sickle cell en

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Sickle Cell Disease

Disease name: Sickle Cell Disease

Synonyms: Sickle Cell Anemia, Sickle-Hemoglobin C Disease, Sickle Beta-PlusThalassemia and Sickle Beta-Zero Thalassemia

Sickle cell disease (SCD) is a grou o! inherited disorders o! the beta-hemoglobin chain"#ormal hemoglobin has $ di!!erent t%es o! hemoglobin & hemoglobin A, A', and "Hemoglobin S in sickle cell disease contains an abnormal beta globin chain encoded b% asubstitution o! aline !or glutamic acid on chromosome **" This is an autosomal recessiedisorder" Sickle cell disease re!ers to a seci!ic genot%e in +hich a erson inherits oneco% o! the HbS gene and another gene coding !or a ualitatiel% or uantitatiel% abnormalbeta globin chain" Sickle cell anemia (HbSS) re!ers to atients +ho are homo%gous !or theHbS gene, +hile hetero%gous !orms ma% air HbS +ith genes coding !or other t%es o! abnormal hemoglobin such as hemoglobin C, an autosomal recessie mutation +hich

substitutes l%sine !or glutamic acid" .n addition, ersons can inherit a combination o! HbSand / -thalassemia" The /-thalassemias reresent an autosomal recessie disorder +ithreduced roduction or absence o! /-globin chains resulting in anemia" 0ther genot%e airsinclude HbSD, HbS0-Arab and HbS1"


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Disease summary

Sickle hemoglobin in these disorders cause a!!ected red blood cells to ol%merie under conditions o! lo+ o2%gen tension resulting in the characteristic sickle shae" Aggregation o! sickle cells in the microcirculation !rom in!lammation, endothelial abnormalities, andthrombohilia lead to ischemia in end organs and tissues distal to the blockage" .nheritanceo! sickle cell disease redisoses to !our main t%es o! crises3 aso-occlusie, slenicseuestration, alastic and hemol%tic" The morbidit% and mortalit% !rom these eents ma% bemani!ested as ain, acute chest s%ndrome (intraulmonar% sickling), ulmonar%h%ertension, cardiac abnormalities, cerebroascular hemorrhage4in!arct, slenic or heaticseuestration, autoslenism, renal disease, lier disease, aascular necrosis o! the !emoralhead, riaism, and li!e-threatening acute hemol%tic or alastic anemia" Triggers !or anacute crisis include h%o2emia, deh%dration, acidosis, stress, in!ection, trauma, h%othermiaor in some cases, no identi!iable redisosing risk !actors"

Current treatment !ocuses on reention o! comlications through earl% diagnosis b%ne+born screening, roh%lactic antibiotics, h%dration, ain management, disease-modi!%ing

theraies, and transcranial Doler screening (+ith subseuent trans!usion thera%) i! needed !or stroke reention" Additional emhasis is laced on disease reention throughgenetic counseling o! gene carriers !or sickle hemoglobin" #e+ theraies to cure or ameliorate s%mtoms include hematooietic cell translantation and h%dro2%urea thera%"5i!e e2ectanc% !or ersons +ith SCD (all genot%es combined) is reorted to be 6$ %ears !or men and 67 %ears old !or +omen"

Due to the ast arra% o! comlications o! SCD, ersons +ith these disorders o!ten reuiresurgical interention !or treatment or reention o! certain comlications" According to theCooeratie Stud% o! Sickle Cell Disease !rom *886, $9 da% mortalit% a!ter surger% +asreorted to be *"*: +ith three deaths reorted to be related to the surgical rocedure and4or anesthesia (9"$:) +ith no deaths in children under *;" This stud% reie+ed


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h%oer!usion and enous stasis, +hich can reciitate sickling" .n addition, an inadertenthigh leel o! block ma% comromise resirator% muscles leading to h%o2emia"

The Cooeratie Stud% o! Sickle Cell Disease !ound that SCD related comlications a!ter surger% +ere more !reuent in atients +ho receied regional anesthesia comared +iththose +ho receied general anesthesia" A more recent randomied, controlled, multicenter

trial, The Trans!usion Alternaties Preoeratiel% in Sickle Cell Disease (TAPS), rea!!irmedthe need !or reoeratie trans!usion rior to surger% to aoid li!e threatening comlicationssuch as acute chest s%ndrome" A combined techniue using regional anesthesia !or intraoeratie and ostoeratie ain control and general anesthesia !or surger% might bethe sa!est aroach"

There are no absolute contraindications to sedation in ersons +ith SCD> ho+eer, itremains crucial to aoid situations +hich ma% trigger an eent such as h%othermia,inadeuate o2%genation, h%oentilation, and4or inadeuate sedation leading to ain andasoconstriction"

Necessary additional diagnostic procedures (preoperative)

• Deendent on rocedure and atient=s comorbidities

• Hemoglobin electrohoresis

• Comlete blood count

• Comlete metabolic anel (should include lier !unction tests)

• Coagulation studies

• T%e ? screen or cross (order earl% to identi!% issues +ith antibodies and bloodaailabilit%)

• Chest 2-ra%4Pulmonar% !unction tests

• 1lectrocardiogram41chocardiogram

• Transcranial Doler (all atients +ith HbSS or HbSB9 disease should undergo TCDannuall% !rom age '-*@)

• Pol%somnogram (in atients +ho hae obstructie slee anea (0SA) or haes%mtoms concerning !or 0SA)

Particular preparation for airway management

Patients +ith SCD +ith a diagnosis o! obstructie slee anea (0SA) or susected 0SA ma%be redisosed to air+a% collase during the erioeratie eriod" Prearation should bemade to manage a otentiall% di!!icult air+a%> other+ise, no articular rearation is seci!icto this disease"


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Particular preparation for transfusion or administration of lood products

Because atients +ith sickle cell disease can receie man% blood roducts starting at a%oung age, these atients remain at high risk !or comlications related to trans!usions, suchas alloimmuniation, dela%ed hemol%tic trans!usion reactions, iron oerload and in!ectionsuch as H., heatitis, and aroirus B*8" These trans!usion issues can make it di!!icult toobtain comatible blood and blood roducts and there!ore deter routine administration o! blood and blood roducts"

The rocess !or t%ing and crossmatching and banking blood should be started earl%, since itma% take seeral hours4da%s to rocure the necessar% units o! blood" Persons +ith SCDshould receie henot%icall% matched blood including minor antigens i! !easible to decreasethe subseuent risk o! allo-immuniation"

Perioeratie blood trans!usion is commonl% er!ormed to reare SCD atients !or surger%"Trans!usion can be accomlished either as a simle trans!usion or as an e2changetrans!usion" The otimal trans!usion arameter to decrease sickling in atients is

controersial> ho+eer, ensuring atients do not e2ceed a hematocrit o! $9: has beensho+n to minimie the risk o! h%eriscosit% s%ndrome" Ho+eer, it is not al+a%s necessar%to increase a atient=s hematocrit aboe baseline !or simle rocedures" 12changetrans!usions hae the added bene!it o! reducing hemoglobin S to less than $9: o! the totalhemoglobin" A stud% comaring conseratie (simle) to aggressie (e2change) trans!usiono! blood !ound the conseratie trans!usion regimen as e!!ectie as the aggressie regimen+ith onl% hal! as man% trans!usion-associated comlications" 0ne stud% suggested thatreoeratie trans!usions might not be necessar% !or electie minor surgical rocedures suchas herniorrhah%, dental4oral surger%, ohthalmological surger% and t%manostom% tubelacements in atients +ho are clinicall% stable and near their reoeratie baseline leel o! anemia" Ho+eer, surgeries such as laarotom%, thoracotom%, tonsillectom% andadenoidectom% ose a much more signi!icant risk o! deeloing ostoeratie comlications"

0erall, di!!erent studies hae had di!!ering oinions regarding reoeratie trans!usions" ACochrane reie+ concluded that recommendations cannot be made and !urther rosectierandomiation studies must be done"

The decision to trans!use blood and blood roducts should be based uon the needs o! eachcase, the surgical risk, and the atient=s underl%ing condition" .! aailable, atients +ith SCDshould be assessed b% a cometent hematologist rior to undergoing surger%"

Particular preparation for anticoagulation

Consideration !or dee ein thrombosis should be gien to atients +ho ma% be immobilied!or a rolonged eriod o! time erioeratiel%"

Particular precautions for positioning! transport or moilisation

1arl% mobiliation combined +ith incentie sirometr% and chest h%siothera% ma% reduceulmonar% comlications" Persons +ith asthma o!ten bene!it !rom ost-oeratie albuterolthera%" Additional bene!it ma% be gained b% reoeratie trans!usion thera% (as aboe)" Aoidance o! !luid oerload and aroriate ain control ma% also mitigate the risk o! acutecomlications" Since man% ersons +ith SCD are narcotic tolerant, it is imortant to kno+ the

atient=s histor% in order to roide su!!icient ain control"


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Proale interaction etween anaesthetic agents and patient"s long term medication

edications taken b% sickle cell atients must be ealuated !or ossible drug interactions+ith anesthetic agents on an indiidual basis"

#naesthesiologic procedure

Consider lacement o! a eriheral intraenous catheter in the reoeratie holding area tobegin reoeratie h%dration or allo+ liberal intake o! clear liuids u to ' hours rior tosurger% time" The use o! a tourniuet !or lacement o! a eriheral intraenous catheter must be monitored to ensure adeuate er!usion to the e2tremit% distal to the tourniuet andto limit the time it is used to onl% the time needed to accomlish the rocedure" Surgicaltourniuets roiding a bloodless !ield reuire care!ul rearation and monitoring o! the SCDatient" Preious reorts re!erred to unusuall% high leels o! !etal hemoglobin, reoeratietrans!usion and inasie monitoring associated +ith use o! surgical tourniuets" Adancedlanning +ith the hematologist and surgeon are +arranted"

outine monitoring should include blood ressure, ulse o2imetr%, electrocardiogram, end-tidal C0' and temerature" aintaining o2%gen saturation aboe 8;: is likel% to imroeoutcomes as +ell as aoiding h%o2ia" Consider increasing the interal o! blood ressuremeasurements i! aroriate !or the rocedure and atient=s medical condition" Also,consider the addition o! inasie monitors as indicated b% rocedure or atient condition"

0erating room temerature should be set at a minimum ';oC or ma2imum achieable

based on atient=s age and underl%ing medical condition" Actie +arming deices are hel!ulin maintaining normothermia" H%othermia resents a signi!icantl% increased risk o! SCD-related comlications"

Standard intraenous or olatile anesthetics can be used, including nitrous o2ide"

Antibiotics should be administered +hen indicated and in set time interals throughoutsurger%"

Patients should be +ell reo2%genated +ith minimal time sent on lar%ngosco% andintubation +ith aoidance o! coughing, bucking or lar%ngosasm to reent h%o2emia,omiting and asiration o! gastric contents"

Sickle cell disease atients ma% hae chronic ain s%ndromes and reuire higher thannormal doses o! oioids and multimodal aduants !or ain control"

5ocal anesthetics ia neura2ial blocks or eriheral nere blocks can be used but atientsshould be monitored closel% as there hae been case reorts o! sickle cell crises inducederiheral neuroath% a!ter neura2ial blocks"

.n atients +ho reuire . contrast !or diagnostic imaging, iodinated contrast is relatiel%contraindicated because o! the high osmolalit% +hich can cause shrinkage and subseuentsickling o! red blood cells" Ho+eer, isotonic contrast has been !ound sa!e to administer"Pre-imaging h%dration is o!ten recommended !or atients reuiring . contrast"


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Particular or additional monitoring

Patient and surger% deendent"

Possile complications

A common comlication is ain !rom a aso-occlusie crisis in the ostoeratie eriod" Thisma% be triggered b% h%othermia, h%oentilation !rom slinting due to surgical ain or inadeuate ain control, inabilit% to mobilie !luids, or insu!!icient o2%genation" Comlicationrates +ere higher !or regional anesthesia than general anesthesia !or surgical rocedures"There!ore, udicious use o! analgesics, h%dration and o2%gen sulementation is critical toaoid or reduce the chances o! a ostoeratie comlication" .t is also crucial to monitor !luidbalance to reent !luid oerload"

Persons +ith SCD are at increased stroke risk comared to the normal oulation" This riskis eleated in atients +ith mo%amo%a s%ndrome or rior stroke" ort% one ercent o! SCDatients +ill be at risk !or recurrent cerebral ascular eents a!ter su!!ering a stroke" #ostandardied anesthesia guidelines e2ist on managing sickle cell atients +ith mo%amo%adisease" Patients need to be com!ortable, +ell h%drated, o2%genated, normothermic andhemod%namicall% stable" Persons on trans!usion thera% !or stroke reention should haetheir surger% otimall% timed to minimie their hemoglobin S burden"

The most common non-sickle cell disease-related comlication +as !ound to be !eer" Thecomlication rate +as lo+er +ith general anesthesia comared to regional anesthesia !or surgical rocedures" This +as also !ound to be true !or in!ections" Sickle cell atients areconsidered immunocomromised and should receie the same considerations in care asother atients +ith immunocomromised states, such as cancer atients receiing

chemothera% or A.DS" Thus, atients should undergo blood cultures and roh%lacticantibiotics as +ell as chest 2-ra%s i! demonstrating ulmonar% s%mtoms"

Postoperative care

Postoeratie destination should be determined on an indiidual basis +hether the atientcan be osted as an outatient or +ill need admission to a regular !loor, monitored !loor or intensie care unit"

Aggressie resirator% thera% +ith o2%gen, incentie sirometr%, chest h%siothera% and

bronchodilators should be instituted as +ell as adeuate ain control and h%dration"

Trans!usion thera% should be administered onl% i! indicated b% disease or due to ost-oeratie li!e-threatening comlications

$nformation aout emergency%like situations & Differential diagnostics

caused by the illness to give a tool to distinguish between a side effect of the anaesthetic procedure and a manifestation of the disease

Trans!usion reactions !rom clerical error or alloimmuniation ose a constant threat to sicklecell atients" Chronic trans!usions can cause deeloment o! antibodies to non-AB0 blood


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grous" This might result in dela%s in obtaining and trans!using blood in an emergenc%"Susicion o! a trans!usion reaction should result in stoing the trans!usion and !ollo+ing theinstitution=s rotocol !or managing atients +ith a trans!usion reaction"

Acute chest s%ndrome is the leading cause o! death among sickle cell atients accounting !or '9: to 76: o! the deaths according to di!!erent studies" The criteria !or diagnosis includes

the onset o! a ne+ lobar in!iltrate, as seen on chest 2-ra%, !eer greater than $7"6oC,resirator% distress, or chest ain" .t can be triggered b% !at embolism and4or in!ection,eseciall% communit%-acuired neumonia" isk !actors associated +ith resirator% !ailureinclude cardiac disease, older age, and a neurologic eent" Aggressie thera% is targetedto imroing o2%genation, h%dration, analgesia, bronchodilator thera%, broad-sectrumantibiotics and trans!usion thera%" The mortalit% rate is $: according to the #ational AcuteChest S%ndrome Stud%"

#mulatory anaesthesia

Consider onl% !or er% minor and lo+ risk rocedures"

'stetrical anaesthesia

Eeneral anesthesia +as identi!ied as a risk !actor !or ostnatal sickling comlications (acutechest s%ndrome, aso-occlusie crisis, stroke) in a stud% inoling 66 arturients, +hile theuse o! ehedrine +as not identi!ied as a risk !actor" The risk o! maternal death in thematernal oulation is *< times greater +ith general anesthesia than regional anesthesia"

#eura2ial techniues can be sa!el% administered but care!ul monitoring !or neuroath% andsickle cell crises is +arranted"


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(iterature and internet links

*" Adu-E%am!i F, Sankarankutt% , ar+a S" Gse o! a tourniuet in atients +ith sickle-celldisease" Can Anaesth *88$>;93';-<

'" Camous , #=da A, 1tienne-ulan , StIhan " Anesthetic management o! regnant +omen +ith

sickle cell disease & e!!ect on ostnatal sickling comlications" Can Anesth '997>663'*9*3$7*38$9-7

8" ohnson CS" Arterial Blood Pressure and H%eriscosit% in Sickle Cell Disease" Hematol 0ncolClin # Am '996>*83 7';'3$;'3*766-@6


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(ast date of modification: #pril )*+,

These guidelines have been prepared by:

#uthor Tae -. /im! 0.D., Clinical Associate, Deartment o! Anaesthesiolog% and Critical Careedicine, The ohns Hokins Hosital, The Charlotte " Bloomberg Children=s Center,

Baltimore, ar%land, GSAtkim6'Mhmi"edu

Co%#uthor 1ommy 2ong 0ershon! 0.D.! Paediatric Anaesthesia ello+, Deartment o! Anaesthesiolog% and Critical Care edicine, The ohn Hokins Hosital, The Charlotte "Bloomberg Children=s Center, Baltimore, ar%land, GSAbhong$Mhmi"edu

Peer revision +#dam Stanley! Deartment o! Anaesthesiolog%, Gniersit% o! Tennessee Health ScienceCenter, emhis, GSA

astanle$Muthsc"edu

Peer revision )3ulie /anter! Director Sickle Cell Disease esearch, Assistant Pro!essor, Paediatric Heme-0nc, edical Gniersit% o! South Carolina, GSAkanterMmusc"edu
mailto:[email protected]:[email protected]:[email protected]:[email protected]:[email protected]:[email protected]:[email protected]:[email protected]

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