Out Of The Old Black Bag

 

OUT OF THE OLD BLACK BAG

 

Perspective on Long-Haul COVID in Children

Part 1:  Is This Related to PANS (Pediatric Acute-Onset Neuropsychiatric Syndrome)?

 

By Anthony Kovatch, M.D.

 

Musical accompaniment: “When You Believe” from the Disney musical “The Prince of Egypt” (1998). Written by Stephen Schwartz

 

Many nights we prayed
With no proof, anyone could hear
In our hearts a hopeful song
We barely understood
Now, we are not afraid
Although we know there’s much to fear
We were moving mountains
Long before we knew we could, whoa, yes

There can be miracles
When you believe

 

Since its iteration in 1998 by Susan Swedo and colleagues at the National Institute of Mental Health, PANDAS (pediatric autoimmune neuropsychiatric disorder associated with streptococcal infections) has been the subject of great enthusiasm, healthy scientific skepticism, and academic polarization. Although widely accepted that the syndrome comprising an abrupt onset (“thunderclap presentation”) of severe obsessive-compulsive disorder, unexplainable acute anxiety, tic disorder, and/or severe food aversion in children greater than 3 years of age and adolescents is a legitimate clinical entity, lack of a proven pathophysiologic paradigm, as well as lack of conclusive efficacy of treatment modalities (prolonged antibiotic therapy, immune-suppressants) in double-blind studies, makes management largely anecdotal. The PediaBlog has reported on the presentations (here and here) as well as on the work-up for this entity (here and here). 

As with any condition under scrutiny, there are staunch supporters and there are detractors. The state of the art in unlocking the mystery of potential childhood autoimmune disorders in genetically-at-risk individuals, including those with autism spectrum disorder, is still in its infancy. The reiteration of the same symptom complex in 2010 expanded to children without a preceding streptococcal infection, PANS (pediatric acute-onset neuropsychiatric syndrome), has fostered inclusiveness, but has made the targeting of treatment modalities more nebulous.

Personally, as a general pediatrician who has cared for patients with debilitating symptoms, including many on the autistic spectrum, over the past 2-dozen years of controversy, I have by default chosen the path of “belief” and aggressive management until the dawn of certainty. However, I believe this mission statement from the NIH Center for Biotechnology Information should be our guide:

Given the severe and debilitating nature of the symptoms and clear effects on family and child functioning, identification of evidence-based, effective therapies and understanding the incidence, prevalence, and biological basis of this syndrome are necessary. These goals can only be achieved by cross-institutional collaboration, deep clinical phenotyping from prospectively collected data through collaborative registries, and well-conducted investigations of underlying biological mechanisms in this cohort.

 

Amen.

When issues are nebulous, it is easier for inquiring minds to speculate. I think this is the case with the persistence of certain symptoms in the wake of documented and appropriately treated COVID-19 infection: long-haul COVID (the colloquial term), otherwise known as post-COVID syndrome.

Characteristically, the clinical presentations of this syndrome are heterogeneous and the symptoms wax and wane. It is postulated that the inciting infection (regardless of the degree of severity, including asymptomatic infection) is followed by prolonged shedding of COVID-19 virus RNA (persistent antigenemia) which produces low CD8 cell counts. This cascade appears to be more common in infection with the Delta strain (10.8%) than with the Omicron strain (4.5%). 

This diminution in CD8 (“killer T-cells” or “cytotoxic T-cells”) compromises the ability of one’s immune system to fight off intercurrent infections of all kinds. This is similar to the diminution in CD4 (“helper T-cells”) characteristic of human immunodeficiency virus (HIV) infections.

As many as one-third of those infected with COVID-19 develop the long-haul syndrome, and, in an unfortunate 10% of them, the symptoms persist beyond one year. The distribution of complaints is consistent:

• Fatigue, especially following exertion: 33%

• Brain fog, a common feature also reported by children with PANS: 30%

• Mood lability, beyond what would be associated with a teenager: 16%

• Myalgias (muscle aches), especially in those with a positive HLA-B27 profile, associated with immunologic dysfunction: 20%

• Shortness of breath: 28%

• Neuropathy, especially peripheral neuropathy with burning as a cardinal symptom: 20%

• Tachycardia (rapid heart rate): 18% 

• Gastrointestinal symptoms, both upper and lower tract symptoms: 44%

• Chronic loss of taste and smell (greater than 9 months): 5%

• Anxiety, widespread but difficult to differentiate as a symptom of the causative disturbance versus a consequence of the frustration surrounding a protracted condition with no solid cure to date. The “thunderclap presentation” of irrational anxiety and severe obsessive-compulsive features has been reported in only a small handful of long-haulers.

 

Scientific research has documented an increased incidence of the onset of type 1 diabetes in the wake of COVID-19 infection. And a hot-off-the-presses-report in the Journal of the American Medical Association highlights a nationwide increase in the volume of adolescent and young adult patients requiring services for eating disorders since the onset of the pandemic. It is noteworthy that anorexia and food aversion are also cardinal features of PANDAS/PANS.   

 

To be continued…

 

source http://www.thepediablog.com/2022/11/30/out-of-the-old-black-bag-35/