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