Transcript خانم بهشتي -آموزش مراقبت از بیماران هموفیلی

Home Treatment In Hemophilia
patients+ case presentation
Noushin Beheshtipoor
MS in Pediatric NURSING
Shiraz university of
medical sciences
Definition:
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Hemophilia refers to a group
of bleeding disorders
resulting from a congenital
deficiency of specific
coagulation proteins.
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Type:
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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
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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 :
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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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severity
Clinical severity Factor activity Bleeding tendency
Severe
(% 60-70cases)
Moderate
<1%
Spontaneous bleeding
without trauma
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:
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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,t
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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 management
Replacement 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 ,
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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
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a. Examination of the head
b. Pupillary reaction
c. Level of consciousness
d. Tendon reflexes
e. Sensory Function
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2. Hematology
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a. Public Display
b. 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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
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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
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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
tre
atment
in
10
steps:
Home
treatment
in
10
steps:
Home treatment in 10 steps
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step1Find 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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Step2Calculating 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
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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
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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
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Don’t touched the exposed needle
Step5-preaparing the
site
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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
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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 8Record and document
you're treatment
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Step 9Watching 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
2-Non allergic
reaction
immediately:
-Skin &Blood infection
-Give
benadryle
- Local bruising, or bleeding
-Sent to near hospital
-call hemophilia treatment center
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Step10-
-Managing pain
Pain caused by venous
access
Pain caused by joint or muscle bleeding
Post-operative pain
Pain due to chronic hemophilic
arthropathy
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Remember Important
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5R
Right Drug
Right Dose
Right Patients
Right Route
Right Rate
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Port A-CATH
A 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
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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 needle
and bandage should be changed once weekly for as
long as you are using your port
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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:
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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
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