Table 3 Summary of recommendations

For chronic pain management use cognitive behavioral therapy (CBT).

B

All

Gerik 2005 [13] (5a), Palermo 2005 [17] (5a)

For acute pain management, offer the patient distraction, hypnosis, visualization, relaxation or other forms of CBT

B

All

Green 2005 [14] (5a), Uman 2006 [21]

Consider habit reversal training, and other psychological techniques for management of pruritus

C

All

Chida 2007 [29] (1a), Ehlers 1995 [32] (2b), Azrin 1973 [30] (4b), Hagermark 1995 [28] (5a), Rosenbaum 1981 [31] (5a)

Basic perioperative assessment and pain treatments should be used as for non-EB patients, with modification

A

All

Goldschneider 2010 [41] (5a), Goldschneider 2010b [42] (5a)

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

Manjushree et al., 2002 [45] (2b); Borland et al., 2007 [46] (2b); Desjardins et al., 2000 [47] (2a)

Perioperative opioid use must account for preoperative exposure, with appropriate dose increases to account for tolerance

B

All

Hartrick 2008 [56] (1a), Mhuircheartaigh 2009 [55] (1a), Viscusi 2005 [54] (2a)

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

Diwan 2001 [51] (5a), Doi 2006 [53] (5b), Englbrecht 2010 [52] (5a), Kelly 1988 [48] (5b), Sopchak 1993 [49] (5a), Yee 1989 [50] (5a)

Maintain optimal nutrition and mobility and treat infections as indicated

D

All

Denyer 2010 [57] (5a)

Consider topical therapies for pain

C

All

Cepeda 2010 [77] (1a), Lander 2006 [76] (1a), LeBon 2009 [73] (1a), Twillman 1999 [72] (5a), Watterson 2004 [74](5a)

Systemic pharmacologic therapy should be adapted to treat both acute and chronic forms of skin pain

B

All

Noble 2010 [59] (1a), Moore, 2011 [ 67] (1a), Nicholson 2009 [65] (1a)

Monitor potential long-term complications of chronically administered medications

C

Pediatric

Huh 2010 [62] (4a), Camilleri 2011 [66] (5a), Chiu 1999 [68](5a), Cruciani 2008 [63] (5a), Gray 2008 [69] (5a)

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

Bell 2009 [85] (1a), Blonk 2010 [84] (1b), Ezike 2011 [82] (2a), Desjardins 2000 [47] (2a), Borland 2007 [46] (2b), Manjushree 2002 [45] (2b), Humphries 1997 [83] (2b), Wolfe 2010 [81] (5a), Ugur 2009 [86] (5a)

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

Green 2005 [14] (5a); Gerik 2005 [13] (5a), Palermo 2005 [17] (5a)

Environmental measures such as adding salt to the water to make it isotonic and keeping the room warm are recommended

B

All

Arbuckle 2010 [78] (5a), Cerio 2010 [79] (5a), Peterson (Poster) 2011 [80] (5b)

Topical treatments are recommended for oral and perianal pain

C

All

Ergun 1992 [98] (4a), Travis 1992 [97] (4b), Marini 2001 [99] (5a), Buchsel 2008 [100] (5a), Buchsel 2003 [101] (5b), Cingi 2010 [102] (2a)

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

Belsey 2010 [112] (1a), Freeman 2008 [98] (4a), Hanson 2006 [113] (4a)

Joint pain should be treated with mechanical interventions, physical therapy, CBT and surgical correction

C

All

Bruckner 2011 [126] (4b), Gandrud 2003 [131] (4b), Martinez 2010 [125] (5a), Lacativa 2010 [127] (5a), Tilg 2008 [128] (5a), Noguera 2003 [130] (5a), Falcini 1996 [132] (5a)

Osteoporosis should be treated to reduce pain in EB

D

All

Levis 2012 [129] (1a), Martinez 2010 [125] (5a)

Back pain should be addressed with standard multi-disciplinary care

C

All

Chou, et al., 2007 [133] (5a)

Care should include general supportive and analgesic care, protecting the eye from further damage, and topical therapies

C

All

Watson, 2012 [136] (1a), Calder 2005 [138] (1a)

Assess patients as needed and prior to and after interventions; health care workers should use validated measures. (Grade: A)

A

Infants

Gibbins 2014 [139] (2a) Stevens 2014 [140] (2a), Hummel 2008 [141] (2a), Krechel 1995 [142] (2b), Lawrence 1993 [143] (2b), Manworren 2002 [144] (2a)

Sucrose solutions should be administered for mild to moderate pain alone or as an adjunct

B

Young infants

Harrison 2010 [150] (1a), Yamada 2008 [149] (1a) Cignacco 2012 [151] (2a)

Standard analgesics should be used in infants as in older patients with careful attention to dosing and monitoring

B

Infants

Tremlett 2010 [153] (5a), MacDonald 2010 [154] (5b)

Assess and manage physical, emotional and spiritual suffering of the patient, while providing support for the whole family

A

All

Craig 2007 [165] (5a), AAP 2000 [166] (5b), WHO [167] (5b)

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

Eisenberg 2009 [174] (1a), Quigley 2010 [172] (1a), Davies 2008 [61] (4a), Bruera 1996 [171] (4b), Watterson 2005 (5b)

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

Reymond 2003 [178] (2b), O’Neil 2001 [176] (5a), Watterson 2005 [87] (5b)

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)

Use environmental and behavioral interventions for itch control

C

All

Nischler 2008 [183] (5b), EB Nurse Website [187] (5b)

Antihistamines are recommended and can be chosen depending upon desirability of sedating effects

D

All

Ahuja 2011 [188] (2b), Goutos 2010 [186] (3a)

Gabapentin, pregabalin, TCA, SNRIs and other non-traditional antipruritics agents should be strongly considered for itch treatment

C

All

Goutos 2010 [186] (3a), Ahuja 2013 [189] (2a), Murphy 2003 [197] (2a)