PEDIATRIC ACUTE CHEST SYNDROME (ACS)

Transcription

1 PEDIATRIC ACUTE CHEST SYNDROME (ACS) Patients with sickle cell disease presenting with 1) a new pulmnary infiltrate n chest radigraphy AND 2) evidence f lwer airway disease (e.g. cugh, shrtness f breath, retractins, rales, etc.) TABLE OF CONTENTS Algrithm- N/A Target Ppulatin Backgrund Definitins Initial Evaluatin-N/A Clinical Management Diagnstic Tests Fluids Nutritin Respiratry Therapy Table 1. Pediatric Asthma Scre Treatment Table 2. Antimicrbial Medicatin Table 3. Pain Medicatin Table 4. Respiratry Medicatin Discharge Criteria References Clinical Imprvement Team TARGET POPULATION Inclusin Criteria Patients with sickle cell disease (SS, SC, Sβ0 thalassemia, Sβ+ thalassemia) Patients f all ages Patients treated year rund Exclusin Criteria Patients withut infiltrate n chest radigraph (e.g. asthma exacerbatin) Patients with severe pulmnary hypertensin Patients pst bne marrw transplant Well children with pneumnia Page 1 f 10

2 BACKGROUND DEFINITIONS Acute chest syndrme (ACS) is the secnd mst cmmn reasn fr hspitalizatin in children with sickle cell disease and a leading cause f mrtality. ACS is defined as a new pulmnary infiltrate n chest radigraph in the presence f evidence f lwer respiratry tract disease (e.g. sme cmbinatin f cugh, shrtness f breath, retractins, rales, etc.). In the majrity f cases f ACS, an etilgy is unable t be identified. The mst cmmn identified etilgy f ACS is infectin but it may als result frm pulmnary vas-cclusin, pulmnary infarctin r fat emblism. The primary infectius agents implicated in ACS include: Chlamydia pneumniae, Mycplasma pneumniae, Streptcccus pneumniae, and viruses. Risk factrs fr ACS include vas-cclusive pain crisis, anesthesia, and surgery. Patients are at increased risk fr strke in the tw weeks immediately fllwing an episde f ACS. CLINICAL MANAGEMENT Hspitalize n hematlgy service Vital signs q 2-4 hurs depending upn degree f respiratry cmprmise Recrd pain scre every 4 hurs Cntinuus cardirespiratry mnitr and pulse ximetry Encurage ambulatin: ut f bed t chair r ambulating at least 2-3 times per day Drplet precautins Cntinue medicatin fr reactive airway disease if applicable DIAGNOSTIC TESTS CBC with differential, platelet cunt, and reticulcyte cunt initially and daily until imprving (cmpare with patient's baseline values) CXR initially, repeat fr clinical deteriratin (be aware that the x-ray ften underestimates the degree f invlvement and may appear wrse when the child is clinically imprving) Cnsider: Type and crssmatch fr severe illness r if Hb is greater than 1 g/dl belw baseline. Request minrantigen-matched, sickle-negative, leukcyte-depleted RBC Bld cultures if febrile (greater than r equal t 38.3 C) r histry f recent fever (d nt need t repeat daily) Arterial bld gas per clinical discretin Renal (BUN, creatinine) and liver (fractinated bilirubin, ALT) functin tests fr severe illness r if diffuse encephalpathy present (rule ut acute multi-rgan failure syndrme) If severe abdminal pain cnsider an ultrasund fr gallstnes Influenza A& B screening during the apprpriate seasn We d nt recmmend rutinely sending a respiratry pathgen PCR Echcardigraphy is nt recmmended rutinely in patients with acute chest syndrme. Cnsult cardilgy if cncern fr pulmnary hypertensin arises (prlnged hypxemia, fixed split S2 r prnunced pulmnary cmpnent f S2, hepatmegaly, persistent peripheral edema, r persistent pulmnary edema despite adequate fluid status) FLUIDS NUTRITION Daily weight Recrd intake and utput strictly Page 2 f 10

3 Maintain "euvlemia". IV + P.O. = 1 x maintenance. Mre fluid is apprpriate nly if patient is dehydrated r if insensible lsses are increased (e.g. persistent fever). IV fluid shuld be D5 1/4 NS t avid exacerbating the sickling prcess. RESPIRATORY THERAPY Cnsult pulmnlgy Clinical features suggestive f asthma r acute brnchspasms make an initial assessment using the Pediatric Asthma Scre (PAS); trial Albuterl 4 puffs with a spacer r 2.5 mg nebulized nce. Reassess using the PAS, a psitive respnse is a decrease f 2 r mre in the PAS. If a psitive respnse is nted, rder Albuterl 2-4 puffs r Albuterl 2.5mg nebulized Q4 and PRN. If at any time PAS wrsens by 2 r mre increase frequency f the Albuterl and ntify the prvider Lung expansin strategies: EzPAP (alng with IS) Q4 hurs fr 72 hurs. After 72 hurs f the initiatin f the therapy, the patient will be evaluated by the respiratry therapist fr pulmnary stability. If the chest x-ray (if dne) remains stable, there are n signs f pulmnary infectin, there is gd aeratin thrughut all lung fields upn auscultatin, and the patient can cnsistently achieve 14ml/kg with the IS, the EzPAP will then be discntinued. The patient will then receive IS Q2 hurs while awake and Q4 hurs at night by their RN Transfer t the ICU if patient is requiring Heated High Flw Nasal Cannula (HHFNC), invasive r nn-inavsive (CPAP, BiPAP) mechanical ventilatin is being cnsidered TABLE 1. PEDIATRIC ASTHMA SCORE Scre Respiratry Rate 2 t 3 years 4 t 5 years 6 t 12 years Older than 12 years 34 r less 30 r less 26 r less 23 r less 35 t t t t r greater 36 r greater 31 r greater 28 r greater Oxygen Requirements Greater than 90% n rm air 85% t 90% n rm air Less than 85% n rm air Auscultatin Nrmal breath sunds t end-expiratry wheeze nly Expiratry wheezing Inspiratry and expiratry wheezing t diminished breath sunds r pr aeratin Retractins Zer t ne site Tw sites Three r mre sites Dyspnea Speaks in sentences, cs and babbles Speaks in partial sentences, shrt cry Speaks in single wrds/ shrt phrases/grunting Nte: Use PAS Scre t guide interventin & respnse t treatment. Older pediatric Page 3 f 10

4 TREATMENT Antipyretics Acetaminphen dse accrding t manufacturer s recmmendatins PRN temperature greater than r equal t 38.3 C after bld cultures have been btained n at least ne ccasin Antibitics (see Table 2 fr chices and dses) Ceftriaxne and azithrmycin is the regimen f chice fr initial inpatient management If a respiratry pathgen PCR is sent fr anther reasn and is negative fr Mycplasma and Chlamydphila, azithrmycin may be discntinued If there is a knwn r suspected cephalsprin allergy, levflxacin is the regimen f chice and azithrmycin can be mitted because levflxacin has adequate cverage f atypical rganisms Strngly cnsider adding vancmycin fr severe illness, r if large infiltrate with pleural effusin present and S. aureus is suspected Prphylactic penicillin shuld be discntinued while patient is receiving antibitics If antibitics are cntinued upn discharge, an apprpriate ral antibitic shuld be cntinued t cmplete a curse f 7-10 days (including inpatient IV therapy received): Amxicillin-clavulanate is recmmended as first line, cefpdxime is the drug f chice in the presence f a penicillin allergy, and levflxacin is the drug f chice in the presence f a cephalsprin allergy. Analgesia (if indicated) If pain is present patients shuld be started n scheduled anti-inflammatries and piates if there are n cntraindicatins Transfusins Start ketrlac, but limit t 48 hur maximum duratin then start ibuprfen PO q6h (nt PRN) if n cntraindicatin present (i.e. gastritis, ulcer, cagulpathy, renal impairment). If pain des nt respnd t anti-inflammatry alne, cnsult the sickle cell vas-cclusin guidelines. Be aware narctic administratin may further suppress respiratin Give simple bld transfusin (10 ml/kg red bld cells) t imprve xygen carrying capacity t peple with symptmatic ACS whse hemglbin cncentratin is greater than 1 g/dl belw baseline and hemglbin wuld nt rise t mre than 10 g/dl. If baseline hemglbin is 9 g/dl r higher, may require red cell exchange transfusin. D nt transfuse acutely t Hb greater than 10 g/dl, Hct greater than 30 percent if percent sickle hemglbin is r is presumed t be greater than 30 percent since it is assciated with inducing pain and strke Urgent exchange transfusin may be indicated after cnsultatin frm hematlgy, critical care, and apheresis specialists when there is rapid prgressin f ACS as manifested by xygen saturatin belw 90 percent despite supplemental xygen, increasing respiratry distress, prgressive pulmnary infiltrates, and/r decline in hemglbin cncentratin despite simple transfusin and/r inability t transfuse due t high baseline hemglbin. May require transfer t ICU and transfusin medicine cnsult fr erythrcytapheresis. Remve femral r central venus catheters as sn as pssible after exchange transfusin t reduce risk f thrmbsis Page 4 f 10

5 TABLE 2. ANTIMICROBIAL MEDICATIONS Medicatin Dsing Indicatin/Ntes Antimicrbial Outpatient 1 st Chice 90 mg/kg/day (maximum 3,000 mg/day if using Amxicillinclavulanate (PO) suspensin and 2,625 mg/day if using tablet) PO Use either amxicillin-clavulanate ES divided TID suspensin mg/5mL r mg tablet frmulatins Antimicrbial Inpatient/Atypical Cverage Azithrmycin (IV/PO) *Highly Biavailable* Cefpdxime (PO) Ceftriaxne (IV) Levflxacin (IV/PO) *Highly Biavailable* Vancmycin (IV) 10 mg/kg/day (maximum 500 mg/day) PO x 1 dse, fllwed by 5 mg/kg/day (maximum 250 mg/day) PO nce daily x 4 dses 10 mg/kg/day (maximum 400 mg/day) PO divided BID 50 mg/kg/day (maximum 2,000 mg/day) IV q24h Less than 5 years: Levflxacin 20 mg/kg/day (maximum 750 mg/day) IV r PO divided q12h Greater than r equal t 5 years: 10 mg/kg/day (maximum 750 mg/day) IV r PO q24h Cntact pharmacy fr recmmended dsing Dsing interval based n age/renal functin) Discntinue if RPP results negative fr Chlamydphila and Mycplasma Nt necessary t use azithrmycin with levflxacin as levflxacin cvers atypical bacteria Antimicrbial Outpatient fr penicillin allergy *Ensure prescriptin sent in advance (variable stck at sme utpatient pharmacies) Antimicrbial Inpatient 1 st Chice Discntinue prphylactic penicillin while patient is receiving brad-spectrum antimicrbials Antimicrbial Inpatient and/r Outpatient fr cephalsprin allergy Discntinue prphylactic penicillin while patient is receiving brad-spectrum antimicrbials Nt necessary t use azithrmycin with levflxacin as levflxacin cvers atypical bacteria Antimicrbial Inpatient fr patients with severe illness r with large infiltrate with pleural effusin present and S. aureus suspected *Must mnitr renal functin (SCr, BUN, urine utput) at baseline and minimum twice weekly thereafter Page 5 f 10

6 TABLE 3. RESPIRATORY MEDICATION TABLE Medicatin Dsing Indicatin Albuterl Prednisne r Methylprednislne Ranitidine 4 puffs OR 2.5 mg nebulized x 1 dse. If effective (imprved wrking f breathing, respiratry rate, wheezing, aeratin) rder scheduled albuterl 2-4 puffs with spacer r 2.5 mg nebulized q4h 1 mg/kg (maximum 40 mg/dse) PO/IV q12h x 5 days Fllwed by sterid wean t prevent rebund: 0.5 mg/kg (maximum 20 mg/dse) PO/IV q12h x 3 days 0.5 mg/kg (maximum 20 mg/dse) PO/IV q24h x 3 days 0.25 mg/kg (maximum 10 mg/dse) PO/IV q24h x 3 days 1 mg/kg (maximum 150 mg/dse) PO q12h OR 1 mg/kg (maximum 50 mg/dse) IVq8h Increased wrk f breathing Cnsider in patients if wheezing/crackles/rales present r histry f asthma GI prphylaxis fr sterid use Fursemide mg/kg (maximum 40 mg/dse) Cnsider if signs f fluid verlad present Page 6 f 10

7 TABLE 4. PAIN MEDICATION Medicatin Dsing Indicatin Acetaminphen Dse accrding t manufacturer s recmmendatins Maximum daily dse 75 mg/kg/day r 4,000 mg/day Temperature greater than r equal t 38.3⁰C Ketrlac 0.5 mg/kg (maximum 30 mg/dse) IV q6h x 48 hurs Pain/Inflammatin Ibuprfen Mrphine Hydrmrphne 10 mg/kg (maximum 600 mg/dse) PO q6 after 48-hurs f ketrlac cmpleted Maximum daily dse 2.4 g/day Intermittent Dsing: mg/kg (maximum 8 mg/dse) IV x 1 dse fllwed by mg/kg (maximum 4 mg/dse) IV q2-4hr PCA Dsing: PCA Dse: mg/kg (maximum 10 mg/hr) with lckut f 8 minutes Cntinuus Rate: mg/kg/hr Intermittent Dsing: mg/kg (maximum 2 mg/dse) fllwed by mg/kg (maximum 1 mg/dse) q3-4hr PCA Dsing: PCA Dse: 2 3 mcg/kg (maximum dse 1.2 mg/hr) with a lckut f 1.2 mg/hr Cntinuus Rate: 3 5 mcg/kg/hr Pain/Inflammatin Pain Pain DISCHARGE CRITERIA Imprved pulmnary symptms and dcumentatin f adequate xygenatin n rm air Negative cultures fr greater than r equal t hurs if applicable Stable hemglbin/hematcrit Taking adequate ral fluids and able t take ral medicatins if applicable Adequate pain relief, if needed, with ral analgesics Fllw-up plans crdinated with sickle cell team Pulmnlgy fllw up arranged Page 7 f 10

8 REFERENCES 1. Vichinsky E et al. Causes and utcmes f the acute chest syndrme in sickle cell disease. Natinal Acute Chest Syndrme Study Grup. NEJM : Yawn B et al. Management f sickle cell disease: Summary f the 2014 evidence-based reprt by expert panel members. JAMA : Hward J et al. Guidelines n the management f acute chest syndrme in sickle cell disease. BJH : Sbta A et al. Crticsterids fr acute chest syndrme in children with sickle cell disease: Variatin in use and assciatin with length f stay and readmissin. Am J Hemat 2010; 85(1): Ahmad F et al. The use f incentive spirmetry in pediatric patients with sickle cell disease t reduce the incidence f acute chest syndrme. J Pedatr Hematl Oncl : Bellet PS, Kalinyak KA, Shukla R et al. Incentive spirmetry t prevent acute pulmnary cmplicatins in sickle cell diseases. NEJM 1995; 333(11): Saylrs R et al. Cmparisn f autmated red cell exchange transfusin and simple transfusin fr the treatment f children with sickle cell disease acute chest syndrme. Pedatr Bld Cancer : Reagan M et al. Multi-mdal interventin fr the inpatient management f sickle cell pain significantly decreases the rate f acute chest syndrme. Pediatr Bld Cancer 2011; 56: Saylrs R et al. Cmparisn f autmated red cell exchange transfusin and simple transfusin fr the treatment f children with sickle cell disease acute chest syndrme. Pediatr Bld Cancer 2013; 60: Turner J et al. Exchange versus simple transfusin fr acute chest syndrme in sickle cell anemia adults. Transfusin 2009; 49: Velasquez M et al. Erythrcytapheresis in children with sickle cell disease and acute chest syndrme. Pediatr Bld Cancer 2009; 53: Page 8 f 10

9 CLINICAL IMPROVEMENT TEAM MEMBERS Chris McKinney, MD Hematlgy Oren Kupfer, MD Pulmnlgy Rachelle Nuss, MD Hematlgy Jyce Baker, RT Respiratry Therapist Abby Kim, PharmD Clinical Pharmacist Paige Krack, MBA, MS Prcess Imprvement Lead APPROVED BY Clinical Care Guideline and Measures Review Cmmittee April 18, 2016 Antimicrbial Stewardship Cmmittee January 22, 2016 Pharmacy & Therapeutics Cmmittee February 10, 2016; minr revisin March 2, 2017 MANUAL/DEPARTMENT ORIGINATION DATE Clinical Care Guidelines/Quality Nvember 24, 2015 LAST DATE OF REVIEW OR REVISION April 18, 2016 APPROVED BY Lalit Bajaj, MD, MPH Medical Directr, Clinical Effectiveness REVIEW/REVISION SCHEDULE Scheduled fr full review n April 18, 2020 Clinical pathways are intended fr infrmatinal purpses nly. They are current at the date f publicatin and are reviewed n a regular basis t align with the best available evidence. Sme infrmatin and links may nt be available t external viewers. External viewers are encuraged t cnsult ther available surces if needed t cnfirm and supplement the cntent presented in the clinical pathways. Clinical pathways are nt intended t take the place f a physician s r ther health care prvider s advice, and is nt intended t diagnse, treat, cure r prevent any disease r ther medical cnditin. The infrmatin shuld nt be used in place f a visit, call, cnsultatin r advice f a physician r ther health care prvider. Furthermre, the infrmatin is prvided fr use slely at yur wn risk. CHCO accepts n liability fr the cntent, r fr the cnsequences f any actins taken n the basis f the infrmatin prvided. The infrmatin prvided t yu and the actins taken theref are prvided n an as is basis withut any warranty f any kind, express r implied, frm CHCO. CHCO declares n affiliatin, spnsrship, nr any partnerships with any listed rganizatin, r its respective directrs, fficers, emplyees, agents, cntractrs, affiliates, and representatives. Page 9 f 10

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