Friday, September 22, 2023

Melatonin concerns

As a growing number of American parents are giving their kids melatonin to help them fall asleep, some experts are warning of potential risks.

Nearly half (46%) of parents in the U.S. have given melatonin to a child under the age of 13, and almost one-third (30%) of parents have given the supplement to a teen over the age of 13 to help him or her fall asleep, according to a recent survey from the American Academy of Sleep Medicine (AASM).

Providing melatonin to children might seem like a natural solution — but a 2022 AASM health advisory warns against using it for children because melatonin is not regulated by the federal Food and Drug Administration (FDA).

The rise in melatonin use has also led to a spike in reports of melatonin overdose, calls to poison control centers and emergency room visits among children, the AASM has stated.

Dr. Anne Marie Morse, a pediatric neurologist in Danville, Pennsylvania, was not surprised to hear about the high prevalence of melatonin use among kids.

"We've seen that in pediatrics, it is much more likely for there to be a prescription to help a child sleep as opposed to adults," she said in an interview with Fox News Digital.

"So, it doesn't surprise me that even before a prescription is given, a parent is asking, ‘What can I do myself? What can I get my hands on to be able to help my child sleep?’"

"Because when your child's not sleeping, the whole house isn't sleeping and it can be extraordinarily disruptive," she added.

Overall, Morse sees melatonin as relatively safe. But she emphasized the need to talk with a doctor before doling it out.

"If you're going to give your child any supplements, you should really have a conversation with your physician first, to make sure it’s the right solution for the problem you’re experiencing," she said. "The doctor can also discuss dosing and how long to utilize it."

As a sleep physician who sees kids and adults, Morse said she "commonly" utilizes melatonin, especially for teenagers who have a delayed sleep phase, meaning that their circadian rhythm has shifted much later than what it should be.

"Melatonin can be very helpful in shifting the circadian rhythm," she said.

When using it for that purpose, Morse usually recommends much lower doses — sometimes as low as 0.3 to 0.5 mg — and using it further away from bedtime.

When using melatonin just for the purpose of helping someone fall asleep, she said she may recommend higher dosing — anywhere between 1 and 10 mg — and recommend taking it closer to bedtime.

As far as how long melatonin is used, Morse said it depends on the patients.

"Research has demonstrated that some populations, such as individuals with autism spectrum disorder, require longer-term treatment with melatonin," she said.

One of the biggest concerns is that melatonin is a hormone produced by the brain, Morse noted, and could potentially influence other hormones the body produces.

"In 2022, there was a 600% increase in poison control calls related to taking too much melatonin."

In animal studies, melatonin has been shown to affect the sex hormones, which can influence the timing of puberty, she warned.

"That's why we tend to be cautious about long-term use if it's not necessary," she said.

Morse’s typical approach is to start the medication, identify whether there's a benefit within three months, then take away the melatonin and gauge whether the benefit is still there.

"The goal is to partner any medication with behavioral strategies that make the medication less necessary for long-term use," she said.

Lack of regulation

Another potential concern, Morse noted, is that there is a "high degree of variability" in how much melatonin may be in any given product.

In a recent study from earlier this year, it was found that there could be anywhere from half of what was being stated on the bottle to as much as four times the amount, she said.

"The challenge is that because it's not a regulated substance in the U.S., there is a lack of predictability," she said.

To reduce the risk, Morse recommended starting with the lowest available dose.

"That way, you know the child won’t get too high of a dose, because there really is no way to predict how much the bottle is going to have."

A study in April by the Cambridge Health Alliance revealed that some melatonin gummies — which can be more appealing to kids — contained amounts far in excess of what the label indicated.

"If consumers decide to try melatonin — particularly if giving it to children — they should seek out a product that is certified by either USP (United States Pharmacopeia) or NSF (formerly the National Sanitation Foundation, now NSF International), as that is the only way to be certain that the product is accurately labeled," study author Dr. Pieter Cohen, associate professor of medicine at Harvard Medical School in Somerville, Massachusetts, told Fox News Digital at the time.

"It’s hard to say whether the short-term benefits outweigh the possible risks."

Although melatonin is a natural hormone, it is possible for people to have adverse effects from taking too much, Morse confirmed.

"In 2022, there was a 600% increase in poison control calls related to taking too much melatonin," the doctor said.

"Thankfully, there hasn’t been any evidence that it can cause something like respiratory depression, coma or death — but it can cause adverse effects like oversedation, nausea and grogginess the next day," she noted.

One study has suggested that melatonin in high doses and for prolonged periods of time could potentially increase the risk of seizures, Morse said, but "that has not been replicated."

While melatonin has a role in improving sleep, the doctor noted that it doesn’t treat all types of sleep disorders.

"Just like any medical disorder, it is important to make sure that you're identifying and treating the right one," she said. "Make sure to talk with your physician and see a sleep doctor if necessary, to make sure you're getting the appropriate treatment."

Dr. Laura Purdy, a board-certified family medicine physician in Miami, Florida, reiterated that the long-term effects of melatonin on kids aren’t known, as there isn’t yet enough research.

"I would use caution on things we don’t know more about long-term," she told Fox News Digital. "It’s hard to say whether the short-term benefits outweigh the possible risks."

Potential short-term risks include increased bedwetting, agitation and mood swings, fatigue and drowsiness, stomach pains and nausea, Purdy warned.

"Just like any medical disorder, it is important to make sure that you're identifying and treating the right one."

"Long-term risks that are being looked into more include worries about negative impacts on your child’s growth and development," she added.

"There are other ways to help your child fall asleep that I suggest trying first," she said. "If you have any questions or concerns, consult your doctor."

Importance of good ‘sleep hygiene’

As far as how much sleep kids need, Morse said it varies by age.

"Generally, the younger you are, the more sleep you require," Morse said. "The pattern and duration of sleep changes over time."

Newborn babies need around 14 to 17 hours a day, fragmented across the 24-hour period, she said.

Five-year-old children generally need 10 to 13 hours of sleep at nighttime.

For adolescents, around eight to 10 hours of sleep is needed.

"All of these are given in a range because it's not one size fits all," Morse said.

The quality of a child’s wakefulness during the day can also help parents understand the personalized amount of sleep that is needed.

"If you're noticing impaired quality of wakefulness, which can look like moodiness, irritability, inappropriately falling asleep or reemergence of naps" — that can indicate the need to see a sleep doctor, Morse said.

For parents of children who tend to be more wound-up or anxious, additional activities, like writing in a "worry journal" before bed, can help promote better sleep.

Good sleep hygiene is also important, Morse said — while it might not fix existing sleep problems, it can prevent them from developing.

"Sleep hygiene refers to behaviors prior to bedtime that align with sleepiness," she said.

That might mean having a consistent "wind-down" routine each night that will trigger the child's brain to associate it with going to sleep.

"That can start with simple things like brushing teeth and washing up, putting on pajamas and then doing a relaxing activity, whether it's reading a book, listening to relaxing or lulling music, or doing some light stretching," Morse said.

For parents of children who tend to be more wound-up or anxious, additional activities, like writing in a "worry journal" before bed, can help promote better sleep.

Multimodal epilepsy surgery in tuberous sclerosis

Ravindra VM, Karas PJ, Lazaro TT, Coorg R, Awad AW, Patino I, McClernon EE, Clarke D, Cairampoma Whitehead L, Anderson A, Diaz-Medina G, Houck K, Katyayan A, Masters L, Nath A, Quach M, Riviello J, Seto ES, Sully K, Agurs L, Sen S, Handoko M, LoPresti M, Ali I, Curry DJ, Weiner HL. Epilepsy Surgery in Young Children With Tuberous Sclerosis Complex: A Novel Hybrid Multimodal Surgical Approach. Neurosurgery. 2023 Feb 1;92(2):398-406. doi: 10.1227/neu.0000000000002214. Epub 2022 Nov 10. PMID: 36637274.


Background: Surgery has become integral in treating children with tuberous sclerosis complex (TSC)-related drug-resistant epilepsy (DRE).

Objective: To describe outcomes of a multimodal diagnostic and therapeutic approach comprising invasive intracranial monitoring and surgical treatment and compare the complementary techniques of open resection and magnetic resonance-guided laser interstitial thermal therapy.

Methods: Clinical and radiographic data were prospectively collected for pediatric patients undergoing surgical evaluation for TSC-related DRE at our tertiary academic hospital. Seizure freedom, developmental improvement, and Engel class were compared.

Results: Thirty-eight patients (20 females) underwent treatment in January 2016 to April 2019. Thirty-five underwent phase II invasive monitoring with intracranial electrodes: 24 stereoencephalography, 9 craniotomy for grid/electrode placement, and 2 grids + stereoencephalography. With the multimodal approach, 33/38 patients (87%) achieved >50% seizure freedom of the targeted seizure type after initial treatment; 6/9 requiring secondary treatment and 2/2 requiring a third treatment achieved >50% freedom. The median Engel class was II at last follow-up (1.65 years), and 55% of patients were Engel class I/II. The mean age was lower for children undergoing open resection (2.4 vs 4.9 years, P = .04). Rates of >50% reduction in seizures (86% open resection vs 88% laser interstitial thermal therapy) and developmental improvement (86% open resection vs 83% magnetic resonance-guided laser interstitial thermal therapy) were similar.

Conclusion: This hybrid approach of using both open surgical and minimally invasive techniques is safe and effective in treating DRE secondary to TSC. Clinical trials focused on treatment method with longer follow-up are needed to determine the optimal candidates for each approach and compare the treatment modalities more effectively.

Pathophysiology of pathological laughing and crying

Lauterbach EC, Cummings JL, Kuppuswamy PS. Toward a more precise, clinically--informed pathophysiology of pathological laughing and crying. Neurosci Biobehav Rev. 2013 Sep;37(8):1893-916. doi: 10.1016/j.neubiorev.2013.03.002. Epub 2013 Mar 18. PMID: 23518269.


Involuntary emotional expression disorder (IEED) includes the syndromes of pathological laughing and crying (PLC) and emotional lability (EL). Review of the lesion, epilepsy, and brain stimulation literature leads to an updated pathophysiology of IEED. A volitional system involving frontoparietal (primary motor, premotor, supplementary motor, posterior insular, dorsal anterior cingulate gyrus (ACG), primary sensory and related parietal) corticopontine projections inhibits an emotionally-controlled system involving frontotemporal (orbitofrontal, ventral ACG, anterior insular, inferior temporal, and parahippocampal) projections targeting the amygdala-hypothalamus-periaqueductal gray (PAG)-dorsal tegmentum (dTg) complex that regulates emotional displays. PAG activity is regulated by glutamatergic NMDA, muscarinic M1-3, GABA-A, dopamine D2, norepinephrine alpha-1,2, serotonin 5HT1a, 5HT1b/d, and sigma-1 receptors, with an acetylcholine/GABA balance mediating volitional inhibition of the PAG. Lesions of the volitional corticopontine projections (or of their feedback or processing circuits) can produce PLC. Direct activation of the emotional pathway can result in EL and the laughing or crying of gelastic and dacrystic epilepsy. A criterion-based nosology of PLC and EL subtypes is offered.

Too many people take too many pills

As a pharmacist in a big hospital in Adelaide, Emily Reeve would often see patients overwhelmed by the number of drugs they took each day. “They’d say ‘I take so many medicines that I rattle when I walk’,” she recalls. And she worried that some of the medications these patients were on seemed useless, or even harmful.

Dr Reeve’s patients are not unusual, at least in the rich world. About 15% of people in England take five or more prescription drugs every day. So do 20% of Americans and Canadians aged 40-79. Since the old tend to be sicker, the number of pills a person pops tends to rise over time. Of Americans who are 65 or older, two-thirds take at least five medications each day. In Canada, a quarter of over-65s take ten or more.

Not all those prescriptions are beneficial. Half of older Canadians take at least one that is, in some way, inappropriate. A review of overprescribing in England in 2021 concluded that at least 10% of prescriptions handed out by family doctors, pharmacists and the like should probably not have been issued. And even properly prescribed drugs have side effects. The more medicines someone takes, the more they will experience...

Getting people off drugs is unfamiliar terrain for modern health systems, which are mostly set up to put patients on them. But that is beginning to change. Doctors, pharmacists and nurses are setting up “deprescribing networks” to try to spread the word. (Dr Reeve, now at Monash University, in Melbourne, runs one in Australia.) England’s National Health Service published a plan to reduce overprescribing in 2021. The first international conference on it took place last year, in Denmark...

Other problems are more straightforwardly medical. Some patients end up taking several drugs that affect the same biological pathway. One example is anticholinergics, which suppress the activity of acetylcholine, a neurotransmitter. Several drugs, including some anti-allergy pills, anti-incontinence drugs and tricyclic antidepressants, work this way. But doctors are not always aware of that, says Dr Reeve.

That can cause overdosing. Loading up on anticholinergics can suppress acetylcholine so strongly that it can leave patients stupefied or confused. Often such effects are wrongly ascribed to old age, or to disease. By cutting away problematic drugs, “we’ve had incidents where we have been able to reverse the [incorrect] diagnosis of dementia,” says Barbara Farrell, an academic and pharmacist at the Bruyere Research Institute in Canada.

Overprescribing can become self-reinforcing, says Dr Steinman. Several common drugs block reabsorption of serotonin, another neurotransmitter. Taking too many can cause tremors, insomnia and jerky movements of the arms and legs. Those symptoms are often mistaken for Parkinson’s disease. So drugs for Parkinson’s are added, in what is known as a “prescribing cascade”. These, in turn, can cause low blood pressure and delirium–which are, of course, treated with yet more drugs...

Perhaps the most common reason is that patients are not told when to stop taking a drug, or forget. In America one in five patients who are given gabapentin, a potent painkiller, after surgery are still taking it 90 days later (the recommended maximum is four weeks). Often prescriptions are renewed automatically by other doctors, who see them on a patient’s notes and assume they have to be continued...

Evidence about how to proceed is nevertheless starting to build up. Brochures have been developed in Canada to help patients wean themselves off a number of common drugs. They explain, among other things, what alternatives are available—such as cognitive behavioural therapy rather than sleeping pills for insomnia. Trials suggest they work.

Automated de-prescribing tools and guidelines for some medicines have also been developed in recent years. Medsafer, one such electronic tool, increased the share of hospital patients for whom drugs were de-prescribed from 30% to 55%, according to a study published earlier this year in jama Internal Medicine. The Drug Burden Index, another tool, tallies the cumulative doses of drugs with anticholinergic or sedative effects.

A medical movement, in other words, is beginning. Its potential impact could be considerable. Keith Ridge, England’s chief pharmaceutical officer, drew an ironic but telling comparison in 2021: “With well over a billion items dispensed each year”, he wrote, “there is a huge prize to be gained in improving the health of millions of people—comparable to a new ‘blockbuster’ medicine—if we can only get this right.”

Thursday, September 7, 2023

Gene therapy and tazarotene/bexarotene therapy for multiple sulfatase deficiency

Promising results from research at The Jackson Laboratory (JAX) and University of Texas Southwestern Medical Center (UTSW) on a gene therapy for the ultra-rare genetic disorder, Multiple Sulfatase Deficiency (MSD), will be presented today during the WORLDSymposium, an annual research conference dedicated to lysosomal diseases. The data paves the way for a gene therapy approach for MSD patients, a very important and exciting step forward for the MSD community.

MSD is an ultra-rare genetic disorder which is often described as “Alzheimer’s in a child,” and leads to premature death, normally before 10 years of age. MSD is caused by mutations in the gene responsible for making formylglycine-generating enzyme, an essential enzyme needed by cells for normal function and to break down cellular waste products, encoded by the SUMF1 gene.

With funding provided by the United MSD Foundation, a parent-led patient advocacy organization that funds research grants for MSD studies, JAX and UTSW partnered to study the SUMf1 gene in mouse models of MSD. The research team utilized a mouse model with the SUMF1 gene “knocked out,” which has similar traits to human MSD patients. While many of the mice die soon after birth, mirroring the short lives of many children with MSD, some of the mice survived past two weeks, providing sufficient time to test possible treatments.

“The experiments were challenging given the early mortality of the mouse model and need to explore several routes of administration and doses,” said Maximiliano Presa , Ph.D., study director at The Jackson Laboratory. “These were big experiments that required a lot of effort, skill and planning by the entire team. The data are encouraging, and we are beyond delighted with the results.”

The researchers used an engineered virus (AAV9) to deliver working copies of the SUMF1 gene into the mouse cells. They tried different delivery methods, locations and time points and found that delivery of the gene through a spinal tap at seven days of age alleviated MSD symptoms. The treated mice showed wide distribution of the SUMF1 gene, no signs of toxicity or neuropathy, improved vision and cardiac function, and no behavioral deficits.

“We are thrilled with these impressive and promising results, and grateful to the scientists and supporters who made them possible,” said Amber Olsen, executive director and founder of the United MSD Foundation. “The United MSD Foundation’s mission is simple: to cure MSD, and this work represents incredible progress toward that cure. Our efforts are now focused on the critical next steps necessary to get gene therapy to children suffering and dying from MSD.”

The data supports that gene replacement therapy could be a therapeutic approach for pediatric subjects, who currently lack any treatment options, and represents a huge milestone for the MSD community.

“We are excited about the results showing successful treatment of mice with MSD, which was the result of a strong collaborative effort between investigators at UTSW, JAX and the United MSD Foundation,” said Steven Gray, Ph.D., associate professor at the University of Texas Southwestern Medical Center.

“MSD is a devastating disease and there are currently no effective treatments for patients. We are highly encouraged by the positive therapeutic response in mice and look forward to further exploring the possible use of this treatment for MSD,” said Rachel Bailey, assistant professor at the University of Texas Southwestern Medical Center.

Through the collaboration with The Jackson Laboratory, UT Southwestern and United MSD Foundation, the MSD community is one step closer to saving the lives of children impacted by this devastating disorder.

Schlotawa L, Tyka K, Kettwig M, Ahrens-Nicklas RC, Baud M, Berulava T, Brunetti-Pierri N, Gagne A, Herbst ZM, Maguire JA, Monfregola J, Pena T, Radhakrishnan K, Schröder S, Waxman EA, Ballabio A, Dierks T, Fischer A, French DL, Gelb MH, Gärtner J. Drug screening identifies tazarotene and bexarotene as therapeutic agents in multiple sulfatase deficiency. EMBO Mol Med. 2023 Mar 8;15(3):e14837. doi: 10.15252/emmm.202114837. Epub 2023 Feb 15. PMID: 36789546; PMCID: PMC9994482.


Multiple sulfatase deficiency (MSD, MIM #272200) results from pathogenic variants in the SUMF1 gene that impair proper function of the formylglycine-generating enzyme (FGE). FGE is essential for the posttranslational activation of cellular sulfatases. MSD patients display reduced or absent sulfatase activities and, as a result, clinical signs of single sulfatase disorders in a unique combination. Up to date therapeutic options for MSD are limited and mostly palliative. We performed a screen of FDA-approved drugs using immortalized MSD patient fibroblasts. Recovery of arylsulfatase A activity served as the primary readout. Subsequent analysis confirmed that treatment of primary MSD fibroblasts with tazarotene and bexarotene, two retinoids, led to a correction of MSD pathophysiology. Upon treatment, sulfatase activities increased in a dose- and time-dependent manner, reduced glycosaminoglycan content decreased and lysosomal position and size normalized. Treatment of MSD patient derived induced pluripotent stem cells (iPSC) differentiated into neuronal progenitor cells (NPC) resulted in a positive treatment response. Tazarotene and bexarotene act to ultimately increase the stability of FGE variants. The results lay the basis for future research on the development of a first therapeutic option for MSD patients.

Wednesday, September 6, 2023

Strict rest should be avoided after a concussion

Patricios JS, Schneider KJ, Dvorak J, Ahmed OH, Blauwet C, Cantu RC, Davis GA, Echemendia RJ, Makdissi M, McNamee M, Broglio S, Emery CA, Feddermann-Demont N, Fuller GW, Giza CC, Guskiewicz KM, Hainline B, Iverson GL, Kutcher JS, Leddy JJ, Maddocks D, Manley G, McCrea M, Purcell LK, Putukian M, Sato H, Tuominen MP, Turner M, Yeates KO, Herring SA, Meeuwisse W. Consensus statement on concussion in sport: the 6th International Conference on Concussion in Sport-Amsterdam, October 2022. Br J Sports Med. 2023 Jun;57(11):695-711. doi: 10.1136/bjsports-2023-106898. PMID: 37316210.


For over two decades, the Concussion in Sport Group has held meetings and developed five international statements on concussion in sport. This 6th statement summarises the processes and outcomes of the 6th International Conference on Concussion in Sport held in Amsterdam on 27-30 October 2022 and should be read in conjunction with the (1) methodology paper that outlines the consensus process in detail and (2) 10 systematic reviews that informed the conference outcomes. Over 3½ years, author groups conducted systematic reviews of predetermined priority topics relevant to concussion in sport. The format of the conference, expert panel meetings and workshops to revise or develop new clinical assessment tools, as described in the methodology paper, evolved from previous consensus meetings with several new components. Apart from this consensus statement, the conference process yielded revised tools including the Concussion Recognition Tool-6 (CRT6) and Sport Concussion Assessment Tool-6 (SCAT6, Child SCAT6), as well as a new tool, the Sport Concussion Office Assessment Tool-6 (SCOAT6, Child SCOAT6). This consensus process also integrated new features including a focus on the para athlete, the athlete's perspective, concussion-specific medical ethics and matters related to both athlete retirement and the potential long-term effects of SRC, including neurodegenerative disease. This statement summarises evidence-informed principles of concussion prevention, assessment and management, and emphasises those areas requiring more research.

Strict rest should be avoided after a concussion, but light-intensity physical activity, such as walking, can help recovery. Screen use should also be limited in the first 48 hours following the injury, according to a new statement released by the Concussion in Sport Group published in the British Journal of Sports Medicine. The new guidelines come following the organization’s 6th International Conference on Concussion in Sport, held in Amsterdam from October 27 to 30, 2022.

The new guidelines also updated the definition of concussion, though “work continues toward a unified conceptual and operational definition.” The consensus statement also highlighted sport-specific strategies for prevention, including policy changes to reduce collisions (such as suggesting disallowing body-checking in hockey), neuromuscular training, and the implementation of optimal concussion management strategies to decrease recurrent concussion rates.

The conference also produced updated versions of the Concussion Recognition Tool-6 (CRT6) and Sport Concussion Assessment Tool-6 (SCAT6, Child SCAT6), designed for use within the first 72 hours (and up to 1 week) following injury. Additionally, new office tools, the Sport Concussion Office Assessment Tool-6 (SCOAT6) and Child SCOAT6, were developed for post-72-hour evaluations and subsequent weeks.

The statement updated return-to-learn and return-to-sport strategies, and underscored the benefits of physical activity and aerobic exercise as early interventions. For athletes experiencing neck pain, headaches, dizziness, and/or balance issues, cervicovestibular rehabilitation was recommended.

For individuals with persisting symptoms lasting more than 4 weeks, a multimodal clinical assessment using standardized symptom rating scales was proposed.

The potential long-term effects of sports-related concussions and repetitive head impacts, sex-based differences in concussion prevention and management, concussion diagnosis and management in para-athletes, and concussions in children aged 5 to 12 years were acknowledged as areas requiring dedicated research, the Concussion in Sport Group noted.

Monday, September 4, 2023

Euthanasia for autism and intellectual handicaps

Netherlands programs have euthanized otherwise healthy individuals with autism and intellectual handicaps in recent years, researchers have found. 

Five individuals under the age of 30, who cited autism as a factor in their decision to seek legal euthanasia, are among the cases reviewed by specialists at the U.K.'s Kingston University. 

"Factors directly associated with intellectual disability and/or ASD were the sole cause of suffering described in 21% of cases and a major contributing factor in a further 42% of cases," Kingston University's report on the issue found. 

The study noted that in many cases, doctors determined there was "no prospect of improvement" for intellectually challenged individuals because there is no treatment for their handicap.

"Reasons for the EAS [euthanasia and physician-assisted suicide] request included social isolation and loneliness (77%), lack of resilience or coping strategies (56%), lack of flexibility (rigid thinking or difficulty adapting to change) (44%) and oversensitivity to stimuli (26%). In one-third of cases, physicians noted there was ‘no prospect of improvement’ as ASD and intellectual disability are not treatable," the study reads.

Palliative care specialist Irene Tuffrey-Wijne — one of the lead authors of the Kingston University report — found Dutch doctors were legally killing patients who sought their own euthanasia because their intellectual disability or mental condition prevented them from leading a normal life, according to The Associated Press.

One record includes the case of a Dutch woman in her 30s with autism and borderline personality disorder. Doctors determined her afflictions prevented her from maintaining relationships and made forming connections with others "too difficult."

"There’s no doubt in my mind these people were suffering," Tuffrey-Wijne said. "But is society really OK with sending this message, that there’s no other way to help them, and it’s just better to be dead?"

Dutch psychologist Dr. Bram Sizoo expressed horror at the trend of autistic youths seeking assisted suicide and euthanasia's expanding acceptance.

"Some of them are almost excited at the prospect of death," Sizoo said. "They think this will be the end of their problems and the end of their family’s problems."

The Royal Dutch Medical Association has left the decision of who qualifies for assisted suicide up to medical professionals with few hard guidelines or rules.

Saturday, September 2, 2023

The ironically named Court of Protection. More end-of-life humanity in the UK

A UK court has ruled a 19-year-old critically ill female patient with a rare disorder cannot make her own decisions about continuing her medical care, as her family battles her doctors' desire to stop treatment and pursue end-of-life care.

The teen, whose identity has been anonymized as "ST" by the court, has a rare genetic mitochondrial disease that is progressively degenerative, according to court documents. Her condition is similar to that of Charlie Gard, the infant whose story drew global headlines in 2017. Charlie's parents lost a bid to bring him to the U.S. for an experimental treatment for his critical condition and he died after the hospital withdrew life-saving care after a months-long high profile legal battle.

Despite previously being a student studying for her A-levels (short for advanced levels), the 19-year-old girl has spent the past year in the ICU, dependent on a ventilator and a feeding tube. She requires regular dialysis due to chronic kidney damage from her disease. "ST" is currently fighting the hospital to be allowed to travel to Canada for an experimental treatment to treat her disease.

The Christian Legal Centre, which is advocating for the patient, argues her case is different than Gard's because she is conscious and able to communicate and argue in her defense.

But her doctor believe "ST" is "actively dying" and has no hope of a cure to resume life outside intensive care. They are asking the court to end her dialysis treatments and pursue palliative care instead. The hospital told the court the 19-year-old is incapable of making decisions about her future medical care because she is under the "delusion" that her death is not imminent.

The teen, who comes from a strong Christian family, confessed she realizes the treatment may not help extend her life but wants to keep fighting.

"This is my wish. I want to die trying to live. We have to try everything," she told clinicians, according to court documents.

Her family has spent their entire life savings to treat the girl, the Christian Legal Centre said, and wants to go to the public to raise funds for the expensive treatment, but cannot due to a "transparency order" requested by the hospital which bars reporting any information which might identify "ST", her family, or the hospital.

The girl's family described the long battle with the hospital as "a year of continuous torture" for them.

"Not only are we anxious about our beloved daughter’s fight for survival, but we have also been cruelly gagged from being able to speak about her situation. We are not allowed to ask people for prayers or for help which she desperately needs. It is a matter of life and death for our daughter to raise money for treatment in Canada, so these arbitrary reporting restrictions are literally killing her," they said via legal representation.

In court this week, a judge determined the teen "is able to communicate reasonably well with her doctors with assistance from her mother and, on occasion, speech therapists." Two psychiatrists assessed the teen was capable of making decisions about her future care for herself.

However, the judge said that, "ST" was mentally incapable of making decisions for herself because "she does not believe the information she has been given by her doctors." The judge ruled that decisions about ST's further care should be determined by the Court of Protection based on an assessment of her best interests.

"We are shocked to be told by the judge that our daughter does not have capacity to make decisions for herself after all the experts have said that she does. We are very distressed by this injustice, and we hope that, by Jesus’s grace, this will be corrected on appeal," the family of the patient said. 

Andrea Williams, the Chief Executive of Christian Legal Centre, blasted the transparency order and called the case "profoundly disturbing."

"This profoundly disturbing case demonstrates the urgent need for an overhaul into how end-of-life decisions are made in the NHS and the Courts," she said.

"What can be more natural or rational for a seriously ill 19-year-old than to leave no stone unturned and to take every chance of survival? ST has wanted to tell her story to the world in order to try and access further treatment but has been prevented from doing so by the ironically named Court of Protection," she said in a statement.

The NHS did not respond to a request for comment.

See the video, Charlie Gard's father: So sorry we couldn't save you.