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An Important Announcement About the IH Registry

An Important Announcement About the IH Registry

It is with great sadness that the Intracranial Hypertension Research Foundation is announcing the closure of our research project, the Intracranial Hypertension Registry. As of this month, June 2020, the IH Registry has ceased all operations. 

This was an extremely difficult decision for us to make. Like many others who have been financially impacted by the Covid-19 pandemic, we have tried our best to come up with an alternative to closing the IH Registry. But after 17 years and with expenses exceeding $10,000 per month, it has become no longer economically possible for IHRF to fund and operate the Registry.

If you are enrolled in the IH Registry, you do not need to do anything concerning your records. All participant records—digital, paper, imaging etc.—will be destroyed in compliance with HIPPA and Oregon Health & Science University regulation. All data in the IH Registry database will also be destroyed.

We would like to thank all of you who took the time to enroll and take part in the IH Registry. You’ve helped contribute to important, novel research and expand the growing body of knowledge about chronic IH.

We would like recognize the efforts of more than 70 IH Registry volunteers—without whom the work of the Registry would not have been possible—including physicians, researchers, University volunteers, graduate OHSU MPH students and pre-med students. We would also like to thank our Senior Volunteer Manager, Ralpha Newton. One word describes the nearly 17 years of Ralpha’s contributions as a volunteer: indispensable!

Our deep gratitude to our IH Registry Research Coordinators, who oversaw the day-to-day function of the IH Registry, streamlined data organization, and greatly assisted in all aspects of research, from ideas to publication:

IH Registry Research Coordinators:

Roshanthi Weerasinghe, M.P.H.
Brenna Lobb, M.P.H.
Sarah Golden, B.S
Jonathan Au, M.P.H.
Carin Waslo, M.P.H.
Veda Varada, B.S.
Nick Miller, B.S.
Sam Hermes, M.P.H.

Finally, we remain hopeful to see that interest in chronic IH continues to grow. And we are gratified that the IH Registry has helped play a role in that process and served as a model for other patient registries.

IHRF will continue to be a resource for the IH community. As always, we welcome your input as we plan our next steps.

Thank you,
IHRF

Published Research of the IH Registry

Mortality among patients with idiopathic intracranial hypertension enrolled in the IH Registry.
Hermes SM, Miller NR, Waslo CS, Benes SC, Tanne E.Neurology. 2020 Mar 27:

Emergency department utilization among individuals with idiopathic intracranial hypertension.
Murphy S, Friesner DL, Rosenman R, Waslo CS, Au J, Tanne E.Int J Health Care Qual Assur. 2019 Feb 11;32(1):152-163

Manifestations of Pediatric Intracranial Hypertension From the Intracranial Hypertension Registry.
Aylward SC, Waslo CS, Au JN, Tanne E. Pediatr Neurol. 2016 Aug;61:76-82.

Acetazolamide-Induced Nephrolithiasis in Idiopathic Intracranial Hypertension Patients.
Au JN, Waslo CS, McGwin G Jr, Huisingh C, Tanne E.J Neuroophthalmol. 2016 Jun;36(2):126-30.
  
The use of acetazolamide during pregnancy in intracranial hypertension patients.
Falardeau J, Lobb BM, Golden S, Maxfield SD, Tanne E.J Neuroophthalmol. 2013 Mar;33(1):9-12.

Idiopathic intracranial hypertension in the USA: the role of obesity in establishing prevalence and healthcare costs.
Friesner D, Rosenman R, Lobb BM, Tanne E.Obes Rev. 2011 May;12(5):e372-80.
 
Weight and visual field deficits in women with idiopathic intracranial hypertension.
Baldwin MK, Lobb B, Tanne E, Egan R.J Womens Health (Larchmt). 2010 Oct;19(10):1893-8.
 
Report on the second Intracranial Hypertension Research Foundation conference.
Tanne E.Cerebrospinal Fluid Res. 2008 Aug 13;5:13

Unpublished IH Registry Research: Conference Presentations and Posters



Long Term Outcomes in IIH Patients Who Underwent Bariatric Surgery
Samuel Hermes, M.P.H. (OHSU), et al. 2020 

Association of Intracranial Hypertension and Ehler Danlos Syndrome
Nickolas Miller, M.S. (OHSU), et al. 2020

Acquired Chiari Malformation in IIH Patients Secondary to Chronic Distal
CSF Leakage
Emanuel Tanne, M.D. (IHRF, OHSU), et al. 2019

The Intracranial Hypertension Registry
Carin Waslo, M.P.H. (OHSU)
Presented at Oregon Academy of Ophthalmology 2018

Understanding Intracranial Hypertension
Emanuel Tanne,M.D. (IHRF, OHSU), et al.
Presented at Oregon Academy of Ophthalmology 2018

Rhinosinus Radiographic Findings in Chronic Intracranial Hypertension: A  New Neuroimaging  Diagnostic Finding?        
S. Vararad, M.D.,et al. 2016

Translaminar  Pressure Difference as a clinical Indicator for Papilledema in
IIH Patients
Emanuel Tanne, M.D. (IHRF, OHSU) et al. 2014

Weight Loss as a Predictor of Resolution of Symptoms in Subjects with Idiopathic Intracranial Hypertension
Zoe Wyse, OHSU M.P.H. degree thesis 2013

Incidence and Correlation of Failure for Ventriculopertoneal and Lumbo peritoneal Shunts in Patients with Idiopathic Intracranial Hypertension
Richard Martin, OHSU M.P.H. degree thesis 2011

IHRF Patient Conference 2011
Multiple Registry investigations
Presented at Nationwide Children’s Hospital 2011

Pediatric Intracranial Hypertension: Surgical Interventions
Emanuel Tanne, M.D. (IHRF, OHSU)  2009

Sinonasal Mucosal Thickening May Reflect Disease Activity in Idiopathic Intracranial Hypertension
Justin Dodge, M.D. (Walter Reed Medical Center), Bronwyn Hamilton, M.D.(OHSU) et al.
Presented at the National Radiology Conference 2010

IHRF Patient Conference 2008
Multiple Registry investigations
Presented at the University of Texas 2008

IHRF Patient Conference 2006
Multiple Registry investigations
Presented at Oregon Health & Sciences University 2006

Does the Choice of Primary Surgical Procedure Correlate with a Significant Difference in Total Number of Surgeries  Needed in Medical Failure Patients with IIH? Comparing Those Undergoing ONSD Vs CSF Shunting as Their Primary Surgery
Susan Benes, M.D. (Ohio State University), et al.
Presented at North American Neuro-Ophthalmology (NANOS) Meeting 2005

Findings at Autopsy in IIH Patients
Emanuel Tanne, M.D. (IHRF, OHSU), et al.
Presented at the 3rd Neural Hydrodynamics Symposium 2005

Other IH Registry Research Initiatives

• Development of First IIH Organ and Tissue Donor Research Program with the National Disease Research Interchange (Nation Disease Research Interchange) 2013
• Original Member Registry, Global Rare Disease Registry (GRDR)
  A Global Registry for all 7000 Rare Diseases, sponsored by National Institute of Health (NIH) 2011
• Member, NASA Working Group on fluid shift hydrodynamics in astronauts

Recognition

• Research Registries: A Tool to Advance Understanding of Rare Neuro-Ophthalmic Diseases
  Blankshain K.D., Moss HE. J Neuroophthalmol. 2016 Sep; 36(3): 317–323.
• NASA Award to Emanuel Tanne, M.D. for outstanding contributions to the Visual Impairment and Intracranial Pressure Summit, Johnson Space Center


Mortality and IIH

IH Registry Study Analyzes Causes of Death in IIH Patients Published in Neurology

The IH Registry (IHR), a project of the Intracranial Hypertension Research Foundation and Oregon Health & Science University, has published a study investigating the causes of death in IIH patients in the March 27, 2020 issue of Neurology, the journal of the American Academy of Neurology.

It is the first time ever that researchers have looked at whether mortality in IIH patients is different from that of the general population of the United States, as well examined the leading causes of death for this particular group of patients.

Understanding mortality rates and risks for all IH patients help to educate and most importantly, prevent unnecessary death.

This retrospective cohort study utilized data from the IHR, a database that contains medical records, imaging studies, participant-reported questionnaire data, and annual follow-up from individuals diagnosed with idiopathic and secondary intracranial hypertension.

A standardized mortality ratio (SMR) was calculated as the ratio of observed deaths, identified by National Death Index (NDI), divided by expected deaths, estimated utilizing indirect standardization with data extracted from the Underlying Cause of Death1999–2017 dataset published by the Centers for Disease Control and Prevention (CDC) Wonder Online Database.

There were 47 deaths (96% female) among 1,437 IHR participants that met the study’s inclusion criteria. The average age at death was 46 years
(range 20–95 years).

Participants of the IHR with IIH experienced higher all-cause mortality than the general population. Suicide, accidents, and deaths from medical/surgical complications* were the most common underlying causes, accounting for 43%
of all deaths.

Significant Discoveries:

• Over 50% of deaths were attributable to four underlying causes. The top two underlying causes of death were intentional self-harm and accidents (7 deaths each). The third most common cause was complications of medical/surgical care, which accounted for 6 deaths. Heart disease accounted for 5 deaths.
• Compared to the general population,  the risk of suicide was over 6 times greater (SMR, 6.1; 95% CI, 2.9–12.7) and the risk of death from accidental overdose was over 3 times greater (SMR, 3.5; 95% CI, 1.6–7.7).The risk of suicide by overdose was over 15 times greater among the IHR cohort than in the general population (SMR, 15.3; 95% CI, 6.4–36.7)
• Participants of the IHR with IIH had a risk of all-cause mortality that was 50% higher than age- and sex-matched members of the general population.
• When compared to age- and sex-matched members of the general population, participants of the IHR with IIH possess significantly elevated risks of all-cause mortality, suicide, and accidental overdose. Taken together, suicide, accidental deaths, and death from complications of medical/surgical treatments accounted for 4 of every 10 deaths in this cohort.

Although IIH itself is not fatal, the elevated risk of death from suicide and accidental overdose among individuals with IIH is a characteristic often observed with chronic disorders that possess fatal outcomes such as multiple sclerosis, amyotrophic lateral sclerosis, and cancer.

Likely explanations for why IIH can lead to suicide and accidental overdose include the intractable nature of the disorder, elevated rates of mental illness, and the medical complexity of many IIH patients.

And while PTSD was only significantly associated with accidental overdose, elevated rates of suicidal ideation, as well as mental illnesses such as depression and bipolar disorder, present as modifiable factors, which may be targeted to reduce the number of deaths among IIH patients in the future.

The development of novel treatments designed specifically for treating IH, rather than the secondary use of existing treatments for other disorders, would also be a major step forward in resolving many of the debilitating signs and symptoms of IIH. Until then, health care professionals should consider mortality as a potential outcome of treatment failure.

*The researchers were not aware of any deaths within this cohort from complications of bariatric surgery, cerebral venous sinus stents, or optic nerve sheath decompressions.

Read the study abstract

IH Registry Study on Emergency Department Usage by IIH Patients

IH Registry Study Examines Emergency Department Usage by IIH Patients

An IH Registry study examining emergency department (EDs) usage by previously diagnosed idiopathic intracranial patients has been published in the International Journal of Health Care Quality Assurance.  Since IIH can be a debilitating disorder that is difficult to identify and treat, a failure to manage IIH symptoms may force patients to present at Eds seeking symptom relief.

The researchers sought to characterize ED use by previously diagnosed IIH patients, a subject that has received little attention but is important to all IH patients, physicians, researchers, administrators, health policy officials and others .

This study includes information from 332 individuals with IIH enrolled in the IHR, and analyzed a 3-year period to understand the influence that untreated/undertreated symptoms and socioeconomic factors have on the use of ED facilities.

The researchers discovered socioeconomic and health insurance factors play an important role in ED utilization: respondents who were non-white, lived in households with incomes below $25,000 and were insured by Medicare or Medicaid were not only more likely to use the ED, but also used it much more frequently than those in higher socioeconomic households

Additionally, among the IIH participants in this study, 39 percent utilized emergency services during the three-year study period and averaged nine visits.

Headaches were reported as a reason for seeking ED treatment in 90 percent of the ED visits for which this information was reported, while blurred vision was a reason given for 43 percent of such visits to the ED. CSF shunting procedures and complications with such procedures were additional factors associated with ED utilization. Over half of respondents who had received a CSF diversional shunt at some points used the ED, which may suggest insufficient symptom control or an adverse reaction.

In general, IIH patients seeking care in the ED found it to be inadequate or unsatisfactory. IIH symptom management is complex and only 21 percent of patients utilizing the ED reported that ED staff was familiar with IH or IH treatment, and only 36 percent felt that their symptoms were taken seriously.

Often, the only services ED staff can offer IIH patients are a lumbar puncture in selected circumstances to temporarily decrease the CSF pressure, narcotics for pain, or referral based on findings and general lack of response to treatment. Over 60 percent of respondents reported that they received a narcotic from ED staff; however, approximately half of respondents felt that they were perceived as being drug seekers.

Addressing the issues of healthcare access and effective symptom management in private healthcare settings may reduce ED utilization among IIH patients. Importantly, future research efforts should lead to more effective clinical treatment plans, both medical and surgical, that can better accomplish improved CSF pressure control.

Ultimately improved pressure control reduces pain management and increases preservation of vision, which should in turn significantly decrease the need for IIH patients to seek ED treatment.

Read the entire study (free)

Diamox Sequels Update

Diamox Sequels (Brand) Update: List of Generic Manufacturers

Many of you have contacted IHRF regarding the discontinuation of brand Diamox Sequels 500 mg extended release (ER) capsules, manufactured by Teva Pharmaceuticals.  There are four pharmaceutical companies that manufacture generic acetazolamide extended release 500mg capsules.  They are:

• Ingenus Pharmaceuticals http://www.ingenus.com
• Heritage Pharmaceuticals http://www.heritagepharma.com
• Nostrum Pharmaceuticals http://www.nostrumpharma.com/contact-us.aspx
• Zydus Pharmaceuticals http://www.zydususa.com

To the best of our knowledge, these are the only companies in the U.S. and possibly overseas that manufacture generic acetazolamide extended release 500mg capsules (if you know of others, please let us know and we will add them to the list). Most pharmacies can order any of these generics, if they do not carry it regularly in stock. Sometimes, it is helpful to try another generic if one is not working, which can be discussed with and overseen by your doctor.

IH Registry and Pediatric Intracranial Hypertension

IH Registry Study on Pediatric Intracranial Hypertension published in Pediatric Neurology

Children represent one of the fastest growing groups of IH patients, yet there has been relatively little research on this group. A new IH Registry study focusing on pediatric intracranial hypertension was recently published in the journal Pediatric Neurology. Researchers at the IH Registry and at Nationwide Childrens’ Hospital in Columbus, Ohio examined different aspects of how the disorder affected both pre-pubertal and post-pubertal children.

The study’s goal was to examine the presenting symptoms, demographics, and interventions in pediatric patients enrolled in the Intracranial Hypertension Registry. Among other discoveries, the researchers found significantly higher rates of obesity in postpubertal females with primary intracranial hypertension. The female-to-male ratios in the postpubertal primary intracranial hypertension and secondary intracranial hypertension groups were also higher than reported in the medical literature.

A total of 203 IH Registry patients—the largest group of pediatric patients examined in such a study—met the criteria for inclusion; 142 (70%) were considered primary (i.e.idiopathic) intracranial hypertension. All patients were 18 years old or less when first diagnosed. Females made up 72.5% (103 of 142) and 75.8% (47 of 61) in the primary intracranial hypertension (PIH) and secondary intracranial hypertension (SIH) groups, respectively.  When divided into pre- and postpubertal status, 32.5% of patients were classified prepubertal; 77.3% of these had primary intracranial hypertension. This resulted in a female to male ratio of 1:1.04 for prepubertal and 6:1 for post pubertal primary intracranial hypertension patients. The body mass index was significantly higher in the postpubertal primary intracranial hypertension group (P = 0.0014). There was no significant difference in opening pressure.

Some of the study’s other discoveries:

•Symptoms most often reported were headache and blurred vision in both PIH and SIH groups.

•Tinnitus was reported in 44.3% of PIH patients and 49.1% of SIH patients in the Registry’s cohort. Tinnitus has been previously estimated at 10% of pediatric PIH patients.

•Bilateral disc edema (papilledema) occurred in 89.3% of the primary IH patients, and in 78.7% of secondary intracranial hypertension patients. 

•The most common cause of SIH among the patients studied was the tetracycline drug family, which has long been associated with increased intracranial hypertension.

•The BMI was higher in the postpubertal group at 30.7 compared with 21.6 in the prepubertal group.

•SIH patients with an opening pressure of less than 28 cm of H20 were less likely to have optic nerve edema when compared with those with 28 cm of H20 (66.7% versus 84.1%). PIH and SIH patients with opening pressure less than 28 cm of H20 were less likely to have a CN VI (seventh cranial nerve) palsy. This finding suggests that there may be a yet unclear link between the degree of increased pressure and development of the palsy.

•SIH patients (62.3%, 38 of 61) were more likely than PIH patients (33.8%, 48 of 142) to have surgical interventions.

Additionally, the common symptoms of intracranial hypertension, including headache, optic disc edema, and vision changes, occurred with similar frequencies in the Registry’s cohort to those reported in the medical literature.

For more information about this study, please read the PubMed abstract.

Focus on Papilledema

Is it always present in intracranial hypertension?

Papilledema, or swelling of the optic nerves, is often considered a hallmark sign of intracranial hypertension. But what does your doctor see when he looks into your eyes? Read on to find out what papilledema is, why it develops, what a venous pulsation is and whether papilledema is necessary for an IH diagnosis.

How does papilledema occur?
The optic nerve is actually an extension of brain tissue, connecting the inner lining of the retina from the eye to the visual cortex in the brain. The nerve is comprised of axons, or fibers of nerves cells, that carry signals from light sensitive cells in the retina to the visual cortex where the signals are converted into formed images.

The drawing below depicts a cross-sectional view of the optic nerve and its three covering membranes, which are the same membranes that envelop the brain. The first and innermost membrane, which is in intimate contact with both the brain surface and the optic nerve, is called the pia mater (shown in pink). The second membrane is known as the arachnoid  (yellow). The outermost and third membrane is the dura mater (purple).

Between the arachnoid and the pia mater is a space known as the subarachnoid space, which surrounds the brain, as well. The subarachnoid space surrounding the brain is normally filled with cerebrospinal fluid (CSF).  The subarachnoid space around the optic nerve is not normally filled with CSF, but with small fibrous strands. However, when intracranial pressure is high, this area—the subarachnoid space surrounding the optic nerve—can become inflated, as pressurized CSF fills it.

As a result, high intracranial pressure will compress the optic nerve and the central retinal vein (not shown) which travels along with the central retinal artery through the middle of the optic nerve and normally drains blood from the inside of the eye. This compression of the central retinal vein and the optic nerve is believed to be the cause of papilledema. 

Compression of the central vein also causes internal venous pressure to rise. Moreover, normal arterial compressions transmitted from the central retinal artery to the adjacent central retinal vein—which are known as venous pulsations and are seen in approximately 80% of normal patients without IH—disappear. Doctors often consider an absence of venous pulsations at the optic nerve head as a sign of IH.

Is it possible to have IH without papilledema?
While many physicians believe papilledema must be present in order to confirm an IH diagnosis, others argue that papilledema does not always have to be present. In some instances, a patient with intracranial hypertension may not show papilledema because of a time lag between the initial rise in cerebrospinal fluid (CSF) pressure and the development of papilledema. Therefore, at the time of diagnosis, papilledema is not seen.

Another theory is that the actual increase in CSF pressure may produce headache but is not high enough to produce papilledema.

Anatomy may also play a role. Variations in the micro-anatomy of the optic nerve can restrict CSF and prevent it from entering the optic nerve’s subarachnoid space. Or CSF pressure may be directed to a point of lesser resistance, such as the sella turcica (pituitary gland fossa) which then produces the appearance of an empty sella on imaging studies.

Other evidence of intracranial hypertension without papilledema is found in the fact that it’s possible to have asymmetric papilledema, in which the optic nerve swelling is either worse in one eye or only develops in one eye, rather than both.

To Learn More:
For more information on papilledema, please see:

Wraige E, Chandler C, Pohl KR. Idiopathic intracranial hypertension: is papilloedema inevitable? Arch Dis Child. 2002 Sep; 87(3):223-4.

Marcelis J, Silberstein SD. Idiopathic intracranial hypertension without papilledema.
Arch Neurol. 1991 Apr; 48(4):392-9.

Brazis PW, Lee AG. Elevated intracranial pressure and pseudotumor cerebri.
Curr Opin Ophthalmol. 1998 Dec; 9(6):27-32. Review.

IH Registry Volunteers

IH Registry Volunteers: Commitment in Action

What do the battlefields of Afghanistan and the rural countryside of El Salvador and Guatemala have in common? If you ask Evan Foulk, he’ll tell you that’s where he got the chance to learn medical skills that he is now honing. Born in Monmouth, Oregon, and raised on a hazelnut farm, he served five years in the Army as a Special Forces Medical Sergeant. A retired member ofthe “A Team,” Evan is now a first-year medical student at Oregon Health & Science University in Portland.

In between, he spent 16 months and about 450 hours as an IH Registry volunteer, requesting records, performing data entry, calling patients and working on his own research project.
“I was already set on my path to go to medical school, but my time at the IH Registry definitely reaffirmed that decision… [The experience] showed me some of the struggles people go through trying to understand their situation and get help. Seeing that will make me more aware as a physician and [better] able to advocate on behalf of my patients.”

Evan represents the type of individual you’ll find at the IH Registry, IHRF’s joint research project with OHSU and the only global chronic IH patient registry of its kind: they are caring, committed volunteers and research coordinators whose daily efforts literally make IH research possible.

Some of the nine current volunteers are pre-med or post graduate students with public health and medical interests, like Evan, who come from local or out of state universities (including Portland State University, Reed College, University of Portland, Stanford Univeresity, University of Washington, et al.) Others are volunteers from within the OHSU community or from the federal program Experience Works, designed to help seniors become job ready. Some, like Ralpha, a retired elementary school principal, chief volunteer mentor and an IH patient, who has been volunteering for more than a decade—have a personal connection to IH, but most do not.

Yet, all share a common desire to help improve the lives of people struggling with chronic IH
.
Carin, the full-time IH Registry Research Coordinator, who recently completed her Master’s degree in Public Health, knows the vital importance of volunteers. “Volunteers are largely responsible for patient recruitment. Often, a volunteer will spend several hours a day sending reminder emails, a task that is absolutely necessary for growing the Registry and achieving Registry research goals.”

And the fruits of their labor?

With every published study using IH Registry data, IH gains prominence. A study on the emergency department (ED) use by IH patients attracts the attention of ED physicians and hospital staff. A study on the cost of treatment and management of IH captures the interest of economists and drug companies. Publishing data in the NIH Global Rare Disease Registry (GRDR)—the IH Registry was among the first patient registries selected to join the GRDR—draws physicians and researchers from the U.S. and around the world, who are examining similar or associated conditions.

Recently, we’ve learned that a new study on Diamox and kidney stones, which grew out of the IH Registry’s continuing collaboration with NASA, has been accepted in the

.

Volunteers at the IH Registry—who give hundreds, even thousands of hours of their time—gain real world research experience. They encounter the difficulties and rewards of data collection; can be involved in ongoing research projects, and have the opportunity to network with researchers around the world. This is how ideas germinate, discoveries are made, and new treatments and a cure are found.

Sometimes, though, listening to a difficult story is just as important. “…Patients express gratefulness for my simple acknowledgement that they are part of an important population that deserves the development of new treatment and management options,” said Carin.

Help us ensure that the crucial research of the 100% donor-funded IH Registry continues, and that today’s volunteers and registry research coordinators become tomorrow’s IH researchers and physicians. To make your tax-deductible gift today, please click on the button below: