Jenna Cohen Final Exam Study Guide Hematologic and Immunologic Dysfunction Part II Assessment Hemophilia multiple hemophilias Missing or deficient clotting factor o Example hemophilia a is a deficiency in factor 8 the left factor 8 that person has the more severe the hemophilia will be o 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 still Hemophilia 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 joints o Pain limited ROM degenerative joint problems Epistaxis See tx in Emergency box on pg 1507 Hemophilia Analysis Risk for Bleeding 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 Safety Prvnt of Injury any organ Perfusion big deal concern o Risk for Injury damaged due to the bleeding o Decreased Intracranial adaptive capacity o Ineffective Tissue Perfusion severe loss of o2 carriers in our rbcs o Impaired Gas Exchange o2 carriers o Acute Chronic Pain o Impaired Physical Mobility Therapeutic Regimen Management Perfusion if severe volume loss and Oxygenation with significant loss of Pain typically bleeding into joints Mobility Health Promotion depending upon how much med and tx they have to manage and how well that is going First Priority Intervention Hemophilia Replace missing factor Amt received depends on severity on a somewhat regular basis o 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 future bleeds o 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 bleeding 1989 changed to non human factor source Prophylactic to prevent bleeds Most are treated at home Steroids hematuria acute hemarthrosis and chronic synovitis NSAIDS use w caution as they inhibit platelet function Amicar 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 may have 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 bleeds o Elevate and immobilize during active bleed o 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 head o 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 sports o 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 children Most 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 and spleen opportunistic infections oral candidiasis o ELISA with Western Blot confirmation for those 15 18 mo and older if then a western blot confirmation o 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 age HIV Analysis Risk for Infection Infection significant concern typically when viral 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 job HIV First Priority Interventions Watchful for Opportunistic infections keep them away from unnecessary infections o PCP candidiasis CMV RSV EBV pneumocystict most dramatic and lifethrestening of opportunistic infection as chance of death who gets pcp is about 50 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 easily will be a concern Immunizations all recommended pneumococcal and flu o Varicella and MMR can be given in periods of strong cell counts to those who have