Produced by The Center for Children with Special Health Needs Children’s Hospital and Regional Medical Center, Seattle, WA
The Critical Elements of Care (CEC) consider care issues across the life span of the child. The
intent of the document is to educate and support those caring for a child with Sickle Cell Disease. The CEC isintended to assist the Primary Care Provider in the recognition of symptoms, diagnosis and care managementrelated to a specific diagnosis. The document provides a framework for a consistent approach to managementof these children.
These guidelines were developed through a consensus process. The design team was multidisciplinary
with state-wide representation involving primary and tertiary care providers, family members and a represen-tative from a Health Plan.
M. Bender, MD, Ph.D. Ruth White, MN, ARNPTrinna E. Bloomquist, BSN
This document is also available on the Center for Children with Special Needs website at:
DISCLAIMER: Individual variations in the condition of the patient, status of patient and family, and the response to treatment, as well as other circumstances, mean that the optimal treatment outcome forsome patients may be obtained from practices other than those recommended in this document. Thisconsensus-based document is not intended to replace sound clinical judgement or individualized consulta-tion with the responsible provider regarding patient care needs.
1997, 2003, 2006 Children’s Hospital and Regional Medical Center, Seattle, Washington. All rights reserved. TABLE OF CONTENTS SICKLE CELL DISEASE I. OVERVIEW
Definition of Sickle Cell Disease ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 1
Diagnostic Testing for the Common Sickle Cell Syndromes ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 2
II. BASIC TENETS OF HEMOGLOBINOPATHY FOLLOW-UP
Hemoglobinopathy Follow-Up Program ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 3
III. GUIDELINES FOR CARE OF CHILDREN WITH SICKLE CELL
Definition of Levels of Care ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 4
Clinic Requirements ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 5
Age-Specific Activities ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 7
Sickle Cell Disease Flow Sheet ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 14
IV. GUIDELINES FOR PAIN MANAGEMENT
Pain Related to Sickle Cell Disease ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 17
General Principles of Pain Management ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 17
Complication-Specific Guidelines: Vaso-Occlusive Pain ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 20
Pain Episode Algorithm ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 21
ER Management: Pain Assessment ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 22
Guide to Determination of Schedule for Maintenance Analgesia in the Hospital ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 23
Assessment Tool 1: The Oucher ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 24
Assessment Tool 2: Pain Intensity Number Scale ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 24
Assessment Tool 3: Work Graphic Rating Scale ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 24
Table 1: Research Dosage Guidelines, Dosing Data for NSAIDS ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 25
Table 2: Research Dosage Guidelines, Dosing Data for Opioid Analgesics ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 26
Critical Elements of Care: Sickle Cell Disease TABLE OF CONTENTS (cont.) SICKLE CELL DISEASE V. APPENDICES
I. Psychosocial Aspects of Sickle Cell Disease ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 27
II. Sickle Cell Disease Algorithms and Complication-Specific Guidelines
Anemia Algorithm ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 29
Sepsis Algorithm ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 30
Acute Chest Syndrome ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 31
Stroke or Acute Neurologic Event ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 32
Priapism ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 33
General Anesthesia and Surgery ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 34
Patient Education Materials Sources ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 35
Counseling References for Parents of Newborns ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 35
General References ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ 36
Critical Elements of Care: Sickle Cell Disease I. OVERVIEW OF SICKLE CELL DISEASE
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Definition of Sickle Cell Disease
Sickle cell disease comprises a group of genetic
disorders characterized by the inheritance of sicklehemoglobin (Hb S) from both parents, or Hb S fromone parent and a gene for an abnormal hemoglobin orbeta-thalassemia from the other parent. The presenceof Hb S can cause red blood cells to change from theirusual biconcave disc shape to a crescent or sickleshape during deoxygenation. Upon reoxygenation, thered cell initially resumes a normal configuration, butafter repeated cycles of “sickling and unsickling,” theerythrocyte is damaged permanently and hemolyzes. This hemolysis is responsible for the anemia that is thehallmark of sickle cell disease.
Acute and chronic tissue injury can occur when
blood flow through the vessels is obstructed by theabnormally shaped red cells. Complications includepainful episodes involving soft tissues and bones, acutechest syndrome, priapism, cerebral vascular accidents,and both splenic and renal dysfunction. Commoncauses of mortality among children with sickle celldisease include bacterial infections, splenic sequestra-tion crisis and acute chest syndrome.
Sickle cell disease affects more than 70,000
Americans, primarily those of African heritage, butalso those of Mediterranean, Caribbean, South andCentral American, Arabian or East Indian ancestry. Itis estimated that eight percent of the African Americanpopulation carries the sickle cell trait, and approxi-mately one African American child in every 375 isaffected by sickle cell disease. Thus, it is among themost prevalent of genetic diseases in the United States(AHCPR Publication #95-2117, DHHS, 1995). Critical Elements of Care: Sickle Cell Disease Solubility Test
indicates thalassemia mutation with reduced (but not absent) production of
the Common Sickle Cell Syndr
, fetal hemoglobin; S, sickle hemoglobin; C, hemoglobin C;
esting for Neonatal Screening Diagnostic Genotype Sickle Cell Disease
cannot be measured in presence of Hb C.
AS, require confirmation with Hb electrophoresis. Syndrome
indicates thalassemia mutation with absent production of o
Hemoglobins reported in order of quantity (e.g. FSA
Hb F levels in rare cases of Hb SS may be high enough to cause confusion with Hb S-Pancellular Hereditary Persistence of Feta
Note that this test does not distinguish sickle cell trait from sickle cell disease syndromes. High fetal hemoglobin level (
I. Overview of Sickle Cell Disease
benign disorder not usually associated with significant anemia or vaso-occlusion. In such cases, family studies and laboratory
Hb F among red cells may be helpful.
tives. False negative results occur during infancy in all sickle syndromes. Critical Elements of Care: II. BASIC TENETS OF HEMOGLOBINOPATHY FOLLOW-UP The Basic Tenets of the Hemoglobinopathy
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• Genetic counseling services should be available
Follow-Up Program are as Follows:
to all families of children with hemoglobinopathies.
• Data on all newborn hemoglobinopathy screens
Every child with sickle cell disease should have
should be centrally maintained so that clinicians can
identify a child’s hemoglobin status without rescreening.
• Wherever possible, well-child care should
• Communication should be maintained between
follow the normal guidelines of the American Academy
of Pediatrics. The primary care provider should be-come familiar with the Management and Therapy of
• Normal patterns of medical confidentiality and
Sickle Cell Diseases publication from the U.S. Depart-
information exchange should be maintained.
ment of Health and Human Services (see Referenceson page 34).
• Every child should have regular consultation
with a physician who has expertise in the sicklingdisorders. Some primary physicians with special interestand skill in the sickling diseases may act both asprimary physicians and consultants.
• Children with major sickle complications
(stroke, acute chest syndrome, renal or cardiac disease)should be evaluated by a tertiary care consultantfamiliar with treating these disorders.
• Positive sickle hemoglobinopathy screening
results should lead to immediate definitive testing by theprimary care physician through qualified diagnosticlaboratories.
• When clinically significant hemoglobinopathies
are confirmed, the primary care provider shouldconsider referral to consultative care. Consultative careshould be established in the first months of life.
• Positive sickle hemoglobinopathy screening
should lead to early prophylaxis of infection andanticipatory family education about the risks to a childwith a sickling disease.
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• The family should have access to 24-hour-a-
day medical services through the primary physician orher/his on-call arrangements. Tertiary level consultationshould be available 24 hours a day to physicians.
• To ensure access to care, other state agencies
should assist the family in identifying financial andother resources. Critical Elements of Care: Sickle Cell Disease III. GUIDELINES FOR CARE OF CHILDREN WITH SICKLE CELL DISEASE Definition of Levels of Care
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Visits listed with the primary care provider
correspond to the current American Academy of
This care plan assumes three levels of care for
Pediatrics well-child guidelines, with the addition of
visits at 15 months, four years, and annually (instead of
1. The primary care physician; 2. A multidisciplinary program skilled in the
Refer to pages 7-12 for the three levels of the
comprehensive care plan for children with sickle cell
3. Tertiary care for management of unusual or
Where skills and resources are appropriate, one
medical site may provide several levels of caresimultaneously. Whenever possible, the regular well-child care and immunizations should be managed bythe primary physician, and disease-specific activitiesmanaged at the multidisciplinary program. The recom-mended timing and substance of visits will be de-scribed, but will vary with the needs of the patient,family and skills of the primary care provider. Ingeneral, infants should have monthly health care visitsthrough the first six months, which can be alternatedbetween primary and comprehensive sites, followed byvisits every three-six months through six years of age. These are guidelines, not standards. Their intent andthe desired quality of care may be met by programsother than those described below.
The comprehensive program visits described
below define counseling and teaching needs for age-specific sickle disease risks. This counseling mayoccur during the course of the normal primary providervisits listed if the primary caretaker is skilled in theproblems of sickle diseases. Alternatively, the counsel-ing and teaching goals may be met by outreach orin-home service providers such as public health nursesskilled in sickling diseases or tertiary program nurseclinicians. However, it is desirable for the child to visitthe comprehensive program by four to six weeks ofage−and at least annually−to establish the rapport andtrust needed in case of major complications, and tokeep abreast of new trends in the treatment of sickledisease. Critical Elements of Care: Sickle Cell Disease III. Guidelines for Care of Children with Sickle Cell Disease Clinic Requirements
Most of the care for sickle cell patients occurs in an outpatient setting. Comprehensive outpatient management has
been shown to reduce morbidity, lessen the frequency of complications, lessen psychologic burdens, and reduce the rateof hospitalization. Primary Care Requirements
Primary caretakers should be familiar with and capable of providing the level of care outlined in The ManagementSecondary Care Requirements
• Ability to obtain and interpret results of screening and definitive tests for hemoglobinopathies. • Ability to provide genetic counseling to affected families. • Provide information about newborn screening program.
• Provide general information about sickle cell diseases. • Ability to follow guidelines for routine ambulatory care, as outlined in Management and Therapy of Sickle
• Access to educational materials to reinforce counseling. • Participation of physicians versed in care of sickle cell patients. • Participation of nursing staff with expertise in sickle cell issues. Nursing staff must have the skill and time
available to provide educational support, perform phone triage, coordinate delivery of services with socialservices, and provide regular family outreach to ensure that families consistently receive care.
• Availability of vaccines specific to the infection risks of sickling diseases. • Availability of social services to coordinate delivery of health care services and provide basic counseling. • Access to nutrition services. • Access to dental care with referral ability to those experienced in issues of infection and anesthesia specific to
• Knowledge of community and family support resources for families of children with sickling diseases.
• Health care staff with experience and resources capable of identifying early signs of and providing initial
treatment for acute and chronic organ damage to include stroke, acute chest syndrome, splenic sequestrationcrises, sepsis, hand-foot syndrome, painful episodes, priapism, leg ulcers, avascular necrosis, sickleglomerulopathy, retinopathy, and sickle lung disease.
• Proximity of secondary level inpatient services, including surgical and medical services capable of providing
initial care and stabilization for the above complications.
• Understanding the unique risks of surgery associated with sickling diseases. • Availability of specialized pain management services, as well as availability of referral services for drug
• Transition strategy for patients transferring from pediatric care to adult care services. • Access to academic and vocational counseling services.
• Birth control counseling and management. • Reproductive counseling and expertise in managing sickle cell patients through pregnancy and delivery. • Understanding of the natural history of sickle cell anemia and development of approaches to monitor patients
Critical Elements of Care: Sickle Cell Disease III. Guidelines for Care of Children with Sickle Cell Disease
• Patient access to expert physician staff available 24 hours a day. Staff must be knowledgeable in sickle
hemoglobinopathies and capable of inpatient management. Comprehensive Sickle Cell Clinic: Tertiary Care
• Physician level genetic counseling services. • Availability of pain management team for design of individualized pain treatment protocols and for application
of coping techniques for chronic pain.
• Neuropsychologist with expertise in recognition of neurocognitive deficits common to sickle cell disease. • Availability of neuro-imaging technology (MRI/angiography, SPECT, etc.) for delineation of neurologic
abnormalities encountered in sickle cell disease.
• Availability of trans-cranial doppler and specialists trained in assessing patients with sickle cell anemia to
• Same as secondary care, but social work and nutrition should have time dedicated to the clinic.
• Same as secondary care, but should be able to provide or directly access definitive care for acute and chronic
• Participation in a tertiary care inpatient center capable of providing definitive medical and surgical care for
• Ability to design and maintain patients on chronic transfusion programs and iron chelation therapy, as well as
understand and monitor for the complications of iron overload and chelation therapy.
• Familiarity with recent advances and ongoing experimental therapy in sickling diseases. • Involvement in clinical trials designed to improve the quality of life and care provided to sickle cell disease
Critical Elements of Care: Sickle Cell Disease III. Guidelines for Care of Children with Sickle Cell Disease Two-to-Four-Week Check by PRIMARY CARE PROVIDER
• Conduct usual two-week, well-child care. • Review results of second state newborn metabolic screen, which includes hemoglobinopathy screening results. • Check if Hepatitis B vaccine given at birth. If not, begin series. When Presumptive Positive Hemoglobinopathy Screen becomes available to PRIMARY PHYSICIAN:
• Discuss usual expectations of well-child care and practice arrangements, including after hours coverage. It is
important to encourage parents to maintain as normal a lifestyle as possible for children with sickle cell disease.
• No immediate confirmatory testing is necessary If the state lab has received two independent specimens as
per standard policy for all newborns.
• Testing, including quantitation of hemoglobin types and for thalassemia, should be performed after consultation
or referral to a pediatric hematologist (a current listing is provided with the newborn screening programnotification letter).
• Begin Penicillin prophylaxis with Penicillin VK 125 mg BID orally to prevent pneumococcal sepsis. • Provide prescription for folic acid supplements, 0.1 mg QD. Folate is consumed at increased rates in
hemolytic anemias. It may be difficult finding liquid formulations; if preferred please contact a pediatrichematologist.
• Emphasize the importance of observing for fever. The family should be taught to take a rectal temperature and
appropriate use of antipyretics. They should be taught to call the primary physician immediately if feverdevelops.
• Emphasize the importance of fluid hydration. • Make referral to your regional genetic counselor for assistance. A list of counselors with expertise in
hemoglobinpathies is provided with the notification letter from the newborn screening program.
• Refer to WIC program for nutrition assistance (if eligible). • Contact the County Health Department Children With Special Health Care Needs Program to have a public
Six-Week Check by COMPREHENSIVE HEMOGLOBINOPATHY CARE PROGRAM (“COMPREHENSIVE PROGRAM”)
• Discuss the identified hemoglobinopathy with the family. Answer further questions. Briefly discuss genetic
basis, and if not already done, refer for genetic counseling. Fever. The parents should check the child for fever if he or she is acting ill (demonstrate taking a rectal tem- perature). The family should be instructed to call the child’s physician or a tertiary care center if fever develops. Overwhelming sepsis should be discussed as well as its normal evaluation and management. The emergent risk of sepsis should be discussed and the need for immediate medical evaluation emphasized. Antibiotic Prophylaxis should be started by four to six weeks of age in patients with SS and Sβ0 Thalas- semia−Penicillin 125 mg BID until age three years, and 250 mg BID from age three to age six years (Gaston et al., 1986). Some comprehensive hemoglobinopathy programs recommend continued prophylactic treatment throughout life, however, a randomized prospective trial for older patients without surgical splenectomy or prior pneumococcal sepsis has demonstrated no benefit (Falletta et al., 1995). Sepsis risk in sickle genotypes other than HbSS (e.g. SC, Sβ+ Thalassemia) is lower and penicillin for these patients may not be indicated. Erythromycin (20mg/kg divided into two daily doses) may be used in cases of penicillin allergy. Splenic Sequestration Crisis. Instruct the family in recognition of splenic sequestration crisis and examina- tion of the spleen. To learn about the exam and their child’s normal splenic size, they should practice this daily when the child is quiet. In cases of irritability, pallor, increasing abdominal girth and tenderness or respiratory distress, they should know to examine the spleen and, if enlarged, seek care at once. Critical Elements of Care: Sickle Cell Disease III. Guidelines for Care of Children with Sickle Cell Disease Other Medical Providers. Discuss the importance of identifying the child’s sickle disease diagnosis with other medical providers.
• Initiate social work evaluation. Include discussion of family structure, strengths, coping mechanisms and
financial resources. Discuss normal reactions to chronic illness in one’s child. Provide information about theparent support group. Where appropriate, refer for financial support for medical care. Where available, refer toa care coordination program.
• Administer second hepatitis B vaccine. • If appropriate and not yet done, refer to WIC or alternate nutrition counseling. • Coordinate nurse review care plan with family. • If appropriate, confirm public health nurse referral. • Begin teaching awareness about coping with common problems associated with children with chronic illnesses. Two-Month Check by the PRIMARY CARE PROVIDER
• Perform routine well-child care and physical exam, and demonstrate spleen exam. Reinforce home palpation
• Reaffirm antibiotic prophylaxis and review emergency care arrangements. • Reinforce teaching about the significance and management of fever. Discuss use of liberal fluids and of
• Review folate therapy. • Give DTAP, IPV, approved H. influenza conjugate vaccine (HIB), Hep B #1 or 2, and pneumococcal conjugate
Three-Month Check by COMPREHENSIVE PROGRAM/ Teaching Goals for Age
• Perform physical exam. • Reinforce earlier teaching. • Highlight:
Pain Episodes, Sickle Dactylitis. Discuss how “colic” or fussiness may be symptoms of pain. Discuss admin- istration of liberal oral fluids and appropriate outpatient pain medications. If pain is not relieved by fluids, rest, and oral analgesics, the child should be medically evaluated. Make available resources for coping with pain. Causes of Sickling. Discuss inciting causes of sickling. Include the kidney’s limited ability to conserve water and consequent need for liberal fluid intake. Discuss fluids appropriate for maintaining hydration in illness or hot weather. Discuss the effects of cold and tiring.
• Initiate dietary/nutrition counseling. Discuss the fact that good nutrition is important for the child’s health but
will not correct sickle diseases. Growth should be followed at each visit. Enroll in WIC if appropriate.
• Social work update. • Coordinating nurse review care plan with family. • Review strategies to maximize health care access and introduce the patient and family to the Emergency Room,
and reinforce strategies for positive interactions. Critical Elements of Care: Sickle Cell Disease III. Guidelines for Care of Children with Sickle Cell Disease Four-Month Check by PRIMARY CARE PROVIDER
• Perform routine well-child care. • Give DTAP, IPV, approved H. influenza conjugate vaccine (HIB), Hep B #2 or 3, and pneumococcal conjugate
• Reinforce teaching about fever, splenic size, fluids, antibiotics, folic acid and pain therapy. • Introduce coping strategies for blood draws and other invasive procedures. Five-Month Check by COMPREHENSIVE PROGRAM/Teaching Goals for Age
• Perform physical exam. • Reinforce earlier teaching. • Highlight:
Acute Chest Syndrome. Discuss how respiratory distress or chest pain may signal problems and call for immediate medical evaluation. Normally, chest x-ray, CBC, retic and blood gases or oximetry would be done. Oxygen should be administered, and simple or exchange transfusion provided in acute chest syndrome. Until infection is ruled out, empiric antibiotic therapy is usually warranted. Consider including antibiotic coverage for chlamydia and mycoplasma infection. Neurologic Complications. Discuss neurologic complications of sickle cell disease. The family should be taught to look for and seek help if seizures, severe headache, weakness, paralysis/paresis, vertigo, visual changes or loss of speech occur. Medical evaluation for CVA should be performed; if fever is present, the possibility of meningitis should be considered. An exchange transfusion is indicated for stroke. The tertiary care program should be contacted for advice. Nurse review care plan with family.
• Collect CBC, diff, platelets and retic count. The child’s normal levels should be established by serial testing. Six-Month Check by PRIMARY CARE PROVIDER
• Perform routine well-child care. • Reinforce previous teaching. • Give DTAP, third Hep B vaccine if not done, and HIB and pneumococcal conjugate vaccine (PCV). • Adjust folic acid dose to 0.25 mg QD. Eight-to-Nine-Month Check by COMPREHENSIVE/PRIMARY CARE PROGRAM/ Teaching Goals for Age
• Review and discuss prior teaching. • Physical exam. • CBC, diff, plt and retic. • Social service re-evaluation. • Nurse review care plan with family. • Influenza booster (initial two-dose vaccine during early first winter).
Note that the eight- to nine-month visit (and subsequent tri-monthly visits through six years of age) may eitherbe performed as a single primary care visit, or separately as a primary care and comprehensive care visit,according to the expertise and comfort of the primary care provider. Critical Elements of Care: Sickle Cell Disease III. Guidelines for Care of Children with Sickle Cell Disease 11-to-12 Month Check by COMPREHENSIVE/PRIMARY CARE PROGRAM/Teaching Goals for Age
• History and PE. • Labs: CBC, diff, retic, plt, BUN, Cr, Bili, Alk P, LDH, ALT, Iron Studies (other than FEP, ZPP), UA. • Quantitate hemoglobins; e.g., HbS, A, A , F, C, evaluate for thalassemia in an approved diagnostic laboratory.
• Tuberculin test. • Adjust folic acid dose to 0.5 mg QD. • Perform blood typing, including all minorblood groups. • Introduce priapism. • Confirm that genetic counseling occurred, and review. • Nutrition counseling. • Nurse review care plan with family. • IPV, MMR• Annually in the fall, give booster influenza vaccine. 14-to-15 Month Check by COMPREHENSIVE/PRIMARY CARE PROGRAM/Teaching Goals for Age
• Routine well-child care. • Review past teaching and examination. • Social service case review. • HIB, PCV #4, DTAP• Nurse review care plan with family. 17-to-18 Month Check by COMPREHENSIVE/PRIMARY CARE PROGRAM/Teaching Goals for Age
• Routine well-child care. • Varicella (optional after age 12 months)• Review past teaching and examination. • Nurse review care plan with family. • Distribue pain questionnaire. 21-Month Check by COMPREHENSIVE/PRIMARY CARE PROGRAM/ Teaching Goals for Age
• Review past teaching and examination. • Social service case review. • Discuss hyposthenuria and enuresis. • Nurse review care plan with family• Discuss Transcranial Doppler Study to identify children at increased risk for stroke (SS and Sβo patients)
24-Month Check by PRIMARY CARE PROVIDER
• Routine well-child care, review previous teaching. • Pneumovax™, meningococcal, Hepatitis A (optional)• Adjust penicillin dose to 250 mg BID. • Adjust folic acid to 1mg QD. • CBC, diff, plt, retic, BUN, Cr, Bili, Alk Phos, ALT, Iron Studies. • Consider discussion of oral hygiene. Critical Elements of Care: Sickle Cell Disease III. Guidelines for Care of Children with Sickle Cell Disease 2-1/2 Year Check by COMPREHENSIVE PROGRAM/Teaching Goals for age (Annually on the half-year)
• Review need and importance of yearly studies. • Review past teaching, PCN prophylaxis and exam. • CBC, diff, plt, retic, BUN, Cr, Alk P, AST, Bili. LDH, Iron Studies• Transcranial Doppler Study at 2 years of age and then yearly for patients with SS or Sβo-thalassemia, and
some patients with Sβ+ thalassemia (should be done at a tertiary care facility by personnel trained to studypatients with hemoglobinopathies).
• Review status of new potential treatments and interventions. • Annually in the fall, give booster influenza vaccine. • Social service PRN. • Nurse review care plan with family. • Review status of new potential treatments and interventions. • Hep A #2
3-and-4-Year Check by PRIMARY CARE PROVIDER
• Routine well-child care. • BP, UA with all subsequent annual visits. • Refer for routine dental care. • Age four: Begin routine hearing and vision screening. • Assess pain status, counsel family on pain management prevention and treatment. • Begin coping strategy teaching with child. • Pneumovax™ booster 1-2 years after initial vaccination. • Assess and teach self-care skills. • Developmental assessment. 5-Year Check by PRIMARY CARE PROVIDER
• Routine well-child care. • DTAP, IPV, Pneumovax
5-1/2 and 6-1/2 Year Check by COMPREHENSIVE PROGRAM/ Teaching Goals for Age
• Review past teaching and examination. • CBC, diff, plt, retic, BUN, Cr, Alk P, ALT, Bili, LDH, UA. • Social service PRN. • Nurse review care plan with family. • Promote self-care, reinforce coping strategies. • Initiate school outreach and provide schools with resources about sickle cell disease. • Continue Transcranial Doppler Study yearly for patients with SS or Sβo-thalassemia and some patients with
Sβ+ thalassemia (should be done at a tertiary care facility by personnel trained to study patients withhemoglobinopathies).
• Review status of new potential treatments and interventions. • Assess and teach self-care skills. • Developmental and neuropsychologic assessment. Critical Elements of Care: Sickle Cell III. Guidelines for Care of Children with Sickle Cell Disease Annual Check by PRIMARY CARE PROVIDER
• Routine well-child care. • Pneumovax™ every three to five years. • dT booster age 15 years. • MMR booster after age five years. • Discontinue penicillin prophylaxis at age six years(children with a history of sepsis should continue on penicillin
Annually from age 7-1/2 to 13 years on the Half-Year Check by COMPREHENSIVE PROGRAM/Teaching Goals for Age
• Review past teaching and examination. • Discuss leg ulcers, priapism, delays in sexual maturation, sexual activity, smoking/drugs, activities and career
goals as developmentally appropriate.
• Abdominal ultrasound for gall bladder stones, as needed for symptoms, and every other year routinely. • Monitor/counsel on pain management. • Monitor school progress and educational intervention as needed. • Social service and nutritional evaluation as needed. • Nurse review care plan with family. • Review status of new potential treatments and interventions. • Assess and teach self-care skills. • Review yearly studies. • Neuropsychologic evaluation q 2-3 years. • Screen for depression. • Pulmonary function tests, CXR, O2 saturation, TCD, ophthalmology and dental evaulations yearly. • EKG every other year. • ECHOcardiogram including documentation of tricuspid regurgitation jet velocity yearly for all patients with a
history of multiple pneumonias, acute chest syndrome or restrictive lung disease.
• Repeat pneumococcal and meningococcal immunization q 3-5 years.
Chronic transfusion programs will usually be managed by tertiary care programs. Transfusion-dependentchildren are at risk of iron toxicity to the liver, heart, pancreas and pituitary gland. Ferritin, Fe, TIBC, as well aspercent HbS are followed closely. At least annually, hepatic and renal function should be tested. Annual24-hour Holter monitoring may be appropriate. Clinical and serologic pituitary function testing, includinggonadotropins, can be used to monitor pituitary function. Liver biopsy to assess for portal fibrosis, chronichepatitis, and iron content on a regular basis may be indicated. HIV and hepatitis serologies should be doneyearly. Critical Elements of Care: Sickle Cell III. Guidelines for Care of Children with Sickle Cell Disease Annually from 14 to 18 years: ADOLESCENCE ISSUES
• Review past teaching and examination.
• Discuss leg ulcers, priapism, potential delays in sexual maturation, sexual activity, smoking/drugs, activities
and career goals as developmentally appropriate.
• Abdominal ultrasound for gall bladder stones, as needed for symptoms, and every other year routinely.
• Genetic counseling directed toward patient early adolescence.
• Monitor/counsel on pain management.
• Monitor school progress and educational intervention as needed.
• Social service and nutritional evaluation as needed.
• Nurse review care plan with family.
• Begin to develop a plan for transition to adult care.
• Neuropsychologic evaluation q 2-3 years.
• Pulmonary function tests, CXR, O2 saturation, TCD, opthomology and dental evaluations yearly.
• ECHOcardiogram including documentation of tricuspid regurgitation jet velocity every two years and yearly
for all patients with a history of multiple pneumonias, acute chest syndrome or restrictive lung disease.
• Repeat pneumococcall and meningococcal immunization q 3-5 years. Critical Elements of Care: Sickle Cell Disease III. Guidelines for Care of Children with Sickle Cell Disease: Sickle Cell Disease Flow Sheet
Name:_________________________Diagnosis:_______________________Birth Date:__________________
Phone:____________________Hospital Phone:_______________________
Primary Care Provider:_____________________Comprehensive Care Clinic/Provider:_____________________
Medications Immunizations
review splenic sequestration SW eval./RN review of care plan verify PHN, WIC and genetic
SW update/RN review of care plan review health care access, interaction
PCN, splenic sequest., etc. introduce acute chest syndrome
cord blood cells from newborn fullsiblings of patients
review previous teaching PE SW update/RN review of care plan
* If unable to follow this schedule, or if the mother of the infant is HepB SAg+, please consult current approved guidelines. ** Influenza vaccination must be addressed early winter of each year. For children under 6mo, household members should be vaccinated. Children
over 6mo of age should receive the two-dose influenza vaccine the first time, then one yearly after. Critical Elements of Care: Sickle Cell Disease III. Guidelines for Care of Children with Sickle Cell Disease: Sickle Cell Disease Flow Sheet
Name:_________________________________ Diagnosis:________________________________________
Medications Immunizations
distribute pain questionnaire initiate development of individual-
PE, review past teaching SW review/RN care review
Critical Elements of Care: Sickle Cell Disease III. Guidelines for Care of Children with Sickle Cell Disease: Sickle Cell Disease Flow Sheet
Name:_________________________________ Diagnosis:________________________________________
For all patients review status of new potential treatments and interventions and inform families of resources to store cord blood cells from newbornfull siblings of patients.
review previous teaching, including chest syndrome
review PCN prophylaxis, neurologic event teaching begin coping strategy teaching w/ child
promote self-care, reinforce coping strategies
refer for routine dental care RBC pitscore annually in patients with SC, Sβ+ Thalassemia increase PCN + 250mg BID at age 3; discontinue PCN prophylaxis at age 6
school outreach quantitate HbF by Hgb electrophoresis
PE, BP, vision/hearing screen discuss leg ulcers, priapism, delays in sexual maturation, sexual activity, smoking, drugs, activities,
career goals as developmentally appropriate
refer for genetic counseling directed toward patient in early adolescence initiate discussions on options and process of transitioning to an adult medical facility discuss and screen for depression yearly
Yearly Labs and Studies
Yearly: cbc, diff., retic, plt, BUN, Cr, Alk P, SGPT (ALT), Bili, LDH, and Iron UA annually Yearly after age 2: in patients with SS and Sβ-thalassemia begin Transcranial Doppler Studies Yearly from 2-3 through 13 years: neuropsychologic evaluation Yearly after age 7 in patients with Hb SS, Sβ0 thalassemia: begin yearly CXR, Oximetry or ABG, EKG Yearly after age 7 in patients with Sβ0 thalassemia, Hb, SS, and SC: begin yearly ophthalmology exam Yearly from 71/2 - 13 years: screen for depression, pulmonary function tests including DICO and pleythsmography, opthomology and dental
evaluation, ECHOcardiogram including documentation of tricuspid regurgitation jet velocity yearly for all patients with a history of multiplepneumonias, acute chest syndrome or restrictive lung disease
U/S in alternating years to assess for gallstones in all patients other**
Immunizations
pneumovax™ booster 1-2 yrs. following initial immunization
repeat pneumococcal and meningococcal immunization q 3-5 years
*Check BP beginning age three years; vision/hearing screen beginning age four years. **Children on transfusion programs should have ferritin, Fe, TIBC; %Hb S monitored; annually, 24-hour Holter monitor; pituitary function testing;liver and renal functions should be tested. Consider liver biopsy to regularly assess hepatic iron content and adequacy of chelation therapy. Critical Elements of Care: Sickle Cell Disease IV. GUIDELINES FOR PAIN MANAGEMENT Pain Related to Sickle Cell Disease
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C. Emphasize the Value of a System-Wide Approach
Severity: Varies from mild to extremely intense and
1. Effective pain management is contingent on
described as “body chewing,” “body biting.”
involvement by administration, manager and practitioners. Character: Deep, aching, tiring, fatiguing Developmental Aspects: Can occur as early as four
to nine months of age when fetal hemoglobin levels
Region: Can occur in any part of the body and may
involve single or multiple body parts. Pain due to
swelling in hands and feet from dactylitis typicallyoccurs in children under three years. Common
3. Pain relief is a quality assurance/continuous
quality improvement issue for children with chronic illness. Care effectiveness must be
4. Develop standards of care/clinical guide
Frequency: Sickle cell pain forms a continuum from
dures, and developing coping strategies.
D. Adequate Assessment is the Cornerstone of
• 20 percent have frequent, severe episodes
(6 percent of patients account for 30 percent
1. Pain assessment should be developmen- tally appropriate and a routine part of Precipitating Factors: the inpatient and outpatient care of children with these chronic diseases. 2. The child’s complaints of pain should be believed. Verbal self-report is primary and cannot be disputed.
E. Assess and Develop a Plan of Care at the
1. Computerized profiles − “recipe cards”:
General Principles of Pain Management
Gathers pain history and then child, parent(s) and health care team develop
A number of general principles can be applied to the
plan on the computer. This plan is modified and updated on a real time basis.
management of pain in sickle cell disease.
A. Pain Must be Viewed Within a Chronic Disease
repeated questioning of child/parents(s),
Continuum: Promotion of Wellness and Develop-
particularly as they enter different hospital
3. A pain problem list should be instituted so
B. Health Care Professionals Have the Account-
that pain stemming from the disease and its
ability/Responsibility for Using a Proactive,
Critical Elements of Care: Sickle Cell Disease IV. Guidelines for Pain Management
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4. Hand-held records: Empowers child and
acute chest syndrome. Incentivespirometry while on continuous
(WHO): Basic foundation for pharmaco- logic management. (See Figure 1 on page
Infants:
Distractions: Music/mobiles, soothing talk,
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Physical: Massage (applicability/efficacy
Toddlers/Preschoolers:
magic game, puppets, kaleidoscopes,counting ABCs, music-sing-along
anticipated return of pain is appro-priate, unless pain is truly episodic
blowing bubbles, “meow-woof”breathing, party blowers
Breathing/relaxation: “Go limp as a rag
doll” or “you’re blowing hurt away,”
Imagery: Stories−use images familiar to the
emphasize informational affectiveaspects of the experience; after
should be done slowly to avoidprecipitating severe pain withdrawal.
Physical: Massage, heat/cold, acupuncture,
School-Age/Adolescents:
Modeling/desensitization: Explanations to
Imagery (older): Pain switch familiar images
Critical Elements of Care: Sickle Cell Disease IV. Guidelines for Pain Management
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Figure 1. The WHO (1986) Three-Step Analgesic Ladder
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Opioid for mild to moderatepain + Non-opioid
Critical Elements of Care: Sickle Cell Disease IV. Guidelines for Pain Management: Complication-Specific Guidelines VASO-OCCLUSIVE PAIN Diagnosis Monitoring Diagnostics Medication/Treatment Discharge General Care Criteria
3. Ibuprofen 10 mg/kg po q 8 hr. or other
anti-inflammatory agent if no contraindi-
dose IV q 2 hr. or 0.01 - 0.1 mg/kg/hr.
prn analgesic orders are not appropriate.
5. Encourage incentive spirometry for all
patients, but it is essential for patients
6. Start oral opiates as soon as tolerated
from a gastrointestinal standpoint, even if
for RBC (minor-antigen-matched if available,
pulse ox >92% or > patients baseline
chest syndrome (see acutechest syndrome protocol),
or cardiovascularcompromise present.
9. Offer heating pads or other comfortmeasures previously used by patient.
10. Consider colace or laxative fornarcotic-induced constipation.
11. See other Clinical Care Paths foracute chest syndrome, acute anemiacrisis, stroke, priapism, if present.
12. Reassess pain control at least twicedaily. Analgesics may be weaned astolerated by decreasing dose, not byprolonging interval between doses.
Modified from Mountain States Regional Genetic Services Network, 1996
Discuss analgesic changes with patient/family. Critical Elements of Care: Sickle Cell Disease IV. Guidelines for Pain Management: Sickle Cell Disease Algorithm PAIN EPISODE ALGORITHM Baseline PAIN EPISODE HOME TREATMENT
A. Acetaminophen and/or NSAIDS only for mild pain
mild opioid given around the clock until episode
B. Always combine pharmacologic with nonpharmacologic
B. Always combine pharmacologic with nonpharmacologic
D. Monthly evaluation of treatment plan with child, parent(s),
evaluation of renal function if on NSAIDS
Characteristics and Modifying Factors of Severe Pain
Home treatment ineffective Disruption of normal lifestyle (function, school
attendance); other medical problems disrupting patient and/or parents work
Consider family/childs perception Degree of coping skills Degree of family support/resources
Critical Elements of Care: Sickle Cell Disease IV. Guidelines for Pain Management ER MANAGEMENT: PAIN ASSESSMENT
Precipitating factors Past therapy that helped
* Determine if pain is sickle cell related (not all pain is sickle cell
ER Management: Immediate Treatment
Hydration (PO or IV depending on status) Non-steroidal anti-inflammatory agent, or non-opioid analgesic (PO or IV depending on status) Administer opioid (PO or IV depending on status) Use established pain treatment plans
Response to initial doses of IV opioid in ER:
Critical Elements of Care: Sickle Cell Disease IV. Guidelines for Pain Management GUIDE TO DETERMINATION OF SCHEDULE FOR MAINTENANCE ANALGESIA IN THE HOSPITAL
(Note- This is a generic plan. Individual patient plans may vary.)
Methods for Administering Opioid Analgesia in the Hospital
• Intermittent IV doses around the clock• IV infusion caution: infusions can result in respiratory compromise• IV infusion with intermittent IV doses offered every two hours• Sustained release or immediate acting agents given PO around the clock, with intermittent IV doses offered every two hours• PCA with basal infusion• Sustained release or immediate acting agents given PO around the clock, with PCA without basal infusion
Critical Elements of Care: Sickle Cell Disease IV. Guidelines for Pain Management: Pain Assessment Tools THE OUCHER Assessment Tool 1
If children can count to 100, they can use the numerical scale; if not, they should use the photographic scale.
A. Let children practice using the Oucher. Ask them to recall times they hurt in the past. Have them de- scribe these episodes to you and then rate them on the Oucher.
B. Collect data and convert to scores.
1. After re-explaining the scale, ask, “How much hurt do you have right now?”
2. If the child uses the numerical scale, the number he/she gives is the Oucher score; if the child uses the photographic scale, the picture he/she selects is converted to the appropriate predeter- mined score shown on the oucher (0, 20, 40, 60, 80 or 100). PAIN INTENSITY NUMBER SCALE Assessment Tool 2
Children Developmentally Later School-Age and Adolescent
1. “I need to know how much pain you have because I can’t feel your pain. I want you to use a scale so you can tell me how much pain you have right now.”
2. “The numbers between 0 and 10 represent all the pain a person could have. Zero means no pain and 10 means pain as bad as it could be. You can use any number between 0 and 10 to let me know how much you have right now.”
3. “Give your pain a number between 0 and 10 so I will know the intensity of the pain you feel now.”
4. Record the pain intensity on the nursing flow sheet as 0/10, 1/10, 2/10, etc. (Wilke, D.J., et. al. The Hospice Journal, 6(1), 1-13. Essentials of Pain Management: A Nursing Handbook. Optioncare: Seattle, WA.)
WORK GRAPHIC RATING SCALE Assessment Tool 3
Children Developmentally Later School-Age and Adolescent
1. Place a straight up-and-down mark on this line to show how much pain you have.
2. Record the pain intensity on the nursing flow sheet as “none,” “little,” “medium,” “large” or “worst pos- sible.”
Critical Elements of Care: Sickle Cell Disease IV. Guidelines for Pain Management: Pain Management References Research Dosage Guidelines Dosing Data for NSAIDS Usual Adult Usual Pediatric Oral NSAIDS Comments
Acetaminophen lacks the peripheral anti-inflammatory activity ofother NSAIDS.
The standard against which other NSAIDs are compared. Inhibitsplatelet aggregation; may cause post-operative bleeding.
May have minimal antiplatelet activity; also available as oral
1000 mg initial dose,followed by 500mg q12 hr.
Available as several brand names and as generic; also available as
Many brands and generic forms available.
May have minimal antiplatelet activity.
Available in generic form from several distributors. Parenteral
Intramuscular dose not to exceed 3 days.
Intravascular and intramuscular dose not to exceed 3 days.
initial dose followedby 15 or 30 mg q 6 hr. Oral dose followingIM or IV dosage:10mg q 6-8 hr.
Note: Only the above NSAIDs have FDA approval for use as simple analgesics, but clinical experience has been gained with other drugs as well. 1 Drug recommendations are limited to NSAIDs when pediatric dosing experience is available. 2 Contraindicated in presence of fever or other evidence of viral illness.
Source: Quick Reference Guide for Clinicians, Acute Pain Management in Infants, Children and Adolescents:U.S. Department of Health and Human Services; Public Health Service, Agency for Health Care Policy, 1972. Critical Elements of Care: Sickle Cell Disease IV. Guidelines for Pain Management: Pain Management References Research Dosage Guidelines Dosing Data for Opioid Analgesics Recommended Recommended Recommended Recommended Equianalgesic Equianalge Starting Dose Starting Dose Starting Dose Starting Dose Oral Dose Parenteral (Adults >50kg (Adults >50kg (Children, adults (Children, adults body wt.) body wt.) <50 kg body wt ) <50 kg body wt ) Parenteral Parenteral
dosing) 60 mg q3-4 hr. (singledose orintermittentdosing)
1 mg/kg q 3-4 hr. 4 Opioid − Antagonist and Partial Antagonist
Note: Published tables vary in the suggested doses that are equianalgesic to morphine. Clinical response is the criterion that must be applied for eachpatient: Titration to clinical response is necessary. Because there is not complete cross-tolerance among these drugs, it is usually necessary to use a lowerthan equianalgesic dose when changing drugs and to retitrate to response.
Caution: Recommended doses do not apply to patients with renal or hepatic insufficiency or other conditions affecting drug metabolism and kinetics.
1 Caution: Doses listed for patients with body weight less than 50 kg cannot be used as initial starting doses in babies less than 6 months of age. Consult the Clinical Practice Guideline for Acute Pain Management: Operative or Medical Procedures and Trauma section on management of painin neonates for recommendations.
2 For morphine (hydromorhone and oxymorphone) rectal administration is an alternate route for patients unable to take oral medications, butequianalgesic doses may differ from oral and parenteral doses because of pharmokinetic differences.
3 Caution: Codeine doses above 65mg often are not appropriate due to diminishing incremental analgesia with increasing doses, but continuallyincreasing constipation and other side effects.
4 Caution: Doses of aspirin and acetaminophen in combination opioid/NSAID preparations must also be adjusted to the patient’s body weight. Critical Elements of Care:Sickle Cell Disease I. Appendix
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Psychosocial Aspects of Sickle Cell Disease Effects of Physical Appearance
Sickle cell disease is life altering for most families.
Children with sickle cell disease may display
Learning to accept, cope and respond to this chronic
physical manifestations of their illness. As a result of
illness requires that the practitioner and family work
short stature, low muscle mass or jaundiced eyes and
together. Cooperation must occur in an environment
nailbeds, ridicule by peers and others is possible. This is
where the family feels comfortable. The practitioner
particularly common in children 8-12 years of age.
sets a tone for the relationship. That tone should
Children and their parents should be prepared to use
encourage the family to view the practitioner as a
coping strategies to help them in these situations.
Gaining knowledge and understanding of their illness isone such strategy.
When working with children and families affected
by sickle cell disease, it is important to develop a
School Attendance and Adjustment
comprehensive approach that encompasses psychoso-
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cial issues. Working to understand the issues faced by
Some children with sickle cell disease are fre-
many of these families will help improve relationships
quently absent from school. These absences may be
the result of a painful episode, hospitalization or otherillnesses. Frequent absences from school may result in
The Status of African Americans
incomplete class work and incomplete development ofsocial skills. Students can feel disenfranchised from
In the U.S., sickle cell disease is primarily a
classroom activities and classmates.
disorder of African Americans. Disproportionatenumbers of African Americans face economic chal-
There are a variety of responses these students
lenges of housing, employment and daily living, and
may have, but the extremes of withdrawal or disruptive
often encounter barriers to health care access. The
behavior are particularly troublesome for school
challenge of overcoming discrimination and racism are
personnel or families. Withdrawal may manifest in a
daily realities for many families. In addition, patients
lack of participation in classroom activities or with
and families often do not feel accepted or welcomed.
classmates, day dreaming, a lack of enthusiasm in theprocess of learning, or opposition to attending school as
Although women are the head of many house-
evidenced by verbalization or behavior. Disruptive
holds, family structures vary. Raising children as a
behavior may be displayed through choices in dress or
single parent is challenging − particularly in the areas of
problems in interacting with other children.
economic support, child care and respite time for theparent. As we have become a more mobile society,
These behaviors may indicate that a child is
single parents often face a lack of family support and
feeling overwhelmed by school work, and s/he may not
experience general feelings of loneliness. Extended
know how to ask for assistance. S/he may not be able
families may include both biological family members
to catch up on missed assignments and may not feel a
and those who are not biologically related but who fill
sense of belonging in the classroom. This can lead to
family roles. It is not unusual to have large numbers of
intense feelings regarding relationships at school. In
“family” who care for a child and take various levels of
most cases the child will not be able to clearly state her/
responsibility for that child. In some cases, extended
his feelings, so s/he may need assistance in defining the
families can be overwhelming for the parents. Parents
problems. This may include testing by a neuropsy-
may need support in articulating their needs in this
chologist experienced in working with children affected
setting and in particular, their need for privacy.
by sickle cell to determine if there is an organic basisfor impaired school performance. A counselor or social
Generally, African Americans have strong spiritual
worker may also be helpful in dealing with the school
beliefs that may be historical and cultural. Some
families may be active participants in a church congre-gation and find great support or assistance from their
We encourage families to contact the school each
church family. Others, while having beliefs, may not
year and to provide information about sickle cell
participate in any organized religious group. Still other
disease to teachers and school nurses. There may be
African Americans have migrated to religious groups
other community professionals or resources to help
like Muslim or Buddhist faiths. It is important to
families with this task. Addressing the needs of sickle
respect these beliefs. Insensitivity or infringement upon
cell patients, such as adequate fluid intake, frequent
a family’s belief system can create a rift between
restroom visits and careful review of academic perfor-
mance, enables the school system to become an ally ofthe family. Critical Elements of Care: Sickle Cell Disease I. Appendix
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modifying unwanted behavior should provide some
Physical Activities
support for hospital staff. Alternatively, it is importantto recognize that some parents may not have adequate
Physical exhaustion can precipitate a painful
strategies. In this case, it is important that a child life
episode in children with sickle cell disease. However,
specialist, social worker or other professional be
children may be expected to participate in physical
consulted as a resource for families and staff. It is
activities at school without necessary supportive
essential to assure patients have support and advo-
measures to prevent difficulties. The educational
cates. This can be from family, community or friends.
process for affected children is to ensure adequate
Children should be encouraged to bring school
knowledge about their disease. When affected children
work to the hospital. Some facilities may have volun-
request fluids or petition for modified physical activity
teers who can assist them, or paid staff members who
they are often seen as problem students who want
fulfill this academic role. The school system may also
special treatment. On the contrary, as children grow to
provide tutors for students under certain conditions.
understand the precipitating factors that affect their
Children should be encouraged to telephone friends and
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illness, the fact that they begin to advocate on their own
family members in an effort to stay connected to life
behalf should be viewed as a positive development.
outside the hospital. These strategies allow the child to
However, balancing between disease-appropriate
stay focused on regular activities rather than focused on
behavior and avoiding a negative label is difficult for
their illness. Living with a chronic illness can result in a
children. It is imperative for parents to be involved each
general apathy about life, which can lead to sadness or
year in their child’s classroom, and that they explain to
teachers and administrators the special needs of their
If frequent admissions have been necessary,
adolescents and their families will know the hospital
As children get older, some may experience an
system well. This means they will know the flaws of
increase in desire to compete in sports. This can result
the system as well, which can create tense moments
from peer or family pressure. The desire to “fit in” or
for staff, patients and their families. For practitioners, it
“be like others” is very important for children aged 8-
may be difficult to be confronted about staffing,
12 years. It may not be possible for some children to
equipment or the lack of communication between
participate in contact sports, particularly strenuous
medical staff and families. Families may not know the
sports, due to problems with easy fatigue or enlarged
best ways to communicate their concerns, so it may be
spleens. The result may be teasing by peers for not
necessary to help them define the problem. Some
being able to participate. The child may look for other
problems, like personality conflicts between certain
ways to prove themselves, or may participate in
staff members and families, may not be easily remedied
activities that are medically risky. At this age, children
by the practitioner, but validating the experience and
need activities that help build their self-esteem and
providing suggestions on how to handle situations can
improve understanding about their illness.
help reduce stress. Many hospital system problems donot have simple answers, although some families insist
Effects of Frequent Hospitalizations
Small children who are hospitalized should be
Mortality and Sickle Cell Disease
encouraged to bring special toys, like stuffed animals toprovide comfort when familiar faces are not around. If
For families, the sickle cell diagnosis raises
possible, consults with pain management personnel can
concerns about the affected child’s life span. It is
provide strategies to reduce the trauma of painful
important to talk openly about this fear with families
procedures (see Pain Management). This is important
and their children. With improvements in medical care,
for children who may experience frequent and pro-
and parents’ involvement in learning about and teaching
their children about the illness, 95 percent of children
Some children require frequent hospitalizations as
will live beyond age 18. The possibility of death should
a result of painful episodes, infections or transfusion
be addressed routinely with encouragement, emphasiz-
protocols. Long hospitalizations can cause boredom,
ing the importance of good care at home and creating a
especially if the facility does not have an orientation
positive attitude toward life in spite of the chronic
toward children’s activities. If a child is having prob-
lems with other children as a result of their illness, it islikely that these behaviors will continue during hospital-ization.
Consulting with families about home strategies for
Critical Elements of Care: Sickle Cell Disease II. Appendix: Sickle Cell Disease Algorithm ANEMIA ALGORITHM SIGNS/SYMPTOMS EVALUATION Critical Elements of Care: Sickle Cell Disease II. Appendix: Sickle Cell Disease Algorithm SEPSIS ALGORITHM SIGNS/SYMPTOMS
1. Physical exam, vital signs, evidence of systems or localized
infection, cardiopulm. assessment, spleen size and neurologic exam
5. Culture other body fluids (as clinically indicated)
A. Obvious infection; or A. Not ill-appearing B. Ill-appearing; or B. Lab evaluation normal C. WBC > 30,000 or < 5,000 D. T>39°C (102.2°F) E. Age < 6mos. with HbSS or Sβ0 thalassemia F. Concerns about compliance
50 mg/kg (2gm maximum)Acetaminophen for fever
2 hrs. after parenteral ceftriaxone.
If stable, discharge to home;follow-up in 24 hrs. for second doseceftriaxone. Critical Elements of Care: Sickle Cell Disease II. Appendix: Complication-Specific Guidelines ACUTE CHEST SYNDROME Diagnosis Monitoring Diagnostics Nutrition, Medications/Treatments Discharge General Care Criteria
2. Acetaminophen 15 mg/kg po q 4 hr.
contraindication present (i.e. gastritis,
5. Cefuroxime 50 mg/kg q 8 hr. IV. (Prophylactic penicillin may be
Clarithromycin 15 mg/kg split q 12 hr. po or other macrolide antibiotic.
d) renal (BUN, creat)and liver (fractioned bili,
severe illness or ifdiffuse encephalopathy
a) simple transfusion for moderatelysevere illness, especially if Hb > 1gm/dl below baseline (do nottransfuse acutely to Hb > 10 gm/dl,Hct > 30%).
b) partial exchange transfusion toHb 10 gm/dl and Hb S or Hb S + C(patient’s RBC) < 30 % for severeor rapidly progressive disease. (May require transfer to ICU forerythrocytapheresis). Removefemoral or central venous cathetersas soon as possible after exchangetransfusion to reduce risk ofthrombosis.
Modified from Mountain States Regional Genetic Services Network, 1996
acute anemic crisis, stroke, priapism, ifpresent. Critical Elements of Care: Sickle Cell Disease II. Appendix: Complication-Specific Guidelines STROKE OR ACUTE NEUROLOGIC EVENT Diagnosis Monitoring Diagnostics Medications/Treatments Discharge Nutrition, Criteria General Care
approximately 10 gm/dl may beconsidered as an alternative to partial
acutely to Hb > 10 gm/dl, Hct > 30 %).
Modified from Mountain States Regional Genetic Services Network, 1996
Critical Elements of Care: Sickle Cell Disease II. Appendix: Complication-Specific Guidelines PRIAPISM Diagnosis Monitoring Diagnostics Medication/Treatment Discharge General Care Criteria
1. Broad-spectrum in antibiotics IV if febrile.
penicillin (if not on broad-spectrum anti-
4. Ibuprofen 10 mg/kg po q 8 hr. or other
anti-inflammatory agent if no gastritis, ul-
5. Morphine 0.05 - 0.1 mg/kg IV q 2 hr. or
0.01 - 0.1 mg/kg/hr. continuous infusion or
PCA pump (max total dose) for severe pain.
anesthetic per urology. Notify urologywithin 2 hours with the goal of performing
the procedure within 4 hours of onset. All
attempts should be made to do this within12 hours of onset.
7. Pseudoephedrine < 2 yr 4 mg/kg/day split
q 6 hr. po; 2-5 yr 15 mg q 6 hr. po; 6-12 yr
8. Never use ice or cold packs.
pulse ox > 92% or > patient’s baseline value. Avoid excessive or unnecessary 0 , which
may suppress the reticulocyte count andexacerbate anemia.
10. Reassess pain control at least twice daily. Analgesics may be weaned as tolerated bydecreasing dose, not by prolonging intervalbetween doses.
11. Transfusion if no evidence of detumes-cence within 12 hr.
a) Partial exchange or erythrocytapheresis to Hb 10 gm/dl andHb S (patient’s RBC) < 30%.
b) May consider simple transfusion asalternative to partial exchange transfu-sion if Hb < 6-7 gm/dl (do not transfuseacutely to Hb > 10 gm/dl, hct > 30%).
12. Surgical drainage (i.e. Winter shunt) isusually indicated if priapism persists for >24 hrs., unresponsive to supportive careand transfusions.
13. Observe for severe headache or neuro-logic signs or symptoms. (Ischemic strokemay occur 1-10 days after onset of pri-apism).
Modified from Mountain States Regional Genetic Services Network, 1996
14. See other Clinical Care Paths for acutechest syndrome, acute anemic crisis, stroke,if present. Critical Elements of Care: Sickle Cell Disease II. Appendix: Complication-Specific Guidelines GENERAL ANESTHESIA AND SURGERY Pre-Op Evaluation Pre-Op Transfusion Day Prior to Surgery Intraoperative Post-Operative
Simple transfusion:RBC’s to increase Hb to
procedures (e.g. PE tubes)with brief anesthetics.
considered a minorprocedure). Recommenda-tions for patients with Hb SCor Sβ+-thalassemia vary. Ingeneral, transfusion is notrequired for smallerprocedures such astonsillectomy and/oradenoidectomy, buttransfuion is required forabdominal surgery. Do to ahigh baseline HCT thesepatients often require partialexchange transfusion.
Modified from Mountain States Regional Genetic Services Network, 1996
Critical Elements of Care: Sickle Cell Disease III. Appendix
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References and Resources PATIENT EDUCATION COUNSELING REFERENCES FOR MATERIALS AND RESOURCES PARENTS OF NEWBORNS WITH SICKLE CELL DISEASE AND TRAIT The Family Connection — Sickle Cell Trait (English,
French, Spanish); The Family Connection —
American Society of Human Genetics Membership Directory. Hemoglobin C Trait (English, French, Spanish); New-
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http://genetics.faseb.org/cgi-bin/ASHG-Search
born Screening for Your Baby’s Health (English, Span-
A searchable database of American Society of Human
ish) Sickle Cell Anemia, New York State Department of
Genetics members, and their contact information.
Health, Newborn Screening Program, Wadsworth Centerfor Laboratories and Research, P.O. Box 509, Albany, NY
Cantor A.B., Miller M.C. III, Larisey L, et al. (1979). A study
of media effectiveness for sickle cell anemia education in
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Grossman L.K., Holtzman N.A., Charney E., et al. (1985).
Rico and the Virgin Islands); Sickle Cell Disease: How
Neonatal screening and genetic counseling for sickle
to help your child to take it in stride — A Parent/
cell trait. Am J Dis Child, 139, 241-244. Teacher Guide Viewpoints. Also available: brochureson sickle cell trait, anemia and other topics, home
Loader S., Sutera C.J., Walden M., et al. (1991). Prenatal
study kit, games and a video on parenting. National
screening for hemoglobinopathies; Evaluation of
Association for Sickle Cell Disease, 3345 Wilshire
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Blvd., Suite 1106, Los Angeles, CA 90010-1880;phone: 1-800-421-8453.
Miller D.R. (1979). Pitfalls of newborn screening for sickle
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of California Genetic Disease Center, phone: 510-412-
Neal-Cooper F., Scott R.B.(1988). Genetic counseling in
1542, leave message with desired number of copies and
sickle cell anemia: Experiences with couples at risk.
contact information. First copy is free, additional copies
The Infant and Young Child with Sickle Cell Anemia (a
Rowley P.T., Mack L. Jr., Lawrence F. (1984). Screening and
guide for parents, in English, Spanish); Pneumococcal
genetic counseling for beta-thalassemia trait in a
Infection and Penicillin; So Your Baby Has the Sickle
population unselected for interest: Comparison of three
Cell Trait (English, Spanish). Also available: brochures
counseling methods. Am J Human Genet, 36, 677-689.
on sickle cell trait, sickle beta-thalassemia, hemoglo-bin C disease, pain in children and various complica-tions. Texas Department of Health, Newborn ScreeningProgram, 1100 West 49th St., Austin, TX 78756-3199;
GENERAL REFERENCES Sickle Cell Anemia — What Is It? Cincinnati Children’s
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Sickle Cell Center, Cincinnati Children’s Hospital
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Armstrong P.J. & Bersten A. (1986). Normeperidine toxicity.
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BCA-clinic Betriebs GmbH & Co. KG Dr. med. Armin Schwarzbach Facharzt für Labormedizin Increasing importance of co-infections for Lyme disease patients – with or instead of a Lyme disease infection An article by Armin Schwarzbach,M.D., PhD., laboratory medicine specialist In the past months presentations of scientific conferences have shown a noticeable increase of the impor
Lebenslauf Name: Ausbildung: St. Gotthard Gymnasium der Benediktiner, Niederalteich, Abitur 1988 Ärztliche Vorprüfung am 03.09.1990 1990- 1994 Friedrich-Alexander-Universität Erlangen-Nürnberg 1. Abschnitt der Ärztlichen Prüfung am 29.08.1991 2. Abschnitt der Ärztlichen Prüfung am 22.03.1994 1994 - 1995 Universität Regensburg 3. Abschnitt der Ärztlichen Prüfung am 04.05.1
IV. Appendix: References and Resources