DR. Irene AKHIDENO FMCA

Egyptian African Critical Care Summit 201710 Jan 2017

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Gullian Barr Syndrome (GBS) is a heterogeneous group of immune-mediated conditions

Variants: Incidence of 1-2/100000/year. Prior history of infection(respiratory or GIT) Require ventilatory support in 20% of cases. Active treatment :

Plasmapheresis intravenous immunoglobulin

Supportive therapy and multidisciplinary approach is very important. 80% :full recovery 16%: neurological deficit 4%: mortality

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15 year old female Prior history of vomiting Progressive history of neck pain and stiffness Inability to walk, weakness in both hands (global hypotonia) Diminishing speech Bulbar palsy and respiratory failure On admission: SpO2 of 71%, RR: 36 cycles/min, ETCO2 of 75mmHg. SIMV mode with an FiO2 of initially 100%, then stabilised at 60%. NG Tube feeding was passed as bowel sounds remained active. ??Administration of Immunoglobulin G Exchange blood transfusion for which only a unit was provided, also due

to cost. Care of the immobilized was instituted with passive physiotherapy,

administration of DVT prophylaxis.

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At 2weeks on admission in the ICU:

Tracheostomy : following consent

Pressure ulcers on the pinna, occiput, scapula, sacrum, elbows and both malleoli

Despite 2hrly patient turning

Air or ripple mattress in use.

Wound dressing with povidone iodine and honey was instituted.

Air ripple mattresses were made available by the 3rd month, as the pressure sores were getting larger, this remarkably improved healing of the pressure sores without formation of any new ones.

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2 weeks in ICU, some spontaneous respiratory efforts,

FiO2 reduced to 30%

SpO2 > 97%

ETCO2 within the 35-45mmHg range.

The programmed respiratory rate was adjusted to encourage her own spontaneous efforts to 6.

34th day in the ICU, her speech had returned and was audible though not sustained due to early exhaustion but progressively improved.

77th day, she had good swallowing reflex ,had stopped drooling saliva however there was no gag or cough reflex.

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At 5th month :

move the neck from side to side,

Return of the gag reflex

feeding was increased to 4hrly due to increase in appetite.

She was also noticed to be depressed,

Escitalopram 5mg daily.

Non-pharmacological methods were also employed such as reassurance and divertional therapy( music, cartoons and movies).

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By the 6th month,

improved spontaneous respiratory efforts ,

ventilatory mode was changed to Adaptive Support Ventilation (ASV) as a better weaning mode and assessment of her respiratory dynamics.

FiO2 of 30%..

She maintained good saturation > 97% ,

Endtidal CO2 range of 35 45mmHg,

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By the 8th month,

cough reflex had returned,

fasciculations were noticed in the muscles of the lower limbs as physiotherapy was twice daily

Commenced feeding by mouth

Celebrated her 16th birthday

9th month:

Attempted switch to spontaneous mode

Good tidal volume for 48hours

However by 72 hours gradual drop in tidal volume,SpO2 and rising ETCO2 necessitating return to ASV mode.

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Current status as at 9month on admission:

Shes still in the ICU,

Mechanically ventilated on ASV mode at 40% Min Vol

Good oxygenation

Stronger cough reflex .

There is still no recovery of motor activity in all in the limbs.

Enjoying her favourite meal ,noodles, by mouth

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Finance: no health insurance coverage

Intravenous Immunoglobulin G: expensive, unavailable

Equipment: Arterial blood gas analyser, financial burden of frequent payment for ABG sample analysis.

Pressure sores: these were obvious by 2 weeks on admission , despite frequent turning, the availability of the ripple mattress by the 3rd month greatly improved the healing of these ulcers as they would have been a nidus for infection and a cause of anaemia

Depression: fear of never walking, going back to school, leaving the hospital alive. Issues of abandonment by her parents as they had to source for funds outside the hospital. No child psychologist in the hospital so shes only seen by the mental health physicians.

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Recurrent urinary tract infection: necessitated fortnight changing of urethral catheter to mitigate this

Anaemia: blood loss from the pressure sores during dressing and nutritional anaemia. Feeds were fortified with blended crayfish to improve protein content

Hypothermia: loss of subcutaneous fat

Future: Home care /palliative care with neurological deficit

??? WAY FORWARD

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ICU staff : Zeal Humane Dedication

The versatile and rugged ventilator that has been functional non-stop for over 9 months has contributed immensely in the management of this patient

A compassionate and patient driven hospital management that has taken over the financial responsibility by waiving fees for medications, consumables and investigations.

Donations from well meaning hospital staff and Nigerians towards her care.

Patient gradual improvement: A first for us An appreciative and intelligent patient with a zeal to live.

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GBS as with other related polyneuropathies

that present with respiratory failure are a challenge to manage in resource poor settings due to unavailability of medication, equipments and all support specialties.

Prognosis is usually good

Recovery is very slow.

A dedicated and multidisciplinary ICU team, acute phase therapy, a versatile and rugged ventilator is vital in improving outcome.

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THANK YOU-SHUKRAN

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