“The Fatal Four”

Four major medical conditions that are more commonly seen among individuals with IDD can lead to serious medical complications: constipation, aspiration, dehydration, and seizures. They can prove to be fatal if not recognized timely and treated appropriately. Community-based primary care clinicians should be familiar with the ways that these common medical conditions present themselves in patients with IDD, in addition to recommended preventive measures. Table 1.2 contains an overview of “The Fatal Four,” and Table 1.3 provides a guide for considerations for each potentially fatal complication.

Table 1.2 “The Fatal Four” major medical conditions associated with IDD


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Table 1.3 Considerations based on the four potentially fatal complications

Constipation Aspiration Dehydration Seizures
Common presenting symptoms
  • Decreased bowel sounds
  • Vomiting
  • Abdominal bloating and rigidity
  • Fever
  • Seizures
  • Behavioral outburts
  • Coughing after swallowing foods or liquids
  • Recurrent pneumona
  • Reactive airway disease
  • Fever
  • Burping
  • Hoarseness
  • Decreased appetite
  • Shortness of breath
  • Increased or decreased respiratory rate
  • Cyanosis
  • Recurrent wheezing
  • Halitosis
  • Excessive sweating
  • Colored sputum
  • Hypotension
  • Dry mouth
  • Decreased skin turgor
  • Delayed capillary refill
  • Tachycardia
  • Seizures
  • Signs of circulatory or cardiovascular collapse
    • Low blood pressure
    • Shallow breathing
    • Weak pulse
    • Clammy skin
    • Cyanosis
    • Low urine output
    • Unconsciousness
  • Generalized convulsions
  • Loss of consciousness
  • Body stiffness and jerking
  • Deep sleeping following seizure activity
  • Involuntary, coordinated motions while conscious
    • Lip smacking
    • Fidgeting
    • Chewing
    • Other repetitive motions
Assessment findings
  • Fever
  • Anorexia
  • Vomiting
  • Pneumonia
  • Seizures
  • Decreased level of consciousness
  • Behavioral outburts
  • Rales or rhonci of lung fields
  • Decreased oxygen saturation
  • Tachycardia
  • Altered mental status due to an underlying illness
Refer to commonly presenting symptoms listed above. Refer to commonly presenting symptoms listed above.
Potential complications
  • Always consider the possibility of bowel obstruction
  • Potential for medication intoxication due to increased absorption
  • Death
  • Development of aspiration pneumonia or sepsis
  • Acute lung injurt
  • Development of acute respiratory distress syndrome
  • Respiratory arrest
  • Death
  • Alterations in electrolytes
  • Death
  • Bodily injury
  • Permanent neurologic damage
  • Death
  • Chemistry panel
  • CBC
  • Flat and upright radiograph of the abdomen
  • Useful tests that may not be well tolerated in patients with IDD:
    • Computed tomography scanning
    • MRI
    • Ultrasonography
  • CBC
  • Arterial blood gas
  • Blood cultures
  • Sputum cultures
  • Chest radiograph
  • Bronchoscopy
  • CT of chest
  • CBC
  • Chemistry profile
  • Urinalysis
  • Serum creatinine level
  • BUN
  • New onset of seizure activity
    • EEG
    • CBC
    • Stat glucose
    • CT or MRI of brain
    • CMP
  • Known seizure disorder without changes in seizure pattern/presentaion may not need diagnostic workup
  • A change in seizure pattern should warrant consideration of:
    • Constipation
    • Medication noncompliance
    • Shunt malfunction
    • Infection
    • Hypoglycemia
  • Maintaining an accurate log of seizure activity
  • Medications may be needed on a daily basis
    • Laxatives
    • Stool softeners
    • Suppositories
  • Manual modalities
    • Enemas
    • Disimpaction
  • Antibiotic therapy
  • Hospitalization and ventilation to support breathing in severe cases
  • Inpatient vs. outpatient treatment for pneumonia
    • Use CURB-65 calculator
  • Fluid and electrolyte replacement
  • Intravenous fluids for severe dehydration
  • Hospitalization for symptoms of circulatory collapse
  • Rule out precipitating factors to assist with determination of treatment
  • Consider early referral to neurologist
  • Consider AEDs once underlying precipitating factors have been ruled out
  • Consider maximizing an AED’s dosage and level before declaring the drug to be a failure
  • Remember slow induction and tapering of all AEDs
  • Consider a vagal nerve stimulator or other modalities if failure occurs with two to three different AEDs
  • Diet modifications
    • Increased fiber intake
    • Promotion of adequate fluid intake
  • Various agents:
    • Bulking agents
    • Softening agents
    • Osmotic agents
  • Feeding evaluation may help guide prevention plan
  • Inclined position
  • Thickened liquid diet
  • Placement of a percutaneous endoscopic gastronomy tube or a jejunostomy tube
  • Consider dietary consultation to assist with prevention plan that addresses fluid and nutritional needs
  • Remember adequate fluid intake in those with alternate feeding routes such as a gastronomy tube
  • Be aware of increased incidence for dehydration during the following:
    • Fever
    • Diarrhea
    • Elevated blood glucose
    • Vomiting
  • Most seizure disorders cannot be truly prevented
  • Effective management is key
    • AED compliance
    • Prevention of precipitation factors such as constipation or hypoglycemia
    • Early treatment of presenting infections
AED, antiepileptic drug; BUN, blood urea nitrogen; CBC, complete blood count; CMP, comprehensive metabolic panel; CT, computed tomography; EEG, electroencephalography; HPI, history of present illness; IDD, intellectual and developmental disabilities; MRI, magnetic resonance imaging.

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