NET Training Day: Whittington Hospital 07/06/17

On a sunny day in June we were welcomed to the Whittington Hospital by Dr Ruvini Dharma. Many thanks to her for organising the day, and for inviting colleagues from further afield to provide a specialist perspective.

Inhaled treatment in COPD – an update from GOLD

We started the day with a welcome update on COPD treatment from Dr Melissa Heightman. She began by discussing the work of the Joint Responsible Respiratory Prescribing group. There are many new inhalers now available, and NICE guidance is old – 2010 – and therefore out of date. We await new NICE guidelines due in 2018, but in the meantime we have to put new evidence into practice. GOLD produced new guidance in February 2017 and a number of trials have reported recently.

In the old NICE guidance inhaler choice was determined by FEV1 – it often suggested triple therapy, even if the patient was not exacerbating frequently. THere have since been concerns due to the link between ICS use and pneumonia risk.
In the FLAME study a LABA/LAMA was put head to head against Seretide. The outcomes were days to first exacerbation, and exacerbation rate. In this study LABA/LAMA was more effective.

For the new GOLD guidelines 267 papers were reviewed. Ch4 of the guideline is on management of stable COPD. Helpfully, teaching slides are available from the website. There is a focus on comorbidity in COPD, and on quality assured spirometry. In the UK a recent change has been the need for accreditation for quality spirometry through ARPT.

The new GOLD grid includes symptoms and exacerbation rate in addition to FEV1  and classes severity of COPD as ABCD. The definition of an exacerbation is important, including whether it is mild vs moderate. This is open to some subjective interpretation which could upstage or downstage a patient in terms of severity and therefore treatment.

New GOLD requires use of CAT score and mMRC dyspnoea scale (remember on mMRC 3=2 on MRC). The statement on bronchodilators in stable COPD is useful, as is the decision not to advocate the use of oral theophylline.

In the TORCH study a combination ICS/LABA was more effective than separate drugs. However, the use of ICS was associated with an increased risk of pneumonia. Triple therapy improves lung function and health status but it is unknown whether triple therapy is superior to LABA/LAMA. Studies are awaited.

PDE4 inhibitor Roflumilast is included in the GOLD guidelines, but is not yet licensed in the UK outside clinical trials.

Azithromycin is supported in GOLD in appropriately selected patients. Mucolytics are supported as adjunctive treatment. Of course, non-pharmalogical therapies are key to optimal patient care.

Mel went onto discuss the work of the Responsible Respiratory Prescribing group which have created new inhaler pathway through an MDT approach, which includes patient representatives. They have settled on a similar approach to GOLD but the pathway is simplified and gives recommendations on specific inhalers/devices (based on patient preferences). It aims to minimise device switching. The project has been collaborative. It has been put to the joint formulary committee for approval.

Tips on inhaler choice :

• If creatinine clearance <50 tiotropium is not recommended. Ellipta Umeclidium is an alternative LAMA.
• If there are any features of concomitant asthma Fostair (ICS/LABA) is recommended.
• Lots of patient feedback is essential when deciding on devices.
• Seretide is dead (on these guidelines anyway).
• Nearly all devices are dry powder form. These allow a greater range of inspiratory flow. Most patients have adequate flow to use these devices. Once daily.
• Consider the environmental impact of device choice – DPIs have a lower carbon footprint than MDIs.

Do you have the RightBreathe app? Very useful, and includes patient instruction techniques for various inhalers

The National COPD Audit: improving patient safety and outcomes

Dr Louise Restrick was up next, with data on the concept of value based care and putting it into practice.

We began by considering the question ‘What is Value in Healthcare?’ as posed by Harvard Prof Michael Porter.

• Porter, Michael E. “What is value in health care?.” NEJM 363.26 (2010): 2477-2481.

One answer is that it is the best outcomes for the cheapest cost. So what are the high value interventions in COPD?
Wales primary care audit data (England did not agree to data sharing), is helpful to analyse as it shows  significant unwarranted variation in care. It also shows that the value pyramid is not as it should be – the most high intervention interventions (flu vaccine, stop smoking, PR) are not the most consistently delivered. Triple inhaled therapy is common. The COPD bundle is evidence based and comes from QI work.

The National COPD audit is prospective, continuous, and importantly public. Unwarranted variation will now be be public. This is a motivator for Trusts and CCGs. The impact of bundles on readmissions is significant. There is a CQIN for COPD bundle delivery. Livery et al PLOS One 2015.

• Laverty, Anthony A., et al. “Impact of a COPD discharge care bundle on readmissions following admission with acute exacerbation: interrupted time series analysis.” PloS one 10.2 (2015): e0116187.

Louise challenged us to answer 3Qs in relation to the National COPD audit:

1. What are the ‘right care components ‘of a respiratory review within 24hrs? Diagnosis of respiratory failure? Can this be one by any doctor? Whose responsibility is it? What involve should acute physicians have?
2. How do we make COPD bundle completion part of the care of every patient? Whose responsibility is this?
3. How will data for the National audit be collected, recorded and uploaded? Whose responsibility is this? What resources are available? What is the opportunity cost?

There are major challenges to timely consistent data collection e.g. when compared to #NOF. The numbers of COPD exacerbations are greater, stroke always goes to 1 speciality, 1 location, 1 team, whereas COPD exacerbations can be anywhere in the hospital under any medical team. Stroke is a definite diagnosis with defined clinical and radiological criteria. COPD exacerbations have a high mortality – these are complex patients with multiple co-morbidities. They need multiple interventions that take time. It is widely acknowledged that there is not enough respiratory resource.

What does the bundle look like in your hospital? What staff are the ‘resource’ available to help? Respiratory SpRs, nurse specialists, Consultants, Physios, Pharmacists, smoking cessation people, community nurses?

There are a number of areas where care could be improved for patients with COPD in hospital

• Make the right diagnosis – not all smokers have asthma.
• What does LRTI mean? Is it an AECOPD or is it CAP? This matters due to differences in treatment, outcomes, and mortality.
• Surviving sepsis – patients with COPD will be excluded from qSOFA if they are not given the correct diagnosis and are labelled as AECOPD
• Dr Restrick advised us not to use DECAF as it includes consolidation.
• In the 2014 COPD audit only 50% had dx by spirometry at any time – this needs to change

We moved on to think about oxygen. Mortality is high if high-flow O2 is used to treat AECOPD.

• Roberts, C. M., et al. “Acidosis, non-invasive ventilation and mortality in hospitalised COPD exacerbations.” Thorax (2010): thx-2010.

O2 prescribing is still poor and patients continue to suffer O2 poisoning. We must get the basics right.
NIV halves mortality under optimal conditions with appropriate patient selection. However, audit data shows that it is not used effectively or appropriately. There is far too much NIV used in an attempt to treat metabolic acidosis!! There is poor documentation of plans if NIV fails. Treatment escalation plans are lacking.

Smoking cessation advice is also still poor, even for COPD patients. We were asked whether we felt it was part of our role to treat tobacco dependence as a Resp SpR? YES of course! Ask, advise, act. Resources available. The Quit at the Whit box is a useful example of a simple intervention to support inpatients. CO monitors can be a valuable adjunct, used as a motivating tool. At the Whittington they managed a 50% 6 month quit rate with varenicline and intensive support. This requires enthusiasm, commitment, and a team approach.

• Ainley, A., et al. “P285 Assessing The Impact Of Varenicline Initiation During Acute Hospital Admission For Current Smokers With Respiratory Diseases: 18-month Experience From An Inner City District Teaching Hospital.” (2014): A199-A199.

In regards to Pulmonary Rehabilitation the story is no better! For every 100 patients 40 complete PR. It is important to motivate patients to attend. Dr Restrick advocates for the question “Would you like to breathe better, feel good, do more?” as a way to introduce PR. To get better access to PR we need to make the case that it is clinically and cost effective. Coding for COPD exacerbations in ICD + HRG are ICD J44 HRG DZ65. Tariffs are related to LOS and complications.

Best Practice Tariffs – there is a new one for COPD which means that the Trust gets a top up payment if ‘best care’ is achieved. This started April 2017. This is worth £280,000 at The Whittington over 2 years. On this basis, the service can make the case for funding for a data manager or specialist nurse. Therefore this has impact! Opportunities are created by financial incentives and targets. The Public dashboard and financial levers give us a chance to improve care at scale and pace. But only if we lead, and take ownership.

A Best Practice Tarriff will be awarded to a Trust where:

• 60% of patients with a primary diagnosis of COPD, admitted for an exacerbation of COPD, receive specialist input to their care within 24 hours of admission; and
• Where they receive a discharge bundle before discharge.

SLT and Clinical Nutrition in Resp Care: a Collaborative Approach  

Mark Livingston and Claire Connelly gave us an overview of the role of the Speech and Language and Nutrition teams in COPD. There is now lots of literature on dysphagia, and vocal cord dysfunction in COPD. Dysphagia may occur due to: weakening of pharyngeal muscles; reflux; poor co-ordination of swallowing and breathing; or previous intubation. As part of the normal physiology, when you swallow you actually have an apnoeic period!

• Gross, Roxann Diez, et al. “The coordination of breathing and swallowing in chronic obstructive pulmonary disease.” American journal of respiratory and critical care medicine 179.7 (2009): 559-565.

Case study: A 56yr old woman with COPD had a 5 month ICU and ward stay. She had a background of bronchiectasis secondary to Radiotherapy fibrosis, Breast cancer and ALL (with allograft BMT). Clearly this was complex and during the admission she was intubated. She was referred to SLT for weaning of trache, communication, and dietetic advice.
NG tube initially.

Trache —> speaking valve. Dysphonia was identified. AFEES was done – useful, can be done at bedside (Video fluoroscopy has to be done in imaging dept). We were shown a video of FESS which showed pooling, and delayed trigger. We also saw a Video fluoroscopy of silent aspiration.

She also had lots of problems with fatigue which was a major barrier to therapy. She was not sleeping at night and was initiated on nocturnal NIV. Once she had a better sleep/wake cycle she was able to start therapy. A major goal for the patient was to eat and drink. She loved food. She made it up to 50% of her nutritional needs being achieved via oral intake. After long discussions she went for a PEG for additional nutritional support.

Recommendations for trainees:

• go and see a FEES
• find out what support is available in the community – generally poor

The evidence for LVRS

Mr Marco Scarci helped us all to consider which of our patients we should be referring for LVRS.

For patients on maximal medical therapy, who have completed PR and remain breathless, surgery is an option that should be considered and is under-utilised.

Surgical options include: transplant, LVRS, and bronchoscopic LVRS techniques.

Surgery today is very different from surgery reported in trials e.g. NETT and therefore risks and morbidity are less.

 

We watched some great videos of surgical interventions in COPD, including VATS bullectomy, talc pleurodesis, pleural tenting etc. YouTube has some great videos of surgery, but is no replacement for going and watching a list yourself. Your local cardiothoracic surgeon will be more than happy to host you!

LVRS in appropriately selected patients leads to improvement in FEV1 and dyspnoea score. So which patients benefit? NETT criteria are now outdated. Patient selection remains crucial. Local surgeons use the Zurich Hospital criteria now. The key is hyper-inflation, which is confirmed on spirometry by a high RV. If the patient is not hyper-inflated they will not benefit. The greatest benefit is when the RV is highest. If FEV1 <20% and DLCO <20% and homogenous disease do not do surgery! These patients have a high mortality and poor outcomes.

When comparing LVRS with medical treatment, there is initially a higher mortality but over time surgery does better. Surgeons will consider patients with homogenous as well as heterogenous emphysema. Heterogenous do better, but there are benefits for others if carefully selected.

Evidence for valves comes from a number of trials, with a recent increase in publications:

• VENT trial 2010 – A Randomized Study of Endobronchial Valves for Advanced Emphysema. Concluded that “Endobronchial-valve treatment for advanced heterogeneous emphysema induced modest improvements in lung function, exercise tolerance, and symptoms at the cost of more frequent exacerbations of COPD, pneumonia, and hemoptysis after implantation.” 
• BeLievER trial 2015 – Bronchoscopic lung volume reduction with endobronchial valves for patients with heterogeneous emphysema and intact interlobar fissures (the BeLieVeR-HIFi study): a randomised controlled trial. Concluded that “unilateral lobar occlusion with endobronchial valves in patients with heterogeneous emphysema and intact interlobar fissures produces significant improvements in lung function. There is a risk of significant complications and further trials are needed that compare valve placement with lung volume reduction surgery.”
• STELVIO trial 2015 – Endobronchial Valves for Emphysema without Interlobar Collateral Ventilation. Concluded that “Endobronchial-valve treatment significantly improved pulmonary function and exercise capacity in patients with severe emphysema characterized by an absence of interlobar collateral ventilation.“
• IMPACT trial 2016 – Endobronchial Valve Therapy in Patients with Homogeneous Emphysema. Concluded that “EBV in patients with homogeneous emphysema without collateral ventilation results in clinically meaningful benefits of improved lung function, exercise tolerance, and quality of life.”

Complications of valves: pneumothorax up to 25%, pneumonia <5%, COPD exacerbation up to 10%, granulation tissue formation 5%, expectoration of valves 5%.

No longer exclusion criteria: alpha-1, homogenous emphysema, LL predominant emphysema, pHTN.

The valves are unidirectional valves. Collateral ventilation can be a problem, so it is important to assess fissure integrity. This is done using quantitative CT. The Chartis system measures air flow from one lobe to another. If there is no collateral ventilation then there is no flow. StratX software is used to analyse fissure integrity after thin slice CT. This helps direct the type of treatment and correlates quite well with Chartis.

Current practice treatment pathway:

• Have to stop smoking at least 6 months before intervention.
• RV 175% predicted, complete fissures —> valve therapy.
• If any doubt —> Chartis +/- valves if appropriate.
• If fissures not intact then go for surgery.

Coils are an alternative but do not have such good data. Trials are ongoing at the Brompton. These are an option for homogenous emphysema.

Familial Pneumothorax 

Dr Stefan Marciniak inspired us all to get excited about the genetics of PSP.

In PSP it is very possible to make interesting genetic diagnoses which can be satisfying for you, and useful for patients. Up to 14% of PSP are familial. The incidence of PSP is 1/10000 population. 25 men, 5 women. 24% familial in women, 25% endometriosis.

We considered the eternal question: does size matter? USA and UK guidelines differ but both are based on ‘expert opinion’. A multicentre prospective audit found that if patients are treated according to BTS guidelines, fewer unnecessary chest drains are inserted, but more patients return needing a procedure at a later date. Since this is not a fatal condition, this seems the right balance.

• Nikolić, Marko Z., et al. “Noninterventional statistical comparison of BTS and CHEST guidelines for size and severity in primary pneumothorax.” European Respiratory Journal 45.6 (2015): 1731-1734.

Simple aspiration was equally effective for PSP in 3 RCTs. Small bore drains <14FF are effective – many studies show this and there really is no need for a large drain.

• Horsley, Alex, et al. “Efficacy and complications of small-bore, wire-guided chest drains.” CHEST Journal 130.6 (2006): 1857-1863.

If the drain is bubbling 48hrs —> put on suction. -10-20cm H2O = 1kPa.

Another frequent debate is to clamp or not to clamp!? There is in fact evidence both ways. There IS a risk of death with a clamped drain, but the risk only occurs if that patient is constantly monitored, so it is very environment dependant. A 3 way tap seldinger drain is potentially more dangerous as it is unclear if the drain is clamped to staff especially on handover. Many units use actual clamps or attach a sign to a clamped drain.

Dr Marciniak also discussed Heimlich valves. There is significant interest in ambulatory pneumothorax management but Heimlich’s are actually not v convenient. The RAMPP study is currently ongoing in Oxford using a new device (Rocket Pleural Vent). The end point is LOS.

We discussed pleurodesis post PSP. In the US patients are offfered pleurodesis after their 1st pneumothorax (risk of recurrent 30-50%). In UK usual practice is to offer the procedure after 2nd pneumothorax (risk of recurrence much higher). The morbidity of the condition and morbidity of the operation (which is now minimally invasive) is becoming more similar, favouring intervention. Options include tetracycline, blood patch, talc, via thoracoscopy (medical or surgical).
Pleurectomy is much more effective (abrasion less good). Recurrence <5%.

Guidelines are vague on when a CT is required. Generally, anyone who is considered for surgery, anyone with a persistent air leak at 5 days for PSP, 2 days for SSP, CXR abnormality, ALL WOMEN (according to Dr Marciniak, since the rate of pickup for interesting diagnoses is good – up to 50%).

Specific disorders associated with pneumothorax:

Acquired:

• Cannabis – leads to emphysema in 30 year olds. There is a characteristic appearance of typical cannabis lung. It is unclear whether the cause is primarily the drugs, the temperature of inhalant, or the technique of inhalation.

Genetic: GeCIP (Genomics England Respiratory) are doing whole genome sequencing. If you see all pneumothoraces for follow up, every 10th patient will have an affected family member with pneumothorax. If you look, you will find interesting conditions.

• Defects of mTOR signalling – BHD, LAM
• Defects of ECM – MFS, LDS, vEDS, others
• Unknown unknowns – about 50% cases.

Birt-Hogg Dube syndrome is a condition in which there are cysts in lower zone/bases. 50% are below level of carina. There are few prospective studies but 30% develop cysts, with recurrence rate of 75%. Up to 10% of familial pneumothorax cases are BHD. It is the most common cause of genetic pneumothorax. Skin lesions: epithelial and fibrous tissue. Fibrofolliculomas, trichodiscomas, acrochordons (skin tags). Develop later – in 40s. 12-34% lifetime risk of Renal Cell Carcinoma. Can be multi-focal and bilateral. RCC happens later 40-60s. Curable cancer if excised before 3cm. Therefore follow-up these patients with lifelong annual screening by MRI (or renal USS? less reliable). Important to inform family members – can save them from death from RCC.

Tuberous Sclerosis Complex / LAM. AD mutations in TSC1 or TSC2. 9/100000 births. Cutaneous angiofibromas. Renal tumours (clear). Pulmonary LAM 26-49% TSC-affected women. Progressive lung loss and will need transplantation later. Must involve National LAM centre in Nottingham. Personalised medicine. Sirolimus slows progression through mTOR inhibition. Patients typically present in pregnancy with pneumothorax. VEGF-D correlates – may have diagnostic and prognostic value.

ECM defects include Marfans syndrome – see the revised Ghent criteria 2010. You cannot just base on Fibrillin protein as v polymorphic. If dilated aortic root and >7 points on systemic score then diagnostic. Get 2 points for having a pneumothorax. Arachnodactyly – Sternberg thumb sign, Murdoch-Walker wrist sign.

Loeys Dietz syndrome. LDS1-5. Features include thoracic aortic aneurysms, OA, skeletal abnormalities. TGFB mutations. Midline fusion defects. Patients need annual ECHO and MRI every few years. Pregnancy assoc complications can occur due to ruptured uterus.

• Chambers, Joseph E., et al. “Spontaneous pneumothorax can be associated with TGFBR2 mutation.” European Respiratory Journal(2015): ERJ-00952.

Vascular Ehlers Danlos syndrome. Collagen 3A1 gene.  10/million. Rare. 80% life threatening problem by 40. Mean age of death 48. V bad! Features include a high arched palate, stretchy skin, hypermobility. A simple screening test for this condition is the Brighton score – we were recommended to do on all pneumothorax patients. Look at Ehlers Danlos website for the test which gives you a score out of 9. Abnormal if 5. These patients may be seen by Rheumatology for what is labelled as ME due to aches and pains. Some have characteristic facial appearance. By making the diagnosis you can extend patients life as they will die of a ruptured aorta if unrecognised. Can intervene with drugs e.g. B-blockers etc to delay dilatation of aortic root +/- offer surgery.

There is a bunch of other small print stuff! Then a load of unknown unknowns. So this is fantastic territory for the 100,000 genome project. It is now recruiting familial pneumothoraces. If you have a patient with familial neumothorax who does not have Marfans or BHD then recruit them! They have to be reviewed in genetics MDT first to exclude these conditions. It is FREE to get sequenced. Can be 1,2,3ary family member. If in doubt, contact Dr Marciniak.

Thanks again to all those involved in organising a fascinating day.

See you at the next training day. Don’t forget GIM training sessions too. Check the latest news and opportunities for an up to date list of training days.