A preview of different features from the Cerebral Palsy Research Network’s website leading to blog post ‘CPRN Turns Five!’

CPRN Turns Five!

The Cerebral Palsy Research Network (CPRN), founded in the summer of 2015, has reached its fifth anniversary. Born out of a 2014 workshop organized by the National Institutes of Health (NIH) and first designed to achieve a national registry for cerebral palsy (CP), CPRN has emerged as a multi-focus initiative and one that has given rise to a lasting partnership between the community and clinician researchers to improve outcomes for people with CP.

A 5-year strategic plan created in 2016 led to the realization of the following major milestones:

  • Engagement of community, clinical and research stakeholders to set and prioritize a patient-centered research agenda. The engagement was accomplished through the establishment of a Community Advisory Committee and the execution of two patient-centered research setting initiatives called Research CP, the first of which was funded by the Patient Centered Outcomes Research Institute (PCORI). This research agenda guides CPRN’s research focus and is influenced, dynamically, via ongoing community engagement in MyCP.org which was launched in the spring of 2019.  MyCP.org is a web portal that provides the community with opportunities to engage in CP research by participating in surveys and/or discussions with clinicians and researchers.
  • Establishment of registry infrastructure to accelerate CP research. This infrastructure includes two registries: a clinical CP registry and a community CP registry. The clinical registry includes data from more than 4,000 patients from 17 enrolling centers and will grow as more of the 28 centers committed to hosting the registry contribute their patient data. The community registry, where community members register themselves and contribute their data and perspectives on CP, launched in 2019 with the inaugural annual survey of adults with CP.
  • Facilitation of multidisciplinary research collaboration. Clinician researchers from multiple disciplines and multiple centers are tackling pressing and patient-centered research questions and quality improvement initiatives designed to improve outcomes for persons with CP. CPRN has submitted multiple grants and has received funding from NIH, PCORI, non-governmental organizations and private foundations for projects such as studying epilepsy in CP, genetics in CP and speech and language predictors of participation in CP. CPRN has also internally funded five quality improvement initiatives: adult care, dystonia, hip health, intrathecal pump infections, and care transitions, all of which seek to rapidly change clinical processes to improve outcomes for persons with CP.

The next five years promise new centers joining CPRN, more data collection and contribution from existing centers, and the development of many new patient-centered research and quality improvement initiatives. The anticipated growth will advance our development as a Learning Health Network that continuously improves treatments and outcomes for CP through research and quality improvement. Our focus over the next five years will give us more ways to engage the CP community and enhance outcomes locally, nationally and internationally.

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.

A preview of pie charts showing the membership of the MyCP portal leading to blog post ‘MyCP.org reaches 1000 participants’.

MyCP.org reaches 1000 participants

MyCP.org, a web portal supporting community engagement in research for cerebral palsy (CP), crossed the milestone of 1,000 users this past week! MyCP.org is owned and operated by the Cerebral Palsy Research Network (CPRN) to provide a place for members of the extended CP community – people with CP, parents and caregivers, advocates, clinicians and researchers – to collaborate on advancing research to improve outcomes for people with CP. Community members can interact to share experiences, advise on research priorities, or learn about existing evidence for various medical treatments. Physicians and therapists that both treat and research the condition of CP often engage in the conversation or seek community input.

MyCP provides several functions for the community including:

Members of the community may contribute their experiences with each other and connect with researchers seeking to understand the experiences of people with CP and partner together for advancing research.  In addition to participation in research, the forum provides members with an opportunity to verify the latest evidence in support of medical and surgical treatments.  For example, this week a community member sought knowledge about an orthopedic surgery and received a reply from a renown CP orthopedic surgeon within 24 hours. (Please note: that MyCP is not a place to ask for medical advice, rather it is a learning and support environment where evidence for the general population may be shared. Talk to your medical team for specific information about you or your loved one’s condition, their specific needs or to understand how existing evidence may or may not apply to your situation and goals. )

MyCP is a resource for the whole community and our programming has been generously promoted by the CP Foundation, CP NOW and UCP.  CPRN thanks these organizations for the sharing our studies with the broader CP community. We look forward to reporting the results of the research back to their audiences over time.

MyCP’s community is made up predominantly of adults with CP, parents of children with CP and medical providers who treat people with CP. It also includes researchers, advocates and industry professionals. People from the community make up 80% of the participants and clinicians are 14%.  Of the 813 members of the community, 58% are people with CP and 37% are parents. There is also a teen only forum. There are many active studies for the community to participate in including “Speech and Language Predictors of Participation for Children with Cerebral Palsy” for the parents of children between 4-17 and the “Adult Study of Personal Wellbeing, Pain and Stigma.”  Please join the conversation and help make a difference in outcomes for people with CP!

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.
A preview image leading to blog post ‘Webinar: Patient Reported Outcomes and Shared Decision Making’.

Webinar: Patient Reported Outcomes and Shared Decision Making

Webinar: Patient Reported Outcomes and Shared Decision Making

Unni Narayanan, MD, MSc

The Cerebral Palsy Research Network (CPRN) announced that its next webinar in its MyCP Webinar Series will feature Unni Narayanan, MD, MSc who is a pediatric orthopedic surgeon at SickKids in Toronto, Canada on Monday, August 17, at 8 pm ET. The topic is “Can we make patient reported outcome measures useful to patients and parents?” Dr. Narayanan has developed several patient-reported outcome (PRO) measures to assess the effectiveness of surgical interventions in cerebral palsy (CP). Now his research is seeking to determine if those PROs can be used to facilitate a shared decision making between patients/caregivers and clinicians. Shared decision making is a process by which clinicians and patients or caregivers collaborate to understand the evidence base behind interventions and the tradeoffs between interventions or alternatives to surgeries and medications. Shared decision making is very important in CP because most surgeries are elective. Dr. Narayanan will present his recently funded research project being done in conjunction with CPRN and then take questions from attendees. The presentation will be approximately 20 minutes followed by an open ended Q&A.

Parents, caregivers and adults with CP can access the PROs developed by Dr. Narayanan by joining MyCP.org where they can be found in the CPRN Community Registry. After completing one of these measures, you can print out the results to share them with your CP physician and facilitate the shared decision making process now!

Interested participants need to register for the webinar to be sent instructions for joining.  Webinars will be recorded and posted for later viewing.  The MyCP Webinar series includes one presentation per month on different aspects of CPRN’s research studies.  Please join us!

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.

A group of men and women clinicians from the Weinberg Family Cerebral Palsy Center gather for a photo.

Columbia’s Weinberg Family Cerebral Palsy Center Joins CPRN

The Cerebral Palsy Research Network (CPRN) announced that the Weinberg Family Cerebral Palsy Center (WFCPC) from Columbia University’s Irving Medical Center has joined the network.  The WFCPC is CPRN’s first site in New York City and provides a large and diverse patient population, including children and adults, for the studies conducted by CPRN.  The participation in CPRN, led by Jason Carmel, MD, PhD, includes an interdisciplinary team from orthopedic surgery, rehabilitation medicine, neurology and other clinical disciplines contributing patient and intervention data to the CPRN Cerebral Palsy (CP) Registry.

The Weinberg Family Cerebral Palsy Center is dedicated to improving the quality of life for people of all ages living with CP by providing comprehensive care, conducting groundbreaking research, and educating medical professionals, patients, and caregivers about the latest advances in cerebral palsy care. Members of the Weinberg CP Center team conducted over 3,700 patient encounters in 2019. Pediatric and adult services include orthopedics, physical medicine and rehabilitation, neurology, genetics, and mental health, along with social work and care coordination services. In conjunction with its clinical care mission, the Center’s academic efforts strive to bridge basic science and clinical research to directly impact the lives of patients living with cerebral palsy.

 “The goals and values of the CPRN are perfectly aligned with those of the Weinberg CP Center, and the CPRN is an incredible resource for multidisciplinary research and collaboration,” said Dr. Jason Carmel. “Our team is excited to begin contributing to the CPRN’s efforts, as well as participate in projects and initiatives that will undoubtably lead to a better understanding of how we can improve the lives of our patients.”

A squadron of pelicans fly in a ‘V’ formation under a cloudless blue sky.

The “ideal” candidate for SDR

This is the second in a series of blog posts on selective dorsal rhizotomy (SDR) in adulthood by guest author, Lily Collison.

Selection criteria for SDR differs between institutions. Characteristics of the “ideal” candidate for SDR at Gillette include:

  • Aged 4 to 7 years.
  • GMFCS level I–III.
  • Primarily spasticity (as opposed to dystonia) that interferes with function.
  • Preterm birth history or injury in the late second or early third trimester of pregnancy.
  • Periventricular leukomalacia (PVL) confirmed by neuroimaging. (PVL is the brain injury that commonly results in the motor problems seen in spastic diplegia.)
  • Energy-inefficient gait.
  • Satisfactory muscle strength, generally defined as antigravity muscle strength at the hips and knees.
  • Fair or good selective motor control at the hips and knees. This means being able to partially isolate joint movement (not moving the joint in a complete pattern). This requires sufficient strength and motor control, i.e., not being reliant on increased spasticity for stability or movement.
  • Good ability to cooperate with rehabilitation.

Other than specific brain injury and age, Tommy met the selection criteria above. Tommy’s primary tone problem is spasticity (minimal dystonia) and the degree of spasticity was problematic for him. Though he does not have the classic brain injury (PVL), it was felt that his brain injury would behave similar to one, in causing spasticity. It would have been far more ideal had Tommy undergone SDR as a child. Tommy missed the opportunity to have it then, as we lived in Ireland and by the time I learned of SDR, he was already aged nine and needed orthopedic surgery to address the muscle and bone problems that had already developed. (Today, Irish children who meet defined selection criteria are able to access SDR in the United Kingdom.) SDR in childhood is better than in adulthood because the older the person, the longer they have been experiencing the negative effects of spasticity on their muscles. SDR in childhood is also better because rehabilitation after surgery (of any type) is more prolonged in adults than in children–adults heal more slowly than children.

Despite the above SDR in adulthood is still beneficial–it reduces the negative effects of spasticity on muscles over a person’s lifetime. Tommy is only 26 with a normal life expectancy. Apart from preserving his muscles, Tommy’s walking will hopefully become more energy efficient, which should translate into greater endurance in walking. Before SDR, his energy consumption in walking was 2.4 times normal. By one year post-op, it is hoped that this will have improved somewhat–it still won’t be normal (nor near normal) but any improvement is valuable. Although SDR is adulthood is beneficial, it is a big consideration. The most difficult short term challenges (i.e., following the surgery and during early rehabilitation) for independent adults following this type of surgery include loss of independence, loss of ability to care for others, and loss of income.

In the next post I’ll address surgical decision-making.

Lily Collison is author of Spastic Diplegia–Bilateral Cerebral Palsy