What do we Know About Pain in Cerebral Palsy?

Irén is a four-year-old girl with dystonic quadriplegic cerebral palsy, GMFCS level V. She is non-verbal and generally a content little girl.  Over the last week she has been holding her muscles tensely, she has a distressed expression for most of the day, and has developed a behavior of constant grunting and gasping.  Her parents come to PT to seek your opinion.  Is she in pain?

Pretest:

Take a moment to scan of your caseload and note the kids you think may be in pain.

How do you know?

Do you have any measurement tools?

Children with CP frequently experience pain:

• Pain in childhood disability is typically under-identified, measured, and treated by clinicians.
•  Pain in childhood disability is complex.  It impacts everyday function and quality of life.  As pain increases, quality of life dramatically decreases.  Parental stress levels increase.
• Pain partially accounts for the association between cerebral palsy and depression. (Whitney, 2018)
• There remains a gap in knowledge between accurate pain assessments and effective treatments.

Challenges to Identifying and Measuring Childhood Pain:

• Many children are unable to self-report pain due to decreased communication, decreased cognition, and physical limitations.
• Proxy reports by caregivers are less accurate. Whenever possible, self-report is the gold standard starting place for pain assessment, especially in kids eight or older.
• The presence of multiple pain sources complicates the ability to isolate the location of the pain.
• There are many limitations with existing pain measurement tools.  This is why it is important to choose the right measurement tool for each unique child (taking into consideration their age, GMFCS level/motor level, cognitive level, and whether they will self-report or have a proxy report)
• As clinicians, we need to accurately measure pain in order to treat pain.

Who is Likely to have Pain?

• A study of a population of children born 1993-2008 with CP living in Skåne, Sweden in 2018 (data from CPUP, a follow-up surveillance program for people with CP in Sweden).  This study demonstrated that GMFCS/MACS level V was associated with the highest prevalence of pain (50%/54%) and level I with the lowest (30%/32%). The GMFCS/MACS Level II group had the second highest prevalence of pain.  Pain was most prevalent in dyskinetic CP (46%) and least frequent in unilateral CP (33%). Feet, knees and hips were the most dominant locations.  (Westbom, 2017)
• A study of 148 three to nineteen-year-old children with cerebral palsy with GMFCS level I-V were assessed at two time points (at least 6 months apart) in Toronto, Canada. The Health Utilities Index 3 (HUI3) was used as a measure of pain and its effect on daily activities. High gross motor level  (GMFCS level 1) patients were likely to have decreased pain at the second visit.  This was not true of the lower gross motor level (GMFCS level V) patients who overall did not have decreased pain levels at the second visit. This was thought to be due to more complex pain presentation and less easily treated pain etiologies. Children with high pain and high gross motor level were the most likely to have a positive outcome. Pain sources were:  hip subluxation/dislocation, contracture, spine deformity, foot deformity, abnormal gait, spasticity/dystonia, constipation, post surgical, acid reflux, choking.  (Christensen, 2016)
• A study of pain prevalence, frequency and intensity in non-verbal children 2-20 years old via parent report.  The children were outpatients in Kuala Lumpur and Klang Valley, Malaysia.  The majority (51%) of children studied were GMFCS V, 65% had spastic quadriplegia. The parents filled out the Comforts and Emotions (section 3) of the Caregiver Priorities of Life with Disabilities (CPCHILD) Questionnaire, as well as the Depression, Anxiety and Stress Scale (DASS-21) and Multidimensional Scale of Perceived Social Support (MSPSS). Physical therapy (stretching) was associated with the most intense and frequent pain. Toileting was also associated with pain.  Older children had more pain.  The study suggests re-examining the role of stretch in physical therapy, especially in light of recent studies which have not proven its effectiveness.  This study established pain as a major priority area in management of children with CP, especially spastic quadriplegia. (Jayanath, 2015)
• A systematic review examining prevalence and characteristics of pain in children and young adults with cerebral palsy reviewed 106 publications from 57 studies, concluding that pain was higher in females, older age groups, and those who are classified in GMFMCS V.  Pain was most common in lower extremities, back and abdomen, and is associated with lower quality of life or health status. (McKinnon, 2018)

A Few Measurement Tools:

• Non-Communicating Children’s Pain Checklist-Revised (NCCPC-R)can be used in all GMFCS levels, but mostly IV & V. Two-hour behavioral observation. 3-18 yrs.   A score of 7 or more indicates that a child has pain with 84% accuracy.
• Revised Face, Legs, Activity, Cry and Consolability Scale (r-FLACC)) The r-FLACC is an observational pain measure based on the Face, Legs, Activity, Cry and Consolability scale (FLACC), a well-established pain measure for young, pre-verbal children.  For each category (Face, Legs, Activity, Cry and Consolability), Total scores range from 0 to 10, higher scores indicating more pain. Observation time of five minutes. 1+ year
• Neonatal Infant Pain Scale (NIPS)  Children less than 12 months.
• Gait Outcomes Assessment List (GOAL) 7-16 yrs- pain/discomfort/tiredness section parent report, ambulant w/ CP.  Evaluates pain/fatigue cycles.
• Caregiver Priorities & Child Health Index of Life with Disabilities(CPCHILD) GMFMCS IV & V. Section 3: Comfort & Emotions. 5-19 yrs. During the past 2 weeks,how often did your child experience pain or discomfort.
• Faces Pain Scale, Revised (FPS-R) 4-17 yrs, best >7 yrs.  Acute situation, self-report,

Now that we know this, what can we do?

• The saying in PT goes: “Motion is lotion!” However,  watch closely for behavioral signs of pain, especially during activities that lengthen muscles.  Using appropriate pain observational tools, therapists can begin to more accurately read the signs of pain stress during a session.
• Use the GMAE-2 software from CanChild to classify GMFCS level in children with CP. It gives the percentile rank for each level.  Know who your GMFCS V’s are and your II’s.
• Follow the Australian Hip Surveillance Guidelines for Children with Cerebral Palsy to identify hip subluxation/dislocation based on the current GMFCS level.
• Follow postural management programs to reduce/ prevent hip migration in children GMFCS III-V
• Use the Hypertonia Assessment Tool to identify spasticity and dystonia. Know your kids that have a component of dystonia.
• Pay attention to abdominal discomfort (gas, constipation, adhesions from surgery).  Institute of Physical Art Proprioceptive Neuromuscular Facilitation (PNF 1), Functional mobilization (FM1) and Dynamic Core 4 Kids Online (DK4K) by Heart Space Physical Therapy for Children.
• Unusual pain situation that requires a different measurement tool?  Refer to the  Chronic Pain Assessment Toolbox for Children with Disabilities when searching for a specific measure.
• Quality of life in children with cerebral palsy is important!  The Study of Participation of Children Living in Europe (SPARCLE) executive summary is a good place for an overview.  In addition to the CPCHILD questionnaire mentioned above, and the Pediatric Quality of Life Inventory (PedsQL) are also frequently used.

In conclusion/Post-test:

Thinking of children on your caseload, who do you think may be experiencing some level of pain?

What tools would will you use to find out more?

Take the Kahoot Challenge to test your knowledge

Pain resources for other diagnoses:

• Medbridge online CEUs :  Pain Management for Children– Susan Stralka, PT, DPT, MS.  This is a comprehensive overview of patterns of physical, affective, sociocultural, behavioral and cognitive changes present in pediatric pain.  The speaker also discusses areas of pain such as Ehlers-Danlos syndrome, sickle-cell disease, complex regional pain, as well as current treatment concepts.
• Joint Hypermobility spectrum: is associated with increased levels of joint pain, increased fatigue levels, and heightened anxiety. Cincinnati Children’s Evidence Based Care Guideline for Identification and Management of Pediatric Joint Hypermobility (4-21 yrs/no spasticity)