Urgent call for comprehensive reform of rare disease care in Hong Kong

Hong Kong Med J 2025;31:Epub 18 Feb 2025
© Hong Kong Academy of Medicine. CC BY-NC-ND 4.0
 
LETTER TO THE EDITOR
Urgent call for comprehensive reform of rare disease care in Hong Kong
Richard SK Chang, FRCP1; Desmond YH Yap, MD, PhD2; KY Chan, MD3; CY Wong, FHKCP3; ML Chan, FHKCPsy4
1 Department of Neuroscience, Central Clinical School, Monash University, Melbourne, Australia
2 Division of Nephrology, Department of Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong SAR, China
3 Palliative Medical Unit, Grantham Hospital, Hong Kong SAR, China
4 Department of Psychiatry, The Chinese University of Hong Kong, Hong Kong SAR, China
 
Corresponding author: Dr Richard SK Chang (richard.chang@alfred.org.au)
 
 Full paper in PDF
 
 
To the Editor—We write in response to an article that highlighted the experience of two Cantopop artists whose son was diagnosed with a rare disease.1 Because of its complexity, immediate discussion is warranted of the critical aspects of managing rare diseases in Hong Kong.
 
Rare disease, which impacts about 300 million individuals globally, encompasses a number of medical conditions across different specialties.2 Neurological disorders and metabolic causes account for 40% and 10% of rare diseases, respectively, with tuberous sclerosis and spinocerebellar ataxia being examples in Hong Kong.3 In general, there is a lack of awareness about rare diseases among healthcare professionals and the public, with consequent delayed diagnosis and treatment.3 Families who cope with diseases often encounter emotional and psychological problems that are compounded by a lack of specialised psychosocial support and palliative care access.4 Moreover, the financial strain of managing diseases is substantial; drug costs for rare diseases are reportedly up to 13.8 times higher than those of more common ailments.3 It is crucial to provide comprehensive care for rare disease patients and their families.
 
The healthcare system for rare diseases in Hong Kong is not as advanced or well equipped as comparable centres in the US2 and Mainland China.5 It faces challenges at different levels. Key issues include insufficient patient support, absence of a specific registry, limited availability of genetic testing, and a high financial burden for patients. To bridge these gaps, Hong Kong could learn from the well-established networks and care models of the US,2 as well as the central registry in Mainland China,5 and adopt supportive policies and financial assistance programmes. As a starting point, the Hong Kong Genome Institute (https://hkgp.org/en/) provides a strong platform from which to promote public awareness of rare diseases in Hong Kong. In addition, with the newly established Genetics and Genomics (Medicine) Fellowship of the Hong Kong Academy of Medicine,6 genetic testing and counselling that target rare disease could be streamlined.
 
Author contributions
All authors contributed to the letter and critical revision of the letter for important intellectual content. All authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.
 
Conflicts of interest
The authors have disclosed no conflicts of interest.
 
Funding/support
This letter received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
 
References
1. Cheng L. Hong Kong star couple Stephanie Ho, Fred Cheng share story of son’s Angelman syndrome diagnosis to raise public awareness. South China Morning Post. 2024 Jun 2: Health & Environment. Available from: https://www.scmp.com/news/hong-kong/health-environment/article/3265045/hong-kong-star-couple-stephanie-ho-fred-cheng-share-story-sons-angelman-syndrome-diagnosis-raise. Accessed 17 Feb 2025.
2. Baynam G, Hartman AL, Letinturier MC, et al. Global health for rare diseases through primary care. Lancet Glob Health 2024;12:e1192-9. Crossref
3. Chung CC, Ng NY, Ng YN, et al. Socio-economic costs of rare diseases and the risk of financial hardship: a cross-sectional study. Lancet Reg Health West Pac 2023;34:100711. Crossref
4. Chan KY, Yap DY, Singh Harry Gill H. Rethinking palliative care in psychiatry. JAMA Psychiatry 2023;80:1089-90. Crossref
5. Guo J, Liu P, Chen L, et al. National Rare Diseases Registry System (NRDRS): China’s first nation-wide rare diseases demographic analyses. Orphanet J Rare Dis 2021;16:515. Crossref
6. Hong Kong Academy of Medicine. Specialty descriptions. 2024 Feb 6. Available from: https://www.hkam.org.hk/sites/default/files/PDFs/2024/SPECRE25%20(Specialty%20descriptions%20-20240206).pdf?v=1736316996530. Accessed 6 Feb 2025.

1,4-Butanediol: legal date rape drug on the loose

Hong Kong Med J 2025;31:Epub 11 Feb 2025
© Hong Kong Academy of Medicine. CC BY-NC-ND 4.0
 
LETTER TO THE EDITOR
1,4-Butanediol: legal date rape drug on the loose
CW Yeung, MB, BS; TM Han, MB, ChB; Kelvin YC Yu, MB, BS; ML Chen, MSc; YK Chong, FHKCPath, FHKAM (Pathology)
Hospital Authority Toxicology Reference Laboratory, Hong Kong SAR, China
 
Corresponding author: Dr YK Chong (cyk280a@ha.org.hk)
 
 Full paper in PDF
 
 
To the Editor—Gamma-hydroxybutyrate (GHB) is a central nervous system depressant with short-term hypnotic and euphoric effects. It is a notorious date rape drug that is extensively abused to enhance sexual activity and pleasure (colloquially known as ‘chemfun’) among men who have sex with men. Gamma-hydroxybutyrate overdose can cause drowsiness, respiratory depression, or coma.1 Gamma-hydroxybutyrate and its pro-drug gamma-butyrolactone are dangerous drugs within the meaning of the Dangerous Drugs Ordinance.2
 
1,4-Butanediol, an industrial solvent, is converted to GHB by hepatic alcohol dehydrogenase and aldehyde dehydrogenase.3 Generally, its onset of action is 5 to 20 minutes after ingestion with effects lasting for 2 to 3 hours.4 Nonetheless there is inter-individual variability in its metabolism to GHB, mainly due to differences in alcohol dehydrogenase activity.3 Importantly, concurrent ethanol intake inhibits conversion of 1,4-butanediol to GHB.5 With a delayed onset of GHB-related desirable effects, inadvertent overdose may occur in those whose intention was to ingest GHB rather than 1,4-butanediol.3 6
 
At the time or writing, 1,4-butanediol is not listed as a dangerous drug. Our laboratory confirmed two cases of 1,4-butanediol misuse and poisoning related to ‘chemfun’ or suspected sexual assault. One of the cases had concurrent ethanol consumption. When encountering cases of suspected GHB poisoning, clinicians should be aware of the possibility of 1,4-butanediol ingestion and educate patients in high-risk groups about the dangers of misusing 1,4-butanediol. To prevent GHBrelated crime, poisoning, or death,1 the government should consider classifying 1,4-butanediol as a dangerous drug, given its accessibility and potential for misuse.
 
Author contributions
Concept or design: All authors.
Acquisition of data: All authors.
Analysis or interpretation of data: All authors.
Drafting of the manuscript: CW Yeung, YK Chong.
Critical revision of the manuscript for important intellectual content: All authors.
 
All authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.
 
Conflicts of interest
All authors have disclosed no conflicts of interest.
 
Funding/support
This letter received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
 
References
1. Dufayet L, Bargel S, Bonnet A, et al. Gamma-hydroxybutyrate (GHB), 1,4-butanediol (1,4BD), and gamma-butyrolactone (GBL) intoxication: a state-of-the-art review. Regul Toxicol Pharmacol 2023;142:105435. Crossref
2. Hong Kong e-Legislation, Hong Kong SAR Government. Cap 134 Dangerous Drugs Ordinance. Available from: https://www.elegislation.gov.hk/hk/cap134!en-zh-Hant-HK?INDEX_CS=N. Accessed 5 Feb 2025.
3. Thai D, Dyer JE, Jacob P, Haller CA. Clinical pharmacology of 1,4-butanediol and gamma-hydroxybutyrate after oral 1,4-butanediol administration to healthy volunteers. Clin Pharmacol Ther 2007;81:178-84. Crossref
4. Drug & Chemical Evaluation Section, Diversion Control Division, Drug Enforcement Administration, US Department of Justice. 1,4-Butanediol. 2024. Available from: https://www.deadiversion.usdoj.gov/drug_chem_info/bdo.pdf. Accessed 21 Jun 2024.
5. Poldrugo F, Barker S, Basa M, Mallardi F, Snead OC. Ethanol potentiates the toxic effects of 1,4-butanediol. Alcohol Clin Exp Res 1985;9:493-7. Crossref
6. Stefani M, Roberts DM. 1,4-Butanediol overdose mimicking toxic alcohol exposure. Clin Toxicol (Phila) 2020;58:204-7. Crossref

Secondary use of dried blood spots from newborn screening

Hong Kong Med J 2024 Aug;30(4):338 | Epub 16 Jul 2024
© Hong Kong Academy of Medicine. CC BY-NC-ND 4.0
 
LETTER TO THE EDITOR
Secondary use of dried blood spots from newborn screening
Christy WM Leung, MB, ChB1; NS Cheng, DNurs2; TF Leung, MD, FRCPCH2,3
1 Faculty of Medicine, The Chinese University of Hong Kong, Hong Kong SAR, China
2 Department of Paediatrics, The Chinese University of Hong Kong, Prince of Wales Hospital, Hong Kong SAR, China
3 Hong Kong Hub of Paediatric Excellence, The Chinese University of Hong Kong, Hong Kong SAR, China
 
Corresponding author: Prof TF Leung (tfleung@cuhk.edu.hk)
 
 Full paper in PDF
 
 
To the Editor—The opt-in, territory-wide Newborn Screening Programme for Inborn Errors of Metabolism relies on collection of a newborn’s dried blood spot (DBS) at birth. The residual DBS (rDBS) samples contain genetic material and may be stored for secondary research purposes. Nonetheless according to the findings by Ngan et al,1 not all healthcare professionals are confident in explaining to hesitant parents the secondary benefits of the Programme. Below is an example of how rDBS may be utilised for translational research through analysis of genes in samples.
 
Acute lymphoblastic leukaemia is a common childhood malignancy of multifactorial pathogenesis and may present before 12 months of age2; such an early onset has inspired research into any prenatal gene abnormalities in diagnosed individuals. Related chromosomal aberrations such as BCR-ABL1 translocation and high hyperdiploidy have been detected in rDBS samples of individuals subsequently diagnosed with acute lymphoblastic leukaemia,3 suggesting in-utero involvement. Closer monitoring of genetically high-risk children may facilitate timely detection of any cancer, although such cost-effectiveness is yet to be assessed.
 
Clinical application of rDBS research is still in its infancy but has potential in population-wide disease tracking and epidemiological studies. Equipping healthcare professionals with updates on relevant studies may facilitate communication with prospective parents. It is hoped that more parents will opt in and contribute precious rDBS samples to the scientific community.
 
Author contributions
Concept or design: CWM Leung.
Acquisition of data: CWM Leung.
Analysis or interpretation of data: CWM Leung.
Drafting of the manuscript: CWM Leung, NS Cheng.
Critical revision of the manuscript for important intellectual content: NS Cheng, TF Leung.
 
All authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.
 
Conflicts of interest
All authors have disclosed no conflicts of interest.
 
Acknowledgement
The authors thank Mr Samson Mak for providing medical editing support.
 
Funding/support
This letter received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
 
References
1. Ngan OM, Tam CJ, Li CK. Exploration of clinical and ethical issues in an expanded newborn metabolic screening programme: a qualitative interview study of healthcare professionals in Hong Kong. Hong Kong Med J 2024;30:120-9. Crossref
2. Cheng FW, Lam GK, Cheuk DK, et al. Overview of treatment of childhood acute lymphoblastic leukaemia in Hong Kong. Hong Kong J Paediatr (new series) 2019;24:184-91.
3. Rüchel N, Jepsen VH, Hein D, Fischer U, Borkhardt A, Gössling KL. In utero development and immunosurveillance of B cell acute lymphoblastic leukemia. Curr Treat Options Oncol 2022;23:543-61. Crossref

Another ketamine analogue on the horizon

Hong Kong Med J 2024 Aug;30(4):337 | Epub 22 Jul 2024
© Hong Kong Academy of Medicine. CC BY-NC-ND 4.0
 
LETTER TO THE EDITOR
Another ketamine analogue on the horizon
TM Han, MB, ChB1,2; Magdalene HY Tang, PhD1,2; HF Tong, FHKCPath, FHKAM (Pathology)1,2; YT Cheung, MB, ChB1,2; Jeremiah SB Tseung, MB, ChB1,2; MK Yip, MB, BS1,2; CK Ching, FRCPA, FHKAM (Pathology)1,2; YK Chong, FHKCPath, FHKAM (Pathology)1,2
1 Hospital Authority Toxicology Reference Laboratory, Hong Kong SAR, China
2 Chemical Pathology Laboratory, Department of Pathology, Princess Margaret Hospital, Hong Kong SAR, China
 
Corresponding author: Dr YK Chong (cyk280a@ha.org.hk)
 
 Full paper in PDF
 
 
To the Editor—Ketamine analogues are new psychoactive substances that share the arylcyclohexylamine backbone of ketamine and produce dissociative effects through antagonistic activity at the N-methyl-D-aspartate receptor.1 Ketamine and its analogues have plagued Hong Kong over the last two decades. Our laboratory has identified outbreaks of multiple ketamine analogues in Hong Kong, including 2-oxo-phenylcyclohexylethylamine in 2017,2 2-fluorodeschloroketamine (2F-DCK) and deschloroketamine in 2019,3 and tiletamine in 2019 to 2022 (according to data on file in the Hospital Authority Toxicology Reference Laboratory).
 
We report identification of a new ketamine analogue, fluoro-2-oxo-phenylcyclohexylethylamine, also known as fluorexetamine (FXE). Recreational use of FXE was first reported in 2018.4 Our laboratory has detected increasing use of FXE in Hong Kong since mid-2023, with FXE now identified in urine samples of 14 patients. Detection of FXE can be difficult since it does not cross-react with bedside ketamine immunoassay and shares common metabolites with 2F-DCK. This may lead to misidentification of FXE metabolites as 2F-DCK metabolites on routine toxicology testing. Clinically, FXE appears to possess similar toxicity to ketamine and 2F-DCK and co-ingestion with other recreational drugs is common, often complicating the clinical presentation.
 
Effective prevention of the emergence of new psychoactive substances can be achieved through prompt communication and accurate toxicology testing. This approach has been successful in halting the upward trajectory of various ketamine analogues. When encountering patients with clinical features of ketamine abuse but negative immunoassay or urine toxicology results, clinicians are encouraged to submit urine specimens to our laboratory for further testing.
 
Author contributions
Concept or design: All authors.
Acquisition of data: All authors.
Analysis or interpretation of data: All authors.
Drafting of the manuscript: TM Han, YK Chong.
Critical revision of the manuscript for important intellectual content: All authors.
 
All authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.
 
Conflicts of interest
All authors have disclosed no conflicts of interest.
 
Funding/support
This letter received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
 
References
1. Morris H, Wallach J. From PCP to MXE: a comprehensive review of the non-medical use of dissociative drugs. Drug Test Anal 2014;6:614-32. Crossref
2. Chong YK, Tang MH, Chan CL, Li YK, Ching CK, Mak TW. 2-oxo-PCE: ketamine analogue on the streets. Hong Kong Med J 2017;23:665-6. Crossref
3. Li C, Lai CK, Tang MH, Chan CC, Chong YK, Mak TW. Ketamine analogues multiplying in Hong Kong. Hong Kong Med J 2019;25:169. Crossref
4. National Drug Early Warning System. Alert from the NDEWS Web Monitoring Team: online mentions of fluorexetamine. 2022. Available from: https://ndews.org/wordpress/files/2023/04/8.12.22.pdf. Accessed 12 Jul 2024.

Cross-specialty point-of-care ultrasound education in The University of Hong Kong

Hong Kong Med J 2024 Jun;30(3):255 | Epub 4 Jun 2024
© Hong Kong Academy of Medicine. CC BY-NC-ND 4.0
 
LETTER TO THE EDITOR
Cross-specialty point-of-care ultrasound education in The University of Hong Kong
Arthur CK Cheung, MB, ChB, FHKAM (Emergency Medicine)1; Pauline Y Ng, MB, BS, FHKAM (Medicine)2; Rex PK Lam, MPH, FHKAM (Emergency Medicine)1; Gordon TC Wong, MD, FHKAM (Anaesthesiology)3
1 Department of Emergency Medicine, School of Clinical Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong SAR, China
2 Critical Care Medicine Unit, School of Clinical Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong SAR, China
3 Department of Anaesthesiology, School of Clinical Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong SAR, China
 
Corresponding author: Dr Arthur CK Cheung (arthurck@hku.hk)
 
 Full paper in PDF
 
 
To the Editor—We read with interest the article by Leung et al1 that offers a glimpse of undergraduate point-of-care ultrasound (POCUS) education in Asia. In The University of Hong Kong, our POCUS curriculum has extended beyond basic theory and e-learning.2
 
Thanks to a generous donation, a pocket-sized POCUS device is now on loan solely to year 5 and 6 medical students during their specialty clerkship. The POCUS device can be easily linked to a smartphone or tablet, empowering students to practise their bedside scanning skills anytime and anywhere.
 
Teachers from different specialties synergise teaching efforts by focusing on relevant organ systems during respective rotations. For instance, the Department of Medicine and the Critical Care Medicine Unit jointly organise the POCUS Boot Camp that offers an intensive hands-on learning experience on basic echocardiography and lung ultrasound. The Department of Emergency Medicine covers the Extended Focused Assessment with Sonography in Trauma and abdominal aorta scan in small-group training, and the Department of Surgery introduces kidney, hepatobiliary and thyroid ultrasound.
 
Ultrasound is not only an essential skill future doctors can use to make better clinical decisions at the point of care, but can also help students visualise clinical signs, such as cardiac murmurs and pleural effusions, detected during physical examination.3 Given the inherited limitations of POCUS and limited practice experience, students are not expected to diagnose disease independently using POCUS and their scan findings need to be verified by qualified practitioners. However, we believe early ultrasound exposure lays a solid foundation for postgraduate training.
 
Author contributions
All authors contributed to the concept of the study, acquisition of data, analysis or interpretation of data, drafting of the letter, and critical revision of the letter for important intellectual content. All authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.
 
Conflicts of interest
All authors have disclosed no conflicts of interest.
 
Funding/support
This letter received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
 
References
1. Leung KY, Bala K, Cho JY, et al. Utility and challenges of ultrasound education for medical and allied health students in Asia. Hong Kong Med J 2024;30:75-9. Crossref
2. Coiffier B, Shen PC, Lee EY, et al. Introducing point-of-care ultrasound through structured multifaceted ultrasound module in the undergraduate medical curriculum at The University of Hong Kong. Ultrasound 2020;28:38-46. Crossref
3. Wong CK, Hai J, Chan KY, et al. Point-of-care ultrasound augments physical examination learning by undergraduate medical students. Postgrad Med J 2021;97:10-5. Crossref

Many systemic diseases may mimic a primary knee disorder

© Hong Kong Academy of Medicine. CC BY-NC-ND 4.0
 
LETTER TO THE EDITOR
Many systemic diseases may mimic a primary knee disorder
John SM Leung, FRCSEd, FHKAM (Surgery)
Department of Cardiothoracic Surgery, St Paul’s Hospital, Hong Kong SAR, China
 
Corresponding author: Dr John SM Leung (leungjohnsiuman@gmail.com)
 
 Full paper in PDF
 
To the Editor—Chan et al1 drew our attention to the fact that tuberculosis can be a great mimicker of other conditions when it affects the knee. Conversely the knee may be a site where problems may mimic other disorders. Knee injuries are among the most common disabling conditions that arise from sporting and other accidents or falls. The presence of septic arthritis may overlap or complicate a traumatic knee condition. Septic arthritis by itself may affect the knee, as well as autoimmune-related arthritis. Yet uncommonly, gout and other crystal arthritis may have a similar clinical and radiological presentation.2 Only when urate or calcium pyrophosphate are identified can the diagnosis be confirmed. The title ‘great mimicker’ was originally applied to syphilis, a disease that declined considerably in the last century but that is recently exhibiting a resurgence due to uncontrolled sexually transmitted diseases.3 Syphilis targets virtually every organ and the knee is no exception. In North America, another spirochete infection, Lyme disease, is known to infect people bitten by ticks or in contact with wild animals, and prominent among its symptoms is arthritis, including that of the knee.4 In Hong Kong, we do not have Lyme disease but we should maintain a high index of suspicion in individuals who have visited North America and who present with fever, fatigue and joint pain. The risk is not confined to recent exposures since the disease may be quiescent for months or even years before a flare-up.
 
Author contributions
The author solely contributed to the concept or design, acquisition of data, analysis or interpretation of data, drafting of the letter, and critical revision of the letter for important intellectual content. The author had full access to the data, contributed to the study, approved the final version for publication, and takes responsibility for its accuracy and integrity.
 
Conflicts of interest
The author has disclosed no conflict of interest.
 
Funding/support
This letter received no specific grant from any funding agency in the public, commercial, or not-for profit sectors.
 
References
1. Chan HM, Fu H, Chiu KY. Tuberculosis of the knee as a great mimicker of inflammatory arthritis: a case report. Hong Kong Med J 2023;29:548-50. Crossref
2. Yun SY, Choo HJ, Jeong HW, Lee SJ. Comparison of MR findings between patients with septic arthritis and acute gouty arthritis of the knee. J Korean Soc Radiol 2022;83:1071-80. Crossref
3. Peeling RW, Hook EW 3rd. The pathogenesis of syphilis: the great mimicker, revisited. J Pathol 2006;208:224-32. Crossref
4. Arvikar SL, Steere AC. Diagnosis and treatment of Lyme arthritis. Infect Dis Clin North Am 2015;29:269-80. Crossref

The maximum dose of atorvastatin and simvastatin as well as rosuvastatin should be restricted in East Asians

Hong Kong Med J 2024 Apr;30(2):184–5 | Epub 12 Apr 2024
© Hong Kong Academy of Medicine. CC BY-NC-ND 4.0
 
LETTER TO THE EDITOR
The maximum dose of atorvastatin and simvastatin as well as rosuvastatin should be restricted in East Asians
Brian Tomlinson, MD, FHKAM (Medicine)1; Elaine Chow, MB, ChB, FHKAM (Medicine)2
1 Faculty of Medicine, Macau University of Science and Technology, Macau SAR, China
2 Department of Medicine and Therapeutics, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong SAR, China
 
Corresponding author: Prof Brian Tomlinson (btomlinson@must.edu.mo)
 
 Full paper in PDF
 
 
To the Editor—Statins are very safe medications when used in doses appropriate for the individual, but the letter from ML Tse highlights the risk of rhabdomyolysis with rosuvastatin 40 mg in Chinese patients.1 It was known at the time of first registration of rosuvastatin in 2003 that plasma levels were twice as high in East Asians (Chinese and Japanese) compared with Caucasians. As 40 mg is the maximum dose of rosuvastatin approved in Western countries, it would seem appropriate to restrict the maximum dose to 20 mg in East Asians. This has been adopted in China, Korea, and Japan.
 
Plasma levels of atorvastatin and simvastatin acid, the active form of simvastatin, are also higher in Chinese and Japanese subjects compared with Caucasians.2 The maximum dose of atorvastatin approved in Japan is 40 mg,3 and the 2023 Chinese guideline for lipid management contains the comment ‘Atorvastatin 80 mg is inexperienced in China, please use with caution’.4
 
The maximum approved or recommended daily dose of simvastatin is 20 mg in Japan and 40 mg in Korea and China. The Clinical Pharmacogenetics Implementation Consortium provides a guideline for genetic testing related to statin myopathy,5 and since 2012 they have recommended that the dose of simvastatin be restricted to 20 mg in individuals with the common c.521T>C variant (rs4149056) in the SLCO1B1 gene that encodes the OATP1B1 transporter. Considering this variant occurs in 11% to 16% of East Asians, it would seem wise to restrict the dose of simvastatin to 20 mg in the absence of genetic testing.
 
The Clinical Pharmacogenetics Implementation Consortium guideline applies to all ethnic groups. The relative risk of myopathy was 2.6 per copy of the SLCO1B1 521C variant in the Heart Protection Study with simvastatin 40 mg.6 The increased risk of myopathy in Chinese patients was seen in the HPS2-THRIVE trial (Heart Protection Study 2: Treatment of HDL to Reduce the Incidence of Vascular Events) where the combination of definite myopathy and incipient myopathy was about 10 times higher in China than in Europe (0.66% per year vs 0.07% per year; P<0.001) in participants taking simvastatin 40 mg in combination with extended-release niacin 2 g plus laropiprant 40 mg daily.7 This was probably due to an unexpected pharmacokinetic interaction between simvastatin and niacin.
 
In 2019, we reported the case of a 69-year-old Chinese male diabetic who had taken simvastatin 40 mg for 10 years and developed rhabdomyolysis, possibly related to unexpected drug interactions with Stevia rebaudiana and/or linagliptin.8 He was a carrier of one copy of SLCO1B1 521C and two copies of the C421>A variant of the adenosine triphosphate–binding cassette transporter G2 gene. That variant is more frequent in Chinese subjects. This illustrates that despite apparent long-term safe administration of simvastatin, it needs only an unpredicted drug-drug or herb-drug interaction or the gradual deterioration in renal function with age, which is more rapid in diabetics, to tip the balance and result in life-threatening toxicity in susceptible patients.
 
Author contributions
Both authors contributed to the concept or design, acquisition of data, analysis or interpretation of data, drafting of the letter, and critical revision of the letter for important intellectual content. Both authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.
 
Conflicts of interest
Both authors have disclosed no conflicts of interest.
 
Funding/support
This letter received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
 
References
1. Tse ML. Cluster of cases of high-dose rosuvastatin-associated rhabdomyolysis and recent reduction of rosuvastatin dose for Asians in other countries. Hong Kong Med J 2023;29:474. Crossref
2. Birmingham BK, Bujac SR, Elsby R, et al. Impact of ABCG2 and SLCO1B1 polymorphisms on pharmacokinetics of rosuvastatin, atorvastatin and simvastatin acid in Caucasian and Asian subjects: a class effect? Eur J Clin Pharmacol 2015;71:341-55. Crossref
3. Naito R, Miyauchi K, Daida H. Racial differences in the cholesterol-lowering effect of statin. J Atheroscler Thromb 2017;24:19-25. Crossref
4. Li JJ, Zhao SP, Zhao D, et al. 2023 Chinese guideline for lipid management. Front Pharmacol 2023;14:1190934. Crossref
5. Cooper-DeHoff RM, Niemi M, Ramsey LB, et al. The Clinical Pharmacogenetics Implementation Consortium guideline for SLCO1B1, ABCG2, and CYP2C9 genotypes and statin-associated musculoskeletal symptoms. Clin Pharmacol Ther 2022;111:1007-21. Crossref
6. SEARCH Collaborative Group; Link E, Parish S, et al. SLCO1B1 variants and statin-induced myopathy—a genomewide study. N Engl J Med 2008;359:789-99. Crossref
7. HPS2-THRIVE Collaborative Group. HPS2-THRIVE randomized placebo-controlled trial in 25 673 high-risk patients of ER niacin/laropiprant: trial design, pre-specified muscle and liver outcomes, and reasons for stopping study treatment. Eur Heart J 2013;34:1279-91. Crossref
8. Chan JC, Ng MH, Wong RS, Tomlinson B. A case of simvastatin-induced myopathy with SLCO1B1 genetic predisposition and co-ingestion of linagliptin and Stevia rebaudiana. J Clin Pharm Ther 2019;44:381-3. Crossref

Prioritising the psychosocial needs of young oncology patients: a call for comprehensive care

Hong Kong Med J 2024 Apr;30(2):186 | Epub 10 Apr 2024
© Hong Kong Academy of Medicine. CC BY-NC-ND 4.0
 
LETTER TO THE EDITOR
Prioritising the psychosocial needs of young oncology patients: a call for comprehensive care
CY Wong, FHKCP1; HY Au, FHKCP1; KY Chan, MD, FHKCP1; Harinder Gill, MD, FRCP2
1 Palliative Medical Unit, Grantham Hospital, Hong Kong SAR, China
2 Department of Medicine, The University of Hong Kong, Hong Kong SAR, China
 
Corresponding author: Dr CY Wong (ashleywcy@gmail.com)
 
 Full paper in PDF
 
 
To the Editor—We write in response to an article published in November 2023 on digital media that discussed the difficulties faced by local young cancer patients who are receiving oncology treatment.1 We would like to highlight the importance of addressing these challenges and propose solutions to help overcome them.
 
In 2021, young oncology patients comprised up to 4.1% of all cancer cases in Hong Kong.2 They experience a range of emotional, social, and financial challenges that greatly impact their overall well-being. Neglecting their needs not only strains their relationship with family, but also hampers their ability to effectively cope with the disease. The financial toxicity of cancer treatment, which includes the expenses, indirect costs and lost income associated with cancer treatment, further exacerbates these challenges.3 4
 
To address these issues effectively it is essential to provide services specifically designed for young cancer patients according to their stage of development. They are at high risk of psychosocial problems and should be prioritised for early integration into palliative care services to improve their quality of life and that of their family.5 Mental health professionals and support groups should be widely available for psychological support. Some medical allowances and social services are provided only for the older adults. Medical-social collaboration and educational resources could improve access by young adults to community support. Additionally, implementing targeted financial assistance programmes and providing employment support will help alleviate the financial burden. It is crucial to recognise and address the obstacles faced by young cancer patients.
 
Author contributions
All authors contributed to the concept or design, acquisition of data, or interpretation of data, drafting of the letter, and critical revision of the letter for important intellectual content. All authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.
 
Conflicts of interest
All authors have disclosed no conflicts of interest.
 
Funding/support
This letter received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
 
References
1. HK01. Hong Kong young cancer patients established a mutual aid platform to help themselves as existing cancer support is mostly targeted at the middle-aged and older adults. [in Chinese]. 2023 November 22. Available from: https://www.hk01.com/article/957641?utm_source=01appshare&utm_medium=referral. Accessed 26 Nov 2023.
2. Hong Kong Cancer Registry, Hospital Authority, Hong Kong SAR Government. Overview of Hong Kong Cancer Statistics of 2021. Available from: https://www3.ha.org.hk/cancereg/default.asp. Accessed 28 Nov 2023.
3. Evan EE, Zeltzer LK. Psychosocial dimensions of cancer in adolescents and young adults. Cancer 2006;107(7 Suppl):1663-71. Crossref
4. Geue K, Götze H, Friedrich M, et al. Perceived social support and associations with health-related quality of life in young versus older adult patients with haematological malignancies. Health Qual Life Outcomes 2019;17:145. Crossref
5. Zimmermann C, Swami N, Krzyzanowska M, et al. Early palliative care for patients with advanced cancer: a cluster-randomised controlled trial. Lancet 2014;383:1721-30. Crossref

Specialised crew resource management programme for non–locally trained healthcare professionals: expediting healthcare cultural adaptation

Hong Kong Med J 2024 Feb;30(1):80–1 | Epub 1 Feb 2024
© Hong Kong Academy of Medicine. CC BY-NC-ND 4.0
 
LETTER TO THE EDITOR
Specialised crew resource management programme for non–locally trained healthcare professionals: expediting healthcare cultural adaptation
Eric HK So, MB, BS, FHKAM (Anaesthesiology),1,2; Victor KL Cheung, MSc (OP), RegPsychol (HKPS)1; Avis SH Leung, MSSc, RN1; SS So, MSc, BBA1; Jeff LK Hung, MSc, BSSc1; Terry ML Yau, MBA, SNM3; NH Chia, MB, BS, FRCSEd (Gen)1,4; George WY Ng, MB, BS, FCICM1,5
1 Multi-Disciplinary Simulation and Skills Centre, Queen Elizabeth Hospital, Hong Kong SAR, China
2 Department of Anaesthesiology and Operating Theatre Services, Queen Elizabeth Hospital, Hong Kong SAR, China
3 Central Nursing Division, Kowloon Central Cluster, Hospital Authority, Hong Kong SAR, China
4 Department of Surgery, Queen Elizabeth Hospital, Hong Kong SAR, China
5 Intensive Care Unit, Queen Elizabeth Hospital, Hong Kong SAR, China
 
Corresponding author: Dr Eric HK So (sohke@ha.org.hk)
 
 Full paper in PDF
 
 
To the Editor—Healthcare delivery is one of the most complex sociotechnical processes with healthcare practitioners working under adverse and stressful conditions despite being adequately trained for medical-technical proficiency. Human factors contribute to 70% to 80% of medical incidents.1 The focus has shifted gradually from individual competence to teamwork as a prerequisite to improve patient safety.1 2
 
Teamwork skills training, crew resource management (CRM), has become an essential and integral part of a corporate-wide teamwork training programme in the Hong Kong Hospital Authority.1 Applying CRM can transform a team of highly specialised experts into an expert team for safe patient care, good working climate and team member satisfaction.1
 
In April 2023, the Hospital Authority launched its first Greater Bay Area (GBA) Healthcare Talents Visiting Programme.1 In September 2023, a Multi-Disciplinary Simulation and Skills Centre at Queen Elizabeth Hospital and the Central Nursing Division co-organised a 4-hour classroom-based interactive group sharing programme for 14 non–locally trained professionals in the Kowloon Central Cluster (Table).3 4 The elements covered in the Cluster’s CRM training include assertiveness, communication, leadership and followership (interpersonal skills), and situational awareness (cognitive skills).
 

Table. Curriculum of crew resource management (CRM) sharing programme for non–locally trained healthcare professionals (4 doctors, 10 nurses) from the Greater Bay Area
 
This pilot programme, titled ‘Sharing Activity for Non–Locally Trained Healthcare Professionals’ (深化醫療團隊協作), aimed to broaden participants’ awareness of the Hospital Authority organisational structure and training centre development and share elements of standard Kowloon Central Cluster CRM training. The objective was improved better clinical teamwork and adaptation among interdisciplinary professionals from diverse training backgrounds.1 5
 
We conducted an evaluation before and after this pilot programme. All items on pre- and post-questionnaires used a 5-point Likert scale (1=Strongly disagree, 2=Disagree, 3=Neutral, 4=Agree, 5=Strongly agree). Our evaluation identified a remarkable increase in understanding of the local healthcare service (score range of the results of items in this category=3.79-4.90; overall increase in knowledge from pre-test to post-test in this category=23%) and elements of CRM (score range of the results of items in this category=4.19-5; overall increase in knowledge from pre-test to post-test in this category=20%). All participants (n=14, 100%) found acquisition of CRM could improve patient safety and 93% (n=13) were confident that they could apply the principles in clinical practice. From the perspective of personal interests and clinical benefits, participants placed a high value on all content but especially ‘Concept of CRM’ (mean ± standard deviation=4.93 ± 0.27) and ‘Simulation technology applied in training and research’ (mean=5). A Self-Evaluated Behaviour Assessment (SEBA-28) addressed the overall impact of the programme on participants’ attitude towards CRM-related behaviours (+10%), in particular ‘Situational awareness’ (+14%).5 When identifying challenges in healthcare cultural adaptation, 93% of participants (n=13) were optimistic that implementing the concept of CRM would mitigate challenges regarding communication, interdisciplinary team cooperation, and cultural diversity.
 
The evaluation demonstrates the potential and value of a CRM programme for non–locally trained healthcare professionals. Various Hospital Authority training centres could play an important role to facilitate integration and interaction of team members from diverse training backgrounds through CRM training. Further study should be planned to fill the knowledge and research gaps and build resilient expert teams in the Hospital Authority.
 
Author contributions
All authors contributed to the concept or design of the letter, acquisition of data, and analysis or interpretation of data. EHK So and VKL Cheung drafted the letter. All authors critically revised the letter for important intellectual content. All authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.
 
Conflicts of interest
All authors have disclosed no conflicts of interest.
 
Acknowledgement
The authors express their gratitude to the Hospital Authority Head Office Medical Grade and the hospital management of the Kowloon Central Cluster for their support and contribution to the Crew Resource Management programme specialised for non–locally trained healthcare professionals from the Greater Bay Area.
 
Funding/support
This letter received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
 
References
1. Chan CK, So EH, Ng GW, Ma TW, Chan KK, Ho LY. Participant evaluation of simulation training using crew resource management in a hospital setting in Hong Kong. Hong Kong Med J 2016;22:131-7. Crossref
2. Hospital Authority. HASLink: strengthen exchange with Greater Bay Area. November 2022. Available from: https://www3.ha.org.hk/ehaslink/issue122/en/feature-1.html. Accessed 5 Sep 2023.
3. So EH, Chia NH, Ng GW, et al. Multidisciplinary simulation training for endotracheal intubation during COVID-19 in one Hong Kong regional hospital: strengthening of existing procedures and preparedness. BMJ Simul Technol Enhanc Learn 2021;7:501-9. Crossref
4. Cheung VK, Chia NH, So SS, Ng GW, So EH. Expanding scope of Kirkpatrick model from training effectiveness review to evidence-informed prioritization management for cricothyroidotomy simulation. Heliyon 2023;9:e18268. Crossref
5. Leung AS, So EH, Chan CN, et al. Embracing human factors in assertiveness, communication, leadership and followership, and situational awareness through O&G specific CRM classroom training. Presented at: KCC Convention; 26 November 2021; Hong Kong SAR, China.

Survival of out-of-hospital cardiac arrest following a return of spontaneous circulation beyond 30 minutes

Hong Kong Med J 2023 Dec;29(6):564–5 | Epub 23 Aug 2023
© Hong Kong Academy of Medicine. CC BY-NC-ND 4.0
 
LETTER TO THE EDITOR
Survival of out-of-hospital cardiac arrest following a return of spontaneous circulation beyond 30 minutes
KL Hon, MB, BS, MD1; Karen KY Leung, MB, BS, MRCPCH1; KL Chan, MB, ChB, MRCPCH1; WF Hui, MB, ChB, MRCPCH1; KT Chau, MB, BS, FRCPCH1; SY Qian, MD2
1 Department of Paediatrics and Adolescent Medicine, Hong Kong Children’s Hospital, Hong Kong SAR, China
2 Pediatric Intensive Care Unit, Beijing Children’s Hospital, Capital Medical University, Beijing, China
 
Corresponding author: Dr KL Hon (ehon@hotmail.com)
 
 Full paper in PDF
 
 
To the Editor—There was a local blog report in Hong Kong of a 5-year-old girl who experienced out-of-hospital cardiac arrest (OHCA) with return of spontaneous circulation (ROSC) after 31 minutes and was discharged with an implantable cardioverter defibrillator.1 However, ROSC within 30 minutes is usually required for a favourable outcome.2 3
 
We performed a literature search to determine the longest time to ROSC and survival rates of OHCA in children (Table). Out-of-hospital cardiac arrest in children has a poor prognosis and prolonged in-hospital resuscitation beyond 30 minutes does not improve survival.3 Predictors of survival to discharge include witnessed arrest (P=0.012), delivery of bystander cardiopulmonary resuscitation (P=0.003), and duration of resuscitation (P=0.028). However, none who received more than 30 minutes of in-hospital resuscitation survived.4 A prospective study found that no patients who required >2 doses of adrenaline or in-hospital resuscitation for longer than 20 minutes survived to discharge.5 However, it is possible that ROSC beyond 30 minutes has not been reported, or that this case is an exception.
 

Table. Selective paediatric references on out-of-hospital cardiac arrest following return of spontaneous circulation
 
Evidence suggests that either death or a poor outcome is inevitable if OHCA occurs more than 30 minutes from the nearest healthcare facility or the resuscitation exceeds 30 minutes.6 7 A 2017 study reported that the survival rate to discharge in Hong Kong was only 2.3%, which was considerably lower than the global survival rate in adults (8.8%).8 9 As OHCA in children has not been evaluated in Hong Kong until 2018,3 and prospective evaluation of OHCA in children has not yet been conducted, we concur with Wu10 who suggested the establishment of an OHCA registry.
 
Many parents and family members who are present during a resuscitation attempt would want to be in attendance if their child were likely to die, and this experience can help with later grieving without impacting on the resuscitation process. If appropriate, family-centred care should be practised and parents should be involved in the decision-making process.6 As paediatricians, although our patient is the child, his/her family members are also important—after all, if the child passes away, it is the family who must shoulder the lifelong emotional burden.
 
In summary, OHCA in children has a poor prognosis and prolonged resuscitation does not improve survival or outcome.
 
Author contributions
All authors contributed to the drafting of the letter and critical revision for important intellectual content. All authors approved the final version for publication and take responsibility for its accuracy and integrity.
 
Conflicts of interest
As an editor of the journal, KL Hon was not involved in the peer review process. Other authors have disclosed no conflicts of interest.
 
Funding/support
This letter received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
 
References
1. Philip. 死去31分鐘的女兒 [in Chinese]. Available from: https://todecidenow.wordpress.com/2021/01/31/死去31分鐘的女兒!/. Accessed 24 Jul 2023.
2. Hon KL, Tse TT, Au CC, et al. Brain death in children: a retrospective review of patients at a paediatric intensive care unit. Hong Kong Med J 2020;26:120-6. Crossref
3. Law AK, Ng MH, Hon KL, Graham CA. Out-of-hospital cardiac arrest in the pediatric population in Hong Kong: a 10-year review at a university hospital. Pediatr Emerg Care 2018;34:179-84. Crossref
4. Tham LP, Chan I. Paediatric out-of-hospital cardiac arrests: epidemiology and outcome. Singapore Med J 2005;46:289-96.
5. Schindler MB, Bohn D, Cox PN, et al. Outcome of out-of-hospital cardiac or respiratory arrest in children. N Engl J Med 1996;335:1473-9. Crossref
6. American College of Surgeons Committee on Trauma; American College of Emergency Physicians Pediatric Emergency Medicine Committee; National Association of EMS Physicians; American Academy of Pediatrics Committee on Pediatric Emergency Medicine; Fallat ME. Withholding or termination of resuscitation in pediatric out-of-hospital traumatic cardiopulmonary arrest. Pediatrics 2014;133:e1104-16. Crossref
7. American Heart Association. Highlights of the 2020 American Heart Association Guidelines for CPR and ECC. 2020. Available from: https://cpr.heart.org/-/media/cpr-files/cpr-guidelines-files/highlights/hghlghts_2020_ecc_guidelines_english.pdf. Accessed 3 Apr 2021.
8. Fan KL, Leung LP, Siu YC. Out-of-hospital cardiac arrest in Hong Kong: a territory-wide study. Hong Kong Med J 2017;23:48-53. Crossref
9. Yan S, Gan Y, Jiang N, et al. The global survival rate among adult out-of-hospital cardiac arrest patients who received cardiopulmonary resuscitation: a systematic review and meta-analysis. Crit Care 2020;24:61. Crossref
10. Wu WY. Out-of-hospital cardiac arrest: the importance of a registry. Hong Kong Med J 2019;25:176-7. Crossref

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