A Pelletier potpourri

I wrote on January 29, 2014:

Regarding the Boston medical child abuse case from:

http://www.bostonglobe.com/metro/2014/01/18/teen-caught-medical-and-custodv-dispute-leaves-boston-children-hospital-after-months/UHgFNo1dzWiCmUNXv6AX7K/storv.html

"Previously, she had been treated for more than a year by Dr. Mark Korson, chief of metabolism at Tufts, and a team of specialists there for mitochondrial disorder, a group of genetic ailments that affect how cells produce energy, often causing problems with the gut, brain, muscles, and heart. But when Justina's mother called last February to report that Justina was having great difficulty eating and walking, Korson directed her to Children's so she could be seen by her long-time gastroenterologist, who had recently moved to that Harvard teaching hospital. In a matter of days, a different team at Children's concluded that Justina's problems were mostly psychiatric and moved to reshape her treatment plan. After her parents objected and attempted to take Justina back to Tufts, the Children's team alerted the state.

During Justina's long hospitalization at Children's, Korson had repeatedly requested, without success, that the hospital and DCF convene a summit of all her providers, to try to build consensus about a treatment plan that would be in her best interests."

This seems to me very interesting. If the article can be taken at face value, the parents were prevented by state order from taking their daughter back to the institution at which she had been receiving her care previously.

Of further note, there has now been a proposal that there be a special session at the UMDF meeting to address an apparently increasing trend in charges of medical child abuse being leveled against presumed and diagnosed mitochondrial disease patients and families.

A reply from A: 

It is striking how terrified journalists are writing about the cases, particularly since, unlike us, journalists don't need to take a position on what truth is. In this context, the 2 part series by the Boston Globe was most remarkable.

As a result of this legal action, the taxpayers of Massachusetts have paid the cost of hospitalization of a child from Connecticut for most of a year. Part of the issue is that in Massachusetts it is hard to find an expert without ties to the hospitals involved in this case. The system needs to be more flexible so a state facing such a situation can do what I would do when faced with such an expensive decision - fly in some expert from another state who sees patients like this all the time such as Bruce Cohen or  Amy Goldstein, and get their opinion. The structural problem is Massachusetts is one in which the mechanism for providing second opinions for legal decisions is underdeveloped, resulting in excessive spending in situations such as this.

One of course feels for the family, and for the various physicians involved whose assessments are being questioned in such a public way, without much opportunity for resolving the questions.

I commented:

I have recently had involvement with two cases where the specter of medical child abuse (formerly Munchausen by proxy) has been raised. These cases seem quite problematic. At what point does zealous and devoted parental care cross the line?...

Accordingly, although medical child abuse implies parental malfeasance, in cases such as these there has to be a physician enabler, who may remain convinced of the propriety of their intervention.

In my earlier a la Nikolayev submission there seems to be a situation where one outstanding medical institution considers another outstanding medical institution to be the enabler and has succeeded in convincing the local child protection services of this, preventing the child's transfer back to the allegedly enabling institution.

I further commented:

It would seem like this case should generate some conversation.

http://www.bostonglobe.com/metro/2014/01/18/teen-caught-medical-and-custody-dispute-leaves-boston-children-hospital-after-months/UHgFNo1dzWiCmUNXv6AX7Kistory.html 

http://www.nydailvnews.com/news/national/parents-accuse-mass-hospital-locking-daughter-psych-ward-article-1.1609998

http://guardianlv.com/2014/02/court-battle-to-free-american-teen-with-rare-disease-from-state-custody/

http://www.glennbeck.com/2013/11/21/kidnapping-hospital-takes-custody-of-teen-girl-after-parents-argue-against-diagnosis/

I further wrote:

I am well aware of the vagaries and limitations of the popular press. Given this, the Justina Pelletier case, on the face of it is quite disturbing. More than a year ago, a now 15 year old girl receiving care from a metabolism physician at Tufts with a diagnosis of mitochondriopathy wanders into Boston Children's and in short order is given a diagnosis of somatoform disorder and her mitochondriopathy diagnosis is totally discounted. When her parents attempt to transfer her care back to Tufts, their parental rights are terminated. Since they sought and followed medical advice from Tufts regarding the diagnosis and management of their child, they are evidently deemed culpable of medical child abuse. No liaison is made between Boston Children's and Tufts regarding Justina's management. Most recently, a judge placed Justina in foster care. A gag order prevents Boston Children's from explaining itself. The evidence available suggests that Justina's clinical status in notably worse than it was prior to her entry into Boston Children's.

See: http://www.foxnews.com/on-air/the-kellv-file/transcript/2014/02/25/mass-iudge-sentences-iustina-pelletier-foster-care 

http://www.foxnews.com/on-air/the-kelly-file/transcript/2014/02/21/father-speaks-out-losing-custody-daughter-over-medical-issues 

http://www.huffingtonpost.com/cristy-balcells/first-do-no-harm-how-we-f b 4843997.html

A reply from B: 

Wait, a confusing story from Fox News?!

Sounds more like POTS. Mito, child abuse, POTS...splitting hairs. It will be interesting to see how that case evolves. I'm guessing this young pediatrician might be in trouble here.

A reply from A:

I think that people are reluctant to discuss this publicly because they don't know the details. One leading mitochondrial expert told me that some of the details in the public record are incorrect. In Massachusetts, people are also reluctant to discuss the case because of fear of angering the institutions and doctors involved.

But the part of this that stands out as unreasonable on the face of it is excluding Mark Korson from the deliberations. Mark is a very reasonable guy. Whatever the answer, excluding Mark seems very odd. Furthermore, excluding Mark send a bad message to all referring doctors and parents that sending a child to Boston Children's Hospital can result in a child being taken away from the family over the objections of the referring doctor.

I replied: 

I am reminded of Jonathan Pollard who allegedly did something oh-so bad, but, if anyone actually knows what this is, they are not telling the rest of us.

A reply from C:

How does Boston Childrens' define somatoform disorder?

My response:

I would assume the standard understanding of somatoform disorder is applied, to wit, from Wikipedia:

A somatoform disorder is a mental disorder characterized by symptoms that suggest physical illness or injury, symptoms that cannot be explained fully by a general medical condition or by the direct effect of a substance, and are not attributable to another mental disorder (e.g., panic disorder). In people who have a somatoform disorder, medical test results are either normal or do not explain the person's symptoms, and history and physical examination do not indicate the presence of a medical condition that could cause them. Patients with this disorder often become worried about their health because doctors are unable to find a cause for their symptoms. This may cause severe distress. Preoccupation with the symptoms may portray a patient's exaggerated belief in the severity of their ill-health. Symptoms are sometimes similar to those of other illnesses and may last for several years. Usually, the symptoms begin appearing during adolescence, and patients are diagnosed before the age of 30 years. Symptoms may occur across cultures and gender. Other common symptoms include anxiety and depression. In order for an individual to be diagnosed with somatoform disorder, they must have recurring somatic complaints for several continuous years.

I wrote:

I know of some practitioners who seemingly diagnose everything that walks with PANDAS, a disorder that I cannot be certain I have ever seen. I knew of a practitioner who seemingly diagnosed everyone who came to him with partial complex 1 deficiency. Do parents who see such a practitioner raise suspicion of medical child abuse? If a neurosurgeon has relatively liberal criteria for surgical intervention, do parents who avail themselves of his/her services raise suspicion of medical child abuse?

In the Pelletier case it is not so much the rectitude or lack thereof of the mitochondriopathy diagnosis that concerns me, as does the nullification of parental autonomy.

To quote from a blog (http://www Idiackson.net/justina- pelletier-a-sick-girl-trapped-between-two-hospitals-and-the-state/): The disorder diagnosed by Boston Children's Hospital is a mental disorder that causes the patient to have imaginary pain. As a result of this diagnosis, medical treatment for mitochondria) disease was stopped and Justina was confined to a locked psychiatric ward, where she was told by workers she would never be going home. Take a look at the before and after picture below and tell me if you think the mental health treatment has been good for Justina....The picture above tells part of the tale. In December 2012, Justina Pelletier was an active teenage girl with her entire life in front of her. In February 2014, she looks as if she is on the door of death. Two things have changed in that span of time. First of all, she is allowed no private visits with her family, and even then, only an hour at a time. No private family interaction at all. Second, her medical treatment has been completely changed. Instead of being treated for mitochondrial disease, she is being treated for a mental health disorder. It is clear to see, her health has not improved as a result of the change in treatment. Because I am a simple man with at least a little common sense, it seems to me Justina should be continuing her original treatment. It was, after all, being effective. Does that not count for something in the medical and legal worlds?

I wrote further:

A Cautionary Tale: How Unconscious Biases Can Deliver Flawed Medical Judgments
http://cognoscenti.wbur.org/2014/01/28/justina-pelletier-lauren-stiller-rikleen

A reply from D:

This story is very sad and disturbing, but when I read the news reports, it is not obvious to me whether Boston Children's diagnosis is incorrect. If they did not allow the Tufts physician to see this girl, then that was not a collegial thing to do but I can't imagine that the attending pediatric neurologist at Boston Children's did not see something strongly suggestive of somatoform disorder before taking such extreme measures. Which of us has not done a lot of soul searching before calling Child Protective Services? I don't think any of us does this lightly. Of course, the greatest tragedy is the harm done to this girl, but it is not yet clear who has done this harm.

A reply from C:

Did this child get to see the Pediatric Neurologist at Children's? It is unclear from the reports and because of the Judge's blackout, it will be a long time before we know.

A reply from E:

Unfortunately in this somatoform argument we have seen holy warriors on both sides some of whom would not hesitate to destroy a colleague’s opinion and reputation on the basis of their own entrenched views. The fact is that there really is no accepted consensus as to when to stop investigating. Early categorizations on both sides can lead to harm to patients. There are of course cases were according to current knowledge the diagnosis of a somatoform disorder may be easily made. Before Prof Segawa described DOPA sensitive dystonias what would we have called these children? NMDA receptor encephalitis patients are still in the early stages characterized as having psychiatric conditions.) have seen a few such in a hospital in the UK where many were adamant that the child had a somatoform disorder. If the tests had come back as negative their entrenched views would have become more entrenched. On the other hand due to delay in giving IVIG (possibly) we have seen rapid development of cerebral atrophy and have regretted. So there are grey areas and one should be a bit circumspect on both sides of the argument till we know all the facts.

I replied:

The Coalition for Diagnostic Rights' letter to the judge
http://www.diagnosticrights.org/1434-2/

A response from F:

Since no one in Boston is able to provide the facts (and thus all comments on this are mere speculation), I suggest that future comments focus on the generic problem of medical child abuse. In particular, I would invite our colleagues to share their experiences with what I would call "mito-quacks," who seem to have joined the ranks of "Lyme-quacks" and "autism quacks" in our field.

A response from E:

Just as the autism quacks and other such there is also a group of extremists on the other side of the fence. These are those who think every medically unexplained illness is somehow a form of abuse and that the practitioner who does not agree with this view is somehow flawed. There are of course many neurological conditions with definitive signs where your scans and other tests may not always reveal an obvious cause. Examples for this include early AIDP/SCIWORA etc. This looking for abuse under every bed led to the anal dilatation test and other such scientifically unproven methods like diagnosing abuse based on the demeanor of the parent .In one highly publicized case this was done by watching a TV program. Pediatric neurology opinion also differs depending on the country and the expertise. Therefore it is a ethical minefield .SUDEP is another condition which by definition is unexpected. Therefore would we take action against a parent/Physician if it happened during drug change or drug withdrawal as you can always find an expert who is full of conviction and can mislead panels and courts.  I am happy to contribute to any debate having occasionally faced the ire of such ayatollahs.

I replied: 

And the PANDAS quacks? Now that another ubiquitous organism, mycoplasma, has been implicated, seemingly producing PANDAM, we now have PANS.

Also, as I mentioned earlier, I knew of a practitioner who found partial complex 1 deficiency to be present with disconcerting frequency.

When we "focus on the generic problem of medical child abuse" and then transition to an invitation for commentary on mito-quacks (certainly without any implication whatsoever that mito-quackery has anything to do with the recent case at hand), it raises again a question I tried to pose earlier. Who is culpable for "medical child abuse", the parent(s) or the physician enabler?

A response from E:

However I feel free debate is the best answer to those who prefer to express their opinion in closed halls and prefer to go unchallenged. Different schools of medicine were a well-accepted concept some years ago .now questioning of orthodoxies like the Munchhausen lobby (strong in some countries) may land you in trouble. Therefore it is important that we allow for debate and the role of self-styled experts of all persuasions in such debates.)

A response from F:

(We need) to be careful how we express our feelings about this case where as you say we only get snippets of the facts and I for one have not heard anything about the Tufts physician's view.

My response:

Similar thoughts from Lynne Vogel in the Daily Kos 3/7/14:

When charges of abuse are levied they target the child's caregivers, not the medical providers. How can it be that hospitals and doctors have been immune from medical child abuse prosecution when they are coparty to the alleged crime?

A child's guardian may deceive pediatricians regarding symptoms but clinical tests, x rays and scans should reveal deceptions. Medical child abuse convictions disclose that most victims received long term inappropriate and dangerous treatment recommended by their physicians.

I wrote:

http://www.bostonglobe.comilifestyle/health-wellness/2014/03/25/permanent-custody-iustina-pelletier-awarded-state-massachusetts/puvPhesGkKE6rGLid2VM2L/story.html

More amply: http://www.bostonglobe.com/lifestyle/health-wellness/2014/03/25/permanent-custody-justina-pelletier-awarded-state-massachusetts/puvPhesGkKE6rGLid2VM2L/story.html

A reply from G:

Second and third hand information can never be considered reliable and we really have no idea of the actual facts of the "Boston" case. However, the situation does touch on the issue of 2nd opinions…

When the provider of the second opinion disagrees with the current management or approach:

1.  I know this is confusing, but I have the advantage of your current neurologists' workup and treatment efforts to date, I believe this is what is going on and this is why. I will communicate my opinions to your neurologist and you can discuss this further. If he/she disagrees with my opinion, you can continue with his/her approach or I'd be happy to see you back to pursue mine. (Again, this is the approach taken when provider of second opinion knows the original neurologist).

2.  I disagree with the approach taken by your current neurologist, I'm on the right side of the river, and I'm right and he/she is wrong.

Sadly, it has been my experience that there are institutions that generate a lot of # 2 responses. Our discipline is of sufficient complexity that one should always allow for the possibility that the other guy, regardless of which side of the river, just might be right.

Humility over hubris is just good policy.

A reply from E:

You are lucky if all that the second opinion giver states is an opinion but if they state this as fact to more official bodies like in Boston the primary opinion giver can get get into trouble.Some also irrespective of your brilliant pedigree misuse their position to run you down.lt is a question of who rather than what.

Part of the problem is the certainty some have especially those on the other side of the Thames or Hudson as the case may be that other opinions are not as valid as their own. l have worked in NY on the right side and in other places on the wrong side of the river. Suffice to say our colleagues are sometimes our worst enemies and often for the wrong reason.

On June 17, 2014, I wrote:

0 frabjous day!

http://www.bostonglobe.com/metro/2014/06/17/judge-orders-custody-iustina-pelletier-returned-parents/mDWtuGURNawSu0b00pDX4J/story.html