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Respiratory Symptoms
And by and by Christopher Robin came to an end of things, and he was silent, and he sat there, looking out over the world, just wishing it wouldn't stop. —A.A. Milne
Of all symptoms that very ill children experience, perhaps the most dreaded and disturbing to patients, parents, and caregivers are pain and difficulty breathing. With regard to the latter, common respiratory difficulties encountered are related to a wide spectrum of conditions. These include illnesses that cause systemic problems such as weakness, anemia, or cystic fibrosis. Other times, localized problems such as airway abnormalities, swallowing problems, aspiration of secretions, or pneumonia may cause breathing difficulties. Metastases from cancer may cause widespread disease within the entire thorax, including multiple tumors, pleural effusions, pneumothorax, or airway compression. This list is by no means exhaustive, but gives some idea as to the variety of causes that can lead to disturbing respiratory symptoms in children, particularly toward the end of life. However, not all respiratory symptoms are necessarily progressive, and are sometimes transient as children recover from other life-threatening illnesses ( Fig. 32-1 ).
An infant receiving oxygen via nasal cannula.
(Provided courtesy of infant's parents.)
Apart from physical or mechanical derangements that lead to breathing problems, anxiety can also compound feelings of shortness of breath and/or difficulty swallowing. There is clearly a role for many specific skills of different health professionals and caregivers to maximize a patient's comfort. These include numerous psychological strategies, careful positioning of the patient, artificial ventilatory supports, and medications. Additionally, children require clear explanations for these distressing symptoms, and reassurance that we will work together to alleviate them.
Psychosocial disciplines such as child life specialists, social workers, spiritual care, and psychology are key to providing care to all of these children, who often have frequent and prolonged hospitalizations with chronic respiratory symptoms. Very often, children don't want to talk about it, whatever it is. And yet many will have fears, questions or concerns that go unaddressed unless we can find non-threatening ways to engage them in assuring their comprehension and expressing their thoughts and emotions about their health. Child life specialists develop trusting relationships with patients by use of normalizing activities as well as therapeutic interventions. This is extremely important for children who require mechanical support for assisted breathing, and helps combat some of the isolation that can accompany requiring such therapy. The normalizing activities include a variety of interactions, from going on walks to engagement through crafts and hobbies that diminish boredom and promote fun. They also encourage sustained connection between the patient and friends and family while children are hospitalized. Therapeutic interventions include art therapy, pre-operative teaching, medical play, diversion, and distraction.
This interdisciplinary approach to care becomes even more important over time, because as technology continues to develop more children who would have died because of their underlying illness now survive. In this context, some of the therapies used to assist with respiratory symptoms also extend life, but with residual respiratory symptoms or limitations lingering in the background. Examples of these therapies range from treating pneumonia with new generation antibiotics to receiving a lung transplant for cystic fibrosis. In other situations, children who cannot be cured of their disease might have their lives extended by months or years by noninvasive ventilation, such as facemask or nose cradle, or tracheostomy, with or without chronic mechanical ventilation. The number of children who live in the hospital or at home requiring respiratory support by biphasic positive airway pressure (BiPAP), continuous positive air pressure (CPAP) or continuous mechanical ventilation by way of tracheostomy has grown significantly in the past decade. Despite this, there are some who believe these procedures are underused in pediatric populations. In addition to the array of medical equipment and operative procedures that may alleviate respiratory symptoms, there are also many medical therapies that may be employed, which will be described later in more detail.
Even with the evolution of respiratory aids and treatments for children, there are still many respiratory symptoms that children experience, which adversely affect their quality of life. In the case of high tech treatment such as ventilation, a child's ability to return home is also adversely affected. This chapter will delve into the many respiratory symptoms that children may experience, as well as the interactive treatments that are offered by nurses, respiratory therapists, physicians, and psychosocial professionals to alleviate these symptoms. The ethical implications of some of these therapies will also be discussed.
Dyspnea
Dyspnea is “a distressful subjective sensation of uncomfortable breathing that may be caused by many disorders, including certain heart and respiratory conditions, strenuous exercise, or anxiety.” The differential diagnosis is vast, and the causes can perhaps best be organized by anatomic location. Dyspnea can result from:
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Fixed or intermittent airway obstruction such as congenital abnormalities of the airway, asthma, external compression, and/or invasion by tumor,
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Intrathoracic extra-parenchymal pathologies such as pneumothorax, hemothorax,
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Intrathoracic lung parenchyma abnormalities such as pneumonia, tumor, pulmonary hemorrhage, cystic fibrosis.
This list is by no means all-encompassing, but gives some idea as to the spectrum of conditions that can lead to dyspnea. Focusing on the underlying pathology and directing treatment to reversing the cause of dyspnea is possible in some cases. Modalities that target reversing or ameliorating the underlying pathology may include medications, surgery, and/or radiotherapy.
In terms of assessing dyspnea, two self-report tools could be located. One has been tested only in hospitalized patients with asthma, and the other has been limited to small focus groups of children with cystic fibrosis or asthma, compared to normal children. They were found to be reliable in children 6 or 8 years of age, respectively, but not younger. In the absence of any validated tool for dyspnea in other diseases, visual analogue scales (VAS) can also be used for children in these age ranges.
There are many studies evaluating both pharmacologic and non-drug treatment of this distressing problem. Results from meta-analyses in the Cochrane Database of systematic reviews are mixed:
“Studies showed that these interventions can help to relieve shortness of breath: vibration of patient's chest wall, electrical stimulation of leg muscles, walking aids and breathing training. There are mixed results for the use of acupuncture/acupressure. Further interventions identified were counseling and support, either alone or in combination with relaxation-breathing training, music, relaxation, a hand-held fan directed at a patient's face, case management, and psychotherapy. There are several non-drug methods available to relieve shortness of breath in incurable stages of cancer and other illnesses. There is currently not enough data to judge the evidence for these interventions. Most studies were conducted in participants with chronic lung disease. Only a few studies included participants with heart failure, cancer, or neurological disease.”
Many children anecdotally do respond positively to practicing deep, slow breathing, singing, or blowing bubbles or pinwheels when they are feeling anxious and short of breath. The use of self-hypnosis in children to manage dyspnea has also been found to be beneficial. This may be directed for younger children by a professional skilled in hypnotherapy. Pediatricians and others can learn to help their patients learn self-hypnosis, which gives the child more immediate and constant access to this form of therapy, through the Society for Developmental and Behavioral Pediatrics. Maintaining a calm and quiet environment is also important, and can be accentuated by use of machines that create light patterns on the ceiling, relaxation carts such a Snoezelen, and favorite music being played quietly.
As for drug treatment of dyspnea, the mainstay medications are opioids and benzodiazepines, titrated to minimal effective dose ( Table 32-1 ). There is sometimes an exaggerated concern on the part of health professionals and parents that initiating opioids to treat dyspnea may cause respiratory depression. Very often, the dose of opioid required for the treatment of dyspnea is a quarter to half that required for the treatment of pain. A recent Cochrane review did report benefits of opioid therapy in ameliorating breathlessness in patients due to both malignant and non-malignant disease. Although the number of studies was small, the results were significant when these medications were given via the enteral or parenteral route. Educating families and colleagues that judicious use of opioids have been found to be safe and beneficial often allays these fears. Also, opioids can sometimes be used transiently, while other therapies aimed at ameliorating the cause of the dyspnea can be employed and given time to take effect. In addition to systemic administration of opioids, there have been many studies evaluating the possible role of inhaled opioid agonists, the theory being that this might confer a direct benefit by stimulating mu receptors in the lungs themselves, thereby reducing systemic side effects such as pruritis, somnolence, and constipation. A recent meta-analysis reveals that this mode of therapy is not beneficial, even in dose ranges from 1 to 40 mg morphine equivalent, despite isolated case reports showing positive effects.
TABLE 32-1
Common Pharmacologic Management of Respiratory Symptoms in Children
| Symptom | Medication | Dosing | Comment |
|---|---|---|---|
| Dyspnea * † | Opioids: | ||
|
|
|
|
| Benzodiazepines: | |||
|
|
|
|
| Bronchospasm | Salbutamol | 2.5-5 mg/dose by nebulizer | Can cause jitteriness, increased heart rate. |
| Secretions | Anticholinergics ‡ : | ||
|
|
|
|
| Cough |
|
|
Or see dosing on over-the-counter formulations by age. |
† Any other potent opioid can be used at ¼ to ½ the dose ordinarily used for analgesia.
* Consider treating all identified underlying causes of dyspnea: antibiotics for infection, transfusion for anemia, drainage and/or pleurodesis or tunneled catheter for pneumothorax or pleural effusion, and radiotherapy for metastases.
‡ Watch for increased anticholinergic effects: urinary retention, constipation.
§ No longer recommended for simple URI children younger than 6 years old.
There is no meta-analysis that evaluates the use of benzodiazepines for relief of dyspnea, though the Cochrane Database has a published protocol for such a study. Other published clinical trials in adults have demonstrated that use of midazolam, in addition to morphine, can further diminish the sensation of dyspnea. There are no similar trials in children, but the combination of morphine or another opioid in conjunction with benzodiazepines are frequently used to treat this symptom in children despite the paucity of data for both children and adults. Occasionally, suffering from symptoms such as dyspnea becomes intractable, despite multiple combined treatments. In this event, many patients and/or families agree to a course of sedation as a last resort to provide comfort, although conscious awareness may be very diminished as a consequence. This treatment might be continued until death occurs, but certainly not always if other treatments such as radiotherapy alleviate the symptom over time in such a way that sedation can be lessened or discontinued. In one study, such sedation was eventually discontinued in a quarter of adult patients.
Other medications that may provide relief, depending on the underlying disease, are bronchodilators, inhaled steroids, or mucolytics. Many patients suffer from orthopnea, and occupational therapists and physiotherapists can often combine their skills to devise modifications to beds and seating such that the patient can maintain a comfortable position even during sleep. Other helpful therapies that may assist with dyspnea include use of a fan for increased air movement in the room. Patients experiencing dyspnea also often feel less symptomatic at an incline of 30 degrees to 90 degrees.
The role of oxygen has been controversial in the management of dyspnea. However, in a 2008 study, patients dying of metastatic lung disease and receiving oxygen therapy were no less dyspneic than those receiving only room air. However, oxygen therapy may have a symbolic role for families, particularly if their child has required oxygen frequently throughout his or her life. Therefore, many patients and families do request that oxygen be provided, particularly in home settings. Having oxygen to provide often seems to enhance patient and parental sense of control, and can be important from that point of view.
Cough
A very aggravating symptom is cough. Cough interferes with activities as basic as eating and sleeping, and also as far-reaching as social isolation that prevents one from attending concerts, movies, etc. Moreover, cough leads to fatigue, abdominal or chest pain, and even vomiting and rib fractures. Persistent cough is a serious problem that healthcare professionals sometimes minimize, perhaps because everyone has had a cough at one time or other. Again, medical treatment to this point is somewhat limited, given that mechanical factors such as secretions in the alveoli and bronchi, and irritation of the carina, are potent stimuli of the cough reflex. No meta-analysis looking at treatment of cough in palliative care could be located. Cough may respond to N-methyl-d-aspartate (NMDA) receptor antagonists, such as dextromethorphan. This is often available in low-dose formulations in over-the-counter cough preparations. There are no controlled trials in children evaluating its role in cough due to progressive respiratory illness. However, one study demonstrated more improvement in parent-report of cough compared with placebo when used for viral upper respiratory infection, though not as effective as ingesting honey. Some patients get a measure of relief from a relatively small dose of opioid. However, for those with a lesion in the bronchi, cough can be fairly intractable without more intense treatments, such as radiotherapy or surgery. With regard to radiotherapy, an area of lung or total lung can be targeted when there is diffuse parenchymal disease, but in the case of bronchial tumors, this can be administered by endobronchial brachytherapy. This is accomplished by delivering a radioactive treatment via an endoscopically placed catheter, which is left in place for a few minutes, then removed.
Secretions
Some children develop noisy breathing due to the movement of secretions in an uncontrolled airway as they lose consciousness prior to death. While there is no evidence of distress to the patient, this situation can be intolerable for parents and other caregivers. Alerting families to the possibility of this occurring is very helpful in terms of anticipatory guidance.
For children who have excessive or difficult-to-manage secretions, suction equipment is often the first line treatment for airway maintenance. In the home, portable suction is easily provided by respiratory equipment vendors, at relatively low cost, or through local home care programs.
When loss of airway control is caused by imminent death, and causes caregivers distress, it can be treated by a variety of anticholinergic medications, although the evidence for these strategies is quite weak. One noninvasive strategy is to use 10% atropine eyedrops sublingually, titrated to effect. Another is to use a scopolamine patch applied transdermally behind the patient's ear. Systemic medications such as glycopyrrolate can also be used, either enterally or parenterally, and this particular medication has the added advantage of not crossing the blood-brain barrier. These medications need to be carefully titrated such that secretions do not become too thick or tenacious. If this complication arises, secretions may become much harder to move within the airways, leading to the formations of large, solid mucous plugs, which can then worsen the child's respiratory symptoms. In these situations, families and caregivers also need to be alert to other anticholinergic symptoms, such as urinary retention, which sometimes requires indwelling urinary catheterization, dry mouth, and worsening constipation.
Pulmonary Hemorrhage
At the mild end of the spectrum, patients may have blood-tinged sputum, progressing to frank hemoptysis. On the other end, one might experience sudden life-threatening pulmonary hemorrhage. Bleeding from the airways is almost always a terrifying event for the patient, family, and healthcare professionals. Causes include a combination of pulmonary disorders exacerbated by bleeding diatheses. In the pediatric population, significant pulmonary hemorrhage commonly arises from complications of advanced lung disease, sepsis, or end-stage liver disease, but also in critically ill patients of any etiology who are mechanically ventilated. For patients in whom pulmonary bleeding might be anticipated, this possibility should be explained to the patient and/or parents and other care providers. In order to diminish the visual impact of bleeding, the patient should as much as possible wear darker clothing and be cared for using darker bed linens, towels, etc. In situations in which the patient is distressed, a dose of subcutaneous, intravenous, or intramuscular, midazolam can be given, titrated to a minimal level of sedation that allows comfort and reduces memory of the event. Initially, midazolam can be given as a bolus dose during the acute event, but if bleeding is recalcitrant, the patient may benefit from a continuous midazolam infusion, given the very short duration of effect from intermittent dosing.
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