Some life care planners use a normal life expectancy for children with cerebral palsy. Is that appropriate?

Except for children with the mildest cerebral palsy, it is clearly erroneous to state that life expectancy is normal. In an article published in the New England Journal of Medicine in 1990, Eyman and colleagues reported on 99,543 persons with developmental disability from the California Department of Developmental Services. The best predictors of mortality were (1) deficits in cognitive function, (2) limitations on mobility, (3) incontinence, and (4) inability to eat without assistance. These investigators provided a life table analysis of survival, based on defining 3 subgroups of children:

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  Subgroup 1: immobile, not toilet trained, required tube feeding

  
  
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  Subgroup 2: immobile, not toilet trained, but could eat with assistance

  
  
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  Subgroup 3: mobile but not ambulatory and could eat with assistance

Subsequently, a significant error in data manipulation in Eyman and colleagues’ study was pointed out, resulting in life expectancies that were too low. The corrected life table for subgroups 1, 2, and 3 is shown in Table 1 .

This was the first of many articles in the medical literature that reported a reduction in life expectancy for most persons with cerebral palsy.

What factors are important in predicting cerebral palsy?

The life span of the child with cerebral palsy is curtailed by the presence of certain key disabilities, summarized in Box 2 . Decreased cognitive abilities are associated with diminished life span, even in the absence of physical impairment. Generally, life expectancy for physically and mentally disabled persons has increased compared with life expectancies from many decades ago, but generally not in the past 10 to 20 years.

The findings in the medical literature with respect to life expectancy and cerebral palsy are consistent. One of the key problems in comparing survival in different population groups of children with cerebral palsy is comparing prognosis in children with similar severity of disability. Some investigators chose more broad categories of disability and others more narrow ones, complicating the analysis. However, subsequent reanalysis of data using similar categories of disability show marked homogeneity in outcomes.

Are there opinions contrary to the mainstream in the cerebral palsy survival literature?

The cerebral palsy survival literature contains some data that vary markedly from the balance of the literature. In 1998, a study was published of 371 children with cerebral palsy in 3 registered nurse (RN)-staffed nursing facilities in the Chicago area. This study divided its study population into groups based on functional abilities in the manner of Eyman to compare statistical results. Survival rates for groups 1 to 4 were notably longer, reaching both statistical as well as clinical significance. The study population was small; only 251 children had significant mental impairment and cerebral palsy. The number of children in groups 4, 5, and 6 were only 11, 9, and 2, respectively. The study population was all inpatient, a notable distinction to the thousands of patients in the California cohort. Details of the data analysis were not provided, leaving the reader uninformed as to the statistical methods used.

Subsequently, it was shown that a Χ ² method was used to analyze the data, a type of statistical test that is not appropriate for a survival analysis. The article was rejected by the American Journal of Children because of statistical problems. Furthermore, the subject count seems confusing: at 1 point, 371 children are reported to have cerebral palsy, yet later in the paper only 367 had. In deposition testimony, the principal author mentioned that patients with developmental disability comprised 31 of the patients, and none had cerebral palsy. The article did not consider the many studies included in topical reviews, instead concentrating only on Eyman’s studies. The study did not note any difference in outcome of children with tracheostomy, in contrast with other studies in the literature. Despite its flaws, the article supports the role of severity of mental retardation, presence and severity of epilepsy, and the importance of arm movement/mobility/rolling as important factors in predicting survival. The choice of journal for publication is questionable, because the publication is outside the literature arena of most people interested in this topic.

The same author published a follow-up study that extended observations to 447 children. Both articles suffer from statistical flaws that ensure that the survival probabilities were too high. The errors are 2-fold: (1) the author did not use a standard survival analysis and instead relied on an incorrect method of his own; and (2) in comparing his own work with Eyman’s, the author used the erroneous data published in Eyman’s original New England Journal of Medicine article, which provided overly pessimistic longevity figures because of a mathematical error, instead of the corrected data (see Table 1 ).

Has there been any improvement in life expectancy in children with cerebral palsy over the last 30 years?

Some data show that children in some subgroups of cerebral palsy are living longer. Investigators following a longitudinal cohort of children with cerebral palsy sought to determine whether there were changes in survival within their population (47,259 persons with cerebral palsy receiving services from the State of California) between 1983 and 2002. In children with cerebral palsy who had severe disabilities, and in adults who required gastrostomy feeding, the investigators found an improvement in life expectancy. Life expectancies reported in earlier studies should be increased by approximately 5 years based on the most recent mortality data. Although survival of these most fragile children improved, no significant increase in life expectancy was seen in other subgroups.

This finding corresponds closely with findings from a group of investigators from Israel who sought to identify risk factors for early childhood mortality with cerebral palsy. One thousand children sequentially referred to 1 development center were followed for more than 20 years. Eighty-one children died, and they were compared with 81 age-matched controls, and 81 developmentally disabled children without neurologic deficits. The principal findings were that (1) children with a lack of mobility or nonindependent feeding had a life expectancy into their mid-teens, and (2) socioeconomic variables were not associated with life expectancy.

Investigators from Sweden studied survival in children with cerebral palsy followed over 15 years. Survival status was known for 99.3% of the whole study population with cerebral palsy, a study strength. The estimated survival was 60% at age 19 years in children with the most severe gross motor limitations, as assessed by the Gross Motor Functional Classification System V (GMFCSV) and who required a percutaneous endoscopic gastrostomy (PEG) tube. GMFCSV indicates children who have the most severe motor deficit. Children with milder forms of cerebral palsy had considerably greater longevity. Gastrostomy tube feeding was associated with a 9-fold increased risk of dying, regardless of GMFCSV level. No difference in mortality was seen between boys and girls.

Australian investigators reported on the survival of individuals with cerebral palsy between 1970 and 2004. Individuals with cerebral palsy had an 80% survival rate to the age of 40 years. The inability to ambulate independently was the strongest predictor of mortality. Compared with those with mild motor impairment, the mortality risk of those with severe motor impairment was 30 times higher. In those with severe motor impairment, survival was determined by the number of additional impairments, including epilepsy, severe/profound intellectual impairment, blindness, deafness, and lack of speech. Consistent with previous findings in the literature, the most severely impaired children had life expectancies of 21 years. Thirty-five percent survived 30 years. Gastrostomy tube use was not presented. Confirming work of others, no improvement in survival was seen in the 30 years between the 1970s and 2000s.

Data from children with cerebral palsy in California and Sweden have been compared and provide additional evidence that survival probabilities are remarkably similar for severely disabled children in developed countries. As reported by the study investigators, “this may suggest that there are not major differences in the type and quality of care delivered in these countries.”

Is cognitive impairment without physical impairment associated with shorter life expectancy?

Life expectancy in children with developmental disability, but with normal physical function, has been well studied over the last 50 years. Diminution of life expectancy is strongly related to the severity of developmental disability. Recent studies continue to show this relationship. Two thousand three hundred and sixty-six persons from the Finnish Population Register Center were followed since 1962, with a mean follow-up of 26.9 years. For persons with mild developmental disability, life expectancy did not differ from the general population. In the group with moderate developmental disability, the decrement was slight, but in the severe and profoundly disabled groups, a 19% to 35% diminishment in life expectancy was observed.

Other researchers have found median life expectancies of 74.0, 67.6, and 58.6 years, respectively, for people with mild, moderate, and severe levels of handicap. Another group assessed excess mortality in persons with intellectual disability (non-Down and Down syndrome) compared with the general population. All-cause and disease-specific mortality were approximately 3 times higher in these groups than in the general population. The most profound differences were noted in persons in their third decade: all-cause mortality was about 9 times higher in men and about 17 times higher in women. In the group excluding those with Down syndrome, both males and females had a standardized mortality ratio of almost 3.0.

What is the life expectancy for children with cerebral palsy?

The life expectancy literature for children with cerebral palsy shows that children with mild cerebral palsy have a normal or near-normal life expectancy. Children with the most profound cerebral palsy have a life expectancy of 19 to 21 years. Intermediate degrees of severity of neurologic deficit are associated with intermediate life expectancy.

What are the initial factors for consideration?

After evaluating the nature and extent of the impairment, the life care plan was opined to be based on a life expectancy to 19 to 21 years, in accordance with findings in the literature presented earlier. In the process of litigation, the expert witness was asked to make a best estimate of future life expectancy. Because there is no crystal ball, the best estimate can be made by systematically reviewing the literature discussed earlier applied to the individual patient.

In terms of expenses, the 3 most important factors for any life care plan are (1) the amount of years surviving; (2) the amount, duration, and intensity of long-term care (ie, nursing services); and (3) the amount, frequency, and duration of therapy services. Thus, each of these factors should have an extensive justification.

What factors play in the estimation of future costs?

The International Academy of Life Care Planners standards of practice specifically outline the basic components and process for life care planning. The methodology remains consistent, although individual differences may present themselves. Within certain diagnoses, as described earlier, there are also consistent areas of impairment. Factors that may remain variable include analysis of the individual patient. Although the life care planning process should be objective and attend to the needs of the child, in litigation there may be opposite views or at least diverse plaintiff and defense views. Although it may be difficult for the novice to envision an appropriate life care plan for the child’s lifetime, it should be pointed out that the life care planner generally has but 1 opportunity to develop a plan (ie, if provisions are not included in the original life care plan, they likely will never be addressed again). What level of care should the life care planner provide for? The short answer is the level of care that is necessary and appropriate to meet the coordination of future medical and rehabilitation services to promote quality of life in the least restrictive environment with respect for independence and human dignity. It may be found in the litigation process that advocacy of the plaintiff and defendant may create a faux friction point regarding this issue. The standards of practice must be used as written, as published in peer-reviewed journals, and generally accepted within the international community of life care planners. The following comments are addressed by common categories within the life care plan.

Projected therapeutic modalities

In life care plans for children with cerebral palsy, the plan is often front-loaded with physical, occupational, and speech therapy. The life care planner should ensure that the plan is realistic in the implementations. If physical therapy, occupational therapy, speech-language pathology, and other therapies are recommended twice per week beyond school needs, little time may be available to successfully implement these therapies within the course of a day. At times, these additional therapies may substitute for the academic curriculum. The life care plan also requires appropriate foundation. Studies in adults with brain injury suggest that persons with moderate neurologic deficit benefit the most, whereas those with profound or minimal deficits less so. The life care planner should consider several issues: what is the evidence that therapies benefit children with cerebral palsy? Are therapy visits needed to supplement what is already provided within the school system? How many visits are needed? Do children with different severities of cerebral palsy have benefit, whereas others little to none? Are therapies necessary to maintain function so that it does not decline?

Physical therapy is typically started early in life in children with cerebral palsy and then continued by parents within the home. The parent’s ability to provide therapy in the home can be affected by several factors. Such factors require analysis, including the health of the parents, the number of siblings the child with cerebral palsy has, and the parent’s employment status. Investigators have suggested that therapy continue to age 12 or 14 years, after which the child plateaus and therapeutic goals are integrated into daily activities. In 1 study the investigators compared 48 infants 12 to 19 months in age who were randomly assigned to either 12 months of physical therapy or 6 months of infant stimulation followed by 6 months of physical therapy. The purely physical therapy group fared more poorly, and significant differences between groups persisted after 12 months. No significant difference was seen in the need for bracing or orthopedic surgery.

Family and individual counseling is another area of consideration. Life care planners may include extensive funds for counseling within the life care plan without due thought or consideration. Investigators have studied patterns in psychotherapy and noted that the average number of sessions is 8. The average number of sessions required to see improvement in about half of patients with depression/anxiety is 13 to 18, and the most dramatic improvement is seen between sessions 7 and 10. Again, individual assessment with appropriate foundation is required. The life care plan is not a theoretic construct.

Diagnostic testing and schooling

The life care planner needs to determine if a reasonable school experience is available within the child’s district, or if another educational/care experience needs to be considered. The adequacy of school systems to meet the needs of the disabled child is the subject of considerable controversy and litigation, which are outside the scope of this article.

The need for ongoing neuropsychological assessment of the child with cerebral palsy may be a routine part of the individualized educational plan, or it may not be performed routinely and needs to be assessed separately. However, although neuropsychological testing is undoubtedly useful, it can be overused and its applicability in real-world settings may be questioned. Consideration of the reasonableness and purpose of routine testing is needed. Is routine testing being completed to assess progress? Is it to aid in developing compensatory strategies?

Wheelchairs, facilitative devices, orthopedic equipment

Costs of durable medical goods are rarely the subject of significant debate. The need for an electric wheelchair is generally based on the child’s physical ability to operate a joystick or similar mechanical interface, and requires the cognitive ability to navigate the chair within their environment. Replacement schedules and amortization of such devices are covered elsewhere in this issue. Many experienced rehabilitation professionals have ongoing records from their clinical practice for appropriate contacts for goods and services for persons with disability, assembled after many years of research. Although the Internet provides ready access to goods, services, and costs, case managers and life care planners are the professionals who work directly with medical and rehabilitation cost identification. They are continuously researching the costs for current and future medical and rehabilitation treatment and services, equipment and other needs. These professionals understand the intricacies of medical billing practices. Although physicians have understanding within their practice, they may, if completing a life care plan themselves, request the services of an individual with this particular expertise.

Drugs and supply needs

Similarly, the costs of medications and disposable medicals should be identified primarily by the current provider of these drugs and supplies. The Life Care Planning Summit of 2012 held in Dallas, Texas resulted in the following consensus statement with respect to drug and supply costs:

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  Verifiable data from appropriately referenced sources

  
  
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  Costs identified are geographically specific when appropriate and available

  
  
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  Nondiscounted/market rate prices

  
  
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  More than 1 cost estimate, when appropriate

Life care planners should use generic medication costs when available. Although the price of a particular drug may be reduced substantially when the drug loses proprietary status and becomes generic, this may not provide a good estimate of future drug costs, because new proprietary drugs may be introduced at higher prices.

Home and facility care

The costs of home and facility care are the largest budget item in a life care plan and may not be fully discussed and justified. The Life Care Planning Summit of 2010, held in Atlanta, Georgia resulted in the following consensus statements pertaining to this topic. The results indicated:

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  When the life care planner includes home care, both private-hire and agency-procured services are options to be considered.

  
  
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  The cost of private-hire home care includes caregiver compensation and associated expenses.

Several points are worthy of further discussion:

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  Care for the disabled child requires extraordinary physical and emotional commitment. The life care planner needs to assess the costs of assistive care as well as respite care when scheduling future needs.

  
  
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  There may be a useful division in assistive care planning before and after 21 years of age. Up to age 21 years, care is most routinely provided by parents and schools. The individual need to care beyond basic parenting during these years should be clearly assessed. Thus, less assistive care may be needed before age 21 years.

  
  
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  The type of long-term care is potentially a sensitive issue. The option of putting someone in a long-term skilled facility is generally less expensive, but advocates for the disabled argue that such facilities are an unsatisfactory setting to care for this population, and they should be reintegrated into the community.

How should care be delegated and priced? Different levels of care are provided by different nursing professionals: certified nursing assistants (CNA), licensed practical nurses (LPN, also known as licensed vocational nurses in some states), and RNs. Different states have differing Nursing Practice Acts defined by their legislature. The issue of delegation has become increasingly prevalent, and careful understanding for those who choose to delegate and those who do not wish to take on this responsibility (liability) should be evaluated and understood. The costs associated with hiring such professionals depend on their level of compensation. Although these rates may vary geographically, costs range from approximately $18 to $20/h for a CNA, $38/h for an LPN/LVN, and $56/h for an RN. The choice of professional is generally determined by the skilled nursing needs of the child. For example, are gastronomy tube feedings needed? Does the child require any type of drugs that are administered intravenously? Is the child partially or completely ventilator dependent? Does the delegation of care to less qualified caregivers put the patient at an unacceptable risk of injury or death? Is the child partially or completely ventilator dependent? These impairments alone may dictate the level of care required. In less physically compromised matters, appropriate foundation is required for using services beyond the obvious.

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  Should the parents be expected to provide some of the care? The purpose of the life care plan is to promote independence in the least restrictive environment with consideration for safety and human dignity. In any setting, including litigation, the parents of a child with a diagnosis of cerebral palsy should undergo a clinical interview to consider the specifics of their individual family. Their ability to provide extraordinary care is likely determined by their health, number of other children, employment status, and other life roles.

  
  
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  It may seem that private-hire care givers can be obtained for considerably less money than those obtained through nursing care agencies; however, associated expenses may alter this bottom line. Nursing care agencies prescreen, perform background checks, cover mandated state and federal employer costs, and ensure staffing needs are met. When hiring privately, an added burden may be placed on the family or they may not possess the capacity to perform the employer duties required to obtain and maintain quality care. The literature is mixed on this issue and provides no clear answer whether survival is better or worse with home versus institutional care. The standards of practice speak to the need for thorough assessment of both options.

Future medical and surgical care

Rechecks with various specialties of physicians generally do not generate significant controversy within a life care plan. However, certain issues may arise:

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  Should routine medical care be scheduled in a life care plan that would be required regardless of disability (eg, routine pediatric care, routine internal medicine care)? Transparency for routine needs and consideration given at the time of assessment and life care planning are appropriate.

  
  
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  Items are included based on an appropriate foundation and their being medically reasonable, necessary, and appropriate. The life care planner develops recommendations for content of the life care plan cost projections for each client and a method for validating inclusion or exclusion of content.

  
  
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  Potentially controversial items include the need for a botulinum toxin, a baclofen pump for spasticity, or selective dorsal rhizotomy for spasticity, release of contractures, hip correction surgery for dysplasia, and scoliosis surgery. These subjects are dealt with each in turn.

  
  
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  Botulinum toxin: the efficacy of botulinum toxin in treating spastic hypertonia in the child with cerebral palsy is beyond the scope of this review, and readers are referred elsewhere. However, botulinum toxin is expensive (as much as several thousand dollars per treatment, with treatment effect lasting only 3 months), and the reduction of spasticity is not a goal in itself. Spasticity should be reduced when it interferes with care of the child, performance of activities of daily living, or gait. A randomized trial showed no difference in a family’s satisfaction with their child’s function after botulinum toxin injection, despite improvement in both spasticity and function.

  
  
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  Baclofen pumps are another expensive option that should be considered only when they ease the patient’s personal care or improve function. Baclofen pumps can cost as much as $15,000 when considering the charges of pump placement, pump replacement, programing and refilling, and the baclofen solution. Whereas oral baclofen is a generic drug, intrathecal baclofen is not. Again, readers are referred to other sources for comprehensive review. However, evidence suggests that pumps reduce tone in children severely affected with cerebral palsy, but less so in ambulatory patients, and they are associated with considerable adverse affects.

  
  
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  Selective dorsal rhizotomy surgery in patients with cerebral palsy has been debated for almost 25 years. Indications for the surgery are still not clear, and whereas some advocate the procedure in potentially ambulatory children with cerebral palsy, others advocate surgery for severely impaired children to ease perineal care.

  
  
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  Contracture surgery is a nonrecurring expense and does not add significantly to the total cost of a life care plan. It may be useful when it eases the care of a markedly contracted patient. Although it may provide benefits in the ambulatory patient, scores that measure gross motor function change little after surgery.

  
  
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  Hip dysplasia surgery is a nonrecurring expense and contributes minimally to the total cost of a life care plan. This surgery often alleviates the pain associated with the dislocated hip.

  
  
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  Scoliosis surgery in the severely disabled child is highly controversial. Although neuromuscular scoliosis surgery may decrease the severe curve, evidence that it prolongs survival is not clear. Furthermore, in the severely impaired child, the ethical dilemma of whether to subject the child to an extremely painful, complication-prone, and costly procedure (approaching $100,000 when including surgical, anesthesiologic, and prolonged hospital stay for critical support) must be considered.

Transportation and housing

When the child with a disability can no longer be lifted or transferred easily to a standard 4-door vehicle, transportation needs require attention. Key questions include the following: (1) is public transportation for the disabled person regularly available? and (2) should the entire cost of a wheelchair van be included in the life care plan, or only the modification costs? That is, if the typical cost of a wheelchair-accessible van is approximately $50,000, should the typical cost of a 4-door vehicle (approximately $26,000) be subtracted, leaving $24,000 as the cost included in the life care plan?

What adaptive housing costs should be included in a life care plan for a child with cerebral palsy? The Model Spinal Cord Injury Data Bank and the Veterans Administration provide useful figures for adaption of a home to wheelchair accessibility. Preliminary cost estimates based on the Department of Veterans Affairs allowance for veterans with service-connected disabilities are one-half the cost for modifications (up to a total of $63,780 allowed), to construct an adapted home or to modify an existing home.