Should I Consider PFAPA?

Patient Presentation
A 5-year-old female came to second opinion clinic with a 1 year history of intermittent fevers. The fevers occurred every 6 weeks for 48-72 hours with documented fevers to 102-103&deg F. Over the year, the fevers increased in frequency to every 3-4 weeks. She had fatigue during the fevers and mild sore throat. The patient was seen for most of these episodes. The primary care physician and an otolaryngologist documented anterior cervical adenopathy of 1.0 cm or more, and ulcerations in the mouth in most of the episodes. She was taking no medication other than acetaminophen. Review of the medical records and diaries from the family showed laboratory studies for strep pharyngitis positive one time, but all other testing for streptococcal pharyngitis were negative. Complete blood counts were normal during and between episodes, except for occasional elevated platelets, or mild leukocytosis (not at the same time) and showed no cyclicity. She also sometimes had a mildly elevated C-reactive protein. Testing for histoplasmosis and other fungal diseases were negative. She had undergone a tonsillectomy and adenoidectomy approximately 2 months ago. She had no fever for 6 weeks afterwards and then had another episode.

The past medical history showed a hospitalization for respiratory syncytial virus as an infant, and 2-3 episodes of otitis media.

The family history revealed that the family was Caucasian of northern European descent. There were no genetic abnormalities, connective tissue diseases, immunodeficiencies or children that died early in life. The younger and older siblings were healthy as were the parents.

The social history showed that the family lived on a grain farm without animals. The drinking water had been tested for infectious diseases and toxins and was negative. She had missed school because of the fevers per the school policy and fatigue but made up all her work quickly and returned to school as soon as possible. She was a good student. There was no history of travel, or exposure to persons who had traveled outside the United States or were in the military. She had two outside dogs who were healthy.

The review of systems showed no nausea/emesis, cough, normal stooling and voiding, no sweating, chills, or rashes. She had been gaining weight and height appropriately over the year.

The pertinent physical exam showed an alert female in no distress. She was afebrile with growth parameters in the 10-50%. HEENT examination showed normal mouth examination and bilateral anterior cervical lymph nodes at the angle of the mandible which were both less than 0.5 centimeters. She had some shoddy groin nodes but no other adenopathy. The rest of her examination was negative.

The laboratory evaluation included electrolytes, urinalysis, erythrocyte sedimentation rate, C-reactive protein, rheumatoid factor, immunoglobulin D and E levels, and titres for cytomegalovirus and Epstein Barr virus. All were negative. The radiologic evaluation of chest and abdominal radiographs were also negative.

The diagnosis of probable periodic fever, aphthous ulcers, pharyngitis and adenitis (PFAPA) was made. The natural history of this syndrome along with possible treatment options were discussed. The family opted to use prednisone with the fevers as a potential abortive agent. The results of the consultation were communicated to the primary care physician by telephone and letter. The patient did not return for follow-up to the second opinion clinic.

Discussion
Fevers that recur can be very difficult to evaluate and treat particularly as the child often appears fairly well. Evaluation is frequently complicated by incomplete history, documentation of physical examinations, and laboratory testing. Evaluations decisions are also complicated by patient and family anxiety about potential serious disease and frustration by lack of a firm diagnosis. Families may also interpret the normal variation of common childhood illnesses that are often accompanied by fever such as common colds and ear infections, as evidence that “there is something wrong with this child.” Normal childhood illnesses are usually supported by family members with similar illnesses or attendance at child care. Occult bacteremia and urinary tract infections should also be considered in the child who presents with fever and no localizing signs.

Learning Point
PFAPA syndrome is characterized by predictable periodic fevers, aphthous stomatitis, pharyngitis, and cervical adenitis. Its cause is unknown and is diagnosed by a through history, physical examination, record review and judicious laboratory studies. It occurs at intervals of 3-8 weeks and usually lasts 3-8 days. The fevers usually begin around 5 years of age, with no ethnic association. PFAPA and Familial Mediterranean Fever are both reported more commonly in the area of Israel. The episodes resolve spontaneously and over time the syndrome itself resolves. Patients are well between episodes, and have normal growth and development. Associated symptoms include chills, sweating, and pain in the head, muscles, bones, joints and abdomen may occur. Infectious diseases and malignancies are uncommon causes of predictable periodic fevers. A history of unusual or severe infections should make the clinician not look at PFAPA as a cause of the periodic fever.

The differential diagnosis for PFAFA can include:

  • Crohn’s disease
  • Immunodeficiencies – congenial and acquired including immunoglobulin deficiencies, T-cell dysfunction/deficiency, complement and phagocytic cells dysfunction/deficiency, cyclic neutropenia and HIV
  • Beçet disease
  • Juvenile idiopathic arthritis
  • Hereditary periodic fever syndromes
    • Chronic infantile neurologic cutaneous articular syndrome
    • Familial cold autoinflammatory syndrome
    • Familial Mediterranean fever
    • Hyperimmunoglobulinemia D with periodic fever syndrome
    • Muckle-Wells syndrome
    • Tumor necrosis factor receptor-associated periodic syndrome

Some studies have supported treatment by tonsillectomy and/or adenoidectomy. Prednisone (1 dose, often at 2 mg/kg/dose) given at the onset of symptoms may also abort the episode in some patients. Cimetidine is also effective in some patients. Other immunomodulators have had less efficacy.

Questions for Further Discussion
1. What signs/symptoms would make you concerned for possible malignancy as the cause of a recurrent fever?
2. What signs/symptoms would make you concerned for an unusual infectious disease as the cause of a recurrent fever?

Related Cases

    Disease
    PFAPA Syndrome
    Fever

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Fever and at Pediatric Common Questions, Quick Answers for this topic: Fever.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

Padeh S, Berkun Y. Auto-inflammatory fever syndromes. Rheum Dis Clin North Am. 2007 Aug;33(3):585-623.

Shinawi M, Scaglia F. Hereditary Periodic Fever Syndromes. eMedicine. Available from the Internet at http://emedicine.medscape.com/article/952254-overview (rev. 11/2/2008, cited 2/17/09).

ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.
    7. All medical and invasive procedures considered essential for the area of practice are competently performed.
    8. Health care services aimed at preventing health problems or maintaining health are provided.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

  • Interpersonal and Communication Skills
    19. The health professional works effectively with others as a member or leader of a health care team or other professional group.

  • Systems Based Practice
    23. Differing types of medical practice and delivery systems including methods of controlling health care costs and allocating resources are known.
    25. Quality patient care and assisting patients in dealing with system complexities is advocated.

    Author
    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

  • What Are the Complications of Mastoiditis?

    Patient Presentation
    A 24-month-old female came to clinic after 7 days of various symptoms. Initially she started to have a low grade fever that responded well to anti-pyretics. Two days later she non-specifically stated that her ears hurt. Her fever continued and two days later she was seen and her examination was normal including her ears. One day later her left ear seemed to be sticking out and she was seen in the emergency room. The physician noted slight erythema of the posterior ear, but with a normal tympanic membrane. Otolaryngology was informally consulted and suggested to use amoxicillin-clavulanate antibiotic. She was seen in clinic two days later with her ear being more prominently displaced anteriorly. She was also fussier and not sleeping well. Her fever continued to be < 101° F. No trauma or foreign bodies were reported.

    The past medical history revealed a fully immunized healthy child who attended a group childcare home 2 days/week.

    The family history was non-contributory. The review of systems showed no dizziness or ataxia.

    The pertinent physical exam showed an unhappy female with normal vital signs and growth parameters. She had a protuberant left ear that was erythematous and tender posteriorly involving the mastoid prominence along the outline of the pinna. The neck was not involved and had full range of motion. Her left tympanic membrane was non-erythematous with some fluid noted. The right tympanic membrane was normal. She had shoddy anterior cervical lymph nodes and groin nodes. She had a normal neurological examination and the rest of the examination was normal.

    The diagnosis of mastoiditis was made. The radiologic evaluation of a computed tomograph of the head showed fluid involving the left mastoid aircells with adjacent soft tissue swelling and abscess but without bony destruction. The patient was taken to the operating room for placement of bilateral pressure equalizing tubes for drainage. The otolaryngologists noted she had a ‘peel’ that covered the tympanic membrane that was giving a falsely non-erythematous visual examination of her left tympanic membrane. She was placed on ceftriaxone intravenously. Cultures of the middle ear fluid eventually were negative.

    The patient’s clinical course showed no improvement over the next 1.5 days and a repeat computed tomography exam also showed no improvement in the abscess. She was again taken to the operating room for incision and drainage. She then improved clinically in the next 24 hours and was discharged home to finish 14 days of oral antibiotics. Three weeks later her audiogram showed slightly abnormal hearing on the left.

    Case Image

    Figure 75 – Axial computed tomography image obtained with intravenous contrast from an exam of the maxillofacial region in bone (above) and soft tissue (below) windows. On the bone window image, the right mastoid air cells are clear and the left mastoid air cells are opacified, representing left mastoiditis. Additionally, on the soft tissue window image, just lateral to the left mastoid air cells in the soft tissues, there is a low density fluid collection with an enhancing rim that represents a Bezold’s abscess. There was no evidence of bony destruction, venous sinus thrombosis, or intracranial extension.

    Discussion
    Children have been having problems with their ears for centuries.
    Dr. Thomas Morgan Rotch in the first edition of his pediatric text quoted the also famous German otologist, Dr. Anton Von Tröltsch, who said “there is an unusually strong disposition to disease of the middle ear, owning on the one hand to the double influence of the peculiar morphological relations of the ear and the pharynx, and on the other hand to the disease and conditions of life to which the child is frequently exposed.”

    Mastoiditis is an inflammatory process of the mastoid air cells. The mastoid is contiguous with the middle ear cleft and therefore it is involved in most patients with acute otitis media. However, acute mastoiditis occurs when the infection spreads beyond the mucosa of the middle ear cleft, there is osteitis in the air-cell system, or there is mastoid process periostitis. These are usually caused by direct bony erosion or through the emissary vein of the mastoid indirectly.

    In the pre-antibiotic era, mastoiditis was a common and feared complication of otitis media in up to 20% of all cases. Treatment was surgical but still complications and death were all too common. The John Simon Guggenheim Foundation which offers numerous annual international fellowships was begun in 1925 after the eldest son of philanthropist Simon Guggenheim reportedly died from mastoiditis. A 1946 study by House documented an 80% decrease in the number of mastoidectomies performed after antibiotics began being significantly used for otitis media treatment.

    Children with mastoiditis are usually young (< 2 years of age). Mastoiditis can present with otalgia, fever, hearing loss, abnormal tympanic membrane, otorrhea, posterior auricular erythema or edema, and protuberance of the pinna. Persistent otorrhea and otalgia while on oral antibiotics especially with any neurological symptoms suggest complications of otitis media and the need for further evaluation. Organisms commonly found in mastoiditis include Streptococcus pneumoniae, Staphylococcus pyogenes, Haemophilus influenza, non-typeable Proteus mirabilis, and Pseudomonas aeruginosa. Mastoiditis can be treated with myringotomy (and/or pressure-equalizing tube placement), abscess drainage or mastoidectomy.

    Learning Point
    Mastoiditis complications include:

    • Bacteremia – with subsequent seeding of other body parts including lung embolization
    • Central Nervous System
      • Epidural abscess
      • Subdural abscess
      • Hearing loss – temporary and permanent
      • Meningitis
      • Sigmoid sinus thrombosis – extension of the septic thrombosis posteriorly can cause blockage of the arachnoid granulations and subsequent otogenic hydrocephalus.
      • Other central nervous system thromboses – cavernous sinus, petrous sinus
      • Osteomyelitis of the skull
    • Cardiovascular system
      • Peripheral thromboses – usually from extension downward from the central nervous system – internal jugular vein, subclavian vein, superior vena cava
      • Carotid artery – abscess, arteritis, spasms
    • Other
      • Bezold abscess – pus in the sternocleidomastoid muscle
      • Facial nerve paralysis
      • Death

    Questions for Further Discussion
    1. What duration of antibiotics is recommended for mastoiditis?
    2. How commonly does mastoiditis recur?
    3. When should a child have an evaluation for a possible immunodeficiency after having mastoiditis?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for this topic: Ear Infections

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    Rotch, TM. Pediatrics. The Hygenic and Medical Treatment of Children. J.B. Lippincott Co., Philadelphia, PA. 1896:1106.

    House H. Acute otitis media. A comparative study of the results obtain in therapy before and after the introduction of the sulfonamide compounds. Arch. Otolaryngol. Head Neck Surg. 1946:43;371-78.

    Redaelli de Zinis LO, Gasparotti R, Campovecchi C, Annibale G, Barezzani MG. Internal jugular vein thrombosis associated with acute mastoiditis in a pediatric age. Otol Neurotol. 2006 Oct;27(7):937-44.

    Spratley J, Silveira H, Alvarez I, Pais-Clemente M. Acute mastoiditis in children: review of the current status. Int J Pediatr Otorhinolaryngol. 2000 Nov 30;56(1):33-40.

    Go C, Bernstein JM, de Jong AL, Sulek M, Friedman EM. Intracranial complications of acute mastoiditis. Int J Pediatr Otorhinolaryngol. 2000 Apr 15;52(2):143-8.

    Brook I, Donaldson, JD.Mastoiditis. eMedicine. Available from the Internet at http://emedicine.medscape.com/article/966099-overview (rev. 07/30/2008, cited 2/11/2009).

    Ferguson A. Campus Benefactors: Simon Guggenheim. Colorado School of Mines “The OreDigger Newspaper”. Available from the Internet at http://media.www.oredigger.net/media/storage/paper1162/news/2009/02/09/Features/Campus.Benefactors.Simon.Guggenheim-3618459.shtml (rev. 2/9/09, cited 2/11/09).

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    4. Patient management plans are developed and carried out.
    7. All medical and invasive procedures considered essential for the area of practice are competently performed.
    9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Systems Based Practice
    26. Partnering with health care managers and health care providers to assess, coordinate, and improve health care and how these activities can affect system performance are known.

    Author
    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

    Steve Randall, MD, MPH.
    Pediatric Resident, University of Iowa Children’s Hospital.

  • How Common Is Pediatric Ocular Trauma?

    Patient Presentation
    During his inpatient rotation, a pediatric resident assisted in caring for two patients with traumatic ocular injuries.
    The first patient was a 3-year-old male who was playing “dress-up” with his sisters. A plastic hair barrette broke and caused a penetrating right globe injury.
    The local emergency medical technicians placed an eye shield and he was transported to the local emergency room. He was then transferred to a local children’s hospital for ophthalmological care where he underwent surgery for a prolapse of the right iris.

    The second patient was a 5-year old male who was a restrained passenger in the front seat of a car involved in a motor vehicle accident. He was hit in the face by the air bag and sustained injuries to both eyes.
    The left eye had a detached retina that required vitrectomy, lensectomy and retinal reattachment.

    The diagnosis of ocular trauma was easily made in both cases. The resident used the Internet and PubMed to review literature on the epidemiology of pediatric ocular injuries and the American Academy of Pediatrics recommendations for prevention of these injuries.

    The first patient’s clinical course showed him to have some amblyopia and minor decreased vision at 8 months after injury.

    The second patient’s clinical course showed him to be able to count fingers with his left eye and had normal vision in his right eye. He required several other surgeries. Both patients were wearing safety glasses daily to prevent additional injuries.

    Discussion
    Ocular trauma is unfortunately a common problem. Eighty percent involve contusions to the external ocular tissues or are nonperforating anterior segment trauma.

    Below are some indications for ophthalmological consultation and notes about treatment and prognosis.

    • Anterior segment injuries (cornea, anterior chamber, iris, and lens)
      • Foreign bodies or abrasions of the cornea – usually do well, consult ophthalmology if unable to remove object or patient has suspected retained object or has non-healed abrasion after 24 hours
      • Eyelid laceration – any laceration felt to possibly compromise the lacrimal system or involve the eyelid margin should be evaluated by ophthalmology
      • Periocular ecchymosis and edema (i.e. “black eye”) – patient should be evaluated by ophthalmology if unable to exam fully because of edema, or there was significant forceful injury as there may be a concurrent posterior segment injury
      • Chemical burns – all should be evaluated by ophthalmology
      • Fractures – commonly involve the floor and medial bones, all suspected fractures should be evaluated because of possible concurrent posterior segment injury and muscle or nerve entrapment
      • Hyphema – all suspected hyphemas should be evaluated by ophthalmology, most problems occur because of glaucoma caused by the red blood cells clogging the anterior chamber outflow tract
      • Traumatic cataracts – all should be evaluated by ophthalmology
      • Traumatic iritis – all should be evaluated by ophthalmology
    • Posterior segment injuries (i.e. vitreous body, retina, optic nerve) – all should be evaluated by ophthalmology
      • Optic nerve – presents as edema and decreased vision, outcomes are variable
      • Ruptured globe – needs prompt treatment, if in doubt, DO NOT TOUCH, cover eye with an eye shield (1/2 paper cup taped to patient) and call ophthalmology immediately.
        Visual outcome is generally poor

      • Retinal detachment – visual outcome is generally poor even with prompt treatment. This can occur in patients with significant trauma but can appear to have only anterior segment injuries.
      • Post traumatic endopthalmalitis (i.e. intraocular infection) – wounds with organic material tend to be worse, visual outcome is generally poor

    Learning Point
    Over 2 million eye injuries occur annually in the United States. Between 35-50% occur in the pediatric age group with injuries occurring more in boys than girls (2-3:1). Adolescent males are the highest risk group. Traumatic events reported were due to projectiles (17%), blunt objects (14%), fingers/fists/other body parts (12%), and sharp objects (10%). Motor vehicle crashes accounted for 5% of injuries with 35% of patients reportedly not wearing seat belts, 25% reportedly wearing seat belts and 40% where seat belt use was unknown. Overall the prognosis for traumatic eye injuries was felt to be good by ophthalmologists who reported that 73% of the patients who sustained eye injuries were expected to fully recover, and 12% would experience mild impairment.

    One author noted, “As children are sometimes unpredictable, and often exhibit naive behavior, their injuries can be caused by activity that is seemingly harmless to adults.” This would bear out in the case of the boy with the hair barrettes. Other injuries are more predictable. Some of the most serious eye injuries are found with use of all-terrain vehicles, paintball injuries and fireworks. Airbag deployment is known to cause injuries to children including death. Young children should not be positioned in the car near airbags. Additionally sports injuries, particularly basketball and baseball are common.

    The American Academy of Pediatrics (AAP) does not recommend use of all-terrain vehicles until a child has a driver’s license and has other specific recommendations for their proper use. The AAP states that all non-powder guns (i.e. air rifles, bb guns, pellet runs and paintball guns) are weapons and should not be characterized as toys. Weapon use is not recommended, but if used, protective eyewear is recommended. Both the AAP and American Academy of Ophthalmology “strongly recommend protective eyewear for all participants in sports in which there is risk of eye injury.”

    Questions for Further Discussion
    1. What questions should be asked of all athletes regarding vision protection?
    2. What are the American Academy of Pediatrics recommendations for child safety seat and seat belt usage?
    3. What resources are available in your community for children with visual impairments?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for these topics: Eye Injuries and Vision Impairment and Blindness.

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    American Academy of Pediatrics Committee on Sports Medicine and Fitness. Protective Eyewear for Young Athletes. Available from the Internet at http://aappolicy.aappublications.org/cgi/content/full/pediatrics;113/3/619 (rev. 3/3/2004, cited 1/28/09).

    American Academy of Ophtlamology. 2007 Eye Injury Snapshot Project Results. Available from the Internet at http://www.aao.org/practice_mgmt/eyesmart/snapshot_2007_results.cfm (cited 1/28/09).

    Salvin JH. Systematic approach to pediatric ocular trauma. Curr Opin Ophthalmol. 2007 Sep;18(5):366-72.

    Upshaw JE, Brenkert TE, Losek JD. Ocular foreign bodies in children. Pediatr Emerg Care. 2008 Jun;24(6):409-14; quiz 415-7.

    American Academy of Pediatrics. Policy Statements. Available from the Internet at http://aappolicy.aappublications.org/policy_statement/index.dtl (cited 1/28/2009).

    ACGME Competencies Highlighted by Case

  • Patient Care
    4. Patient management plans are developed and carried out.
    6. Information technology to support patient care decisions and patient education is used.
    7. All medical and invasive procedures considered essential for the area of practice are competently performed.
    8. Health care services aimed at preventing health problems or maintaining health are provided.
    9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    12. Evidence from scientific studies related to the patients’ health problems is located, appraised and assimilated.
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.
    14. Knowledge of study designs and statistical methods to appraisal clinical studies and other information on diagnostic and therapeutic effectiveness is applied.
    15. Information technology to manage information, access on-line medical information and support the healthcare professional’s own education is used.

  • Systems Based Practice
    23. Differing types of medical practice and delivery systems including methods of controlling health care costs and allocating resources are known.
    25. Quality patient care and assisting patients in dealing with system complexities is advocated.

    Author
    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

  • What Health Problems Are More Common in Homeless Children?

    Patient Presentation
    A family was encountered by a pediatrician at a local daycare center that provided temporary and emergency childcare for families who were homeless, involved with domestic violence or the foster care system. The family had moved from another state after they had lost jobs and were living in a shelter. There were two adult sisters, one of whom was pregnant, one sister’s husband, and 4 children under age 5. One adult had secured employment and the other two were searching for work and permanent housing. The one mother stated, “It’s so nice that you have that table of free clothes. We need so much.”

    The diagnosis of a homeless family with multiple needs was made. The pediatrician inquired to the center director how the center interacted with other agencies to help support families. The center director discussed how they worked with the state’s human services department, the homeless shelter, the domestic violence program, several local food programs, early childhood education programs and school district, clothing agencies and local programs for free or reduced cost for medical care. The center director noted that she had helped the one sister obtain prenatal care on the second day the children had come to the center, and the children were seen in a clinic down the street within a week of coming to the center. The center director also explained that the childcare center was able to function itself through the generosity of a local church that provided the space for the center, local food programs that provided meals and snacks, and agencies that provided transportation between the shelters and domestic violence programs.

    Discussion
    The United States Federal definition of a person considered homeless is one who “lacks a fixed, regular, and adequate night-time residence; and… has a primary night time residency that is: (A) a supervised publicly or privately operated shelter designed to provide temporary living accommodations… (B) an institution that provides a temporary residence for individuals intended to be institutionalized, or (C) a public or private place not designed for, or ordinarily used as, a regular sleeping accommodation for human beings.” About 3.5 million people of which are 1.35 million children are likely to experience homelessness in a given year. Note that homelessness is not permanent but persons are likely to move into and out of a homeless state over time. The average period of homelessness is 10 months and 25% of children are homeless more than once during childhood. Children < 18 years accounted for 39% of the homeless population with 42% of these children being under the age of five in 2003. Unaccompanied minors (including runaways) were 5% of the urban homeless. Rural populations have an increased risk of homelessness than urban populations. Families with children are among the fastest growing homeless populations, with a 2007 study, finding families with children comprising 23% of the homeless population in urban areas.

    As much as 25% of homeless persons are employed but are not making adequate income. One study stated that “…in the median state a minimum-wage worker would have to work 89 hours each week to afford a two-bedroom apartment at 30% of his or her income, which is the federal definition of affordable housing.” Racial and ethnic variations in homelessness depend on geographical location. Other groups with higher rates of homelessness include domestic violence victims, persons with mental illness or addiction disorders and veterans.

    Opportunities to assess adequate housing during a medical encounter include:

    • Patient registration – confirming an address, noting numerous changes in address or other contact information or insurance, and single mother headed households
    • Reviewing immunization records – having no records, inadequate records, or multiple sources of immunization administration
    • Interactions with health care professionals – asking about situations that may influence the doctor or nursing plans such as family stress, problems with travel, a sick relative, a housing change, etc. This can be done when the patient is placed into the room and at other points of contact such as immunization administration.
      • Reviewing and re-taking social histories may give clues including history of frequent changes of child care, schools or employment, and having little family, friends or other support available.
      • Responding to parental communication such as inquiring about sample medication availability, coverage of medication/health services by insurance, requests for supplies such as diapers, formula, snacks or food, or requests for transportation vouchers, etc.

    Learning Point
    Children who are homeless or inadequately housed have higher rates of many health problems. These rates are beyond those of children who live in poverty but are adequately housed.
    Children who are homeless experience a higher rate of:

    • Overall health is poorer – homeless children have more health problems, more severe health problems and more multiple health problems.
    • Child abuse and neglect – domestic violence is common among homeless mothers and families. Children are often witnesses to abuse or are victimized themselves. Child abuse investigation prevalence with homeless children is 24-35%. Children are often witnesses to abuse or are victimized.
    • Dental – poor dentitia and caries
    • Developmental delays – speech and language is the most common delay in younger children. Any developmental area can be affected though. School age children have more problems with verbal skills, vocabulary and reading. They also have more grade repetition and overall below-average school performance.
    • Mental health and behavior problems – especially depression
    • Immunization – under- and over-immunization for age occurs because of inconsistent health care
    • Infant mortality
    • Infectious disease
      • Diarrhea
      • Ear infections
      • HIV and AIDS
      • Lice
      • Scabies
      • Sinusitis
      • Wound and skin infections
      • Upper respiratory tract infections
    • Injuries – shelters and temporary housing are less structured and generally less safe so injuries are common
    • Lead – although there is a small amount of data, multiple risk factors make homeless children a high-risk group
    • Nutrition – obesity, malnutrition, growth stunting and anemia
    • Pregnancy
    • Respiratory diseases – asthma, bronchitis, pneumonia
    • Substance abuse
    • Visual acuity problems

    Questions for Further Discussion
    1. What resources are available locally to assist for homeless children and families?
    2. How does your practice assist homeless families so they can receive adequate healthcare?
    3. How can you adapt your practice to provide improved care for homeless families?
    4. How can health care providers advocate for their homeless families?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for this topic: Homeless Health Concerns

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    Karr C, Kline S. Homeless children: what every clinician should know. Pediatr Rev. 2004 Jul;25(7):235-41.

    American Academy of Pediatrics Policy Statement. Providing Care For Immigrant, Homeless, and Migrant Children. Pediatrics Vol. 115 No. 4 April 2005, pp. 1095-1100.

    Grant R, Shapiro A, Joseph S, Goldsmith S, Rigual-Lynch L, Redlener I. The health of homeless children revisited. Adv Pediatr. 2007;54:173-87.

    National Coalition for the Homeless. How Many People Experience Homelessness? Available from the Internet at http://www.nationalhomeless.org/publications/facts/How_Many.html (rev. June 2008, cited 2/5/09).

    National Coalition for the Homeless. Who is Homeless? Available from the Internet at http://www.nationalhomeless.org/publications/facts/who.html (rev. June 2008, cited 2/5/09).

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

  • Practice Based Learning and Improvement
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

  • Interpersonal and Communication Skills
    19. The health professional works effectively with others as a member or leader of a health care team or other professional group.

  • Professionalism
    20. Respect, compassion, and integrity; a responsiveness to the needs of patients and society that supercedes self-interest; accountability to patients, society, and the profession; and a commitment to excellence and on-going professional development are demonstrated.
    21. A commitment to ethical principles pertaining to provision or withholding of clinical care, confidentiality of patient information, informed consent, and business practices are demonstrated.
    22. Sensitivity and responsiveness to patients’ culture, age, gender, and disabilities are demonstrated.

  • Systems Based Practice
    23. Differing types of medical practice and delivery systems including methods of controlling health care costs and allocating resources are known.
    25. Quality patient care and assisting patients in dealing with system complexities is advocated.
    26. Partnering with health care managers and health care providers to assess, coordinate, and improve health care and how these activities can affect system performance are known.

    Author
    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital