Background
Methods
Study citation | Study type | Population (setting, patients) | Intervention/Comparison groups | Outcomes |
---|---|---|---|---|
Chiu 1999 [68] | Case report | Country: Canada Setting: Children’s Hospital Age: 11 years Gender: Male Patient with Severe JEB | Amitryptiline (25 mg at night) was prescribed and patient started on a program of cognitive behavioral training (hypnotic imagery, distraction). Oral midazolam (7.5 mg) was initiated 20 minutes prior to bath or dressing change | Pain management |
Review articles | Country: United States Setting: Children’s Hospital Patients with EB | Pain management and prevention | Pain management | |
Herod 2002 [44] | Review article | Country: England (London) Setting: Children’s Hospital Patients with EB | General pain management | Pain management |
Mellerio 2007 [152] | Review article | Country: United States, England, Chile Setting: Hospital Patients with EB | Medical management | General pain management |
Saroyan 2009 [156] | Case report | Country: United States Setting: Hospital Female infant with EBS, severe, Dowling-Meara subtype | Use of IV ketamine given orally Oral administration of IV ketamine (10 mg/mL, Monarch Pharmaceuticals) at a starting dose of 0.5 mg (0.125 mg/kg/ dose) Over four days, the dose was titrated to 3 mg (0.75 mg/kg/dose) in response to observed effect | Achieve analgesia during painful dressing changes |
van Scheppingen 2008 [4] | Qualitative study (Interviews) | Country: Netherlands Setting: Center for Blistering Diseases Age: 6 to 18 years Children with different (sub)types of EB) | Interviews conducted at homes or in hospital Questions explored were: (i) What problems do children with EB actually experience as being the most difficult? (ii) What is the impact of these problems on their daily life? (iii) Are there differences in experiences between mildly and severely affected children? | Themes of pain for severe disease (generalized blistering with motion impairment) and for mild disease (localized blistering or generalized blistering without motion impairment). |
Watterson 2004 [74] | Case report | Country: United States Setting: Hospital Children with EB using peripheral opioids | Topical morphine gel applied to the most painful areas of skin at that time for each child | Pain scores |
Quality level | Definition |
---|---|
1aa or 1ba
| Systematic review, meta-analysis, or meta-synthesis of multiple studies |
2a or 2b | Best study design for domain |
3a or 3b | Fair study design for domain |
4a or 4b | Weak study design for domain |
5a or 5b | General review, expert opinion, case report, consensus report, or guideline |
5 | Local Consensus |
Application | Level of recommendation | Target age group | Key references (evidence grade) | |
---|---|---|---|---|
A. Psychological therapies offer effective approaches to management of chronic and acute pain as well as itching.
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For chronic pain management use cognitive behavioral therapy (CBT). | B | All | ||
For acute pain management, offer the patient distraction, hypnosis, visualization, relaxation or other forms of CBT | B | All | ||
Consider habit reversal training, and other psychological techniques for management of pruritus | C | All | ||
B. Postoperative pain can be handled as for other patients in the same setting, with modifications.
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Basic perioperative assessment and pain treatments should be used as for non-EB patients, with modification | A | All | ||
Transmucosal (including intranasal fentanyl and transbuccal opioids) should be considered for short procedures and pain of brief duration when intravenous and enteral routes are unavailable | B | All | ||
Perioperative opioid use must account for preoperative exposure, with appropriate dose increases to account for tolerance | B | All | ||
Regional anesthesia is appropriate for pain resulting from a number of major surgeries. Dressing of catheters must be non-adhesive and monitored carefully | C | All | ||
C. Skin wounds and related pain are the hallmark of EB of most subtypes. Prevention and rapid healing of wounds through activity pacing, optimal nutrition and infection control are important. A number of pharmacologic treatments are available
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Maintain optimal nutrition and mobility and treat infections as indicated | D | All | Denyer 2010 [57] (5a) | |
Consider topical therapies for pain | C | All | ||
Systemic pharmacologic therapy should be adapted to treat both acute and chronic forms of skin pain | B | All | ||
Monitor potential long-term complications of chronically administered medications | C | Pediatric | ||
D. Baths and dressing changes require attention to both pain and anxiety
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Anxiolytics and analgesics should be used for procedural pain and fear. Care must be taken when combining such medications due to cumulative sedative effects | B | All | ||
Cognitive behavioral techniques should be implemented as the child becomes old enough to use them effectively. Specifically, distraction should be used for younger children | B | All | ||
Environmental measures such as adding salt to the water to make it isotonic and keeping the room warm are recommended | B | All | ||
E. EB affects the gastrointestinal tract in its entirety. Pain from ulcerative lesions responds to topical therapy. GERD and esophageal strictures have nutritional as well as comfort implications and should be addressed promptly when found. Maintaining good bowel habits and reducing iatrogenic causes of constipation are crucial.
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Topical treatments are recommended for oral and perianal pain | C | All | ||
Therapy should be directed to manage gastroesophageal reflux and esophageal strictures using standard treatments | C | All | Freeman 2008 [95] (4a) | |
Constipation should be addressed nutritionally, with hydration and addition of fiber in the diet to keep stool soft, by minimizing medication-induced dysmotility and with stool softeners | C | All | ||
F. Bone pain treatment must account for factors that include nutrition, mobility, potential occult fractures and is treated by combinations of nutritional, physical, pharmacologic and psychological interventions.
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Joint pain should be treated with mechanical interventions, physical therapy, CBT and surgical correction | C | All | ||
Osteoporosis should be treated to reduce pain in EB | D | All | ||
Back pain should be addressed with standard multi-disciplinary care | C | All | Chou, et al., 2007 [133] (5a) | |
G. Corneal abrasions are common in EB, prevention and supportive care are appropriate
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Care should include general supportive and analgesic care, protecting the eye from further damage, and topical therapies | C | All | ||
H. Pain in infants is as widespread as in any other age, but unique pharmacologic, developmental and physiologic issues must be accounted for in infants with all types of EB
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Assess patients as needed and prior to and after interventions; health care workers should use validated measures. (Grade: A) | A | Infants | ||
Sucrose solutions should be administered for mild to moderate pain alone or as an adjunct | B | Young infants | ||
Standard analgesics should be used in infants as in older patients with careful attention to dosing and monitoring | B | Infants | ||
I. End of Life pain care is an expected part of care for EB, which in many cases is life-limiting in nature. All basic principles of palliative care apply as they do for other terminal disease states.
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Assess and manage physical, emotional and spiritual suffering of the patient, while providing support for the whole family | A | All | ||
Opioids are the cornerstone of good analgesia in this setting. Opioid rotation may need to be considered to improve analgesia and reduce side effects, and adjuncts may need to be added | B | All | ||
Consider targeted medication for neuropathic pain when pain proves refractory to conventional therapies | D | All | Allegaert 2010 (5a), Saroyan 2009 (5a). Clements 1982 (5a), Watterson 2005 [87] (5b) | |
Continuous subcutaneous infusion of combinations of medication is an option when parenteral therapy is needed in the terminal phase | C | All | ||
Where needed, breakthrough medication can be given transmucosally (oral or nasal) for rapid onset and avoidance of the enteral route | B | All | Zeppetella 2009 [182] (1a) | |
J. A combination of environmental, cognitive-behavioral and pharmacologic therapies are available for use for EB-related pruritus, which can be a severe symptom of the disease.
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Use environmental and behavioral interventions for itch control | C | All | ||
Antihistamines are recommended and can be chosen depending upon desirability of sedating effects | D | All | ||
Gabapentin, pregabalin, TCA, SNRIs and other non-traditional antipruritics agents should be strongly considered for itch treatment | C | All |
Dimension | Definition |
---|---|
Grade of the body of evidence | High, Moderate, Low, Not Assignable |
Safety/harm (side effects and risks)
| Minimal, Moderate, Serious |
Health benefit to patient | Significant, Moderate, Minimal |
Burden on patient to adhere to recommendation | Low, Unable to determine, High |
Cost-effectiveness to healthcare system | Cost-effective, Inconclusive, Not cost-effective |
Directness of the evidence for this target population | Directly relates, Some concern of directness, Indirectly relates |
Impact on morbidity/mortality or quality of life | High, Medium, Low |
Updating procedure
Results and discussion
Psychological and integrative approaches
Introduction
Types of therapies
Efficacy of psychological interventions in chronic and acute pain management
Psychological interventions for itch
Integrative medicine therapies
Good practice points
Acute pain care: postoperative pain management
Introduction
Assessment
Systemic therapies
Preoperative tolerance
Regional anesthesia
Non-pharmacologic therapies
Good practice points
Chronic and recurrent pain care
Skin and wound pain
Introduction
Environmental and behavioral approaches
Systemic approaches
Topical approaches
Good practice points
Bathing and dressing changes
Introduction
Environmental treatment
Analgesics
Anxiolysis
Other behavioral comfort measures
Good practice points
Pain related to the gastrointestinal tract
Upper gastrointestinal tract
Topical treatments
Gastroesophageal reflux
Esophageal strictures
Lower gastrointestinal tract
Constipation
Colitis
Perianal pain
Good practice points
Musculoskeletal pain
Introduction
Joint pain
Bone pain
Back pain
Good practice points
Eye pain
Good practice points
Special Topics
Pain care in infants with EB
Introduction
Pain assessment
Procedural pain
Bathing and dressing changes
Severely affected hospitalized infants
Good practice points
End of life pain care
Introduction
Opioids
Adjunctive measures for neuropathic pain
Infusions and issues of drug delivery
Breakthrough pain medication at the end of life
Good practice points
Pruritus
Introduction
Non-pharmacological approaches
Pharmacological therapies
Good practice points
Conclusions
1. | Availability of resources (for example, medications and equipment) |
2. | Legal and social restrictions on the use of various medications and therapies. |
3. | Limited and uneven distribution of knowledge and expertise |
4. | International dissemination of guidelines and EB-related information to local care providers and families (includes translation and access to electronic and print media) |
Psychological and integrative approaches:
| |
1. | Test the efficacy of well-established cognitive behavioral interventions for acute and chronic pain management in EB. |
2. | Develop EB-specific pain assessment measures for both acute and chronic pain. |
3. | Evaluate the efficacy of cognitive behavioral therapy for EB-related pruritus |
4. | Evaluate the role for Integrative Medicine techniques for the EB population. |
Acute pain:
| |
1. | Improve the balance between analgesia and side effects specific to EB (for example, itching). |
2. | Establish optimal treatment of needle-related pain. |
3. | Define the role for ketamine and other non-opioid agents. |
Chronic and recurrent pain:
| |
1. | Evaluate topical therapies including opioids, local anesthetics and NSAIDs. |
2. | Determine optimal environmental interventions for bath and dressing changes including bath additives (salt, bleach, oatmeal). |
3. | Define optimal perianal pain therapies. |
4. | Clarify the role of bone density screening in preventing bone pain and fractures. |
5. | Determine the role of topical NSAIDs in treatment of corneal abrasion pain. |
6. | Explore the role for various physical and occupational therapy interventions for joint, bone and back pain. |
Infants:
| |
1. | Validate observational pain scales in the setting of bandaged infants. |
2. | Determine the safety and dosing of adjunct medications, such as gabapentin and topical agents. |
Pruritus:
| |
1. | Establish the mechanisms of pruritus in EB and effective treatment thereof. |
2. | Refine the management of opioid-exacerbated itch. |
End of life:
| |
1. | Define how best to integrate palliative care into the overall care of patients with EB prior to end of life. |
2. | Define optimal treatments for pain at the end of life. |