Practicing Medicine in Bush Alaska—Some ABCs

From Guide to YKHC Medical Practices
Revision as of 10:56, 27 June 2019 by JenniferH (talk | contribs)
(diff) ← Older revision | Latest revision (diff) | Newer revision → (diff)
Jump to navigation Jump to search

Animal Bites

With a preponderance of dogs on the Delta, you may be faced with many bites. The important issues concern the fear of rabies and the risk of infection. Any unprovoked attack, or an attack by an unknown animal or unimmunized animal, should be considered for treatment for rabies. All bites should be reported to OEH. (543-6420)

There are “Rabies Investigation Report Forms” that should be filled out – (all the health aides and ER have) and there is always an OEH person on call if you have questions. (The operator keeps the list.) If the bite is severe it may need to come to Bethel. The rabies vaccine series may need to be given. The animal often needs to be caught and the brain sent for studies. The animal is usually quarantined for 10 days for observation before the decision to send the brain for rabies is made.

  • Local care of any dog bite includes washing with soap and water.
  • Fox bites are especially rabies prone.
  • The patient should receive a tetanus shot if they haven’t had one within 5 years.
  • High-risk
  • Bulleted list item
  • wounds probably require prophylaxis, but this is controversial. Oral flora usually complicate the wound and treatment should be considered for:
    • any wound showing signs of infection
    • any would that is difficult to clean
    • any wound in a critical area (face, hand, etc.).
  • Organisms usually include
    • Streptococci
    • Eikenella Corrodens*
    • S. Aureus
    • Bacteroids
    • Peptococcus
    • Petostreptococci
    • Pasteurella (14-50% dogs, 50-75% cats)

Penicillin resistant gram-negative rods are infrequent pathogens. *Eikenella is unusual in that it is often sensitive to PCN and Ampicillin, but not to oxacillin/methecillin/nafcillin/clindamicin.

Amoxicillin/clavulanate would be the best choice for prophylaxis if it were needed. for prophylaxis if it were needed.

Resources:

Botulism

Three types of Botulism

  1. Food (preformed toxin ingested) -- The main topic here
  2. Wound (organism grown in wound and forms toxin)
  3. Infantile (intestinal tract organism with formation of toxin)

Background

Botulism is very common here due to the fermented food that is considered a delicacy. Fish heads will be buried in the tundra for weeks and then dug up and eaten. If they were covered with Saran wrap or in a plastic container – they may be contaminated with botulism. Also seal oil that is sealed tightly is another risky food.

Botulism is a gram-positive anaerobic bacillus that can exist as a spore and resist killing. The toxin it releases interferes with neurotransmission at the peripheral cholinergic synapses preventing acetylcholine release. Fortunately, the toxin, which causes food poisoning, can be killed with boiling for 10 minutes or maintaining T+80c for 30 minutes.

Because lab tests for botulism take several days – the initial diagnosis depends on rapid clinical assessment. The incubation period is usually 12-36 hours.

Classic diagnostic pentad for botulism symptoms

  • Diplopia -blurry vision – due to eye dilation,
  • Dysphagia
  • Dilated Fixed Pupils
  • Dry Throat or mouth
  • Nausea or Vomiting

The three major areas of clinical symptoms are gastrointestinal, neurological, and muscular:

  • GI: Nausea/vomiting, ileus, diarrhea early, constipation late, and dry mouth.
  • Neurologic: symptoms may follow the ingestion by 3 days and include dry mouth, blurry vision, diplopia, dilated or unreactive pupils, dysphagia, decreased gag reflex.
  • Muscular: Symmetrical skeletal muscle weakness, respiratory muscle paralysis, fatigue, dyspnea

Possible contaminated foods need to be sent to the public health department in Anchorage. As soon as a case of botulism is suspected, the State Epidemiology Lab, Public Health and YKHC’s Office of Environmental Health (OEH) need to be notified IMMEDIATELY. This is considered a public health emergency and therefore contacting people after hours and activating appropriate resources is essential.

Treatment

  • Observation: the patients are followed with observation and Q 1 hour monitoring of their FVC.
  • Equine produced antitoxin: will prevent further deterioration, but not resolve the symptoms. Because of our relatively high incidence of botulism here in the Yukon-Kuskokwim Delta region, we have several antitoxin kits available in the pharmacy and additional kits can be gold-streaked out from Anchorage from their stockpile if there is a large outbreak (many members of the same family ate the contaminated food, for example). There is a Botulism step-by-step protocol located in the ER as this is where most of the cases are sent once they are recognized. The protocol has very specific directions that accompany the antitoxin. Read them closely and consult with Anchorage CDC to see if it should be initiated. The instructions also include very specific directions for blood to be drawn PRIOR to administration of the antitoxin.
  • Pre-emptive intubation and ventilation management should be considered if FVC falls to less than 80% of predicted or is diminishing over time in those with chronic lung disease. Please consult with an experienced provider when considering the management of botulism.

Resources:

Bronchiolitis/Wheezing

Wheezing is a common complaint on radio traffic. In infants, wheezing is most likely due to bronchiolitis or asthma. Listen carefully for a history of recurrent wheezing in the past as a clue toward asthma. For infants presenting for the first time or with associated stridor, think foreign body. Wheezing in older children should not be diagnosed as “bronchitis” as this is not a disease seen in children. Older wheezing children have asthma, a viral process or pneumonia. Be tuned in to a reported past history that points to undiagnosed bronchiectasis (productive cough greater than 3 months). These children should be seen in Pediatric Clinic for an evaluation.
Pathophysiology:
Inheritance:
Demographics:
Signs/Symptoms:
Diagnosis:
Management:
Critical Times for Affected Patients:
Resources:

Bronchiectasis/Chronic Cough

Pathophysiology:
Inheritance:
Demographics:
Signs/Symptoms:
Diagnosis:
Management:
Critical Times for Affected Patients:
Resources:

Carntitine Palmitoyl Transferase, Type 1A Arctic Variant (CPT1A Acrtic Variant)

(Adapted from Pocket Guide to Alaska Native Pediatric Diagnoses)
Pathophysiology: Fatty acid oxidation disorder: difficulty breaking down fatty acids from both food and body fat.
Inheritance: Autosomal recessive
Demographics:

  • Considered to be the wild-type (normal) gene in the Yu'pik population in Alaska (50% are homozygous for the Arctic Variant)
  • Total incidence per year in newborns in Alaska =7%
  • Found at a higher rate in all circumpolar coastline populations including Inuit populations in Canada and Greenland and indigenous populations of northern Siberia
  • General population = <1/1,000,000 (general CPT1A deficiency)

Signs/Symptoms:

  • Most children never have symptoms, but if they do, symptoms are more likely to be seen in children <2 years old
  • Initial signs of metabolic crisis: sleepiness, irritability, poor appetite
  • Metabolic crisis: Hypoglycemia (hypoketotic), seizures due to hypoglycemia, Death (especially associated with a concomitant infectious disease, although rare)

Diagnosis: Alaska Newborn Screen (processed in Oregon) - added to screen in the fall of 2003.
Management:

  • Avoid fasting states
  • When healthy, children with CPT1A Arctic Variant should eat like any other child their age
  • When sick, if infants and toddlers are unable to tolerate glucose-containing fluids (Pedialyte, juice, sports drinks) or food for more than 6 to 8 hours, they should see a health care provider immediately to consider IV or NG glucose-containing fluids.
  • Children with CPT1A Arctic Variant who are NPO on IV fluids should always be on dextrose containing fluids (D5-NS or D5-1/2NS). A normal maintenance rate is all that is needed.

Critical Times for Affected Patients:

  • Fasting or illness during first 2 years of life
  • fever, moderate/severe infection, dehydration, Surgery

Resources:

Cellulitis -- Community acquired MRSA Abscesses

There is a tremendous amount of cellulitis and abscesses in the YK Delta. We have a lot of community acquired MRSA infections here, probably from the lack of running water in many of our villages. I and D is our first line treatment for all boils. Many of the health aides can I and D simple abscesses – but small children and complicated ones we have sent to Bethel – to be seen in clinics or ER. Please culture all abscesses when you do an I&D so we can get a sensitivity on the organism!!!!!! That is the only way we know what we are treating. We have two great cellulitis and abscess guidelines (one for outpatient evaluation and treatment and one for severe) – please refer to them. If the erythema is over 10x10 cm, antibiotics are recommended after I&D. First line is Septra then Doxycycline for po treatment.

If the cellulitis is huge – they may be started on IV meds. As the resistance to fluorquinolones is increasing – we have been using mostly IV Vancomycin. If the patients are stable we may have adult patients come back Q12 hours to get their antibiotics on an outpatient basis (pediatric patients must be admitted for IV treatment). If the cellulitis doesn’t seem to be resolving, they will be admitted. Lower extremity cellulitis should have a low threshold for admission.

Resources:

Congenital Adrenal Hyperplasia (CAH)

(Adapted from Pocket Guide to Alaska Native Pediatric Diagnoses)
Pathophysiology: Inherited disorders of adrenal steroidogenesis resulting from deficiency in 1 of 5 enzymes necessary for normal cortisol synthesis. 21-hydroxylase deficiency accounts for 90% of CAH.
Inheritance: Autosomal recessive
Demographics: Yupik population = 1:280 live births. General = 1:15,000 live births
Signs/Symptoms: (Initial Presentation)

  • Newborn - Ambiguous genitalia (females, classic)
  • 1-2 weeks old - Adrenal crisis (males, classic salt-losing/wasting):Failure to thrive, dehydration, hyponatremia, hyperkalemia
  • 2-4 years old - Early virilization with pubic hair, growth spurt, adult body odor (males, classic non-salt-losing/wasting)
  • School age - Hirsutism, menstrual irregularity, early pubarche, sexual precocity (non-classic, school age children)

Diagnosis:

  • Newborn Screen - looks for high levels of 17-OH-progesterone seen in classic CAH
  • If NBS (+) or family history of CAH, check levels of 17-OH-progesterone (by mass spectroscopy) and electrolytes
  • If any concerns for CAH, consult YKHC Pediatricians who will help coordinate assessment and management with Pediatric Endocrinology at ANMC

Management:

  • Refer to Pediatric Endocrinology
  • If genital ambiguity and non-palpable gonads, run diagnostic tests and then treat empirically in discussion with Pediatric Endocrinology. Draw blood for diagnostic tests before starting treatment
  • Treatment: Hydrocortisone, Fludrocortisone, Sodium Chloride
  • Adrenal Crisis management
    • call YKHC Pediatrics on call if any concerns who will co-manage with Pediatric Endocrinology
    • Fluids (NS 20mL/kg bolus then D5NS or D10NS at 1.5xmaintenance)
    • monitor glucose and electrolytes
    • stress-dose hydrocortisone (IV or IM)
      • <3y/o: 25mg bolus followed by 30mg/day
      • 3-12y/o: 50mg bolus followed by 60mg/day
      • >12y/o: 100mg bolus followed by 100mg/day
      • (also listed in Raven note from Pediatric Endocrinology titled "Sick Day Plan")

Critical Times for Affected Patients:
Any time that could trigger adrenal crisis (hypotension, hyponetremia, hyperkalemia, metabolic acidosis, hypoglycemia)

  • first 1-4 weeks of life (prior to diagnosis or starting treatment)
  • when ill or severely stressed (infectious diseases, surgical procedures, etc)

Resources:

Congenital Sucrase-Isomaltase Deficiency (CSID)

(adapted from Pocket Guide to Alaska Native Pediatric Diagnoses)
Pathophysiology: Lack intestinal brush border enzyme to breakdown di- and oligosaccharides including sucrose & isomaltose
Inheritance: Autosomal recessive (potential mild form in carriers)
Demographics:

  • likely 3-10% of Alaska Natives (exact numbers not known)
  • 3% of Canadian Inuit (28.5% are carriers
  • 5-10% of Greenland Inuit
  • 0.2% of European-descended North Americans

Signs/Symptoms:

  • Onset usually around the time that solid foods are introduced.
  • Watery osmotic diarrhea when fed sucrose-containing food (breast milk and many infant formulas have only lactose), abdominal pain/distention, failure to thrive, malnutrition.

Diagnosis: Genetic Screen (blood), run at University of Washington:

Management:

  • Dietary Modification: Avoid sucrose, isomaltose and maltose (corn syrup is sucrose)
  • Enzyme Replacement: Sucraid (sucrose digestion only), costs ~$2000/month, not covered by Alaska Medicaid at this time.

Critical Times for Affected Patients:

  • Consider first exposed to sucrose (some formulas; most often when starting solids around 6m/o)
  • Consider this diagnosis when you have an infant or toddler with chronic diarrhea who has recently started solids or transitioned from breast milk or formula to whole milk and other foods.

Resources:

Fever in infant <90 days

We follow the generally accepted policy that infants under 3 months of age with a fever of 100.4° or greater should be evaluated in Bethel for severe bacterial infection and sepsis work up as appropriate. The CHA’s are not allowed to give infants less than 3 months of age antibiotics in the village without a physician’s order, and these infants should be evaluated in Bethel before being started on any antibiotic therapy.

Infants with fevers 100.4° or greater that have a normal exam and are clinically stable do not need to be medevaced, but they do need to come to Bethel on the first plane in the morning.

Infants with fever who appear ill, have respiratory distress, or are toxic in appearance should have a medevac arranged, and IM ceftriaxone should be given in the village if any delay is expected. A blood culture should be obtained prior to Ceftriaxone if the patient is in a SRC. Our guideline on management of fever in infants better details our usual management style.

If you have questions upon starting at YKHC, feel free to consult a pediatrician.

Resources:

Fish Finger

(AKA Seal Finger, Spaek Finger, Speck Finger, Spekk Finger, and Blubber Finger)

Fish finger is an infection that develops after handling fish, fish products, seal, or walrus. It is considered an occupational hazard for wildlife and marine workers and aquarium personnel. It may also occur from handling items exposed to the above such as slime covered nets, knives, etc. In the Delta, the infection may be noted on any extremity that comes into contact with aquatic creatures. Patients may describe both arm and leg exposure to animals such as walruses, ie when kneeling on a carcass while butchering. Fingers, however, are the primary area involved. A complete history is warranted when investigating the presentation of a painful, red, swollen digit. Patients may insisted they have used intact gloves with butchering, yet on later history they reveal gutting lake trout without gloves.

The infection is characterized by an incubation time ranging from hours to 3 – 4 days. Infection may occur in initially intact tissue, although history of seal or fish bite, knife cut, etc may precede infection onset. Fish finger develops rapidly, with severe pain, intense swelling, and often adjacent joint involvement. Ascending lymphangitis and adenopathy may be seen. Without treatment, the joint symptoms may progress for months or years, leading to cellulitis, tenosynovitis, and/or arthritis. Pathological examination has revealed neither necrosis nor abscess formation. Prior to antibiotics, sealers were known to opt for amputation because of the pain and disability resulting from infection.

The causative organism is unknown. Three different types of mycoplasm are suspect. Unfortunately these cannot be appreciated on routine culture. Other organisms that may cause similar infection from saltwater organism exposure include erysipelothris rhusiopathiae and vibrio vulnificus. This differential is important because while vibrio and mycoplasm infection are treatable with tetracyclines, erysipeloid infection is treated with penicillins.

With a straightforward presentation of a dull red. hot, swollen, very painful finger with a rapid onset and clear history of fish/walrus/seal exposure, fish finger is suspect and doxycycline is the drug of choice. This will cover the common sources of infection. Erysipelothris is suspect, however, is there is not a good response to doxycycline, if the erythema is intense, and if the infection spreads peripherally with violaceous color and distinct raised borders. Erisipelothris is a serious infection with risk of bacteremia and endocarditis, thus is important to keep in the differential. Erisipeloid may be cultured as well.

It is wise to keep village patients with fish finger in town for a recheck the following day, in order to observe response to doxycycline. If they return to the village without a recheck, there is the possibility that they will be “weathered in” and be unable to promptly return to Bethel should treatment failure occur. This places them at risk of bacteremia and endocardititis should they be infected with the more serious erysipeloid.

Frostbite

Frostbite is, for obvious reasons, a common problem. If someone is in a village with frostbite and there is a chance of refreezing - DO NOT THAW. If you can guarantee the affected extremity can be kept warm and at body temperature (95-100 degree) a bath can be used to rewarm the affected area. If it’s just blisters, they usually stay in the village with local (pun intended) care there. If you elect to transport the patient in, don’t let the affected area get bumped on the way.

Once here, the patient may need daily whirlpools, they will need NSAIDS and avoidance of all pressure to the area. Bear in mind that the longer you wait and treat conservatively, the less tissue damage will occur. A foot that initially looks bad enough that all the toes may be lost will often do much better than expected. (A toe saved, a toe earned - be patient and do no harm). Both clear and hemorrhagic blisters often occur-the books will tell to unroof the hemorrhagic blisters; practical experience shows that keeping them intact until they break on their own is better. Debride as needed to prevent bacterial trapping. Of note, aloe gel is helpful as a dressing. We also have very experienced physical therapists skilled in wound care. Use these resources.

Warn patients that paresthesias are common and they have a high risk of re-injuring the area in future exposures. Avoid all tobacco to increase blood flow.

Update Tetanus vaccination.

Resources:

H. pylori

Pathophysiology:
Inheritance:
Demographics:
Signs/Symptoms:
Diagnosis:
Management:
Critical Times for Affected Patients:
Resources:

Haemophilus influenza type a (Hia)

Pathophysiology:
Inheritance:
Demographics:
Signs/Symptoms:
Diagnosis:
Management:
Critical Times for Affected Patients:
Resources:

Hepatits B

Hepatitis B is common here with the probable major mode of transmission being sexual or close contact. There is a high carrier rate and the “Hepatitis B” program does an excellent job in following AFPs and LFTs to screen for hepatomas bi-annually in these carriers. Children not vaccinated at birth and all Alaskans and health care workers working in Alaska should receive the three part vaccination. Pregnant women who are carriers need to have their HepBeAg tested. If it is positive the child at birth should get the Hep B immunization as well as the immunoglobulin. If the mother is just HepBsAg positive – they do not get the immunoglobulin. If the mother is just HepBsAg positive—they do not get the immunoglobulin—Epidemiology per our State recommendations.

Resources:

HLA-B27

This is frequent in this population with all of its associations (Reiters syndrome, Rheumatoid arthritis, and spondyloarthropathies are much more common here. We manage patients with rheumatic arthritis on Methotrexate with the assistance of ANMC specialist. Remember the labs that need to be done Q 1-2 months – kidney, liver, and blood count – CBC and Comp Chem.

Kuskokwim Syndrome (Arthrogryposis-like syndrome)

(Adapted from Pocket Guide to Alaska Native Pediatric Diagnoses)
Pathophysiology: Mutation in the FKBP10 gene resulting in the impaired collagen cross-linking and disorganization of collagen molecules causing congenital joint contractures
Inheritance: Autosomal recessive
Demographics:

  • rare, incidence unknown
  • Found only in the Yupik population in the Bethel Area

Signs/Symptoms:

  • range and severity of contractures varies greatly
  • contractures are generally present at birth, worsen during childhood, then stabilize
  • often contractures of large joints, especially knees and elbows
  • other joints may also be involved, especially in lower extremities
  • milder skeletal features are common including
    • Spine: scoliosis, lordosis, spondylolisthesis
    • Feet: bunions (hallux valgus), flat feet (plano valgus), club feet (talipes equinovarus)

Diagnosis: Genetic testing - discuss with YKHC On-call Pediatrician
Management:

  • refer to Pediatric Orthopedics
  • Bracing and surgical correction of lower extremity contractures to allow ambulation
  • Occupation Therapy and Physical Therapy to enhance upper limb movement for self-care and lower limb movement for ambulation

Resources:

Metachromatic Leukodystrophy (MLD)

(Adapted from Pocket Guide to Alaska Native Pediatric Diagnoses)
Pathophysiology: Lysosomal storage disease causing progressive demyelination of central and peripheral nervous sytem, also affecting kidneys and other visceral organs due to accumulation of cerebroside sulfate
Inheritance: Autosomal recessive
Demographics:

  • 1:2,500 in Navajo (closely related to Athabascan)
  • 1:40,000-1:100,000 in northern Europe and North America

Signs/Symptoms:

  • Children have normal development until onset of disease
  • Late infantile onset = 6m/o - 2y/o (up to 4y/o). regression of motor skills, gait difficulties, seizures, ataxia, hypotonia, extensor plantar responses, optic atrophy, fussiness/pain/distress - thought to be due to neuropathy or dystonia
  • Juvenile and adult onset => 4y/o. gait disturbance, ataxia, seizures, intellectual impairment, behavioral difficulties, upper motor neuron signs, peripheral neuropathy

Diagnosis:

  • Brain MRI: symmetric white matter lesions with periventricular predominance (early) and cortical atrophy (late)
  • Genetic testing for deficient ARSA (arylsulfatase A) gene activity
    • Option 1: blood draw 6-8mL green top (min 2mL) plus optional 1-2mL lavender top for DNA extraction if worried about aged specimens
    • Option 2: blood spots on PKU card

Management:

  • Consult YKHC on call Pediatrician and assign CPP status
  • refer to Pediatric Neurology
  • no curative treatment
  • Bone marrow transplant, gene therapy and hematopoietic stem cell transplant are all investigational with goal of slowing the disease course
  • Prognosis for late infantile and early juvenile onset is poor (death within 5 to 6 years)

Critical Times for Affected Patients:

  • Recognition of symptoms and accurate diagnosis

Resources:

Micro-aspiration (in Apparently Neurologically Typical Children)

(Adapted from Pocket Guide to Alaska Native Pediatric Diagnoses)
Pathophysiology: Unknown
Demographics: Unknown, but appears to be a significant number of otherwise normal children under age 3 from Western or Northern Alaska
Signs/Symptoms:

  • Overt signs/symptoms not always present
  • frequent cough
  • aspiration with feeds during URIs
  • frequent (or any) pneumonia, especially right upper lobe

Diagnosis: Clinical vs Video Fluoroscopic Swallow Study (VFSS) evaluation. The radiation exposure from VFSS as well as the cost of transporting patients to Anchorage are higher risk and cost than initially treating symptomatic patients
Management:

  • Slow-flow nipple. Change to Dr. Brown level 1 or Dr. Brown Preemie nipple to slow down flow rate. Ensure that families do not cut or alter nipples in any way
  • Thicken liquids to nectar thick (using Thick-it). If flow is too slow once feeds are thickened, try Dr. Brown's Level 2 or 3.
  • If still experiencing overt symptoms of aspiration or if no resolution of lung disease within 3 months of proactive thickening, child should be referred to ANMC for a VFSS or modified barium swallow study
  • Continue thickened liquids until the patient experiences 12 symptom free months, then gradually wean off thickener. If child does not tolerate wean, refer to ANMC for swallow evaluation.

Risks of Proactive Thickening

  • Child's swallow pattern may not change; child may still be aspirating but with reduced signs and symptoms
  • if above is true, child is now aspirating corn starch and additional sugar from thickener.
  • if still aspiration, the heavier consistency of thickened liquids make them more difficult to clear via spontaneous cough

Critical Times for Affected Patients: Respiratory illness in infancy
Resources:

Otitis Media/History of Mastoiditis

Lots and lots on the Delta. Please refer to our Otitis Media Guideline. If the TMs are red and have no mobility – then recommendation is to treat them. Our antibiotic recommendation include—first line high dose Amoxicillin, second line Augmentin, third line Omnicef, and lastly Rocephin x 3 days. If the child has a long history of ear infections – referral for audiology for hearing eval . Tube placement referral is very common – it is done in Anchorage.

Many people over the last few decades had mastoiditis and had mastoidectomies to treat it in our population. They should be seen by ENT every 1-–2 years to have their mastoid bowls cleaned out and examined.

In general, children should be direct referred to ENT for PE tube placement if they have recurrent (>3 in 6 months or 4 in a year) infections or persistent effusion >3 months (especially with hearing loss). You may also refer these patients to audiology if there is a question of hearing loss/speech delay secondary to recurring infections; this is another route to ENT care as the audiologists routinely do telemedicine consults with the ANMC ENT’s using TM photos. We no longer do antibiotic prophylaxis for recurrent OM’s, as this has not been shown to be effective. All infants fewer than 3 months of age that are diagnosed by a CHA to have otitis media should be seen and evaluated in Bethel before being started on any antibiotic.

Resources:

Pneumonia

We have lots of lower respiratory infections in this region. Children who otherwise appear well and have relatively normal vital signs may have large infiltrates on the CXR. We tend to obtain CXRs for all children with a chief complaint of fever and cough and treat if indicated.

We have 10x the Strep Pneumonia rate of infection, as the rest of the state – so we take additional precautions in our newborns with fever. We have 50x the RSV Bronchiolitis incidence – as the rest of the world. Breastfeeding has been the only positive thing to decrease a child’s risk at this time. We do give Synagis to our high-risk pre-termers or cardiac complicated children. Look at our pneumonia guideline for recommendations for work up and treatment.

Resources:

Septic Joint

Any children with a red, swollen joint with or without fever and refusal to use the limb should be evaluated in Bethel. Suspected toxic synovitis should also be evaluated here and not managed in the village. Some joints can be tapped in Bethel for evaluation; several of the family practitioners and ER providers are trained in aspiration of some joints, although hip joints and other complicated joint aspirations are usually done by ortho in Anchorage. You can always consult with other providers here or with ortho in Anchorage if you are uncertain of how to manage a patient.

Septo Optic Dysplasia

(Adapted from Pocket Guide to Alaska Native Pediatric Diagnoses)
Pathophysiology: Disorder of early brain development resulting in wide variation of findings including hypopplasia of optic nerve, agenesis of corpus callosum and septum pellucidum, and/or pituitary hypoplasia.
Inheritance: Usually sporadic; occasionally autosomal recessive
Demographics:

  • 1:10,000 live births
  • unknown, but anecdotally higher incidence for Alaska Native populations

Signs/Symptoms:

  • Hypoplasia of optic nerve = impaired vision (one or both eyes), nystagmus
  • abnormal midline brain structure formation (corpus callosum) = intellectual disability, other neurologic problems including seizures
  • Pituitary anomalies (hypoplasia, ectopia, etc.) = growth hormone deficiency (most common), pan-hypopituitarism (also possible, at risk for adrenal crisis, hypothyroidism, micropenis)
  • Occasionally can have seizures, developmental delay, abnormal movements

Diagnosis:

  • Brain and pituitary MRI - thinning of optic nerves & chiasm, absence of septum pellucidum, Agenesis of the corpus callosum, Pituitary hypoplasia or posterior pituitary ectopia
  • Ophthalmology exam
  • Endocrinology evaluation
  • can be suspected initially based on prenatal ultrasound

Management:

  • varies depending on individual
  • consult YKHC Peds on call and assign CPP status
  • refer to Pediatric Endocrinology for regular endocrine evaluations
  • refer to Ophthalmology
  • refer to Family Infant Toddler (FIT)
  • refer to Pediatric Neurology in setting of seizures and neurologic deficits

Critical Times for Affected Patients:

  • vary depending on individual
  • If hypopituitarism, times of stress (fasting, illness, surgery, trauma) are high risk as well as newborn period due to: ACTH/Cortisol deficiency => adrenal crisis in the first week of life (similar to CAH; does not show up on newborn screen); Thyroid deficiency (can show up on newborn screens as low T4); GH deficiency and ACTH deficiency => hypoglycemia

Resources:

Strep Pharyngitis

This is usually caused by a Group A (strep pyogenes), but can be caused by groups C and G. It usually occurs in children ages 5-10 with peak incidence in the first few years of school. The transmission is through direct contact via respiratory or nasal secretions. There can be food or water borne outbreaks and the incubation period is 2-4 days. We have a great deal of strep throat in the Delta as well as peritonsillar abscesses.

Clinical onset in older children and adults is abrupt onset of ST, HA, malaise and feverish.

The pharynx is usually red and edematous with hyperic/hyperplastic tonsils with white exudate, tender lymphadenopathy, and T>101. Symptoms usually last 3-5 days. It may develop into a peritonsillar abscess – with a enlarged asymmetrical tonsil – exquisitely tender. This may need to be drained by needle aspiration. Exudative pharyngitis in children less than 3 is rarely streptococcal. Type specific antibodies are seen in 4-8 weeks and protect against infection with organisms of the same M-type.

Treatment

Positive strep in a patient with no allergies can be treated with LA bicillin x 1 – with age appropriate dosing. If the family requests PO treatment – it is now recommended to give penicillin – 750mg po Q day x 10 days. You can use the daily dosing to increase compliance.

Evaluate for dehydration – as some of our severely ill patients require IV fluids – as they are so dehydrated due to decreased oral intake from the pain. If a peritonsillar abscess looks likely – it will need to be drained with needle aspiration – using hurricane spray for numbing. Ask for assistance with this as it can be tricky. Be aware that there can many complications from strep – and a review of common complications and management is important.

Tuberculosis

A large percentage of the elderly on the Delta have had active TB in the past. You’ll see a lot of abnormal x-rays and find a lot of +PPD’s recorded in charts. If a patient doesn’t know if or how they were treated for TB, you can often find a report under the “x-ray” section of the chart on old “TB screening” x-ray reports. The public health nurses may also have a record of the patient’s treatment. The RPMS health summary of ten indicates TB status as well.

The most important thing to remember about TB is to remember TB. You may see new “converters”, new disease in a previous converter, reactivation. Be suspicion is the key to diagnosis of. CXR’s should be done on all converters (to determine skin test + vs. disease) and any person with a +PPD and symptoms suspicious for disease. Sputum’s can be sent to the State labs in “cans” x 3 for AFB and culture and sensitivity.

Public Health Nursing does most of the screening, follow up, and other investigation. They provide skin testing, and school screening. MWF 1-3 is a good time for them. (907-543-2110) PPDs can also be placed in villages, or on outpatient units.

The hospital has several physicians designated at TB Control Officer (Drs Roll, Bowerman, Chyi and Mondesir) by virtue of their interest and special training - they do all of the prescribing of anti-tuberculosis meds. Please notify this resource.

A TB isolation designation should be used for any patient admitted to the hospital for ‘rule-out TB”. Questions about this can be directed to infection control nurse or physician.

Resources: