PEDIATRICS Vol. 122 No. 3 September 2008, pp. 629-632 (doi:10.1542/peds.2007-2355)
EXPERIENCE & REASON |
Critical Illness and Amputation in Meningococcal Septicemia: Is Life Worth Saving?
a Centre for Child and Adolescent Health, University of Bristol, Bristol, England; Departments of
b Pediatric Occupational Therapy
c Pediatric Intensive Care, St Mary's Hospital, London, England; Departments of
d Pediatrics
e International Child Health, Imperial College, London, England
ABSTRACT
Amputation is an infrequent but devastating outcome of meningococcal septicemia. We assessed daily living functions and quality of life in a cohort of children and young people, 3 to 5 years after limb amputations following severe meningococcal disease. All participants lived with their families in the community, with minimal assistance. Participants used effective strategies to compensate for motor impairment and generally had good quality of life, despite ongoing health problems (predominantly musculoskeletal). The degree of amputation did not predict the functional outcome. The surprisingly good outcomes we report should discourage clinicians from withdrawing intensive care support because of presumed poor outcomes after multiple amputations in severe meningococcal disease.
Key Words: amputation • critical care • developmental outcomes • meningococcal disease • quality of life
Abbreviations: AMPS, Assessment of Motor and Process Skills
Fulminant meningococcal sepsis may progress rapidly to shock and multiple-organ failure. Those who develop purpura fulminans, with severe peripheral ischemia and gangrene, may require amputation of limbs and digits. When limb ischemia develops in a critically ill child who is also suffering from shock and multiple-organ failure, there is often a prolonged delay before limb segments are amputated, to determine fully the extent of demarcation. Clinicians caring for the child and the family often face the difficult decision of whether to continue aggressive intensive care for a child who may survive with multiple mutilating amputations.
Amputation following meningococcal septicemia is frequently multiple. There may be associated skin necrosis, abnormal sensation, contractures, tissue breakdown, and poor stump healing or abnormal growth. Affected patients need long-term multidisciplinary rehabilitation, but published outcome reports have been limited to data on survival, range of movement, and achievement of independent mobility.1–4 There are few published data on how these patients fare subsequently in everyday life, apart from a single case study.5 For clinicians and families, assumptions about future quality of life may color decisions on whether to continue aggressive intensive care in the presence of multiple-organ failure and the need for multiple amputations.
CASE REPORT
A previously well, 8-year-old girl was transferred from her local hospital to a regional PICU. She had been unwell for 12 hours and developed a rapidly spreading purpuric rash. She had tachycardia and white peripheries with very delayed capillary return, but she was alert, with no meningism. Initial investigations showed acidosis, leukopenia, thrombocytopenia, low potassium and calcium levels with impaired renal function, and severe coagulopathy.
The patient required mechanical ventilation for 18 days, because of acute respiratory distress syndrome. Ongoing shock necessitated large-volume fluid resuscitation (210 mL/kg in 6 hours) and high-dose inotropic support. The patient needed renal support (hemofiltration) until day 30. All 4 extremities became severely ischemic; the patient was treated with heparin and tissue plasminogen activator and underwent bilateral tibial compartment fasciotomies. She developed prolonged fever with negative culture results, which resolved only with bilateral below-knee amputations. She subsequently underwent bilateral below-elbow amputations. With significant progress in her multidisciplinary rehabilitation, she was able to go home on day 72.
As part of our research on the long-term consequences of the disease, the patient was reassessed at age 11. A bright, literary, and determined girl (before and after her illness), she has goals of owning a car, writing a book, and having a career. Attending to her appearance was a key aspect of her successful rehabilitation, with obtaining aesthetic prostheses suitable for a young girl being an important step in her resuming an active social life. Because of their appearance, the patient was not using upper-limb prostheses and had learned to write faster with her stumps than her fastest classmate. She was able to perform almost all activities of daily living (excepting putting toothpaste on a toothbrush) herself, although she was accepting assistance in a number of areas for the sake of speed.
Together with other families with disabled children, the patient's parents had fought for adaptations in the school, using knowledge of legislation and complaints where needed. The patient's acceptance into and adjustment to school life had been facilitated by visits to the school by members of a meningitis and septicemia support charity, who talked with other students and staff members to diffuse prejudice and fear regarding a child with severe disability attending the school. Despite her severe disability, the patient had gone on camping holidays and participates in all significant school and family events.
METHODS
To evaluate the long-term outcomes of children suffering amputation following meningococcal septicemia, we studied a cohort of young people (<18 years of age at the onset of disease) who were admitted to a single PICU during a 5-year period. We identified 13 patients who had undergone amputations (exclusions were limb loss limited to a single fingertip on the nondominant hand or a single nongreat toe). Two patients declined to participate, 1 was living abroad, and 1 could not be traced. Nine participants 5 to 22 years of age (median: 12 years) at assessment consented to the study. They were visited at their homes with their families, 3 to 6 years after the onset of disease. Standardized assessments were made of functional abilities (Assessment of Motor and Process Skills [AMPS]6) and quality of life (Short Form-36 if >16 years of age or the equivalent Child Health Questionnaire7), together with a clinical examination and a semi-structured interview about current health and everyday routines, which was designed to address function and quality of life in more detail.
This study was approved by the St Mary's Hospital Local Research Ethics Committee. Participants and their families provided written informed consent for their involvement in the study and for publication of the photographs presented here.
RESULTS
At admission to the hospital with severe meningococcal disease (Glasgow Meningococcal Septicemia Prognostic Score: 12–15),8 the patients were 9 months to 17 years of age. All patients were treated for shock and multiple-organ failure, which necessitated mechanical ventilation and inotropic support. All except 1 required renal replacement therapy, and 1 had cerebral involvement (encephalopathy with cortical microhemorrhage). The degree of amputation (Fig 1) varied from loss of a single toe to bilateral below-elbow or below-knee loss. In 5 cases, triple or quadruple amputations were required.
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At the time of assessment, with the exception of 1 child who had lost a single toe only, all patients had and used lower-limb prostheses; only 1 used a cosmetic hand. One patient used crutches because of stump pain and hyperesthesia, and another was using a wheelchair because of a fractured femur.
Ongoing health problems were predominantly musculoskeletal (stump and prosthesis problems, pain or abnormal sensation, postural difficulties, asymmetric limb growth, joint pain or effusion, and muscle atrophy). One patient had undergone renal transplantation because of sepsis-induced chronic renal failure. Other health problems were poor growth, migraine, poor memory or concentration, and fatigue. Despite this, all participants were living with their families in the community, with minimal assistance (5 were at mainstream schools, 1 was unemployed, 2 had paid employment, and 1 was a parent), and had life goals similar to those of their peers (Fig 2).
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As expected, functional assessments showed low scores for motor skills, in comparison with age-matched normative values (Fig 1). However, process (organizing and trouble-shooting) skills were normal (except for the individual with cerebral involvement). Clinical assessments and these scores together indicated that these young people were using effective strategies to compensate for their motor impairments.
Quality-of-life scores varied but were generally good. Only 1 patient reported quality-of-life scores >1 SD below the mean on both physical and psychological scales, despite less-extensive amputations. We observed no relationship between the degree of amputation and subsequent functioning and quality of life (Fig 1).
DISCUSSION
The surprising findings of this small cohort survey are that, 3 to 5 years after this catastrophic illness, young people with multiple disfiguring amputations are living full and valuable lives in the community with their families, using effective strategies to compensate for motor impairments and reporting generally good quality of life. The lack of a simple relationship between functional or patient-centered outcomes and disease severity has been observed for many other conditions.9–12 The only participant in our series with poor outcomes in physical and psychological evaluations, despite less-severe amputation, also had brain involvement. This might be an important association, because cognitive skills may have an important protective or compensatory function. The lower AMPS score for process skills may be critical, with this young person not finding the creative ways around obstacles that the others managed.
Despite the small size of this study and the fact that it was based in a single unit, our findings support an optimistic approach in continuing aggressive intensive care for children with meningococcal disease and severe peripheral limb ischemia. The favorable quality of life and functional outcomes of patients who have experienced mutilating surgery and limb loss suggest that, unless there is evidence of severe permanent neurologic damage, full intensive care support should be continued, even if there is likely to be subsequent significant limb loss.
CONCLUSIONS
The families involved in this study remind us of the extraordinary resilience, resourcefulness, and determination of young people striving to make the most of their lives despite being faced with apparently insuperable adversity. This report should help clinicians involved in the acute care of these young people to continue treatment even when faced with such catastrophic illness and the prospect of quadruple amputation.
ACKNOWLEDGMENTS
This study was funded by a grant from the Meningitis Research Foundation. The Meningitis Research Foundation approved the funding proposal and invited presentation of the results but had no part in study design or analysis, interpretation, or presentation of results.
We thank the participants and their families. We thank Helen Betts and Rebecca Biggs for help in identifying potential participants, Eva Bower, University of Southampton, and Prof Alan Emond, University of Bristol, for advice, and the Meningitis Research Foundation (especially Linda Glennie) for support and encouragement.
FOOTNOTES
Accepted Dec 20, 2007.
Address correspondence to Tom Allport, MD, Centre for Child and Adolescent Health, University of Bristol, Hampton House, Cotham Hill, Bristol, BS6 6JS, England. E-mail: tom.allport{at}bristol.ac.uk
The authors have indicated they have no financial relationships relevant to this article to disclose.
REFERENCES
- Landham TL, Datta D, Nirula HC. Amputation for gangrene of the limbs following severe meningococcal infection. J R Coll Surg Edinb. 1991;36 (1):11 –12[Medline]
- Genoff MC, Hoffer MM, Achauer B, Formosa P. Extremity amputations in meningococcemia-induced purpura fulminans. Plast Reconstr Surg. 1992;89 (5):878 –881[Web of Science][Medline]
- Hegarty SE. Meningococcal septicaemia and the management of patients who have suffered limb loss. BAPOmag. 1999;25 –28
- Bache CE, Torode IP. Orthopaedic sequelae of meningococcal septicemia. J Pediatr Orthop. 2006;26 (1):135 –139[CrossRef][Web of Science][Medline]
- Lowe KG, Boyce JM. Rehabilitation of a child with meningococcal septicemia and quadrilateral limb loss: a case report. Arch Phys Med Rehabil. 2004;85 (8):1354 –1357[CrossRef][Web of Science][Medline]
- Fisher AG. Assessment of Motor and Process Skills. 2nd ed. Fort Collins, CO: Three Star Press; 1997
- Landgraf JM, Abetz L, Ware JE. The Child Health Questionnaire (CHQ) User's Manual. 2nd ed. Boston, MA: HealthAct; 1999
- Sinclair JF, Skeoch CH, Hallworth D. Prognosis of meningococcal septicaemia. Lancet. 1987;2 (8549):38[Web of Science][Medline]
- Vogel LC, Klaas SJ, Lubicky JP, Anderson CJ. Long-term outcomes and life satisfaction of adults who had pediatric spinal cord injuries. Arch Phys Med Rehabil. 1998;79 (12):1496 –1503[CrossRef][Web of Science][Medline]
- Singer LT, Hill BP, Orlowski JP, Doershuk CF. Medical and social factors as predictors of outcome in infant tracheostomy. Pediatr Pulmonol. 1991;11 (3):243 –248[CrossRef][Web of Science][Medline]
- Mayou R, Bryant B. Outcome in consecutive emergency department attenders following a road traffic accident.
Br J Psychiatry. 2001;179
(6):528
–534
[Abstract/Free Full Text] - Wood RL, Rutterford NA. Demographic and cognitive predictors of long-term psychosocial outcome following traumatic brain injury. J Int Neuropsychol Soc. 2006;12 (3):350 –358[Web of Science][Medline]
PEDIATRICS (ISSN 1098-4275). ©2008 by the American Academy of Pediatrics
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