Pain in Children with Special Health Care Needs

Overview

Many kinds of pain affect children and youth with special health care needs (CYSHCN), some of whom have intellectual disability that makes assessing and understanding their pain more challenging and others who have ongoing acute pain as part of their condition.

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) 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).

Helpful Articles

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

Miró J, McGrath PJ, Finley GA, Walco GA.
Pediatric chronic pain programs: current and ideal practice.
Pain Rep. 2017;2(5):e613. PubMed abstract / Full Text

Pearls & Alerts

Parent intuition
Parents with CSHCN are sometimes frustrated when primary care, emergency room, and other clinicians don’t 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. Notably, this type of irritability may respond to gabapentin. [Collins: 2019]

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 cause include ear infections, dental caries, fractures, constipation, skin lesions, urinary tract infection, and abdominal pain.

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. Medications for sleep 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 cause 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.
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.
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 are discussed in [Quinn: 2018].
The last 2 may be particularly useful for children with developmental/intellectual delay or autism.

Ongoing Acute Pain

Ongoing 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.

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 include:

Medications

There is little evidence to guide the use of medications for pain relief in children. Generally, children should be started on a low dose of a medication familiar to the clinician, which should then be increased as needed slowly while carefully watching for side effects. Absent over-riding concerns, start with an over-the-counter medication, such as ibuprofen, acetaminophen, or naproxen. Topical agents may be tried alone but are usually used as an add-on for pain and a counter-stimulant/distraction (See Behavioral Health considerations below). Opioids should be used only when necessary and with a definitive plan for their use and, ideally, in consultation with a pain service.
If acute pain medications aren’t helpful, consider adding a medication that can change the pain sensation over time, including the gabapentin-like medications gabapentin and pregabalin, the tricyclic antidepressants amitriptyline and imipramine, and the serotonin norepinephrine reuptake inhibitors (SNRIs) duloxetine and venlafaxine. Carbamazepine, oxcarbazepine, and phenytoin are used for trigeminal neuralgia.
Treatment of peripheral and central neuropathic is addressed by [Szok: 2019].

Cognitive Behavioral Therapy

Families are often uncertain about the role of behavioral health in pain management. Cognitive Behavioral Therapy (CBT) is a widely researched, time-limited therapeutic approach shown to be effective for managing pain and other related conditions. CBT management of chronic pain, either alone or as a part of an integrated pain management program, is successful for improving function over time. Evidence suggests that improved functioning also improves quality of life for a variety of chronic pain conditions.
CBT is a talk therapy where the therapist works with a patient to analyze and redirect thoughts, feelings, and behaviors related to headache and response to headache in a way that improves management by learning new ways of using thoughts and behaviors to control how the brain responds to pain signals. Key components of CBT for pain are:
  • Self-management training, including pacing and learning to accomplish things in a thoughtful and systematic way
  • Relaxation training, which could include biofeedback, relaxation techniques, hypnosis, mindfulness, and others
  • Behavioral Activation that seeks to increase a child’s or adolescent’s ability to engage in activity
  • Maintaining exercise, sleep, nutrition, and hydration needs
Insurance benefits vary, and these therapies fall under mental health coverage, even though they are addressing a medical problem. Contact the insurance company for specific coverage information.

Holistic Approaches

An increasing body of evidence suggests that a holistic approach to pain management is likely to be more effective than a pharmacologic approach. Pain is a biopsychosocial construct and typically requires a comprehensive and interdisciplinary approach.
Nonpharmacologic options are often helpful as part of a comprehensive multidisciplinary plan, including psychological therapies, acupuncture, meditation, aromatherapy, music therapy, pet therapy, play therapy, and others. Physical therapies such as massage, ultrasound, stretching, and transcutaneous electrical stimulation (TENs) may be helpful and are generally prescribed by a physiatrist.
Learning behavioral management and tolerance skills, including counter-stimulation, distraction, cognitive control, and relaxation, can provide considerable relief for many children with chronic pain.

Children’s Pain Programs

If treatment is not effective using the above approaches, local resources, such as a palliative care service, pediatric pain clinic, or anesthesia service at a local children’s hospital, should be considered.
Interdisciplinary pain programs are designed to include pediatric patients as part of an active treatment team, focused on the person rather than the pain. These programs provide patients and families with a holistic approach to pain treatment and involve a comprehensive team to help children in pain live a full life. Because chronic pain is a complex problem, it is important that a pain team provides children with the physical and psychological components needed for success.

Spiritual Community and Support

Sometimes the role of faith in a person’s pain will need to be addressed. A simple spiritual assessment may be helpful with the acronym “FICA:”
  • Faith - does the person have a faith or spiritual belief system?
  • Importance - how important is that belief in their life and in their understanding of the illness/condition? Sometimes people feel they are being punished by God, or that God has abandoned them, or are confused as to why a benevolent God would allow this to happen to them.
  • Community - is their faith community available to them for daily support, spiritual support, etc.?
  • Address in your care - how should issues of faith be addressed in their care? Don't mention it, bring in the chaplain, pray for/with them, etc.
See the FICA Spiritual History Tool for more information.

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 leading to pain chronification. A multidisciplinary approach is usually needed.

Resources

Information & Support

Behavioral Management of Pain
Behavioral management and tolerance skills, including counter-stimulation, distraction, cognitive control, and relaxation, which can provide considerable relief for many children with chronic pain.

For Professionals

Caring for Children Who Have Severe Neurological Impairment: A Life with Grace
Written by an expert physician, this book empowers parents to make informed decisions about their child’s care.

Pediatric Pain and Symptom Management Guidelines (PDF Document 98 KB)
An excellent resource for caring for children with pain from Julie Hauer and her group at the Dana Farber Cancer Institute at Boston Children’s Hospital.

Services for Patients & Families Nationwide (NW)

For services not listed above, browse our Services categories or search our database.

* number of provider listings may vary by how states categorize services, whether providers are listed by organization or individual, how services are organized in the state, and other factors; Nationwide (NW) providers are generally limited to web-based services, provider locator services, and organizations that serve children from across the nation.

Authors & Reviewers

Initial publication: July 2020
Current Authors and Reviewers:
Authors: Lynne M. Kerr, MD, PhD
Deirdre Caplin, Ph.D.
Reviewer: Joan Sheetz, MD

Page Bibliography

Bailey B, Gravel J, Daoust R.
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

Fournier-Charrière E, Tourniaire B, Carbajal R, Cimerman P, Lassauge F, Ricard C, Reiter F, Turquin P, Lombart B, Letierce A, Falissard B.
EVENDOL, a new behavioral pain scale for children ages 0 to 7 years in the emergency department: design and validation.
Pain. 2012;153(8):1573-82. PubMed abstract

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

Landry BW, Fischer PR, Driscoll SW, Koch KM, Harbeck-Weber C, Mack KJ, Wilder RT, Bauer BA, Brandenburg JE.
Managing Chronic Pain in Children and Adolescents: A Clinical Review.
PM R. 2015;7(11 Suppl):S295-S315. PubMed abstract

Lomax MR, Shrader MW.
Orthopedic Conditions in Adults with Cerebral Palsy.
Phys Med Rehabil Clin N Am. 2020;31(1):171-183. PubMed abstract

Malviya S, Voepel-Lewis T, Burke C, Merkel S, Tait AR.
The revised FLACC observational pain tool: improved reliability and validity for pain assessment in children with cognitive impairment.
Paediatr Anaesth. 2006;16(3):258-65. PubMed abstract

Miró J, McGrath PJ, Finley GA, Walco GA.
Pediatric chronic pain programs: current and ideal practice.
Pain Rep. 2017;2(5):e613. PubMed abstract / Full Text

O'Connell N.
Clinical management in an evidence vacuum: pharmacological management of children with persistent pain.
Cochrane Database Syst Rev. 2019;6:ED000135. PubMed abstract

Ostojic K, Paget S, Kyriagis M, Morrow A.
Acute and Chronic Pain in Children and Adolescents With Cerebral Palsy: Prevalence, Interference, and Management.
Arch Phys Med Rehabil. 2019. PubMed abstract

Quinn BL, Solodiuk JC, Morrill D, Mauskar S.
CE: Original Research: Pain in Nonverbal Children with Medical Complexity: A Two-Year Retrospective Study.
Am J Nurs. 2018;118(8):28-37. PubMed abstract

Raffaeli G, Cristofori G, Befani B, De Carli A, Cavallaro G, Fumagalli M, Plevani L, Mosca F.
EDIN Scale Implemented by Gestational Age for Pain Assessment in Preterms: A Prospective Study.
Biomed Res Int. 2017;2017:9253710. PubMed abstract / Full Text

Szok D, Tajti J, Nyári A, Vécsei L.
Therapeutic Approaches for Peripheral and Central Neuropathic Pain.
Behav Neurol. 2019;2019:8685954. PubMed abstract / Full Text

Wilmshurst JM, Ouvrier RA, Ryan MM.
Peripheral nerve disease secondary to systemic conditions in children.
Ther Adv Neurol Disord. 2019;12:1756286419866367. PubMed abstract / Full Text