A CHILD IN NEED OF CARE — GUILLAIN BARRÉ SYNDROME L

Guillian Barré-sindroom is die mees algemene polineuritis. Motoriese funksies word tot ’n groter mate as sensoriese funksies aangetal en die diagnose berus gewoonlik op simmetriese, distale en perifere spierswakheid. Sterftes aan die sindroom is gewoonlik die gevolg van respiratoriese arres en dus is vroeë diagnose en respiratoriese ondersteuning noodsaaklik. Terapeutiese sowel as verpleegsorg berus veral op goeie waarneming van die omvang van verlamming en die voorkoming van komplikasies. In die verpleging van ’n kind met die sindroom is die erkenning en hantering van die kind se vrese weens die verlies van Iiggaamsfunksies belangrik. Derhalwe is die daarstelling van goeie kommunikasie ’n noodsaaklike element van sorg.

It was a busy F riday afternoon when A nne was adm itted to the paediatric intensive care unit.A nne was four years old with mousy coloured hair, long eyelashes and very expressive brow n eyes.She was adm itted with m uscular w eak ness and her diagnosis was possible G uillain B arré Syndrom e.

GUILLAIN BARRÉ SYNDROME (Infectious Polyneuritis)
Infectious polyneuritis, also known as infectious neuronitis and L an d ry's or G uillain B arré syndrom e, is probably the m ost com m on form of polyneuritis.It is an acute polyneu ropathy in which m o tor dysfunction predom inates over sensory distur bances.It is characterised by sym m etrical ascending paralysis, in cluding bilateral facial paresis or paralysis, and occasionally w eak ness of the bulbar and respiratory m usculature.dicates that all ages, all races and both sexes are affected.A lthough children are less often affected than adults, the incidence in the paedia tric age group appears to be increas ing, with higher susceptibility in children betw een the ages of four and ten years (W haley, L .F .;W ong, D .L .1983: p 1492-1493).

Incidence
The average incidence of the syn drom e is 1.

Etiology
The precise etiologic-agent is un known.The syndrom e has recently been considered to be an im m une reaction in which the prim ary target is the peripheral nervous system.The im m une process m ay be trig gered by exposure to an exogenous agent, recent infection o r vaccina tion.
A n a n tig e n -a n tib o d y re a c tio n occurs, causing lym phocytes to becom e sensitised to the peripheral nerve antigen and to attack the myelin of peripheral nerve tissue (Sm ith, J.B .1983 : p 283).
Precipitating factors include: Ebstein B arr virus; m easles, m um ps, influenza, enteric viruses, m um ps/ ru b e lla v a c c in a tio n , d ip th e r ia , p e r tu s s is , te ta n u s v a c c in a tio n , (Shelov, S.P. et al 1984).

Pathophysiology
The pathological changes th at take place are prim arily of segm ental dem y e lin a tio n o f th e p e r ip h e r a l nerves, that is loss of the fatty m yelin sheath (see figure 1).A lthough the myelin is affected, the structure of the axon is usually spared, these degenerative changes affect spinal and cranial nerve roots in ascending order.T he result of these degenerative changes is the slowing or total blockage of nerve conduction, producing a rapidly as cending partial or com plete p ara lysis of muscles innervated by the nerves involved.
C o n c o m i t a n t c h a n g e s a r e oedem a, inflam m ation and com pression of nerve roots within the dural sheath.The anterio r spinal roots or motor neurones are primar ily affected, causing disturbance in m ovem ent.Posterior spinal roots or sensory neurones are also affected resulting in disturbances of sensa tion.O ther nerves involved are the cranial and autonom ic nerves.

Clinical presentation
Paralysis is usually preceded by a mild influenza-like illness or sore throat.
The prim ary presenting feature of Guillain B arré syndrom e is sym metrical, distal, peripheral muscle weakness.Leg weakness is pro nounced but arm weakness may also be present and may progress to full flaccid paralysis with loss of'reflexes.
Facial, extraocular, lingual, pha ryngeal and laryngeal muscles may be affected.Evidence of intercostal and phrenic nerve involvem ent in cludes breathlessness with shallow irregular respirations.Most patients complain of muscle tenderness, cramps or sensitivity to slight press ure.Urinary incontinence or re tention and constipation are fre quently present.
The height of paralysis is usually reached within a few days after onset, although the progression of the paralysis may persist for up to three weeks.
Sensory involvem ent is variable.Position sense as well as sense of touch, pain and tem perature may be im paired.Paraesthesia (num b ness or tingling) of toes is present in a glove-and-stocking fashion, tend ing to be interm ittent during the ini tial stage of the illness.
Cranial nerve involvem ent is p re sent to some degree in the m ajority of patients.The facial nerve is most commonly affected.Dysphagia and laryngeal paralysis implicate the glossopharyngeal nerve.
A utonom ic dysfunction manifests itself mainly by orthostatic hypoten sion and/or transient hypertension.
Excessive sym pathetic activity is evidenced by hypertension, persist ent sinus tachycardia, episodes of delirious behaviour, sudden profuse sweating and peripheral vasocon striction.Insufficient sym pathetic activity results in orthostatic hypo

Prognosis of Guillain Barré syndrome
Guillain Barré Syndrome occurs in various degrees of severity, but all affected patients will require hospi talisation.Some children may never progress to full paralysis and may only require observation, whereas others will require acute care.The general health of the child and the extent of the paralysis will influence the outcom e of the illness.Almost all deaths result from res piratory failure, therefore early diagnosis and respiratory support are essential.Muscle function may begin to return any time between two days and two weeks after onset of the disease, recovery being de pendent on the extent of demyelination.The longest peripheral nerves are the last to recover and the greater the degree of paralysis the longer the recovery phase, which may extend from a few weeks to months.

Diagnostic evaluation
C urrently, there is no specific test to confirm the diagnosis of Guillain Barré syndrom e.The diagnosis is based on the collective inform ation gained from the p atien t's history, clinical sym ptom atology, progres sion and laboratory results.C ere brospinal fluid analysis reveals an increased protein concentration but in essence other laboratory studie; are non-contributory.

Therapeutic management
The m anagem ent of Guillain B arré syndrom e is sym ptom atic and de pends upon the stage of illness and the extent of m uscular involvem ent.The m anagem ent regime is one of highly supportive care.
C orticosteriod therapy has been used without conclusive results.Plasmapheresis has been investi gated, based on the idea that the etiology is im munologic -by re moval of the circulating antibody specific for peripheral nerves, clini cal im provem ent should follow (Smith, J.B .1983: p286).

Nursing Care
The therapeutic care of this child is highly supportive and so is the nurs ing care.The em phasis of care is on close observation to assess the extent of the paralysis and on pre vention of com plications.
During the acute phase of the disease the child's condition should be carefully observed for possible difficulty in swallowing and respira tory involvem ent.T here should be a respirator attached to the patient with a cardiac m onitor on standby.Suction apparatus is essential, with a tracheostom y tray and vasocon strictor drugs available at the bed side.Vital signs and level of con sciousness are m onitored halfhourly.
For the child who develops respi ratory dysfunction, the care is the same as that for any child with res piratory distress requiring m echan ical ventilation.
Throughout the recovery phase special em phasis is placed on pre vention of com plication -includ ing good postural alignm ent, fre quent change of position and pas sive exercises.Children with oral and pharyngeal involvement are usually fed by m eans of a nasogas tric tube to ensure adequate feed ing.
Bowel and bladder care are needed to avoid constipation and retention of urine.Sensory im pair m ent makes the child susceptible to trophic ulcers therefore skin care and care to pressure areas is im por tant to prevent a pressure sore.
Physiotherapy is limited to a pas sive range of m ovem ents during the acute stage of the illness.L ater, as the disease stabilises and recovery begins, an active physiotherapy program m e is im plem ented to p re vent contracture deform ities and to facilitate recovery of muscles.This will include active exercise, gait training and p erhaps the use of cal lipers.
Communication R ecognition of the child's fears and feelings of loss o f body control is a prim ary elem ent in providing care during the acute phase of the syn drom e.In som e instances, children may find them selves totally p a ra lysed, intubated and helpless within 24 hours after adm ission to the pae diatric intensive care unit.
Initially the paren ts will also be fearful and helpless.It is of the u t m o s t i m p o r t a n c e t h a t c o m m unication be established.The child and family need to feel a sense of security in th e intensive care en vironm ent.
If the child still has facial muscle control it is possible for him to com m unicate by m eans of eye signals and head nodding.T he use of pic ture boards and language charts is helpful.A t th e bedside, the nurse should always speak to the child, tell the child w hat will happen, talk about topics fam iliar to the child and rep o rt news from hom e.The parents can becom e involved by reading stories and assisting in the child's daily care.
Establishing a daily routine is helpful in allowing the child to gain some control over the environm ent and assisting th e child to becom e fa miliar with the hospital personnel.
The recovery period of G uillain B arré syndrom e m ay be as long as several m onths, therefore open com m unication and the establish m ent of trust are extrem ely im por tant.

Resumé
A nne was an only child who lived with her parents on a farm not far from the city.She loved the anim als and would spend as m uch tim e as possible with h er fath er helping him with certain activities on the farm .E ven though she was only four years old, her father described her as his little helper.
A nne had not really been ill before, apart from the occasional cold.T he course of her illness, Guillain B arré syndrom e, had fol lowed very much the sam e pattern as that described in the text books.She experienced a cold and sore throat about a week before the onset of sym ptoms of muscle w eak ness.The progress of the disease had been rather rapid and within 36 hours of admission she had been in tubated and was receiving m echan ical ventilation.
It is custom ary in most intensive care units, particularly in the paedi atric units, to have a child cared for throughout his/her stay by the same team of people as far as possible.H o s p ita lis a tio n is fr ig h te n in g enough for the child, let alone ad mission to an intensive care unit.I was on duty at the tim e of A n n e's adm ission and she becam e my patient/responsibility/friend.Anne was the first child with G uillain B arré syndrom e that I had nursed.I becam e extrem ely interested in her care, which had to be highly sup portive.
O ne thing w orried us and that was our inability to com m unicate adequately with A nne (rem em ber she had been intubated and was on a ventilator).W e w ere continuously m et with fearful brow n eyes.We tried pictures, stories, and animal noises -but nothing seem ed to help.N ot even her parents seem ed to m anage and they tried so hard.We were all beginning to despair and then C harlie the clown came into her life.
As I walked past a toy shop one day I was w ondering if there was anything that could possibly help us to com m unicate with A nne.I stopped to look into the window and there was C harlie, a hand puppet, alm ost jum ping up and asking to be bought.T here w asn't a second thought and the next m orn ing C harlie and I w ent on duty to gether.
My first inclination was to in tro duce C harlie straight away.H ow ever, I decided to wait for a quieter m om ent.T here was a certain am ount of apprehension on my part as I feared C harlie's rejection.The big m om ent arrived and for the first tim e since intubation we saw those big brown eyes smile.
Charlie was to play an im portant role in A n n e's care -he was always available to help us with medical and nursing procedures and the games he played m ade physioand occupational therapy fun.His help was invaluable and above all, he helped to foster two-way com munication.
A nne was intubated for two weeks after which she was weaned from the ventilator.She continued along a slow but steady p ath to re covery.She spent ano ther three weeks in the intensive care unit and was then transferred to an outlying ward.H ere, with C harlie's help, she w ent from strength to strength until discharge two m onths later.R esidual dam age was a persistent paralysis of one of her toes.C harlie and A nne had becom e inseparable and I am told that this situation re m ained so for many years.

Conclusion
The facts which have been related em phasise the im portance of com m unication in the care of our patients, a subject about which much has been w ritten.W e cannot m anage our lives w ithout com m unication.
As nurses each of us has special patients whom we rem em ber long after discharge.A nne and Charlie are special as they have contributed to both my professional and perso nal growth.
Statistical d ata relating to the inci dence of G uillain B arré Syndrom e in South A frican is unobtainable.H ow ever, A m erican L iterature in-Miss L Acres, RN, RM, Registered Pae diatric Nurse, DNE(Wits), DNA(Wits).Senior tutor, College for Advanced Mid wifery and Paediatrics, Baragwaneth College of Nursing.
7 p er 100,000 population in A m erica and geographic loca tion, season and clim ate m ay affect the incidence of G uillain B arré Syn drom e (N elson, D et A l 1979 : p 1029-1033).

Figure 1 :
Figure 1: Segmental demyelination of the peripheral nerves

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tension and poor tone, causing pooling in dependent areas.Exces sive parasym pathetic activity results in bradycardia, facial flushing and generalised extrem e warm th.Insuf ficient parasym pathetic activity re su lts in s p h in c te r d is tu rb a n c e s (Lichtenfeld, P 1971 : p 771-780).