26 Dermatologic Conditions and Symptom Control
Kimberly A. Bower, Julie Good, Anke Reineke, Cristina Gordon, and Michele R. Burdette-Taylor
Beauty is not in the face; beauty is light in the heart
kahlil gibran
Introduction
Skin disorders are often encountered in seriously ill patients and can have significant physical, emotional, social, and spiritual impact. The skin is the largest organ in the body and accounts for 15% of a person’s body weight. It provides protection from the external environment, allowing intimacy through the ability to touch and be touched. The skin is visible to the external world, thus strongly affecting appearance, which in turn impacts social interactions and self-image. Appearance also influences parents’ perception of their child’s health and well-being. For all of these reasons it is critical to use an interdisciplinary team to take a whole-person approach to the treatment of dermatologic conditions (Table 26.1).
Table 26.1 The roles of team members in the treatment of wounds
| Discipline | Role |
| Psychosocial clinician Psychologist Social worker | Identify that there is a wound or that there is emotional distress related to a wound Provide presence and support to the patient and family Assess the emotional impact of the wound on the child Assess the emotional impact of the wound on the child’s family members Provide therapy and counseling to the patient Provide therapy and counseling to the family Provide education and support in the patient’s school for the child’s classmates Provide activity therapies to the patient and family, such as art, music, and play |
| Child life specialist | Identify that there is a wound or that there is emotional distress related to a wound Provide presence and support to the patient and family Provide activity therapies to the patient and family, such as art, music, and play Prepare the patient for what to expect during a dressing change Provide distraction during a dressing change |
| Spiritual counselor | Identify that there is a wound or that there is emotional distress related to a wound Provide presence and support to the patient and family Assess whether the wound is causing spiritual distress to the patient Address the spiritual distress that may be caused by the wound Identify and address the spiritual needs of the patient’s family Perform spiritual rituals that may support the patient and family |
| Nurse | Identify that there is a wound or that there is emotional distress related to a wound Provide presence and support to the patient and family Assess the physical signs and symptoms associated with the wound Provide hands-on medical care including dressing changes, medication administration, and repositioning Educate the family about wound care and wound prevention Educate the school nurse about the patient’s medical and emotional needs |
| Certified nurses assistant | Identify that there is a wound or that there is emotional distress related to a wound Provide presence and support to the patient and family Provide repositioning and skin care |
| Physician | Identify that there is a wound or that there is emotional distress related to a wound Provide presence and support to the patient and family Assess the physical signs and symptoms associated with the wound Provide medical care including dressing changes, medication administration, and repositioning Prescribe a treatment plan |
| Psychiatrist | Identify that there is a wound or that there is emotional distress related to a wound Provide presence and support to the patient and family Assess the emotional impact of the wound on the child Assess the emotional impact of the wound on the child’s family members Make a psychiatric diagnosis such as anxiety disorder or depression Prescribe a treatment plan including medications |
| Wound care specialist | Identify that there is a wound or that there is emotional distress related to a wound Provide presence and support to the patient and family Make recommendations to the team about the best management plan to treat the wound Provide hands on medical care including dressing changes |
Clinical Vignette
Matt is a 13-year-old boy with hereditary sensory autonomic neuropathy type II. He presents as significantly younger than his stated age and academically is at a first-grade level. His ability to sense pain is very limited, and for this reason he has developed multiple wounds throughout his life. He has also been treated several times for osteomyelitis and has had his left leg amputated below the knee. He was recently admitted to the pediatric intensive care unit (PICU) for sepsis. He is wheelchair bound and incontinent of urine and stool. His appetite is poor and his albumin is 2.5. He has three large Stage III wounds on his coccyx. There is an unpleasant odor coming from the wounds. Matt is very self-conscious and is reluctant to have anyone other than his mother look at his wounds.
Matt lives with his mother, 11-year-old brother, and 9-year-old sister. The family is Catholic and has limited resources. Matt has not attended school for the past year because his mother believes that his wounds get worse when he is at school. He also does not leave the house very frequently because he is self-conscious.
An interdisciplinary care team consisting of a nurse, social worker, spiritual counselor, bereavement counselor, physician, psychiatrist, wound care nurse, and dietitian started working with Matt and his family. A treatment plan for the wounds was devised. The odor from the wounds resolved, and the wounds slowly began to decrease in size. The wound care nurse ensured that Matt had a pressure-relieving mattress on his bed and a pressure-relieving cushion for his wheelchair. The nurse did regular dressing changes while the social worker provided Matt with distraction and emotional support during the procedure. The nurse also educated his mother on wound care and prevention. The dietitian worked with the patient to identify ways to improve his nutritional status. The psychiatrist diagnosed Matt with depression and started him on an antidepressant medication. The social worker helped to identify resources for the family and provided emotional support to the patient and his siblings. The patient’s mother worked with a bereavement counselor to address her fear about the possibility of Matt dying prematurely, and the spiritual counselor provided prayer and addressed Matt’s mother’s questions about why God had made her son sick.
With support from the team Matt’s mood improved and he started to leave the house and go on outings more frequently. In fact, he became restless and started to look for any excuse to leave the house. The team started to work with Matt’s school on a plan to allow him to return to school.
Given Matt’s underlying medical condition, he will always have wounds and a risk of osteomyelitis, sepsis, and death. Matt’s medical condition will continue to affect him physically and emotionally. Exacerbation of his physical symptoms will likely cause new emotional and spiritual distress. Matt’s siblings will be significantly affected by his illness, and his mother will continue to benefit from help locating resources and from counseling and spiritual support. Matt’s story highlights the need for a family-centered interdisciplinary approach to the care of children with chronic medical conditions and wounds.
Psychosocial Impact of Dermatologic Conditions
Body Image
Body image is a central part of self-concept and self-esteem and is broadly defined as “the composite of thoughts, values, and feelings that one has for one’s physical and personal self at any given time.”1 Patients with obvious differences—or changes—in appearance report that they feel embarrassed, at times experiencing a “loss of self” that prevents them from connecting with their own identity.2 Children’s responses to their own appearance—whether “different” as a defining feature of a disorder or a significant change from their own “normal”—can be highly individual. Influencing factors include whether an affected body part or function is particularly emotionally laden for the child and whether there is visible disfigurement. As a result of physical changes in appearance, others may stare or avoid looking at a child. Thereafter, the child may suffer emotionally and may withdraw socially. Throughout the literature, studies have suggested that altered body image can interfere with daily functioning and has been associated with grief, anxiety, depression, social introversion, social avoidance behaviors, decline in self-esteem, avoidance of intimate relationships, and overall poor quality of life.3,4,5
Cancer is an example of a diagnosis in which both the disease and the treatment frequently lead to changes in physical appearance. Some effects are temporary (e.g., hair loss, presence of central venous catheter) and other can be permanent (e.g., skin changes due to chemotherapy, scars from surgeries). Such body-altering effects of cancer have been reported by adolescents as the worst aspects of living with the disease,6 especially when others tease or shun them, or engage in other hurtful social interactions. Physical changes due to cancer or its treatment can be devastating to the child’s self-image and can place the child at an increased risk for psychological adjustment issues.7,8,9 Physical appearance and attractiveness are major issues confronting adolescents, and their body image becomes a central aspect of identity development.10 The adolescents’ sense of self-worth can be affected to such a degree that they may withdraw from their friends and family. Older adolescents facing changes in body image may be less likely to establish intimate relationships.11
A skin disorder may similarly affect children’s sense of self-competence and self-worth in their relationships with family and friends. Young children rely primarily on their parents and siblings for support, whereas adolescents turn more to their peers, who are in turn influential in their self-definition and self-evaluation. If children or adolescents have diminished social contact in any of their relationships, this may have significant implications on their ability to cope with the skin disease and can have negative effects on their overall quality of life.
Epidermolysis bullosa (EB) is a rare disorder in which “the skin tears apart, blisters, and shears off leading to severe pain, disfigurement, and wounds that may never heal.” Negative body image in EB can be related to poor self-esteem, anxiety, and depression.12 In one study, psychiatric symptoms, particularly depression, anxiety, and behavioral disturbance, were experienced by 80% of patients with EB.13 It is important to keep in mind that all the psychological responses described may not be simply the result of the skin condition per se, but may be the child’s response to the overwhelming impact of living with a life-threatening illness.14
Family Reflection
Becky (mother of Clara, age 6, who has severe generalized EB simplex)
Pain
The experience of pain is a crucial consideration in the psychological impact of skin conditions and their treatments. A wound is a constant reminder to children of the traumatic changes that their body has undergone. Clinicians’ attentiveness and sensitivity to children’s responses during wound care are of paramount importance, as patients often link their depression and anxiety to the experience of pain.15,16 In children with EB, the pain is associated with frustration, embarrassment, sadness, anxiety, and fear of being hurt.17 When pain, skin breakdown, and scarring lead to limitation of motion and activity, even therapies with the goal of improving function become implicated in children’s overall distress.18 The critical contribution of expert consultation in pain and symptom management cannot be underestimated for these children.
Comfort of Touch and Intimacy
Regardless of the underlying medical diagnosis, intact skin allows for intimacy and the ability to touch and be touched—a powerful way for a child’s family to express their care. Any change in a child’s body or body image may compromise that physical closeness, either through the child’s own reactions or those of the family. Loss of this intimacy may in turn elicit psychological responses that become enduring and pathological, such as depression and social anxiety.
Impact on the Family
How the family, both parents and healthy siblings, respond to and cope with a child with a skin disorder is a new area of research. In patients with EB, reactions of family members to the condition have both direct impact on the child and, ultimately, on the family as a whole.13 Parents of children diagnosed with EB identified distress around themes including their own painful struggle to accept their child as visibly different from others, witnessing their child suffering from pain and sometimes causing the pain while taking care of the wounds, witnessing others’ reactions to the child’s appearance, feelings of uncertainty, restrictions in employment or leisure time, and guilt at not paying attention to other family members, including siblings.17 Parents occasionally mention feeling ashamed of their child’s appearance—a feeling that in turn engenders enormous guilt. Parental support is crucial and a protective component to improve resilience and lead to more positive psychosocial outcomes for their children.19,20
All too often, however, parents feel unprepared, even overwhelmed, with how to manage appearance-related issues.21 The impact and adjustment on healthy siblings of having a brother or sister with a serious illness and a visible disfigurement are yet not well understood. Although the presence of chronic illness may increase siblings’ distress, the adjustment can vary by age, sex, and type of illness.22 Clinical experience suggests that siblings’ reactions are especially complicated when their brother or sister’s illness is highly visible or disfiguring in its manifestation.
Intervention
Optimal care for a child’s dermatological condition—both medical and psychosocial aspects—ideally includes ongoing and specialized approaches (educational, psychological, and integrative), support for the children and families, and access to an interdisciplinary team. A powerful mediator of adjusting to the impact of a serious skin disorder is effective communication that is open, honest, acknowledges emotions, and actively involves the child, family and the care team.17 “Psychoeducational” approaches combine the transmission of critical information about the condition and treatment with extreme sensitivity to the impact on the child and family of receiving such information. The team can guide children in adjusting to differences or changes in their appearance while validating their emotional experience throughout the illness.
Early and ongoing psychological guidance for parents—regarding the care of both the patient and the healthy siblings—is invaluable. When available, individual psychotherapy (including play therapy for young children) and expressive therapies (e.g., art and music) permit children to safely explore their extraordinary life circumstances without being overwhelmed by a sense of vulnerability. When children feel validated in their concerns, they are more able to think through their situation and move toward some sense of mastery over the challenges. Family therapy can play a critical integrative role for all members in managing unremitting challenges and stress.
Encouraging and facilitating connections between patients and their peers (with others who are living with a similar condition and with healthy friends) provides a critical opportunity for them to share their experience and gain support.23 These interactions may be in person or telephone or through various media, including e-mail, texting, and social networking websites.
The care team can recommend and offer integrative modalities to provide relaxation and respite for children, as well as give them a certain sense of control over their symptoms. These include guided imagery, massage, healing touch, hypnosis, acupuncture, and aromatherapy, as well as other mind-body skills. Teaching parents how to implement selected techniques for their child can assuage their sense of helplessness and opens an important avenue of comfort for the child embedded within the family.
Greater attention to understanding the impact and efficacy of interventions for children with skin disease is needed to ease profound suffering and enhance their overall well-being. The integrative palliative team can play an important supportive role in enhancing quality of life for the child and family.
Pruritus
Pruritus is defined by the International Forum for the Study of Itch (IFSI) as a sensation in the skin that provokes the desire to scratch.24 Itch can be distressing and sometimes difficult to treat. It may lead to sleep disturbance, difficulty concentrating, anxiety, depression, and agitation. Left inadequately managed, it can have a significantly negative impact on a child and their families quality of life.25 Itch may impact physical function, impair social relationships and school performance26,27 and lead to self-consciousness and lower self-esteem.28 Parents may face financial burdens secondary to the cost of dermatologic care and poor reimbursement for some topical products, struggle with the time commitment for treatment, and experience difficulty meeting the needs of other family members including siblings while taking time to care for a child’s skin.29 The relationship between psychological and dermatological health may be bidirectional, and a comprehensive approach to management is recommended,27 including multidisciplinary treatment, education, and psychological support.30,31
The IFSI identifies six categories for the classification of pruritus based on underlying etiology: dermatological, systemic/neurogenic, neurological, psychological/psychosomatic, mixed, and other.32 Because the causes of pruritus are numerous, identifying the etiology of pruritus can be helpful when formulating a therapeutic approach. This section of the chapter focuses on common causes of pruritus in seriously ill children. The reader is further directed to the European S2K Guideline on Chronic Pruritus for additional general and disease specific recommendations.24
Pathophysiology of Pruritus
Our understanding of pruritus perception and transmission has progressed over the past decade, with promising treatment targets beginning to emerge while still others are in earlier stages of development.33 Overall, the neuronal pathways for the transmission of pruritus are thought to be closely related to pain pathways (Figure 26.1).34
Figure 26.1 Neuroanatomy of itch. Itch and pain transmission occurs via unmyelinated C nerve fibers that excite the lamina I subset of neurons in the dorsal horn of the spinal cord. Two subsets of pruritoceptive C-fiber neurons (which respond to histamine and cowhage, respectively) are conducted through distinct spinothalamic pathways, with projections to the thalamus. Processing of itch through either pathway activates several regions of the brain, which are similar to those involved in pain (see Figure 26.1). Concomitant painful stimuli can reduce the sensation of itch, possibly by a descending inhibitory mechanism resulting from activation of the periaqueductal gray matter.
(Adapted from Figure 5.1 Yosipovitch G, Akiyama, T. Pruritus 2018, In Bolognia, Schaffer MD, Cerroni MD editors: Dermatology, 4th ed., Elsevier34)
In chronic pruritus (itch that lasts >6 weeks), peripheral stimulation by a pruritogen has been shown to lead to nonhistaminergic/mechano-sensitive c-fiber and A-delta fiber pathway activation.35,36,37 Meanwhile, histaminergic/mechano-insensitive c-fibers play a lesser role in chronic itch but are still important for acute dermatological conditions like urticaria, insect bites/infestations, and phytodermatoses. These findings help to explain the observation that antihistamines are useful for some itch but are not helpful in others.38
Molecules currently known to mediate itch in the periphery include39,40,41,42
• Amines: Histamine, serotonin, proteases (e.g., kallikrein 5 and mucunian), cytokine-interleukins
• Phospholipid metabolites: Leukotriene, endocannabinoids
After initial stimulation, G-protein coupled receptors (e.g., transient receptor vallinoid cation channel subfamily V1 [TRPV1], transient receptor potential ankyrin 1 [TRPA1], and voltage-gated sodium channels) depolarize the nerve, leading to sodium influx and potentiation of signal from the periphery to the spinal cord.43,44
At the spinal cord level, sensory neurons release gastrin-releasing peptide to activate gastrin-peptide–specific dorsal horn neuron receptors45 that subsequently carry the itch signal via the spinal cord to the thalamus. Functional magnetic resonance imaging (fMRI) shows activity in multiple areas of the brain which encode sensory location and intensity, emotion, attention regulation, cognitive/evaluative, motor control, and goal-directed movement aspects of itch.46
Itch of the neurological classification arises secondary to damage of nerves anywhere along the afferent pathway. Nerve damage can be seen in peripheral neuropathies, nerve compression, or cerebral processes such as tumors, abscess, or thrombosis. Itch caused by psychiatric and/or psychosomatic disease can occur in disorders such as obsessive-compulsive disorder and parasitophobia. It is also believed that both acute and chronic stress can trigger or enhance pruritus.47
Overview of Proposed Treatments
Itch may be inhibited by peripheral, spinal, and/or supraspinal mechanisms. These mechanisms include gamma aminobutyric acid (GABA), glycine, dynorphin, endocannabinoid, norepinephrine, and serotonin and mechanical (scratch) inhibition.48
Systemic mediators of itch responses that are under investigation for specific clinical utility in chronic pruritus syndromes39,40,41,49,50,51 include
• Neurokinin 1 (NK-1) antagonists: Aprepitant, serlopitant, tradipitant, orvepitant, fosaprepitant
• Kappa-agonists: Nalfurafine, pentazocine, nalbuphine, difelikefalin
• Mu-opioid receptor antagonists: Naloxone, naltrexone, nalbuphine
• Interleukin antagonists: Cyclosporine, nemolizumab dipilumab, secukinumab, ixekizumab
• Janus kinase (JAK) inhibitors: Tofacitinib, others
• First- and second-generation histamine receptor antagonists: Bilastine, rupatidine, others
• Leukotriene receptor antagonists: Montelukast
• Bile acid transporter inhibitors
Topical and local therapies under investigation for itch52 include
• Transient receptor potential vanilloid 1 (TRPV1) inhibitors: Capsaicin, tacrolimus
• Tropomyosin-receptor-kinase A (trka) inhibitors: Pegcantratinib
• Transient receptor potential melastin 8: Menthol creams
• Proteinase-activated receptor 2 (PAR-2) inhibitor: Polidocanol
• Phosphodiesterase-4 (PDE-4) inhibitors: Crisaborole
• Topical analgesics: Ketamine, amitriptyline, lidocaine
• Endocannabinoids: N-palmitoylethanolamine, N-acetyl ethanolamine
Assessment
Among seriously ill children the most likely etiologies of pruritus include underlying medical disorders, drug therapy, and dry skin. The etiology of pruritus can frequently be determined by history and physical exam. History should include localized versus generalized location, description, temporal profile, provoking factors, effect of medications, current medication list, atopic history, and travel history. Physical exam should include evaluation for dry skin, scabies, icteric conjunctivae, weight loss, and mental status changes. Radiologic and laboratory workup can be considered based on clinical presentation and goals of care.
Validated, universal clinical assessment tools that quantify the severity of pruritus and impact on quality of life are needed in adults and children.53 The pediatric tools available assess global quality of life (QOL) for children with skin disease54 or assess itch severity but are designed to be used in specific conditions, such as post-burn pruritus55 or cholestatic itch.56 Several existing tools designed to measure disease severity and QOL for children with atopic dermatitis need further validation.57,58 In children older than 6 years, visual analog scales may be used to evaluate itch intensity.59 In the research setting, quantitative measures of pruritus can be obtained by devices that assess motor movement at the wrist (actigraphy) or by accelerometers that measure the vibrations of the fingernails in the act of scratching, although relationship between motor movements and subjective experience is influenced by multiple factors.60
Management of Specific Conditions
The most effective treatment for pruritus is treating the underlying cause of the itch. This is not always possible, and even when it is possible the itch often does not resolve immediately. For this reason it is important to identify treatments specifically aimed at quickly and effectively relieving itch to the extent possible regardless of the state of the patient’s underlying condition. In this section, we review management strategies for pruritus that are caused by conditions frequently seen in seriously ill children (Table 26.2).
Table 26.2 Treatment of pruritus by cause
| Cause | Treatment |
| General measures for all forms of pruritus (including prevention and management of xerosis) | Insure air is humidified Keep ambient temperature cool and avoid rapid temperature changes in environment Avoid very (thermally) hot, and spicy foods Avoid emotional stressors i.e. extreme excitement, strain Learn relaxation techniques Select coping tools/distracting behaviors to engage in when itch is severe Maintain hydration of body as a whole (enteral, or parenteral if necessary) Wear breathable cotton in layers Bathe in cool/lukewarm bath, add one or more of the following to the bath: sodium bicarbonate, oatmeal, potassium permanganate Select low pH cleaners and moisturizers Use oil-based emollients (avoid- water or alcohol-based) Avoid fragrances, any irritating ingredients specific to your child Keep fingernails short Wear gloves or mittens if scratching is subconscious/occurring at night (remember to allow babies and toddlers time out of gloves to explore sensory world each day) |
| Opioid-induced | Opioid rotation Add analgesic adjuvants to allow opioid dose to be lowest possible Opioid receptor antagonist Opioid receptor agonist-antagonist Mixed results with: 5-HT 3 receptor antagonists, D2 receptor antagonists, nonsteroidal anti-inflammatory agents, lidocaine, subhypnotic propofol, midazolam |
| Cancer-specific | Anticancer therapy H 2 receptor antagonist cimetidine in Hodgkin’s lymphoma and polycythemia vera Paroxetine Mirtazapine Possibly: Ondansetron, corticosteroids, H1 antihistamines, gabapentinoids, thalidomide, naloxone, butorphanol |
| Cholestasis | If extrahepatic: Cholestyramine, bile acids If intrahepatic and obstructed: Phenobarbital Any cause: • Liver transplantation in severe, refractory cases depending on goals of care |
| Uremia | Enhance dialysis regimen Correct calcium, phosphorus, and magnesium Treat hyperparathyroidism Ultraviolet (UV-B) light therapy Cromolyn sodium Gabapentin Mu-opioid receptor antagonist naltrexone Kappa-opioid receptor agonist nalfurafine (not available in United States at time of publication) Capsaicin cream |
| HIV/AIDS | Assess for secondary etiology: Infection, peripheral neuropathy, xerosis, primary skin condition, systemic disease, drug reaction, elevated cytokines If HIV is cause: Indomethacin |
Practical steps can be taken to prevent, treat, and mitigate pruritus regardless of the etiology.24 Preventive measures which may help include use of a humidifier if air is dry, maintaining a cool ambient temperature, avoiding rapid changes in environmental temperature, limiting the intake of very hot drinks or spicy food, and avoiding emotional stressors including extreme excitement and strain when possible. It can also be beneficial for children to learn relaxation techniques as well as coping strategies to prevent perpetuation of the itch–scratch cycle.
Interventions that maintain or improve skin moisture and integrity are helpful in soothing itch and include: oral rehydration (when dehydration is present); wearing soft, breathable cotton clothing in layers to prevent excessive sweating; restricting time (<20 min) in the bath or shower; bathing in cool or lukewarm water instead of hot water; dabbing skin when wet; and applying emollients to the skin after bathing and on a regular basis (avoiding alcohol- or water-based products in favor of oil-based products). Some children may find adding oatmeal or potassium permanganate to the bath soothing to itchy skin. High skin surface pH has been noted in several skin disorders, including atopic dermatitis, and uremia. Using low pH cleansers and moisturizers, or preparing baths with sodium bicarbonate added to the bath water can address this issue. Attention should also be paid to avoiding skin irritants, including fragrances.
Since it can be quite difficult to keep children from unconsciously scratching areas that itch, it is crucial to keep a child’s fingernails cut short to mitigate damage to the skin from scratching. Gloves or mittens can be placed on children’s hands, but they are often difficult to keep in place and can limit important developmental exploration of the world early on and impair function in older children.
Additional research is needed to determine treatment efficacy for pharmacologic and nonpharmacologic interventions for chronic pruritus of unknown origin.61
Opioid-Induced Pruritus
Pruritus that may localize to the face and trunk is a common complication of parenteral or neuraxial opioid administration. Pruritus can also be seen with the administration of oral opioids. It is difficult to estimate the incidence of opioids-induced pruritus in children for several reasons including limited data, variation between opioids and routes of administration, and lack of uniformity in methods of evaluation of pruritus. The incidence has been reported to be between 2% and 50% for parenteral administration and between 20% and 100% when neuraxial opioids are administered.62,63
The mechanism of opioid-induced pruritus is not fully understood. It is known that orally, intradermally, or parenterally administered codeine, morphine, and meperidine can cause the release of histamine from mast cells, but fentanyl, alfentanil, sufentanil, buprenorphine, and naloxone do not.64,65 For this reason, histamine cannot play a singular role in opioid-induced pruritus. Furthermore, in studies done in primates, the administration of a histamine antagonist did not attenuate scratch induced by morphine.66 Some opioid-induced pruritus is known to be mediated centrally through mu-opioid receptors,67 and additional mechanisms proposed include modulation of serotonin pathways,68 prostaglandins, the role of an “itch center” in the central nervous system (CNS), medullary dorsal horn activation, antagonism of inhibitory transmitters,69 and D2 receptors.70
In the initial management of pruritus caused by morphine, it may be helpful to change from morphine to an alternative opioid such as hydromorphone or fentanyl. If this is not effective, an opioid receptor antagonist can be administered in conjunction with the patient’s opioid. It is important, however, that the opioid antagonist be used in a low enough dose that it does not reverse the analgesia achieved by the opioid. Both naloxone and naltrexone have been studied. Naloxone can be administered at a starting dose of 0.25 mcg/kg/hr to 1 mcg/kg/hr. Doses of more than 2 mcg/kg/hr are likely to cause an unacceptable degree of reversal of the analgesic effect of the opioid. A pilot study done in children in sickle cell crisis found that patients tolerated co-administration of naloxone and morphine.71 Two additional small studies of 46 and 59 pediatric postoperative patients, respectively, found that concomitant administration of low-dose naloxone and morphine through patient-controlled analgesia (PCA) significantly reduced opioid-associated pruritus.72,73
Opioid agonist-antagonists, including nalbuphine and butorphanol, have also been studied as agents to reduce pruritus. These drugs have conceptual advantages over pure opioid antagonists in that they do not reverse the analgesic effect to the opioid or risk precipitation of opioid withdrawal syndrome. These medications act as antagonists at the mu-receptor and as agonists at the kappa-receptor, which attenuates morphine-induced itch without interfering with analgesia in monkeys.74 Results of human clinical trials have been mixed, with only limited studies in the pediatric population. One study of 184 pediatric patients found that nalbuphine 50 mcg/kg IV given as a single dose was not effective in the treatment of postoperative opioid-induced pruritus.75 Nonetheless, a subsequent review found nalbuphine more effective for opioid-induced pruritus when compared to placebo, control, diphenhydramine, naloxone, or propofol in patients receiving neuraxial opioids.76
Beyond opioid antagonists and opioid agonist/antagonists, there has not been investigation or consensus specifically in children for other agents to prevent or manage opioid-induced pruritus.77,78 In adults, and especially laboring women 5-HT3 receptor antagonists,68 D2 receptor antagonists,70,79 the sodium channel blocker lidocaine80 and prostaglandin-blocking nonsteroidal anti-inflammatory agents (NSAIDs),81 have been studied as possible treatments for opioid-induced pruritus, with mixed results. While studies in adults may be promising for some agents (e.g., propofol82 and midazolam83), these studies are in adults exclusively and limit subjects to those receiving intrathecal or epidural opioids. These factors make it difficult to predict the effect that these medications may have in the treatment of opioid-induced pruritus in seriously ill children. It may be that the use of opioid-sparing analgesic strategies that lower the total opioid dose and secondarily mitigate pruritus (e.g., adjuvants such as acetaminophen and NSAIDs, regional techniques, gabapentinoids, etc.) deserve consideration.
Cancer-Specific Pruritus
Many children with cancer experience pruritus. Although the incidence of pruritus in children is not known, estimates in adult cancer range from 6% to 50%, with higher prevalence in hematologic malignancies.24 Children with cancer may experience greater incidence of pruritus than adults with cancer.84,85 The most common types of cancers that cause pruritus in the pediatric population are cancers of bone, skin, liver, and blood—particularly leukemias and Hodgkin and non-Hodgkin’s lymphomas.84 The exact mechanism by which cancer causes pruritus is unknown, although several factors under investigation include degree of inflammation, presence of eosinophils, role of CD4 helper T cells, and timing of production of IL-31 in the model of cutaneous T-cell lymphoma.86
In order to choose the most appropriate treatment, it is important to consider the cause of the symptom. The pruritus may be a direct result of the cancer84 or a side effect of the child’s treatment.87 Possible etiologies for secondary pruritus include xerosis, radiation desquamation, drug reactions, cholestasis, uremia, side effects from chemotherapy and biologic agents, or graft versus host disease.
The most effective treatment for cancer-related pruritus is anticancer therapy. In advanced illness, however, patients may respond poorly to or are no longer receiving disease-modifying therapies. In these patients, other approaches to the treatment of the pruritus must be identified. There are very limited data on the treatment of pruritus associated with cancer in children, necessitating extrapolation from the adult literature. In Hodgkin’s lymphoma88,89 and polycythemia vera,90 case reports suggest that the H2-receptor antagonist, cimetidine, is effective in treating pruritus. A case series91 that included pediatric patients suggested that pruritus caused by solid tumors may respond to the serotonin selective reuptake inhibitor (SSRI) paroxetine, which was also determined to have moderate evidence of efficacy for pruritus in adults with advanced cancer.92 While paroxetine’s antidepressant effects can take weeks to see, its antipruritic effects may be seen in as little as 24 hours after administration, with the current hypothesis that the more rapid antipruritic effect is serotonin mediated. Mirtazapine has also been shown similarly to improve pruritus related to cancer in three elderly cancer patients93 in other more recent case reports,94,95 with recommendation in systematic reviews of the literature to consider paroxetine and mirtazapine as therapeutic options for cancer-related pruritus.95,96 Anecdotal evidence from small case reports and uncontrolled studies also exist for other medications (e.g., corticosteroids, H1 antihistamines, gabapentinoids, ondansetron, thalidomide, naloxone, and butorphanol), but too little data exist to support their use over the aforementioned medications. Combined therapy may be needed and efficacious to address multifactorial causes of pruritus from immune, peripheral, and CNS stimulation.97
Cholestasis
Cholestasis occurs in children of all ages, but it is particularly common in the neonatal period. The incidence of neonatal cholestasis is estimated to be approximately 1 in 2,500 live births.98 The mechanism by which cholestasis causes pruritus is unclear; while mechanisms including accumulated bile acids, steroid-derived metabolites, endogenous mu-opioids, and serotonin have been observed, they are also disputed as causative mechanisms.99,100 The pruritus of cholestasis is not mediated by histamine, and any relief that patients perceive with the administration of antihistamines is likely related to the sedating effects of the medication. It is of interest to note that pruritus often precedes accumulation of bile salts, and it often resolves if patients progress to liver failure.100
Therapy for pruritus caused by cholestasis is typically administered in a progressive order until efficacy is achieved, starting with medication directed at decreasing the level of circulating bile acids, including the nonabsorbable anion exchange resin cholestyramine.101 However, nonabsorbable anion exchange resins are not effective in the case of intrahepatic biliary obstruction because the bile acids must reach the intestine for the medication to be effective; instead, phenobarbital can be used.102
If obstruction is partial, and pruritus is refractory to cholestyramine (or cholestyramine is contraindicated), rifampin is considered next. Rifampin appears to act by inhibiting autotaxin expression as its mechanism for reducing cholestatic pruritus.24,103 The third- and fourth-line agents for cholestatic pruritus are naltrexone then sertraline, respectively.101,104 Care should be taken when administering mu-opioid antagonists in the seriously ill patient when they are also receiving opioid agonists for pain as there is a risk of loss of analgesia at higher doses of naltrexone.
Although initial studies in adults appeared to show that the 5-HT3 receptor antagonist ondansetron was effective,105 subsequent studies106 failed to show effect. Clinical practice guidelines and systematic reviews consider the use of ondansetron, other medications, and nonsurgical therapies to have insufficient evidence of efficacy and safety.97,100,101
Surgical interventions may also be helpful if medical management has not been successful and surgery makes sense within the goals of care. Procedures including partial external biliary diversion,107,108 terminal ileal exclusion, and gallbladder to colon diversion have shown a trend toward decreased pruritus in some children with intrahepatic cholestasis.109 In pruritus caused by extrahepatic disease, stenting the bile duct is often the best treatment for pruritus. In extreme cases of pruritus that is refractory to treatment and that is causing significant negative effects on a patient’s quality of life, liver transplant may be considered.101
Uremic Pruritus
Pruritus from uremia is seen in patients with chronic renal failure but rarely in those with acute renal failure. The rate of pruritus is higher in patients receiving dialysis than in those not receiving dialysis, and, in adults, the presence of severe uremic pruritus is a predictive factor for death.110 The mechanism by which uremia causes pruritus is not fully understood, but, as in other systemic illnesses, it is likely caused by the accumulation of pruritrogens in the blood. It is intriguing to note that renal transplantation has been found to reduce, and in some cases relieve chronic pruritus,111 but the effect is not uniform for all patients.
Limited research has been done in the pediatric population on pruritus secondary to uremia.112 Therefore, treatments are often based on evidence from the adult literature, which is also lacking in definitive approaches to the condition.113,114 Initial management in patients on dialysis includes enhancing the dialysis regimen and correcting the patient’s calcium, phosphorus, and magnesium. Xerosis, which is very common in uremic patients, should also be treated.115 If a patient is found to have hyperparathyroidism secondary to renal failure, pruritus can resolve after parathyroidectomy.116,117 Beyond these steps, ultraviolet light (UV-B) therapy, gabapentin, oral cromolyn sodium, topical capsaicin, the mu-opioid receptor antagonist naltrexone, and the kappa-opioid receptor agonist nalfurafine have shown some promise in adults,97,118,119 but have yet to be studied systematically in children. As in pruritus caused by most other systemic diseases, antihistamines are not helpful in the treatment of uremic pruritus.
HIV/AIDS
Pruritus is very common in patients with HIV infection. It can be secondary to multiple etiologies, including infection, infestation, peripheral neuropathy, xerosis, a primary skin condition, systemic disease, drug reaction, or elevated levels of cytokines. A thorough evaluation for the most likely causes of the pruritus should be done and should direct treatment. Idiopathic HIV pruritus is uncommon and is diagnosed by the exclusion of other causes. For patients with pruritus associated with HIV infection, a recent Cochrane review in adults concluded that indomethacin may be most effective, but results are tentative and further investigation is necessary to determine ideal therapy.97
Mucositis
Oral and gastrointestinal mucositis, referred to collectively as alimentary tract mucositis, is very common in children receiving cytotoxic chemotherapy, molecularly targeted agents, hematopoietic stem cell transplantation, and/or radiation to areas encompassing the gastrointestinal tract, including the head and neck. Younger patients develop oral mucositis (OM) more frequently and heal more quickly than older patients receiving similar treatments. This is thought to be secondary to an increased epithelial mitotic rate in children as compared to adults. Mucositis of the oral cavity and esophagus can lead to ulceration, oral pain, dysphagia, and odynophagia while mucositis of the gastrointestinal tract can lead to gastritis, abdominal pain, diarrhea, malabsorption, and bloating. Mucositis can be the dose-limiting factor in administration of chemotherapy, and affected children are at risk of bleeding, local infections, sepsis, and compromised nutritional status. Because mucositis limits treatment, causes serious complications, and leads to significant symptom burden, increasing attention has been paid to understanding its pathogenesis, exploring preventative measures, and investigating treatments. Despite this increased attention, there are few interventions that are supported by a strong evidence base. In 2019, the Multinational Association of Supportive Care in Cancer and the International Society for Oral Oncology (MASCC/ISOO) published their updated evidence-based guidelines for mucositis.120 These guidelines provide a strong foundation for the evidence-based care of mucositis.
Given that the groups of patients who are likely to develop mucositis is known, it would be ideal to implement a therapy prior to high-risk intervention to reduce the incidence or severity of the symptom. Efforts to prevent OM have traditionally focused on modifying oral microbial load to decrease inflammatory response and subsequently OM severity. The fact that there have been inconsistent results with the use of antimicrobial therapies such as chlorhexidine rinses has led to the exploration of the concept that oral flora dysbiosis observed in patients during cancer therapy is a predisposing factor in the development of OM and achieving oral flora symbiosis rather than sterilization of the oral cavity may be the goal.121 At this time, though multiple preventative approaches have been studied, the primary recommendation of the MASCC/ISOO is to implement a multiagent combination oral care protocol for the prevention of OM during chemotherapy, head and neck radiation therapy, and hematopoietic stem cell transplantation.122 These protocols should be formulated by a interdisciplinary team including medical, dental, nursing, and child life/psychosocial professionals. Protocols vary between institutions, but typically include recommendations regarding the timing and frequency of regular assessment of the oral mucosa, tooth brushing, flossing, and the use of a bland mouth rinse such as a saline or sodium bicarbonate rinse. Providing education to patients and families about these protocols and appropriate oral care, though not evidence-based, is generally believed to be helpful. The evidence base reviewed by the MASCC/ISOO was not strong enough to create a guideline around professional oral care, but expert opinion supports dental evaluation and treatment prior to cancer therapy.122
Another common preventative measure used to minimize mucositis caused by chemotherapy is cryotherapy. This intervention involves having the child suck on ice for 20–45 minutes while receiving chemotherapy. The ice cools the mucosal tissue and leads to vasoconstriction, decreasing the exposure of the tissue to the chemotherapeutic agent. The MASCC/ISOO guideline supports the use of oral cryotherapy in patients undergoing autologous hematopoietic stem cell transplant (HSCT) with high-dose melphalan conditioning protocols and patients receiving bolus 5-fluorouracil chemotherapy.123 Growth factors and cytokines have been studied for the prevention and treatment of OM and only keratinocyte growth factor-1 (KGF-1) has been shown to be effective in patients undergoing autologous HSCT conditioned with high-dose chemotherapy and total body irradiation regimens.124 Laser therapy and other light therapies that have trophic, anti-inflammatory, and analgesic effects have been trialed for both prevention and treatment of OM. In patients receiving HSCT or head and neck radiation this type of therapy may be effective for prevention of OM and related pain.125
For the prevention of gastrointestinal mucositis, several therapies are being investigated including palifermin, glutamine, sodium butyrate, and dietary interventions, but the evidence base for these interventions is not strong. The MASCC/ISOO does recommend the use of probiotics containing Lactobacillus spp. for prevention of chemotherapy- and radiation-induced diarrhea in patients with pelvic malignancies.126
The primary symptom associated with OM that interdisciplinary teams are asked to address is pain. The mainstay of treatment for pain associated with OM remains systemic opioids. In the setting of oral pain and difficulty swallowing, intravenous PCA with a basal rate and a bolus dose may provide the most immediate and effective pain control. Opioids can be very helpful, but they frequently do not completely control the pain and their use may be limited by side effects such as sedation. For this reason many oral rinses have been trialed as adjuvant therapy. Topical agents are often mixed in different combinations and referred to by various colloquial names such as “magic mouthwash.” Though these combined rinses are common, the evidence does not support their use. In fact the American Academy of Nursing Choosing Wisely statement recommends against the use of mixed medication mouthwashes to prevent or manage cancer treatment–induced OM. Topical agents that have been tried in the treatment of OM-induced pain include analgesics such as viscous lidocaine, morphine, ketamine, diphenhydramine, capsaicin, doxepin, benzydamine, maltodextrin, and dexamethasone. Mucosal protective agents such as aluminum hydroxide and magnesium hydroxide containing liquid antacids, natural honey, Gelclair, Episil, kaolin/pectin, and propolis have also been tried. The evidence base is not strong enough to recommend the use of any one of these adjuvant treatments over another. Caution needs to be taken when considering the use of topical anesthetics due to the theoretical concern over an increased risk of aspiration secondary to the numbing effects of the drugs and concern over systemic absorption through damaged mucosal surfaces. The MASCC/ISOO guidelines do recommend the use of topical 0.2% morphine mouthwash for the treatment OM pain in head and neck cancer patients treated with radiation and chemotherapy.127 It is hypothesized that opioid receptors on peripheral nerves could be blocked by topical morphine leading to reduced neuronal excitability of peripheral nociceptors.127
One factor that may increase the oral pain associated with OM is secondary infection with fungal organisms, bacteria, or herpes simplex virus. In patients with mucositis, evaluation should be done for any signs of infection, and appropriate treatment should be started based on the results of the evaluation. In immunosuppressed patients, including patients on systemic steroids, topical treatment of oral infections is typically not sufficient and systemic treatment should be considered.
When children have OM, particular care should be taken to make sure they are eating foods that don’t increase their pain. Foods that are acidic, salty, dry, or that require a significant amount of chewing should be avoided.
The primary symptom associated with gastrointestinal mucositis that interdisciplinary teams are asked to address is diarrhea. It is important to ensure that adequate hydration is maintained. Diarrhea can be treated with standard antidiarrheal medications including loperamide. If loperamide is not effective, then octreotide can be considered.
Diaper Dermatitis
Diaper dermatitis is a broad term used to describe an inflammatory reaction of the skin within the diaper area. Common causes include allergic contact dermatitis (ACD) and irritant contact dermatitis (ICD).
Allergic contact dermatitis can be caused by exposure to an allergen leading to a T-cell–mediated type IV delayed hypersensitivity reaction. The inflammatory response requires sensitization caused by an allergen penetrating the epidermal skin barrier followed by a subsequent exposure to the same allergen.128 Common allergens include components of topical creams such as lanolin and formaldehyde releasers, preservatives such as benzalkonium chloride used in baby wipes, and topical medications including neomycin, antifungals, and steroids. The clinical presentation in the acute phase typically spares the skin folds and causes erythema, scaling, vesicles, and bullae.128 Infants and toddlers may be less likely to develop ACD secondary to the immaturity of their immune systems.129 Treatment includes avoidance of the allergen, regular use of emollients that enhance the barrier function of the skin, and topical corticosteroids. In very severe cases a short course of systemic corticosteroids can be considered.128
The most common cause of diaper dermatitis is ICD caused by impairment of the skin’s barrier function and subsequent exposure to irritants such as those in urine and stool. Commonly the initial presentation includes erythema, mild maceration, and scaling which spares the inguinal folds. More severe presentations can include papules, pustules, and skin breakdown with open areas130 (Figure 26.2). Complications of the initial condition can include granuloma gluteal infantum,131 Jacquet’s erosive diaper dermatitis,132 pseudoverrucous papules,133 and secondary infection.134 The incidence of ICD peaks at 9–12 months of age,129,134,135 but in seriously ill children incontinence and prolonged use of diapers is common and ICD can occur at any age.
Figure 26.2 Diaper dermatitis.
The initiating event for ICD is overhydration of the skin from prolonged exposure to urine and stool which is potentiated by the physical occlusion of the diaper.130,134 Friction from the diaper against the overhydrated skin causes damage to the stratum corneum and epidermis. This increases the skin’s permeability to irritants.130,134 In addition, the pH in the diaper area is increased when fecal ureases catalyze the conversion of urinary urea to ammonia.130,134 Urease-positive bacteria in feces, which are more common in children who drink cow’s milk, can also degrade urea and release ammonia.129 The elevated pH activates fecal proteases and lipases which are considered the most significant irritants leading to ICD.129 The fecal enzymes further increase the skin’s permeability to bile salts and other irritants.136 It follows that there is a positive correlation between the number of stools per day and the development of ICD.135 Of note, the faster stool moves through the intestine the higher the proteases and lipases concentration, leading to an even further increased susceptibility to ICD in children having diarrhea.129 Broad-spectrum antibiotics can play a role by causing diarrhea and increasing the susceptibility to the development of a candida infection.129 Other factors that may increase the likelihood of ICD are zinc and biotin deficiencies.129 Additionally, premature infants can take several weeks to develop proper skin pH and full barrier function leading to the need for increased attention to this issue in the neonates.134 One protective factor against ICD is breastfeeding which results in stools with a lower pH.136
The prevention and treatment of ICD starts with trying to keep the skin in the diaper area dry. This can be achieved by ensuring that the diaper is changed as soon as it becomes wet or soiled. An additional helpful approach is exposing the diaper area to air as often as possible,130 which has the added benefit of decreasing friction on the skin from the diaper. Emollients can be applied to support the skin’s barrier function.137 When the diaper is changed, the skin should be gently cleansed completely removing urine and stool while minimizing friction. Research suggests that there is no significant difference between using a wet washcloth or a baby wipe to clean the skin.138,139 Traditional soap with an alkaline pH should be avoided and mild cleansers with a slightly acid pH should be used instead.137 Wipes should not contain alcohol, fragrances, essential oils, soap, or harsh detergents that can lead skin irritation. They should contain well-tolerated preservatives and a pH buffer to maintain slight acidity of the skin.137 Once the skin is clean, a barrier cream containing zinc oxide or petroleum can be used to reduce the skin’s contact with urine and stool. The barrier cream does not need to be fully removed between diaper changes as long as all of the urine and stool has been removed. Modern disposable diaper technology that includes super-absorbent gels, petrolatum-based lotions, breathable outer layers, and a mesh like aperture top sheet has helped in preventing ICD. These modern disposable diapers may be more effective in preventing ICD than cloth diapers.140,141,142 If a severe ICD develops that is not responsive to the preceding measures, a short 3–7 day course of low-potency, nonhalogenated topical corticosteroid such as 1% hydrocortisone can be applied twice a day before the barrier cream. High-potency topical steroids and combination antifungal/potent steroid creams should be avoided as there can be systemic absorption leading to adrenal suppression. Topical vitamin A has been studied for treatment and prevention of ICD, but it is unclear whether it is efficacious.143
In the setting of diaper dermatitis with skin breakdown and an elevated skin pH that alters the cutaneous microbiome, there is increased risk for developing a secondary fungal or bacterial infection. The most common fungal infection is Candida albicans.136 It commonly involves the deep skin folds and presents as erythematous and scaly plaques with satellite papules and pustules. Microscopic slide examination (KOH) or fungal culture can aid with diagnosis. If diaper dermatitis persists for more than 3 days after initiation of the standard treatments, it is likely that there is a secondary infection with C. albicans. Cutaneous infections can usually be easily treated with topical antifungals.144 Persistent or recurrent infections could be a sign of diabetes mellitus, chronic mucocutaneous candidiasis, or an underlying immune deficiency. Bacterial infections in the diaper area can also occur and often present with vesiculopustular lesions that can be mistaken for fungal infection. The most common bacterial infection is Staphylococcus aureus.136 Bullous impetigo caused by S. aureus often presents as large bullae that break down, leaving collarets of scale behind. Newborns are especially prone to development of bullous impetigo. Staphylococcus sp., Escherichia coli, and Bacteroides sp. are common.145 Appropriate antibiotic therapy can be tailored based on bacterial culture results and organism susceptibility profiles. Typically topical antibiotic treatment is sufficient, but oral antibacterial treatment may be needed for more extensive infections and when topical therapy is not effective.146 In the event of fever or ill appearance, an evaluation by a healthcare professional is indicated.
For persistent dermatoses that present in the diaper area and are unresponsive to conventional treatment approaches, dermatologic consultation and skin biopsy may be indicated. Skin diseases such as chronic inflammatory dermatoses (atopic dermatitis, seborrheic dermatitis, and psoriasis), infectious diseases (scabies, congenital syphilis, and primary herpes simplex), metabolic diseases (acrodermatitis enteropathica), autoimmune diseases (lichen sclerosis and Kawasaki disease), and neoplasms (Langerhans cell histiocytosis) may present with prominent groin involvement.129,144
One study147 documented parental guilt secondary to a child developing diaper dermatitis, therefore care should be taken not to imply poor parenting when providing education about prevention and treatment of ICD. At the same time child abuse and/or neglect should be listed in the differential diagnosis of severe, recalcitrant, or atypical diaper dermatitis. ICD not responding to treatment may be the result of neglect and the diaper area is a possible site for burns and bruises.
Wounds
Unlike wound management in the general population, the first determination that should be made when managing a wound in a seriously ill child is the goal of treatment. Frequently, the goal is to heal the wound. However, in some cases, particularly when the child is near the end of life, there may not be time to heal the wound. The goal then becomes to stabilize the wound and control the symptoms associated with the wound including pain, odor, bleeding, and exudate. Under these circumstances care should be given to managing the periwound area.
Types of Wounds
Pressure Injuries
Pressure injuries in the pediatric population, while not as prevalent as in adults, are still a significant dermatologic occurrence that must be appropriately addressed. The incidence of pressure injuries in the pediatric population ranges from 1.4% to 28.8% depending on risk factors, with children in critical care being more likely to develop a pressure injury.148,149,150 Minimal data are available on the overall incidence of pediatric pressure injuries, especially in seriously ill children. Data on the incidence and point prevalence of pediatric pressure injuries in different care settings, such as home setting versus hospital, are not available.
Children who are bed- or chair-bound or who have limited ability to reposition themselves are at particularly high risk for developing pressure injuries. Poor nutritional status and conditions leading to moist skin, such as incontinence, are compounding risk factors. It is important to identify patients who are at high risk and take steps to prevent pressure injury formation. If a child is identified as being high risk, his or her skin should be inspected daily so that issues can be identified before they become problematic. A bed-bound patient should be repositioned every 2 hours and a chair-bound patient every hour. An appropriate pressure-reducing mattress is essential to off-load areas of higher pressure.151 In addition care should be taken to ensure that patients have appropriate cushions for their wheelchairs and other frequently used seating. Donut-type cushions should be avoided because they reduce blood flow to the tissue within the donut. Children should be lifted with the help of a draw sheet or trapeze when repositioned to prevent shearing forces. Pillows, foam wedges, and flotation devices or boots should be used to prevent bony prominences, such as the heels and ankles, from coming into contact with each other and surfaces.
When patients are in a side-lying position, pressure directly on the trochanter should be avoided. When a child is incontinent, an absorbent diaper or brief should be used, and it should be changed as soon as the child becomes wet or soiled. In addition, a barrier cream should be used to protect the skin from moisture. Excessive skin dryness is also a risk factor for skin breakdown. It should be avoided by applying liberal amounts of moisturizer directly to skin that is still damp after bathing (reapply up to 3–4 times daily) and subsequently overlapped with an emollient to serve as an occlusive agent thereby preventing moisture loss. When a child is being cared for at home it is important that parents and other caregivers are educated on how to prevent pressure injuries and how to identify pediatric skin failure at the earliest stage.152
Pressure injuries occur in similar locations in both adults and children. The most common locations are the heels, neck, sacrum, and scalp (specifically the occiput), or when any medical device is in contact with skin.153,154 Whenever possible, avoid adhesives, rotate medical devices, dry skin whenever it is wet, and use appropriate skin barriers.150 The primary concern, regardless of location of the injury, is addressing and eliminating the source of pressure, shear, friction, and or excess moisture. Examination of pressure sources includes evaluation of support surfaces, splints, braces, clothing, and any other material or device that is in contact with the skin. Unless the underlying etiology is identified, the opportunity to intervene and/or prevent further injury and promote healing is lost and may lead to continued tissue damage and severe injury. Whenever using any product on a child, whether a pressure-reducing surface, device, or other product, it is important to review the information related to age-specific use.
A child’s report of pain from a cast, splint, or other supportive device should always be taken seriously pending complete review of the source of the pain. If in doubt, the supportive device should be removed to allow for a full assessment. Medical device–related pressure injuries (MDPIs) most commonly occur with external monitoring, oxygen delivery, and airway maintenance.153 Sources of pressure that may require removal include but are not limited to casts, other immobilization devices and boards, masks, pressure cuffs, and tubing.
Identification of the underlying pressure injury etiology is critical to optimal outcomes. When treating pressure injuries, it is important to rule out other potential etiologies. These etiologies include fluid extravasation; surgery-related wounds; electrical, thermal, and chemical burns; incontinence-related erosions or lesions; congenital abnormalities; trauma from dressings, adhesives, and clothing; and systemic diseases. Potential abuse should be included in the differential diagnosis based on the presentation and history. Failure to treat underlying pathophysiology will delay or prevent closure of the wound.
Pressure injury staging is the same in adult and pediatric populations. The most commonly accepted system for staging and describing pressure injuries is from the National Pressure Injury Advisory Panel (NPIAP), shown in Box 26.1. The NPIAP updated its name in November 2019 from National Pressure Ulcer Advisory Panel (NPUAP) to reflect a shift to the internationally preferred term “pressure injury” in place of “pressure ulcer” to acknowledge that lesser degrees of skin damage due to pressure may not be associated with skin ulceration. The description and identification for each stage was updated to reflect additional mechanisms and areas of injury (e.g., device-related and mucosal pressure injuries).155,156
Box 26.1 Revised National Pressure Injury Advisory Panel (NPIAP) Pressure Injury Staging System
Pressure Injury: A pressure injury is localized damage to the skin and underlying soft tissue usually over a bony prominence or related to a medical or other device. The injury can present as intact skin or an open ulcer and may be painful. The injury occurs as a result of intense pressure, prolonged pressure or pressure in combination with shear. The tolerance of soft tissue for pressure and shear may also be affected by microclimate, nutrition, perfusion, comorbid conditions and condition of the soft tissue.
Stage 1 Pressure Injury: Non-blanchable erythema of intact skin. Intact skin with a localized area of nonblanchable erythema, which may appear differently in darkly pigmented skin. Presence of blanchable erythema or changes in sensation, temperature, or firmness may precede visual changes. Color changes do not include purple or maroon discoloration; these may indicate deep tissue pressure injury.
Stage 2 Pressure Injury: Partial- thickness loss of skin with exposed dermis. Partial-thickness loss of skin with exposed dermis. The wound bed is viable, pink or red, moist, and may also present as an intact or ruptured serum-filled blister. Adipose (fat) is not visible and deeper tissues are not visible. Granulation tissue, slough and eschar are not present. These injuries commonly result from adverse microclimate and shear in the skin over the pelvis and shear in the heel. This stage should not be used to describe moisture associated skin damage (MASD) including incontinence associated dermatitis (IAD), intertriginous dermatitis (ITD), medical adhesive related skin injury (MARSI), or traumatic wounds (skin tears, burns, abrasions).
Stage 3 Pressure Injury: Full-thickness skin loss. Full-thickness loss of skin, in which adipose (fat) is visible in the ulcer and granulation tissue and epibole (rolled wound edges) are often present. Slough and/or eschar may be visible. The depth of tissue damage varies by anatomical location; areas of significant adiposity can develop deep wounds. Undermining and tunneling may occur. Fascia, muscle, tendon, ligament, cartilage or bone are not exposed. If slough or eschar obscures the extent of tissue loss this is an unstageable pressure injury.
Stage 4 Pressure Injury: Full-thickness loss of skin and tissue. Full-thickness skin and tissue loss with exposed or directly palpable fascia, muscle, tendon, ligament, cartilage or bone in the ulcer. Slough and/or eschar may be visible. Epibole (rolled edges), undermining and/or tunneling often occur. Depth varies by anatomical location. If slough or eschar obscures the extent of tissue loss this is an unstageable pressure injury.
Unstageable Pressure Injury: Obscured full-thickness skin and tissue loss. Full-thickness skin and tissue loss in which the extent of tissue damage within the ulcer cannot be confirmed because it is obscured by slough or eschar. If slough or eschar is removed, a Stage 3 or Stage 4 pressure injury will be revealed. Stable eschar (i.e. dry, adherent, intact without erythema or fluctuance) on an ischemic limb or the heel(s) should not be softened or removed.
Deep Tissue Pressure Injury: Persistent non-blanchable deep red, maroon or purple discoloration. Intact or non-intact skin with localized area of persistent non- blanchable deep red, maroon, purple discoloration or epidermal separation revealing a dark wound bed or blood filled blister. Pain and temperature change often precede skin color changes. Discoloration may appear differently in darkly pigmented skin. This injury results from intense and/or prolonged pressure and shear forces at the bone-muscle interface. The wound may evolve rapidly to reveal the actual extent of tissue injury, or may resolve without tissue loss. If necrotic tissue, subcutaneous tissue, granulation tissue, fascia, muscle or other underlying structures are visible, this indicates a full thickness pressure injury (Unstageable, Stage 3 or Stage 4). Do not use DTPI to describe vascular, traumatic, neuropathic, or dermatologic conditions.
Additional pressure injury definitions.
Medical Device Related Pressure Injury: This describes an etiology. Medical device related pressure injuries result from the use of devices designed and applied for diagnostic or therapeutic purposes. The resultant pressure injury generally conforms to the pattern or shape of the device. The injury should be staged using the staging system.
Mucosal Membrane Pressure Injury: Mucosal membrane pressure injury is found on mucous membranes with a history of a medical device in use at the location of the injury. These ulcers cannot be staged.
Used with permission of the Prevention and Treatment of Pressure Ulcers/Injuries: Clinical Practice Guideline. The International Guideline 2019. Third edition released November 2019 by the European Pressure Ulcer Advisory Panel, National Pressure Injury Advisor Panel, Pan Pacific Pressure Injury Alliance.
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