MARCH, 1972 P H Y S I O T H E R A P Y Page 5 TREATMENT OF SPINA BIFIDA CYSTICA AS USED AT THE QUEEN ELIZABETH HOSPITAL FOR CHILDREN LONDON E2 JA N IC E B E H R (nee Green) D ip. Physio. (U .C .T.) IN T R O D U C TIO N The aim o f this article is to present som e o f o u r theories and practice in th e treatm ent of children w ith Spina Bifida. Various different m ethods are used in o th er hospitals treating this type o f handicap and overlap in m any respects. I hope to be o f help to the physiotherapist handling these children fo r the first time. We are fortunate in our hospital in th a t the medical and nursing staff, medical social w orker, physiotherapist and occupational therapist all w ork in close co-operation to the best advantage o f th e child and th e family group. It is very likely th a t these families will present a disturbed pattern due to the traum a o f initial separation o f m other and child a t birth, plus the added physical defects o f the child. This will all naturally affect all members o f the family. We find, therefore, th a t the m ost im portant early factor in the treatm ent o f these children is the thorough preparatory care-w ork provided by o u r medical social worker to help these parents to accept their handicapped baby. C ontinued support is provided fo r these families by the hospital medical social w orker so long as the child continues to attend this hospital. A part from the obvious physical difficulties presented by these children, factors often overlooked are th e associated problems, the m ajo r one a t an early stage being hydro­ cephalus w ith recurrent urinary problem s due to the p ara­ lyzed bladder. T he child requiring frequent hospital admissions because o f blocked valves an d urinary infection will be delayed in all phases o f developm ent a t this stage. The gross family problem s which m ay arise due to the uncertainty o f the outcom e o f this condition. The anxiety o f the parent will lead to over-protection o f the child, which may delay development. The role o f the physiotherapist in treatm ent is now described: TREATM ENT PR O G R A M M E The first stage o f treatm ent covers th e new born baby to the pre-moving phase. Admission and Initial R eferral The children are adm itted to o u r hospital within a short time o f birth from a num ber o f m aternity units in N orth- East London, Essex, H ertfordshire an d K ent regions. The reason for this early and urgent adm ission is the surgical necessity o f early closure o f the myelomeningocele or meningocele. This requires a paediatric surgeon w ho special­ izes in this procedure. Muscle Charts The baby is usually referred to the physiotherapy depart­ ment for initial muscle charting prior to surgery. This is the first muscle c h art and often th e m ost difficult to produce accurately due to the follow ing:— (1) The baby may still be sluggish from birth traum a. (2) Primary reflex activity will interfere w ith actual muscle power. (3) Leg deformities m ay m ask true muscle power, though these do often indicate the presence o f muscle activity. The muscles are charted in basic groups as accurately as Possible, care being taken to differentiate, where possible, between reflex activity an d actual muscle pow er. Exam ple: |.ln stim ulation o f the p lantar surface o f the foot may elicit a flexor w ithdrawal pattern o f the leg. A second muscle c h art is com pleted post-operatively, observing the sam e procedure as before. W henever possible a th ird and final initial in-patient ch art is com pleted between 14 and 28 days, depending upon the degree o f hydrocephalus and th e time th e surgeons select to insert the valve. Once the baby has settled from this procedure, th e chart is com ­ pleted and it appears th a t this is the most accurate assess­ ment. TREA TM EN T O F A SSO C IA TE D D E F O R M IT IE S M any o f these children present w ith congenital paralytic deform ities which require correction. Talipes Equino Varus (1) R obert Jones strapping w ith passive m anipulations. ' F elt an d zinc oxide strapping o r Elastoplast alone are used depending upon skin reactions and ease o f m anipulation. (2) Modified R obert Jones strapping to below the knee. (3) F oam -backed Zim mer finger splintage £ in. to 1 in. width, bent to th e required angle an d strapped on to the foot to hold the corrected position. (4) D ennis-Brown bootees on a b a r fo r th e older baby and child. (5) Surgical intervention with the older child. (6) M inim al deform ities are corrected w ith daily m anipu­ lations and passive movements. Calcaneo Feet Deformities Corrected by passive stretching, or the third m ethod as above. H ip and Knee Contractures (1) Passive movements. (2) P ro n e lying for hip flexion contractures. D islocated hips will be corrected surgically, depending on the genera] condition o f th e child. This is usually perform ed between the ages o f one year and three years. In som e children (pre- o r post-operatively) a passive, full range o f m ovem ent is never obtained. Spinal Deformities Scoliosis and lum bar/thoracic kyphosis to o may have to be corrected surgically a t a later date. Physiotherapy contact with the parents is established a t a suitable stage, this will usually be p rio r to the first discharge. It is essential th at, during these early stages, a constructive an d friendly rap p o rt is established w ith the child’s family. H om e instruction is norm ally the only necessary physio­ therapy and, if needed, weekly visits for restrapping o f foot deformities are arranged. W e feel th a t the minim um instruc­ tion should be given at this early stage, and th a t the parents should be encouraged to visit the departm ent w ith their child on norm al outpatient appointm ents. F u rth er details o f treatm ent program m es are given when th e family next attend, an d have become cognisant w ith th e child’s special difficulties. I t m ay be necessary to repeat muscle charts throughout these early stages. F rom th e very first, all disciplines must em phasize to the whole family the necessity o f allowing the baby to be as norm al as possible and to p u t him on the floor at the usual time. F oam wedge pillows, etc. are sometimes found help- R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. ) Page 6 P H Y S I O T H E R A P Y MARCH, 1972 ful fo r the children to lie over, with toys placed on the ground in front. These parents need alerting to the necessity of bringing different experiences to the baby to compensate fo r its loss o f natu ral movement. SE C O N D P H A S E O F TREA TM EN T Assessment o f Movement O bservation by the therapist is m ade, wherever possible, o f the child’s ability to move, pivot, sit, crawl and show alertness in personal and social situations. C om parison w ith the norm al milestones is difficult, for even th e child w ith m inim al paralysis m ay be retarded in his early stages, owing to hospitalization and often over­ protection by the family. If, a t 9-12 m onths it is apparent th at further active treat­ m ent an d help are necessary, then in some cases we have found th at fu rth er advice to parents has proved adequate, but in some cases it is found necessary to institute regular attendances in the departm ent to stim ulate both intellect and movem ent. C are m ust be taken by the therapist in interpreting reports given by the parents o f the child’s progress at home. Parents cannot be expected to function as trained observers, e.g. m isinterpretation o f pivoting for a definite backwards or forw ards m ovem ent o f the child. A guide o f actual move­ m ent for parents to report upon is a six-foot directional crawl in any form . Thus the non-m overs will fall into the next treatm ent group. Treatm ent Programme In this departm ent we feel strongly th a t these children should establish each stage o f development, i.e. floor mobility, pre-walking training, standing, through to calipered walking in sequence. T he occupational therapist is also concerned with the establishm ent o f program m es for intellectual and em otional stim ulation, which will run parallel to the movem ent ones. W e feel th at the child needs to be encouraged in all disciplines fo r any one o f these to be established successfully. T he child who has no interest in play will not have the m otivation fo r moving. Floor Mobility (1) Prone lying — lifting head and p u sh in g 'u p . o n arm s. Some children with hydrocephalic heads halve diffi­ culty with this movem ent. A larger wedge m ay be used to elicit the above response. We have had the occasional child who starts to move principally to remove him self from the wedge! (2) Rolling to sitting from supine through side flexion is taught but the child w ith a high paralytic lesion may be unable to achieve this movem ent. Rolling may be unobtainable fo r the sam e reason until the child is older. (3) Independent sitting is delayed and is a t first achieved with a forw ard lean and until the child can balance his own body weight, he cannot achieve a good sitting position. Falling play in this position is im portant for the child to gain self confidence in independent sitting. (4) M erm aid crawl: We teach the child to pull on his forearm s in prone to stim ulate forw ard movem ent on the floor, but first the idea o f head lifting in prone m ust be acquired. This activity will take time to achieve, especially with the poorly m otivated child. Parents should be encouraged to give the child plenty o f opportunity to practise this at hom e — a shiny surface, such as lino, should be used at first an d all form s o f play incorporated. Some children do not manage the m erm aid crawl and develop a “ bottom shuffle” in the sitting position. Any movem ent in any form is a great achievement, especially w ith the slow starters. (5) Spina Bifida Trolleys: This gives the child a n idea of speed o f m ovem ent and a means o f joining in play w ith other children. A trolley is a m eans o f reducing frustration in the irritable child. Some children will not yet be moving on the floor, although mobile in the trolley, and this m ay lead to the “ trollev bound” child which is no t a desired result. Pre-W alking Training (1) T he child is taught falling from standing in all directions. This will overcom e his fear o f falling from this strange and high position o f standing. There are many games and m uch fun from this form o f exercise. (2) T he back extensors are strengthened w ith specific exercises in th e standing position, incorporating the aims o f the above. B oth this an d above activities will need to be carried out w ith support from the therapist to the child to m aintain knee extension and a standing balance. (3) W hen the child can move on the floor — o u r minimum o f six feet — Q ueen Elizabeth H ospital standing splints are fitted. These should be w orn as much as possible during the day, as w ith calipers, and play in the upright position can now be m ore easily super­ vised by the parent. A t this stage it is not felt that a pelvic band fo r the additional support will be needed but, at the time o f writing, these splints are on trial and are proving very successful. It is helpful to suggest to the parents th a t play surfaces, e.g. sandpits, water- play, etc., be on a level w ith th e child’s best standing position. (4) F o r arm strengthening and developm ent o f a down thrust the children play various games. O ne th at we find m ost helpful is “ pushing off” from various objects placed on either side o f the child in a sitting position. This will lead to the required action for crutch walking. Secondly, this same action is used in the forw ard, lean-standing position in some falling games. Thirdly, throughout all phases th e parent and therapist support the child’s arm s w ith extended elbows and a p alm ar grasp to enable the child to thrust down when walking. Calipers and Walking T hroughout o u r series and fro m experience w ith many Spina Bifidas treated at this hospital, we have found th at a child is no t ready fo r walking training until he has achieved a good form o f floor mobility an d has overcom e the fear o f falling and m ust show the desire to walk as well as being m ature enough to concentrate. T his stage m ay not be reached until the child is nearly fo u r years old. W hen the child is ready, we start w alking-training in standing splints while aw aiting the supply o f calipers. We prefer no t to use hip locks o n full calipers to allow m ore freedom an d security in falling. The child with frequent admissions to hospital and the added difficulty o f over-protection from disturbed parents, will invariably be late in all developm ental stages and may not be ready fo r walking training a t the arb itrary age of three. E ach case m ust be considered on its own merits, but we do not push walking before this age. M ost o f these children require time to achieve a w alking p attern and have a limited concentration span fo r such a difficult task. In o u r survey group we have found th at the older children who, for various reasons, could no t be calipered until 4J-5, have all been able to walk within a couple o f m onths on quadripod sticks. In th e early stages either a swing through o r a reciprocal gait is taught so th a t the child can appreciate an d enjoy m ovem ent in the vertical position. A swing through gait m ay lead to the realization o f a reciprocal gait. T he older child o r adult usually reverts to a swing through gait fo r speed when using crutches. R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. ) MARCH, 1972 P H Y S I O T H E R A P Y Page 7 Walking Aids Rollators and quadripod sticks are used as walking aids in conjunction w ith parallel bars, in the initial stages of training, progressing later to crutches. Rollators have the advantage o f moveable wheels which allow the child to appreciate movem ent. We find the square fram ed rollator the preferable model, as the child is able to place his arm s in the correct position later needed fo r crutch gaits. PH YSIO TH ER A PY A SSO C IA TE D W IT H O RTH OPA ED IC M A NA GEM EN T Reduction of Dislocated Hips Modified tum m y trolleys have been used m ost successfully while the child is in a uni- o r bilateral plaster hip spica. This m aintains m obility while in plaster and helps to have a happier child. A wide rear end on the trolley is necessary to accommodate the abducted position o f the legs. When the plaster is removed, the child is adm itted to hospital fo r m obilisation — approxim ately tw o weeks. Great caution m ust be exercised at this stage because fractures, due to osteoporosis, are likely to occur w ith the slightest stress. The children are a t present put into standing splints following rem oval o f plaster. These help to prevent fractures by m aintaining a straight leg. T he lower end o f the femur fractures when the child pushes up in prone and a fracture m ay occur at the lower end o f the plate in the femur when the child rolls, leaving p art o f the leg to one side. It is hoped th a t the standing splint will be a help in reducing the fracture risk, as already the num ber o f post­ surgery m obilisation fractures has dropped since these were first applied after the rem oval o f plaster. T he mobilisa­ tion programme is carried out w ith these splints, which are worn continually except fo r short periods, a few days after removal o f plaster when knee flexion is required. 1. Passive movem ents are found to be unnecessary and may be traum atic. T he child will m anage the m ajority o f his mobilization w ith positioning by the therapist and from instruction to the nursing staff and parents. 2. A program m e o f graduated sitting is commenced to flex the hips until approxim ately 90° is reached. U ntil this stage, the child is limited to bed — usually two days. (Discretion m ust be used w ith the child who sits on his lum bar spine and does not require the same degree o f flexion to obtain a good sitting position.) 3. W hen the child has achieved this required sitting position, he will be allowed up in his standard trolley and to sit at the table to play. While in this chair sitting position splints are removed and, with the thigh fully supported, gravity will act as an aid to knee flexion. T he upper leg m ust be supported to the knee when the knee is flexed. F ractures have occurred when a child has slid forw ard an d the fem ur protruded over the edge o f the chair. Feet m ust be adequately supported. A t all times care must be taken in handling the limbs o f the child when lifting, carrying and moving in bed. 4. A fter ten days the child recommences standing with support and floor mobility in creeping and sitting, graduating stresses and strains to continue program m e as above. A CKNOW LEDGEM ENTS T h in k s are due to Miss D . M. G reenhill, Superintendent Physiotherapist, Q ueen Elizabeth H ospital fo r Children, London, E.2, fo r her valuable criticisms and encourage­ ment, also Miss R . B arnitt, head occupational therapist, and Miss Collyer, typist, fo r the preparation o f this article. SUMMARY In this paper we have shown how, in o u r hospital, Q ueen Elizabeth H ospital fo r C hildren, L ondon, E.2, we treat spina bifida cystica during the neonatal, pre-walking m obilization and early walking calipered life. The aim s o f the whole treatm ent team — doctor, therapists and medical social w orker — being to help the total family unit w ith the m anagem ent o f this child. O ur hope is that, by the time th e child reaches five, he will only require m inim al supervisory physiotherapy an d be able to concentrate on his school activities. M rs. Behr, who trained a t the Physiotherapy School, University o f Cape Town, spent several years a t the Queen Elizabeth H ospital fo r Children, London, E.2. She recently returned to Cape Town, South Africa. OCCUPATIONAL THERAPY FOR THE SPINA BIFIDA CHILD R . B A R N IT T Occupational Therapist Queen Elizabeth H ospital fo r Children, London, E.2 There are four areas in which th e occupational therapist should combine with the physiotherapist to provide a co­ ordinated program m e fo r the Spina Bifida child. (1) Activities to encourage o r distract the spina bifida child during his specific physiotherapy treatm ent. T his m ay take the form o f using a teddy bear o r a doll to do exercises a t the same time, o r providing music to which group exercises are perform ed. M any variations can be provided if some im agination is used. (2) W hen the baby first comes to the departm ent the m o th er will often ask fo r advice on handling an d play. T he suggestions given are directed to prom oting m aturity o f eye/hand skills an d increasing the range o f stim ulation th a t is provided in the home. The parents are advised to place toys and visually stim u­ lating objects within the bab y ’s focal range, an d to encourage eye follow ing; to interest the child in his hands by attaching bell bracelets, to provide as m uch tactile stim ulation as possible by carrying the child around the house an d garden touching things. W hen the child is old enough to sit, a soft foam floor chair m ay be m ade and bags o f textured m aterial, blocks and o th er toys suitable to the age, placed o n the floor near the child. F rom a year onw ards great emphasis is placed on handling different m aterials an d messy play as these children will n o t be prepared for saving, propping and pushing until they have experienced the relevant preparatory play. (3) W ith the older nursery age children group situations are provided. M any o f these children will have little opportunity fo r mixing w ith o ther children o f this age, and an ability to socialize will m ake the start o f school easier. In these groups a com m on type of play is provided such as pretend cooking o r painting. T he children encourage each o th er an d the physio­ therapist will be able to use the group fo r standing practice and as an incentive fo r play after treatm ent. (4) Some spina bifida children have perceptual difficulties; th e most com m on th a t we have found is a faulty body image. To prevent this the parents are encouraged to play with the child from an early age, games o f touching an d nam ing parts o f the body, especially the lower limbs. L ater activities can include m irror pain t­ ing, dressing dolls an d cardboard cut-out figures. R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. )