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SSM Health Medical Minute: New Pediatric HHT Clinic Opens At SSM Health Cardinal Glennon Children's Hospital
ST. LOUIS – A new, highly-specialized, multidisciplinary center focused on pediatric Hereditary Hemorrhagic Telangiectasia (HHT) has opened at SSM Health Cardinal Glennon Children's Hospital.
Dr. Andrew J. White, Cardinal Glennon's Pediatric Chair, leads the clinic. He's a nationally-recognized expert on pediatric HHT and one of only a few such pediatric specialists in the country. He sits on the Global Research and Medical Advisory Board for HHT Foundation International, and is a member of its HHT Guidelines Expert Panel.
Dr. White notes that while HHT is a rare disease, it is more common than many realize and is both under-recognized and under-diagnosed.
What is HHT?
HHT is a genetic disorder of the blood vessels, also known as Osler-Weber-Rendu disease. In individuals with HHT, blood vessels develop abnormally, leading to fragile and/or malformed blood vessels (AMVs) in the nose, lungs, brain, or liver. HHT affects one out of 5,000 people in the United States, but up to 90% have never been diagnosed.
"If one of the parents has it, 50% of the children will have this disease, or each child has a 50% chance of having it, of inheriting the gene," White said. "The other reason that it's under-diagnosed is that some of the symptoms, at least in the beginning of the illness, can be very trivial or minor, who hasn't had a nose bleed."
Symptoms of HHT in children can include:
How is HHT diagnosed and treated?
Because it's a genetic disease, HHT is detected most accurately through genetic screening. Sometimes, the disease is detected after symptoms arise and families are encouraged to consider genetic testing for other related family members. If a child is found to have HHT, they should be screened for the malformations (AVMs), particularly if they are symptomatic.
Fortunately, malformed blood vessels in the brain or lungs can be identified and corrected before problems arise using catheter embolization or various surgical procedures. A child may be prescribed supplemental iron and/or blood transfusions if iron deficiency anemia is present.
For more information about the SSM Health HHT Clinic, click here.
The SSM Health Medical Minute airs Wednesdays on News 11 at 7 p.M. And FOX 2 News at 9 p.M.
What Preceded The Abrupt Resignation Of UB Pediatrics Chair?
With Dr. Steven E. Lipshultz's arrival in Buffalo in late 2018, the region landed a renowned pediatric cardiologist, a pioneer in pediatric cardio-oncology and a prolific researcher ranked among the top of National Institutes of Health-funded scientists.
He immediately became one of the top local officials shaping health care for Western New York children, assuming the titles of chair of the Department of Pediatrics at University at Buffalo's Jacobs School of Medicine and Biomedical Sciences, president and CEO of UBMD Pediatrics and pediatric chief of service at John R. Oishei Children's Hospital.
But in the late morning March 8, Children's Hospital told employees Lipshultz had been replaced by Dr. Anne Marie Reynolds as its interim chief of service for pediatrics. That evening, UB announced Lipshultz had resigned from his leadership roles in the pediatrics department and at UBMD Pediatrics but would remain as a university professor.
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UB, UBMD Pediatrics and Kaleida Health have not explained what led to the sudden change for a prominent physician who previously held similar leadership roles at medical schools at Wayne State University and the University of Miami.
But two lawsuits and interviews with more than a half-dozen people familiar with the situation paint a picture of a work culture under Lipshultz that could be hostile, dismissive and retaliatory, which caused some physicians to leave.
Since 2020, at least 20 physicians, including several division chiefs and program directors, have left UBMD Pediatrics and their teaching faculty positions within the medical school's pediatrics department, The Buffalo News found.
That turnover and disruption is significant because of the relationship between UB, UBMD Pediatrics and Children's Hospital. UB's pediatrics department employs more than 100 physicians in general pediatrics and pediatric subspecialties, who provide care through UBMD Pediatrics – one of 18 practice plans within UBMD Physicians' Group. And Kaleida then contracts with UBMD Pediatrics for medical directorships and physicians for Children's Hospital, the region's safety net children's hospital on the Buffalo Niagara Medical Campus.
The situation boiled over late last month with Lipshultz's firing of Dr. Taosheng Huang, the chief of human genetics at UBMD Pediatrics who made his displeasure over his termination without cause widely known.
Dr. Taosheng Huang came to Buffalo in September 2020, a well-known physician-scientist recru…
In an interview, Huang called his termination "totally unbelievable." About a month before he received his termination letter, Huang said he had raised concerns in an internal meeting at the medical school about the culture under Lipshultz.
"In the meeting, I said, 'This is a serious issue for the health of children in Buffalo, and I have no choice but to speak up,'" Huang said.
Since his termination, Huang has retained attorney Lindy Korn to represent him in pursuing resolution or litigation.
Lipshultz did not respond to multiple calls and a text seeking comment. In an email, he referred all questions to UB and UBMD Pediatrics.
In response to detailed questions, UB and UBMD Pediatrics issued separate general statements to The News.
"While the University at Buffalo does not comment on pending litigation or specific personnel issues, UB is committed to a work environment across the university in which the dignity of everyone is respected," UB said in its statement.
"The university responds directly to allegations of misconduct, unprofessionalism, and violations of our workplace violence and/or discrimination and harassment policy and has a zero-tolerance policy for retaliatory action against anyone acting in good faith who has made a complaint in this regard," the statement said.
Dr. Steven E. Lipshultz is no longer the hospital's pediatric chief-of-service, Children's Hospital President Dr. Stephen Turkovich wrote in an email late Wednesday morning to hospital staff.
UBMD Pediatrics said that all of its actions, which include recruitment, retention and personnel changes, are consistent with laws and regulations and are driven by the practice plan's purpose of providing health care services to Western New York children and families.
"The high-quality health care provided to patients remains uninterrupted during this time and the team continues to provide specialty and subspecialty care to children in need across the region," UBMD Pediatrics said.
In response to a question about staff turnover under Lipshultz, UB said turnover within the pediatrics department "was typical for a department of its size and scope, particularly so during the pandemic when the medical profession nationwide encountered many challenges, which led to staff changes at many medical institutions."
UBMD Pediatrics said it is growing and added nine new physicians last year, with more hiring anticipated this year.
Leading the department and UBMD Pediatrics now is Reynolds, who has been a member of UBMD Pediatrics and the pediatrics department since 2004.
When UB hired Lipshultz, the university touted his research prowess as a principal investigator of several large National Institutes of Health studies. In 2010, Lipshultz's group was among the top 2% of NIH-funded investigators.
From 2013 to 2018, Lipshultz was the Carman and Ann Adams Endowed Chair of Pediatric Research at Wayne State University School of Medicine in Michigan.
He had also been the chair of Wayne State's Department of Pediatrics until then-medical school dean Dr. Jack Sobel fired him from that position on Aug. 23, 2016, according to reporting by Crain's Detroit Business in 2017.
Reached by The News recently, Sobel declined to discuss details but said that Lipshultz did not meet the standards of leadership behavior expected of a department chair.
"He failed to meet the dean's conventional criteria for being an adequate chair of a very large department," Sobel said. "He just was not a good chair."
Asked whether he was surprised to see Lipshultz hired at another university as a department chair, Sobel said UB "never called me to find out what I thought of him."
In the 2018 announcement, which came after what UB called a "comprehensive national search," then-Jacobs School Dean Dr. Michael Cain said Lipshultz "rapidly emerged as the top candidate, possessing the administrative, scientific, clinical, leadership and visionary skills needed to move the Department of Pediatrics in the Jacobs School forward."
Kaleida said in a statement that UB engaged firm Russell Reynolds in its 2018 search for a new pediatrics chair. The health system deferred questions regarding that process to UB.
UB's statement did not address whether it was aware Lipshultz had been fired from his prior department chair job, though The News asked that specific question.
In its statement, UBMD Pediatrics addressed the two pending lawsuits filed against the practice plan, noting "the former team members in question voluntarily resigned from their positions and left the organization."
But court paperwork in each of the cases indicates the two doctors felt they had no choice but to leave.
The first complaint was filed in late 2020 by Dr. Corinne Leach against UBMD Pediatrics, Kaleida, Reynolds and Lipshultz.
Leach, a longtime neonatologist who was hired by UBMD in 1988, claims she was forced to retire from her position with UBMD Pediatrics after she had been raising concerns of patient safety and regulatory compliance to her supervisors for months.
Around the same time, Leach – 66 at the time the lawsuit was filed – had requested in November 2019 to transition to a part-time schedule in February 2020.
During a meeting Jan. 27, 2020, the complaint states, Lipshultz "threatened Dr. Leach with the possible repercussions" of a Kaleida medical executive committee investigation unless she retired immediately. That investigation would have concerned an internal complaint that claimed Leach had been abrupt during an exchange with a physician assistant over a patient care issue, the complaint notes.
The complaint claims Leach felt she had no choice but to retire immediately. In court records, attorneys for UBMD Pediatrics and Kaleida have denied allegations that they threatened or suggested an investigation if Leach did not retire.
Around the time of Leach's meeting, Kaleida – in conjunction with UBMD Pediatrics – was trimming several staff positions for nurses, respiratory therapists, dietitians and social workers, which were tied to the care provided in multiple pediatric subspecialty clinics, according to a complaint filed in March 2021 by Dr. Jamie Wooldridge against UBMD Pediatrics.
Following the cuts, Wooldridge, then-UBMD Pediatrics' division chief of pulmonology, and two male division chiefs met on Jan. 30, 2020, with then-Children's Hospital Chief Medical Officer Dr. Stephen Turkovich to express their concerns about the position eliminations, operational struggles and the effect on patient care, the lawsuit states. Those concerns, the complaint says, had been brought to UBMD Pediatrics but received no response.
At the meeting, Turkovich said he would discuss with Lipshultz whether a position of vice chair of clinical operations would be appropriate for the pediatrics department – something the group felt would address gaps in patient care, according to the complaint.
At a previously scheduled meeting on Feb. 7, 2020, Lipshultz began the session by talking to Wooldridge and saying he was "very disappointed" to find out she had met with Turkovich and, the lawsuit states, "accused her of lobbying for the position of vice chair of clinical operations." In the meeting, the complaint claims, Lipshultz told Wooldridge it "was cute" that she wanted to be vice chair.
Further, the complaint states, Lipshultz also rescinded a $30,000 stipend Wooldridge had received after serving as interim chair before Lipshultz was hired.
Meanwhile, the complaint notes, neither of the two male division chiefs who participated in the meeting with Turkovich and supported the same recommendations faced any reprimand from Lipshultz.
By mid-October 2020, after Wooldridge had been removed as division chief, she was "constructively discharged from her employment" with UBMD Pediatrics as a result of "intolerable discrimination and retaliation" as well as UBMD's failure to investigate her complaints, the complaint claims.
"We believe there was a pattern of conduct toward women and those that opposed or raised concerns about patient care," said Kevin P. Wicka, Wooldridge's attorney and a partner at the Tarantino Law Firm. "My client, along with two other male physicians, raised issues, and it was only my client that received adverse employment action for doing that."
After receiving complaints from faculty members, Dr. Allison Brashear, dean of UB's Jacobs School, called an internal meeting with the pediatrics division chiefs on Jan. 27.
At this meeting, Huang was among those who raised concerns about the situation under Lipshultz.
The News sent a specific question about this meeting to UB, which did not address it in the statement it provided.
Less than a month after that meeting, Huang opened his email Feb. 23 to find a letter signed by Lipshultz terminating his employment with UBMD Pediatrics, effective May 25. A copy of the letter, which provided no cause for the termination, went to Brashear via hand delivery, according to a copy of the email reviewed by The News.
UBMD Pediatrics said that, in some cases, it "exercises its contractual right to, without cause, end its relationship with a physician."
When Huang's termination from UBMD Pediatrics becomes complete, he also will no longer have the title of medical director of genetics and metabolism at Children's Hospital, Kaleida confirmed, adding that UBMD is obligated to fill that role.
"We continue to ensure uninterrupted care for children at OCH," Kaleida said when asked whether Huang's firing has created a gap for the treatment of genetic and metabolic conditions in children.
Less than two weeks after Huang's termination letter, Children's Hospital President Turkovich sent an email to hospital managers just after 11 a.M. March 8, informing them that Reynolds was the new interim chief of service for pediatrics at the hospital, replacing Lipshultz.
About eight hours later, Brashear sent an email to colleagues, alerting them to Reynolds' interim appointments and about Lipshultz resigning from his leadership roles at the Jacobs School pediatrics department and UBMD Pediatrics.
Jon Harris can be reached at 716-849-3482 or jharris@buffnews.Com. Follow him on Twitter at @ByJonHarris.
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Organization Publishes Official Statement On Race, Ethnicity And Pulmonary Function Test Interpretation
The American Thoracic Society has issued an official statement for clinicians that explains why race and ethnicity should no longer be considered factors in interpreting the results of spirometry, the most commonly used type of pulmonary function test (PFT). The statement was endorsed by the European Respiratory Society. The full statement is available online in the American Journal of Respiratory and Critical Care Medicine.
Spirometry is a breathing test that measures how much air is going into an individual's lungs, and how rapidly air is inhaled and exhaled. It can be used to diagnose and track the severity of such respiratory diseases as asthma and chronic obstructive pulmonary disease.
Race-specific equations or adjustments are currently used in the interpretation of PFT results. This approach requires results from Black patients to be lower—in some cases by 15 percent—than those from white patients of the same sex, height and age in order for the PFT measurements to be interpreted as abnormal. In 2021 the ATS convened a diverse workshop panel of clinicians and investigators to evaluate the use of race/ethnicity in PFT interpretation, make recommendations and provide clinical guidance. Although there was no initial consensus, and concerns that changing the existing guidelines might harm patients, five research articles were subsequently published that provided strong evidence to eliminate the use of race in PFT reporting and interpretation.
"The recent scientific evidence shows superiority of a race-neutral approach to PFT interpretation for assessing overall and pulmonary prognosis, and for assessing the impact of exposure to tobacco smoke," said panel co-chair Nirav R. Bhakta, MD, Ph.D., associate professor, critical care specialist and pulmonologist, University of California, San Francisco School of Medicine. "Reviews of clinical algorithms throughout medicine in the past decade have spawned concerns about bias and harm when race is used as a variable and has led to revisions of these algorithms."
He added, "In many areas of the world, the categorization of people by race is associated with structural racism and its negative effects. Globally, race/ethnicity is a social construct that changes across geography and time, making it difficult to envision it as a fixed characteristic of people; this is true now more than ever before with increased movement of people and mixing of cultures."
Widespread views among the white population dating to the 19th century that race distinguishes people based on innate and immutable physical features have resulted in the organization of pulmonary function measurements into categories of race and ethnicity. These beliefs predate investigation of the many factors determining lung health. This race-based approach led to the observation of differences in pulmonary function between some racial/ethnic groups and a recommendation to use race/ethnicity-specific reference equations for these measurements. Today, the scientific community has increasingly recognized that "race" is based on superficial appearance and does not encompass biological characteristics.
"Significant heterogeneity within these categories and lack of consistency of the definitions across time and geography further undermine attempts to use race and ethnicity to achieve precision in describing individuals," says Dr. Bhakta. "Normalization of perceived differences through the use of race/ethnicity-specific equations in PFT interpretation potentially contributes to medical harms caused by the lack of attention to modifiable risk factors for lower pulmonary function including those related to structural racism. People of color may experience delayed disease diagnoses or reduced access to therapies. Recent evidence from the United States demonstrates improved consistency of predictions of important clinical outcomes across racial/ethnic categories through the use of race-neutral compared to race/ethnicity-specific equations."
The ATS panel's new recommendation to use an average reference equation (race-composite GLI Global) instead of race/ethnicity-specific equations in PFT laboratories and clinical practice represents an evolution in thought supported by evidence presented since the most recent technical standards were published.
"We expect discussion and research to continue," Dr. Bhakta emphasizes. "There is a need to move beyond a simple statistical description of normal pulmonary function by examining the association between pulmonary function and meaningful health outcomes. Threshold-based decisions that lack evidence of benefit should be re-evaluated. Studies examining the potential impact of race-neutral and race/ethnicity-specific approaches on all people should continue. Further discussion on what the ideal reference population is and the development of reference equations without assigning racial or ethnic labels to the source data are still needed to achieve race neutrality."
The authors state that further research in more diverse populations across the world is also needed to identify modifiable risk factors for reduced lung function and on how to measure these factors in a way that may be translated to public policies and the application of pulmonary function testing in the clinic.
In conclusion, "There is a burden of proof of benefit for any continued use of race and ethnicity in PFT interpretation." Key conclusions and recommendations are available in the statement.
An accompanying editorial by Kevin Wilson, MD, chief of ATS Documents and Patient Education, may be found here.
The ATS 2023 international conference will include presentations addressing disparities, including the use of race and ethnicity in PFT interpretation and health disparities in lung nodule management.
More information: Nirav R Bhakta et al, Race and Ethnicity in Pulmonary Function Test Interpretation: An Official American Thoracic Society Statement, American Journal of Respiratory and Critical Care Medicine (2023). DOI: 10.1164/rccm.202302-0310ST
Citation: Organization publishes official statement on race, ethnicity and pulmonary function test interpretation (2023, March 30) retrieved 30 March 2023 from https://medicalxpress.Com/news/2023-03-publishes-statement-ethnicity-pulmonary-function.Html
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