Pain in Children with Special Health Care Needs

Many kinds of pain affect children and youth with special health care needs (CYSHCN). Some children may have intellectual disability that makes assessing and understanding their pain more challenging, and others may have ongoing acute pain as part of their condition. This resource provides primary care clinicians with some insight into pain assessment and management strategies for children with special health care needs. See related topics for more details:

Key Points

Parent intuition
Parents with CYSHCN are sometimes frustrated when primary care, emergency room, and other clinicians do not respond to concerns about their child being in pain or “not acting like himself.” In general, parents should be considered the best source of information about what a non-verbal child is feeling. Parental feelings about pain or something being wrong should be taken seriously.

Neuro-irritability
In non-verbal children, there is a phenomenon called neuro-crying, neuro-irritability, and/or neuro-irritation that can be difficult to distinguish from pain. This crying might be the result of an immature or abnormal nervous system, but this is a diagnosis of exclusion; other sources of pain should be ruled out. Even without finding a source of pain, the medical home should work with the family on ways to alleviate the crying, e.g., upright vs. supine posture, changes in diet, and calming measures. The parents should be encouraged to take some time away, even for an hour or 2, when stressed by the crying. Notably, this type of irritability may respond to gabapentin. [Collins: 2019]

Guidelines

Harris J, Ramelet AS, van Dijk M, Pokorna P, Wielenga J, Tume L, Tibboel D, Ista E.
Clinical recommendations for pain, sedation, withdrawal and delirium assessment in critically ill infants and children: an ESPNIC position statement for healthcare professionals.
Intensive Care Med. 2016;42(6):972-86. PubMed abstract / Full Text

Hauer J, Houtrow AJ.
Pain Assessment and Treatment in Children With Significant Impairment of the Central Nervous System.
Pediatrics. 2017;139(6). PubMed abstract

Diagnosis

Interpreting the manifestations of pain should account for the developmental age of the child and their verbal ability/interest. In non-verbal children, it is often difficult to distinguish pain from agitation, such as a child with dystonic cerebral palsy whose agitation episodes may be due to an environmental stimulus that is not pain-related. Presentations of pain in children with special health care needs can vary widely and are often non-specific. Quinn et al. identified irritability, feeding intolerance, change in mental status, vomiting, breath-holding, and increased muscle tone or spasticity as presentations of pain in children with medical complexity. [Quinn: 2018]

Common sources that should be considered when evaluating a child with pain/irritability of unknown causes include ear infections, dental caries, fractures, constipation, skin lesions, urinary tract infections, and abdominal pain.

Several scales that focus on a specific age group or setting are available for assessing pain in infants and children. These and other scales are discussed in [Beltramini: 2017]; the use of such scales in children with medical complexity is discussed in [Quinn: 2018].

  • For children with a mental age of ≥6 years, visual analog scales (VAS), generally using icons of faces reflecting various levels of discomfort/distress, are recommended. [Bailey: 2012]
  • For those with a mental age of ≤5 years, behavioral pain assessments are recommended, including the:

The last 2 may be particularly useful for children with developmental/intellectual delay or autism.

See Pediatric Pain Assessment & Rating Scales.

Co-occurring Conditions

Anxiety

Although it is not a type of pain, it is important to realize that anxiety about and focus on pain, particularly with ongoing acute episodes of pain, such as frequent IV infusions, for example, may worsen the pain experience for children. See Procedural Anxiety.
Anxiety regarding pain can be magnified by a mental process called catastrophization, where even relatively small amounts of pain consume a large part of the child’s and the family’s thought processes. If present, this should be addressed by behavioral health experts for both the child and family.

Inadequate sleep

Many children with pain have difficulty falling and staying asleep. Sleep should be optimized; typical sleep hygiene, including sleeping in a quiet, dark, cool place, a sleep schedule, and decreasing use of electronics before bedtime, should not be ignored. Sleep Medications on a short-term basis may be needed to get a typical pattern started. Please see Behavioral Techniques to Improve Sleep and Sleep Medications.

Management by Type of Pain

Acute Pain

Acute pain has a recent, obvious onset and is expected to resolve within a short period (days to weeks). Examples include pain from injury, headache, and post-surgical pain. These should be treated with over-the-counter pain medications or, rarely, prescription non-steroidal medications or opioids as needed. See Acute Pain in Children & Adolescents.
Treatment of this type of pain is similar for typical children and CYSHCN; however, a key challenge for clinicians and families of children who are developmentally delayed/intellectually disabled, have autism spectrum disorder, or are too young to describe their pain lies in understanding when and to what degree they are in pain. A first step is to assume that pain expression will be at the developmental level of the child. For example, a 12-year-old with the intellectual reasoning of a 4-year-old will express pain at about the 4-year-old level.

Ongoing Acute Pain

Recurrent or prolonged acute pain, such as that observed in children with spastic or dystonic cerebral palsy, can be challenging. Although cerebral palsy is not a progressive condition, musculoskeletal complaints due to spasticity or dystonia often worsen over time. [Lomax: 2020] [Ostojic: 2019] Surveillance of children with cerebral palsy and spina bifida is key to identifying this type of pain. Ongoing pain can also result from a variety of conditions, including deposition of mucopolysaccharides in Hunter syndrome and cancer and its treatment. The Gustave-Roussy Child Pain Scale (DEGR) was designed for children 2 to 6 years old with prolonged pain due to cancer.
Over-the-counter pain medications and opioids are the basis of treatment for most ongoing acute pain; however, other medications, such as long-acting opioids and gabapentin-like medications, may also be prescribed. Complicating factors include adverse interactions, such as respiratory depression, with other medications being taken. [O'Connell: 2019] [Cirillo: 2019]
Regional anesthesia, used in conjunction with other pain treatments, is an option for severe pain – local anesthetics are placed close to relevant nerves to block pain signals from the affected region of the body from reaching the brain. This approach includes epidural catheters and those placed at peripheral nerves and may be used both inpatient and at home.
Children with these conditions will often need help from pain specialists, which involve a pain clinic associated with a hospital, rehabilitation medicine, anesthesiologist, palliative care, or critical care, depending on local availability. As many of these conditions are progressive, a Hospice referral may be indicated once the patient approaches the last year of life. This can be difficult to predict in children with medical complexity.

Neuropathic Pain

Neuropathic pain is poorly understood but is found in many situations, such as phantom limb pain, hereditary motor sensory neuropathy, and Charcot Marie Tooth syndrome, post-chemotherapy, post-herpetic neuralgia, reflex sympathetic dystrophy, and local nerve damage (causalgia). This type of pain typically does not respond well to over-the-counter medications or opioids.
The type of pain may respond to modalities like transcutaneous electrical nerve stimulation (TENS), physical therapy, acupuncture, or gabapentin-like medications. Those modalities are likely best used as part of an integrated multidisciplinary approach or clinic. [Szok: 2019] [Wilmshurst: 2019]

Chronic Pain

Chronic pain refers to processes where peripheral pain receptors have a prolonged reaction to pain due to release of biochemical mediators (e.g., prostaglandins, cytokines) and peripheral and central sensitization that amplifies the perception of pain. Although not causative, depression, exhaustion, anxiety, and stress exacerbate the pain response. Once chronic pain has started, it can be very difficult to treat and will usually require a multidisciplinary approach that may include cognitive behavior therapy. It is important in this type of pain to avoid opioids as they do not work and may prolong the chronic pain process. [Landry: 2015] [Fisher: 2018]
Headaches, which may have acute and chronic pain qualities, are addressed in the Portal’s Headache (Migraine & Chronic).
Treatment options for chronic pain may include medications, cognitive behavioral therapy, holistic approaches, children’s pain programs, and spiritual community and support. See Chronic Pain in Children & Adolescents.

Prevention

Children who will be exposed to large numbers of procedures or will have ongoing pain issues (e.g., rheumatoid arthritis) should be followed by therapists, including Child Life, while in the hospital and Behavioral Health, to manage and minimize long-lasting effects of acute pain and preventing more complex, chronic pain leading to pain chronification. A multidisciplinary approach is usually needed.

ICD-10 Coding

ICD-10 offers many codes that are specific to the location or type of pain. These are generally found in the section related to the body system or anatomical part, such as abdomen pain (R10.xxx), headache syndromes (G44.xxx), and spine pain (M54.xx).

Under G89 (pain, not elsewhere classified), there are 12 codes for acute and chronic pain related to trauma, post-thoracotomy, other postprocedural, and neoplasms, along with pain not elsewhere classified, and chronic pain syndrome (G89.4), defined as associated with significant psychosocial dysfunction.

G89.2, chronic pain, not elsewhere classified, the broadest billable code below G89 excludes all the localized pain types, as well as the complex regional pain syndromes (G56.4 and G57.7) and reflex sympathetic dystrophy (G90/5).

See ICD-10 Coding for Pain Not Elsewhere Classified (icd10data.com) for more details.

Authors & Reviewers

Initial publication: July 2020; last update/revision: February 2024
Current Authors and Reviewers:
Author: Zainab Kagen, MD
Authoring history
2020: update: Dominic Moore, MD, FAAPR
2020: first version: Lynne M. Kerr, MD, PhDA; Deirdre Caplin, Ph.D., MSA; Joan Sheetz, MDR
AAuthor; CAContributing Author; SASenior Author; RReviewer

Page Bibliography

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Reliability of the visual analog scale in children with acute pain in the emergency department.
Pain. 2012;153(4):839-42. PubMed abstract

Beltramini A, Milojevic K, Pateron D.
Pain Assessment in Newborns, Infants, and Children.
Pediatr Ann. 2017;46(10):e387-e395. PubMed abstract

Cirillo A, Collins J, Sawatzky B, Hamdy R, Dahan-Oliel N.
Pain among children and adults living with arthrogryposis multiplex congenita: A scoping review.
Am J Med Genet C Semin Med Genet. 2019;181(3):436-453. PubMed abstract

Collins A, Mannion R, Broderick A, Hussey S, Devins M, Bourke B.
Gabapentin for the treatment of pain manifestations in children with severe neurological impairment: a single-centre retrospective review.
BMJ Paediatr Open. 2019;3(1):e000467. PubMed abstract / Full Text

Fisher E, Law E, Dudeney J, Palermo TM, Stewart G, Eccleston C.
Psychological therapies for the management of chronic and recurrent pain in children and adolescents.
Cochrane Database Syst Rev. 2018;9:CD003968. PubMed abstract / Full Text

Harris J, Ramelet AS, van Dijk M, Pokorna P, Wielenga J, Tume L, Tibboel D, Ista E.
Clinical recommendations for pain, sedation, withdrawal and delirium assessment in critically ill infants and children: an ESPNIC position statement for healthcare professionals.
Intensive Care Med. 2016;42(6):972-86. PubMed abstract / Full Text

Hauer J, Houtrow AJ.
Pain Assessment and Treatment in Children With Significant Impairment of the Central Nervous System.
Pediatrics. 2017;139(6). PubMed abstract

Hauer JM.
Pain in Children With Severe Neurologic Impairment: Undoing Assumptions.
JAMA Pediatr. 2018;172(10):899-900. PubMed abstract

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Orthopedic Conditions in Adults with Cerebral Palsy.
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O'Connell N.
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Ostojic K, Paget S, Kyriagis M, Morrow A.
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Quinn BL, Solodiuk JC, Morrill D, Mauskar S.
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Szok D, Tajti J, Nyári A, Vécsei L.
Therapeutic Approaches for Peripheral and Central Neuropathic Pain.
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Wilmshurst JM, Ouvrier RA, Ryan MM.
Peripheral nerve disease secondary to systemic conditions in children.
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