Saint Patrick and alpha-1 antitrypsin deficiency

Perhaps the most significant of the “…mad March days” is the 17^th – Saint Patrick’s day. Saint Patrick is of course the patron saint of Ireland and this month we bring you work from a team in Dublin who describe the phenotype of SZ alpha-1 antitrypsin deficiency (page 298); see also the linked editorial by Quinn and colleagues on page 214. The ZZ genotype is linked to COPD regardless of smoking and the MZ genotype leads to COPD, mainly in smokers. What then of SZ? Using registry data from 486 patients, with spirometry and CT outcomes, the authors find that the SZ alpha-1 antitrypsin deficiency phenotype resembles the milder MZ rather than the severe ZZ form. Saint Patrick used the three leaved shamrock to explain the holy trinity. The same symbol might also be used to explain the unholy trinity of alpha-1 antitrypsin deficiency: ZZ, PZ and SZ.

A cargo of specialised gym equipment?

In Masefield’s poem, the “Dirty British coaster…” carries a cargo of very basic equipment: “…Tyne coal, Road-rails, pig-lead, Firewood, iron-ware, and cheap tin trays.” In this month’s Thorax, Patel and colleagues (page 264) describe pulmonary rehabilitation for COPD, with basic equipment vs rehabilitation conducted with access to a specialist gym. The study includes 318 consecutive patients undergoing the low tech intervention who were propensity score matched to 318 patients attending a gym. The authors find that pulmonary rehabilitation conducted in church halls and community centres is non-inferior to delivery in a specialist gym. The potential role of iron-ware and cheap tin trays has yet to be evaluated…

Cargoes, topographies and clinical outcomes

The ships of Masefield’s poem come from a variety of topographies, from the Middle East, to Tyneside by way of the tropics. Their cargoes are equally varied, ranging from precious stones to “…sweet white wine”. Similarly different topographies of the lung (from the subpleural tissue to the airway) carry differing “cargoes” of micro-organisms. In this month’s journal, Valenzi and colleagues (page 239) use 16 s rRNA sequencing to characterise microbial communities in the lungs explanted from patients with idiopathic pulmonary fibrosis (IPF). In IPF they find a higher bacterial load in the airways (compared with sub-pleural tissue). The authors suggest there is a distinct subgroup of patients with IPF with higher bacterial load and worse clinical outcomes. Sadly, the clinical outcome for John Masefield was a gangrenous infection which was ultimately fatal. He is buried in Poets’ Corner in Westminster Abbey.

Mad March Hare Sportive

Less cultured members of the Editorial team were concerned that March madness might elude them, but as luck would have it March is often when cyclists test their reliability after a winter of, well, wintery weather. Early season rides are used to assess reliability, responsiveness and difference in form from the previous season. They tend to lack objectivity, and therefore is of little generalisable value, although a great way to start the cycling year.

It is not just cyclists who understand the importance of activity, Pulmonary Rehab for patients with COPD has highlighted the importance of activity in people with lung disease, but objective assessment can be a challenge in determining the value of Minimally Important Differences. In this issue of Thorax (page 228) Garcia-Aymerich and colleagues describe the detailed assessment of two activity tools, the Daily-PROactive and Clinical visit-PROactive Physical Activity (D-PPAC and C-PPAC) instruments, that combine questionnaires and activity monitors. They found that the activity scores were a reliable and valid across a range of COPD populations and could measure clinically relevant changes following both pharmacological and non-pharmacological interventions. Maybe, these should be used to assess the effect of winter training, rather than putting yourself through the pain and suffering of a reliability ride. If you are not convinced sign up for the Mad March Hare ride instead!

Remember, All Things Will Die

Two wonderful poems summarise the inevitability of the human condition. Tennyson’s classic All Things Will Die (https://genius.com/Alfred-lord-tennyson-all-things-will-die-annotated) describes the paradox that is the wonder of life mixed with the pain of death and Remember is a beautiful poem by Christina Rosetti helping loved ones come to terms with loss (https://www.poetryfoundation.org/poems/45000/remember-56d224509b7ae).

The role of an ILD specialist is not only to prolong life but also to facilitate a good death. However, as Koyauchi et al (page 248) show in this issue of Thorax, patients with ILD had a lower quality of dying and death with poorer access to palliative care and decision-making than patients with lung cancer.

Given the predictability of both the human condition and the disease trajectory of Interstitial Lung Disease, especially pulmonary fibrosis, it is saddening to read these results. Maybe the ILD community should heed Dylan Thomas’ advice and should ‘not go gentle into that good night, Old age should burn and rave at close of day; Rage, rage against the dying of the light. It’s time to take a stand to ensure our patients get a ‘good’ death.

From Masefield to Shakespeare

Our teaser image is from the case based discussion from McDill and colleagues (page 313) and evokes Shakespeare rather than Masefield “To drain or not to drain?”