Maria Goddard, MD – Brain Blogger Health and Science Blog Covering Brain Topics Wed, 30 May 2018 15:00:03 +0000 en-US hourly 1 Facing the Future Mon, 01 Sep 2014 11:00:47 +0000 Nine years have passed since the first partial face transplant was performed in France but the questions regarding both the physical and psychological ramifications for patients remain.

One of my first contributions to Brain Blogger examined the debate surrounding face transplantation, written in 2008 after the completion of the first partial procedure in the United States. A few days ago, GQ Magazine announced that Richard Norris, the recipient of the first successful complete face transplant, had been photographed and interviewed for the latest edition of the monthly publication. His surgery was performed in 2012 by Dr. Eduardo Rodriguez, a Baltimore plastic surgeon, in a 35 hour procedure.

Unlike a presentation in a medical journal which would focus more on clinical outcomes, the GQ feature is a short narrative of a day in Norris’ life spent with journalist Jeanne Marie Laskas. It addresses some of the questions raised in the beginning when this procedure was proposed.

From Laskas’ account, Norris and his family are proud of the role that he has played in the advancement of medicine despite the many restrictions imposed on him. Like any other transplant, there is a risk of rejection and he will require a strict lifelong regimen of medications that leave him at risk of disease due to being immunocompromised. However Norris enjoys being able to walk through life without a mask over his face as he did in the past. While article reports his difficulties as a patient, he also attends medical meetings as an example of how positive this surgery can be for patients and as a role model for further developments.

Earlier this year in April, Dr. Rodriguez and his colleagues were published in scientific journal The Lancet reviewing the 28 face transplantation cases that have been performed around the world. The study shows that none of the patients have reported any psychiatric difficulties, have accepted their new physical appearance and that some have returned to work. Functional and neurological results have also been favorable.

However, some data are limited as there is not currently a standardized reporting method for outcomes to be measured and follow-up to date remains short-term. As the medical teams involved perform more of these surgeries and the number of patients increase, we will begin to have a clearer picture of the effects on all aspects of their lives.

The benefits of facial transplantation lie beyond the aesthetic for patients who have sustained severe trauma. The ability to see, begin the process of food digestion, and speech can all be affected by injury or disease. The ability to restore all of these functions and others requires transplantation of soft tissue, bone, nerves and blood vessels from the recipient to the donor in a technically complex surgery. The key to successful long-term outcome lies not only with the operation itself but also in establishing a multidisciplinary team to monitor patients mental and physical well-being following recovery.

The debate is certainly not over regarding the long-term implications of this surgery, but for patients like Richard Norris the risk appears to be worth it.

Khalifian S, Brazio PS, Mohan R, Shaffer C, Brandacher G, Barth RN, & Rodriguez ED (2014). Facial transplantation: the first 9 years. Lancet PMID: 24783986

Laskas, Jeanne Marie. July 2014. The New Face of Richard Norris. GQ Magazine

Image via ChaNaWiT / Shutterstock.

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Is Your Doctor Happy or Burnt-Out? Fri, 04 Dec 2009 22:33:59 +0000 Is your doctor happy? This is something that may have never crossed your mind: the idea that your personal physician may not be pleased with the current state of his or her life. Even if it has, perhaps you were of the opinion that it was not of minor importance if it did not affect the quality of medical care and attention which you received.

The general public’s image of the lives that medical professionals face is colored by 50 minute sitcoms where problems related to the characters, both personal and professional, are often neatly resolved by the time the final credits roll. The trauma patient who was crashing at the beginning of the episode is seen smiling and leaving the hospital by its conclusion. Furthermore there is a misconception by many that most doctors have amassed vast riches and lavish lifestyles at the expense of the illness of others. Does this translate to the real lives of medical doctors?

A recent study appeared in the American Journal of Bone and Joint Surgery which examined the quality of life among orthopedic surgery residents and attendings. While this article focused on a specific group of individuals, the issues identified as both protective and risk factors are of relevance to everyone. Some of the items which showed a high correlation to dissatisfaction were concern over regular use of alcohol, difficulty disconnecting from work life while at home and high levels of sleep deprivation. Protective factors included making time for exercise and hobbies, spending quality time alone with partner, sense of support or mentor and taking non-work related vacations. Curiously, although high levels of stress were reported, 80% of residents and 88% of the faculty members who responded felt that the sacrifice was worthwhile, and many reported that they would still make the same career choice.

Combined with the 80 hour work week guidelines, this new information leaves us to wonder if patient care is affected. Earlier this year a multi-center study performed in three United States hospitals and one in Canada evaluated whether the outcome of orthopedic surgery performed after-hours was different to that conducted during the day. After-hours surgery refers to procedures conducted outside of the confines of a usual work day, usually at night or in the early hours of the morning. The results of this investigation by Ricci and colleagues showed that most factors were similar, including infection rate, but the after-hours group required more re-operations to remove painful hardware. However, the article was unable to identify what caused this one difference: whether it was related to surgeon fatigue or the varying levels of experience between surgeons who operated during the day or night. Other than that one factor, we can feel reassured that our surgical outcome will be similar regardless of the time of day it is done. To gather more information, more studies into the role of physician quality of life on level of medical care will have to be carried out.

Perhaps these findings will improve the doctor-patient relationship, by ensuring that patients receive a high standard of care regardless of when they see their doctor. Physicians are subject to the same stresses their patients are and, hopefully, will feel more e comfortable addressing risk factors affecting their quality of life.


Sargent MC, Sotile W, Sotile MO, Rubash H, & Barrack RL (2009). Quality of life during orthopaedic training and academic practice. Part 1: orthopaedic surgery residents and faculty. The Journal of bone and joint surgery. American volume, 91 (10), 2395-405 PMID: 19797575

Ricci WM, Gallagher B, Brandt A, Schwappach J, Tucker M, & Leighton R (2009). Is after-hours orthopaedic surgery associated with adverse outcomes? A prospective comparative study. The Journal of bone and joint surgery. American volume, 91 (9), 2067-72 PMID: 19723981

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Hope for Future Treatment Options for Lou Gehrig’s Disease Tue, 24 Mar 2009 14:37:45 +0000 Neuroscience and Neurology CategoryAmyotrophic lateral sclerosis (ALS) is a devastating neurological disorder which always results in death. This condition is often called Lou Gehrig’s disease after the American baseball player Henry Louis “Lou” Gehrig who died from disease complications in 1941 at the age of 37. In ALS nerve cells are damaged in a rapidly progressive fashion resulting in paralysis of all muscles. The usual cause of death is collapse of the respiratory system when these muscles become involved. Disease incidence peaks around the age of 60 and men are more commonly affected than women.

BaseballCurrently, there is no known cure for ALS and the medication riluzole (Rilutek) is the only one approved for use by the Food and Drug Administration (FDA) for treatment. This drug slows progression of the disease by lowering the release of glutamate and reducing the rate of damage to neurons. However, this drug has not been exceedingly effective, with most persons dying within 5 years of diagnosis.

New hope is on the horizon with the results of recent studies reported in Science. Scientists at the University of Massachusetts Medical School and King’s College in London report that they have identified that at least one heritable form of ALS may be due to mutations in the FUS/TLS gene located on chromosome 16. Furthermore, similar mutations may account for between 5-10% of all inherited cases. The researchers found mutations in 17 different families with familial ALS while no mutations were found in cases of spontaneous ALS. Of note is the fact that these families all come from a single study group, with a highly conserved gene pool; they may not be typical of a larger population. The mutation causes proteins which should be transported into the cell nucleus to remain in the cytoplasm. Either the aggregation leads to cell death or the missing protein is necessary for healthy cell function but this is not yet clear. A total of 13 mutations were identified in the study population and with identification of the gene, therapy for the treatment of this fatal disease can be more directed and hopefully more effective.


Kwiatkowski, T., Bosco, D., LeClerc, A., Tamrazian, E., Vanderburg, C., Russ, C., Davis, A., Gilchrist, J., Kasarskis, E., Munsat, T., Valdmanis, P., Rouleau, G., Hosler, B., Cortelli, P., de Jong, P., Yoshinaga, Y., Haines, J., Pericak-Vance, M., Yan, J., Ticozzi, N., Siddique, T., McKenna-Yasek, D., Sapp, P., Horvitz, H., Landers, J., & Brown, R. (2009). Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis Science, 323 (5918), 1205-1208 DOI: 10.1126/science.1166066

Vance, C., Rogelj, B., Hortobagyi, T., De Vos, K., Nishimura, A., Sreedharan, J., Hu, X., Smith, B., Ruddy, D., Wright, P., Ganesalingam, J., Williams, K., Tripathi, V., Al-Saraj, S., Al-Chalabi, A., Leigh, P., Blair, I., Nicholson, G., de Belleroche, J., Gallo, J., Miller, C., & Shaw, C. (2009). Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6 Science, 323 (5918), 1208-1211 DOI: 10.1126/science.1165942

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How Many Babies Is Too Many? Mon, 16 Feb 2009 16:54:44 +0000 Opinion CategoryLike many of you, I have watched as the world has been captivated by the birth of the octuplets to Nadya Suleman. To say that this case has opened heated debates on the ethical and psychological issues related to in vitro fertilization (IVF) would be a gross understatement. What makes this case especially newsworthy is the fact that Ms. Suleman not only has a total of 14 children, all with the help of IVF, but is also single and unemployed.

This is not the first octuplet birth to rock the medical community worldwide. In London 12 years ago the The Lancet produced an editorial on the story of Mandy Allwood. That case was also interesting for its many ethical questions: Ms. Allwood was secretly taking fertility drugs without medical supervision or informing her significant other. In the early stages of the pregnancy, doctors also recommended that she reduce the number to twins in order to ensure safe delivery and development, advice which she refused. Accusations that decisions were made based on financial incentives were compounded by statements made by her public relations consultant Max Clifford. He implied that the more children who were born, the more money there was to be made. Tragically, all eight of the babies died within an hour of birth at 22 weeks.

Exactly how many children can a woman carry safely to term and are there any limits imposed on fertility doctors?

DucklingsRecommendations have been made by the American Society for Reproduction Medicine that a maximum of two embryos should be placed in a female. However, there are those who would argue that fertilized embryos are already a life, that selective termination of some fetuses to increase survival of the others should not be done and that all embryos should be implanted and given the opportunity to become a full term pregnancy. Last year, Evans and Britt presented a review that showed fetal reduction in cases of multi-fetal pregnancies improved the overall outcome. Despite these results, it is understandably a difficult decision for many to make. IVF parents are especially reluctant and feel a special attachment part partly because of their previous difficulties conceiving.

One of the major issues that has arisen out of the Suleman octuplet story is that the fertility doctor should not have made the choice to implant this many embryos. Also, there is the question of what if any, psychological screening was conducted on Ms. Suleman before proceeding with the procedure.

What are your thoughts on this issue? Should restrictions be placed on the practices of IVF? How far away are we from so-called “designer” babies, selecting not only gender but other desirable characteristics as well? Should there be stricter psychological testing for women and couples undergoing IVF and why should the process be different for women who have children naturally?


M DEAN (1996). British octuplet pregnancy upsets the medical applecart The Lancet, 348 (9027), 605-605 DOI: 10.1016/S0140-6736(05)64810-6

American Society for Reproduction Medicine

MI Evans, DW Britt (2008). Fetal reduction 2008. Current Opinion in Obstetrics and Gynecology. 2008 Aug;20(4):386-93.

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The Memory of an Elephant Sat, 14 Feb 2009 13:14:32 +0000 Neuroscience and Neurology CategoryAre there days in your life that you would rather forget ever happened? Falling down the stairs in front or your entire class or an embarrassing fashion faux pas? By the same token, there are some occasions we wish could remain as fresh in our mind’s eye as the day they occurred — whether it is a wedding day, the birth of a child or a graduation. Scientists have found 4 individuals who possess what is being referred to as “super-memory” — the ability to recall in uncanny detail both private and world events that have taken place. Three of the individuals are male; the lone female was the first to be recognized.

MemoryFunctional MRI (fMRI) of the brain showed two enlarged regions: the caudate nuclei and the area of the temporal lobe responsible for retaining information (see our reviews on fMRI, part 1 and 2). Many studies, including a publication by Dr. Norman White from the Department of Psychology at McGill University, have shown that damage to the caudate nucleus results in impaired memory. Huntington’s disease, a hereditary disorder with memory loss and abnormal movements, is caused by a lesion in this area of the brain.

The inability to put details out of your mind can be a blessing and a curse. Suppression of thoughts or memories which cause distress is considered by psychologists to be one of the mature coping mechanisms. It appears as though those who have super-memory would be unable to accomplish this successfully. Jill Price, the first person identified, relates her in book The Woman Who Can’t Forget that she often feels anguish over the constant flood of recollections.

Investigations into this phenomenon are still in the preliminary stages as scientists continue to recruit potential subjects. In the future, studies on persons with super-memory may provide researchers with answers as to how we retain information and may be the key to diseases where memory is affected such as Alzheimer dementia and Parkinson disease. However I’m sure that there are times when these four people yearn to just forget.


N WHITE (2008). Some highlights of research on the effects of caudate nucleus lesions over the past 200 years Behavioural Brain Research DOI: 10.1016/j.bbr.2008.12.003

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New Year, New Resolutions – How Long Will They Last? Tue, 27 Jan 2009 16:03:06 +0000 Psychiatry and Psychology CategoryI love the beginning of a new year, full of hope and promise and the idea that the slate has been wiped clean and a new start can be achieved. All over the world as the clock strikes midnight and amidst the streamers and celebrations, millions make resolutions to change in the New Year. The more popular ones include losing weight and saving money. Many of you may have already broken or forgotten yours.

Recently Mind, a British mental health charity, suggested that the process of making New Year’s resolutions can be unhealthy as we focus on the negative aspects of ourselves. I do not agree. I think we can only improve ourselves as human beings if we recognize areas of our lives that need correcting or revamping. Without a continued process of renewal and re-evaluation, how will we know how far we are in reaching our goals and what changes need to made? Only when impossible targets are set such as losing 50 pounds in one month will there be an element of frustration which leads to failure. Constant checks and balances ensure that we remain on the right path or are able recognize the wrong direction and correct it.

New YearIn 1998, Miller and Marlatt from the University of Washington’s Addictive Behaviors Research Center conducted a study of over 260 subjects to determine what makes a person more likely to fulfill their decisions. They found that persons who constantly reviewed their progress and found ways to resolve set-backs were more likely to attain their goals.

I think of the beginning of a new year as an opportunity to make a life audit — a 12-month “To Do” list of sorts — and then I make a plan of how I will accomplish each item. I ensure that it is written down so that as each month passes, I am reminded of what I wanted to achieve. There is nothing more satisfying than crossing an item off as “completed”. I wish you the greatest luck and hope that at the end of your year you will have been successful in at least one of your resolutions.


Miller, E.T. & Marlatt, G.A. (1998). How to Keep Up with Those New Year’s Resolutions: Researchers Find Commitment Is the Secret of Success.

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Recognizing the Man in the Mirror Sat, 27 Dec 2008 14:09:35 +0000 Psychiatry and Psychology CategoryThree short years ago, the first partial face transplantation was performed in France and recently the first of these procedures to be conducted in the United States was successfully accomplished. This was only the fourth operation of its type but experts estimate that perhaps as early as next year the first complete face transplant will be attempted.

It is one thing to receive a donation of an organ such as a heart or liver, which is inside of one’s body and never seen, but quite another to look into the mirror everyday and see someone else’s face. While the underlying bone structure of the affected patient will largely determine the facial construct, the knowledge of the organ’s origins may be too much for some to bear psychologically. In addition, there may be some difficulty convincing the family members to allow the use of their loved one’s face for transplantation purposes. Herein lays the ethical dilemma of this groundbreaking procedure. Faces are considered to be individualistic; they express feelings and show personalities. However, this must be balanced by imaging the psychological consequences of the patient going through life with a disfigured face. Whether we care to admit it or not, humans are judged on their appearance, so being able to interact with society and continue normal daily activities and conduct their everyday life is essential. Some may even argue that the damaged face is not theirs either and transplantation offers them the chance at a normal life.

MirrorIn December 2007, the 18 month follow-up of the index transplantation patient in France was reported in the New England Journal of Medicine with good medical results. Psychological support was given at regularly scheduled intervals as part of her recovery protocol. Although no formal psychological testing was conducted, she reported being pleased with the aesthetics and felt comfortable interacting with other people.

Reading about these advances also reminded me of a medical condition called prosopagnosia or “face blindness”. This is a neurological condition where affected persons do not recognize faces, sometimes including their own. It can be caused by brain damage to the occipital and temporal lobes and there is some evidence of a congenital form of the disease. Studies have shown that patients have great anxiety in social situations and may become reclusive as a result. What happens when the face is not actually your own?

Organ transplantation is already a difficult procedure medically, requiring the patient to take lifelong immunosuppressive drugs. Combine this with the many unanswered ethical questions and facial transplantation becomes even more complicated. Therefore patient selection for this surgery must focus not only on organ compatibility but also on the emotional and mental capacity of the individual.


J.-M. Dubernard, B. Lengele, E. Morelon, S. Testelin, L. Badet, C. Moure, J.-L. Beziat, S. Dakpe, J. Kanitakis, C. D’Hauthuille, A. El Jaafari, P. Petruzzo, N. Lefrancois, F. Taha, A. Sirigu, G. Di Marco, E. Carmi, D. Bachmann, S. Cremades, P. Giraux, G. Burloux, O. Hequet, N. Parquet, C. Frances, M. Michallet, X. Martin, B. Devauchelle (2007). Outcomes 18 Months after the First Human Partial Face Transplantation New England Journal of Medicine, 357 (24), 2451-2460 DOI: 10.1056/NEJMoa072828

L YARDLEY, L MCDERMOTT, S PISARSKI, B DUCHAINE, K NAKAYAMA (2008). Psychosocial consequences of developmental prosopagnosia: A problem of recognition Journal of Psychosomatic Research, 65 (5), 445-451 DOI: 10.1016/j.jpsychores.2008.03.013

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The Doctor Can’t See You Right Now, He’s Napping Sun, 21 Dec 2008 15:12:51 +0000 Health and Healthcare CategoryOn December 2nd, the Institute of Medicine (IOM) released a report suggesting that resident physicians have further limits on work hours than those enacted in 2003 by the Accreditation Council for Graduate Medical Education (ACGME). While the number of hours per week would remain at the ACGME maximum of 80, the IOM recommends on 30-hour shifts that a “protected sleep period” of 5 hours occurs between 10pm-8am. These measures are aimed at reducing physician fatigue and the number of resultant medical mistakes.

At face value, this seems like a step in the right direction but what are the wider implications? Many programs in labor intensive specialties such as general surgery argue that the quality of education and training has been compromised by the restrictions. They dispute that patients will be safer if doctors are exposed to fewer cases and clinical contact. There have been difficulties with some programs maintaining the current policy and further restrictions will potentially stretch many training institutions to their limits. The older generation of medical practitioners views long work hours as a rite of passage which they successfully survived. They believe that this helps to develop the strong character and discipline of a physician. Younger doctors however are becoming increasingly focused on job satisfaction and overall quality of life outside of medicine.

SleepOne way to address this problem would be to increase the number of residency positions. However, this would require that more funding be available to hospitals for these salaries. In an already overburdened healthcare industry, where will this money come from? In addition, a recent survey by the Physicians’ Foundation showed that there is a reduced interest in primary care practice; even those currently in practice are dissatisfied with their jobs. Many plan to drastically lower the amount of time in practice or to leave the field altogether. Add to this increasing wait times in emergency rooms, the failing economy leading to unemployment and loss of health insurance coverage and the situation appears bleak. In these uncertain times can the public afford for doctors to work less? Many rural areas in states such as Georgia are already concerned over the lack of readily available primary care.

When the new political administration takes over in 2009, it will be interesting to see what direction they take to protect and improve healthcare in America. The citizens of a country are its greatest resource and without adequate access to medical care, the country will not be able to prosper.


Resident Duty Hours: Enhancing Sleep, Supervision, and Safety. Institute of Medicine of the National Academies, December 2nd, 2008

The Physicians’ Perspective: Medical Practice in 2008. Survey conducted on behalf of The Physicians’ Foundation by Merritt Hawkins & Associates, October, 2008.

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