Resources for adults with cerebral palsy, such as research and educational presentations, plus an interactive forum you can join today!

 

An old conical metal buoy standing on a windswept headland overlooking the ocean under a cloudy blue sky in the fading daylight

Exercise and physical activity in spastic diplegia – part 2

[This post is part of our Knowledge Translation/Education Tuesday series. Guest author Lily Collison, author of Spastic Diplegia–Bilateral Cerebral Palsy, continues the series. You can ask questions of the author on the MyCP Forum].

Thankfully, with our lockdown here in Ireland COVID case numbers are decreasing. The 5km restriction here means that both my photo and I have to stay local! The photo this week is of an old mooring buoy for ships (dating from 1908) that sits forlorn on the headland.

Last week I looked at exercise and physical activity in children and adolescents with cerebral palsy (CP) but what about in adulthood? Consistently strong evidence demonstrates that people with CP participate in less physical activity and spend more time engaged in sedentary behavior than their able-bodied peers throughout the life span [1]. Studies have shown that:

  • Adults with CP who reported preserved mobility throughout adulthood attributed it to regular physical activity, participation, and maintenance of strength, balance, and overall fitness [2].
  • Adults with CP who engaged in regular physical activity were at lower risk of decline in mobility. Deterioration in gait was strongly associated with inactivity [3].
  • “What are the best long term exercise/strength training strategies to improve activity, participation and health, minimize pain, and maximize function in each GMFCS category across the lifespan?” was the number one research topic identified in a collaboration between stakeholders, to set a patient-centered research agenda for CP (Research CP) [4].

The World Health Organization (WHO) notes that participating in regular physical activity reduces the risk of many health conditions, including coronary heart disease and stroke, diabetes, hypertension, colon cancer, breast cancer, and depression. Additionally, physical activity is a key determinant of energy expenditure and thus is fundamental to energy balance and weight control [5].

Verschuren and colleagues (2016) published a set of exercise and physical activity recommendations for people with CP under the following headings [6]:

  • Cardiorespiratory (aerobic) exercise
  • Resistance (muscle strengthening) exercise
  • Daily moderate to vigorous physical activity
  • Avoiding sedentary behavior (i.e., not being physically inactive)

The following table details their recommendations. Note that these are lifetime recommendations; it may take at least eight to 16 consecutive weeks of exercise to see the benefit. Their recommendations are similar to (and based on) the WHO’s guidelines for able-bodied people [5]. Though these recommendations are relatively recent, the concept that “exercise is medicine” is not new [7].

Verschuren and colleagues (2016) Verschuren and colleagues (2016) My notes
Type of exercise/physical activity Recommendations for people with CP  
Cardiorespiratory (aerobic) exercise ➡3 times per week
➡> 60% of peak heart rate*
➡Minimum time of 20 min per session
➡Regular, purposeful exercise that involves major muscle groups and is continuous and rhythmic in nature
This is the type of exercise that gets the heart pumping and the lungs working.
Resistance (muscle strengthening) exercise 2–4 times per week on non-consecutive days Muscle strengthening is especially important for people with spastic diplegia because muscle weakness is a feature of the condition. It is important for all muscles but particularly the antigravity muscles: the hip extensors (gluteus maximus) and the ankle plantar flexors (gastrocnemius and soleus). Other muscles to be considered include the hip abductors, the ankle dorsiflexors, the core muscles, and the upper limb muscles, if there is upper limb involvement.
Daily moderate to vigorous physical activity 60 minutes ≥ 5 days per week This is the ordinary movement we do in our everyday lives. Physical activity counts as long as it is moderate to vigorous. It is less taxing than cardiorespiratory exercise but is more vigorous than gentle movement. Walking, going up stairs, and household chores are all included in this category.
Avoiding sedentary behavior (not being physically inactive) Sit for less than 2 hours/day or break up sitting for 2 minutes every 30–60 minutes One can be physically active but still sedentary; they are separately measured. For example, if the person meets the recommendation for moderate to vigorous physical activity but sits for long periods watching TV or playing computer games, then they are physically active but sedentary. Prolonged sitting in one position, particularly with bad posture, is not good for any person, but it is particularly ill-advised in spastic diplegia.
*Peak heart rate can be approximated as 220 minus age. For example, at age 15, peak heart rate is 205 (220 –15). 60 percent of peak heart rate is approximately 120 beats/minute (205 x 0.6).

Note that there is no lower (or upper) age limit on the exercise and physical activity recommendations for people with CP. There is no denying these recommendations are very high. However, research has found that typically developing infants can take up to 9,000 steps in a given day and travel the equivalent of 29 football fields [8]. It is important to be aware of the recommendations and aim to meet them as much as possible. And remember, any activity is better than no activity.

References

Seen from behind, the ‘Waiting On Shore’ monument, depicting a woman with reaching arms by the shore in Rosses Point, Ireland.

Prevalence of Cerebral Palsy

[This post is part of our Knowledge Translation/Education Tuesday series. Guest author Lily Collison, author of Spastic Diplegia–Bilateral Cerebral Palsy, continues the series. You can ask questions of the author on the MyCP Forum].

The sculpture above “Waiting on shore” is located in our village (Rosses Point) on the Atlantic coast. It reflects the age-old anguish of seafaring people who watched and waited for the safe return of loved ones. It’s a gentle reminder to future generations to remember a proud history of courage and survival, of loss and grief.

In the last two posts, I addressed causes of and risk factors for CP. This week I’ll cover the prevalence of CP. The prevalence of a condition is how many people in a defined population have the condition at a specific point in time. Prevalence rates can vary geographically. A 2013 worldwide review found that the overall prevalence of CP was 2.11 per 1,000 live births*1. A recent (2019) study, however, reported that the birth prevalence** of CP declined across Australian states between 1995 and 20092. The percentage of children with CP whose disability was moderate to severe also decreased. A 2020 report on collaborative research between the European and Australian Surveillance Networks found similar decreasing prevalence of CP in Europe3. This is encouraging.

Some further points to note:

  • CP is the most common cause of physical disability in children4.
  • Males are at higher risk of CP than females. Data from Australia found that 57 percent of those with CP were male, while males represented 51 percent of all births5. This may be because males have certain nerve cell vulnerabilities that may result in CP6. It is noteworthy that there are frequently more male than female participants in CP studies.
  • Relative to its prevalence and its impact on the life span of those with the condition, funding for CP research is very low. The NIH reports research funding by condition. Although the reported prevalence of CP is twice as high as that of Down syndrome (0.2 percent versus 0.1 percent), funding allocated to CP research in 2019 ($28 million) was significantly lower than that of Down syndrome research ($86 million)7. Funding estimates for 2020 and 2021 are $29 and $26 million, respectively, for CP and $113 and $105 million for Down syndrome.
  • An analysis of National Institutes of Health (NIH) funding for CP research from 2001 to 2013 found that only 4 percent went toward studies of CP in adulthood8. Thus research on CP in adulthood receives only a small percentage of an already small budget.

*Births up to 2004.
**This was formerly referred to as “incidence,” but the term “birth prevalence” is now felt to be more accurate2.

1Oskoui M, Coutinho F, Dykeman J, Jetté N, Pringsheim T (2013) An update on the prevalence of cerebral palsy: a systematic review and meta-analysis. Dev Med Child Neurol 55: 509–519.
2Galea C, Mcintyre S, Smithers-Sheedy H, et al. (2019) Cerebral palsy trends in Australia (1995–2009): a population-based observational study. Dev Med Child Neurol 61: 186–193.
3Sellier E, McIntyre S, Smithers-Sheedy H, Platt MJ, SCPE and ACPR Groups (2020) European and Australian Cerebral Palsy Surveillance Networks Working Together for Collaborative Research. Neuropediatrics 51(2): 105-112.
4Graham HK, Rosenbaum P, Paneth N, et al. (2016) Cerebral palsy. Nat Rev Dis Primers 2: 1–24.
5Australian Cerebral Palsy Register (ACPR) Group (2013) Australian Cerebral Palsy Register Report 2013. [pdf] Available at: .
6Graham HK, Thomason P, Novacheck TF (2014) Cerebral palsy. In: Weinstein SL, Flynn JM, editors, Lovell and Winter’s Pediatric Orthopedics, Level 1 and 2. Philadelphia: Lippincott Williams & Wilkins, pp 484–554.
7National Institutes of Health (NIH) (2020) Estimates of Funding for Various Research, Condition, and Disease Categories (RCDC). [online] Available at: .
8Wu YW, Mehravari AS, Numis AL, Gross P (2015) Cerebral palsy research funding from the National Institutes of Health, 2001 to 2013. Dev Med Child Neurol 57: 936–941.

A golden-brown loaf of braided challah on a parchment paper covered tray resting atop the stovetop burners.

Adult SDR: update on my progress

This is the last in a series of blog posts on selective dorsal rhizotomy (SDR) in adulthood as part of Knowledge Translation Tuesday (KTT). Tommy Collison wrote this update at the recent five months post-op point. KTT will continue on CPRN on September 8, 2020.

Quickfire round:

Adult SDR: update on my progress

Challah Bread

  • Broadly, everything is great — zero complaints, except that gyms/pools/physical therapists are closed.
  • Generally feeling pretty solid on my feet. I had a fall a few weeks ago, but no ill-effects beyond some scrapes to my hand. I’ve been testing my standing endurance by cooking more — to the right is a pic of some challah I made last weekend. (Had the wrong kind of flour, it didn’t turn out just right. Reattempting right now with the correct flour: writing while it proves.)
  • I think it’s still a little bit early to try and guess at effects of the surgery, especially since it’s an apples-to-oranges comparison of my life pre- and post-surgery, but some things I’ve noticed:
    • More flexibility than before in certain muscles: imagine sitting on a chair and bringing your heel up onto the chair, as if you’re putting on a sock. I’m pretty sure I wouldn’t have been able to do that before.
    • General fatigue — this is the one I’m really excited about. Before surgery, if I had a big/ long day (gym → work for 8-10 hours → dinner with friends → home), I would feel it in my legs. Not pain so much as a… heaviness. Just a dog-tiredness. Not sure the right phrasing. I think that’s lessened or gone now. Yesterday, I hung out with friends in the backyard, went for a 6 mile cycle, and then walked downtown and back, getting back just after 10pm. I got into bed and it wasn’t the same sort of “whew, glad today is over” fatigue, where I feel grateful to be lying down and not having to do anything. There was general tiredness, but less muscle tiredness.

  • I’m staying down in Menlo Park, which is nice because it’s within walking distance (about a mile — 25 mins walk) to downtown, where I can get a takeaway coffee. I’ve had success with that sort of habit-stacking (need caffeine, also need to walk) and am walking downtown ~5 times a week.
  • Doing stretching and stretching in the interim. Probably not as much as I should, but PT and I are seeing progress, especially in the squats, and I’m sending her videos. Another interesting bit of progress: we used to practice sit-to-stands and they were SO difficult. The other day, I got out of a chair hands-free without thinking about it. Those little steps forward are so nice.

My Amazing Bike
  • Using two crutches + AFOs outside, and one crutch inside.
  • Got an AMAZING three-wheel bike that’s much safer, because three wheels mean that basically all balance considerations are taken care of. I have a nice 6 mile route that I’m doing 2-3 times a week, and I ordered a Peloton, so lots more cycling in my future.

Want to ask Tommy questions about his SDR? You can find him on the forum at https://cprn.org/ by posting a question with “Tommy” in the subject.

SDR surgery and early rehabilitation

This is the fourth of five in a series of blog posts on selective dorsal rhizotomy (SDR) in adulthood by Lily Collison — the inaugural author for Knowledge Translation Tuesday for the Cerebral Palsy Research Network (CPRN).  You can comment and discuss the article with Lily on MyCP.org.

Today I will explain a little more about selective dorsal rhizotomy (SDR) and then describe Tommy’s experience of SDR and early rehabilitation.

SDR only reduces spasticity, not other types of high tone. Of the various tone-reducing treatments (oral medications, botulinum neurotoxin injection, phenol injection, intrathecal baclofen, and SDR), SDR is the only irreversible tone-reducing treatment. What do the three words –“selective dorsal rhizotomy” mean?

  • Selective: Only certain abnormal nerve rootlets are cut.
  • Dorsal: “Dorsal” refers to the sensory nerve rootlets–it the sensory nerve rootlets that are cut. (The sensory nerve rootlets are termed “dorsal” because they are located toward the back of the body. The motor nerve rootlets are termed “ventral” because they are toward the front.)
  • Rhizotomy: “Rhizo” means “root,” and “otomy” means “to cut into.”

Putting it all together, “selective dorsal rhizotomy” means that certain abnormal, dorsal nerve rootlets are cut. SDR is a major operation, and the better the rehabilitation, the better the outcome is likely to be. Just as the operation itself varies between institutions, different institutions have different rehabilitation protocols post-SDR. Typically patients undergo intensive physical therapy lasting approximately one year starting in the first days after surgery.

Tommy travelled to St. Paul on Wednesday February 5, 2020 for tests on Thursday followed by his SDR surgery on Friday. The plan was that he would spend four weeks in St. Paul for the initial intensive rehabilitation and then return to work/continue his rehabilitation back in San Francisco, where he lives. My husband and I travelled to St. Paul to support him there. Tommy was admitted on the morning of surgery and wasn’t unduly nervous. (He even pitched the idea of a career change to the anesthesiologist–“Hey come to Lambda School–lots of people are changing career and learning to code” [Tommy works at Lambda School, an online coding school] ?.)

There are two SDR techniques, the cauda and conus, named after the level of the spinal cord at which each procedure is performed. The choice of technique is provider-specific but also depends on the patient. The cauda technique was used in Tommy’s case. Dr. Kim (neurosurgeon) performed the surgery with Dr. Ward (Physical Medicine and Rehabilitation physician) monitoring. The surgery involved removal of the back of the vertebrae (the lamina) in order to access the spinal cord. The dorsal nerve roots were dissected into rootlets, and the rootlets were individually electrically stimulated to determine whether they triggered a normal or abnormal (spastic) response. If a rootlet triggered an abnormal response, it was cut. If not, it was left alone. 30% of dorsal nerve rootlets from L2 to S1 were cut during Tommy’s six hour surgery.

For the first three days post-op, Tommy was confined to lying on his back to allow healing of the dura–the cover of the spinal cord. His pain level was manageable; he did have some stiffness in his back and some unusual sensations in his feet–numbness and hypersensitivity (likely due to the handling of nerve rootlets which would have caused temporary nerve damage). Wound healing progressed well. Three days post-op he was gradually brought to a sitting position and closely monitored for headache (to ensure that the dura was fully healed–no cerebrospinal fluid leakage). He received physical and occupational therapies as an in-patient. He wore knee immobilizers 50% of the time as per plan. He left hospital using a rented wheelchair four days post-op, a day earlier than scheduled. (Indeed, he was well enough to have dinner that evening in the restaurant of our hotel–a goal of Tommy’s.) Over the next three weeks, he attended twice daily out-patient physical therapy and did exercises at home. Posterior leaf-spring AFOs (PLOs) were prescribed and manufactured. During that time he progressed from using a wheelchair to walking with a walker and then to walking with two crutches. The altered sensations he felt in the immediate post-op period, diminished with time. Four weeks post-op (March 4th)–he returned home to San Francisco. Little did we know what was to unfold with COVID-19.

Here are some photos.

SDR surgery and early rehabilitation Day 4- Post op

Day 4 post-op: Leaving hospital.

Day 4 post-op: Leaving hospital.
Rough grey ocean waves crash against rocky brown-and-black cliffs under a grey misty sky.

Surgical decision-making

This is the third in a series of blog posts on selective dorsal rhizotomy (SDR) in adulthood by Lily Collison — the inaugural author for Knowledge Translation Tuesday for the Cerebral Palsy Research Network (CPRN).  You can comment and discuss the article with Lily on MyCP.org.

We hear a lot about evidence-based medicine. Evidence-based medicine combines the best available external clinical evidence from research with the clinical expertise of the professional. When Tommy was undergoing Single Event Multi-Level Surgery (SEMLS) at age nine in 2004, there were a number of outcome studies from different international centers supporting SEMLS. These outcome studies together with the expertise clearly evident at Gillette, gave my husband and me the confidence to take our nine year old abroad for surgery. This year, sixteen years later–whilst there are a large number of studies from many centers supporting SDR in childhood (including long-term outcome studies)–there is a dearth of research evidence supporting SDR in adulthood. I could find just two studies from one center. Research conducted by CPRN has shown that 5% of individuals who underwent SDR, were aged over 18 years. There is a need for more outcome studies evaluating SDR in adulthood.

Decision-making for undergoing surgical procedures such as SEMLS and SDR is interesting. Parents of young children and later the adolescent and adult themselves are co-decision makers with the clinician in the medical process. We, Tommy’s parents, were largely the decision makers for Tommy’s SEMLS at age nine. (He and I clearly recall discussing the proposed surgery on a long car journey–he was happy to proceed if we felt it was the right thing to do.) The decision to proceed with SDR this year was totally Tommy’s. Whilst it’s easy to understand that parents largely make the decision for children undergoing procedures and adults make the decision for themselves, there is a “grey area” when it comes to adolescents. Thomason and Graham (2013) made the very interesting point that adolescents must be given the freedom to make their own informed decisions about surgery and rehabilitation. They added that an adolescent who feels they have been forced into surgery against their will or without their full consent is likely to be resentful and may develop depression and struggle with rehabilitation. I fully support this view.

For Tommy’s surgery this year, he asked if as a “fly on the wall”, I would accompany him to the multidisciplinary appointment to decide if he was a suitable SDR candidate. Watching from that vantage point, a few thoughts struck me:

  • The evaluation truly was multidisciplinary. The three consultants discussed the surgery in detail together and with Tommy–it was a robust four-way discussion. The clinicians’ decision, that Tommy was a good SDR candidate was unanimous.
  • I was happy to observe that Tommy fully understood what was involved. When the possibility of SDR was first raised, he told me that he read that section in my book and felt it explained SDR very clearly for him (positive endorsement–our offspring are often our harshest critics!) Tommy was making an informed decision to proceed with the surgery–he was an effective co–decision maker in the medical process.
  • The fact that Gillette offers continuity of care for individuals with CP–from childhood right through to adulthood–is hugely important. Tommy has been receiving care at Gillette since he was nine. This continuity of care has benefits on so many levels. It also makes “handing over the baton for healthcare management” from parent to adolescent, so much easier.
  • As a parent, changing role from being an active participant in the medical process, to being a “fly on the wall” (and only then by invitation), takes discipline, but is so worth it.

Thomason P, Graham HK (2013) Rehabilitation of children with cerebral palsy after single-event multilevel surgery. In: Robert Iansek R, Morris ME, editors, Rehabilitation in Movement Disorders. Cambridge: Cambridge University Press, pp 203–217.

Author note: The photo was taken off the northern Californian coast. It was also there that I took last week’s photo of pelicans flying in V-shaped formation. One of the goals of SDR surgery is to reduce the energy cost of walking. Flying in a V-shaped formation is one of the tricks birds use to reduce the energy cost of flying.

CPRN Launches Study of Adult Health and Pain

Adults with Cerebral Palsy

CPRN launched study of adults with CP

The Cerebral Palsy Research Network (CPRN) launched its first study of adults with cerebral palsy (CP) today. The study is open to anyone with CP that is 18-years-old or older and is available on the web at https://mycerebralpalsy.org. The study consists of a series of surveys about an individual’s health, motor abilities, social and emotional health, and experience with chronic pain. It should take approximately 20 minutes to complete. The study of adults with CP was a key priority that was identified in the National Institutes of Health Strategic Plan for Cerebral Palsy (link) that was released in October 2017.

Mary Gannotti, PT, PhD

Mary Gannotti, PT, PhD, professor of Physical Therapy at University of Hartford

“The quickest way to get information about the issues adults with cerebral palsy are facing with aging, including functional changes, stigma, pain impact and pain treatments that work, is to ask adults with cerebral palsy,” said Mary Gannotti, PT, PhD, and co-chair of the CPRN Adult Study Panel. “We are thrilled to have MyCerebralpasly.org launch these surveys!” she added, “and we hope to people post their thoughts or comments about these topics on the website comments page!”

The adult study was designed by a panel of expert clinician researchers who treat adults with CP. The CPRN Community Advisory Committee provided input into the development of these studies. The Research CP initiative ranked the study of adults with CP as the highest priority research area and we are excited to launch this foundational study to address the needs of the community of people with CP.

Adult Patient Reported Registry Nears Completion

Clinicians and researchers of the Adult Study Group have been meeting regularly to refine questions for the CPRN Patient Reported Outcomes registry. Input from the adults with cerebral palsy on the CPRN Community Advisory Committee this summer indicated that consumers wanted more information about pain management, pain prevention, optimizing function with aging, and issues with social and emotional function.

The study group adapted a survey widely used to study pain and pain management. It is developing a survey to ask individuals about perceived changes in function with aging, how they are managing these changes, social and physical supports, community and employment activities, satisfaction with life, anxiety, and depression.

Two initial surveys are being created. The first survey will focus on pain, and will allow answers to questions about pain location, interference, and management.  It will provide useful information for clinicians and consumers.

The second survey will focus on physical, social, and emotional function. It will allow the adult study group to describe self-reported functional changes, social challenges and successes, and emotional struggles and victories.  It will provide useful information for clinicians and consumers.

The study group is planning to have the surveys piloted tested in December by the community advisors for release to the broader CP community in Q1 2017. People interested in CPRN surveys should sign up for CPRN alerts.

This is an important step to begin to address the needs of adults with cerebral palsy. Studying the adult population with CP was determined to be a critical need at the 2014 Workshop on the State of the science and treatments in CP that was held by the National Institutes of Health.

CPRN Expands Patient Engagement to Include Adults with Cerebral Palsy

The Cerebral Palsy Research Network (CPRN) has invited adults with cerebral palsy and caregivers of adults to join an adult registry study panel. The panel will partner with clinicians and providers who have led the creation of adult registry elements for the CPRN Registry. The panel will help determine study questions that are most important to adults with CP and have an opportunity to participate in the research process. The CPRN Adult Registry group is co-led by Mary Gannotti, PT Ph.D. and Debbie Thorpe, PT Ph.D. and includes providers from a wide range of disciplines who treat adults with (or children or both) CP. The adult registry study panel will be organized by Michele Shusterman of CP Now Foundation and CP Daily Living in her role as the Patient Engagement Leader on the CPRN leadership team. The panel members will represent a group of people with CP with diversity among age, race, gross motor function, and cognitive abilities.

CPRN plans to invite a similar panel to participate in the CPRN Registry efforts for children. Both groups will be extended opportunities to influence the overall research agenda for CPRN at a future planning meeting. The engagement of patient stakeholders in research has been demonstrated to enhance the impact of clinical research and is integral to the mission of CPRN.

Adult Cerebral Palsy Registry Completed

The Cerebral Palsy Research Network (CPRN) completed its adult cerebral palsy registry data elements. The adult planning group formed in July 2015 under the leadership of Mary Gannotti, PT PhD of the University of Hartford and Debbie Thorpe PT PhD of the University of North Carolina Chapel Hill. The diverse team included clinicians, therapists, social workers and educators that work with adults and children with CP including:

  • Anita Bagley, PhD, MPH Shriners of Sacramento
  • Hank Chambers, MD, Rady Children’s Hospital, and University of California San Diego
  • Marina Gazayeva, FNP-BC, Columbia University Medical Center, Weinberg Family Cerebral Palsy Center
  • Jill Gettings, MD, Gillette Lifetime Specialty Healthcare Clinic
  • Ed Hurvitz, MD, University of Michigan, Physical Medicine and Rehabilitation
  • Sherry Lanyi, MA, CCC-SLP, Perlman Center, Cincinnati Children’s Hospital Medical Center
  • Michael Msall, MD, University of Chicago Medical Center, Pediatrics
  • Garey Noritz, MD, Nationwide Children’s Hospital, Pediatrics
  • Gadi Revivo, MD, Rehabilitation Institute of Chicago, Physical Medicine and Rehabilitation
  • Beth Ann Loveland Sennett, EdD, University of Hartford, Department of Education
  • Robert Wagner, MD, Gillette Lifetime Specialty Healthcare Clinic
  • Lisa Zimmerman, LSW, Perlman Center, Cincinnati Children’s Hospital Medical Center
  • Marcia Greenberg, PT, MS, Center for Cerebral Palsy at UCLA

The adult cerebral palsy registry is an extension of the CPRN clinical registry that predominantly adds patient reported outcomes (PROs) to the existing pediatric focused registry elements. These PROs focus on quality of life issues including employment, education, functional decline, intimacy and pain among other areas. The adult cp registry is expected to provide valuable data for long term outcome research for cerebral palsy.

CPRN plans to integrate the adult cerebral palsy patient outcomes into its patient reported outcomes platform that will allow participation from adults who are not served by CPRN centers. CPRN plans to launch its adult cerebral palsy registry in the Fall of 2016.