Quality of Life in Children Following Treatment for a Malignant Primary Bone Tumour Around the Knee

Purpose. We report on the quality of life following treatment for a malignant primary bone tumour around the knee in skeletally immature children. Patients. Patients (n = 41; mean age = 18 years; range 8–28) had all experienced chemotherapy in a neo-adjuvant setting, surgical excision of the tumour and endoprosthetic replacement. Methods. Interviews were conducted separately with the child and mother and focused on mobility, body image and the impact of treatment on schooling, employment and plans for the future. Results. Mobility in the group was variable. Only 12% reported that they could run with any confidence. The proportion who were able to swim (49%) or ride a bike (46%) was higher. All had experienced major disruption in schooling (mean absence following diagnosis = 12 months). Eight had repeated a school year and 41% patients reported that their schoolwork was affected. As a result of their experience, eight (six females and two males) chose health-related employment. Concerns for the future were highest among males and those with manual jobs. Three patients were receiving psychiatric support, in relation to extreme concern about the risk of recurrence. All expressed satisfaction with treatment, and older patients believed that the prosthesis gave a better quality of life than amputation. Discussion. Our data suggest that outcome following limb-salvage surgery is variable. Education is disrupted. Even so, only two left school with no qualifications. Employment is most restricted among males with few qualifications who may benefit from sensitive vocational counselling.


Introduction
T he incidence of bone tumours (osteosarcom a and Ew ing' s tum our) in children is small. Prognosis has im proved in recent years 1 and consequently it is now timely to consider the unique experiences and dif® culties experienced by these patients. T reatm ent involves neo-adjuvant chem otherapy according to the relevant treatm ent protocol and the surgical excision of the tum our. D ue to problem s of lim bsalvage surgery in children, som e centres advocate prim ary am putation for children who present with a m alignant prim ary bone tum our. 2 O ptions described for lim b salvage in children include endoprosthetic replacement, allografting 3 and van N es rotationplasty. 4± 6 O ur centre has perform ed lim b-salva ge surgery for skeletally im m ature children with m alignant prim ary bone tum ours of the lower extremity since 1976.
An extensible endoprosthetic replacement consists of a m assive replacement of bone that can be lengthened, a constrained knee joint and a sliding com ponent. 7,8 T he sliding com ponent is situated in the distal fem ur in proxim al tibial replacements and in the proximal tibia in distal fem oral replacem ents. It consists of an intram edullary stem that crosses the physis perpendicularly and is designed to allow growth to continue at that physis.
At the time of diagnosis the expected grow th is calculated according to data provided by T upm an. 9 If the expected grow th exceeds 4 cm (bone age less than 14 years in boys or 12 years in girls), an extensible endoprosthetic replacem ent is contemplated. O therw ise, a non-extensible endoprosthetic replacement w ith a sliding com ponent is inserted. 7,8 The surgical procedure involves en bloc resection of the tum our and replacem ent. T he children w ho have had an extensible endoprosthetic replacement 1357-714 X/97/010039± 07 Ó 1997 Journals Oxford Ltd require regular hospital adm issions in order to lengthen the prosthesis. O n average, two lengthening operations per year from time of diagnosis to skeletal m aturity are required in order to m aintain lim b length equality. 10 Lim b-salvage treatment appears to offer the possibility of better psychological functioning, and intact body im age. At the sam e time, there appears to be no difference in term s of overall survival between lim b salvage and am putation.
N evertheless, there are som e disadvantages with lim b-salva ge surgery com pared with am putation. Additional hospitalizations m ay be necessary depending on speci® c com plications, including infections or loosening of the prosthesis. Care m ust be taken that the limb is not dam aged, and children are advised not to participate in contact sports. Salvaged lim bs vary in their degree of function and there can be som e discom fort. Fear of injuring the effected lim b, failure to reintegrate successfully with peers and reduced sporting prowess m ay m ean that at least som e children adopt a restricted life-style. Repeated school absence m ay also com promise school achievem ent. G iven the extent of m edical treatment involved, and the known potential com plications, it is necessary to determ ine how far these procedures com prom ise children' s quality of life, both in terms of physical and psychological function.
O ur study is therefore concerned with the im plications for quality of life of endoprosthetic replacem ent for a m alignant prim ary bone tumour around the knee in skeletally im m ature children. A sm all num ber of studies have com pared adult patients treated by am putation or lim b surgery in terms of psychological outcom e. 11,12 These have included patients across a wide age range and have not focused on the speci® c dif® culties of young people. Although Sam mallahti et al. 13 concluded that there w as no increased incidence of psychiatric problems in children with osteosarcom a, all those included in this study had been treated by am putation. T here has, therefore, been no study to date which has speci® cally addressed the impact of lim b-salvage surgery on quality of life in children and young adults.
There is currently no abso lute agreement regarding the m easurem ent of quality of life in children, 14 and no agreem ent about the dom ains or areas of function which contribute to quality of life. 15 W e therefore developed an appropriate interview schedule, to cover the main areas w hich have m ost frequently been cited as relevant. 14,16 In this paper, we report: (i) school and educational achievem ents; (ii) current employm ent status; (iii) physical functioning: ability to get around independently, participation in sports; and (iv) pain and body im age. F or patients over 18 years, general well-being was assessed using the SF-36. 17 In addition, we asked w hether or not the patient was receiving psychiatric or psychological counselling. As is com m on following amputation or surgical procedures of this kind, physical functioning was assessed using the Functional Evaluation Index. 18 In recognition of the increasingly aggressive nature of m odern m edicine, particularly in the treatm ent of rare or potentially life-threatening disorders, there has been a burgeoning interest in`quality of life' work with children w ho have been treated for a range of diseases. W hile this work is believed important to determine progress in the ® eld, it is not known how far fam ilies ® nd it distressing to be asked to participate in such studies. In order to address this question, participants were asked to rate how distressing they had found the interview.

Patients and m ethods
Approval to conduct the study was sought and obtained from the appropriate Ethics Com mittee. All patients who had an extensible endoprosthetic replacement of the distal fem ur or proxim al tibia at the Royal O rthopaedic Hospital, Birm ingham since 1976 were traced through m edical records. For inclusion in the study, we required that patients were at least 7 years of age and had a m inim al follow-up of 6 m onths. Patients who were experiencing m etastatic disease and undergoing active treatm ent were excluded. Sixty-three patients were initially identi® ed. O f the 63 patients identi® ed through m edical records, six w ere not contacted either because they had since been treated by am putation or were in relapse. N ine could not be contacted; they did not answ er at least tw o letters to the last know n address. O ne fam ily was excluded because the parents did not speak English. A further three declined to participate. (O ne of these had previously refused lengthenings treatm ent and had not been followed up for 4 years and two m others anticipated that they would be too distressed.) Forty-four fam ilies were traced and agreed to take part in the study (an overall response rate of 70%). In 38 fam ilies, both child and m other were interviewed. In three fam ilies the patients took part but not their m others, and in a further three fam ilies the m others agreed to be interview ed themselves but did not wish to involve their child. (This included one 11-year-o ld who w as thought to be too young by his m other and two older m ales, both of whom were working full-tim e and w ere`too busy' .) In this paper, w e report the results of the 41 interviews with patients. The m ean age of the group was 18 years (range 8± 28), and included 24 m ales and 17 fem ales. Five had been treated for Ewing' s sarcom a. For the total group, 35 had received an extensible endoprosthetic prosthesis and nine a non-extensible prosthesis as previously described.
Additional demographic and m edical data describing the sample are shown in T able 1. activity. T hey w ere also asked to report if they used analgesics and how frequently. W ith regard to body im age, patients were asked their view s about the app earance of their leg and w hether or not they dressed to disguise it.
(v) Emotiona l well-being. W e recorded whether or not the patient was receiving, or had ever received, psych iatric help or psych ological counselling. In addition, patients above 18 years com pleted the SF-36. 17 T his m easures generic health and includes eight m ulti-item scales containing between tw o and ten items each, and a single item m easure of reported health transition. The subscales are designed to m easure: physical functioning (extent to which health lim its physical activities such as self-care, walking, climbing stairs); role physical functioning (extent to which physical health interferes w ith work or daily activities); bodily pain (intensity of pain and effect of pain on normal work); general health (personal evaluation of health); vitality (feeling energetic com pared with feeling tired and w orn out); social functioning (extent to which physical health or em otional problem s interfere with norm al social activities); role functioning em otional (extent to which em otional problem s interfere with work or daily activities); m ental health (general mental health, including depression, anxiety); and reported health transition (evaluation of health com pared with 1 year ago). T his scale is currently being used widely and was chosen because of the availab ility of norm s for the British population. 17 (vi) Functional evaluation of reconstructive procedures. 18 T his scale is used as a standard assessm ent of function following surgical treatment for bone tum ours. It includes ratings of six categories: pain (none-severe); function (no restriction± total disab ility); and emotional acceptance of the lim b (enthusi-astic± dislikes); use of supports (none± crutches); lim itations in walking (unlim ited± unable without aid); and gait (norm al to m ajor handicap). Ratings are m ade on a 0± 5 scale and scores sum m ed to give an overall rating. A num erical score and percentage rating are calculated, with higher scores indicating better functioning and em otional acceptance. This scale was com pleted by patients only.
Treatment of data. Standardized m easures were scored as directed in the appropriate publications. Interviews were transcribed and later coded by two authors independently. Inter-rater reliability was initially good (92%) and disagreem ents resolved by discussion. C ategorical data were sum m arized as percentages. Student t-tests were used to com pare m ean scores on the SF-36 with population norm s and to compare ratings of function m ade by patients Patients were initially approached by letter, outlining the purpose of the study. For patients below 14 years of age, the letter was sent directly to parents. For those over 14 years, separate letters w ere sent to patient and parent. F or all fam ilies, participation of both patient and parent was requested. H ow ever, the letter suggested that it was also acceptable for only one mem ber of the fam ily to participate if that was preferred. Included w ith each introductory letter w as a reply slip, for fam ilies to return and suggest a convenient time for the interview to take place. All but three interviews were conducted in fam ily hom es. In these three cases, patients felt it would be m ore convenient to be interviewed at the hospital on a routine follow-up visit. M others and patients were alw ays interviewed separately.

M easures
An open-ended interview was conducted w hich enabled survivors to expand on their own experiences and raise particular issues which they considered unique to them selves. The interview focused on a num ber of themes: recall of the diagnosis, understanding of the cause, rationale for treatment, possibility of complications and need for future treatment, perception of how well inform ed they had been, and their own opinion about how and how much children should be told about the disease. (These data w ill be reported separately.) (i) Impact on schooling. Estim ation of length of time absent from school, both following diagnosis and for subsequent lengthenings, examinations passed, any course still being studied, whether or not patients had repeated a school year or needed special help and self-ratin gs of learning dif® culties.
(ii) W ork. Em ploym ent status, w ork satisfaction, im pact of illness on choice of work.
(iii) M obility. Com petence to take part in different sporting activities, ability to run, ride a bike, swim, w alk upstairs, walk dow nstairs, drive a car (m anual or autom atic), dif® culties experienced travelling.
(iv) Pain and body image. Patients were asked if they experienced any pain at all or only after strenuous w ith those m ade previously by surgeons.
Signi® cance was accepted at the p , 0.05 level.

Results
C om parisons were ® rst m ade betw een those with Ew ing' s sarcom a and the rest of the sam ple. N o differences w ere identi® ed in terms of Enneking scores 18 nor on any of the psychological measures. D espite the increased toxicity of treatment for those w ith Ewing' s sarcom a, the groups did not differ in self-estimates of learning dif® culties, time away from school, or whether or not they had repeated a school year. In all rem aining analyses, therefore, the groups w ere com bined. these absences (beyond the hospitalization period) w as asso ciated with practical dif® culties of returning to school (fear of being knocked over by crowds, inability to use stairs). Eight had repeated or were currently repeating a school year. A further three children were having extra teaching help in school on a regular basis. T his included one patient (male) who had dyslexia problems which pre-dated the diagnosis. Tw o fem ales reported learning dif® culties which they felt were asso ciated w ith treatm ent. In practice, they described dif® culties in concentration and understanding instructions. (There w ere no patients with Ew ing' s sarcom a in these groups.) Am ong the group, six were too young to have attempted any public exam inations. Am ong the rem ainder, 12 (29% ) were taking or had com pleted education to G CSE standard (or the equivalent Intercert awarded in Ireland). Seven (17% ) were taking vocational courses (e.g. hotel catering). T wo left school with`A' levels and nine (22%) were registered or had com pleted a course leading to a degree, and three (7.3%) w ere taking or had completed higher degrees. O nly two (5%) had left school with no quali® cation and had no plans to change this.
Tw enty-four (59% ) reported that the illness or its experience had no effect on their choice of subjects studied. H owever, 18 (41%) felt that their choices had been reduced because of lost schooling. (French and maths were m ost frequently cited as subjects where dif® culties w ere experienced m aking up for lost time.) (ii) C urrent employm ent status. Tw enty-six (63%) were still in full-tim e education. Am ong the rest, three (7%) w ere unemployed, 11 (27% ) were in full-time em ploym ent and one w as a housewife and m other. Four reported that their work was too demanding for them and six thought it was`som etimes' . O ne fem ale w as wanting to give up her clerical post in order to return to full-tim e education.
All patients were asked whether they thought the illness had m ade or w ould m ake any difference to their choice of w ork. W e were able to identify four groups: (a) E ight patients did not make appropriate responses.
All w ere am ong the youngest in the sam ple and had no idea what they would do or were very unrealistic (wanted to be a pop star!). O nly one male (and no fem ales) anticipated that there m ight be work problems in the future. Although currently employed in a skilled m anual job, he recognized that it w ould not be possible to continue inde® nitely and was planning to take a business course. (In addition, it is important to note that from the separate interviews involving m others, w e learned that the two m ales w ho were`too busy' to be interview ed were in full-tim e m anual employm ent.) (iii) Physical functioning, ability to get around independently, participation in sports. Patients' reports about their com petence to perform each physical activity (run, sw im , ride a bike, w alk upstairs, w alk dow nstairs) were coded, so that low er scores indicate m ore dif® culty. Ability to drive was recorded in term s of four categories: (too young 5 1, unable to drive but of an approp riate age 5 2, able to drive an automatic car only 5 3 and able to drive a m anual car 5 4). Results are shown in Table 2. T here was variability in com petence on all tasks. Although all but one was able to get up and down stairs, a higher percentage reported dif® culties with swim m ing or riding a bike, and only ® ve (12% ) were able to run. In addition to these questions, we asked how m any had m obility problems at school or work. N o dif® culties were reported by 38% . O thers had dif® culties with steps (43%), or being in a crowd (18% ). The problem of crowds w as m ost acute for children at school who often were allowed to leave lessons early in order to avoid the between-lesso n crush. N one of the sam ple reported that they took part in contact sports at least at any com petitive level. (M any m ales quali® ed this and reported that they did play`friendlies' .) Participation in all sports was generally very low, with patients com m enting that although they were allowed to play non-contact sports, their inability to run very m uch lim ited w hat they could do. Five m ales and two fem ales expressed considerable regret that they could not participate in sports. Tw o patients reported dif® culties w ith travelling, due to problem s of keeping the leg in one position for any length of time.
Seventeen had taken up som e activity to m ake up for the fact that they could not participate in active sports (e.g. pool, ® shing, breeding dogs, sailing). In term s of leisure activities, the m ajority (66%) did not belong to any clubs. Three (8% ) belonged to sedentary clubs (chess), eight (20%) belonged to a sports club and one belonged to a club for the disabled.
Patients were not directly ask ed w hether or not they understood that there w as a risk they m ight have been treated by am putation. However, 23 raised the question of amputation themselves. N one felt they had been offered a choice in treatment, rather that prosthesis w as the preferred policy of the hospital. All were pleased they had had a prosthesis rather than an am putation. O nly one reported hav-ing som e regrets given the fact that he had not achieved as good m obility as he had hoped. T hose who did not discuss the possib ility of am putation were younger than those who did (p , 0.05).
(iv) Pain and body image. Ten (24% ) reported that they had no pain associated with the prosthesis at all. T hree (7% ) reported pain when it was cold, and six (15%) reported pain only after very strenuous activity. N ineteen reported m ore serious and continuous pain. As a result, six (15% ) reported occasional use of analgesics and four reported regular use.
Seven (two m ales and ® ve fem ales) of the sample were distressed by their scar. T he rem ainder were not em barrass ed or at all self-conscious of the scar, and six (four m ales and tw o fem ales) reported themselves quite proud (`I wear it like a m edal' ). M uch m ore distress was experienced in relation to m uscle wasting, uneven growth and m is-shap en knees (n 5 25). M any continued to feel embarrassed by the appearance of their leg; am ong the m ales, seven reported that they did not w ear shorts or sw im m ing clothes, while am ong the females, eight always chose to wear trousers or long skirts.
(v) W ell-being. T hree patients were receiving or seeking psychiatric help. In all three cases, extreme fear about recurrence w as the m ajor concern.
Patients over 18 years com pleted the SF-36 (n 5 30). M ean scores for the group on the separate subscales are shown in Table 3 along with the published norm s. A series of t-tests to com pare m ean differences w as conducted. The group had scores below population norms on all subscales, and these were signi® cant on physical functioning, physical role perform ance, pain, general health and social functioning (see T able 3). In addition to the norm ed scores, patients were asked to rate their health now com pared w ith 1 year previously. T hree reported that their current health was`much better' or`slightly better' than the previous year, 17 that it was the sam e, and 2 that it was`w orse' or`much worse' .
Fertility. Am ong the 18 patients over 16 years of age, two fem ales and one m ale had children. (There were four children in total.) The remainder were aware that they m ight be unable to have children.

D iscussion
T his study suggests that there is som e variability in physical and psychological outcom e among young people treated with endoprosthetic replacements. As a group, they app ear to do well in public exam inations, despite lengthy school absence. W hile the m ajority were not distressed or felt able to disguise the app earance of their leg w ith the right clothes, a proportion went to som e lengths to disguise the way they looked. They all report some lim itations on their m obility. T his is m ore of a disadvantage for those at school w ho are expected to participate in PE program m es com pared with those w ho are at work. W hether at school or work, patients reported being afraid of being knocked, and as a result, tended to avoid crowded places. F or som e, the lack of a visible handicap represented a disadvantage of the prosthesis over an amputation, since they felt that others w ere less likely to m ake allow ances. N one reported that they experienced restrictions in terms of their opportunities to socialize or travel. None expressed regret about the course of treatment that had been taken.
O ur data suggest that, on the standard functional evaluation of reconstructive procedures, 18 patients rate their m obility and satisfaction with the treatm ent lower than surgeons. Several studies point to parallel ® ndings, that patients or their fam ilies rate quality of life to be low er than ratings m ade by m edical staff. 19 Such discrepancies should not be unexpected, and m ay be attributed to the surgeons' m ore lim ited awareness of the patient' s functioning in a range of situations, rather than sim ply in a hospital setting. In addition, patients m ay very m uch want to present themselves to m edical staff in a positive light. Even so, it is im portant to recognize that surgeons m ay inevitably be unaware of the degree to which patients experience dif® culties in their daily lives.
From an educational point of view they perform ed well. D espite very lengthy school absences, m any individuals had reached a satisfactory level of education. H owever, academic progress is certainly vulnerable, as evidenced by the num ber repeating a school year.
The m ajority of patients w ere`not at all' or`a little' distressed by the interview. M any patients were, in fact, very positive about their involvem ent, em phasizing that this was their ® rst opportunity to talk the`whole thing' through. Although anyone who experienced distress w as invited to term inate the interview , no one w ished to do so.
The extent to which quality of life is com promised is dependent on how far an individual is prepared or able to develop other skills to com pensate for the inevitable lack of physical strength and m obility. In this respect, those individuals with m ore academ ic leanings are undoubtedly in better positions than those w ho would expect to take m anual or unskilled work. T he lim itations experienced by these patients, especially m ales, m ay be aggravated in environm ents where the stereotype of app ropriate male work is physical. It is this group of patients (m ale unskilled) who may need speci® c counselling to deal with their disability. All recognized that som e jobs which involved too m uch standing or lifting were not possible. T hese restrictions w ere of course m ore lim iting for those with less education, who m ight have expected to w ork in shops, hairdressing, etc. O thers would not consider jobs which involved wearing a uniform which would fail to conceal their scar (e.g. air hostess).
Although the group app ears to be functioning as well as m ight be expected for a group from very different social backgrounds, the m ajority were still young and in relatively protected environm ents. M ost w ere in school or higher education and still living with parents. As yet, they have not experienced dif® culties in obtaining employm ent, although given their functional lim itations, a proportion at least m ust expect to experience this. T he extensible prosthesis is a tem porary device intended to ensure equal lim b length throughout childhood and prepare the lim b for subsequent insertion of an adult prosthesis. Consequently, it m ust be anticipated that children will require revision to the adult type at some stage. This will inevitably m ean a m ajor disruption in further education and em ploym ent prospects. T he need for longer-term surveillance of these patients app ears, therefore, to be essential.
There is no doubt that lim b-salvag ing surgery of the kind experienced by these patients is potentially a m ajor set-back to the attainm ent of a normal life-style. Treatm ents are very lengthy, the interruptions to norm al school or work attendance great, and the possibility of recurrence or problems w ith the prosthesis are high. T he alternativeÐ am putationÐ does not appear to be asso ciated with less m orbidity 20 and m ay be less acceptable to patients from social and psychological perspectives. Even so, it is not possible to predict the function of a prosthesis w hen initially em barking on surgery. O ur data provide som e baseline against which to inform patients and their parents of the likely outcom e in terms of physical function. Although it m ight be useful to have com parable data involving other treatments, there is no obvious control group against which to com pare the functioning of these patients. Given that lim b saving is the treatment of choice (at least from patient perspectives), it is not appropriate to com pare this group with those who have experienced amputation, since at least in our centre, patients are only treated by am putation w here lim b salvage fails. H ow ever, the question of w hether or not experience of a prosthesis enables patients to better accept an am putation if it becom es necessary is a very real question for future work.
Future work m ust, therefore, seek to clarify ways in w hich quality of life can be optimized for young people undergoing lim b salvage procedures. M ore system atic coordination between national and regional centers at all levels (m edical, nursing, social w ork and physiotherapy) is necessary, as well as the establishm ent of a counselling service w hich is inform ed about the particular needs of these patients.

A cknowledgem ent
T his work was funded by the Cancer Research C am paign (CP1019/0101).