they will probably need to be present to supervise. Generally a nurse can only infuse medication that they have prepared or for which they have supervised the preparation. Remember that you may need to repeat information about your bleeding disorder, and the treatment that you need to receive more promptly in order to get the care that you need.

15. ER Doctor • If necessary, inform the doctor of the need for prompt infusion to stop any bleeding prior to doing any tests. • Make sure that they know the bleeding disorder that you have, the type of bleed that you have, the treatment product that you use and if you have an inhibitor. • Make sure that your treatment is fully explained to you. Don’t be afraid to disagree if you feel that the treatment suggested isn’t the best one for your condition. • Remember that you may be more informed with regard to your own bleeding disorder than either the ER doctor or the Hematologist on call. • If you feel that you are not getting the right care, call your HTC or the hematologist on call and explain the situation.

16. Stay Calm The ER can be a noisy and hectic place. You may feel stress because of the situation. Staying calm can be difficult, but it is important. The ER staff will find it easier to understand you!! Listen Listen carefully to what the ER staff says so that you understand what they propose to do. Don’t be afraid to ask them to explain if you’re not sure you understand

17. Speak Up! Be Assertive! Tell the staff what you need and why you need it. State what the problem is and what concerns you have. If you don’t understand something or disagree with a treatment, SAY SO!!

18. Be Proactive when communicating with the ER Staff Don’t sit there worrying and feeling frustrated that you have to wait. The staff have probably heard of hemophilia or VWD before, but you can help them by reminding them that people with bleeding disorders suffer from prolonged bleeding if not given prompt effective treatment.

19. Be knowledgeable about your bleeding disorder ER staff cannot possibly know everything about your bleeding disorder. Provide information to the best of your ability. Do Say: “Hemophilia is an inherited bleeding disorder that affects mostly men. Hemophilia patients have low levels of clotting factor in their blood which results in prolonged bleeding. I am under the care of DR.____________ at the __________Hemophilia treatment center. Here is the phone number. There is someone on duty who can advise you on my treatment.”

20. Focus on the Problem…... Not the People! You are there for a reason that could be complicated by a bleed- that is the problem at hand. If necessary, repeat to the staff why you are there and why prompt treatment is important. Do Say: “I need to receive prompt treatment for this knee bleed. If not, there could be serious damage to the joint.” Don’t Say: “This is ridiculous how long I have been waiting here. What are you people doing back there? It’s your job to provide treatment for me so where is it? I’m calling the hospital administration about this.”

21. Use “I-statements” ...not “You-statements” Don’t tell staff what to do. It is more effective to focus on your own needs. Do Say:“I understand that you are very busy but I have hemophilia and I may be bleeding internally. I need to be seen and receive treatment with factor concentrates quickly.” Don’t Say: “You have to infuse me now before you do any x- rays.”

22. Be Prepared for Difficult Situations At times you may find yourself in a difficult situation where medical staff fails to understand your need for prompt treatment. They may refuse to follow your advice. This can be very frustrating.

23. Remember to Be Polite and Courteous The healthcare professionals are trying to do their jobs to the best of their abilities. Remember that patients with bleeding disorders such as hemophilia and vonWillebrand Disease are rarely seen in the ER. The person you meet may never have had the opportunity to treat anyone with your condition. It is unfair to expect them to know a lot about it.

24. Situations • I can’t have you infuse until I have determined the cause of the bleeding. I’ll need to order some blood work and a few x-rays first to be sure. • I think this bleed in your leg will just go away by itself over time. It’s probably just a pulled muscle. I really don’t see the need for any treatment now. • It’s 3 a.m. I’m not going to call the clinic and get someone out of bed for advice. I’ll just run some tests and we’ll figure things out.

25. In Conclusion Many ER visits go extremely well. You are seen quickly, the staff listens to your concerns, treatment is prompt and effective, and you go home with appropriate follow-up instructions and the peace of mind that the problem has been taken care of. On the other hand, you must be prepared for the times when treatment does not go so smoothly. You may be confronted with a long line-up, staff that may not understand your need for prompt care or that refuse to follow your advice. Remember that you have the right… • To be treated in a respectful, courteous manner • To participate in decisions with respect to your health care • To be informed about any treatment proposed • To consult with another person before you consent to any procedure • To refuse to consent to treatment

26. Remember… Hope for the best but be prepared for the worst!!

27. Sources • The Journal of Emergency Medicine, Vol. 39, No. 2, pp.158-165, 2010 • http://www.hemophiliaemergencycare.com/ • http://www.hemophilia.ca/files/er2.pdf