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PATHOPHYSIOLOGY

Non modifiable Risk Factors

Carrier mother of Hemophilia B

Male

Labs: Blood Typing: O+

Genetic predisposition

Factor IX deficiency

Tx: BT of FFP

HEMOPHILIA B

Impact of trauma

Meds: Diphenhydramin 50 mg IV prior to transfusion of FFP Furosemide 20 mg IV after blood transfusion

Easy bleeding

Easy bruising

Delayed clotting

Ankylosis

Labs: PT: 13.6 secs PTT: >300 secs

S/Sx: Hematoma Left hip hemathrosis Right elbow hemathrosis Immobility of joints Limitation of movements on joints

DIAGRAM
Modifiable Risk Factors

Chronic Smoker: 3 to 8 sticks per day

Alcohol Drinker: 3 bottles everyday

Possible Infection of H. pylori

Cytotoxic effect

Increased gastrin level

Increased gastric output

Decreased bicarbonate secretion

Altered mucosal blood flow Inflammatory response Meds: Omeprazole 40 mg PO OD Tramadol 50 mg q6h prn for pain Allow back diffusion of hydrogen ions

Affection of mucous production

Tissue damage (Mucosal defects)

Labs: EGD Body: erosion, linear bile stained mucosa Antrum: areas of erythema, edema, bile stained mucosa 2nd part Duodenum: positive areas of hematoma Post-endoscopic Diagnosis: Esophagitis LA Class A Hiatal hernia Erosive gastropathy Bille reflux gastropathy Duodenal hematoma

Diffusion of acid and pepsin into the cell

Epigastric pain

Ulceration of mucosal barrier BLEEDING PEPTIC ULCER DISEASE Further damage of blood vessels

Gastric outlet

UPPER GASTROINTESTINAL BLEEDING

Blood volume depletion Poor perfusion

Accumulation of blood in GI tract

Increased peristalsis

Nursing Diagnosis Impaired Comfort r/t abdominal pain and compression of somatic nerves of right elbow secondary to hemathrosis due to Hemophilia B

Nursing Interventions: Encourage verbalization of feelings about the pains Fears and concerns can increase muscle tension and lower threshold of pain perception. Verbalization of ones feelings presents to the client that it the feeling of pain is not made up and can be alleviated with proper interventions. Provide comfort measures such as frequent changes of position, back rubs, and support with pillows. Changes in position, back rubs, and back support with pillows relieves discomfort and creates a therapeutic state of analgesia to affected areas. Schedule rest periods, provide quiet environment. Rest periods prevent further fatigue and over metabolizing of the bodys nutrients thus producing slower tissue repair. Rest promotes quicker cell regeneration and over fatigue of the heart. Provide non-pharmacological methods. -Distraction techniques. Heighten ones concentration upon non-painful stimuli to decrease ones awareness and experience of pain. Emphasis will be given to breathing control. -Relaxation exercises. Techniques are used to bring about a state of physical and mental awareness and tranquility. The goal of these techniques is to reduce tension, subsequently reducing pain. Use of analgesics as prescribed Higher grade of pain may not be alleviated with non pharmacological approaches thus the need for PRN drugs that induces analgesia.

Sources: Brunner and Suddharth. (2007). Textbook of Medical-Surgical Nursing, 10th Edition. Bulechek, G., Butcher, H., Dochterman, J., (2008) Nursing Interventions Claissifications (NIC). Doenges M., Moorhouse M.F., & Murr A. (2010). Nurses pocket guide: diagnoses, prioritized interventions and rationales (12th Edition). Philadelphia, Pennsylvania: F.A. Davis Company. Doenges M., Moorhouse M.F., & Geissler A. (2000). Nursing care plans: guidelines for individualizing patient care (Edition 5). Philadelphia, Pennsylvania: F.A. Davis Company. Moorhead, S., Johnson, M., & Maas, M. (Eds.) (2004). Nursing Outcomes Classification (NOC) (3rd ed.). St. Louis, MO: Mosby. Porth, C. (2005). Pathophysiology: concepts of altered health states (7th Edition). Lippincott Williams and Wilkins.

Vomiting (hematemesis)

Decreased O2 carrying capacity

Increased blood loss

Hypoxemia

Anemia

Labs: RBC: 5.49x10^12/L Hgb: 173 g/L Hct: 0.523

S/Sx: Cold temperature CRT: >2secs General pallor

S/Sx: Easy Fatigability General weakness

Nursing Diagnosis Ineffective Peripheral Tissue Perfusion r/t Mechanical Reduction of blood flow s/t Upper Gastrointestinal Bleeding from Bleeding Peptic Ulcer Disease. Hemophilia B

Nursing Diagnosis Risk for Injury (Bleeding) r/t impaired blood coagulation s/t Hemophilia B

Nursing Interventions: Begin active ROM to all extremities. Rationale: Minimizes muscle atrophy, promotes circulation, helps prevent contractures. Consult with physical therapist regarding active, resistive exercises and patient ambulation. Rationale: Individualized program can be developed to meet particular needs/deal with deficits in balance, coordination, strength. Monitor patients ability to perform normal tasks/ADLs, noting reports of weakness, fatigue, and difficulty accomplishing tasks. Rationale: Assess patients ability to perform normal tasks/ADLs, noting reports of weakness, fatigue, and difficulty accomplishing tasks. Monitor BP, pulse, respirations during and after activity. Note adverse responses to increased levels of activity. Rationale: Cardiopulmonary manifestations result from attempts by the heart and lungs to supply adequate amounts of oxygen to the tissues. Assist patient to prioritize ADLs/desired activities. Alternate rest periods with activity periods. Write out schedule for patient to refer to. Rationale: Promotes adequate rest, maintains energy level, and alleviates strain on the cardiac and respiratory systems. Plan activity progression with patient, including activities that patient views as essential. Increase activity levels as tolerated. Rationale: Promotes gradual return to normal activity level and improved muscle tone/stamina without undue fatigue. Increases self-esteem and sense of control. Monitor laboratory studies, e.g., Hb/Hct, RBC count and PT and PTT. Rationale: Identifies deficiencies in RBC components affecting oxygen transport and treatment needs/response to therapy. Explain to the patient the importance and effects of massage in controlling pain level and providing comfort. Rationale: Secures willingness of patient to do massage on him. Do not use tapotement as a massage technique on the patient. Instead, use effleurage or petrissage. Rationale: Contraindicated in patients since it may elicit pain. Massage the extremities using long strokes from distal to proximal areas. Rationale: Helps in the return of blood to the heart.

Nursing Interventions: Provide information regarding health condition that may result increased risk of injury Identify safety devices to promote safe physical environment and individual safety Monitor the patient closely for hemorrhage indicates active bleeding Note hemoglobin and hematocrit levels before and after blood loss, as indicated to monitor for severity of bleeding Monitor for signs and symptoms of bleeding to check if there is an active bleeding Monitor PTT specifically as factor IX belongs to the extrinsic pathway Monitor orthostatic VS, including blood pressure as hypotension may be indicative of blood loss Administer blood products as appropriate to replace blood loss Protect patient from trauma Avoid injections, as appropriate to minimize source of bleeding Use soft toothbrush for oral care to prevent gum bleeding Coordinate timing of invasive procedures with platelet or fresh frozen plasma transfusions, as appropriate to ensure blood replacement Administer medication, as appropriate Instruct patient to avoid aspirin and other anticoagulants as these prevent clot formation Avoid constipation to prevent bleeding from GIT Instruct caregivers of signs of bleeding and report to physician to decrease risk of hypovolemia Remove potentially harmful objects from the environment. There are many possible triggers of bleeding, safety precautions such as removal of possible triggers would reduce the chances of bleeding occurring. Keep padded side rails up. Keeping the side rails up would be a safety precaution for It prevents the client from falling out of bed and having physical damage from the fall Avoid doing things that would cause you to bump or cut yourself. Wear non-skid slippers or socks when you are out of bed. This will help keep you from slipping or falling. Do not blow your nose hard. Cut nails short to avoid scratching the skin which may cause bleeding and bruising Increase intake of foods rich in Vitamin K to increase production of clotting factors If you start bleeding, control the bleeding by putting pressure on the bleeding area. Ice packs cool the area and decrease blood flow. Put the ice in a plastic bag and cover it with a towel. Place this over the injured area as directed by your caregiver. Call your caregiver if the bleeding does not stop after pressure and ice.