Rehabilitation and Aids during the Developing Years in Italy①

Martino Avellis,Alda Pellegri,Andrea Cazzaniga,Stefania Chierici,Marida Farina,Antonio Cinquegrana,Ciro Grazioli,Giovanni De Angelis

1.Experiences by La Nostra Famiglia di Bosisio Parini and Brain Injury Center Villa Beretta Hospital,Como,Italy

2.La Nostra Famiglia,Ponte Lambro,Italy

3.Fomagalli,Ponte Lanbro(CO),Italy

4.Garbagnate Milanese Hospital,Garbagnate Milanese,Italy

5.National Health Service for Naples Area,Rome,Italy

1 Rehabilitation—when,why and where during the age of development

Early rehabilitation of a child with disabilities is a tenet of the Italian care system.

"......early,timely intervention—from the first instant of clinical diagnosis of a potential disability—prevents potential worsening of the disability—both physical and psychological;

supply of suitable aids allows better management of the difficulties or of the incapacities,rendering care less onerous and consenting,in many cases,a good level of autonomy and all of this reduces welfare charge subsequently favouring,at the same time,promotion of the individual and the reduction in global costs......"

Law 104 from1992 which has often been referred to in previous articles,has,among its principal aims,the planning of a system of interventions with regards to"......avoiding and removing the invalidating conditions that prevent the development of a human being,the achievement of the maximum autonomy possible and participation of the disabled person in community life and to pursue functional and social recovery of the person affected by physical,psychical and sensorial impairment,guaranteeing services and assistance for the prevention,cure and the rehabilitation of disabilities......"and in particular,with regards to subjects during the age of development,establishes that concrete policies are implemented to

—guarantee primary and secondary prevention in all the phases of growth and development of the child to avoid or counteract opportunely the onset of impairment or to reduce and overcome damage from additional impairment;

—guarantee the supply and repair of equipment,prostheses and technical aids necessary for the treatment of disabilities and to avoid handicaps.

And,always relative to small and older disabled children during development,Law 104 also defines

—the right to education and instruction from the youngest age.

—A handicapped child aged 0 to 3 years is guaranteed inser-tion in a day nursery.The right to education and instruction is guaranteed to a disabled person in nursery schools,common classes of any type and level in scholastic institutions and university institutions.Scholastic integration has the aim of developing a disabled person's potential in learning,communication,relationships and in socialisation.

Ministerial guidelines always insist on the necessity of intervening intensively at the first onset of damage."......Rehabilitation intervention begins at the same moment in which the damage starts......"and even more so this must occur in the developmental stage.A child,right when he is tiny,must receive the message that he has goals to conquer,targets to reach;we cannot leave disabled children to grow believing they are incapable and useless and postpone the assessment of their capabilities to when their personality has already been formed.

Exactly because it is a delicate task,the guidelines place the Units for serious disabilities during the age of development between three intensive High Speciality rehabilitation Units,defining them as highly specialised structures expressly aimed at facing the complex and serious diagnostic,evaluative and re-educational problems of motor and cognitive,congenital or acquired,pathologies in the age of development.

The Unit for serious disability during the age of development must be equipped with specifically trained and qualified personnel,comprising neonatologists,paediatricians,infantile neuropsychiatrists,physiatrists,nurses,physiotherapists,speech therapists,neuro-psychomotor therapists,occupational therapists,psychologists,orthopaedic technicians,social care-workers,professional educators.

2 Among the duties,also the designing of aids

The activities of the Unit for serious disability during the age of development is mainly aimed at：

·close diagnostic examination relative to serious impairment and child disability;

·operative technical formulation of rehabilitative project and the therapeutical programme as well as control of its execution;

·design and validation of assistive technologies dedicated to the age of development and experimentation of innovative materials;

·clinical research activities and documentation of scientific progress in the childhood sector;

·observation of epidemiological data.

Unit for serious disability during the age of development must guarantee suitable information and formalised training for the families and/or helpers who will carry out home care relative to the following problems;respiratory care,cognitive problems,behavioural and psychological disturbances,prosthetic care and overcoming of architectural barriers,necessity of check-ups,where to effect them and how to access them.

The fact that among the duties of the unit for serious disability during the age of development there are also those of defining the characteristics and functional requisites of assistive technologies shows how,for Italian culture,there is an indissoluble connection between aids and rehabilitation.

It is rehabilitation that assesses the deficits and potential of a person,that establishes the goals that can be reached and also defines which instruments and how instruments must be made that will allow a child to stay seated better,more comfortably and more"correctly",to slowly slowly learn to stand up,to try to walk with supports that are increasingly less complex.

The first"Operative Unit for serious disability during the age of development"in Italy were"La Nostra Famiglia(Our Family)"Rehabilitation Centres.Founded in 1952,it very soon became a point of reference for the whole of Italian rehabilitation for small and older disabled children and the organisation of its own centres,the activities carried out in these,the teams of professionals that interact in every structure have made the model which inspired the compilers of the Rehabilitation Guidelines to define the requisites of the operative Unit for serious disability during the age of development.

In these centres in the sixties,next to rehabilitation treatments,the first aids for children were designed：aids for seating position,aids for the erect position,aids and supports for walking.

They were the first aids in Europe specifically invented and studied to respond to the particular needs of children with motor disabilities,designed to render possible those specific rehabilitative treatments with which,at La Nostra Famiglia/Our Lady,every child could hope to reach his best level of autonomy and participation possible.

Thanks to these aids many seriously disabled children have managed to sit up to prevent the worsening of their disability,prevent the setting in of deformity or pathological postural behaviour,to stand up and to walk,to write using that part of their body they managed to control best,to communicate.They especially learnt that disability can render walking more difficult but not necessarily prevent the reaching of goals,also ambitious ones.Many of these children who couldn't even sit up are now IT engineers,teachers,journalists and writers,accountants,lawyers......

We can certainly say that the aids invented our'La Nostra Famiglia'Centres have made history,in Europe,and have surely inspired the description with which the Italian Government indicates the requisites that aids for the age of development must possess in order for them to be supplied free of charge by the National Health Service.

"La Nostra Famiglia and aids during the age of development"

The first Rehabilitation Centre for disabled children began its activity in Ponte Lambro(Como)in 1952.It was a"La Nostra Famiglia"Centre and was the first to receive ministerial recognition from the Italian Government.

The basic concept which guided the operators,intervention(doctors,therapists,teachers)was that every child needs to,before anything"develop all his potential".This thought influenced the attitude and choices of all those who shared therapy,daily life and free time with the children.

"You can do"is the suggestion that motivates the children to dare,to overcome their difficulties,to become autonomous,to make themselves useful as much as they can,to feel capable and to increase their self-esteem.

"Doing for them"is the temptation of parents and operators driven by two things：

a)the pressing for time,

b)what is visible to the eye,what looks good

If we only consider what appears we negate the value of careful and patient research into what functions in every child,placing the right merit on the result,which can also be minimal,but it is the best we can do.

Developing functionality means intervening so that routines are structured in a way that is necessary to everyday care,to expressiveness and the construction of useful and meaningful objects.In order for routines to evolve,it is necessary that specific stimuli get sent to the damaged brain of the pathological subject.For this reason,in the Ponte Lambro Centre many aids suitable for stimulating the perceptions and many types of grasping functions have been realised.

It was immediately clear that"movement therapy"is not sufficient to activate functions and neither to maintain reached levels of articulation and functional mobility.It is necessary to act in a targeted way on the development of functionality.

The Rehabilitation sector specific for the development of autonomy and functionality is the Occupational Therapy sector.Within this sector the following takes place：

a)The research of every minimum ability which can be acted upon to render the disabled child capable of doing something for himself;

b)The training of all personnel that deals with the child so that anything effected in"therapy"sessions can be continued in any sphere;

c)Finally,the privileged field of occupational therapy,the adaptation of orthoses and aids.

The aid inserted in the rehabilitative project must have the characteristic of adaptability to every single child,so that he feels good,safe,helped to develop playing activities,the taking care of himself—abilities typical in school learning.

During all the various stimulation techniques applied to the sensorial apparatus,to movement and to deep sensitivity,the fragile point in the treatment is verifying the level of conscious attention and participation of the patient,in particular if the child,within the project that is being developed,leads to the achievement of the goals that have been identified for him.Motivating stimulus must always be found.

The autonomy goal is fundamental for the child and is often a hard achievement also faced with the difficult comparison with normal children of the same age who are increasingly more able in their movements,not always taught,like disabled people,to find new ways of gaining autonomy.

The motivation to movement and to competition is very high in a child so everything that allows him to move quickly,to be able to race,to be skilful at play,to realise positive results at school and in constructive ability is gladly accepted.

The aid,created in the 60's at the Ponte Lambro Centre had an occupational therapist,a parent and sensitive and capable craftsmen as protagonists.

The ideas were born one after the other due to the close observation of children's needs：

1.care of posture

2.facilitation of movement

3.stabilisation of segments conditioned by motor pathology

4.facilitation in communication

5.facilitation in the integration in the own context of life

6.preparation for working activities

7.study of the instruments capable of facilitating functional grip allowing：

a)feeding

b)individual and group play

c)reading and writing

d)painting expressivity

e)constructional ability.

The aid evolved exploiting the availability of technology,always respecting a few fundamental rules though,such as：

A)lightness

B)the adaptability to the single child that can use different parts of the body

C)safety

D)comfort and well-being

E)inexpensiveness.

Comparing the aids that are built today with the models of the past we can say that the original matrix has been our school and every operator has built on this bringing initiative,care,imagination,professionalism.

La Nostra Famiglia has indeed three Aid Centres located in Bosisio Parini(Lombardy),Pieve di Soligo(Veneto)and Ostuni(Apuglia).The operators responsible for the Sector work in strict connection between each other and the companies that supply aids,both for movement and for communication and learning(IT technicians),involving themselves with training so that all the therapists have the indispensable indications to operate well,also thanks to this important technical support.

The basic motive that drove and continues to drive the operators that deal with this sector of rehabilitation is the attention to the child and his needs.

This is the message of"la Nostra Famiglia"taken from their Website：

"The Association's Mission"

Our"mission"is that of protecting the dignity and improve the quality of life-through specific rehabilitation interventions-of people with disabilities,especially during the developing years.

"La Nostra Famiglia"intends to take on,not only disabilities in themselves,but also personal sufferance and that of the family that accompanies them.

The Association proposes making their own contribution to the development of research and scientific knowledge in the field of development pathologies."

The Association's style

When the founders,Luigi Monza and Zaira Spreafico,first Association President,welcomed the first disabled children,the concept of bringing up autonomous children worthy of being respected in their dignity,despite their disabilities,they were ahead of their times.Their indications were to"operate with welcoming and competent capacity"which characterised the style of la Nostra Famiglia and they are still the guarantee of being able to give a suitable answer to the needs of the children they take care of.

The welcome and respect of each life,however it is,orientates all choices:rehabilitative,educational,scientific,ethical and social.

Other"la Nostra Famiglia"structures that deal with Rehabilitation exist in the word and they also take their inspiration from the Mission of the first Association born in 1945：

Usadc(Usratuna)in Khartoum in Sudan 1999

A Nossa Familia in Santana in Brasil 1994

Nuestra Familia ad Esmeraldas in Ecuador 1996

In these contexts too,care of the motor disabled child through the use of aids has developed.In fact,in Juba(Sudan)and at Esmeraldas,two Aids Centres are active that use the experience of the Italian"La Nostra Famiglia",creating significant adaptations in relation to the culture of the countries in which they are located.We can say that for us,to rehabilitate signifies developing autonomy,well-being and self-esteem.Rehabilitation has been massively enriched developing sectors and innovative fields.

The aid was born with us and has accompanied us on a difficult but stimulating journey.Today we have disabled people who grew up in our centres and now,as adults,they advise us on how to still increase autonomy today with the help of aids,as every achievement has to be maintained and every disabled person can tell us something new.

Dott.ss Alda Pellegri,president of"la Nostra Famiglia"

3 Aids for children supplied free of charge by the Italian Health Service

When with aids we turn out attention to children,the first element we must take into account is the fact that their morphology,personality and disability are not fixed,constant data,but they are intrinsically and physiologically destined to change.

First of all there is"physiological"growth：from 0 to 18 years old the changes are continual and impose very close periodical checks of the maintenance and dimensional suitability of the aid：it is the aid that has to adapt to the person and not the person to the aid.

Another element that is always present is the modification of the disability situation.The impossibility of maintaining appropriate and comfortable postural behaviour or to carry out harmonious movements and the search and maintenance of unnatural posture to compensate for asymmetries or instability favour the beginning and fixing of articular limitations,muscular retraction,deformity and it is important to monitor the possible negative evolution to intervene timely also by modifying and correcting the supports.Suitable rehabilitative treatments can lead to important positive changes and,sometimes,quick changes：a child who cannot even sit up by himself can,if the potential exists,with the suitable treatment and aids,learn to stand up and,maybe to walk even,also in the space of a couple of years.

Within the Italian system of free supply of aids for rehabilitative projects,if for adults fixed renewal times are foreseen(for example,5 years for a wheelchair),the particular needs of this sector are recognised and the norms foresee"......For devices supplied to those beneficiaries below the age of 18,minimum renewal times do not apply,the local health authorities authorise the substitution or modification of supplied prosthetic devices,based on foreseen clinical controls and according to the therapeutic programme".

The adaptability of the aid to the individual characteristics of the child and the possibility of easily and constantly modifying it over time is an indispensable element(at least for the more complex and expensive aids)for the aid to be reimbursed by the national health service.

The knowledge that a person,and even more so a developing child,can stay the whole day seated in the same position and that for some activities such as eating,writing or drawing,reading,using the hands to do things means that they must have a particular type of seat,different from that used by the parents when they take their child out.This has led to two types of seating being foreseen by our regulations.These can both be supplied to the same child：a more active postural seat such as the"postural highchair with variable configurations"and more simple systems for brief trips outside,for example：from home to school：the"pushchairs".

In the descriptions of types of aids for the seated position that can be supplied free of charge in Italy,we find all the important elements that should characterise active seating(postural seating)and passive seating(pushchair,present on the list in two versions：one more simple and the other more containing).

Following below are examples of how some types of aid for the seated position for children are described in the new version of the list of aids that can be supplied free of charge to disabled children.

18.09 aids for the seated position

18.09.21 special seats

18.09.21.006 Postural highchairs and adjustable configuration

An aid indicated for beneficiaries with serious and complex postural problems that permits assumption of several functional positions for the specific necessities explicitly reported in the individual rehabilitative project.Characteristics：base frame equipped with pirouetting wheels and service brake,height adjustable to enable drawing up to a work surface,with a device allowing seating variation,tilting seat with system allowing tilting with the child seated,a removable seating system to also allow use on a base for outside use,padded seat,backrest,side panels and armrests;covered in a washable material,tilt-adjustable leg rest,height and tilt adjustable footrest,height and inclination adjustable backrest,seat depth and width adjustable,armrests height and inclination adjustable.

Prescribable accessories

18.09.91.003 Padded abductor,adjustable and extractable

18.09.91.006 Lateral adjustment of abductor

18.09.91.009 Pelvic harness,padded

18.09.91.012 Chest belt or at 45°on pelvis,padded

18.09.91.015Brace strap,padded

18.09.91.018 Padded thorax pads,adjustable in height and transversally(pair)

18.09.91.021 Padded front pads,adjustable in height and transversally,folding(pair)

18.09.91.024 Padded clavicular pads adjustable in height,angulation and transversally,folding(pair)

18.09.91.027 Padded headrest,height and depth adjustable

18.09.91.030 Padded headrest,height,depth and angulation adjustable

18.09.91.033 Padded headrest,height,depth,angulation and transversally adjustable

18.09.91.036 Padded headrest,height,depth,angulation and transversally adjustable with dynamic anti-flexion head containing straps

18.09.91.045 Small wrap around table height and tilt adjustable

18.09.91.048 Transparent wrap around table,height and tilt adjustable

12.27 vehicles and means of transport

The pushchair is an aid aimed at the occasional transport of disabled people(usually children)by a helper in conditions of suitable comfort and safety and designed to facilitate loading into a car or onto public transport.It is normally composed of a rapidly and easily reducible or foldable frame with wheels suitable for outside trips and equipped with a service brake,seating system(seat/backrest/safety belt)in washable material suitable to specific postural needs of the user.It also has a couple of leg rests with footrests(the latter always height adjustable)and push handles.The pushchair is not suitable to support the user for lengthy periods of time,for which other aids are foreseen(wheelchairs,postural systems).

Indications：children with severe motor disabilities and important postural problems who often need to be transported.In any case,if priority postural and particularly critical needs exist,prescription should be directed towards modular postural systems mounted on a mobile base. (cod.18.09.39)

12.27.03 pushchairs

12.27.03.003Umbrella closing pushchair

Foldable lengthwise and transversally with a single manoeuvre in order to obtain the minimum bulk for transport,equipped with height adjustable footrest and brace-type safety belts.Indicated for short trips on even ground,for children not exposed to important postural problems.

12.27.03.006Reducible pushchair

Foldable or dismantable with a single manoeuvre in order to obtain the minimum bulk for transport;padded seat and backrest,seat height and depth adjustable,backrest tilt adjustable,height ad-justable footrest,brace-type safety belts.Suitable for any type of trip for children who need particular support for containment and posture.

4 Basic characteristics of aids for the seated position

These descriptions with which the legislator defines the compulsory characteristics that an aid must have so that it can be supplied free of charge by the Health Service represent the fundamental constitutive elements of aids aimed at small and older children.

Adaptability is,in fact,one of the elements that cannot be renounced for every aid for children,both to adapt them suitably to the child at the moment of supply and to modify them over time,following development.

Let us try to examine the various characteristics required：

When we speak about seating,the choice of dimensions,adjustments and kinds of structure are extremely important for achieving the goals of the rehabilitation programme.If any details or variables are underestimated,the seating could be completely ineffectual and useless for daily activities.In some cases it could even aggravate the condition of the person rather than improve it.

In order to evaluate the seat adjustments some systems can be used for an objective evaluation,for example,pressure mapping,or the graphical and numerical trends of pressure between the body of the person and the support on which it rests.These systems,however,in order to obtain correct and comparable data must be used in accordance with standard protocols,repeatable and comparable;the protocol of Calgary for example identifies the actions that need to be carried out and how evaluations are to be carried out.

The width of the seat is one of the basic parameters for a posture system,a seat that is too wide could lead to asymmetries and obliquities that,over time,could create deformities,a seat that is too narrow would be uncomfortable and unbearable over long periods creating situations of risk from pressure sores.

In the picture on the side the seat is too wide creating an asymmetry resulting in a weight shift to one side.

The seat depth is another key factor which,if poorly identified could be totally ineffective during everyday life.If this is too short,the surface is very reduced and posture is uncomfortable,if too long it causes the risk of kyphosis of the spine and elevated pressure in the popliteal area.In the image the seat is too short and the contact area is very small,as the thighs don't touch the surface they are unable to absorb weight which is completely loaded onto the ischial area.

The height of the footrests must be measured with regards to the length of the legs.

Incorrectly considering this adjustment could mean reducing drastically the touching surface or creating a situation of sliding and seating instability.In the top picture the footrests are too high and do not allow the front of the thighs to touch.The bottom picture highlights peak pressures typical of slipping in the area distal to the ischia.

Reclining of the backrest must be assessed as when the backrest is reclined sliding usually occurs and pressure is then concentrated in the sacrum/coccyx area reducing comfort and increasing the risk of pressure sores.

The two images show the transition from an upright backrest(top)to a backrest reclined by 40°,we see the increase in peak pressure in the area of the coccyx.

Tilt in space,contrary to backrest recline,is able to maintain seating without slipping,distribute the weight more onto the back and create a situation of comfort and better trunk control.

The images show evolution of the pressure before tilting(above)and after having changed the seat angle to 135°(below).Note the decreased values.

The position of the armrests,if used,must support arm and elbow and be comfortable for the shoulder joint and effective to load weight from the seat.In the top picture with respect to the one below,the armrests are too low and therefore are not effective for loading of weight—the higher peaks of pressure can,in fact,be noted.

Each kind of cushion behaves differently,knowing the performance and behaviour of each cushion,the right one can be chosen to suit the needs of each person to achieve the best and most comfortable seating.

The images show the various acquisitions made with the same person and different types of cushion,in sequence：

-viscoelastic cushion

-hollow fibre cushion

-anatomically shaped cushion

-air bubbles cushion 7 cm high

-air bubbles cushion 10 cm high

Reference

1995"Valutazione di Cuscini Antidecubito—Studio Comparativo"Centro Propara Montpellier.

1999 A.Cavicchioli-Glossario."Immobilità,allettamento e possibili complicanze：dalla sorveglianza epidemiologica all,impiego di ausili"—Atti del Convegno Chianciano Terme.

Hess CT.Guida clinica alla cura delle lesioni cutanee.Masson SpA：Milano,1999.

Dalla Valle I,Battisti N,Puglisi C,Di Gioia S,Cuscini Antidecubito.la Riduzione della Pressione Quale Indicatore Reale della Loro.

Efficacia.Giorn Ital Med Riabil 1992;4(VII)：369-374.

Defloor T,Grypdonck MHF.Sitting Posture and Prevention of Pressure Ulcers.Applied Nursing Research,1999,12(3)：136-142.

Michael Clark.Pressure Ulcers—Recentr advances in tissue viability,2004.

Aids in the developing years:2 experiences

We will report two experiences which show the use of postural aids in developing years supplied free of charge by our National Health Service in situations of severe disability.

1.Marco,13 years old,affected by Metachromatic Leukodystrophy

In this case it is highlighted how the use of different elements described by our national government system for"postural highchairs with variable configurations"is determining in making a child affected by a severe degenerative dystrophy assume a symmetric and active position.The pathology of this child is such that he is unable to autonomously maintain an appropriate seating position and leaving him without suitable support would favour the fixing of pathological postural behaviour with the consequent setting in of serious deformity.

2.Nine cases of children afflicted with spinal muscular atrophy(SMA).

The use of seating systems which adapt perfectly to the characteristics and needs of each child and the supply of suitable equipment allows for children to be discharged from hospital structures where they were compelled to live and taken back into their families.

Constant clinical observation and objective assessment with measurement of concentrations of oxygen in the blood through a saturometer has allowed verification that the maintenance of a comfortable seating posture also improves the ventilation/perfusion ratio,something extremely important for these patients as respiratory capacity constitutes one of the most critical elements.

Case 1:Marco,affected by Metachromatic Leukodystrophy

1 case：Serious degenerative leukodystrophy：from non-postural seating to postural seating Photographic documentation.

Marco is 13 years old.

He suffers from a Metachromatic

Leukodystrophy(MLD,also called

Arylsulfatase A deficiency),a severe degenerating dystrophia.

Marco is not able to control his position and slips down on his seat with no way to keep his arms and legs in an appropriate position.

Legs balance the generally asymmetric postural seating.

His mother is always repositioning her son's hands,legs and head.

His trunk is getting more and more asymmetric;his backbone tends to curve to scoliosis

His head is held by head side supports,correctly made to guarantee a wider surface and allow Marco to keep a central position.

The unstable equilibrium of the legs creates generally asymmetric postural seating.

Combination of a more symmetric position for legs and the best comfort is very difficult.

Let's try and find Marco a more active position,that won't let him slip down.

Let's change the technical aid;and let's start working with the pelvis and on the trunk.

First of all the width of the seat is correctly dimensioned so that there is about one centimetre space between the sides at the level of the trochanter and the lateral supports.The length of the seat is also dimensioned so that there are about two centimetres between the end part of the seat and the popliteus hollow of the knee.Supports are positioned on the backrest that maintain the position stable.

A lumbar cushion maintains the physiological curve,providing support.

Let's move on to look for a better head support.

A wide,comfortable head support is tried which,though,doesn't support his head sufficiently.

Other supports are tried with the aim of finding a more active position.

Finally,we choose a shapeable finger headrest that is height,side,inclination and depth adjustable.

The most suitable position is looked for,often adjusting the depth in order to give the right support：not too far back,not too far forwards.The lateral supports are precisely adapted to the dimensions of Marco's head.

Now Marco is much better：his postural seating is more"active".

Then,we try to improve his legs,working on leg-rest inclination;footplate;calf support…

We must decide whether to use separate footrests to correct the postural asymmetry,or a single,more comfortable footrest with leg-rests.

By observing Marco's position after having seen to the pelvis,trunk and the head,we notice that his legs seem to assume a more symmetrical position and therefore we decide that a single footrest is sufficient.

Seat tilting is also evaluated and fixed at about 10°.

Even better!

Now let's focus on trunk adjustments,while replacing the vest with a belt with braces,thus granting more freedom.

A tray with a good surface for the forearm and with a small basin so Marco does not need to flex his wrists and that improves his handling of objects....

Finally for Marco an indoor/outdoor base is chosen：very compact it allows his family to take him into any room of the house and also,for short periods,outside,in the parents'small garden.

What will Marco be like in a year's time?

What would Marco be like in a year's time?

The question that we ask ourselves each time when faced with such severe cases is：can a suitable posture system effectively counteract the onset of deformity,tendon and muscular retraction,scoliosis and other pathological behaviour?In Italy limited situations such as the one figured beside are becoming ever rarer and,when they occur,they are often the consequence of the unsuitable management of postural supports,rehabilitation treatment and clinical intervention.

The results that we systematically observe,when intervention is early and followed up at very close intervals adopting each time the necessary modifications up to changing the seating system when it is not longer suitable,confirm the possibility of averting the setting in of more serious damage.

Another,non secondary element,is represented by the increased inclusion within the family and social life of the child/youth：a good seating system allows for a more active posture that stimulates the disabled child to better participation but which also invites those within his family and social context to turn to him and include him more frequently.

Marco's parents state that ever since Marco has been sitting on the new system they take him more frequently with them into the various rooms of the house.They also say that his little brother turns to him much more often,asking him questions and trying to involve him in his games,even if he is aware that Marco cannot answer nor interact with him.

Case 2:nine children affected by SMA

9 cases of spinal amiotrophy.Good seating posture allows a person to participate more fully in life in the family home and also prevent damage to the muscular-skeletal;respiratory and digestive apparatus.Methods and results are reported.

CHANGING THE DISABILITY THAT ISOLATES INTO"TOLERABLE DIVERSITY"

This project was developed to respond to two different problems：to help seriously disabled children live with their families and to realise a seating system that could be adapted to children's postural needs.

The rehabilitation team operating in the area of Naples admitted a group of children whose severe disabilities had not,until then,allowed them to live outside of hospital to assess the possibility of suitably equipping their houses to allow them to return to their families,as the parents wanted.

The houses were equipped with the necessary equipment and initially the children,as soon as they were discharged,lived the whole time in their beds,or,for a few minutes,in the arms of their family members.This though,left them isolated all the time compared to the daily life of the family and the team,composed of a physiatrist,a therapist and a paediatrician decided to find a seating system that would allow the children to spend their day in the rooms where their families lived,rather than in their equipped bedrooms.

It was thus necessary to find a seating system that would allow the children to assume a reassuring and comfortable position.

Agreements were made with a company that made systems for disabled children to implement the modifications necessary to reach their goals.

The seating position has permitted a significant integration of the children in their families.Even though unable to carry out any activities,the simple possibility of being present in every moment of daily family life has produced positive effects both in the children and in their families.

An unexpected and very interesting result is that there has also been a significant clinical improvement.

Introduction

The Vesuvian area pertaining to the Sanitary Regional Institute Naples 5 suffers a particular phenomena：an extremely high influence of children afflicted with SMA(in particular Werdnig Hoffmann disease)much more than what indicated as per WHO statistics.Therefore the Sanitary Regional Institute Naples 5 wants to prepare a program to safeguard these children,in order to grant them,first of all,adequate home medical care.For the project realization the best seating postural system has been chosen in order to suit the specific needs of this particular pathology,and this has been obtained with appropriate adjustments as well as options that permit personalisation of the seating unit to adapt to each child：we would like to help them not only with regards to the children's pathology but also with all the other targets of the rehabilitation project on the whole.An industrial project is necessary in this situation that tests the effects the adoption of a postural seating system will have in the life of children suffering heavy disability.

Besides this,further important aims of the test are：

· To grant the child suffering from severe disability and his family the best possible autonomy,reducing as much as possible their"solitude"due to their unavoidable pathology;

· To organize a working team for a multidisciplinary approach to the complexity of the pathology;

· To reduce the single caregiver's load.

The project involved 9 children who were followed for 9 months.

Methods

The Italian National Health System previously supplied the child and his family the following items,as per specific needs:

· Electromedical equipment for the maintenance of vital abilities;

· Technical aids(postural seating system);

· Home Rehabilitation Therapy(PT,ST,OT,PM);

· Nursing;

· Domicile Caregivers;

· Multiprofessional and health team for specific problems.

At a later date the children were supplied with a special seating postural system and,using some opportune monitoring schedules,the effects of this system in the everyday life of both the child and also his family could be assessed.

The monitoring schedules used were：

1.P.U.L.S.E.S.profile(Physical conditions,Upper limb function,Lower limb function,Sensory components,Excretory function,Support factors)for the enlistment criteria;

2.postural test for the sitting position evaluation;

3.Certain/IHQL for costs/benefits(Certain：Cost-Effective Rehabilitation Technology through Appropriate Indicators;IHQL：Index Health-related Quality of Life);

4.evaluation schedule for the seating postural system;

5.follow up schedule.

Besides these instruments,the Paediatric Clinical Evaluation(that monitored the health level of the child)used the value of the peripheral oxygen saturation under a double indication of value：first of all as absolute value for the oxygenation of the children having a compromised respiratory system,and then as indirect agent of evaluation for the best possible postural sitting positioning.

The posture system used allowed the perfect adjustment of the width and depth of the seat and the creation and application underneath the contact surfaces of the backrest and seat of a personalised conformation of modules.These allowed for the creation of personalised seating for each child,able to provide comfort and stability.The position of the modules was varied often to find the most suitable arrangement;in particular,two children asked for the modules to be repositioned about ten times in the course of two months before being satisfied with the results.

It was in fact necessary to wait until the children were used to the new position(until then they had been in a lying down position or,sometimes,they were held)and that they found a comfortable postural position autonomously.

Another interesting element was the possibility of tilting the seating system—inclination of the whole seat without varying the backrest angle.

Results

Thanks to the above explained analysis we obtained the result that the children's variable characteristics could be assessed and the right postural sitting positioning could be identified.

The use of a special postural system that,due to its own project characteristics,can be adjusted to suit the different specific child's needs,together with the multi-professional team,made up of community health nurses and public caregivers,allowed the foreseen targets to be reached,and,in particular：

· the rehabilitation of a child at home improves the quality of the child's and his family's life;

· everyday autonomy both at home and outside is increased;

· the measurement of the peripheral oxygen saturation of these children has highlighted the correlation between the registered values and the time of use of the postural system;besides this,there is also a difference between the right sitting positioning and the sitting positioning with the best oxygenation.

Under the evaluation,manufacturing companies can see that the postural system has been accepted with interest by the families and the multi-professional team involved in the project;furthermore,in respect of the complexity of the children,situation,the team has pointed out some critical points of the postural system that have been perfected,as per structure sizes,the adjustments and further necessary optionals.

Conclusions

This project made both the parents and the nursing operators of the area aware that the problem in this area is serious and requires the guarantee of adequate autonomy.

The life prospects for these children has increased,not only with regards to"age"but also with regards to quality.

The everyday use of a suitable postural sitting system allowed the child to reach a seating position and to take part in family life translating into better physical and also emotional life.

Furthermore,thanks to the unique aim of the Hospital and the nursing operators it is possible to reach goals more easily with no responsibility overlapping and no defaults.

Reference

?John B.West,Fisiologia della respirazione.L'essenziale(3aedizione italiana sulla 7a in inglese),Padova,Piccin,2006.

?Tinsley R.Harrison;Kasper,Braunwald,Fauci,Hauser,Longo,Jameson,Principi di Medicina Interna(16aedizione),Milano,McGrawHill,2005.

?J.B.West,"Fisiologia della respirazione",Piccin ed.

?Goldman A,Hain R,Liben S,eds.Oxford Textbook of Palliative Care for Children.Oxford,UK：Oxford University Press;2006.

?American Academy of Pediatrics.Committee on Psychosocial Aspects of Child and Family Health.The pediatrician and childhood bereavement.Pediatrics,2000.

?Racca F,Bonati M,Berta G,et al.Long term ventilation of children in Italy：preliminary data from questionnaire survey[abstract].Intensive Care Medicine,21st ESICM Annual Congress,2008.

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?Ottonello G,Ferrari I,Pirroddi IM,et al.Home mechanical ventilation in children：Retrospective survey of a pediatric population.Pediatrics International 2007.

?Carnevale FA,Alexander E,Davis M,et al.Daily living with ventilator-assisted children at home,distress and enrichement：the moral experience of families.Pediatrics,2006.

?Oskoui M,Kaufmann P.Spinal muscular atrophy.Neurotherapeutics,2008.

In Italy rehabilitation in children and aids for children cannot be separated.We have spoken about seating positions but we should also speak about the erect position or about aids for walking：for everyone there is a single denominator：this is the rehabilitative project which,defining necessity and potential,challenges industry to manufacture products that are increasingly more adaptable,comfortable,pleasant and effective.

The Italian state considers each adapted aid,supplied to a child through the correct path that assesses moment by moment the reached results and modifies intervention in relation to the achieved results as an investment for the future：because the person who has had the right aid today can become an autonomous citizen tomorrow,who therefore can take care of himself and can also be effectively inserted into the workplace.

Careful about waste,the Italian state is also implementing intelligent forms of supply,purchasing aids that can easily be reused for other people so that when an aid is no longer suitable for a child,it can be"made new again"and used for somebody else.