FormalPara Key points for decision makers

There are currently few UK data on the rehabilitation costs of cases of meningococcal disease with lifelong sequelae

Lifetime rehabilitation costs are substantial for survivors of severe cases of disease and are particularly high during the early years after recovery

Lifelong costs associated with severe cases of meningococcal disease are presented in a format appropriate for use in cost-effectiveness analysis from a National Health Service/Personal Social Services and government perspective

Results from this costing exercise may be used by economic analysts to represent the cost of severe illness when looking at the cost effectiveness of immunization programmes against meningococcal disease

1 Introduction

Meningococcal disease is the foremost cause of bacterial meningitis in the UK, where it is also a major infectious cause of death in early childhood [1]. Although the majority of people who survive appear to recover relatively unscathed, some are left with severe disabilities, and their long-term care imposes a considerable burden on healthcare resources. Meningococcal meningitis can result in major sequelae, such as mental disability, seizures, hearing loss, motor impairment, hydrocephalus and loss of vision [2], while limb loss, growth plate damage, renal failure and scarring can occur in cases of meningococcal septicemia [3, 4]. According to a recent systematic review of studies in children surviving meningococcal disease, 4 % have hearing loss, 7 % have neurological sequelae, 3 % have amputations, 3 % have other orthopedic problems, and 13 % have skin necrosis and scarring [1].

Economic evaluations such as cost-effectiveness analyses are becoming increasingly important in determining whether immunization programmes should be implemented [5]. Accurate cost-of-illness data is a fundamental component of such an analysis; however, a study of cost effectiveness of meningococcal serogroup C conjugate vaccination pointed to a lack of published information in the UK of costs associated with the treatment and rehabilitation of survivors of meningococcal disease with serious after effects [6].

As Meningitis Research Foundation (MRF) has unique contact with families affected and professionals involved in acute care and rehabilitation, we have attempted to fill this gap in the current UK literature through an estimation of the lifetime rehabilitation costs associated with severe cases of meningococcal disease. We present these costs in a format appropriate for use in cost-effectiveness analyses.

2 Methods

We developed two severe scenarios of meningococcal disease with major sequelae; one that presented acutely as septicemia (patient A) and the other that presented as meningitis (patient B). Scenarios were based on MRF members who have experience of meningococcal disease with severe outcome and systematic reviews of the literature describing the sequelae of meningococcal disease [1, 2, 7]. The compiled scenarios were refined through discussions with intensive care pediatricians, pediatric neurologists, pediatric orthopedic surgeons and pediatric infectious disease specialists responsible for managing children with meningococcal disease over many years. They agreed that our final scenarios were representative of cases at the severe end of the spectrum.

Once we had outlined the scenarios, we interviewed the parents of six children with sequelae similar to those of patient A, and the parents of six children with sequelae similar to those of patient B. This enabled us to compose a list of health, social service, educational, and other resources used by patients during and after their acute illness. From this we identified and consulted with relevant professionals and academics in health, social care, and education. Consultation with these professionals allowed us to estimate the amount of National Health Service (NHS), local authority and Personal Social Services (PSS) resources that these patients would use. Lifelong needs for continuing care and support were considered as the child grew, attended school, and made the transition to adulthood.

Initial consultations were carried out over the telephone or in person and the MRF members’ experiences of resources were used as an initial template for discussion. Amendments to patient-reported resource use were made according to comments made by the consultees. A consultation document was then produced that detailed all of the assumptions made about the acute illness, immediate, long-term and late-onset sequelae, and consequent acute care, rehabilitation and long-term service provision. This document was sent to all of our professional consultees for validation and then amended according to the responses received. The revised document was then submitted to our consultees, who agreed that this was an accurate representation of resource use for such patients. Tables 1 and 2 summarize the NHS/PSS resources used by patients A and B, respectively. Full assumptions are provided in the appendix (Online Resource 1).

Table 1 Patient A’s National Health Service/Personal Social Services resource use
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Table 2 Patient B’s National Health Service/Personal Social Services resource use
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The resource use identified during the consultation period was costed following recent guidelines on costing of public services as part of economic evaluation [8]. Currently, UK cost-effectiveness analyses (CEAs) are confined to an NHS/PSS perspective [8]. However, there is continuing debate about whether broader perspectives should be used in economic evaluation of medical innovations [9], so we have presented costs from both an NHS/PSS and government perspective.

As the costs presented are distributed across a lifetime, discounting has been used to give less weight to future costs compared with current costs. In keeping with UK Treasury recommendations [10], all costs have been presented at a discount rate of 3.5 % for the first 30 years and at 3 % thereafter. Costs have been based on a lifespan of 70 years of age (7 and 12 years less than the respective average life expectancies for UK males and females [11]). Several pediatric specialists argued that the mobility limitations in these scenarios might give rise to cardiovascular problems that could shorten lifespan, and it has been reported that survivors of meningococcal septicemia report poorer general health than the population as a whole [12].

2.1 Unit Cost Data

All costs are expressed in pounds sterling. The costs of hospital stays, inpatient and outpatient appointments were derived from English Department of Health reference costs 2008–2009 [13]. Unit costs of community health and social services were largely calculated using unit costs of health and social care [14], although some costs such as short-break provision and educational services were based on independent research (Lam SC, personal communication) [15]. Medication costs were obtained from the British National Formulary [16, 17]. Prosthetic component costs were obtained from the NHS catalogue of prosthetic components at the NHS supply chain. All unit costs represent, or have been indexed to, 2010–2011 prices.

3 Results

3.1 Patient A

Patient A contracted meningococcal septicemia at 12 months of age, suffering severe septic shock, acute respiratory distress syndrome and renal failure. He also developed gangrene of the limbs due to purpura fulminans. He spent 31 days in the pediatric intensive care unit (PICU) during the acute episode. His respiratory and renal problems were resolved in the PICU, but both legs had to be amputated above the knee and one arm below the elbow. He also underwent various skin grafting operations during his initial hospital stay, which lasted a total of 6 months.

Once discharged from hospital, patient A became a lifelong outpatient of a disablement services centre for prosthetic limbs and attended regular appointments with the hospital pediatrician, orthopedic surgeon and plastic surgeon. Community therapists were involved in his rehabilitation at home and at school and he required specialist equipment to help him with his mobility. He experienced stump overgrowth and scar contractures on his residual limbs and as a result needed corrective operations throughout his childhood to trim his bones and alter the skin coverage of his amputation stumps.

At 4 years of age he started to display difficult behaviour. He saw a psychiatrist throughout his school years and took medication to help his concentration.

Patient A attended a mainstream maintained nursery and school. He had a special educational needs statement because of his physical and behavioural problems. During his early years he had a learning support assistant to help him with his mobility and daily physiotherapy exercises. He had free transport to and from school, and the school also underwent adaptations to accommodate his needs.

Patient A was one of two children in a two-parent family. After becoming ill, one parent gave up work to help care for him, which put a financial strain on the family. The child’s disability and the reduced family earnings entitled the family to a range of government grants and benefit payments.

Patient A went on to further education at age 18 years and at age 21 years he secured a part-time job and moved from his parent’s house into rented accommodation. Whilst he lived independently, he received direct payments which allowed him to employ a homecare worker for 7 hours a week to help with certain household tasks such as shopping. He worked 16 hours a week until he retired at the age of 65 years.

3.2 Patient B

Patient B contracted meningococcal meningitis at 3 years of age. She was hospitalized with raised intracranial pressure and intractable seizures, and required ventilation and intubation. Acute hydrocephalus was immediately treated by insertion of an external ventricular drain and later by insertion of a shunt. She required prolonged airways management for neurological complications, including repeated seizures. She spent 26 days in the PICU. Once stable she was transferred to a pediatric ward where she spent time recovering before moving to a neuro-rehabilitation unit for 5 months.

Patient B was left with severe neurological damage, including severe cognitive deficits, epilepsy, severe hemiplegia, homonymous hemianopsia, communication problems, and profound deafness. Although she eventually learned to walk, this was only for very short distances. She was predominantly a wheelchair user and had problems with her posture.

Once discharged from hospital, she had regular neurological outpatient appointments. She also had regular visits from community therapists and was provided with specialist equipment to help with her mobility and posture. She had bilateral cochlear implants and became a lifelong patient of the cochlear implant centre.

Patient B attended a special needs nursery and a maintained special needs primary and secondary school. Throughout her childhood and into adulthood she spent the occasional weekend in a residential unit so that her parents had a break from caring. Her parents also received direct payments so that they could employ home help for a few hours each week.

One parent gave up work to help care for Patient B shortly after she became ill, which put financial strain on the family. The child’s disability and the reduced family earnings entitled the family to government grants and benefit payments. Patient B lived with her parents until the age of 40 years, when they became too frail to care for her, and then moved into full-time residential care.

3.3 Costs

The lifetime government and NHS/PSS costs associated with both patients are outlined in Table 3. Prosthetic provision was the primary cost driver for patient A, followed by the hospital length of stay. The most costly aspect of care for patient B was the residential care home. Cochlear implantation was the most costly medical treatment that she received over her lifetime.

Table 3 National Health Service/Personal Social Services and government costs (discounted and undiscounted) according to category for patients A and B over a lifespan of 70 years
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The average undiscounted costs per year for patients A and B are shown in Tables 4 and 5, respectively. In both cases, considerable costs were accrued during the first year after disease onset. This is largely due to the prolonged stay in the PICU and the subsequent long spell in hospital undergoing multiple surgical procedures (in the case of patient A) and rehabilitation (in the case of patient B).

Table 4 Average yearly undiscounted cost for patient A (a severe case of meningococcal septicemia)
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Table 5 Average yearly undiscounted cost for patient B (a severe case of meningococcal meningitis)
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Yearly undiscounted costs to the NHS/PSS tend to be higher in the early years following the acute illness in both cases. For patient A, this is due to increased prosthetic provision in childhood, when limbs and their interfaces need to be changed more regularly to keep up with the growth of the child and due to skin stump fragility in the first years after healing, along with increased physiotherapy and occupational therapy soon after the amputation and throughout childhood. Increased costs for patient B were partly due to more intensive habilitation after cochlear implantation in the first 3 years post-implantation. Yearly costs for patient B increased again in later life because of the high cost of residential care, which was required from the age of 40 years onwards.

3.4 Univariate Sensitivity Analysis

To account for uncertainty associated with both costs and resource use in our scenarios, we adjusted cost category totals by 25 %. Acute care is a significant contributor to overall costs so we also examined how varying the length of stay in the PICU and length of stay in hospital by 25 % affected the lifelong costs. Sensitivity analyses are presented in Figs. 1 and 2.

Fig. 1
figure 1

Tornado plot showing results of univariate sensitivity analysis of various cost categories for patient A. Parameters were adjusted by ±25 %. NHS/PSS National Health Service/Personal Social Services, SEN special educational needs

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Fig. 2
figure 2

Tornado plot showing results of univariate sensitivity analysis of various cost categories for patient B. Parameters were adjusted by ±25 %. NHS/PSS National Health Service/Personal Social Services, SEN special educational needs

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4 Discussion

4.1 Main Findings

Severe cases of meningococcal disease which require 5- to 6- month stays in hospital can result in costs to the NHS/PSS of around £160,000–£200,000 in the first year alone. These costs have not been taken into account in a recent UK cost-effectiveness model for meningococcal B vaccines because acute care costs in this model have been based on average hospital inpatient day costs for meningococcal infection alone (which do not include costs associated with surgical procedures such as amputations, skin grafting, cochlear implantation, neurosurgery, or other inpatient rehabilitation, which may take place in complicated cases) [18].

This study demonstrates that resource use (and therefore costs) in severe cases tend to be higher in the early years after recovery from acute illness. Discounting scales down future events so that the further into the future they occur, the less important they are to a decision maker today, and the discount rate applied in a CEA can dramatically affect the resulting cost-effectiveness ratio [19]. It may be important to the outcome of CEA that these higher costs in the early years post-illness are fully represented by staggering the costs accordingly.

This study estimates costs over a lifetime of survivors of meningococcal disease with major sequelae approached from two different perspectives: NHS/PSS and government. A government perspective on costs gives a broader picture of the cost to society as a whole, and includes additional costs associated with the education of children with major sequelae. The government perspective also includes benefit payments such as disability living allowance, carer’s allowance and disabled facilities grants which acknowledge (but do not fully compensate for) the increased cost to the family of bringing up a disabled child. In these scenarios, discounted costs from a government perspective are 1.5- to 2.3-fold higher than NHS/PSS costs alone.

We did not attempt to cost these cases from a full societal perspective as costs to individual families are more difficult to quantify. Previous studies have reported that the day-to-day costs to a family with a disabled child are three times those for a family of a non-disabled child [20], and one study found that the minimum essential budget required to bring up a disabled child was £7,355 per year compared with £2,100 for other children [21]. Reasons for these additional costs to the family may include increased heating bills due to the lack of mobility of the child, increased washing because of incontinence, and the need for specialist equipment to help with day-to-day activities and mobility. These higher day-to-day living costs in addition to a lower income (due to one parent perhaps needing to stop work to care for their child) mean that families with disabled children are 4-fold more likely to live in poverty [22].

4.2 Strengths and Limitations

A limitation of this study is that relying on experts with experience and interest in the sequelae of meningococcal disease to estimate resource input may produce estimates representing good practice care rather than actual practice. However, we have been careful to be realistic about the medical treatment given and included only such care as our experts considered would be available on the NHS for a child in these scenarios. For example, some children are able to get running legs by applying to their Primary Care Trust, but such expensive specialist equipment was not included in our scenario. Additionally, patient B was cared for by her parents until she reached 40 years of age rather than being cared for in a residential care home from an earlier age at a considerably higher cost.

Drug costs have been obtained from the British National Formulary, which may overestimate costs because NHS Trusts often have separate contracts for suppliers. Additionally, this could be the case for certain equipment costs where the only available price was a direct quote from suppliers; however, as these costs only make up a small proportion of the total, they are unlikely to significantly alter the overall amounts.

Although we describe severe examples of disease, these scenarios do not represent the most complex cases. For example, the extent of injury to the circulatory system in severe septicemia leaves a proportion of children with permanent renal failure, and adrenal or other endocrine damage in addition to amputations. Our scenarios are also conservative in that they have not taken into account additional costs to the NHS due to ill health of other members of the family. Previous studies have indicated that ill health is more prevalent in families with disabled children compared with other families as members of such families are particularly prone to stress-related illnesses such as depression and anxiety. It has also been reported that well siblings of children with chronic illness or disability are 1.6- to 2.0-fold more likely to experience behavioural and emotional problems [23].

5 Conclusions and Implications

At the time of writing, the first ever vaccine to protect against different strains of serogroup B meningococcal bacteria has been given a positive opinion by the European Medicines Agency and will soon be a licensed product in Europe. Another vaccine is in late stage development. CEA is used as a tool to decide whether licensed vaccines should be introduced into the national schedule.

Economic analysts who create cost-effectiveness models are often faced with estimating costs for meningococcal disease survivors with sequelae with limited information. Until now, there has been no UK literature detailing the costs of caring for and treating survivors of meningococcal disease with major sequelae. This study has identified the high costs associated with the acute treatment of severe cases of meningococcal disease, which to date have been unaccounted for in CEA of vaccination for meningococcal disease. We have also identified cochlear implantation and prosthetic limb provision to be amongst the most costly aspects of caring for survivors of meningococcal disease with severe sequelae. One recent study suggests that around 1.3 % of serogroup B meningococcal disease survivors will be left with disabling amputations and another 2 % will survive with profound bilateral hearing loss, although the proportion of survivors with severe brain damage is more likely to be less than 1 % [24]. Consideration of these long-term rehabilitation costs within CEA would certainly increase the cost effectiveness of a vaccine that protects against serogroup B meningococcal disease. It remains to be seen, however, whether their inclusion would be enough to reduce the cost of a particular vaccine to within the NICE £20,000–£30,000/quality-adjusted life-year (QALY) cost-effectiveness threshold.

In addition to the cost/QALY from an NHS/PSS perspective, it is imperative that decision makers and budgetary and service planners recognize the overall economic and human impact of such diseases when considering whether to implement vaccines that protect against them. As our study identifies costs from a wider government perspective, it highlights some of these wider impacts.