Harold O. Conn, MD was a world-famous doctor specializing in diseases of the liver.  After his retirement he developed Normal Pressure Hydrocephalus (NPH) and thus embarked on his second career which was to study and spread awareness of this condition.  His perspective was unique and informed, and his work was generous and insightful.  In this article HA board member Marvin Sussman, PhD and Carlos Hakim, PhD pay tribute to Dr. Conn and his work.

Tribute to a Passionate NPH Physician Advocate — The Passing of Harold O. Conn, MD

By Marvin Sussman, PhD

Harold O. Conn, MD, a world renowned liver specialist (Hepatologist), author and a pioneer in the basic understanding and treatment of advanced liver disease, died on Oct. 9, 2011 of natural causes at age 85 in Pompano Beach, FL.  Dr. Conn was a physician and 50-year faculty member at the Yale University School of Medicine.  He wrote more than 400 peer-reviewed articles for national medical publications about hepatic encephalopathy, detailing lethal liver diseases.  His unique sense of humor made these articles more enjoyable.

Dr. Conn earned BS (1946) and MD (1950) degrees from the University of Michigan and interned at the Johns Hopkins Hospital in Baltimore.  He was the chief resident at Yale-New Haven Hospital, earning a two-year fellowship with Dr. Gerald Klatskin, a pioneering Hepatologist. Dr. Conn later established a liver unit at the West Haven Veterans Administration Hospital and worked closely with Dr. Klatskin for over 30 years. Today, more than 100 Klatskin or Conn trained Hepatologists are scattered throughout medical centers around the world.  One of his greatest professional accomplishments was “The Histopathology of the Liver” by Klatskin and Conn, published in 1995. This book, a benchmark reference for the diagnosis of chronic liver diseases, was Dr. Conn’s last big project until he was diagnosed with a condition that was unknown to him, normal pressure hydrocephalus (NPH).

Dr. Conn’s health began to fail shortly after his retirement; and he had difficulty walking. Over the next decade, with a misdiagnosis of Parkinson’s disease, he developed other symptoms, including loss of short-term memory and a decrease in responsiveness, reaction time, and mental sharpness. A second opinion by a young neurologist in 2003 revealed the correct diagnosis of NPH. A neurosurgeon drained 60 milliliters of cerebral spinal fluid, and his symptoms vanished. A shunt was implanted, and the symptoms were controlled.  At age 78, Dr. Conn passionately launched himself into a study of the disease, which was identified in 1965 by his friend, Salomon Hakim, M. D., Ph. D. He became an expert spokesperson for NPH awareness, publishing articles in medical journals and appearing on national radio and TV programs.  A YouTube presentation by Dr. Conn may be viewed at:

http://www.youtube.com/watch?v=XwzRBP86vZI

A 2008 paper in the Yale Journal of Biology and Medicine by Dr. Conn and Francis M. Lobo, M.D., demonstrated that a significant lack of awareness of NPH remained among physicians surveyed:

“What Do Physicians Know About Normal Pressure Hydrocephalus and When Did They Know It? A Survey of 284 Physicians”

Harold O. Conn, MD and Francis M. Lobo, MD

As a Good Samaritan, Dr. Conn enthusiastically made himself available to advise patients and the families of friends about the diagnosis and treatment of the condition.  In the ensuing decade he wrote a dozen meaningful articles about NPH, its prevalence and heredity, and he also appeared on national radio and TV programs.

Personal Perspective on Harold O. Conn’s Interest in NPH

By Carlos Hakim, Ph. D

Harold Conn, M.D. contacted me by letter in December 2003.  This was a few months after his diagnosis of normal pressure hydrocephalus and the implantation of a shunt, resulting in his successful recovery from this disease. Coincidently, his winter home was in Pompano Beach, Florida, only five minutes away from my parent’s winter home. A month later, when my father, Salomon Hakim, M.D., Ph.D. was in Florida, the three of us got together. This was the beginning of what would become a warm and fruitful friendship during the next seven years. As Dr. Conn would frequently state, he was the newly adopted member of the Hakim family and their ongoing research program.

Dr. Conn’s interest in NPH grew tremendously, to the extent that he gave up his specialty of hepatology (liver disease) and devoted his new career as an amateur “NPHologist”. His two new primary goals in his life surrounded NPH. First, he wanted to learn everything he could about NPH. Second, he wanted to make as many practicing physicians as possible aware that NPH is not a rare disease and that in most patients it is reversible, even when it is in its terminal stages.

Harold Conn was very impressed with Salomon Hakim’s description of NPH in 1964, which at that point was an unknown disease. He was captivated by the half-century of work produced by the Hakims and coworkers, including published articles and the creation of several implantable shunt-valves. These valves permitted precise and extrinsic control of the CSF-systemic shunt pressure, restoring virtual normal life to thousands of patients who would otherwise be disabled or deceased.

A very important aspect of Harold Conn’s contributions was the fact that his observations of NPH were from his perspective as a physician with the illness. When Salomon Hakim met Harold Conn in 2003, Dr. Hakim mentioned that Dr. Conn was the first physician with NPH that he had met, and that he thought his medical training would have given him different insights from a non-medically trained patient. Indeed, they did. As an experienced academic physician with a lot of free time, he saw the opportunity to study NPH, a poorly understood, and not fully accepted, but reversible illness and he accepted the challenge.

Even though Dr. Conn learned that some experts doubt the existence of NPH and that the efficacy of shunting is questioned by many neurologists and neurosurgeons, he promptly abandoned his interest in the liver and dedicated his new life to making NPH a familiar term for physicians and lay people.  Additionally, he commented on the shunt complications that he experienced. He learned about NPH the hard way; he developed it.  Dr. Conn published several articles and lectured about NPH, including, among others:  “Normal Pressure Hydrocephalus: Case Report by a Physician who is the Patient with Observations of the Patient”.

At the time of his death, he was quite advanced in the process of writing a book on NPH and his experiences with it. Dr. Conn was also beginning to organize a symposium on NPH to commemorate the 50th anniversary of the initial description of the condition (March 10, 1964).

I greatly enjoyed the numerous meetings and discussions we had. He was an avid writer and his enthusiasm with this subject was clearly displayed in his accomplishments during the years he dedicated to NPH. A few months before his death, I mentioned to him that I greatly admired his dedication and stamina and he responded:

“After a decade of depressing diagnoses I didn’t immediately accept that I had this relatively new, mysterious, reversible illness until my neurosurgeon had performed a lumbar puncture that induced an instantaneous, miraculous, remission.  I had had difficulty climbing onto the gurney, but 10 minutes after the drainage of 60 ml of spinal fluid had started I could walk again.  It was such an immediate, dramatic change that I, as an objective scientist, who does not believe in casual miracles, did not believe that it could have occurred so rapidly.  As an objective observer I assure you that this miracle did occur.  Immediately after the CSF drainage I could again walk normally and felt and acted like my old self.  For the past seven years I have enjoyed every day the miraculous results of my shunt procedure”.

HA is pleased to announce two recipients of the Hydrocephalus Association’s grant program focusing on CSF Production, Flow, and Regulation. The long term goal of these grants is to create therapeutic interventions such as a pill that could control intracranial pressure. The grantees are:

Pat McAllister, Ph.D.

Miles Johnston, Ph.D.

Pat McAllister, Ph.D., Professor of Neurosurgery and Director of Basic Hydrocephalus Research at the University of Utah, will investigate abnormal development along the ventricular walls in the brain which causes blockage of normal cerebrospinal fluid flow. His team hopes to use this information to develop novel approaches to protect or repair a hydrocephalic brain.

Miles Johnston, Ph.D., Professor of laboratory medicine and pathobiology at the University of Toronto and Senior Scientist at the Sunnybrook Research Institute, will be investigating cerebrospinal fluid (CSF) drainage mechanisms, specifically the role that the lymphatic vessels of the brain play in relation to CSF clearance. His group hopes to test how these lymphatic vessels respond to pharmaceutical intervention in relation to CSF drainage.

The research awards granted to Dr. McAllister and Dr. Johnston total $400,000 in new funding for hydrocephalus research and, along with prior awards, represent major progress around HA’s five year research plan which includes the following three priorities:

• Supporting the field of Hydrocephalus Research with Mentored Young Investigator Awards and the sponsorship of scientific conferences
• Supporting clinical research aimed at reducing shunt failure and improving outcomes
• Making basic science investments to understand the root causes of hydrocephalus

These new awards, aimed at increasing our understanding of the dynamics of cerebral spinal fluid, are HA’s first investments in the pathophysiology of CSF as part of the third priority of the five year plan. The Hydrocephalus Association has now funded grants totaling $1,367,000 since it initiated its commitment to support and fund research in 2009.

Dear American Brain Coalition Member Organizations:

Recently, the Chair of the House Appropriations Subcommittee for Labor, Health and Human Services released his funding recommendation for NIH, which included a $1 billion increase above the FY11 level. This is a tremendous success for health research advocates and your advocacy efforts played a role in making this possible.

Now is the time for ABC members — and your individual members — to contact House and Senate members and urge that NIH be properly funded in any final agreement that is reached on the FY12 Labor-HHS-Education Appropriations bill.  Currently, there is a difference between the House and Senate bills. The House produced a bill that funds NIH at $31.7 billion, accepting the $1.0 billion increase over FY11 funding that was recommended by the President.  The Senate bill, on the other hand, funds NIH at $30.5 billion, a $200 million reduction below current levels.

Please contact your Members of Congress today to request that any bill passed this year would support the House bill’s funding request for NIH of $31.7 billion. This is the minimum amount needed to at least maintain the current level of support for biomedical research in the United States.

The American Brain Coalition asks that you share this information with your own members so they can demonstrate the brain community’s strong support for NIH in FY12.  We are pleased to provide CapWiz, our online legislative action center, to make it as easy as possible to contact your Representatives.  Please visit: http://capwiz.com/americanbraincoalition/home

There will be a blue ‘Take Action!’ box with two items.  Click on “Ask Congress to Support NIH Funding in FY 2012″ and follow the steps.

For those organizations that have their own online outreach software, please share your response numbers with me in coming weeks.  We appreciate your help in this important effort.

If you have any questions, please feel free to contact me.

Sincerely,

We are extremely pleased to have Gavin Reed join the Hydrocephalus Association staff as our Research Associate.  For the past two and a half years, Gavin has been a part of the pediatric neurosurgery research team at Children’s Hospital of Alabama in Birmingham. During that time, he helped evaluate treatments and patient outcomes relative to several different neurological diseases, chiefly epilepsy and hydrocephalus. For several months, Gavin worked to gather data and enforce surgical protocol for the Hydrocephalus Clinical Research Network (HCRN).

On his new position, Gavin notes “I am very excited to have the opportunity to be part of HA’s research team and to help improve treatments and work toward defeating hydrocephalus.  I look forward to interacting with those in the hydrocephalus community, and I will strive to help promote and facilitate any research that may lead to better treatments, improved quality of life, or a cure for the individuals and families facing this condition.”

Gavin joins HA in the middle of a grant making process which will award funding to established researchers in the area of CSF Production, Flow and Regulation.  We hope to fund projects that will advance our understanding of CSF dynamics and lead to effective treatments for hydrocephalus.  We have many promising proposals, and are looking forward to making awards before the end of the year.

Welcome Gavin!

I am pleased to announce that Paul Gross, Chairman of Hydrocephalus Association’s (HA) Board of Directors, has been selected to join the 18 member National Advisory Neurological Disorders and Stroke Council -the major advisory panel to the National Institute of Neurological Disorders and Stroke (NINDS).  Paul joins three other new members and brings to the Council his entrepreneurial perspective, successful business experience, and a deep, passionate commitment to finding answers for people living with hydrocephalus.  

NINDS, a component of the National Institutes of Health (NIH), is the nation’s primary supporter of basic, translational and clinical research on the brain and nervous system.  Its 18-member Council, composed of physicians, scientists and representatives of the public meets three times a year to review applications from scientists seeking government grants to support biomedical research on disorders of the brain and nervous system. Members also advise the Institute on research program planning and priorities.

“In just a few years, Mr. Gross has done a remarkable job of engaging engineers, scientists and clinicians in plans to develop research that will lead to better treatment for people with hydrocephalus,” said Story Landis, Ph.D., Director of the NIH National Institute of Neurological Disorders and Stroke (NINDS).  “I am delighted that he will be a member of the NINDS Advisory Council and look forward to his participation.”

We are delighted as well and offer our sincere congratulations to Paul on this very exciting and important appointment.

As we put the final touches on our five year Research Initiative Plan, we wanted to find an additional way to convey our hope for the impact of increased hydrocephalus research. We were going to release this video with the plan but because September is National Hydrocephalus Awareness month, and with the exciting news that came out last week about Dr. Chun’s research on hydrocephalus, we decided to share it early. As we set our sights on our mission to eliminate the challenges of hydrocephalus, we think this video offers a powerful expression of our vision – a future with no hydrocephalus.

We are honored to have Dr. Chun as the Review Chair for HA’s current round of research grants focused on cerebrospinal fluid dynamics.  He is also the mentor to postdoctoral fellow Yun Yung, PhD, who was a grantee from our inaugural Mentored Young Investigators award in 2009 and contributed to these findings around LPA and congenital hydrocephalus.  We are very excited about the potential for their findings and we congratulate Dr. Chun on the recent publication of his results.  You can read more about his findings here.

Also, Dr. Kestle was interviewed about this paper on Medscape.com, which has broader media coverage and is helping to move awareness of hydrocephalus forward.  The Hydrocephalus Association would like to congratulate Dr. Kestle and the HCRN for the great work they are doing on behalf of our community.

Congress is making decisions now on how to allocate health care funds for next year.  If you or somebody you know is a constituent of one of the following Congressmen, or a resident of his state, please contact them by clicking on their link and let them know how important it is to you and your family to support expanded research for hydrocephalus.

1. Jack Kingston, Georgia’s 1^st Congressional District
2. Rodney Alexander, Louisiana’s 5^th Congressional District
3. Mike Simpson, Idaho’s 2^nd Congressional District
4. Denny Rehberg, Montana’s Congressman at Large

Here is an example of what to say to your Congressman.

Script for emailing  your Representative:

I am here in (your home town/state) asking for your support for expanded research for hydrocephalus – a condition caused by excessive fluid in the brain.

Hydrocephalus is a brain condition that affects approximately 1 million people in the US (including me, my son, my daughter, my spouse, etc.)

In addition to the 1 in 500 babies born with hydrocephalus each year, there is a growing incidence among Iraq/Afghanistan soldiers suffering traumatic brain injury (TBI), and a growing recognition that people develop the condition as they age, which often masquerades as dementia or Alzheimer’s.

Many people think hydrocephalus is quickly curable:  implantation of a shunt drains away the excess fluid.  In fact, shunt failure is a lifelong, dangerous and very costly medical problem for hundreds of thousands of people.  Shunts are 50 year old technology that fail frequently, causing costly additional surgeries; alternative treatment is essential.

Since you were a cosponsor of the legislation that designated a special month as hydrocephalus awareness month, I am asking you to also support expanded research for hydrocephalus in the Labor, Health and Human Services Appropriations report. Can I count on your support?

*Mention your home town

I attended the National Institutes of Health (NIH) National Institute of Neurological Disorders and Stroke (NINDS) nonprofit forum on June 1st.  Of the 27 institutes that make up NIH, NINDS is the largest potential grantor in hydrocephalus research.  The event is put on by the NINDS staff to allow patient advocacy organizations and research funders to get more information about how to effectively work with NINDS and the other institutes.

The day included a mix of education sessions on NIH basics, breakouts and networking sessions with NINDS program directors that are responsible for different portfolios of research across NINDS.  It opened with some sobering news from NINDS Director Story Landis explaining that NIH’s budget has been cut by 1%.  The major ramification of that budget cut is that fewer applications will get funded in coming years.  On the positive front, Dr. Landis referred to hydrocephalus during her opening talk thereby acknowledging that we are on NINDS radar. Much of the discussion in the breakouts was about how we (the nonprofits) could collaborate to bring more funding to NIH and work across our diseases/conditions to make scientific breakthroughs.

In one-onone conversations with program directors, we also got very positive feedback on the accomplishments of our partner and grantee the Hydrocephalus Clinical Research Network (HCRN). While the funding environment has become tighter, our strategy of funding young investigators was validated as a tactic to increase the potential for future NIH funding of hydrocephalus research. The Hydrocephalus Association has begun to build important relationships with a number of NIH program directors that will help us advance our government’s support of hydrocephalus research.