Children With Special Healthcare Needs (CSHCN)
Only 13–18% of children have special healthcare needs, but they are responsible for approximately 80% of pediatric healthcare costs. The complexity of their care often results in emergency department (ED) visits, and they are much more likely to be hospitalized as children without disabilities. In addition, approximately 1 in 1000 CSHCN requires technology-assistive care, and complications from these devices are often the reasons for ED visits. The family may also use the ED as the medial home, especially if there is no established relationship with a primary healthcare provider.
The ED physician must determine whether the chief complaint is a manifestation of the underlining diagnosis or a new acute problem. The patient may also have difficulty cooperating and communicating, adding to the challenge of making a diagnosis. In addition, there are over 800 congenital syndromes responsible for childhood disability, so no physician can be familiar with every aspect of a given disease. Moreover, obvious inexperience with a CSHCN may make a parent less trusting. It is important to understand the child’s functional status and abilities prior to the incident that brought them into the ED. Incorrect assumptions about the patient’s capabilities may obscure the physician’s clinical assessment.
Some CSHCN may also present with behavioral challenges that can cause examination, imaging, or simple diagnostic testing to be more challenging. The patient may be nonverbal, have a decreased ability to communicate, or be unable to cooperate. Therefore, attempt to communicate in a manner that is familiar to the patient, including the use of augmentative communication devices or sign language when relevant. Also, take steps to ease the stress for the child and family, such as reducing stimulation, using objects from home that may provide some comfort, and minimizing the number of staff that are interacting with the patient. If available, a child life specialist may be helpful. When behavioral modifications are not effective, the ED physician may have to use pharmacological methods in order to perform procedures safely.
Many CSHCN require an enterostomy tube because of oral motor problems, dysphagia, inadequate calorie intake, unpalatable medications, or severe aspiration. Tubes are classified as gastrostomy (GT), gastrojejunostomy (JT) or jejunostomy, depending upon the location of the distal end. Advise the parents to keep a detailed record of their child’s current tube size and type in order to facilitate any future replacements.
Dislodgement is the most common reason for an ED visit concerning a GT or JT. This may occur due to trauma, tension on the tube (intentional or unintentional), rupture of the balloon, or unintentional balloon deflation. A feeding tube may also malfunction due to deterioration over time or obstruction.
Dislodgement in the first four weeks after insertion is the most common complication of feeding tubes. A tract for the tube will begin to mature within 7–10 days, although it may not be complete for up to three months. However, if a tube dislodges within the first 2–4 weeks, there is a risk that the stomach may separate from the anterior abdominal wall, so that blindly reinserting a new feeding tube may result in placing it within the peritoneal cavity. Using aseptic technique, stent the site with a replacement tube, then consult the service that originally placed the tube, as there is considerable institutional variation as to when a tract is considered to be mature. Do not use the feeding tube at this point. Instead, place an NG tube while awaiting consultation.
If the dislodged tube was from an older stoma, immediately replace it with either a Foley catheter or an appropriate-sized replacement tube. Lubricate the distal end with petroleum jelly using aseptic technique. Reinsert the tube carefully to avoid placing it into the peritoneal cavity through a false tract. Always inflate the balloon with sterile water (not saline). Abdominal pain upon balloon inflation is an indication of improper position; remove the tube and retry.
If excessive time has elapsed prior to the family seeking medical attention, the stoma may have constricted. To avoid this, do not delay replacement while seeking the correct size replacement tube. If the proper-size feeding tube is unavailable, insert a Foley catheter to stent the stoma until a replacement can be obtained. If the stoma has partially closed and the original size gastrostomy tube or Foley does not fit, use a smaller tube, but do not use excessive force when attempting reinsertion.
After a GT has been reinserted, check for proper placement. To confirm, aspirate gastric fluid and test the pH (should be <5) and/or inject 10–15 mL of air while listening over the stomach for borborygmi. Otherwise, obtain an abdominal film after injecting 5–10 mL of water-soluble radiocontrast material to confirm the location of the tube. Apply an abdominal binder or a loose-fitting ace bandage wrap to secure the enterostomy tube flush against the stomach and reduce the risk of recurrent dislodgement.
A dislodged JT must be replaced by either interventional radiology or surgery, as there is a risk of subsequent ischemia and bowel necrosis.
Removing a GT
Generally, there is no reason to remove a GT in the ED. However, if it is necessary, use a 20 mL syringe to deflate the balloon prior to removing the tube. Alternatively, some tubes have an internal bumper. In order to determine whether there is an internal bumper or a balloon, inspect the outer ports. If the port is meant for inflating a balloon it is typically white and labeled with the volume of fluid needed to maintain the balloon. If the situation remains uncertain, use a syringe and aspirate the contents of the port.
Frequent complaints are redness or bleeding around the tube site due to leakage, tape sensitivity, infection, or granulation tissue. If the tube is not properly secured against the skin, the stoma may become widened over time and gastric contents can leak around the tube. It is important to determine whether leakage is caused from a defect in the tube itself, or from gastric contents refluxing around the tube. Shield the skin from leaking gastric contents with a plastic barrier (e.g., Tegaderm), or a barrier agent, such as sucralfate, hydrocolloid agents, or zinc oxide. Add an antacid to the feeding regimen to increase the pH of the leaking gastric contents, and refer the patient to the primary physician for further care.
Granulation formation around the tube is a common complaint. While these areas are usually painless and only rarely become infected or cause obstruction of the stoma, on occasion they may bleed. Apply warm saline compresses and cauterize with silver nitrate. Then apply a topical antibiotic cream and dress with sterile gauze. Alternatively, use a topical steroid ointment (e.g., triamcinolone), which may help reduce the redness and size of the granuloma.
Approximately 20% of patients will experience a local infection after placement of an enterostomy tube. Although most infections are minor, there is a small risk of progression to a necrotizing fasciitis. See Cellulitis (pp. 108–109) for the treatment of a local infection in a nontoxic-appearing patient.
Vomiting is common in a child with an enterostomy tube, although it is most often related to gastroesophageal reflux disease or dysphagia. However, there are several possible tube-related causes: tube occlusion, tube migration, and intestinal obstruction due to adhesions.
Gastrostomy tube occlusion may occur in up to 45% of patients, secondary to kinking or obstruction of the lumen by the accumulation of formula or medications. The usual complaint is an inability to flush the tube or infuse liquid into it. Attempt to flush the tube with sterile water using a 30 mL or larger syringe to avoid excessive pressure. Use 20–30 mL of warm water to unclog PEG tubes. If conservative measures fail, replace the gastrostomy tube or use a Foley catheter of equal size. However, newly placed gastrostomy tubes, as well as jejunostomy tubes, and nasoenteric tubes may be difficult to replace. In these cases, consult the appropriate subspecialist and do not remove the tube.
Although rare, it is possible for a GT to migrate. A traditional GT or JT (not a button) will have about 4–6 cm of tube outside the stoma, although this may vary based on the size of the patient. Compare the centimeter markings on the outside of the tube with what the parent has typically observed. If there is a discrepancy, gently pulling back on the tube until the balloon is against the abdominal wall may relieve an obstruction.
Buried bumper syndrome occurs when the internal bumper erodes through the stomach wall with subsequent re-epithelialization which covers or buries the bumper. Often this is a result of excess traction on the enterostomy tube. It usually presents as resistance to flow or vomiting and/or abdominal pain during feedings. Suspect a buried bumper if the tube cannot be freely rotated. Consult a surgeon or gastroenterologist to plan removal and replacement of the tube.
Tracheostomy tubes are sized according to three dimensions: the inner diameter, the outer diameter, and the length. Regardless of the brand, the inner diameter is consistent among manufacturers and is always imprinted on the flange. In contrast, outer diameters and lengths are not consistent and may or may not be printed on the tube. Discrepancy in the outer diameter may cause a replacement tube to not fit into the stoma, so it may be necessary to select a smaller-size tube. Therefore, before replacing a tracheostomy, always have a smaller-size back-up available in case the original size cannot be reinserted.
The proper tube diameter optimizes airway resistance and limits the risk of aspiration, without irritating the mucosa or damaging the airway wall. Typically, the outer diameter is less than two-thirds of the tracheal diameter and the end of the tube is >2 cm beyond the stoma, but no closer than 1–2 cm from the carina. To prevent esophageal obstruction, trachea-esophageal fistula, or a tracheo-innominate artery fistula, use a curved tube to ensure that the distal end is concentric and parallel to the trachea. The standard pediatric tracheostomy tube has a 15 mm connector at the proximal end that allows for a connection to a bag-mask or a ventilator. However, a metal tube will not have this adapter.
A tracheostomy tube may be cuffed or uncuffed. The limited indications for a cuffed tube include a patient with chronic aspiration or one who requires high positive pressure for ventilation. The patient cannot speak with the cuff inflated.
The tracheal lumen may be fenestrated or non-fenestrated. A fenestrated tracheostomy tube is usually reserved for a patient who is able to speak, but may require intermittent mechanical ventilation.
The most common causes of hypoxia are infection related, either tracheitis or pneumonia. However, dislodgment or obstruction also occur frequently and must be identified. A false tract is also a possibility, especially in tracheostomy tubes less than a week old or that have been recently changed by an inexperienced caregiver.
Any significant bleeding from the tracheostomy site requires an endoscopic evaluation of the airway. If the tip of the tracheostomy tube rests against the tracheal wall it may cause irritation, inflammation, and ulceration with bleeding. This tends to be more common in a patient receiving mechanical ventilation. If the erosion occurs on the anterior tracheal wall it has the potential to cause a hemorrhage of the innominate artery, which typically lies 9–12 rings below the cricoid cartilage. This is a potentially life-threatening event and a surgical emergency which presents with a pulsating tracheostomy tube, bleeding around the tracheostomy site, or massive hemoptysis. If an innominate artery hemorrhage is suspected, do not remove the tracheostomy, as it may be the only way to ensure an adequate airway. If the patient does have a cuffed tracheostomy, overinflating the cuff may help to tamponade the bleeding.
A patient with a tracheostomy, especially one requiring ventilatory support, is at high risk for infection involving the stoma and lower respiratory tract. A child with a neuromuscular disease is at particularly high risk. A tracheostomy is usually colonized with potential pathogens, including Pseudomonas aeruginosa, Streptococcus spp., Staphylococcus aureus, Haemophilus influenza, and Candida albicans. Signs of an acute infection include a change from the baseline respiratory status, fever, tachypnea, increased oxygen requirement, and changes in tracheal secretions, cough, or accessory muscle use. Minor bleeding may also be a sign of infection if accompanied by other symptoms.
A tracheoesophageal fistula can occur when the posterior wall of the trachea is exposed to chronic pressure from the tip of the tracheostomy. This is potentially life-threatening because of bacterial contamination of the tracheobronchial airway. Symptoms include more copious secretions, new or increased aspiration of food contents, dyspnea, cuff leak, or gastric distention. The diagnosis can be made with a CT scan or barium esophagography. Treatment is usually surgical.
This is the most frequent complication of a tracheostomy and can be caused by frictional trauma from the tube, inflammation from stasis of secretions, infection, poor tube position, or traumatic suction technique. Granulomas are most common just superior to the internal stoma site on the anterior tracheal wall, but may also be seen along the posterior tracheal wall. Up to 80% of pediatric tracheostomies develop suprastomal granulation tissue, but in the majority of cases they are small and asymptomatic and require no intervention. Large granulomas may cause bleeding and may delay decannulation or pose an obstruction for recannulation after accidental decannulation. Treat granulation tissue along the external stoma with silver nitrate.
Stenotic lesions are classified according to the anatomical site, including suprastomal, stomal, cuff, and at the tip of the cannula. Most patients with tracheal stenosis remain asymptomatic until the original tracheal lumen diameter is reduced by 50–75% or the actual diameter is <5 mm. Symptoms include cough, inability to clear secretions, and dyspnea.
Skin Breakdown and Pressure Injuries
Pressure injuries are common in a patient who is immobilized, wheelchair-dependent, or has insensate skin. Other predisposing factors are fecal or urinary incontinence, chronic steroid use, muscle atrophy, elevated tissue temperatures, chronic malnutrition, and improper transfer techniques. In addition, many CSHCN are malnourished due to oral motor control issues, predisposing them to pressure ulcers. The most frequently affected sites are bony prominences, such as the sacrum, greater trochanter, and ischial tuberosity, as well as the occipital region in a younger child. Ulcers can be further complicated by osteomyelitis and sepsis. Use the National Pressure Ulcer Advisory Panel Pressure Ulcer Staging System to assess skin breakdown (Table 27.1).
|I||Non-blanchable erythema of intact skin|
|II||Partial-thickness skin loss with exposed dermis|
|III||Full-thickness skin loss|
|IV||Full-thickness skin and tissue loss|
|Unstageable||Obscured full-thickness skin and tissue loss|
|Deep tissue injury||Persistent non-blanchable deep red, maroon, or purple discoloration|
When evaluating skin breakdown, document the anatomical location, pressure injury stage, length, width, depth, type of tissue present at the wound base and its color, presence of exudate (none, minimum, moderate, large amount), and odor. Note whether there is undermining (destruction beneath intact tissue) or tunneling at the wound base, which can create dead space and lead to abscess formation. Several factors may interfere with an accurate assessment, including the presence of either an eschar or copious necrotic material, although necrosis may begin deeper within the tissue at the bony prominence. Also, multiple ulcers within a limited area may be indicative of interconnecting fistula tracts. Areas of deep tissue and pressure injury may present as a dark purple/maroon discoloration, particularly over a bony prominence, and can be mistaken for a bruise. These are potentially serious injuries.
Clean the wound to remove the necrotic, devitalized tissue and exudate while minimizing trauma to the wound bed. First, irrigate with a 30–35 mL syringe attached to an 18 or 19 gauge needle (delivers 8 lb per square inch). Use normal saline, but do not add an antiseptic solution, which may be cytotoxic. Debridement is then necessary if there is a fibrinous exudate. Use autolytic debridement, which involves keeping the wound moist to allow the body to debride on its own (may use Medihoney), or enzymatic debridement with a topical medication containing collagenase (e.g., Santyl). Use calcium alginate, foam (Mepilex) or hydrogel dressing (Aquacel Ag or Kaltostat) (Table 27.2) for exudative wounds.
|Dressing type||Properties||Brand names|
|Calcium alginates||Fibers absorb exudates and convert into a gel, providing moisture for healing||Kaltostat|
|Absorbs moderate to heavy drainage||Aquacel Ag|
|Controls minor bleeding||(antimicrobial)|
|Provides a moist healing environment|
|Use under a dressing (ABD pad, gauze or transparent film)|
|Enzymatic debriding agents||Will chemically lyse necrotic tissue||Medihoney|
|Must use within moist environment||Santyl|
|Will chemically lyse necrotic tissue|
|Film dressing||Use on skin tears||Bio Occlusive|
|Use on wounds with little or no drainage||Tegaderm|
|Allows water and oxygen into wound, keeps bacteria out||Op-site|
|If too much exudate in wound it will interfere with evaporation and oxygen diffusion and cause maceration|
|Foam dressing||Absorbs moderate to heavy exudates||Mepilex Border|
|Hydrocolloid||Forms occlusive gel by interacting with wound barrier||Duoderm|
|Provides barrier to external contaminates|
|Use with low to moderate exudates|
|Provides autolytic debridement|
|Do not use with infected wounds|
|Hydrogel||Use with minimal to moderate exudates||Saf-gel|
|Promotes autolytic debridement of devitalized tissue/eschar|
|Maintains moist environment|
|May ease pain and inflammation|
Wound cultures are not useful, as most wounds above Stage I are colonized with bacteria. Arrange for daily cleansing and dressing changes to help control colonization as wound healing will not occur if the bacterial count is excessive. Give antibiotics only if there is a cellulitis (erythema and warmth) or the patient has signs of a systemic infection (fever, chills, toxicity). If a wound infection is suspected, consult a burn specialist or dermatologist to perform a wound biopsy or obtain fluid via needle aspiration. A bacterial count >105/gram of tissue indicates an infection. Prescribe topical mupirocin, bacitracin, or polymixin, all of which are effective against Gram-negative and Gram-positive organisms.
Wounds heal better in a moist environment, which enhances cell migration, granulation tissue formation, and white blood cell (WBC) effectiveness. Select a dressing (Table 27.2) which provides an appropriate environment while managing the amount of exudate that the wound is producing. Use these guidelines as a framework to provide temporary care measures until the patient can be evaluated by a wound management expert. All pressure injuries require close follow-up, as superficial-appearing wounds may reflect a deeper injury.
For Stage I and II injuries, use transparent film, hydrocolloid, or foam. For Stage III and IV wounds, use calcium alginate, foam, or hydrogel, but obtain surgical consultation to determine whether inpatient management is needed. When applying a dressing leave at least a 1–1½ inch margin around the wound. In addition, removing the weight load/pressure to the injured area is a priority. Limit the use of an orthotic device that may be causing pressure and refer the patient to the orthotist to modify or fabricate a new orthosis.
If the injury is in an area of continued pressure, use a hydrocolloid dressing (e.g., Duoderm) or foam dressing (Mepilex) to protect the skin. Review with the parents the proper techniques for preventing wounds, including recognizing potential skin hazards such as radiators, car heaters, hair dryers, and hot plates on laps that can cause burns. In the summer, warn parents about hot sidewalks, metal storm drains, and hot sand. Teach proper positioning, including weight shifts every 15–20 minutes for 30 seconds while sitting, and bed turns every two hours. Encourage the patient to participate in wound checks and arrange for follow-up with either the primary care provider or a local skin specialist (i.e., physiatrist, plastic surgeon) the next day.
Special Considerations in Patients With Spinal Cord-Related Conditions
Autonomic dysreflexia is a life-threatening syndrome characterized by excessive uncontrolled sympathetic output below the level of a spinal cord injury, particularly in a patient whose injury level is above T6. A noxious stimulus causes an afferent impulse along an intact spinal reflex mechanism below the level of injury, leading to hypertension. Above the level of lesion there is an excess of parasympathetic output that results in peripheral vasodilation and the symptoms that characterize this condition. The most common causes of autonomic dysreflexia include bladder distention, fecal impaction, pressure sores, infection (most commonly urinary tract infection [UTI]), ingrown toenails, fractures, hemorrhoids, heterotopic ossification, and hip dislocation. Less common triggers include menstruation, appendicitis, gallstones, urinary stones, delivery, syringomyelia, testicular torsion, deep vein thrombosis, and pulmonary emboli. Medications such as nasal decongestions, methylphenidate, or illicit drugs such as cocaine may also produce these symptoms.
The presenting symptoms include hypertension, pounding headache, sweating above the level of injury, bradycardia or tachycardia, piloerection, blurred vision, and anxiety. In the infant or young child there may be sleepiness or irritability. If unrecognized, the hypertensive episodes can lead to retinal hemorrhage, stroke, subarachnoid hemorrhages, seizures, and cardiac arrhythmias (including atrial fibrillation).
When evaluating a child or young adult with a spinal cord injury, it is important to recognize that resting blood pressure is lower due to decreased tone below the level of the injury. The median systolic blood pressure for a patient with a spinal cord injury is (90 + [age in years × 2]). A systolic blood pressure of 150 mmHg or 20–40 mmHg above baseline is consistent with autonomic dysreflexia.
Obtain IV access and place the patient on a cardiac monitor. Sitting the patient upright, with the legs dangling off the stretcher, will cause an orthostatic decrease in blood pressure. Loosen the patient’s clothing and remove any anti-embolism stockings or abdominal binder, if present. Catheterize the bladder, but first apply lidocaine jelly. If there is an indwelling catheter, check for obstruction and either irrigate it or replace it. If the blood pressure is labile, apply 2% nitropaste (nitroglycerin) ½–1 inch above the level of the injury. If the above measures do not decrease the blood pressure, add nifedipine (0.25–0.5 mg/kg, maximum 10 mg), using the bite and swallow method rather than sublingually, which can cause rebound hypotension. If symptoms persist, manually disimpact the bowel, but use lidocaine jelly first. Consult with a neurologist and admit the patient if symptoms persist and no precipitant is found that can be successfully addressed in the ED.
A patient with a spinal cord injury or spina bifida with resultant neurogenic bladder is at increased risk of developing a UTI due to incomplete voiding, elevated intravesical pressure, and catheter use. An indwelling catheter is the most important risk factor, while repeated antibiotic exposure increases the likelihood of resistant organisms.
For a patient with a neurogenic bladder, significant bacteriuria is defined as >102 CFU/mL for catheter specimens from a child with intermittent catheterization; >104 CFU/mL for clean void specimens from a catheter-free male using condom catheter devices; or any detectable growth from a patient with an indwelling catheter or from a suprapubic aspirate. The patient may reuse catheters, which may become colonized with bacteria and then yield confusing results. Therefore, use a clean catheter to obtain a culture specimen. If the patient has an indwelling catheter, replace it before collecting a specimen. Since chronic catheterization may cause pyuria in the absence of a UTI, use 50 WBC/hpf in an unspun urine to suggest a UTI.
Signs and symptoms suggestive of a UTI (pp. 692–693) include fever, pain over the bladder or kidney, urinary incontinence, increase in spasticity, cloudy urine with increased odor, malaise, lethargy, feelings of anxiety, or autonomic dysreflexia (see pp. 793–794).