Don't laugh at me

Don't laugh at me (Mark Wills)

Dedicated to my long-time friend Shirley Forry for providing me with the inspiration for this blog.

I. The emotional toll of hydrocephalus

As I prepare to leave for Washington, D.C. on September 11th, I have been challenged by the Hydrocephalus Association (HA) to update my hydrocephalus education and awareness blog as often as possible. Having survived the condition unshunted for 53+ years, I have no shortage of information that I can share, but I wanted to do at least one blog that looks at the emotional toll it takes on those affected by the condition. For most of us - myself included - that would be the cruel and hurtful remarks made by some people. Why is this? Simply put, we live in a society that promotes a narrow standard for attractiveness. Take the images at the top-right and mid-left side of this blog. Most people (especially other children) would look at the image of the me and go "eww". By comparison, the would look at the image of the "normal child" and say nothing.  Why is this? According to one author it's because "Digitally altered faces and bodies - beautiful, yet unrealistic - become imprinted in our minds as normative. Anyone outside the bell curve is a potential victim of bullying".

II. How do people with hydrocephalus look?

Perhaps the most noticeable characteristic of people with hydrocephalus (or, at least, those without a shunt) is a larger than normal skull. Take my head for example, it is 9 7/8 inches in circumference. Over the years - I am 53 now - I have been made fun of, teased, and even told that I was "evil" because of how my head looks. The sad part is that it doesn't look any different than an "average" head, just larger. In addition to a larger-than-normal head, many children with hydrocephalus also suffer from "sun-setting" eyes. This condition involves an upward gaze paresis (weakness) while the eye itself appears driven down. It occurs in approximately forty percent (40%) of children with obstructive hydrocephalus and thirteen percent (13%) of children with shunt dysfunction. Although it doesn't have a direct correlation, many people have seen my head and assume that I am developmentally delayed (I will not use the "R" word). According to HA, while developmental disabilities are a possibility, the more frequent problem is the development of social skills -- something I have never had a problem with.

III. Conclusion

What can be done for what amounts to bullying? Parents need to teach their children that it is imperative that they treat ALL people with both respect and kindness. They should learn that so-called norms depend upon context and that they will vary from one family to another and, on a larger scale, from one culture to another and ARE NOT based on the air-brushed ideals propagated by the media. Even more importantly it is incumbent upon us, as adults, to remind children that human beings everywhere are interesting and memorable because of - not in spite of - their unique real features.

For additional information: About hydrocephalus, Social skill development in children with hydrocephalus, Sunset eye sign  Teased about your looks? Bullying that goes under the radar

Does it hurt? Hydrocephalus and the headaches associated with it (Part 2 of 2)

III. Why are hydrocephalus-related headaches difficult to diagnose and treat

According to Dr. Rick Abbott (Beth Israel Hospital), Dr. Fred Epstein, and Dr. Jeffrey H. Wisoff (New York University Medical Center) the difficulty in diagnosing and treating headaches associated with hydrocephalus is due to the fact that, in many cases, there has been no change in ventricular size and the headache(s) are more chronic, non-progressive nature. This can be the result of intracranial hypotension (negative pressure within the brain cavity often seen following a shunt implant) or intracranial hypertension (positive (or elevated pressure within the brain cavity). In such cases, medical professionals recommend the use of Intracranial pressure (ICP) monitoring where the patient is hospitalized for 24 - 48 hours and the ICP is continuously monitored. During this time he/she is alert and active so that the pressure(s) recorded is relative to both body position as well as activity. If pressure changes can be correlated with the patient's symptomatology, the shunt can be revised to either a higher or lower pressure valve.

IV. Conclusion

As I have stated in previous blogs, hydrocephalus is not a disease (emphasis added), it is caused by the brain reacting to a blockage. Currently placement of a shunt device is the only way to control a blockage. It should be noted, however, that approximately fifty percent (50%) of those treated with a shunt will require a revision or revisions during their lifetime.

Normal pressure hydrocephalus: the "grown-up"version of an under-recognized problem (Part 2 of 3)

In part one of "Normal pressure hydrocephalus: the "grown-up" version of an under-recognized problem" we looked at what normal pressure hydrocephalus or NPH is and what symptoms are experienced. In part two, we will look at what causes it, how it is diagnosed, and what treatment options are available.

III. What causes normal pressure hydrocephalus?

In the majority of normal pressure hydrocephalus (NPH) cases are classified as idiopathic meaning that it occurs spontaneously or that the exact cause is unknown. It should be noted, however, that there are known causes for NPH including: head injury, cranial surgery, subarachnoid hemorrhage, tumor, or cyst. Additionally, it can result from subdural hematoma, bleeding that occurs during surgery, or meningitis. It is interesting to note that all of the causes outlined above can predispose the patient to inflammation (which affect the cerebrospinal fluid [CSF] pathways) which impedes the CSF flow.

IV. How is NPH diagnosed?

In many cases, it is often the affected person or close family member who first brings the symptoms of NPH to the attention of a doctor. Additionally, there have been cases where the enlarged ventricles have been found on a brain image (such as a CT scan) that is performed for a totally different reason. If a physician has reason to suspect his/her patient has NPH, the doctor can order a variety of test to confirm this diagnosis.

Once NPH is suspected by the affected person's physician, it is imperative that a neurologist, neurosurgeon, or neuropsychologist be brought on board. Their involvement from the diagnosis stage onward is helpful not only from the standpoint of interpreting test results, but also in discussing the surgical option (if indicated), potential side effects and follow-up care.

As the diagnostic process moves forward, the decision to order a specific test might hinge on the given clinical situation. These might include, but are not limited to:

Clinical exam to evaluate symptoms consists of an interview (with the patient and their family) and/or a physical/neurological exam. (Hyperlink goes to a YouTube video.)

It is important to note the presence of the Hakim triad of symptoms is not necessary to confirm a diagnosis of NPH. The medical team will consider both the pattern and severity of impairments (combined with the results of other tests outlined below) to differentiate NPH from other possible outcomes.

CSF tests to predict shunt responsiveness and/or determine shunt pressure include lumbar puncture ("spinal tap"), external lumbar drainage, measurement of CSF outflow resistance, intracranial pressure (ICP) monitoring, and isotopic cisternography (pictured at left).

Lumbar puncture allows for an estimation of both the CSF pressure as well as analysis of the fluid. Performed under a local anesthetic, a thin needle is passed into the spinal fluid space of the lower back. The doctor then removes up to one (1) cubic centimeter (cc) of CSF are removed to see if a reduction in CSF volume relieves symptoms being experienced by the patient.

External lumbar drainage (also known as continuous lumbar drainage) is a variation of the lumbar puncture where a flexible catheter is left in place to facilitate drainage of the CSF. It allows for either continuous or intermittent drainage of CSF over several days to emulate the effect a shunt would have. It also allows for a more accurate reading of CSF pressure as opposed to the "moment in time" measurement provided by the lumbar puncture.

For intracranial pressure (ICP) monitoring or spinal pressure monitoring a small pressure monitor is inserted through the skull into one of three places: 1) The brain itself; 2) The ventricles of the brain; or 3) The lumbar region to monitor ICP. It is utilized to detect an abnormal pattern of pressure waves as well as either low or high pressure. The results from this testing can be utilized to select an initial pressure setting if a shunt is being implanted.

Isotopic cisternography is no longer frequently used because a "positive" cisternography result does not reliably predict whether the patient will respond to implant of a shunt and the (cisternography) results are often ambiguous.

For additional information: NPH booklet

Does it hurt? Hydrocephalus and the headaches associated with it (Part 1 of 2)

The other evening I was looking at what I had written about so far and thinking of additional topics when a long-time friend suggested "anything but the typical 'does it hurt' '" that she and I have heard too much as we grew up with a disability and even now as we are in our adult years. Following her comment, I began looking and came up with the idea of doing a blog on hydrocephalus and the associated headaches suffered by some of us.

I. Mechanics of hydrocephalus and headaches

The combination of brain volume (volume for the average adult is 1,260 cubic centimeters), blood volume, and cerebrospinal fluid (CSF) volume determine intracranial pressure or ICP. If one of these increases, pressure in the brain will rise unless one of the others compensates by decreasing. In a person - such as myself - with hydrocephalus this balance is distorted and a unnatural condition takes place. In a person not affected by hydrocephalus, they should have one ounce (1 oz.) of CSF in the ventricles and about four ounces (4 oz.) of CSF surrounding the outside of the brain. When the components of the brain are functioning normally - without a shunt - the brain has the ability to be elastic. This means it is able to compensate accordingly for an increase (or decrease in CSF pressure). In hydrocephalus patient's who have been shunted, this is no longer the case.

Physiology of hydrocephalus

In studying to present this blog, I learned something that I had never heard before and that is that when our bodies enter rapid eye movement (REM) sleep (typically about 80 - 90 minutes after falling alseep) plateau waves occur resulting in increased intracranial pressure (ICP). In shunted patients who undergo ICP monitoring overnight, those changes are considerably more drastic that with the average person. Those things that would normally be expected to cause an increase in ICP result in huge changes in a shunted patient.

Placement of a shunt results in an unnatural situation. The brain fills the intracranial space while the shunt drains essentially of the available CSF from the ventricles.The end result is a larger-than-normal brain in a fixed (NOT elastic) skull with little room for changes in the ICP (which is characteristic of a person with hydrocephalus. If changes in blood flow occur, resulting in blood volume within the intracranial space, increased intracranial pressure will occur and can result in a headache.

Headaches can also be the result of altered pressures within the skull once a shunt has been implanted. Interestingly, the can occur both if the pressure is too high or too low.

II. Treatment

Like everyone else, both children and adults with hydrocephalus experience periodic headaches. It is the frequency and severity of these that determine whether they are a result of the hydrocephalus. According to Dr. Gordon McComb, Chief of Neurosurgery, Children's Hospital Los Angeles, "If the headaches are getting progressively worse, many times it's an intermittent malfunction of the shunt". Dr. McComb explains that ventricle size is also not always an accurate indication of whether the shunt is working correctly. "The ventricles are going to remain the exact same size whether the pressure is normal or elevated".

The slit ventricle is the white streak
visible pointing toward the
left side of the brain

With slit ventricle syndrome, small ventricle size is not the cause of the problem. The problem occurs if the shunt becomes clogged and the ventricles don't dilate. If this situation happens repeatedly (accompanied by acceleration in the occurrence of headaches) it can indicate a blockage the builds up and then releases. Replacing the proximal ventricular catheter in the shunt will normally correct this problem.

When hydrocephalus problems experience repeated headaches it is imperative to sketch out a plan of action which involves a conference between the patient and his/her doctor(s). This conference sets up treatment parameters and, if those parameters are exceeded, the medical team moves forward with the replacement of the shunt.




For additional information: Hydrocephalus and headaches

Normal pressure hydrocephalus: the "grown-up"version of an underrecognized problem (Part 1 of 3)

Before I go any further into my topic, I will ask my reader's to please not take offense at the illustration above. It is a light-hearted way to explain the effects of a condition that affects an estimated 700,000 Americans and often goes undiagnosed and untreated. According to the Hydrocephalus Association, an estimated 80% of cases remain unrecognized by the medical community.

I. What is normal pressure hydrocephalus (NPH)?

Many people aren't family with the term Hydrocephalus but, if you refer to it as "water on the brain" they immediately know what you are referring to. In older adults it manifests itself as Normal pressure hydrocephalus or NPH. The term "water" is a misnomer, however, because the substance that creates the problem is cerebrospinal fluid (CSF). As the CSF builds up, it can cause dangerously high intracranial pressure (ICP) and affect how the brain functions. There are a number of factors that can cause NPH including: head injury, surgery, or a brain bleed (hemorrhage).

II. What are the symptoms of NPH?

The term Normal pressure hydrocephalus was first coined by Dr. Salomon Hakim (pictured at right) in his 1964 paper which spoke of a type of hydrocephalus where there is little or no increase in the ICP and occurred primarily in older adults. One thing that made NPH so dramatically different from other forms of hydrocephalus was what Dr. Hakim referred to as the triad of symptoms. These are: 

Gait disturbances which can range from a mild imbalance to a total inability to stand or walk at all. Often described as a "magnetic gait" characterized by wide-based, short steps with a noticeable shuffle. Additionally, persons with NPH might have problems picking up their feet making the navigation of curbs and stairs difficult and placing them at a higher risk for falls.

Mild dementia can be described as the loss of interest in activities, becoming forgetful, difficulty performing daily routine tasks, and short-term memory loss. Typically these symptoms are less severe than those seen with full-blown dementia and are often overlooked for years or dismissed as a consequence of aging. Persons with NPH usually do not  (emphasis added) lose language skill, but their deficits might not be as obvious to them and they might deny there is even a problem.

Impairment of bladder control is usually characterized by both urinary frequency and urgency, however, the complete loss of bladder control (urinary incontinence) can occur in more severe cases. As a point of education, urinary frequency is a need to urinate more frequently -- sometimes as much as every one to two hours. Urinary urgency is the strong, immediate sensation of the next to urinate. In some cases, this urge is so strong that it cannot be held back resulting in incontinence. In very rare cases, fecal incontinence can also occur.

For additional information: Hydrocephalus facts and stats, NPH article NPH booklet

Shunting as a means to manage hydrocephalus

I. Overview

Subarachnoid spaces in the brain

I. Overview

Over the years, the management of hydrocephalus has challenged neurologist, engineers, and medical device manufacturer alike due to the unique nature of cerebrospinal fluid (CSF) dynamics in each person. Known as a "CSF diversion device", a shunt has become the primary therapy used in hydrocephalus management for over sixty (60) years. The shunt, which is surgically implanted within a ventricle in the patient's brain (or in the subarachnoid spaces around the brain), works by diverting the CSF to another part of the body where it is absorbed. This creation of an alternate pathway typically restores the physiological balance between CSF production, flow, and absorption when one or more of these functions has been impaired. Once it has been inserted, valves within the shunt's pathway act like on/off switches, opening when the differential pressure, that is, the pressure difference across the valve, exceeds the valve's opening pressure.

Blog author Walter Little with his
grandparents, James and Sara Sanders,
and Officer Dorsey Goss (1973)

II. What are the complications of shunting?

As I alluded to above, a shunt provides an alternate pathway through which CSF can bypass obstruction(s) in the fluid compartments (ventricles) of the brain. Such a bypass relieves the excess fluid backup the causes hydrocephalus. When both the CSF production and absorption are in balance, the hydrocephalus is considered to be "compensated". In contrast, when production exceeds aborption, complications such as elevated pressure or overdrainage occur and can mimic a malfunctioning shunt.

More detailed information can be found on the Hydrocephalus Association website, but here is a brief overview of some of the most common complications:

Malfunction

As is the case with any piece of equipment, a shunt might break. Additional they are subject to becoming disconnected, migrating (moving), or, most commonly, becoming blocked. (It should be noted that the disconnection and migration are particularly prevalent in children with a shunt due to the child's growth.)

Infection

Shunts can become colonized with bacteria or - in rare instances - fungi which typically occurs at the time the shunt is implanted. In an effort to combat this, some manufacturers have began to add anti-microbial coating to their shunts which appears to be reducing the rate of post-surgical infection.

Material degradation

Originally, barium sulfate (BaSO4) was mixed with silicone to allow the shunt catheter to be visible on an x-ray. These would eventually dissolve making the tubing surface rough. When tissue in-growth occurred to the tubing, it would bind at that location resulting in deterioration and/or breakage. Over time the design of shunt tubing has changed and a clear silicone elastomer now covers the surface of the tubing greatly decreasing the likelihood of degradation.

 Shunt revision(s)

I put an "s" on revision because the average person with hydrocephalus undergoes 2.66 shunt revisions over the course of their lifetime. These can be required at any time to correct one (or more) of the complications outlined above or to compensate for growth in the case of children and young adults. If a blockage is suspected, it must be confirmed by a neurosurgeon who evaluates the implanted system to determine whether the problem is the result of a complete or partial blockage, if a disconnection has occurred, or whether the current system just can't no longer meet the needs of the individual.

Additional information:

Shunt system fact sheet

What is hydrocephalus? (Part 2 of 2)

In part one of "What is hydrocephalus?" I discussed what it is and what causes it, today I will look at how it effects those with the condition (and how it is treated) as well as what is being done to combat it.

III. What are the effects of hydrocephalus and how is it treated?

What effect does hydrocephalus have on the body? The most notable is an increase in pressure -- not only in the Cerebrospinal Fluid (CSF), but also by the heart which has to work harder in order to deliver blood to the brain. If the CSF pressure continues to rise, it will eventually interfere with blood supply to the brain thus depriving it of both oxygen and glucose which is needed in constant amounts in order to maintain functionality of the brain. Initially this results in tiredness, irritability, and drowsiness; left untreated, however, the patient soon looses conciousness as the brain begins to shut down.

Ventricular tap

The immediate effects of this interference with blood supply will disappear if CSF pressure is returned to normal either by means of insertion of a ventricular tap (pictured at right) or insertion of a shunt (pictured at right). In most cases, however, the diminished blood supply has been going on unnoticed for sometime before a diagnosis of hydrocephalus is made. This results in a "dying back" of the very fine blood vessels located in the brain. Without treatment the "dying back" continues resulting in the progressive damage to nerve cells in the brain which will result in their eventual destruction.

Currently, there is no known way (emphasis added) to either prevent or cure hydrocephalus. As I alluded to yesterday, the most effective treatment involves the surgical insertion of a shunt such as the ventricular-peritoneal pictured above. The shunt is flexible tube (typically made of silicone or similar material) inserted into the ventricular system of the brain to divert the flow of  into another region of the body such as the peritoneal cavity which is located in the abdominal wall. A valve within the shunt maintains CSF at a normal pressure within the ventricles.

IV. What is being done to combat hydrocephalus?

As much as it pains me to say this, not much. Despite the fact that it is a MAJOR (emphasis added) public health burden affecting an estimated 1,000,000 people, very little public (or private) investment is made into understanding hydrocephalus or improving the outcome for those affected by the condition. 

For additional information:


The effects of hydrocephalus
Shunt fact sheet

What is hydrocephalus? (Part 1 of 2)

Countdown to Washington: 3 weeks and four (4) days

I. What is hydrocephalus?

Taken shortly after my birth,
my hydrocephalus was clearly evident

After completing my latest blog last night, it occurred to me that some might not know and/or understand what hydrocephalus is. It is a condition caused by an abnormal accumulation of cerebrospinal fluid (CSF) in the ventricles (pictured above) of the brain.  The word comes from the Greek hydro (meaning "water") and cephalus (meaning "head). Often, the term "water head" is used in a derogatory sense to make fun of a person suffering from the condition.

Under normal conditions the CSF - which is produced in the ventricles - circulates through the ventricular system before being absorbed into the blood stream. It is constantly being circulated and serves many functions in ensuring the health of both the brain and the spinal cord. Perhaps one of its most important functions is to surround both the brain and spinal cord and serve as a cushion against injury. In persons with hydrocephalus (or "hydro" for short) there is an imbalance between the amount of CSF produced and the rate at which it is absorbed into the blood stream. As it builds up within the ventricles, it causes them to enlarge and the pressure to increase.

II. What causes hydrocephalus?

First of all, it's important to differentiate that hydrocephalus is a condition and not a disease  (emphasis added) as I have heard people mistakenly say over the years. It can develop for a variety of reasons including as a part of another condition.

Hydrocephalus that is congenital (meaning that it is present at birth as mine was) is thought to be the result of a complex interaction of both genetic and environmental factors. One of the most common causes of hydrocephalus is Aqueductal stenosis where there is a blockage of the cerebral aqueduct known as the Aqueduct of Sylvius.  It is estimated that hydrocephalus occurs in 1.5 per 1,000 births.

In addition to congenital hydrocephalus, it can also be acquired as the result of intraventricular hemorrhage, meningitis, head trauma, a tumor, or a cyst.

Yet a third cause of hydrocephalus - known as normal pressure hydrocephalus or NPH - is prevalent in older adults. Unlike congenital or acquired hydrocephalus, NPH causes little or no appreciable increase in pressure within the ventricles.

For additional information: Hydrocephalus fact sheet

Mr. Little goes to Washington

The nation's capital at dawn

Countdown to Washington: 3 weeks and five (5) days

As one of my favorite expressions says "this isn't my first rodeo" as far as visiting Washington, but it will be my first time going to represent a cause that effects so many lives. As I alluded to in my last blog, hydrocephalus occurs in one to two births out of every 1,000 making it as prevalent as Down syndrome and more common than spina bifida or brain tumors combined. Interestingly, according to an article I found on Medscape, hydrocephalus occurs in an estimated 15 - 25 percent of children with open myelomeningocele (a form of spina bifida) at birth. Of those, 80 - 90 percent of those will need surgery to implant a shunt.

As you read this, you might ask what good going to Washington will do. Currently, there is no cure for hydrocephalus only shunting which decreases cranial size by allowing the excess Cerebrospinal Fluid (CSF) to drain from the skull. Done at birth, shunt surgery can give a child with hydrocephalus a near-normal size head. In going to Washington for the rally (which is a yearly event) I and others "give a face" to a condition that many of the elected officials have probably never heard of. But, as my family has reminded me, it's bigger than that because I am representing everyone with disability and proving that we can be productive citizens that give back to our community.

A conversation starter

For those who might not have seen my picture, this is me. I have always been told I "view the world differently" and the way I see my hydrocephalus is the perfect example. Over my 53 years, I have never seen it as something to be ashamed of, but something I was proud of AND a great conversation starter. I have lost track of the number of times I have sat down in a restaurant, at the bus stop, etc. and the person next would ask what caused it. I love to talk about my "hydro" and help people to understand not only what causes it, but also that it doesn't automatically make a person retarded. (I add the latter at some point in my discussion because there have been more people than I can count who assumed that it made me a real-life version of "Baby Huey".)

As I was just telling a fellow hydrocephalus survivor, we have to use such experiences as a teachable moment. Over the years I have explained to people that it IS NOT (emphasis added) like a contagious disease that can be passed from one person to another or that it DOES NOT (emphasis added) an evil or sinister person. Another one that was related to me by my grandparents were comments made to them when I was growing up about "God must be punishing you for [fill in the blank] sin". No, it's not a punishment either; it is a congenital birth defect that caused the ventricles in my brain to not properly develop.

Another way that I am different as far as my perspective of my hydrocephalus is that I welcome people who want to touch my head. For the most part - especially if the person is in the medical field - I know what they are wanting to feel and that's whether I have a shunt (pictured at right). I don't but, again, it opens the pathway to educating them about the condition. Education is key because, according to the Hydrocephalus Association one to two babies in every 1,000 are born with hydrocephalus making it as prevalent as Down syndrome and more common (emphasis added) than either spina bifida or brain tumors. Even so, there is currently no cure for hydrocephalus, so it is imperative that people learn about it and join the bandwagon to find a cure.

2017 Hydrocephalus scholarships awarded

I had planned to post this back on Monday (July 31st) but, due to health issues, I was delayed until today. Earlier in the year, I received an e-mail from the Hydrocephalus Association saying that I was in the pool for one of their 2017 scholarships. Several months passed and I didn't hear anything regarding my application or essays, so I assumed I didn't make the final cut. Well, last Sunday evening (July 30th) I received a telephone call from David Roberts of the Hydrocephalus Association's Atlanta office letting me know that I had indeed been awarded a scholarship and would receive it in the mail within the next two weeks. This came just in time and should "push me over the top" as far as paying for my final quarter at school! (Speaking of, I had been beating myself up for taking longer than four (4) years to finish my degree until I talked with a friend who explained few people actually finish in four years. That being said, let's do this.)

Until next time . . .

Walter