NATURE AND SCOPE OF THE HEMOPHILIA NURSE John C NATURE AND SCOPE OF THE HEMOPHILIA NURSE John C. Urgo RN, BSN Rush Medical Center USA

Mission Statement of an HTC The goal of an HTC is to foster self-supporting and self- sustaining behavior in patients with hemophilia and associated bleeding and clotting disorders. We believe the use of aggressive preventive measures, such as patient and family empowerment, home therapy, factor prophylaxis and promotion of joint health will decrease patient morbidity and mortality, promote integration of the patient into the community, and enhance quality of life.

Needing to Moving Forward Most hematologists focus their practice on malignant diseases and only provide care for patients with bleeding disorders out of necessity. Competence and the ability to provide multidisciplinary care to patients with Hemophilia is lacking in many areas of the country and the world.

CLINICAL PRACTICE: EDUCATION BLEED IDENTIFICATION Joint Muscle Soft tissue Nose Mouth ASSESSMENT Bruising, Swelling, Warmth, Range of Motion TREATMENT Determined by Type of bleed Available blood products

Daily Nursing Duties Scheduling patients for clinics. Room patients, VS, history, immunizations, confirm medications, social issues and school issues. Triage phone and message pool calls, return pages. Education an re-education daily as well as reinforcement of said education. Meeting with other clinic staff on a inpatient level regarding patient care. During Comprehensive Clinic visits the following clinical needs are assessed and discussed with the clinical care team. (i.e. Dental, Physical Therapy, Pharmacy, Nutrition, Surgery, Psychosocial, Genetics, Pain management ) Teach patients, parents and caregivers to infuse on their own.

NURSES ROLE: EDUCATION Bleed Management Pharmacologic Factor Amicar Non-pharmacologic Rest, Ice, Compression, Elevation (RICE) Pain Management Analgesics Narcotics

SIGNS & SYMPTOMS OF Bruising Very common Rarely dangerous Treatment may not be required For severe bruising, use an ice pack Contact parents if needed. May look unsightly and lead to teasing, body image sensitivity or suspicion of child abuse.

SIGNS & SYMPTOMS OF: BRUISES

TYPES OF BLEEDS: Bruising

TYPES OF BLEEDS: SOFT TISSUE

SIGNS & SYMPTOMS OF: Muscle bleed Warm to the touch Pain Swelling Tenderness Limited range of motion Guarding

TYPES OF BLEEDS: Muscle

TYPES OF BLEEDS: MOUTH

TYPES OF BLEEDS: Mouth

CLINICAL PRACTICE: EDUCATION Complications: Physical (vascular; neurological) Hepatitis (A, B, C, ) HIV Inhibitor Thromboembolism Allergic reactions

CLINICAL PRACTICE: HISTORY Bleeding: Signs & Symptoms, Management Pain level, Management Bleed Management Psycho-social issues Barriers to Care

Comprehensive Clinic Nursing Evaluation History was obtained from self on 9/8/16.   Since the time of his last comprehensive visit on 9/15 Anthony has been well. The interval history is 6 months. There have been 2 ED visits for elbow bleeds. Immunizations up to date: Yes No There has been 2 episodes of joint bleeding, these have been in the following joint: bilateral elbows   soft tissue bleeding: gum bleeding   There is acute or chronic pain. Management of the pain involves the use of marjuanna. Is a treatment log kept? Yes No Was it brought with you? Yes No  The activities of daily living   restricted (limited recreational activity, limited school/work, requires assistance for self care, requires assistance for all activities). Anthony is right handed.  The following assistive devices are used: cane, crutches, ACE wraps or splints, wheel chair, brace.  Days absent from school: n/a and from work 0.   Medical alert device used: bracelet, pendent, anklet, wallet card, other.---ordering this in clinic today.

Comprehensive Clinic Nursing Evaluation  Current factor replacement therapies used include:(regimen: primary prophylaxis, secondary prophyalxis, on-demand, enhanced on demand, immune tolerance) Benefix 13,450 IU once weekly as prophy Last Dose: 2 days ago  Product choice reviewed yes and patient assistance programs available to the patient reviewed yes. Home therapy Yes. No. Self -infusion If no, who infuses factor? Mother The home care company Diplomat. Venous access: PIV, PAC, CVAD, AVF Problems or concerns raised regarding venous access: Yes. No Additional teaching or support requested? Yes. No Are infusion records maintained? Yes. No If yes, in what form? n/a Nursing estimate of adherence with prescribed treatment? 100% (0-100%)

CLINICAL PRACTICE: SKILLS Venous Access Peripheral Central venous line: Port-a-cath, Hickman, Broviac Access Heparin Blood draws Dressing care

CLINICAL SKILLS: FACTOR INFUSION: SUPPLIES NEEDED Central Line Factor Hueber Needle Alcohol Pad Gauze Bandaid Saline Flush Heparin Flush

CLNICAL SKILLS: FACTOR INFUSION: SUPPLIES NEEDED Peripheral Factor Needle Butterfly Angiocath Tourniquet Alcohol Pad Gauze Bandaid Saline Flush

CLNICAL SKILLS: ADMINISTER VACCINATIONS Administration of vaccines Hepatitis A Hepatitis B

CLINICAL PRACTICE: COMMUNICATION Documents patient assessment, plan, and interventions Patient advocate Resource for multidisciplinary providers Arrange homecare

Contact Information John Urgo RN BSN Rush University Medical Center Direct Line 312-942-4715 Fax Line 312-942-8975 John_urgo@rush.edu