Lukes Presentation 1

8/17/2019 Lukes Presentation 1

1/28

Sickle-CellAnemia


2/28

Sickle cell anemia is a

disease passed down

through families in which

red blood cells form anabnormal crescent shape.

Defnition


3/28

Causes/EtiologySickle Cell Anemia is an inherited disorder in which a child

inherits one abnormal gene from each parent. This is known asautosomal recessive inheritance .


4/28


5/28


6/28

• These abnormal genes produce an abnormal type of bloodhemoglobin called hemoglobin S .

•

RBC containing HbS loses its round pliablebiconca!e disk shape and becomes deformedrigid and sickled-shaped

• Sickle-shaped cells deli!er"#$ to the body%stissues. They also can clog more easily in small

blood !essels and break into pieces that disruptblood &ow the patient may ha!e pain swellingand fe!er

• 'f the RBC is again e(posed to ade)uate amount ofo(ygen before the membrane becomes too rigid itcan re!ert to a normal shape

• Cold can aggra!ate the sickling process


7/28

*#* +#,' 'AB /

0. Hereditary

$. Race

Sickle cell anemia is most common in people of African'ndian +iddle /astern or +editerranean descent.

*e!ertheless it can occur in all peoples.

Risk Factor


8/28

Signs and symptoms of sickle cell anemia usually show up afteran infant is 1 months old'ncludes2

Anemia RBCs usually li!e for about 0$3 days before they die and

need to be replaced. Howe!er sickle cells die after only 03 to $3days.

4ithout enough red blood cells in circulation your body can%t getthe o(ygen it needs to feel energi5ed.

Signs and Symptoms


9/28

Delayed growthRBCs pro!ide your body with the o(ygen and nutrients you need

for growthShortage of healthy red blood cells can slow growth in infants and

children and delay puberty in teenagers

Jaundice't is a yellowing of the skin and eyes that occurs because of li!er

damage or dysfunctionSickle cell anemia ha!e some degree of 6aundice because the li!er

which 7lters harmful substances from the blood is o!erwhelmed by therapid breakdown of red blood cells


10/28

• Hand !oot syndromeSwollen hands and feet may be the 7rst

signs of sickle cell anemia in babies. Theswelling is caused by sickle-shaped redblood cells blocking blood &ow out of theirhands and feet.

• Fre"uent in!ectionSickle cells can damage your spleen an

organ that 7ghts infection. This may makeyou more !ulnerable to infections.


11/28

#year old and up$ision %ro&lem

Tiny blood !essels that supply your eyes may becomeplugged with sickle cells. This can damage the retina8portion of the eye that processes !isual images9.

Episodes o! pain 'Sickle Cell Crisis(

:ain de!elops when sickle-shaped red blood cells blockblood &ow through tiny blood !essels to your chestabdomen and 6oints. :ain can also occur in your bones.

The pain may !ary in intensity and can last for a few hoursto a few weeks.


12/28


13/28

)est Defnition %urpose

Hemoglobinelectrophoresis

Hemoglobin

electrophoresis is a testthat

measuresthe di;erenttypes of the

o(ygen-carryingsubstance

8hemoglobin9 in theblood.


14/28

Sickle Cell)est

A sicklecell testlooks !or

thepresence

or

a&senceo!

a&normalhemoglo&in in the

&lood thatcauses

sickle cellanemia+

)his testis done totell i! you

ha,ea&normalhemoglo&

in thatcauses

sickle celldisease

and sicklecell trait+

*egati,e SickleCell )rait

SickleCell

Anemia


15/28

Complete-loodCount

'C-C(

.t measuresthe o!

R&Cs0 o!1-Cs0 totalamount o!H& in the

&lood0 2 o!the &lood

composed o!R-Cs'hemat

ocrit(

pro,idesspecifcin!ormationthe si3e andhemoglo&incontent o!indi,idualred &loodcells+

.t is ascreening

test used todiagnose

and managenumerousdiseases+

R-C45ale46+7 to8+#millioncells/mc9Female4 6+:to ;+6 millioncells/mc9

1-C4 60;


16/28

• *ursing +anagement• :ro!ide information about what the client may feel• /ncourage )uestions and dialogue about fears and apprehensions• 'nform the client of the time period before the results will be a!ailable


17/28

5anagement

• +edical• :atients with sickle cell disease need ongoing treatment e!en when they

are not ha!ing a painful crisis.#+ Acetaminophen

Classi7cation2 AnalgesicAction2 't is used for the relief of fe!er as well as aches

and pains associated with many conditions. 'ndication2 +ild :ain= e!er >eneric 8Brand9 names2 Acetaminophen' )empra0 )ylenol(


18/28

$ . Hydro@yurea

Classi7cation2 Antimetabolite

Action2 inhibits production of Hb which inhibits sickling

'ndication2 to reduce fre)uency of painful crises and

need for blood transfusions in adult

patient with Sickle Cell Anemia with recurrent

moderate to se!ere painful crises

>eneric 8Brand9 names2 Hydro@yurea 'Dro@ia0 Hydrea(


19/28

? + -lood )rans!usion

'n a red blood cell transfusion red blood cells are

remo!ed from a supply of donated blood. These donated

cells are then gi!en intra!enously to a person with sickle cell

anemia.Blood transfusions increase the number of normal red

blood cells in circulation helping to relie!e anemia. 'n

children with sickle cell anemia at high risk of stroke regular

blood transfusions can decrease their risk of stroke.


20/28

1. Supplemental o@ygen

Breathing supplemental o(ygen through a breathing

mask adds o(ygen to your blood and helps you breathe

easier.


21/28

• *on %harmacological Highlights

0. Supplements of olic Acid8@itamin B9

-essential for producing red blood cells

$. luids-help pre!ent dehydration- gi!en either by mouth or through a !ein

?. Heating pads


22/28

• Surgical• -one marrow transplant

This procedure replaces bone marrow a;ected by sickle cellanemia with healthy bone marrow from a donor who doesn%t ha!ethe disease. 't can be a cure but the procedure is risky and it%sdi cult to 7nd suitable donors.

Bone marrow used for a transplant must come from a closelymatched donor. This is usually a close family member who doesn%tha!e sickle cell anemia.

Bone marrow transplant re)uires a lengthy hospital stay. Afterthe transplant you%ll need drugs to help pre!ent re6ection of thedonated marrow.


23/28

,iagnosis2 Acute pain related to tissue hypo(ia

'nter!entions2

-promote calm and rela(ing en!ironment to di!ert client sawareness to pain

-di!ertional acti!ities like watching tele!ision use of imagery etc.-perform warm compress on the site of pain to pro!ide relief

-encourage families to maintain a normal life for the child with SCAnemia

#utcome2 Client will be able to relie!e pain.

*ursing Diagnosis


24/28

,iagnosis2 Risk for infection related to inade)uate secondary defense

'nter!entions2

-promote childhood immuni5ation program-encourage adults to update immuni5ations to reduce thetransmission of global infection

-emphasi5e necessity of taking anti!iral=antibiotics

-re!iew indi!idual nutritional needs appropriate e(ercise programand need for rest

-encourage fre)uent proper hand hygiene

#utcome2 Client will be able to pre!ent the spread of potentiallyinfectious microorganism.


25/28

,iagnosis2 'mpaired gas e(change related to o(ygen carryingcapacity of the blood

'nter!entions2-re!iew o(ygen-conser!ing techni)ues

-instruct in the use of rela(ation stress reduction techni)ues

-encourage appropriate acti!ity and e(ercise to impro!e)uality of life

-emphasi5e the importance of nutrition in impro!ing staminaand reducing the work of breathing

#utcome2 Client will be able to ha!e an ade)uate o(ygenationof tissue.


26/28

'n the past sickle cell patients often died from organ failurebetween ages $3 and 13. Thanks to a better understanding andmanagement of the disease today patients can li!e into their 3sor beyond.

Causes of death include organ failure and infection. Somepeople with the disease e(perience minor brief infre)uentepisodes. #thers e(perience se!ere long-term fre)uent episodeswith many complications.

%rognosis


27/28

Red grape skin may help sickle cell su erers' Bcto&er =#0 :ESTA >a. F An e(tract in red grape skin may be a new treatment forsickle cell disease +edical College of >eorgia researchers say.

Study sing $iagra .ngredient to )reat Sickle Cell

Cut Short ' July :>0 :


28/28

• Brunner and Suddarath s GTe(tbook of +ed-Surgical *ursing 00 th /dition

• Io5ier ans /rb s undamentals of *ursing• Taber s Cyclopedia +edical ,ictionary .A. ,a!is• ,isease and disorders *ursing Therapeutic +anual• Http2==en.wikipedia.org=wiki=SickleJCellJ,isease• http2==www.nlm.nih.go!=medlineplus=ency=article=333 $K.htm• http2==www.mayoclinic.com=health=sickle-cell-anemia=,S33?$1• *urses s :ocket >uide 00 th /dition

Re!erences

Lukes Presentation 1

Documents

Transcript of Lukes Presentation 1