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Jenna Cohen- Final Exam Study GuideHematologic and Immunologic Dysfunction Part IIAssessment – Hemophilia multiple hemophilias- Missing or deficient clotting factoro Example: hemophilia a is a deficiency in factor 8, the left factor 8 that person has the more severe the hemophilia will beo Factor VIII deficiency (Hemophilia A)o The less Factor VIII, the more severe the disease- Patho: Hemophiliacs have 2 of the 3 factors required for clotting: platelets and vascular influence; Therefore they may bleed longer but not faster just missing full cascade of factors- expected to bleed longer* have two of the three factors needed stillHemophilia - Assessment- 80% are X-Linked recessive inherited- Hematomas presentation to expect (mainly hemophilia a-bleeding possibilities main concern) with little cause or without cause: brusining, petechiae- any sx of bleeding under the skin or tissues- Hemarthrosis: bleeding into jointso Pain, limited ROM, degenerative joint problems- Epistaxis (See tx in Emergency box on pg. 1507)Hemophilia – Analysis- Risk for Bleeding -Perfusion big deal concern ourselves with significant bleeds that could leave to hypovolemia as well as bleeding into certain areas of the body, particularly gi bleeds, cerebral bleeds- Complications r/t bleeding – depends on site and severity:o Risk for Injury -Safety: Prvnt of Injury any organ damaged due to the bleedingo Decreased Intracranial adaptive capacityo Ineffective Tissue Perfusion -Perfusion if severe volume loss and severe loss of o2 carriers in our rbcso Impaired Gas Exchange -Oxygenation with significant loss ofo2 carrierso Acute / Chronic Pain -Pain typically bleeding into jointso Impaired Physical Mobility -Mobility- Therapeutic Regimen Management -Health Promotion depending upon how much med and tx they have to manage and how well that is goingFirst / Priority Intervention – Hemophilia- Replace missing factor: Amt received depends on severity on a somewhat regular basiso Routinely when hemarthrosis occurs o Prophylactic: 3 – 4 x / week occurs to prevent bleeds are getting more efficient products and don’t need as frequent replacement but prophylactic prevents futurebleedso Episodic: during an active bleed- Steroids: - during acute hemarthrosis and synovitis inflammation of synovial membranes to decrease the inflammation which will decrease swelling and likelihood to bleed- NSAIDS should be limited; they inhibit platelets if already deficient in clotting factors, don’t want to be deficient in another area of clotting process such as platelets- Amicar: prevent clot destruction, used with mouth or trauma surgery something given prophylactically to a hemophiliac to prevent them from breaking down materials unnecessarily and causing additional bleeding1989: changed to non-human factor sourceProphylactic: to prevent bleedsMost are treated at homeSteroids: hematuria, acute hemarthrosis and chronic synovitisNSAIDS: use w/ caution as they inhibit platelet functionAmicar: prevents clot destruction. Used with mouth or trauma surgery- Dental hygiene – modifications bc of tendency to easily bleed- soft toothbrushes, gentle methods rather than harder, bristled tooth brushes, if significant issue at the moment mayhave them just use swabs or towel for oral care- Medical alert bracelet if acute medical situation those taking care if they’re bleeding there is issue of missing factor, need to therapeuticly treat them in the right way opposed to trying to give them RBCs- SQ over IM route for injections if possible IM leads to more bleeding based on tissue its going into- Crippling effects of bleedso Elevate and immobilize during active bleedo Active ROM after acute episode, exercise and diet important o Adverse effects of medications, treatments Hemophilia- Evaluate:o Pain, VS and other signs of compensation, neuro status if concerned about significant amounts or areas of breathing; neuro status if concerned about bleeding in heado Daily life effects bc of need for repeated tx and meds, and for them to be careful with certain acitivities; don’t want them involved in big contact sportso Adverse effects of medications, treatments anytime talking about using these types of factor replacements, even though not human replacement, still run a risk of reaction to that product- Trends:o Look for signs of acute serious bleeds progressing (think shock signs, neuro changes, profuse bleeding)HIV in childrenMost prenatal / birth transmission prevention w/ prenatal meds- Most others are diagnosed early those transmitted via mom- Good prognosis if treated early and persistently biggest thing is compliance with med so that resistance doesn’t develop and let the virus take over- Assessment for possible later Dx: Failure to thrive, developmental delay, hepatosplenomegaly, oral candidiasis in childhood or other exposures- enlarged liver andspleen; opportunistic infections (oral candidiasis)o ELISA with Western Blot confirmation: for those 15 – 18 mo and older if + then a western blot confirmationo PCR: under 15 mo; Infant carries maternal antibodies for up to 18 mo making other tests inaccurate these time frames are in some shift, indicates different testing guidelines but this is a good baseline knowledge for which tests and which ageHIV – Analysis- Risk for Infection -Infection significant concern typically whenviral loads are high and t cell counts are low- Psychosocial concerns -Coping either being born into a social situation that’s concerning or have to be placed into different family setting based on psychosocial concerns prior to being born, often serious concerns; as child gets older, their friends/family knowledge of diagnosis how info is communicated and handled and addressed as child grows older is something we hae to think about- Therapeutic Regimen Management -Health Promotion daily meds are key to ot having hiv develop into aids and also making appts happen can be a big jobHIV – First / Priority Interventions- Watchful for Opportunistic infections keep them away from unnecessary infectionso PCP**, candidiasis, CMV, RSV, EBV pneumocystict… most dramatic and lifethrestening of opportunistic infection as chance of death who gets pcp is about50% within getting pcp for the 1st time, very significant keep them healthy from these things, candidiasis in the mouth particulatrly, rsv and cmv more prone and at risk for resp illnesses, Epstein barre virus – anything that can attack a more wekaned immune system

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SC NURS 412 - Final Exam Study Guide

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