Table 3.
Prophylaxis for the most common pathogens in patients with sickle cell disease, including those living in austere environments.
| Infection/system | Micro-organism(s) | Prophylaxis*+ |
|---|---|---|
| Bacteremia/sepsis | S. pneumoniae, S. aureus, GNR (Typhi and non-typhi Salmonella, E. coli, Klebsiella sp., H. influenzae), Bacteroides sp. (49–51) | - Diphtheria/tetanus/pertussis/H. influenzae type B/polio/13-valent pneumococcal vaccine at 2, 4, and 6 months (13-valent pneumococcal and H. influenzae type B also at 12–15 months); - S. pneumoniae 23-valent vaccine (at 2 years of age and 5 years later) at least 2 months after the 13-valent vaccine (68) - Penicillin V prophylaxis; erythromycin if penicillin allergy. Starting at age of 2 months until 5 years or for a history of pneumococcal sepsis or surgical splenectomy and continued lifelong (68) - Salmonella typhi vaccine for travel to resource-poor areas |
| Meningitis/central nervous system | S. pneumoniae, H. influenzae, and N. meningitidis (11, 52); Pasteurella multocida and Capnocytophaga sp. (in the presence of dog bite), viruses (Enteroviruses, herpes simplex viruses, mosquito-borne viruses); Cryptococcus neoformans and cerebral Toxoplasma gondii (especially in the presence of HIV) | - Diphtheria/tetanus/pertussis/H. influenzae type B/polio/13-valent pneumococcal vaccine/meningococcal vaccine+ at 2, 4, and 6 months (13-valent pneumococcal/H. influenza type B also at 12–15 months and meningococcal vaccine+ at 12 months). - S. pneumoniae 23-valent vaccine (at 2 years of age and 5 years later) at least 2 months after the 13-valent vaccine (68) - Meningococcal vaccine+2, including travel to resource-poor areas. - Meningococcal B vaccination+3, can start at 10 years of age. - Penicillin V prophylaxis; erythromycin if penicillin allergy. Starting at age of 2 months until 5 years or for a history of pneumococcal sepsis or surgical splenectomy and continued lifelong (68) - Avoid bites, scratches, and have mindful contact with dogs (Capnocytophaga sp., Pasteurella multocida). |
| Upper and lower respiratory tract infection (sinusitis, epiglottitis, tracheitis, bronchitis, pneumonia) | Viruses (influenza viruses, respiratory syncytial virus, adenovirus, metapneumovirus, rhino-enterovirus, parvovirus B-19, parainfluenza viruses, cytomegalovirus, Epstein-Barr virus, and herpes simplex viruses, etc.) (55, 56); bacteria (S. pneumoniae, H. influenzae, Chlamydophila pneumoniae, Mycoplasma pneumoniae, Legionella sp., S. aureus (methicillin susceptible and resistant) (11, 57–59) | - Annual influenza vaccine from 6 months. - Diphtheria/tetanus/pertussis/H. influenzae type B/polio/13-valent pneumococcal vaccine at 2, 4, and 6 months (13-valent pneumococcal and H. influenzae type B also at 12-15 months). - S. pneumoniae 23- valent vaccine (at 2 years of age and 5 years later) at least 2 months after the 13-valent vaccine (68) - Penicillin V prophylaxis; erythromycin if penicillin allergy. Starting at age of 2 months until 5 years or for a history of pneumococcal sepsis or surgical splenectomy and continued lifelong (68) |
| Musculoskeletal (skin and soft tissue infection, septic arthritis, fasciitis, myositis, osteomyelitis) | Typhi and non-typhi Salmonella, Gram-negative enteric bacteria, other Gram-negative (Kingella kingae, especially in the presence of negative cultures), S. aureus (methicillin susceptible and resistant), S. pneumoniae (51, 52, 60–62) H. influenzae type B | - Diphtheria/tetanus/pertussis/H. influenza type B/polio/13-valent pneumococcal vaccine at 2, 3, and 4 months (13-valent pneumococcal/H. influenza also at 12–15 months). - S. pneumoniae 23-valent vaccine (at 2 years of age and 5 years later) at least 2 months after the 13-valent vaccine (68) - Penicillin V prophylaxis; erythromycin if penicillin allergy. Starting at age of 2 months until 5 years or for a history of pneumococcal sepsis or surgical splenectomy and continued lifelong (68) - Salmonella typhi vaccine for travel to resource-poor areas. |
| Gastrointestinal (cholelithiasis/choledocholithiasis, cholecystitis, cholangitis, intussusception), gastroenteritis | Enteric Gram-negative pathogens, including Typhi and non-typhi Salmonella, Enterococci, and anaerobic bacteria (65) | - Prevention of vaso-occlusive crisis: avoid hypoxia, acidosis, hypothermia, infection, hypovolemia, etc.) - Decreased hemolysis/gallstone formation: hydroxyurea, ursodiol, low-fat diet - Prophylactic cholecystectomy? - Salmonella typhi vaccine for travel to endemic areas, known exposure to a carrier or laboratorian who works with S. typhi. |
| Urogenital (urinary tract infection, pyelonephritis, renal abscess, urosepsis) | Gram-negative pathogens | Prevention of vaso-occlusive crisis: avoid hypoxia, acidosis, hypothermia, infection, and hypovolemia. |
| Malaria | Plasmodium falciparum, Plasmodium vivax, Plasmodium ovale, Plasmodium malariae, and P. knowlesi | - Lifelong antimalarial chemoprophylaxis in patients living in malaria-endemic countries. - Travel to malaria-endemic areas: a. Avoid exposure to mosquitoes and avoid areas with outbreaks of mosquito-borne infections. b. Mosquito barriers: b.1. Wear Permethrin clothing that fully covers arms and legs and closed shoes, especially during early morning and late afternoon. b.2. Bed nets, screens, and nets tucked around strollers and other confined spaces where young children are placed. Insecticide-treated nets. b.3. Use Environmental Protection Agency-registered mosquito repellents. c. Avoid outdoor activities at dawn and dusk in malaria-endemic areas). d. Malaria chemoprophylaxis□ |
| Tuberculosis (Mycobacterium tuberculosis) | Mycobacterium tuberculosis | - Annual tuberculin skin test or interferon-gamma release assay for HIV-infected persons and incarcerated adolescents. - Bacille Calmette-Guerin (BCG) immunization for those living in endemic areas, in U.S.: when risk of exposure is unavoidable and failure or unfeasibility of other control methods. - Latent tuberculosis infection identification and treatment. |
| Human immunodeficiency infection | Human Immunodeficiency Virus (HIV-1 and HIV-2); HIV-2 is mainly prevalent in Western Africa | - Safe sex practices. - Pre-exposure prophylaxis for men who have sex with men, heterosexual couples, and injection drug userse. - Post-exposure prophylaxis (sexual, other non-occupational exposure, occupational exposure)e. - Prevention of HIV transmission from infected pregnant mother-to-childΩ. |
| Dengue fever, dengue hemorrhagic fever/dengue shock syndrome | Arbovirus (Flaviviridae family; genus Flavivirus) | - Avoid exposure to mosquitoes and avoid areas with outbreaks of mosquito-borne infections. - Eliminate local mosquito breeding sites (elimination/drainage of receptacles for standing water), keep swimming pools, children's wading pools, and bird baths clean; clearing clogged rain gutters. - Mosquito barriers: a. Wear Permethrin clothing that fully covers arms and legs and closed shoes, especially during early morning and late afternoon. b. Bed nets, screens, and nets tucked around strollers and other confined spaces where young children are placed. Insecticide-treated nets. c. Use Environmental Protection Agency- registered mosquito repellents.- Avoid outdoor activities during daylight hours in Dengue-endemic areas. - No chemoprophylaxis available. - In May 2019, Dengvaxia was FDA approved for 9-45 year olds with laboratory-confirmed prior dengue virus infection. Other vaccine candidates are under clinical trials. |
| Parasitic infections | Helminths: Ascaris lumbricoides, Ancylostoma duodenale, Trichuris trichiura, Strongyloides stercoralis, Schistosomiasis (S. mansoni, S. haematobium, S. japonicum), Toxocara canis, T. filariasis (Onchocerca volvulus) | - Sanitary disposal of human feces (all parasitic infections). - Vegetables cultivated in areas where uncomposed human feces are used as fertilizer must be washed thoroughly and cooked before eating (A. lumbricoides). - Wear shoes to avoid contact with contaminated soil (A. duodenale). - Chemotherapy prophylaxis: albendazole, mebendazole) to pre-school and school-aged children in areas with >20% prevalence of infection (A. lumbricoides, T. trichiura). - Screening and treatment of high-risk groups (e.g., children, agricultural workers, and immigrants from endemic areas (A. duodenale). - S. stercoralis serology in all people with unexplained eosinophilia. - Elimination of the intermediate snail host in endemic areas; avoid contact with fresh water streams, rivers, ponds, or lakes in endemic areas (schistosomiasis). - Proper disposal of cats and dog feces, deworming of dogs and cats, covering sandboxes when not in use (T. canis). - Repellents and protective clothing (long sleeves and pants) to decrease exposure to black flies' bites during the day; community-wide mass ivermectin treatment: filariasis). |
| Parasitic infections | Protozoa (other than malaria): Entamoeba histolytica, Entamoeba coli, and Giardia lambli | Protozoa (other than malaria): Entamoeba histolytica, Entamoeba coli, and Giardia lamblia- Chronic Giardia infection with secondary chronic intestinal malabsorption and failure to thrive. - Toxic megacolon, fulminant colitis, ulcerations on colonic mucosa and secondary perforation; hepatic, pleural, lungs, and pericardium abscesses (E. histolytica). - E. histolytica: a. Asymptomatic cyst excreters (intraluminal infection): paromomycin or diiodohydroxyquinoline/iodoquinol. b. Invasive colitis or extraintestinal disease: metronidazole or tinidazole followed by diiodohydroxyquinoline/iodoquinol or paromomycin. c. Percutaneous or surgical aspiration of large liver abscesses. d. Piperacillin-tazobactam or Meropenem if peritonitis. - Giardiasis: metronidazole, nitazoxanide, or tinidazole. - Hand hygiene after defecation, before preparing or eating food, after changing a diaper or caring someone with diarrhea, and after handling an animal or its waste. |
| - Sanitary disposal of fecal material. - Treatment of drinking water (boiling, chemical disinfection with iodine or chlorine, use of filters). - Sexual transmission: use of condoms and avoidance of sexual practices that may permit fecal-oral transmission (E. histolytica). - Refrain from using recreational water venues (e.g., swimming pools, water parks) until asymptomatic and completed treatment. |
*
The standard vaccine series of childhood according to the American Academy of Pediatrics and the Advisory Committee on Immunization Practices should also be provided to all patients with sickle cell disease.
+
CDC-guided immunization schedule (for 2019) with notes for those with sickle cell disease: https://www.cdc.gov/vaccines/schedules/hcp/imz/child-adolescent.html.
+2
Menveo (groups A, C, Y, W-135) or MenHibtix (groups C, Y, and Haemophilus b Tetanus Toxoid conjugate). Menactra not given until child is 2 years old, with two doses given 8 weeks apart unless travel to country with endemic disease—can start at 9–23 months and given 12 (preferred) or 8 weeks apart.
+3
Can be given to adolescents 16–23 years of age at clinical discretion. Bexsero: two-dose series at least 1 month apart. Trumenba: three-dose series at 0, 1–2, and 6 months. Bexsero and Trumenba are not interchangeable, and the same product must be given for all doses used in a series.