[This post is part of our Knowledge Translation Tuesday series. Guest author Lily Collison, author of Spastic Diplegia — Bilateral Cerebral Palsy, continues the series on her journey with her son and cerebral palsy (CP). Author note: The is the view out over the Atlantic on Sunday as we climbed Knocknarea–the hill I pointed out in last week’s post.]

In coming to terms with our child’s CP diagnosis, we almost always ask the question why? This week I will write about cerebral palsy causes and risk factors. As we will see below, very often no specific cause is identified. This was the case with our son.

The term cause is self-explanatory. The term risk factor can be defined as any attribute, characteristic, or exposure of an individual that increases the likelihood of developing a disease or injury. Causes thus have a stronger relationship with CP than risk factors. Significant deprivation of oxygen to the infant’s brain, for example, is a cause of CP. Preterm birth is a risk factor but not a cause of CP—in other words, not every preterm baby is found to have CP. There are many possible causes of brain injury, including events before and during pregnancy, during birth, or in early infant life. Much is known about the causes and risk factors for CP, but much remains unknown as well. Depending on what you read, you may come across different lists of causes and risk factors for CP.

Causes of CP
Developing fetuses and infants (up to age two to three) can develop CP if they experience brain injury or disruptions in brain development caused by¹:

• Bleeding in the brain before, during, or after birth.
• Infections of the brain, including meningitis or encephalitis.
• Shock—a state in which organs and tissues do not receive adequate blood flow.
• Traumatic brain injuries, such as from a serious car accident.
• Seizures at birth or in the first month following birth.
• Certain genetic conditions.

Risk factors for CP
Risk factors for CP include¹:

• Preterm birth and low birth weight. A typical pregnancy lasts 40 weeks. Babies born before 37 weeks have a greater risk of having CP. The risk increases the earlier a baby is born and the lower the baby’s birth weight. Twins and other multiple-birth siblings are at particular risk because they tend to be born earlier and at lower birth weights.
• Serious illness, stroke, or infection in the mother. CP is more common in children whose mothers:
  – Experience certain viral and bacterial infections and/or high fevers during pregnancy.
  – Have coagulation (clotting) disorders or experience blood clots during pregnancy.
  – Receive excessive exposure to harmful substances during pregnancy.
  – Have thyroid problems, seizure disorders, or other serious health concerns.
• Serious illness, stroke, or infection in the baby. Infants who experience serious illnesses, strokes, or seizures around the time of birth are at greater risk of having CP. Such illnesses might include:
  – Severe jaundice. (Kernicterus is a rare kind of preventable brain damage that can happen in newborns with jaundice.)
  – Seizures during the first 48 hours after birth.
  – Infections of the brain, such as meningitis or encephalitis.
  – Strokes caused by broken or clogged blood vessels or abnormal blood cells.
• Pregnancy and birth complications. For example, not enough nutrition through the placenta or a lack of oxygen during labor and birth. Incompatible blood types between mother and baby.
• Genetic issues.

I will continue with part 2 next week.

¹Gillette Children’s Specialty Healthcare (2019) What Is Cerebral Palsy? [online].

[This post is the continuation of our Knowledge Translation Tuesday. Guest author Lily Collison, author of Spastic Diplegia — Bilateral Cerebral Palsy, continues the series on her journey with her son and cerebral palsy (CP).]

For a multitude of reasons, I believe it is very important for parents of young children and adolescents and adults with CP to fully understand the condition. I was that mom who didn’t understand my son’s diagnosis and therefore didn’t know how best to help him. My adult son now needs to have a good understanding to best help himself. When I was invited to write for CPRN to expand its knowledge translation objective, Paul (Gross) asked me to first write about SDR in adulthood because my son, Tommy had just undergone this procedure. Once I’d finished that series of posts, I debated whether it was best to work back to childhood or start from childhood. I was also conscious of recent data which showed that people who themselves have CP–adolescents/adults with CP are the biggest single group (58%) in MyCP. In the end, I decided to go back to the start and follow a logical sequence of subjects from childhood to adulthood. Issues in adulthood to some extent build on issues in childhood. It’s like the Wordsworth quotation “The Child is father of the Man.“

Let’s start with the actual definition of CP. Over the years there has been much discussion of the definition of CP, and different definitions have been adopted and later discarded. The most recently adopted definition, published in 2007, is as follows:¹

Cerebral palsy (CP) describes a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, perception, cognition, communication, and behavior, by epilepsy, and by secondary musculoskeletal problems.

(Here is a link to a table explaining each term in that definition.)

CP is a lifelong condition and there is currently no cure, nor is one imminent, but good management and treatment can help alleviate some or many of the effects of the brain injury. When the brain injury occurs is important. The consequences of a brain injury to a fetus developing in the womb are generally different from those of a brain injury sustained at birth, which in turn are different from those of a brain injury acquired during infancy. It is generally accepted that only brain injuries occurring before the age of two or three fit the definition of CP. A brain injury occurring after that age is called an acquired brain injury. This cutoff is due to the differences in brain maturity when the injury occurs. In my son’s case I’m not certain when his brain injury occurred–he was born after an uneventful pregnancy and delivery–most likely it occurred during pregnancy.

Returning to the definition of CP, although this definition is very useful, I’m not sure it sufficiently alerts us to the secondary conditions that may arise in adulthood. As O’Brien, Bass, and Rosenbloom (2009) explained, the definition was developed to be used in childhood—it was not intended to infer that progressive problems might not appear in adult life.²

(The photo is one I took yesterday evening at Rosses Point, Sligo, Ireland, where I live. Yeats had close connections with Sligo. The hill in the left background is Knocknarea at 1,073 ft. Tommy was very proud when he first climbed it as a child.)

¹Rosenbaum P, Paneth N, Leviton A, Goldstein M, Bax M (2007) A report: the definition and classification of cerebral palsy April 2006. Dev Med Child Neurol 49 Suppl 2: 8–14.

²O’Brien G, Bass A, Rosenbloom L (2009) Cerebral palsy and aging. In: O’Brien G, Rosenbloom L, editors, Developmental Disability and Aging. London: Mac Keith Press, pp 39–52.

Lily Collison, Author, Spastic Diplegia — Bilateral Cerebral Palsy

This is the third in a series of blog posts on selective dorsal rhizotomy 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.

Lily Collison, Author, Spastic Diplegia — Bilateral Cerebral Palsy

This week’s Knowledge Translation Tuesday article from the Cerebral Palsy Research Network (CPRN) is about the “ideal” candidate for a selective dorsal rhizotomy (SDR) but also details an example of SDR in adults.  Author Lily Collison describes why making a decision to get an SDR is different for adults.

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