Informal Notes of Meeting with Majid Hashemi on 18 December 2014 regarding Achalasia at St John & St Elizabeth Hospital An informal meeting and question and answer session with the London Achalasia Meetup Group, which is linked to the Oesophageal Patients’ Association, was held at the above hospital by Majid Hashemi Mb, ChB, FRCS (Gen), Lead Consultant UGI Surgeon, Senior Lecturer. The meeting was arranged by Amanda Ladell, of London Achalasia Meetup Group, and Alan Moss, of the Oesophageal Patients’ Association. Majid Hashemi has had 26 years in surgery, with 15 years in oesophageal work. Thanks are due to David Marshall, Chief Executive, for allowing us to hold this meeting which was very much appreciated by the 27 guests (this includes 7 partners) who attended. Oesophagus is approx. 20 cm long and finishes at oesophagus junction with stomach. It is an active organ, with peristaltic contractions. Need progressive peristalsis, timing and pressure important Sphincter needs to function properly Hiatus needs good angle with oesophagus Sling of muscles like an arch. Need stomach and small bowel to empty well + saliva lubricating the passage well. Manometer good for pressure measurement 1 hr. manometry test, tube from the nose, not a pleasant test but gives a definitive answer What is it? Non relaxing lower oesophageal sphincter High resting pressure in lower oesophagus Poor contraction in the oesophagus Simultaneous and badly co-ordinated contractions in the oesophagus Contractions happening simultaneously Sphincter should relax, lose the inhibition of tone High resting tone and non-relaxing tone Low pressure. Simultaneous high pressure and non-relaxing sphincter Bolus non-clearance because sphincter not opening After treatments, use gravity for food to go to stomach Vigorous achalasia – high pressure contractions of 180 mm Hg – ‘nutcracker’ oesophagus. Healthy oesophagus generates 80 – 120 mm Hg Can get a whole range of pressures Chaga’s disease mimics achalasia Pseudo-achalasia can be diagnosed Chest pains hardest to treat by surgery and tricky to treat afterwards Multi-factors cause it including tiredness and how fit one is Myotomy doesn’t stop the spasm Oesophagitis can be the cause of the spasm Bananas help a lot Informal notes of meeting 22.12.14 Ann Elms 1 Treatment of the spasms Try nitrates Try calcium channel blockers Try Buscopan Gentle pressing downward in the middle area of the chest Deep breathing Stretching upwards Relaxation Standing on tiptoes and cracking down on heels vigorously – the shock helps to push the food down Role of fermentation and reflux causes pain Sucralfate can help as a temporary stopgap to give pain relief Try drinking warm water Try holding a hot water bottle on the chest to give pain relief Eat a piece of banana Pathology Loss of inhibitory gangli Viral – measles, varicella zostervirus, Chaga’s Genetic Nitric oxide Auto immune Research on vagal nerve & sympathetic nerves Sometimes colon does not have much peristalsis May have predisposition to cardiomyopathy Common presentation Dysphagia Regurgitation Reflux Pain Respiratory problems Laryngeal problems Can get aspiration pneumonia Post nasal drip Nocturnal cough – need to be most concerned about this Weak voice after 1 hour Get tired with having to talk loudly Diagnosis and Investigation History 1/3 – ½ do not pick up achalasia, mostly reflux is the cause endoscopy Barium swallow Informal notes of meeting 22.12.14 Ann Elms 2 Manometry “Bird’s beak” shaped oesophagus Sometimes treatment leads to wide open sphincter and oesophagus can fold over on top of itself and pooling can occur If treatment effective, this pooling doesn’t happen Who does it affect? Men and women 1 in 100,000 annually diagnosed 10 in 100,000 prevalence any age 16-80’s 1st presentation 30-60 years most common Some genetic predisposition Treatment Two parallel strategies Avoiding stress and exacerbating factors Medical Endoscopic treatment Surgery First treatment is the best chance of getting it right Type 1 – no peristalsis Type 2 – oesophageal pressurization Type 3 – vigorous - chest pains General question and answer session Botox has helped with chest pain, has had myotomy and is going to have POEM. Symptoms bad at night, cough if lying on the right side. Mostly people have problems if lying on the right. May have sigmoid oesophagus A machine for moisturising air helped nocturnal cough Lying on stomach can help cough but not recommended. Will be less likely to aspirate but not recommended as a position If you have aspiration problems, get it checked. Advise some elevation, upright with pillows is best ? many dilatations – 6 or 7 dilatations are safe Need to have durable solution and dilatation lasts only about 14 months Really effective dilatation or myotomy as first treatment is best Dilatation if too old for surgery. 40 mm dilatation with achalasia balloon is done first. Do a post dilatation swallow immediately. One achalasia sufferer had dilatation from age 19, now 42. Most people who had botox, need further treatments. Route if fit is to go either for dilatation or surgery. If that doesn’t work, then one still has the choice of dilatation or surgery Informal notes of meeting 22.12.14 Ann Elms 3 Third time surgery is not very easy with dilatation If unfit – dilatation or botox is the treatment Choice is linked to local expertise and patient choice. If you don’t have a wrap, you may get severe reflux. Fundoplication is wrap to reduce reflux. Wrap and fundoplication is essential and surgery is recommended if fit. Tablets to switch off acid, such as proton pump inhibitors like omeprazole, can lead to benign polyps and osteoporosis. Best not to be on them for long term but use them for a while and then stop. Presence of an ulcer – need endoscopy. Extremely rare to develop squamous cancer – irritation of oesophagus (much more common 20 years ago). Stasis and irritation of lower gullet Duodenal ulcer – not a side effect of achalasia Gastrin levels, amount of acid – biopsy and keep an eye on it What is likelihood of wrap loosening over time? Can have the wrap tightened. Measure the manometry pressure. The Mucosa is stronger if you haven’t had dilatation. Don’t want too tight a wrap or it might restrict too much. Some surgeons are pioneering the POEM procedure, but it is not recognized as standard procedure at the moment. It is more complex, and does not in itself provide the means for providing a “valve” to prevent reflux. Current thinking for many surgeons would be to recommend keeping to the safer and more controlled option of myotomy and a form of wrap for most situations. Candida A brief paper was tabled by a member who has suffered from candida of the oesophagus and how she dealt with it over the years before achalasia was finally diagnosed. It is a major contribution to dysphagia. If oesophagus is not emptying, it will become infected and one can get run down. Conventional treatment is with Nystatin which can be taken up to 5 times a day, or fluconazole – these can have side effects. Get source of candida reviewed Barium swallow and brushing After treatment, check if it has gone – swabs from throat? Endoscopy and brushing? Try the different tips for a short period. Watch use of probiotic as one can get over-growth of bacteria in the colon. Other effects of achalasia Can get extreme burping and tiredness 14 people in the room had had treatment, 4 had not had treatment Two have not had manometry Anything from oesophagus can be referred to back of throat All should have follow-up. 80% of people get 80% improvement If myotomy hasn’t worked, can have dilation Informal notes of meeting 22.12.14 Ann Elms 4 Can have revision myotomy Should not leave expanded oesophagus. If getting regurgitation will affect throat. Most achalasia sufferers eat less than they previously did. Buttermints can help. Pineapple juice can help. Need drinks at room temperature Mr Hashemi’s team are getting a trained psychologist on benign reflux team. For the next meeting, Mr Hashemi will aim to have a clinical psychologist and a dietician present. It is hoped to hold this in the summer. Informal notes of meeting 22.12.14 Ann Elms 5