american academy of pediatrics health supervision …health supervision for children with sickle...

PEDIATRICS Vol. 98 No. 3 September 1996 467

AMERICAN ACADEMY OF PEDIATRICS

Health Supervision for Children With Sickle Cell Diseases

and Their Families

Committee on Genetics

This set of guidelines is designed to assist the

pediatrician in caring for the child in whom thediagnosis of a sickle cell disease (SCD) has beenconfirmed by hemoglobin electrophoresis. The SCDs

include 55, SC, SB#{176}thalassemia (refers to �3 thalasse-mia mutations that do not produce any f3-globin),SD, SE (the latter being noted after the influx of

Southeast Asian immigrants), and others. Becausesome of these variants (eg, SC or SE) may be associ-ated with minor symptoms, the information con-tamed in these guidelines about complications, mor-bidity, and mortality may be overstated for these

disorders. Therefore, this statement has been devel-oped primarily for the health supervision and treat-ment of patients with 55 disease. Because manage-

ment issues may be complex, consultation, referral,and/or comanagement with a pediatric hematologist

or comprehensive sickle cell program is advisable.Medical treatment, home environment, patient

and family support, and education can significantlyaffect the clinical outcome of children and adoles-cents with SCD and can facilitate their transition toadulthood. The following outline is designed to helpthe pediatrician in caring for children with SCDs andtheir families.

In addition to assuring compliance with the sched-ule, “Recommendations for Preventive PediatricHealth Care” of the American Academy of Pediatrics

(AAP), the following areas require ongoing assess-ment throughout childhood and should be reviewed

at each visit or periodically at appropriate ages (Ta-

ble 1).

I . Establishment of baseline hemoglobin, white cell

count, absolute neutrophil count, and reticulo-cyte count, which should be repeated periodi-cally;

2. Monitoring growth parameters and nutritionalstatus;

3. Observation for pallor and jaundice;4. Recording of liver and spleen size;

The recommendations in this statement do not indicate an exclusive course

of treatment for children with genetic disorders but are meant to supple-ment anticipatory guidelines available for treating the healthy child pro-

vided in the AAP publication Guidelines for Health Supervision. Diagnosis

and treatment of genetic disorders are changing rapidly. Therefore, podia-

tricians are encouraged to view these guidelines in light of evolving scien-

tific information. Clinical geneticists may be valuable resources for the

pediatrician seeking additional information or consultation.

PEDIATRICS (ISSN 0031 4005). Copyright © 1996 by the American Acad-

emy of Pediatrics.

5. Examining for the presence of cardiac murmur;6. Obtaining an interim history regarding clinical

symptoms, hospitalizations, and blood transfu-sions;

7. Monitoring compliance with penicillin prophy-laxis;

8. Reviewing personal support available to the fam-ily;

9. Periodically reviewing other financial and med-ical support programs (including comprehensivesickle cell programs) for which the child andfamily may be eligible; and

10. Discussing appropriate exercise and sports activ-

ities.

THE PRECONCEPTION AND/OR PRENATAL VISIT

Although the child’s first visit to a pediatrician isusually in infancy, the pediatrician may be called on

to advise a couple who are carriers or who have beengiven a prenatal diagnosis of a SCD. In some settings,the pediatrician may be the primary resource for

counseling. At other times, counseling may be pro-vided by a clinical geneticist and/or obstetrician. As

appropriate, the pediatrician should cover the fol-

lowing topics with the family:

I . Review autosomal recessive inheritance and of-

fer carrier diagnosis to potential parents in at-risk groups. The preferred testing should include

a complete blood count (with mean corpuscularvolume) and hemoglobin electrophoresis. Acomplete blood count with mean corpuscularvolume will potentially identify carriers of the/3-thalassemia genes that would not be deter-mined by hemoglobin electrophoresis, which

will show only quantitative changes in �3-globin

structure.2. Discuss the clinical manifestations and manage-

ment of SCD.’43. Inform parents who are carriers of the availabil-

ity of a prenatal diagnosis by chorionic villous

sampling or amniocentesis using molecular ge-netic techniques.

4. All patients with SCD should receive accurateinformation at puberty and periodically through-out their reproductive lives about methods of

contraception,5 genetic transmission of SCDs, theneed for carrier testing of their partners, preg-nancy planning, availability of prenatal testing,

and the increased difficulty and responsibility of

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be performed; S, subjective by history; 0, objective, by a standard testing method; + , at time of diagnosis; D, discuss referral to specialistor comprehensive sickle cell center; and (I), as clinically indicated.

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TABLE 1. Health Supervision for Children With Sickle Cell Disease*

468 HEALTH SUPERVISION FOR CHILDREN WITH SICKLE CELL DISEASES

Diagnosis

Discuss diagnosis

Carrier status of parents

Genetic counseling

Mother with sickle cell disease

Carrier status of father/partner

Carrier status of siblings

Recurrence risks

Reproductive options

Family support

Support groups

Long-term planning

Medical evaluation

Interim historyGrowth

Cardiac murmurLiver size

Spleen size

Baseline complete blood count

Reticulocyte count

Bilirubin level

Urine analysis

Chest radiograph

Electrocardiogram

(echocardiogram optional)

Psychosocial

Development and behavioral

School performance

Sexuality

Prenatal Infancy, I mo-I y

Neo- 2 4 6 9

natal mo mo mo mo

12 15 18 24

mo mo mo mo

. (I) #{149}(I)

. (I)

raising children while coping with a chronic dis-

ease.5. If the patient is pregnant and has SCD:

0 Management requires coordinated care by ob-stetricians and hematologists. The risks forpregnant patients are increased, and fetal loss

is more likely.6’7 All female patients shouldreceive accurate information at puberty andperiodically throughout their reproductive

lives about the risks of pregnancy and theneed for high-risk obstetrical management. Al-

though careful management is necessary forpregnant women with SCD, there is no reasonthat they cannot have children.

I Discuss the increased morbidity and mortalityof patients with SCD during pregnancy and

delivery.0 Discuss increased fetal morbidity and mortal-

ity due to placental microvasculature damagefrom sickled red cells.

0 Obtain the partner’s carrier status (completeblood count, mean corpuscular volume, and

hemoglobin electrophoresis) to provide ap-propriate genetic risk assessment for the fetus;note the availability of prenatal testing.

0 Discuss the option of pregnancy termination.

6. Offer to see the patient again during the preg-nancy, if desired.

HEALTH SUPERVISION FROM BIRTH TO 1 YEAR:INFANCY

Forty-three states, the District of Columbia, Puerto

Rico, and the Virgin Islands jurisdictions have man-

datory newborn screening programs for hemoglobi-nopathies. Because these programs vary from state tostate, the pediatrician should be familiar with thespecific screening test performed, its interpretation,the follow-up confirmatory procedures recom-mended, the listing of the state newborn screeninglaboratory, and a listing of area comprehensive sickle

cell programs, pediatric hematologists, and clinical

genetic units. In states where mandatory newbornscreening is not available, all at-risk infants (eg, inaddition to African-Americans, Hispanics from Pan-ama, South America, and the Caribbean and thosewhose ancestors are from the Mediterranean, India,or the Near East) should have routine hemoglobinscreening at the first visit or at least by 2 months ofage, because overwhelming sepsis”4 has been re-ported as early as 2 to 3 months of life.

Examination

1 . Confirm the diagnosis by protein-based (hemo-globin electrophoresis) or DNA-based methods.

Sickle cell preparations and solubility testsshould not be used as screening tests or to estab-lish a diagnosis or carrier status, because other


AMERICAN ACADEMY OF PEDIATRICS 469

hemoglobin variants will be missed. Current

methods permit diagnostic confirmation of es-sentially all patients, even in the first months oflife (using citrate agar Eacidi electrophoresis, iso-electric focusing, or high-performance liquidchromatography).

2. Review the diagnosis with the family, and coun-

sel the family regarding the need for immediatemedical attention for the following clinical man-

ifestations associated with the disorder. Thesemanifestations should be introduced during thecourse of the first three or four visits occurring

during the first 6 months of life. At the first visit,emphasize manifestations of life threatening-

complications due to the sepsis and splenic Se-questration. Because stroke and pain are unlikely

to occur in the first few months of life, the pedi-atrician can introduce these topics at later visits.Recognizing that parents can assimilate a limited

amount of information about the disease at anyone visit, the essential information should bedelivered and reinforced incrementally duringthe course of ongoing care. By checking the par-

ents’ understanding of each visit, the pediatri-cian can individualize the delivery of new infor-mation until the parents learn about all the

clinical manifestations of the disease. The topicsto cover include:. Fever;. Abdominal distention;. Sudden pallor and listlessness;

S Jaundice;S Swelling of the hands and feet;. Recurrent joint, abdominal, chest, or muscular

pain or limping;. Paresis or other symptoms compatible with a

stroke;. Respiratory distress;

. Chronic anemia; and

. Signs of infection.

Physical Examination and Laboratory Studies

I . Observe the infant’s color (for pallor and jaun-

dice).2. Record the presence or absence of a palpable

spleen; estimate and record size.3. Note the presence or absence of a cardiac mur-

mur.

4. Establish a hematologic baseline when the childis well, including a complete blood count, mean

corpuscular volume, reticulocyte count, and se-rum bilirubin level.

5. Perform liver and renal function tests, urinalysis,

and chest radiographs (note pulmonary mark-ings and cardiac size) annually as needed.

6. Perform quantitative glucose-6-phosphate dehy-

drogenase analysis (optional).7. Obtain a full red cell antigenic phenotype after

the age of 6 months or before the first anticipated

transfusion.

Anticipatory Guidance

I . Review the diagnosis with the family, and coun-

sel the parents regarding the need for immediate

medical attention for signs and symptoms of sep-

ticemia,’#{176}’3 painful crises,’4’6 sequestration cri-ses (abdominal distention from splenomegalywith sudden pallor and listlessness),’7’8 aplastic

crises,’9 hyperhemolytic crises (pallor, jaundice,and splenomegaly), hand-foot syndrome,2#{176} he-

maturia, priapism,2’ and neurologic symptoms(including stroke, acute myelopathy, and audi-

tory and ocular problems).�24 The frequency of

this review will vary with the needs of the fam-ilies but should probably occur at each visit.

2. Begin penicillin prophylaxis as soon as the diag-

nosis is established and preferably by 2 monthsof age.8’#{176}Therapy with 125 mg of penicillin V orC twice a day is recommended. If the child isallergic to penicillin, erythromycin may be sub-

stituted. It should be noted that Streptococcuspneulnoniae strains with resistance to penicillin C

have been identified from many regions of theUnited States. Penicillin C may not provide ad-

equate treatment for S pneurnoniae infections, andtesting for antibiotic sensitivities is l I

3. Instruct the family in home management of pain

and when to contact a physician (Table 2).4. Stress the need for adequate hydration and

avoiding extreme heat and cold.5. Teach spleen palpation to the parents.6. Review compliance with penicillin prophylaxis

for 55 and SB#{176}thalassemia. Penicillin prophy-laxis for SC disease is optional.

7. Begin folic acid therapy. Usual doses are 0.1mg/d from 0 to 6 months; 0.25 mg/d from 6 to 12

months; 0.5 mg/d from I to 2 years, and I mg/dbeyond the second birthday.

8. Follow routine immunization protocol, includingHemophilus influenzae type B and hepatitis B.

9. Provide appropriate literature on SCD.10. Inform parents of the resources available, such as

the Sickle Cell Disease Association of Americaand other state or regional agencies, (see “Bib-liography and Resources for Parents and Fami-lies”), and provide them with appropriate liter-ature. Encourage parents to contact a communitysickle cell agency for additional support and fol-low-up information.

I I . Give the family a card that identifies the infant toemergency medical services. Consider having

the infant wear a Medic Alert bracelet.12. Recommend that the patient enroll in a compre-

hensive sickle cell center or provide consultationor comanagement with a pediatric hematologist.

I 3. Review the genetic possibilities for future preg-nancies and other family members; offer or rec-

ommend testing for parents, siblings, and par-

ents’ siblings.

HEALTH SUPERVISION FROM 1 TO 5 YEARS:EARLY CHILDHOOD

Examination

The child should be examined every 3 to 4 months.

Physical Examination and Laboratory Studies

1. Evaluate the child for the presence of cardiacmurmur and cardiac enlargement (clinically).


TABLE 2. Recommended Doses and Frequency of Administration of Selective Analgesics to Obtain Pain Control*


Drug Dose Administration Frequency

Mild pain

Ibuprofen 5-10 mg/kg, children; 400-600 mg, adolescents P0 Every 6-8 hAcetaminophen 10-15 mg/kg, children; 300-600 mg, adolescents P0 Every 4 hCodeine 1.0 mg/kg/dose P0 Every 6 hCodeine in combination with aspirin,

acetaminophen, or ibuprofenenhances analgesia

Ketorolact 0.5-1 mg/kg; 10 mg, adolescents P0 Every 6 h

Severe or moderate painMorphine 0.15 mg/kg/dose

or0.1-25 mg/250 mL of saline

Infuse at 0.4 mL/kg (0.04 mg/kg)/h;

increase slowly until pain is relieved

IV, SC, IM,

PCA

IV

Every 3-4 h

Continuous infusion

Meperidine� 1.5 mg/kg/dose1.5 mg/kg/dose (maximum 100 mg/dose)

IM, IVP0

Every 2-4 hEvery 4 h

Hydromorphone 0.02 mg/kg/dose

0.04 mg/kg/doseIM, IV

P0Every 3-4 h

Every 4 hOxycodone 5-10 mg (1-2 tablets)/dose P0 Every 4 h

* The medication plan should be reevaluated every 24 hours. When acute pain has subsided, intravenous drug doses should be tapered,

but the interval should not be changed. Once the patient tolerates a 50% decrease, oral analgesics should be considered, and parenteraltherapy should be discontinued. Carefully record narcotic prescriptions, number of tablets dispensed, and refills. P0 indicates oral; IV,intravenous; SC, subcutaneous; IM, intramuscular; and PCA, patient control anesthesia.

t Can be given IM or IV; not approved by the Food and Drug Administration for use in children as yet.� Increases incidence of seizures. Avoid in patients with renal or neurologic disease or who receive monoamine oxidase inhibitors.

2. Record the presence or absence of a palpablespleen; note the measurement.

3. Record and review with parents acute illnesses,crises, hospitalizations, and blood transfu-sions?�5-27

4. Obtain a complete blood count and reticulocyte

count every 6 months for patients with 55 andSB#{176}thalassemia and every 12 months for thosewith SC.

5. Perform annual liver and renal function tests andurinalysis.

6. If the child has received transfusions, monitor forthe human immunodeficiency virus and hepati-

tis status (B and C) and for ferritin levels (if thechild received numerous transfusions).


I . Stress the importance of hydration and diet.

2. Review the signs and symptoms of medicalemergencies, complications, and when to call aphysician.

3. Review pain management.28 (Table 2)4. Review patient compliance with penicillin pro-

phylaxis for 55 and SB#{176}thalassemia (SC, op-tional) and folic acid treatment. At 3 years of age(approximately 35 lb), increase the penicillin dos-

age to 250 mg twice a day.”45. In addition to the currently recommended AAP

immunization schedule, also administer pneu-mococcal, meningococcal, and influenza vac-

cines. Give pneumococcal vaccine at 2 years ofage. Although the academy recommends abooster at 3 to 5 years,” some hematologistsrecommend the booster at I to 2 years after theinitial immunization. For meningococcal pro-phylaxis, administer a single quadrivalent me-ningococcal vaccine when the child is older than2 years. (It is unclear at this time whether chil-

dren need booster doses of meningococcal vac-cine.) Give influenza vaccine annually.

6. Review genetics of SCD and family planning.7. Discuss the child’s medical condition in relation

to preschool and school planning.

8. Add a pediatric dental examination to the annualtesting battery.

HEALTH SUPERVISION FROM 5 TO 13 YEARS:LATE CHILDHOOD

Examination

The child should be examined every 4 to 6 months.


I . The report of the Prophylactic Penicillin Study II(1995) provides revised recommendations for

continuing the preventive regiment beyond 5years of age.29 The report concluded that childrenwith sickle cell anemia (55 or SB#{176}thalassemia)

who have not had prior severe pneumococcalinfections or splenectomies and are receiving

comprehensive care may safely stop prophylac-tic penicillin at 5 years of age. However, parentsmust be aggressively counseled to seek medical

attention for all febrile events.2. Newer potential modalities of therapy, including

hydroxyurea and 5-azacytidine, which increase

fetal hemoglobin production, have been shownin experimental situations30” to reduce the mci-

dence of crises in some severely affected adult

patients. Early clinical trials are being performedin children. For such children with frequent cri-

ses, discussion and referral to a comprehensivesickle cell center regarding newer beneficial ther-apies is advisable.

3. Begin to discuss the nature of the disease withthe child and answer questions at an age-appro-

priate level.


AMERICAN ACADEMY OF PEDIATRICS 471

4. Review the child’s school attendance and perfor-mance in relation to SCD.

5. Discuss the child’s participation in appropriatesports activities. Indicate the need for adequate

hydration with activity and increased environ-mental temperaturet3

6. Add an electrocardiogram (thereafter optional

on a yearly basis) and cardiologic examinations.7. Stress the importance of dental hygiene; dental

examinations need to be performed twice a year.

8. Add an ocular examination by an ophthalmolo-gist for retinopathy during this age group.

9. Discuss the prevention and local care of leg ul-

cers.10. Examine the child for possible avascular necrosis

of the femoral head with radiography if clinically

indicated.I I . Review for symptoms of gallstones (obtain a his-

tory and perform imaging studies as indicated).

12. Explain the principles of pain management to thepatient.

HEALTH SUPERVISION FROM 13 TO 21 YEARSAND OLDER: ADOLESCENCE TO EARLY

ADULTHOOD

Examination

The patient should be examined every 4 to 6months


I . Discuss the nature of the disease with the pa-

tient, and review concerns and issues related tothe impact of the disease throughout adoles-

cence.2. Review the adolescent’s school attendance and

performance in relation to SCD.3. Discuss the adolescent’s participation in appro-

priate sports activities. Indicate the need for ad-

equate hydration when involved with activity orwhen exposed to increased environmental heat.

4. Discuss the prevention and local care of leg ul-cers.

5. Explain the principles of pain management to thepatient.28

6. Review for symptoms of gallstone disease (ob-tam a history and perform imaging studies asindicated).

7. Discuss sexuality and birth control with parentsand patients, as age appropriate, including therisk of using an intrauterine device. Discuss theincreased risk of thromboses with combined oral

birth control pills5; progesterone-only forms oforal or injectable contraception may be prefera-ble.

8. Review pregnancy risks with female patients.9. Discuss plans for independent living.

10. Review genetics with the patient and the possible

chances of having affected children if the partneris also a carrier. Stress the need for carrier testing

of the patient’s partner.

:i1 . Provide counseling regarding education, post-secondary education, and vocational planning.

12. Facilitate the transfer to adult medical care, asappropriate or desired.

COMMITrEE ON GENETICS, 1995 TO 1996Franklin Desposito, MD, ChairpersonSechin Cho, MDJaime L. Frias, MDJack Sherman, MDRebecca S. Wappner, MDMiriam G. Wilson MD

LIAIsoN REPRESENTATIVES

Felix de la Cruz, MDNational Institutes of Health

James W. Hanson, MDAmerican College of Medical Genetics

Jane Lin-Fu, MDHealth Resources and Services Administration

Paul G. McDonough, MDAmerican College of Obstetricians andGynecologists

Godfrey Oakley, MDCenters for Disease Control and Prevention

AAP SECTION LIAISONBeth A. Pletcher, MD

Section on Genetics and Birth Defects

BIBLIOGRAPHY AND RESOURCES FOR PARENTSAND FAMILIES

The Infant and Young Child with Sickle Cell Anemia (a guide forparents in English and Spanish). Texas Department of Health,Newborn Screening Program, 1100 West 49th St. Austin, TX78756-3199; phone: (512) 458-7000

Sickle Cell Anemia, (Medicine for the Public). National Institutes ofHealth publication 90-3058. Clinical Center Communications,

9000 Rockville Pike, Building 10, Room 1C255, Bethesda, MD

20892; phone: (301) 496-2563

Sickle Cell: A Selected Resource Bilbliography (catalog No. D002) andSo I Have the Sickle Cell Trait (catalog No. B050). National Ma-ternal and Child Health Clearinghouse, 8201 Greensboro Dr,McLean, VA 22102; phone: (703) 821-8955

Sickle Cell Disease in Newborns and Infants: A Guide for Parents.Agency for Health Care Policy and Research, Publications pub-lication 93-0564. Agency for Health Care Policy and Research,Publications Clearinghouse, P0 Box 8547, Silver Spring, MD20907; phone: (800) 358-9295

Hemoglobin S (Spanish), Hemoglobin C (Spanish and English), andAll You Ever Wanted to Know About Sickle Cell Trait. NorthernCalifornia Comprehensive Sickle Cell Center; Phone: (510)

428-3651

The Family Connection-Sickle Cell Trait (English, French, and Span-ish), The Family Connection-Hemoglobin C Trait (English, French,and Spanish), Newborn Screening for Your Baby’s Health (Englishand Spanish), Directory ofAvailable Sickle Cell Services in New YorkState, and Sickle Cell Anemia. New York State Department ofHealth, Newborn Screening Program, Wadsworth Center for

Laboratories and Research, P0 Box 509, Albany, NY12201-0509; phone: (518) 473-7552

Sickle Cell Anemia-What Is It? Cincinnati Comprehensive SickleCell Center, Childrens Hospital Medical Center, Cincinnati, OH45229; phone: (513) 559-4200

Your Child and Sickle Cell Disease. Mid-South Sickle Cell Center, LeBonheur Childrens Medical Center, Memphis, TN 38103; phone:(901) 522-6792

Brochure for Parents of Children with Sickle Cell Disease. HowardUniversity, Comprehensive Sickle Cell Center, 2121 GeorgiaAye, Washington, DC 20059; phone: (202) 806-7930

Help (resource book listing sources of care for patients with sicklecell disease in the United States, Puerto Rico, and Virgin Is-lands). National Association for Sickle Cell Disease, 3345



Wilshire Blvd, Suite 1 106, Los Angeles, CA 90010-1880; phone:

(800) 421-8453

Thalassemia Information Sheet and Sickle Cell Anemia PublicHealth Information Sheet. March of Dimes, Birth Defects Foun-dation, 1275 Mamaroneck Aye, White Plains, NY 10605

Note: The above listings are not all-inclusive. Ad-ditional material may be available from your own

state or local health department, sickle cell agency, or

community agency.

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1996;98;467Pediatrics Committee on Genetics

Health Supervision for Children With Sickle Cell Diseases and Their Families

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1996;98;467Pediatrics Committee on Genetics

Health Supervision for Children With Sickle Cell Diseases and Their Families

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American Academy of Pediatrics. All rights reserved. Print ISSN: 1073-0397. American Academy of Pediatrics, 345 Park Avenue, Itasca, Illinois, 60143. Copyright © 1996 by thebeen published continuously since 1948. Pediatrics is owned, published, and trademarked by the Pediatrics is the official journal of the American Academy of Pediatrics. A monthly publication, it has


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