Why I wrote “Spastic Diplegia–Bilateral Cerebral Palsy”

Spastic diplegia, also known as bilateral spastic cerebral palsy (CP) or simply bilateral CP, is a subtype of cerebral palsy. It is a lifelong condition characterized by limb muscles that are extremely tight (spastic) with the legs more affected than the arms (diplegia). However, spastic diplegia involves much more than tight leg muscles. I wrote the book “Spastic Diplegia–Bilateral Cerebral Palsy” in conjunction with senior medical staff at Gillette Children’s Specialty Healthcare (hereafter referred to as Gillette), in Minnesota, to provide a detailed explanation of spastic diplegia and to show how the condition develops over time. The book describes the best management and treatment of the condition (at the time of writing) in childhood, adolescence, and adulthood.

My third son, Tommy, was born at term in Ireland in 1994. His older brothers, then aged four and six, had been easy babies, so by then my husband and I felt quite relaxed as parents. Tommy was born after an uneventful pregnancy and delivery, but from birth he cried incessantly. At three weeks our family physician insisted that I give up breastfeeding. She could see how frazzled I had become due to Tommy’s constant crying, his difficulty with feeding (many feeds were being returned), and the fact that neither he nor I were getting much sleep. 

At three months the incessant crying suddenly stopped; Tommy became a serene, happy, and placid child, and we all relaxed again. However, a few months later, at a routine developmental check, he was deemed “developmentally delayed.” That started a long journey, beginning with the diagnosis of spastic diplegia when he was a year old and continuing with the management and treatment of his condition in our community as well as at the Central Remedial Clinic (CRC) in Dublin. In the early years he also received conductive education, and in adolescence he had a number of surgeries at Gillette. Tommy, now an adult, is a college graduate, working full-time and living an independent life in the US.

Why a book on spastic diplegia was needed

CP is the most common cause of lifelong, childhood-onset physical disability in most countries, with an estimated prevalence of 17 million people worldwide. Approximately one-third of those with CP have spastic diplegia. Spastic diplegia is thus a common subtype of CP, affecting an estimated 6 million people worldwide—roughly the population of Ireland. If the book helps a tiny fraction of those affected by spastic diplegia, writing it will have been worthwhile.

A book on spastic diplegia that is accessible to laypeople did not exist. It did not exist when Tommy was diagnosed approximately 25 years ago, and it still did not exist when I wrote one. Many of the specialist centers that treat CP had well-developed websites, but again, they did not offer much information specific to spastic diplegia.

Good texts on CP existed, but many covered all subtypes of CP together. Explaining the different subtypes together is like having one book to explain all types of cancer and their treatments. Each subtype of CP is different, and each has a different prognosis for mobility and associated health challenges. Subtypes vary from generally mild to severe. Anyone with a condition is understandably rather selfish—they only want to learn about their specific condition. If my diagnosis is throat cancer, I only want to learn about throat cancer. If my diagnosis is spastic diplegia, I only want to learn about spastic diplegia. I do not need to know about health challenges that do not concern me.

Spastic diplegia is complex. How can a baby born with what appear to be typical bones, muscles, and joints grow into the young child who may walk with a gait typical of a child with spastic diplegia? Although spastic diplegia is a complex condition, it becomes more understandable when logically explained.

Spastic diplegia is a lifelong condition, and we parents are key influencers of outcomes. My lack of understanding always held me back. My background is in science, and I was thirsty for knowledge that would further my understanding of the condition. In those early days, before the internet, I scoured bookshops in any city I visited, but all I could find were books on CP in general. The first book I found that offered specifics on spastic diplegia was one written by Dr. Jim Gage (1991), an orthopedic surgeon at Gillette who has since retired. (This book was recommended to me by Tommy’s community physical therapist, who had previously worked at Great Ormond Street Hospital in London and had visited Gillette.) Though I learned a lot from it, this book was not fully accessible to me because it was written for medical professionals. It took me many hours and much hard work to find the information I needed to understand the condition. That hard work culminated in my completing a master’s degree over a decade ago—a case study providing a detailed picture of single-event multilevel surgery (SEMLS) and rehabilitation, and evaluating outcome.

The book seeks to provide the reader, with an understanding of spastic diplegia without having to invest so much time and effort. Indeed, Bailes and colleagues (2018) pointed out that most of the time, health professionals underestimate the amount of information parents and caregivers want about their child’s condition. This problem extends to adulthood: lack of information about their condition was the highest area of unmet need reported by young adults with CP. This included information on complications, consequences, and causes of CP.

The parent of a child with spastic diplegia does not have the full, long-term view of how spastic diplegia develops over time. Medical professionals have the benefit of their training and their clinical experience to put things in context, but a parent does not. In the early days, I felt like I was trying to do a jigsaw puzzle without having the picture on the box as a reference. I had pieces but did not understand how they fit together. I wanted to help my child walk, but his physical therapist wanted me to help him roll. 

A good explanation gives us parents a much better understanding of what we can and cannot change about our child’s condition. Greater understanding should give us more confidence when helping our child. I so often had doubts: “am I doing this correctly?” Greater understanding should lead to greater motivation to carry out the exercises we are given to do at home.

Parents also put in a huge effort to prevent future, possible events—events of which we have no experience. Success is avoiding negatives. In some ways, that feels unsatisfactory. I’d never seen the problems resulting from excessive W-sitting; they were as vague to me as being told as a young Catholic child, “Don’t do this or you won’t go to Heaven.” Heaven was vague to the younger me. Hip subluxation and contractures were vague to the older me. I’d never seen them, nor the problems they caused. People who have plenty of money do not really understand the pain of poverty. People who have plenty to eat do not really understand the pain of hunger. People who already understand do not really feel the pain and frustration of just not understanding. Many prospective parents read books such as What to Expect When You’re Expecting to understand the different stages of pregnancy; my book could just as easily have been called What to Expect When You or Your Child Has Spastic Diplegia.

Having a book just for spastic diplegia protects against unnecessary anxiety. Books and websites on CP frequently include a long list of other possible problems separate from the movement disorder, such as epilepsy or learning disabilities. However, the presence of other problems largely depends on the CP subtype. Generic lists are not helpful and cause unnecessary anxiety for us parents, who already have enough to worry about. The opposite also holds true: if I have a child who is unlikely to ever walk independently, what use is it to read about gait analysis? 

On the subject of anxiety, some may argue that if parents read the book—a book which details the issues associated with spastic diplegia in childhood, adolescence, and adulthood—they will not be able to handle the full story and will become even more anxious. I disagree. I would counter by noting that we parents find out quickly that our child’s spastic diplegia is not going to go away. We see adolescents and adults with spastic diplegia in treatment centers and in the community, which gives us an idea of how the condition develops. Ignorance is not bliss. Fear of the unknown can be a bigger problem. Indeed, if we are given the long-term picture of how our child’s particular condition will develop, it may be better than we fear. It may also mobilize us to early action. Outcomes in adulthood very much depend on management and treatment in childhood and adolescence (though spastic diplegia also presents its own challenges in adulthood). If the reader is a parent of a young child with spastic diplegia, I would advise them to read the book in full, including the chapter on adulthood, to gain a full-life perspective on the condition. 

Despite their best efforts, medical professionals use medical terms. This is a new language for us parents; we’re not familiar with terms like “adduction” and “dorsiflexion.” Professionals can either refrain from using such terms or continually explain them to parents. Alternatively, parents can gain an understanding of these commonly used terms. I think the latter is much easier all around. The book includes an explanation of this “new” language for parents.

Treatment of CP does not involve just one discipline. It involves professionals from a number of disciplines, including physical therapy (PT, also termed physiotherapy), occupational therapy (OT), speech and language pathology/therapy (SLP/SLT), nursing, orthotics, pediatrics, neurology, neurosurgery, orthopedic surgery, and physical medicine and rehabilitation (PMR, also termed physiatry). Within the multidisciplinary team, no one professional is responsible for explaining the condition to parents; it is very ad hoc. At appointments, there is little time for explanation because the child is present and their treatment takes priority. (In addition, the child will get bored if the adults are talking too much.) Having medical professionals explain the condition to parent after parent is an inefficient system. It would be preferable, in my opinion, to have well-developed resources available to parents, and professionals can then answer specific questions. 

Parents are co-decision makers with medical professionals. Unless we have a good understanding of the condition, how can we contribute in a meaningful way to this very important process? How can we be effective advocates for our child? A survey of 1,214 parents and caregivers of children with CP found that they judged available medical information to be inadequate to guide their decision-making. Educating the parent is investing in a most vital member of the child’s multidisciplinary team. Obviously, in time the role of the parent passes on to the adolescent—and then the adult—themself.

How can we know if our child is getting the best management and treatment if we don’t know what best management and treatment looks like? In an ideal world, management of spastic diplegia for all individuals would be at the current limits of medical science. My book details the best treatments available and the evidence base supporting each one. I regret that I did not know about selective dorsal rhizotomy (SDR) back when it would have been a treatment of choice for Tommy. (For a number of reasons, the “ideal” candidate for SDR is aged four to seven years; reasons include that secondary contracture development is still minimal. By the time I learned of SDR, Tommy was nine and had already developed contractures.)

Parents are also bombarded with treatment ideas from well-meaning family and friends. In addition to describing evidence-based treatments, my book includes information on treatments that have been disproven or still lack an evidence base.

My book also explains the expert consensus on the best management plan for spastic diplegia. I would encourage readers to ask about their center’s management plan, which may not exist—but if it doesn’t, asking about it puts pressure on centers to develop one. I also hope that the book puts pressure on centers to provide the best, evidence-based treatments—or, in their absence, to facilitate access to centers where such treatments are available. Treatment should not be limited by zip or postal code.

The world is changing. In all areas of medicine, patients now want good information. My book is motivated by that drive.

How the book was written

I was convinced of the need for the book but was not sufficiently knowledgeable or qualified to write it alone. I proposed the idea to Dr. Tom Novacheck at Gillette, and I am eternally grateful that he understood the need. Under his stewardship and with the great help of Jean Stout, Amy Schulz, and Candice Johnson, the book exists today. Indeed, the book bears testament to the fact that at Gillette, parents and professionals work well together.

The title of the book is Spastic Diplegia, Bilateral Cerebral Palsy. Throughout Tommy’s life the term “spastic diplegia” was used to describe his condition, and this term remains in use in the US today. Over the past 20 years, the term “bilateral spastic CP” or simply “bilateral CP” has been adopted in Europe and Australia because it is thought to provide a more accurate description of the condition. If Tommy were diagnosed in Ireland today, his condition would be called “bilateral CP.” Indeed, all three terms are used in the scientific literature.

The Gross Motor Function Classification System (GMFCS) is a five-level classification system that describes the functional mobilities of children and adolescents with CP. Level I has the fewest limitations and level V has the greatest. The GMFCS offers an indication of the severity of the condition. The book is relevant to those at GMFCS levels I–III: people who are capable of walking independently or with a handheld mobility device. GMFCS levels I–III account for the majority of children, adolescents, and adults with spastic diplegia. Because self-mobility is limited in spastic diplegia, GMFCS levels IV and V, these levels are best addressed in the book Children and Youth with Complex Cerebral Palsy.

At GMFCS levels I–III, the descriptors “spastic diplegia” and “bilateral spastic CP” are largely interchangeable. For simplicity, I used just one descriptor for the condition in the text. Because “spastic diplegia” is the descriptor with which I was familiar, and because of its continued use in the US, I chose to use it throughout the text. The book equally applies to people with bilateral spastic CP (or simply bilateral CP), GMFCS levels I–III. Indeed, while the book focuses on spastic diplegia, much of what is addressed also applies to other forms of spastic CP at GMFCS levels I–III (hemiplegia and spastic quadriplegia).

Problems with bones, muscles, and joints (musculoskeletal problems) and with walking are generally the most significant issues one encounters in spastic diplegia, GMFCS levels I–III. Because of this, the development and management of musculoskeletal and mobility problems is the main focus of the book. However, children may have problems in other areas, such as with communication or with using their hands. These problems are beyond the scope of the book.

Though each chapter can be read independently, many build on information presented in previous chapters. It is best to first read the book in its entirety to get an overall sense of the condition; after that, the reader can ignore chapters that are not currently relevant and revisit them only if they become pertinent. Throughout the book, medical/scientific information is interspersed with personal experience. Orange-colored boxes are used to separate personal experience from the other information. In addition, one chapter is devoted to the experiences of people who live with this condition. My son Tommy wrote the epilogue. At the back of the book is a glossary with definitions of key terms.

A companion website for the book is available here. This website contains several resources, including: all “Useful web links” included throughout the book, collated on one page.

For simplicity, throughout the book I refer to parents and children; I acknowledge, however, that family structures vary. The term “parent” is used as a generic that includes grandparents, relatives, and carers (caregivers) who are raising a child with spastic diplegia.

Who the book is for

The book is aimed at parents of young children with spastic diplegia as well as adolescents and adults who have the condition. It should also be useful for teachers, extended family members, and students taking undergraduate therapy and other courses (such as special education). It should be helpful for members of multidisciplinary teams because it provides a holistic view of spastic diplegia. A greater understanding of spastic diplegia should help answer many of the questions people have, keep pressure on treatment centers to provide the best evidence-based treatments, and improve quality of life for people who live with this condition.

From reading the book, readers will learn that:

  • Spastic diplegia arises from a brain injury. The brain injury causes problems with movement, and as a consequence, over time, muscle growth and bone development are affected. Intelligence is typically not affected in spastic diplegia.
  • There is currently no cure, nor is one imminent.
  • People with spastic diplegia have a relatively normal life expectancy.
  • The severity of spastic diplegia (i.e., GMFCS level) can be determined by age two. It is not possible to be certain of severity before this age because the baby’s brain is still developing.
  • Spastic diplegia is generally mild or moderate, not severe. 
  • Almost all children with spastic diplegia GMFCS levels I–III walk in childhood.
  • A number of treatments are used individually or in combination (e.g., therapies, home program, orthoses, and tone reduction). These treatments are used to increase stretching (to stimulate muscle growth) and to facilitate the development of mobility. The aim is to prevent or delay the onset of muscle and bone abnormalities. The muscle and bone abnormalities that may develop may be addressed with orthopedic surgery.
  • Disability can increase with age, and aging can occur earlier than normal in those with spastic diplegia. Good management in childhood and adolescence can optimize outcomes in adulthood. 
  • Though spastic diplegia affects muscle and bone development and mobility, Paralympians with spastic diplegia are able to compete in a variety of sports. They are proof that spastic diplegia does not have to be a barrier to achieving great levels of fitness and skill.
  • The chapter on adulthood includes studies which determined the percentage of adults with CP who, for example, had chronic pain, experienced a decline in walking, or were unemployed. There is much readers can do to ensure that they or their child will be among those adults with CP who do not have chronic pain, do not experience a decline in walking, and who are employed.
  • There is much that the parent, adolescent, or adult can do to control how much spastic diplegia affects their lives.

The above is an extract from Spastic Diplegia–Bilateral Cerebral Palsy available here.

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