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Acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF) and progressive pulmonary fibrosis (AE-PPF) continue to present complex challenges in intensive care settings, say researchers of a new paper, underscoring the high mortality associated with these events and the importance of early decision-making conversations and intervention strategies.¹

Recent estimates suggest that AEs occur in up to 10% of patients with IPF and PPF annually. These episodes, marked by sudden respiratory decompensation and radiologic evidence of new ground-glass opacities on a background of usual interstitial pneumonia (UIP), are associated with dismal outcomes: in-hospital mortality reaches 50%, and rates approach 90% among those requiring mechanical ventilation.

IPF and PPF both face poor survival rates, and the pair share similar clinical features, leading to similar treatment approaches, which are limited and nuanced.

“Treatment options remain limited, often necessitating consideration for lung transplantation. Careful deliberation is required when deciding on intubation, and early integration of palliative care is strongly recommended from diagnosis through the disease course,” explained the researchers in Critical Care Nursing Clinics of North America.

Corticosteroids remain a cornerstone of AE-IPF and AE-PPF treatment following diagnosis, though their use is increasingly scrutinized based on questions surrounding their effectiveness and potential harm. While high-dose steroids may show some benefit in PPF flares, studies have failed to demonstrate survival benefit in AE-IPF, and concerns remain over immunosuppression risks. Retrospective studies comparing pulse-dose regimens (>250 mg/day of methylprednisolone) versus non-pulse doses (1 mg/kg/day) showed no significant outcome differences, also raising questions about optimal dosing strategies.

When patients get admitted to the intensive care unit for flares, delivery of oxygen varies based on specific needs of the patient. Options include high-flow nasal cannula (HFNC), preferred for its ability to provide enough oxygen and flow to help the patient breathe, as well as intubation and mechanical ventilation (MV).

Due to its association with high (90%) mortality, clinical guidelines hint at a recommendation against intubating patients, highlighting the need for early discussions with patients and their families to make decisions ahead of AEs. Researchers of the paper highlight that in cases of intubation, a careful approach should be taken to minimize any additional stress, such as ventilator-induced lung injury or ventilator-associated pneumonia, to the already compromised lungs.

Protective ventilation strategies include limiting plateau pressures (<30 cm H₂O), low tidal volumes (4-6 mL/kg), and optimizing positive end-expiratory pressure to reduce driving pressure.

Extracorporeal membrane oxygenation (ECMO), especially veno-venous ECMO (VV-ECMO), is emerging as a preferred alternative to MV in transplant-eligible patients, considered an evolved form of life support by acting as an artificial lung. ECMO supports gas exchange while minimizing lung injury and allowing physical therapy. Studies show favorable outcomes when ECMO is used as a bridge to transplant, with survival to discharge in more than half of patients. However, ECMO without a transplant plan confers limited benefit, with mortality as high as 75%.

Given the unpredictability and high mortality of acute exacerbations, early palliative care integration is essential in the disease states, explained the researchers. In a separate pilot study, researchers found that implementing an early palliative care program for patients with IPF significantly reduced the rate of deaths in a hospital setting based on patient preference. Notably, the study showed favorable survival among those who received early palliative care, though the study was limited to a single center and included a small group of 46 patients.²

While data have suggested benefits of integrating palliative care early on, referrals remain underutilized. One study showed that 92% of ICU survivors died within 2 months of discharge, emphasizing the need for clear goals-of-care discussions well before patients reach critical deterioration.

References

1. Ali A, Glassberg MK. Managing acute exacerbations in idiopathic pulmonary fibrosis and progressive pulmonary fibrosis in the intensive care unit setting. Crit Care Nurs Clin North Am. Published online July 4, 2025. doi:10.1016/j.cnc.2025.05.002

2. Bassi I, Pastorello S, Guerrieri A, et al. Early palliative care program in idiopathic pulmonary fibrosis patients favors at-home and hospice deaths, reduces unplanned medical visits, and prolongs survival: a pilot study. Eur J Intern Med. Published online May 23, 2024. doi:10.1016/j.ejim.2024.05.024

Trolleys piled high with decapitated silicon monster heads, tattooed dealers lurking in alleyways, bin bags of contraband hidden behind shop counters: welcome to the world of Lafufus.

Fake Labubus, also known as Lafufus, are flooding the hidden market. As demand for the collectable furry keyrings soars, entrepreneurs in the southern trading hub of Shenzhen are wasting no time sourcing imitation versions to sell to eager Labubu hunters. But the Chinese authorities, keen to protect a rare soft-power success story, are cracking down on the counterfeits.

“Labubus have become very sensitive,” says one unofficial vendor, in her small, unmarked, fake designer goods shop hidden on the 17th floor of a bland office building in Shenzhen’s Huaqiangbei district, an area known for cheap electronics. “We don’t dare to talk about it,” her colleague adds.

Labubus, a furry bunny-eared elf sold by Chinese toy company Pop Mart, have gone viral this year. Touted by celebrities from Rihanna to Blackpink’s Lisa, the “ugly-cute” dolls have been so in demand that in the UK Pop Mart pulled the grinning monsters from all stores because of the risk of fights breaking out between customers. In the UK they retail for £17.50, while official versions in China sell for between 99 and 399 yuan (£10.30 – £41.40), with resale prices soaring much higher.

The hype has been embraced by the Chinese authorities, who have hailed Pop Mart as the latest Chinese brand to gain popularity overseas, following the likes of the viral video game Black Myth: Wukong and AI company DeepSeek.

In June, People’s Daily, the Chinese Communist party’s official mouthpiece, praised Labubus as representing the shift from “Made in China” to “Created in China”. “Labubu’s rise fuses China’s strong manufacturing base with creative innovation, tapping into the emotional needs of global consumers,” the article said.

Pop Mart’s elevation to the status of national hero also appears to have motivated the authorities, in a country trying to shed its reputation for being a land of knock-offs, to aggressively crack down on fakes. In April, customs authorities in the eastern city of Ningbo intercepted a batch of 200,000 goods suspected of infringing Labubu’s intellectual property, according to state media, with another sting last month catching over 2,000 fake goods.

About 40km (25 miles) across town from the Huaqiangbei store, 59-year-old Li Yang* has never heard of a “Labubu”. But she spends hours each day sitting on a low plastic stool in her high-rise apartment building slicing apart hundreds of moulded silicon monster heads that will later become Lafufus.

Surrounded by piles of flesh-coloured components, Li and her neighbour, Wang Bi*, another stay-at-home grandmother engaged in the painstaking work, spilled out into the hallway of their apartments. “Since we’re staying at home, taking care of the kids, doing housework, we wanted to find some gig work,” Li says.

Li didn’t know where the monster heads came from or were sent back to. The boss of a nearby factory reported by Chinese media to be producing Lafufus flatly denied any involvement, despite the presence of a pile of suspiciously Labubu-like heads piled high in the hallway.

From business interest to national interest

“China has never been so determined to fix IP [intellectual property] thefts, thanks to Labubu’s contribution not just as a global bestselling toy but as a soft power tool,” said Yaling Jiang, a Chinese consumer trends analyst. “Defending Labubu’s IP is no longer just about business interest, but [about] national interest.”

So the Lafufu market is going underground. Authorities in Shenzhen’s Huaqiangbei recently said they would be inspecting vendors for “counterfeit and shoddy” Labubus. But it doesn’t take long to find a dealer.

After a quick phone call made by one of the street-side vendors hawking fake designer bags and watches, a slight, tattooed man, his canvas tote bag dripping in cutesy furry keyrings, appeared out of nowhere.

He led the Guardian into a busy shopping mall and over to a counter selling hairdryers and sunglasses. With a few furtive glances, the smartly dressed shop assistant whipped out a black plastic bag from behind the counter, full of Lafufus, for sale for 168 yuan (£17.40) each.

Fakes likely come from a range of sources. But Li’s business model works like this: every few days, a courier wheels over a trolley piled with bags stuffed with hundreds of moulded monster heads to Li’s apartment building. The heads are moulded by a machine, but the act of splitting them into two, so that they can be stuffed and reassembled into a finished toy, is fiddly. It requires cutting along the curved edge of the toy’s head by hand, using a sharp knife.

So Li and her neighbours, all elderly women, are enlisted to slice the heads by hand, with the mystery factory paying them 0.04 yuan a piece. Every time the courier arrives, Li hauls down several large bags of split-open heads, and collects a new batch of elfin models, ready for dissection. One woman estimated she can cut through 800-1,000 heads a day, earning up to 40 yuan.

None of the workers interviewed by the Guardian had any idea what a Labubu was. Wang was shocked to hear that the finished products, fake or otherwise, sold for several hundred yuan. But one person in the home factory knew exactly what the toys were. As Li’s young granddaughter wandered into the hallway to find her grandmother inspecting a finished toy, she screamed: “Labubu!”.

*Name has been changed

Additional research by Lillian Yang