Health Supervision For Children With Sickle Cell Disease
From the American Academy of Pediatrics , 2011
This statement provides pediatricians in primary care and subspecialty practice with an overview of the genetics, diagnosis, clinical manifestations, and treatment of SCD. Specialized comprehensive medical care decreases morbidity and mortality during childhood. The provision of comprehensive care is a time-intensive endeavor that includes ongoing patient and family education, periodic comprehensive evaluations and other disease-specific health maintenance services, psychosocial care, and genetic counseling. Timely and appropriate treatment of acute illness is critical, because life-threatening complications develop rapidly. It is essential that every child with SCD receive comprehensive care that is coordinated through a medical home with appropriate expertise.
What Are The Possible Side Effects And Risks Of Penicillin
People with SCD do not have any more risks associated with penicillin than people without SCD. Side effects and allergic reactions to penicillin are rare, but can occur. Some side effects of penicillin VK include:1,3
Get emergency help immediately if you notice symptoms of an allergic reaction, such as:3
- Fever or chills
- Puffiness or swelling around the face
- Shortness of breath
- Skin rash, hives, itching
These are not all the side effects of penicillin. Talk to your doctor if you notice any changes in your childs symptoms.
Children can use other antibiotics, such as erythromycin, if they are allergic to penicillin.1,7
Another concern is that pneumococcus bacteria will become resistant to penicillin over time. This happens when the bacteria develop ways to prevent penicillin from working. Some studies have shown that people with SCD who take penicillin are no more likely to have penicillin-resistant bacteria. However, it is still a concern that those with sickle cell may experience the emergence of resistant pneumococcal bacteria, so they should work closely with their doctor about the proper use of penicillin and how to treat acute infections. Doctors may still use other types of antibiotics to treat an acute infection.1,8,9
These are not all the possible side effects of penicillin. Talk to your doctor about what to expect or if you experience any changes that concern you during treatment with penicillin.
Things To Know About Penicillin
Once children with SCD reach 5 years old, they are much less likely to experience a pneumococcus infection. This is because their immune systems have developed the ability to respond to pneumococcus. Studies have shown that penicillin does not significantly reduce the risk of infection after age 5.1,10
Many children stop taking penicillin after 5 years old. However, some continue taking it, especially if they have had their spleen removed or have had a severe pneumococcus infection before.1,11
Sticking to a penicillin prophylaxis plan is often difficult. Children with SCD have a higher risk of infection when they do not take penicillin. Infections can happen quickly even after 1 missed dose . Continued education to parents and caregivers about the importance of penicillin has helped improve rates of people sticking to the plan.1,12,13
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Infection And The Pathophysiology Of Scd
Deoxygenation of HbS alters the structure of the -chain of hemoglobin, resulting in reduced solubility, hemoglobin polymerization, and diminished membrane flexibility . Distortion of globin chains and exposure of intracellular heme iron intensifies intracellular oxidant stress, cell membrane damage, and erythrocyte dehydration . Vascular occlusion, tissue ischemia, and a cascade of systemic inflammation activate endothelial cells to interact with erythrocytes, activated leukocytes, the coagulation cascade, and activated platelets . Potent vasoconstrictors are liberated from the endothelium in response to injury . These events result in endothelial dysfunction, inflammation, and tissue ischemia, which correlates with SCD symptom severity . The clinical presentations of SCD-specific complications and serious infections can overlap. Care of the critically ill SCD patient must address infection while supporting the underlying pathophysiology of the disease to prevent or mitigate SCD-related complications. These interactions are summarized in Figure 2.
Chronic endothelial inflammation and dysfunction cause progressive vital organ system deterioration over time. Relevant organ system considerations for critical care management are summarized in Figure 3.
Differential Diagnosis Of Fever And Infectious Syndromes In Scd
Fever without a source is a common problem in SCD patients and should be regarded as a medical emergency requiring rapid evaluation and initiation of intravenous antibiotics. The presentation of sepsis is similar to children without SCD. The presentation of bacteremia may be subtle, however, and can include sudden fever, few prodromal features with a relatively well appearance, and then rapid deterioration sometimes associated with adrenal hemorrhage, progressive shock, and death .
Leukocytes and platelet counts may mimic findings in infections and are similar to children without SCD. An increased hematocrit can signify dehydration due to poor fluid intake or increased fluid losses, whereas decreased hematocrit may result from infection-induced hemolysis, acute chest syndrome, splenic sequestration crisis, malaria, or viral suppression of the bone marrow. Splenomegaly or hepatomegaly may indicate splenic or hepatic sequestration, malaria, or a variety of viral infections.
All febrile children should be evaluated for evidence of early focal infection of the respiratory system, central nervous system, abdomen, and musculoskeletal systems as well as SCD-specific complications, including acute chest syndrome, stroke, vaso-occlusive crisis, aplastic crisis, splenic sequestration, and hepatic sequestration.
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Appropriate Antibiotic Prophylaxis For Children With Sickle Cell Disease
Measure Domain: Management of Chronic ConditionsMeasure Sub-Domain: Sickle Cell TreatmentPQMP COE: Q-METRICAssociated NQF # and Name: 3166, Antibiotic Prophylaxis Among Children with Sickle Cell Anemia
Measure Technical Specifications and Other Supporting Documents:
Internet Citation: Appropriate antibiotic prophylaxis for children with sickle cell disease. Content last reviewed August 2021. Agency for Healthcare Research and Quality, Rockville, MD.https://www.ahrq.gov/pqmp/measures/scd-antibiotic-prophylaxis.html
Determine If The Surgery Is Really Necessary
Given the high risk of both surgical and postoperative complications in patients with SCD, it is imperative to first determine whether conservative management strategies have truly failed. Consider the risks associated with surgery vs continuing with conservative management, including referral to a specialist center offering less invasive options. Ascertain a patient’s personal motivations for and expected outcomes of having surgery to determine whether surgery will allow them to achieve their personal goals. This process should be a shared decision between the patient and health care providers.
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Predictors Of Antibiotic Adherence
We evaluated potential associations between receiving 300 days supply of antibiotics and the following predictors: age, sex, use of health services , calendar year, and state of residence. Our approach adjusted for state of residence as a confounder to partially account for the unmeasured variation of these factors between states. Classification of health care encounters was expanded to include any mention of sickle cell disease to account for potential misclassification of sickle cell subtype within the encounter.
Doses For Penicillin Prophylaxis
In the PROPS study, all patients received penicillin V potassium 125 mg twice daily. This has become the dose recommended for children younger than 5 years of age.15,16 In patients over the age of 5 years, penicillin V potassium 250 mg twice daily is suggested.16 Children should be started on penicillin prophylaxis once the diagnosed is established or at least by 2 months of age.16 Penicillin suspension can be used in place of penicillin tablets, but the shelf-life of the reconstituted suspension is only 14 days.17 If compliance is considered to be a problem, monthly injections of 600,000 units of benzathine penicillin IM have been used in Jamaica.18 However, these injections are painful, and the effectiveness of the injection for the later half of the month is questionable.11 Amoxicillin 20 mg/kg/day is an alternative to penicillin.16 For patients who are allergic to penicillin, erythromycin 125 mg twice daily is given to children younger than 5 years of age, and erythromycin 250 mg twice daily is given to children 5 years of age or older.18
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Critical Care Considerations In Scd
An exhaustive discussion of critical care support of sepsis with organ dysfunction is beyond the scope of this review. Critical care support of patients with severe sepsis and organ dysfunction is similar to that provided to patients without SCD with additional considerations specific to persons with SCD.
In this section, we will review the considerations relevant specifically to the management of SCD patients with severe sepsis. We present issues related to hemodynamic assessment and support, respiratory considerations, the role of transfusion therapy in critically ill persons with SCD, and precautions related to granulocyte colony-stimulating factor and coagulation factor replacement.
Circulatory support must be individualized and frequently reassessed. No single inotrope or vasopressor can be recommended, as therapy must be determined on the basis of serial hemodynamic evaluations. Vasoconstrictor medications, though often necessary to maintain vital organ perfusion pressure, should be minimized and weaned whenever possible as they promote sickle hemoglobin polymerization by prolonging transit through the vasculature. Individuals with SCD typically have lower blood pressure than matched African American controls , therefore, blood pressure should be viewed in the context of direct and indirect measures of adequate oxygen delivery.
Why Is Penicillin An Important Treatment For Sickle Cell Disease
Children with SCD are vulnerable to certain types of bacterial infections. This is because blocked blood flow in the spleen prevents the spleen from working. The spleen is important to filter out certain types of bacteria from the blood, including Streptococcus pneumoniae .1,2
Penicillins are a group of antibiotics that kill or block the growth of bacteria. Penicillins weaken the cell wall of these bacteria. This causes bacteria to eventually burst and die. The type of penicillin used in SCD is called penicillin VK. Penicillin VK is given by mouth and is most effective against infections caused by spleen disorders.1,3
Pneumococcus infections can quickly become severe in children with SCD. Because of this, preventing infections is usually more effective than treating them. Children with SCD should take penicillin twice a day from 2 months old to 5 years old. This is often called penicillin prophylaxis because it is taken to prevent an infection.1
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Risk Of Bacterial Infection
Bacterial infections are a major cause of morbidity and mortality in SCD. This increased susceptibility is mainly a result of impaired splenic function, however other factors, such as defects in complement activation, micronutrient deficiencies, tissue ischemia and inflammation also contribute. S. pneumoniae, H. influenzae type b, and non-typhi Salmonella species, have been identified as important causes of infection in SCD. Other bacteria associated with frequent infections in SCD include Mycoplasma and C. pneumoniae, which cause pneumonia, ACS and Yersinia enterocolitis.
One reason for the ongoing risk of pneumococcal infection in patients with SCD may be genetic changes among the bacteria. A study looking at the genotypes of more than 300 S. pneumoniae strains isolated over 2 decades from SCD patients showed a shift to non-vaccine serotypes and other mutations affecting antibiotic resistance, metabolism and iron transport. These adaptations to the SCD host may confer advantages to S. pneumoniae and reduce the effectiveness of vaccines and antibiotics. .
How Do We Know That Penicillin Prevents Infections In People With Sickle Cell Disease
Penicillin greatly reduces the risk of infections in people with SCD. The first evidence of this was published in 1986. In the study, children between 3 months and 3 years old took either penicillin or placebo twice a day. The study ended early because penicillin was so effective. Penicillin reduced the incidence of pneumococcus infection by 85 percent.4
The study also found that penicillin is more effective when it is started earlier. This is the main reason why we now screen every newborn for SCD. Early diagnosis allows doctors to start penicillin treatment before the age of 4 months.1,4
Until the 1990s, up to 30 percent of children with sickle cell anemia died from infections. Early diagnosis and penicillin treatment have reduced this to only 3 percent.4-6
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Duration Of Penicillin Prophylaxis
Older children experience a dramatic decline in the incidence of pneumococcal infection when compared to children less than 5 years of age. This is thought to be a result of older children’s increased ability to produce an immunologic response to encapsulated organisms such as S. pneumoniae.11,19 In addition, there are concerns about higher rates of penicillin-resistant organisms associated with the use of penicillin prophylaxis in older children who no longer have the same risk of pneumococcal infection.
To determine whether penicillin prophylaxis should be continued for children with SCA after the 5 years of age, the Prophylactic Penicillin Study Group conducted PROPS II.19 This was a randomized, double-blinded, placebo-controlled trial to evaluate the effects of discontinuation of penicillin prophylaxis at 5 years of age in children who had received penicillin prophylaxis for at least two years prior to enrollment into the study. Patients were seen or contacted every three months in order to obtain medical history and dispense study medication. If a child experienced a febrile illness, they were examined and blood was collected for cultures. Those who continued penicillin prophylaxis received penicillin V potassium 250 mg twice daily. Those randomized to have their penicillin prophylaxis discontinued received a matching placebo that was administered twice daily.
The Management Of Sickle Cell Disease
From the National Heart Lung and Blood Institute , 2014
The practice guidelines best supported by scientific evidence are:
- Penicillin prophylaxis prevents pneumococcal sepsis in children
- Pneumococcal vaccine prevents pneumococcal infection in children
- In surgical settings, simple transfusions to increase hemoglobin levels to 10 g/dL are as good as or safer than aggressive transfusions to reduce sickle hemoglobin levels to below 30 percent
- Transfusions to maintain a hematocrit of more than 36 percent do not reduce complications of pregnancy
- Transfusions to reduce Hb S levels to below 30 percent prevent strokes in children with high central nervous system blood flow
- Hydroxyurea decreases crises in patients with severe sickle cell disease
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Approach To Antimicrobial Coverage
Appropriate initial empiric antibiotic therapy for the most likely pathogens is presented in Table 2. Initial antibiotic selection for patients presumed to be bacteremic should be broad and active against encapsulated organisms as well as other common pathogens. Antibiotic selection is further influenced by local epidemiology and antibiotic resistance patterns . The empiric use of vancomycin and a third-generation cephalosporin provides coverage against: resistant pneumococci, meningeal penetration, and Staphylococci and enteric Gram-negative pathogens. Antibiotic coverage should be tapered based on culture results to minimize development of bacterial resistance and opportunistic fungal infection. The addition of anti-pseudomonal antibiotics should be considered if hospital-acquired pneumonia is present, and oseltamivir should be administered if influenza is considered likely.
Viral Pathogens In Scd
Respiratory viruses can trigger significant complications in patients with SCD, including acute chest syndrome, bacterial superinfection, aplastic crisis, splenic sequestration, and painful vaso-occlusive crisis . Viral respiratory pathogens promote the development of acute chest syndrome by inducing lung inflammation, injury to the microvasculature of the lung, airway hyper-reactivity, mismatch of ventilation and perfusion, and in some instances, secondary bacterial infection .
The prevalence of HIV seropositivity in SCD patients varies between 0 and 11.5% . Few data are available regarding the impact of coexistent HIV infection and SCD but both diseases increase the risk for stroke, splenic dysfunction, avascular necrosis, and pulmonary arterial hypertension . SCD patients with HIV may be more susceptible to infection with encapsulated bacteria as well as opportunistic pathogens. In a U.S. hospital-based study, SCD with HIV infection conferred a greater risk for hospitalization for bacterial infection and sepsis but less risk for vaso-occlusive crisis. Inpatient case fatality data for children with SCD and HIV were not different from that of children with SCD alone but fatalities were lower than those of children with HIV infection only . SCD may confer protection against HIV infection because of upregulation of inflammation, iron metabolism, and auto-splenectomy, which are not favorable for HIV replication .
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Roadmap For Research And Implementation
Therapies designed to interfere with the sickle cascade at many levels of the pathway are ongoing. Such efforts include strategies to reduce hemoglobin polymerization through the stabilization of HbS with medications such as voxelotor and l-glutamine , as well as interference with interactions between the cellular elements of the blood and the endothelium with the monoclonal antibody crizanlizumab . Molecular strategies designed to nullify the cellular interactions that characterize the pathophysiology of SCD may someday delay or prevent acquired spleen dysfunction, thus allowing the infant’s maturing immune system to develop.
In resource-rich environments, morbidity and mortality related to infectious sequelae have been greatly reduced. In such environments, research should include more effective vaccines to more completely immunize SCD children against bacterial and viral pathogens. Likewise, investigation into barriers that prevent 100% compliance with vaccination and antibiotic prophylaxis is necessary to ensure that children capitalize on the availability of optimal nutrition, disease-modifying medications , antibiotic prophylaxis, and vaccination participation. Ongoing surveillance into the development of microbial resistance to antibiotics and immunization strains is needed to stay current with effective prophylaxis and immunization.
Penicillin Prophylaxis In Sickle Cell Disease
|The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details.|
|First Posted : October 28, 1999Last Update Posted : March 25, 2016|
|Anemia, Sickle CellHematologic DiseasesHemoglobinopathiesInfection Pneumonia||Drug: penicillin|
For over 20 years children with sickle cell anemia have been known to have an increased susceptibility to severe bacterial infection, particularly due to Streptococcus pneumoniae. Meningitis, pneumonia and septicemia due to this organism have been recognized as the major causes of death for these children, with children under three years of age being at highest risk. The annual incidence of pneumococcal septicemia among young children with sickle cell anemia appears to have remained remarkably constant over the last two decades at approximately 10 percent. This illness can often be fulminant, progressing from the onset of fever to death in less than 12 hours, with a case fatality rate ranging as high as 35 percent.
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