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Home Treatment In Hemophilia patients+ case presentation
Noushin Beheshtipoor
MS in Pediatric NURSING
Shiraz university of medical sciences
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Definition:
Hemophilia refers to a group of bleeding disorders resulting from a congenital deficiency of specific coagulation proteins.
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Type:
Hemophilia A or classic (factor VIII def) = 75% all cases 1/10,000 male population
Hemophilia B or Christmas disease (FIX def) 1/100,000
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Hemophilia C ( FXI def) 2-3 % all hemophilia Vonwilebrands disease
1-3% US population over looked or under thereat in women ( lack of V W factor) does not function as it should in platelet adhesion or as a carrier of F VIII.
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Etiology :
In a bout 80% of all cases. The inheritance pattern is demonstrated as X – linked recessive or gene mutation.
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Clinical manifestation:
Hemophilia is manifested in different by bleeding tendencies that range from mild to moderate or severe .
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severityClinical severity Factor activity Bleeding tendency
Severe
(% 60-70cases)
<1% Spontaneous bleeding without trauma
Moderate 1-5% Bleeding with trauma
mild 5-50% Bleeding with severe trauma or surgery
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We should suspect Hemophilia when massive bleeding:
from site of umbilical (70%) after circumcision ( 30-40%) after falls with tooth eruption after minor trauma (ecchymosis) after minor trauma nose or gum bleeding after minor surgical procedure after IM or SQ injection
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Con,tCon,t
Limited in motion Tenderness, swelling Hemarthrosis Contractures Retroperitoneal and intra cranial bleeding
Can be life threatening
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Diagnostic evaluation: History of bleeding episodes Pedigree Carrier detection Bleeding time PT,PTT Factor VIII and F IX assays
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Therapeutic managementReplacement of missing clotting factor: VIII or IX concentrate DDAVP ( 1 desamino- 8- D- argining
vasopressin. Only in mild hemophilia type A and VWD [type I,IIA] )
Cryoprecipitate Plasma Prophylaxis (3 times/ week H.A , 2 times/week H.B)4/25/2013
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Nursing considerations:
1-Short term objective: Recognize and prevent
bleeding (RICE) Factor or suitable injection Reduce pain Impaired physical mobility Support patient and family
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cont
Assessment of neurological system
a. Examination of the headb. Pupillary reactionc. Level of consciousnessd. Tendon reflexese. Sensory Function
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2. Hematology
a. Public Displayb. Skin: (pallor, petechiae, bruising, bleeding from the mucous membranes or wounds or puncture vein injection)c. Abdomen (enlarged liver, spleen
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2-Long term objective
Knowledge deficit Self care and self infusion Educate the effect of
chronic disease on life style (Side of med)
Cope with disease Identify persons at risk
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Avoid taking Temperature rectally or use suppository Avoid CK blood pressure by cuff Avoid IM injection Avoid use Aspirin or ASA product Use finger stick for sampling
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Non invasive procedures without first replacing missing factors
Apply pressures 3-5 minute post venipuncture
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Child Abuse Adverse childhood experiences
emotional abuse crime emotional neglect substance abuse Depressed affect Anger
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Home Treatment
Home treatment for hemophilia includes:
Learning how to recognize when bleeding has started, administering clotting factors, eating well, and exercising regularly.
Learning how to care for yourself or a child with hemophilia at home can lead to a better quality of life.
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Home therapy allows immediate access to clotting factor and hence optimal early treatment,
resulting in decreased pain, dysfunction, and longterm disability and significantly decreased hospital admissions for complications.
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Home treatment must be supervised closely by the comprehensive care team and should only be initiated after adequate education and training.
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Home treatment in 10 steps: Home tre atment in 10 steps: Home treatment in 10 steps
step1- Find a responsible assistant Never Infuse alone Make sure some one is with you If complication arise it is critical
that some be treat to assist you or
call for helps
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Step2-
Calculating dosage
Check with treatment center the number Calculate of factor (U/Kg) Noticed that vials vary in the number
of units each one contains, check it
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The number of units varies according to:
Type of hemophilia A or B Type factor product selected Type and site of bleeding Kind of invasive procedure or
surgery
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Step3---and washing
Hands should be washed with soap and warm water for 15 second
Before infusing put gloves After removing gloves, wash
you're hands
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Step4- Reconstituting factor
Check the expiration date Store the vials in the refrigerator ( 2-8 C) Bring the bottle and water to room
temperature Rolling them between you're hands Remove protective cap Use Double ended needle (transfer
needle) Prevent foaming Don’t touched the exposed needle
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Step5-preaparing the site Select site -the back of the hand
-Forearm
-Inside of the elbow Do not use the same vein each
time Place tourniquets (rubber tissue) Put hand in warm water or Encourage fluid intake
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Step6-infusing the factors Select straight length of vein Use tourniquet Cleans the skin (alcohol pad) Insert needle to vein with 15-20
degree angle If blood return ,infuse factor by
pushing slowly(100 unit/minute)
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Step 7-
-Disposing of waste
cleaning up you're
devices
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Step 8-
Record and document you're treatment
Step 9-
Watching for:
1-allergic reaction -check
itching,rach,fever,runny nose,cough,wheezing,back pain,swelling,tight constricted feeling in throat
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If this signs appear immediately: -Give benadryle
-Sent to near hospital
-call hemophilia treatment center
2-Non allergic reaction -Skin &Blood infection - Local bruising, or bleeding
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Step10-
-Managing pain
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Pain caused by venous accessPain caused by venous Pain caused by joint or muscle bleeding
Post-operative pain
Pain due to chronic hemophilic arthropathy
Remember Important 5R Right Drug Right Dose Right Patients Right Route Right Rate
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Port A-CATHA port is a special device, which is used to
allow easier, more dependable access to veins. It is made up of an injection
port and a long hollow tube.
Same Names:
Broviac®, Port-A-Cath®, Hickman®, PICC® line
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Caring for Venous Access Device: Port‐a‐Ca
This is a sterile procedure An “L”Shaped needle (Huber Needle) will be
put through your skin and into the rubber part of the port
A sterile bandage will be put over the needle to hold it in place and to prevent infection
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The needleand bandage should be changed once weekly for as long as you are using your port
Flushing the Port. The port needs to be flushed every 30 daysflushed with
a heparin solution to prevent blood clots from forming inside the catheter
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When to report symptoms:
Temperature over 100.5 F Your face or neck gets swollen The area around the port is red, swollen, and
warm and/or painful. These are signs of infection. The pain in your shoulder, arms, and neck does
not go away or gets worse.
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Case Presentation:
7 Y/O BOY
From Fassa
Over protected with parents
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Thank you
For your Attention