Feb 15 2011

Reporting Medical Cases as Human Interest Stories: Chase Britton Edition

I have not been shy about discussing journalistic behavior that I despise – so here’s another one. Take an unusual medical case and report the following about it: 1) Doctors are baffled,  2) this challenges everything we thought we knew, 3) some are calling this remarkable case a miracle, 4) the patient (or their parents) did not listen to the doctor’s negativity, and bravely persevered.

In the reporting of the case make sure you emphasize the unknown as much as possible. Doctors are just besides themselves with how dang impossible the whole thing is. Then find a physician or other expert who is relatively clueless about how to deal with the media and goad them into saying all kinds of irresponsible but very sensational statements. In order to showcase the triumph of the human spirit, exaggerate as much as possible how much better the patient is doing than they should be, according to those nasty skeptical doctors.

Now before someone accuses me of being a curmudgeon, let me say that I get the human interest angle of unusual medical stories. I have no problem with showcasing brave and optimistic patients or parents, or even overly enthusiastic therapists. But I do object to rank mystery-mongering, getting the facts wrong, and not talking to proper experts. I also find it annoying when physicians or scientists who are not media savvy ram their feet down their throats.

Chase Britton

The story of Chase Britton is certainly an interesting one, and it’s unfortunate that the reporting on this case has been so dismal. Chase has pontocerebellar hypoplasia. According to reports he has an absent pons and cerebellum, although I could not find any imaging online. It is possible that his cerebellum is atrophied to the point that it is not visible (or easily visible on MRI scan), but there is probably a remnant there. The pons also cannot be missing. That’s like saying someone’s neck is missing. It is just atrophied – perhaps the ventral pons is missing or atrophied.

The picture above is an MRI scan of a patient with pontocerebellar atrophy – not Chase Britton. I include it to help you visualize the anatomy. This is a sagittal view, meaning a vertical slice down the middle from front to back. The cerebellum is a separate structure at the base and back of the brain. In this image it is visible, but very atrophied – just a shriveled walnut. Immediately in front of the cerebellum is the pons – part of the brainstem. In this image the dorsal (back) part of the pons appears atrophied. As further evidence for atrophy there is a big fluid-filled black space where the cerebellum should be.

The story of Chase Britton is actually consistent with published case series – despite the fact that headlines declare doctors “baffled”. For further background there are different types of pontocerebellar hypoplasia. One type is genetic – mutations that result in abnormal brain development. These children tend to die either in infancy or young childhood, usually from impaired respiration. Chase does not appear to have this variety.

The other is acquired – in this type early sonograms reveal the presence of a cerebellum, but the child is born with a severely atrophied cerebellum and pons. At some point the development of these structures is impaired. This category can be further divided, with one subcategory who have suffered from a bleed, stroke, or infection in the brain that damaged that part of the brain impairing its development. Chase also has no evidence of this.

The category that Chase does fit into is pontocerebellar hypoplasia resulting from prematurity. Here is a case series that fits Chase very well. He had evidence of a cerebellum on early sonograms, and he was premature. There are other possible factors as well. Here is a case of a child with pontocerebellar atrophy apparently resulting from vitamin A deficiency.

So what is so unusual about Chase’s case that baffles doctors? I don’t know.

Perhaps it is that Chase is able to function as well as he does. Again – I don’t want to sound like the curmudgeon. I hope that Chase exceeds all expectations, and his parents seem to be highly motivated to give Chase every opportunity to maximize his function. Perhaps this has allowed him to do better than average.

But his degree of neurological function (as evidenced by the video, not by the description of what he can do) seems to be in line with his anatomical deficits. He can move, but he has very poor coordination. He walks with a walker, and does not seem to have the balance to walk without assistance. He is also legally blind.

The cerebellum is primarily involved with motor coordination. It is not part of the primary motor pathways. It is like a parallel pathway that processes motor information and helps coordinate different groups of muscles together in order to produce balance and precise movements. It is also involved in memory for learned motor tasks – like shooting baskets.

So without the cerebellum you can still move. Your brain will do the best it can without the dedicated coordination processing of the cerebellum, but the best it can do will be clumsy movements. You can think about it this way – imagine if walking were not an automatic function for you and you had to think about every move. You might be able to stumble along, but it would never be graceful and you would never be able to walk on a balance beam or do backflips.

Take a look at the video of Chase – that’s basically what we are seeing. There’s nothing baffling or miraculous about this.

I understand optimism, but the interview with the therapist seemed disconnected from reality. She said that Chase can use scissors better than her, but the video shows someone helping him use scissors in an obviously clumsy manor. She says he can ride a bike – without further clarification. This would be remarkable for any 3-year old, and there is simply no way Chase can ride an actual bike. In one shot, however, you can see in the background a four-wheeled cycle (not a “bike”) and that is probably what the therapist was referring to. I’m sure if you strapped him into that cycle, or held him in place, he could manage to work the peddles.

Conclusion

The story of Chase Britton and his parents is interesting from a neurological point of view. It’s a shame the reporting missed out on the real story here and chose instead to go for cheap sensationalism and the tired cliches of “baffling miracles.”

Also, we can be uplifted by the courage and strength of Chase’s parents, who seem to be meeting this challenge with optimism and a very productive “can do” attitude, without exaggerating the truth. I’m sure the real story is more complex than this, but we can acknowledge human strength and virtue without turning a blind eye also to human frailty. We’re not children, despite the journalist’s apparent attitude toward their viewers.

I am tempted to be critical of the doctor in the report, but I have learned from past experience that often time such doctors (or other experts) were horribly misrepresented by the journalist. This is possible here. It is almost certain that he was filmed for a couple of hours, and we are seeing those bits that were plugged into the narrative the journalist had already written. But at the very least, scientists and experts have to learn how to deal with the media. You have to give crisp soundbites that can all stand on their own. You have to resist the urge to take the bait when a reporter invites you to make a sensationalistic statement.

It also seems this doctor, while being an expert in fetal medicine, was not a neurologist. It did not take me long to find a case series that put the case of Chase Britton nicely into context. He could have done this in prep for the interview, or deferred to a neurologist with experience in this syndrome.

This story does not, as we are told, force us to reconsider what we thought we knew about the brain. It is an uncommon, interesting, but wholly unremarkable case that conforms to existing knowledge. It is, however, further evidence for the terrible state of science reporting. This report would serve only to confuse and misinform the public, which the journalist would likely justify (if they bothered to do so) with the fact that they were making people feel good.

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28 responses so far

28 Responses to “Reporting Medical Cases as Human Interest Stories: Chase Britton Edition”

  1. Michael Meadonon 15 Feb 2011 at 9:09 am

    Thanks for blogging this, Steve. Demystifying these kinds of stories is important.

  2. superdaveon 15 Feb 2011 at 9:25 am

    Steve, what’s a realistic prognosis for someone with this condition?

  3. Michael Meadonon 15 Feb 2011 at 9:32 am

    Btw, this affair reminds me of the following fantastic quote from Darwin:

    “Ignorance more frequently begets confidence than does knowledge: it is those who know little, not those who know much, who so positively assert that this or that problem will never be solved by science.”

  4. Steven Novellaon 15 Feb 2011 at 9:38 am

    He will likely do fine in terms of survival, and with physical therapy can have significant function, but will always be clumsy. He will probably always need some form of assistance to walk. So functionally it will be like having cerebral palsy, except he is also blind.

  5. dhbritishon 15 Feb 2011 at 9:49 am

    You are welcome to contact me…get the facts…discuss Chase. I appreciate the way you gave your opinion while being kind.

    As Chase’s Mom though, I know best. lol Ok, not doctor best, but I’ve heard all the info everyone has on him over and over again. I haven’t responded to much I have read but found this very interesting. Are you a neuro? Dr. du Plessis focus is on fetal cerebellar development, if you research you’ll see the very many articles he has published, which is why we searched him out. He was interviewed for an hour, yet they only used two blank look comments, so I agree with you there.

    Many doctors have had the “atrophied but some remaining” theory. But it’s not the case. If you are a neuro I’d be happy to send you MRI images of Chase’s brain if you fax me a signed statement that you will not share them.

    Let me know if you’d like to discuss this further, pass aong a little background to me, and maybe an email address if you’d like.

    Thank you for taking the time to look into this…

    Heather Britton aka Chase’s extremely proud Mom

  6. dhbritishon 15 Feb 2011 at 9:51 am

    Oh, and he’s legally blind…he can see quite well except for what we assume could be “swiss cheese vision” spots of darkness mixed with spots of vision. He has bilateral optic nerve colobomas.

  7. Steven Novellaon 15 Feb 2011 at 10:11 am

    dh- thanks for leaving a comment. I am a neurologist practicing at Yale.

    I would like to see the MRI scan, and I would like more details than were available in the news reports. As I wrote – your son’s case is very interesting. The point of my post was to criticize the quality of media reporting and to give some real background to help put the story into medical perspective.

    You can e-mail me privately at snovella (at) theskepticsguide.org

  8. CrookedTimberon 15 Feb 2011 at 10:30 am

    These types of stories seem to play to a strange fascination much of the public has with seeing experts taken down. This situation is plenty fascinating without inventing that spin.

    @Heather
    Good luck, Chase seems like an inspiring kid!

  9. Ur23on 15 Feb 2011 at 10:30 am

    Thank you Steven for this article – I have a feeling that this particular media piece will be quite often (ab)used by dualists (and other kinds of people) still looking for brain-independent soul. Posts such as yours are really very much needed.

    I also hope that the fact that Mrs. Heather Britton contacted you will result in a more detailed post on this very interesting case. It would be really great to hear something more about it.

  10. bmcon 15 Feb 2011 at 10:45 am

    It probably goes without saying (but, of course, I’ll say it anyway): If you do review his MRI, and you can share additional insights without violating the terms or spirit of your agreement with Chase’s mom, this reader would definitely be interested in a follow-up article.

    Brian Clapper

  11. tmac57on 15 Feb 2011 at 11:22 am

    @dhbritish-I am happy to see that you have joined the discussion.I was a bit disturbed by the quote from the article of:

    And then we’d go to the neurologists and they’d say, ‘That’s impossible.’ ‘He has the MRI of a vegetable,’ one of the doctors said to us.”

    Is that accurate? If so I am quite surprised that a neurologist would use such insensitive language as this while talking with a parent of a child.
    Best wishes to you and your family.

  12. Steven Novellaon 15 Feb 2011 at 1:07 pm

    Yeah – the “brain of a vegetable” comment got me too. This is just nonsense. His cortex appears to be intact. The pons is atrophied, not absent, and this is not incompatible with consciousness.

    I also did not mention plasticity. When damage or developmental problems occur in utero, brain plasticity is still very high and the hardwiring can partly compensate. Sure, if you took a fully developed adult and removed that much of their pons they would probably be comatose, but that does not apply to a fetus.

  13. SARAon 15 Feb 2011 at 1:10 pm

    Well, whatever else was wrong with the report, I think it will generate more help for Chase. More experts get interested and help, more thought, diagnosis and therapy options are considered.

  14. Wholly Fatheron 15 Feb 2011 at 1:25 pm

    To Heather:

    You mentioned Chase has optic nerve colobomas. There are several syndromes associated with colobomas, including COACH syndrome (and closely related Joubert syndrome) which also have cerebellar atrophy/hypoplasia. I don’t know if Chase has other features of these syndrome, or whether or not these have been considered.

    Here is a link to the Online Mendelian Inheritance in Man (OMIM) page on the subject:

    http://www.ncbi.nlm.nih.gov/omim/216360

    Best wished to you and your family.

  15. daijiyobuon 15 Feb 2011 at 1:55 pm

    Per “the terrible state of science reporting”,

    even the New York Times has Anahad O’Connor reporting City homicides and the like, while also doing interesting sciencey articles

    http://topics.nytimes.com/topics/reference/timestopics/people/o/anahad_oconnor/index.html .

    -r.c.

  16. James Foxon 15 Feb 2011 at 2:06 pm

    @Heather
    Best wishes and you have my admiration for being willing to have this type of open discussion and for making the sacrifices and efforts necessary to care for your child. I spend my days investigating and working with parents who abuse and neglect their children or who fail to get necessary medical care for their children. Hearing about a child with this level of need and parents who are responding to the challenge as best as they can is a story worth hearing in itself.
    ~J

  17. bluedevilRAon 15 Feb 2011 at 3:41 pm

    I’m glad you brought up plasticity. I was wondering about that. If young patients that have undergone left hemispherectomies can compensate by developing language centers in the right, could Chase somehow have compensated for his pons/cerebellar deficits? I imagine it would be difficult for other areas of the brain to adopt these roles given the unique structure and function of the pons and cerebellum. So a very small structure but still functional structure seems the most likely. I am reminded of the French man who had the extensive hydrocephalus with a greatly reduced cerebral cortex and he was still mostly functional.

  18. dhbritishon 15 Feb 2011 at 4:55 pm

    negative for Coach and no Molar tooth sign of Joubert…it’s the colobomas that take away from some syndromes that he might have otherwise fit into.

    I like discussing it because I want to learn…

    Maybe there will be a CHASE SYNDROME coming from this!?

  19. roroon 15 Feb 2011 at 7:31 pm

    I wonder if neuroplasticity is at work full-time what parts of Chase’s brain are compensating for the deficit?

  20. norrisLon 15 Feb 2011 at 11:51 pm

    Not suggesting for one moment that this applies in any way to Chase, but as veterinarian I am aware that the feline panleucopaenia virus will cause kittens to suffer from cerebellar hypoplasia. These kittens can survive although would tend to be a little inccordinated, depending upon the degree of cerebellar hypoplasia.
    All the best to Chase
    Stuart

  21. Mikeon 16 Feb 2011 at 3:19 am

    Dr Novella: If it turns out that your explanation is wrong (and the total cerebellum is missing, rather than just being atrophied), then surely wouldn’t this just be a case of cerebellar agenesis? There have been a few cases of this, I think, with the most famous just being known by the initials H.C.

    Then, as mentioned by others above, neuroplasticity helps to compensate for some of the lost functions.

    Great article though, and it’s encouraging to see Chase’s mother is taking an active interest in her son. I think all too often people will hear that (idiot) doctor’s opinion that their son has the “brain of a vegetable” and stop asking any more questions..

  22. Watcheron 16 Feb 2011 at 10:02 am

    This should be on SBM, or at least a link to here on the website. Although, maybe there would be too many cooks in the kitchen at that point. Then again, people from such a diverse number fields could be helpful in the discussion.

  23. dhbritishon 17 Feb 2011 at 9:03 am

    Any way you could remove my more detailed posts? I’m finding more and more links to this and had thought it was a more quiet forum of professionals. THANK YOU!!!!

  24. shalliton 17 Feb 2011 at 10:15 am

    There’s a related case reported by Lorber:

    http://www.sciencemag.org/content/210/4475/1232

    I gather Lorber’s claims were somewhat exaggerated – but do you know anything more about that case?

  25. Watcheron 17 Feb 2011 at 11:03 am

    Heather, you may want to email Steve if you want something done quickly.

  26. cyn1972on 17 Feb 2011 at 11:49 am

    I have a nephew with the same condition. My brother would LOVE to speak to the parents of Chase. I saw pictures of Chase and it was like looking at a picture of my nephew 15 years ago. My brother would love to speak with you! My e-mail info is cyn1972@aol.com – please contact me!

  27. Steven Novellaon 17 Feb 2011 at 2:02 pm

    Mike – apparently early sonograms showed the presence of a cerebellum. So this cannot be complete agenesis.

    If these sonograms are correct, then the cerebellum must have atrophied. It’s possible it was so small that it was just resorbed. Perhaps there is more going on than just failure to develop – such as something causing actual damage to the cerebellum. But the MRI did not show evidence of this.

  28. Mikeon 17 Feb 2011 at 9:05 pm

    @Dr. Novella – Ah, fair point. But watching the video of the news story on YouTube, the doctor (I’m not sure if he was the primary doctor or just some quack they found to make a soundbite) seemed doubtful whether there was any cerebellum during development – of course, that didn’t stop him from claiming that there was. I assumed this doubt was because the quality of the ultrasounds weren’t good enough to make any certain judgements.

    I know that previous complications resulted in the doctors performing more ultrasounds than standard practice, but to get scans of the brain wouldn’t they have had to use higher frequency imaging? What would the doctors have been looking for that would require that level of imaging?

    Your atrophy theory does sound more plausible to me though, and I suppose if you’re able to have a look at the scans you’ll be able to know one way or the other.

    Thanks for the reply.

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