Nurses play a vital role in preventing, recognizing, and treating pediatric oncology emergencies, which can happen at diagnosis, as a complication of therapy, or at disease progression or recurrence.
Pediatric oncology nurses—in constantly assessing their patients—are in a key position to recognize complications early, and providers depend on them to do so. The earlier a potential emergency is identified, the lower the risk for the child.
This article focuses on four common types of emergencies: superior vena cava syndrome, spinal cord compression, tumor lysis syndrome, and septic shock (the box on the next page shows additional types of pediatric emergencies).
Superior Vena Cava Syndrome
Superior vena cava syndrome (SVCS) refers to the symptoms that occur from compression or obstruction of the superior vena cava—and it is usually caused by either thrombosis or an anterior mediastinal mass. SVCS is most commonly caused by a mediastinal mass that is present at diagnosis or recurrence. Thrombosis, however, is not as predictable and can develop at any point during therapy, leaving patients at continued risk.
The most common symptoms of SVCS are cough, shortness of breath, wheezing, pleural effusions, pericardial effusion, chest pain, facial swelling, syncope, headache, orthopnea, hoarse voice, jugular venous distention, blood pressure changes, and respiratory arrest.1,2 Diagnosis is usually confirmed by a chest radiograph.
Treatment for SVCS, regardless of cause, starts with supportive care from the nurse: giving the patient supplemental oxygen (positioned to minimize obstruction), maintaining a calm environment, and admitting the patient to an Intensive Care Unit.
There is currently no established standard of care for mediastinal masses—but, treatment may include radiation therapy of 200 cGy given once or twice daily to reduce the size of the mass, which leads to decompression of the organs involved.2
Children with mediastinal masses caused by lymphoid malignancies may respond to corticosteroids without the requisite demand of radiation, but sarcomas typically do not respond as well. The use of intravenous methylprednisolone along with radiation therapy is an option in children with leukemia or lymphoma, as the steroid reduces radiation side effects such as edema.2
Finally, SVCS caused by thrombosis is best treated with low molecular weight heparin. If the clot is caused by an indwelling catheter such as a central line, the catheter may need to be removed.2
Spinal Cord Compression
Spinal cord compression is seen in 2.7 to 5 percent of children with cancer at diagnosis, progression, or recurrence of disease.3,4 Although spinal cord compression is not life threatening, it can cause irreversible neurologic damage if not recognized early and treated.
Spinal cord compression is a result of a mass or tumor invading the vertebrae and collapsing the spinal cord or causing increased pressure in the spinal canal.5 Compression of the vertebral venous plexus causes cord edema, venous hemorrhage, and ischemia.3,4
Spinal cord compression has been seen in almost every tumor type including leukemia and Wilms' tumor. The majority of cases, though, are caused by sarcomas that have metastasized to the spinal cord followed by neuroblastoma, germ cell tumors, lymphoma, and metastases of primary CNS tumors.4
Pediatric oncology nurses should act immediately if a child with cancer is complaining of back pain. Data show that 80 percent of those complaints are associated with spinal cord compression.2,4,5 The back pain is often worse at night or when a child lays flat.
Other symptoms of spinal cord compression include weakness, gait abnormalities, bowel and bladder dysfunction, numbness, tingling, and decreased ability to differentiate between dull and sharp pain. Once a neurologic deficit develops, paraplegia and quadriplegia can occur within hours or days and may be irreversible.3,4
Early diagnosis and treatment are essential to decrease the risk of permanent neurologic deficits. Oncology nurses should look for changes that may signal spinal cord compression during physical exams and regular monitoring, such as pain, motor weakness, and loss of sensitivity to touch. Diagnosis is normally made based on a complete neurologic exam and magnetic resonance imaging.
Treatment can include the use of high-dose steroids, emergent radiation, chemotherapy, and surgical decompression via laminectomy.2–5 And throughout treatment, the oncology nurse—in addition to providing medical care—helps educate the patient on how to cope with the diagnosis.5
Tumor Lysis Syndrome
Tumor lysis syndrome (TLS) is caused when tumor cells die and release potassium, phosphates, and nucleic acids into the bloodstream, which lead to hyperuricemia and hypocalcium. TLS is usually seen with acute lymphoblastic leukemia and high-grade lymphomas.
It can present prior to the start of chemotherapy, but is normally seen within six to 72 hours of starting therapy—and in some cases can occur up to seven days later.
Symptoms of TLS include decreased urine output, fatigue, muscle cramps/twitching, nausea, vomiting, diarrhea, respiratory distress, edema, hematuria, acute kidney injury, seizures, cardiac arrhythmias, neurologic changes, and death secondary to multi-organ failure.4–7
Signs that could predict children at high risk for developing TLS include: large tumor burden, rapidly growing malignancies, renal insufficiency, and dehydration.
Once a child is recognized as high risk, hydration becomes critical to prevent renal damage. A child should receive intravenous fluids without potassium. Children who are at low or intermediate risk should be given allopurinol prior to the start of chemotherapy to prevent the formation of uric acid. Those on allopurinol may also need sodium bicarbonate added to their fluids to alkalize their urine.
Children at high risk should also start rasburicase prior to the start of chemotherapy. Rasburicase starts breaking down uric acid within four hours, decreasing the risk of renal damage (and the need for dialysis). Children with G6PD deficiency should not receive rasburicase.6–10
The oncology nurse needs to closely monitor the child's intake, output, and weight. Ongoing assessment for edema, respiratory, neurologic and/or cardiac changes, and gastrointestinal complaints are crucial observations when caring for a child with TLS. Tumor lysis lab tests should be drawn as ordered—typically every four to six hours, or every eight to 12 hours, depending on risk factors and treatment.5, 6
Infection continues to be a major cause of morbidity and mortality in pediatric oncology patients (which can lead to septic shock in severe cases). Patients are at risk for infection because of impaired immune systems, and are at a higher risk if there is an indwelling catheter.
The risk of developing infection is greatest during periods of neutropenia. An estimated 10 to 40 percent of children with fever and neutropenia develop an infection.
Fever is the most common symptom and often the first sign of a severe infection that could lead to septic shock (which is life-threatening).2,10–12 Other symptoms include impaired tissue perfusion, immune dysregulation, inflammation, dysfunction in more than two organ systems, cardiovascular dysfunction, and suspected or proven infection.13
Recognizing septic shock early is imperative to decrease the risk of mortality. At the first sign of a child going into septic shock, rapid fluid boluses (20 ml/kg of isotonic crystalloid solution each) should be started immediately, and the oncology nurse should assess for perfusion after every bolus. Boluses should be given until tissue perfusion is restored and blood pressure is adequate.
Vasopressor agents may also be necessary to maintain blood pressure and perfusion. Intravenous broad-spectrum antibiotics should be started immediately after blood cultures are drawn, and should be fully infused within one hour of the start of symptoms. Data show that the mortality risk increases by eight percent for every hour delay in the start of antibiotics.13
The pediatric oncology nurse typically has the first opportunity among all medical personnel to recognize a child going into septic shock. Children at risk need to have their vital signs monitored continuously, as well as have ongoing physical assessment so the nurse can recognize subtle changes that may be signs of infection and septic shock.
Oncology nurses also must be aware that children can deteriorate physically before there are changes in vital signs.5
Oncology nurse need to be educated about these emergencies and know the initial steps to take when they occur. If a nurse has not been exposed to dealing with oncologic emergencies, he or she can shadow other nurses or providers who have experience handling oncologic emergencies to better prepare themselves.
In addition, obtaining certification in pediatric hematology/oncology offers further education on the topic.
JALANE THEIS, RN, MSN, CPNP, is a pediatric oncology nurse practitioner on the leukemia team at Texas Children's Cancer Center, where she also coordinates the Advance Practice Providers Fellowship as well as the care of oncology patients in the post-anesthesia care unit.
Common Pediatric Oncology Emergencies
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