Post Polio Syndrome Presentation

Here's a presentation and dysphagia case study I made recently on Post-polio syndrome. I had never even heard of post-polio syndrome and didn't know much about Polio, so found i needed to do a lot of research.

Post Polio Syndrome and Dysphagia

Introduction

·   Polio is a highly infectious viral disease, which invades the nervous system and can cause total paralysis within hours.

·   No cure

·   1956 – routine polio vaccination program in the UK

·   Polio remains a widespread problem in 3 countries: Nigeria, Afghanistan and Pakistan.

Post Polio Syndrome (PPS)

·   NHS England estimates that there are around 120 000 people living in the UK who survived polio infections.

·   Many will develop PPS 25-80%

·   Unknown cause, main theory: ‘gradual deterioration of motor neurons damaged by the polio virus’

Jubelt et al 2000

Criteria for PPS

1.     Past poliomyelitis infection with residual motor neuron loss. 2.     Period of neurologic/functional stability after recovering from the acute illness (15+ years) 3.     Gradual/abrupt onset of new weakness/ abnormal muscle fatigue or generalised fatigue.

Clinical presentation

·   Generalised fatigue.

·   Muscle weakness, predominantly in muscles affected during the acute illness but also in muscles which were affected subclinically e.g. bulbar muscles.

·   Joint pain

·   Abnormal muscle fatigue and delayed recovery.

·   New weakness may result in respiratory insufficiency and bulbar muscle dysfunction (dysphagia, dysarthria, aphonia, facial weakness).

Sonies et al 1991

Dysphagia in patient’s with the post polio syndrome

·   Investigated the cranial nerves and swallowing of 32 patients with PPS using videofluoroscopy, ultrasound and a cranial nerve examination.

·   14 of the patients developed dysphagia alongside PPS.

Results

·   Mild – moderate cranial nerve impairments were found in all but one patient. For many these did not cause symptomatic dysphagia.

·   Impaired tongue activity was the most common sign of dysfunction with many patients requiring tongue pumping to initiate a swallow.

·   Uncontrolled bolus flow into the pharynx was very common.

·   Unilateral transport of the bolus through the pharynx was common (bolus moving asymmetrically and with less force).

·   Delayed oesophageal motility.

·   Pooling in the valleculae + pyriform sinuses

·   Delayed initiation of a swallow reflex

·   Aspiration was rare in all patients

·   Patient’s who had clinical bulbar involvement in the acute polio infection were much more likely to develop dysphagia in PPS.

Author’s hypothesis

·   Infrequent aspiration may be due to compensation for longstanding dysfunction by using accessory muscles or by postural adjustments when swallowing.

Key points

·   PPS can cause progressive dysphagia/ cranial nerve impairments.

·   Decreased pharyngeal transit/ feeling of food catching in the throat was the most common deficit of swallowing.

·   New dysphagia was more severe if there had been previous bulbar involvement.

Sonies, B and Dalakas, M. (1991) Dysphagia in patient’s with the Post-Polio Syndrome, The New England Journal of Medicine, 1991, 324, pp 1162-1167.

Jubelt,B. And Agre,J (2000) Characteristics and management of post polio syndrome, JAMA, 284(4), pp412-414.

The patient has not had any recent chest infections.
- The patient can walk (mobilise) with a frame.
- The patient has a foam neck brace which puts pressure on her larynx. She is unable to stabilise her head on her neck independently. When eating/drinking the patient takes off her neck brace. She leans on her armchair to support her head and is able to hold it upright for short periods.
- The patient's larynx did not appear to be in the midline. She appeared to have more severe left sided neck weakness.

-       The patient reported that her swallowing varies from day to day.

- Daily consumption - bread with marmalade and tea + small Oakdale mini meals. The patient reported that recently she has not been able to finish meals.


-       
Cranial nerve examination


-       VII - reduced left eyebrow raising, difficulties maintaining a lip seal when puffing out cheeks with air, the patient reports dribbling from the right side occasionally.


-       
V - NAD



-       XII - NAD


-       
X - Difficulties changing the pitch of her voice and the volume of her voice. Reflexive cough present, high pitch voice, slightly wet sounding voice. Voluntary swallow - effortful laryngeal elevation and difficulty triggering a swallow. 


-       
IX - nasal voice quality, pitch breaks on /a:/



-       Oral cavity - hydrated, no sign of infection


-       
Swallow assessment –

-       Consistencies: water + custard and bread with marmalade + biscuit



-       Pre -oral - the patient ate and drank independently when set up. She rested her head on her armchair to keep it in position. When eating some bread with marmalade the SLT fed the patient while she supported her chin in order to hold her head up and more in midline. 



-       oral stage - adequate transfer of bolus', adequate bolus preparation when eating crestless bread, no oral residue post swallow. Prolonged oral stage when eating a biscuit and effortful swallowing of the biscuit bolus.



-       Pharyngeal stage - difficulties triggering a pharyngeal swallow on solids more than on liquids, delayed pharyngeal swallow trigger, no significant desaturation of oxygen on fluids/ solids, hyolaryngeal elevation appeared effortful with some bobbing. The patient fatigued after 6-7 swallows and required a rest. Some throat clearing post swallow on solid foods.



-       A: Impression - the patient appears to have muscle weakness, difficulties with head support and with hyolaryngeal elevation and excursion. This may lead to reduced upper oesophageal sphincter opening and difficulties swallowing. The patient also fatigues rapidly when eating/ drinking. The left side of the patient's neck appeared weakest resulting in her larynx not being in the midline and resulting in her needing to rest her head on her armchair. The patient’s oral stage was prolonged for normal foods and her anterior to posterior bolus transfer was delayed. She may have base of tongue weakness/ reduced base of tongue to posterior pharyngeal approximation, which causes her to have greater difficulties swallowing solid foods. The patient did not show any overt signs of aspiration and does not have a history of chest infections. She did show intermittent signs of penetration.



-       Recommendations:
- Soft mashed foods
- thin fluids
- small mouthfuls 
- breaks when eating/ drinking.
- small meals throughout the day to manage fatigue



-       P: dietician referral 
- contact physiotherapy team about head support.
- review in a week and consider swallowing exercises for hyolaryngeal elevation but thinking about the impact of fatigue.

Presentation

 Polio aged 3, recent decline in function (Post Polio Syndrome) – was walking in communal areas, now difficulties walking due to weakness and lack of neck support, coughing on foods and not managing to eat full meals- severely underweight, deteriorating health. Lives in supported housing (one carer is on call), restrictive lung function – not getting enough oxygen when sleeping, care package twice a day (morning and evening), friend prepares all meals.

Initial Assessment

• Fatigue
• Laryngeal muscle weakness
• Effortful swallowing of normal foods
• Prolonged oral stage on normal foods
• No signs of aspiration
• Residue on normal solids may cause throat clearing. Could be due to reduced pharyngeal constrictor action or base of tongue approximation.

Task

• Eat a bite of biscuit or take a sip of water.
• Now tilt your head forwards and at an angle, so that your chin is almost touching your left shoulder.
• Eat/take a sip of water in this position.
• Reflect on how difficult it was to swallow in the tilted position/ whether more effort was needed.

MDT involvement

OT – advise on neck/head support, seating, fatigue management and clarifying the patient’s care package.

Dietician – estimated the patient’s BMI as 11.7 kg/m2 – significantly underweight (5.1 stone) and vitamin D deficient. Fresubin juicy found too thick and difficult to swallow due to taste. Calogen added to meals + protein and energy boosters.

GP – information on Post- Polio Syndrome

Patient’s friend – prepares her meals

Positioning

Papers on positioning in patient's with cerebral palsy showed that pelvic alignment supports trunk positioning in the midline and therefore supports neck alignment. If a patient is not well supported/ positioned it will lead to greater fatigue and effort required when eating/drinking.

Intervention: Fatigue Management

• Soft Mashed diet for main meal (less fatiguing). Soft snacks (crustless sandwiches) throughout the day.
• Main meal at lunchtime.
• Positioning – head control is affected by trunk alignment which depends on pelvic stability.
• Pillows under arms to increase stability.
• Small meals throughout the day.
• Rest period inbetween lunch and dinner.

Challenges

• Supported living – report able to provide patient with rest periods but only one carer to 42 people.
• Patient did not attend one of her orthotics clinics due to poor weather and feeling not 100% - waited months for the next one.
• Patient reports that she does not want plaster on her neck – this is needed for a personalised orthotic.
• Seating – patient does not fit criteria for the purchase of a chair. Small frame – unable to receive neck/ head support form behind.
• Diet – disliked puree/ soft mashed foods unless prepared by a friend.

Plan for the Future

• Liaise with the orthotics clinic (neck brace and eating and drinking).
• Liaise with the dietician re- goals for oral intake.
• OT to support the patient with purchasing suitable seating.

Reflections and questions