Hydrocephalus and the individualized education program (IEP) (Part 1 of 2)

I. How did it all begin?

Although the legal precedence for inclusion can be traced back to Brown v. Board of Education (347 U.S. 483 [1954]), it was really the parent's of children with a disability that encouraged legislators to adopt the  Education for All Handicapped Children Act (EAHCA) (PL 94-142) in 1975 which required school district's to both include and educate children with special needs as well as to create specialized academic plan (later to be known as an individualized education program or IEP) for them.

Because of its honorable beginnings, it only makes sense that the IEP (sometimes also referred to Individualized Education Plan) process was intended to be centered on the student. That being said, which students qualify for an IEP? Below is a listing of the disabilities or impairments that are eligible for an IEP: Autism, Deaf-blindness, Deafness, Emotional disturbance, Hearing impairment,  Intellectual disability, Multiple disabilities, Orthopedic impairment, Other health impairment, Specific learning disability, Speech or language impairment, Traumatic brain injury and Visual impairment.

II. Having your child / student evaluated for an IEP


For a parent, it is very simple if you believe that your child is qualified to receive special education services. It's just a matter of going to their teacher, principal, or the school's psychologist. Once you have asked about you child being evaluated for an IEP , a group of qualified personnel (including those outlined above) will decide whether or not an evaluation is warranted and create an appropriate plan if an IEP is deemed necessary. From the time the parent gives consent to proceed with the evaluation, the district has sixty (60) days to complete the evaluation. Any decision regarding the IEP evaluation must, by law, include parental input.

It should be noted that a child's teacher can also refer students for evaluation, but only after attempts have been made to remedy the problem without special education services. If such attempts have been made (and the student is continuing to struggle), the next step is initiation of an intervention under RTI (response to intervention) or convening the school's student services team (SST) to discuss their performance. In this meeting, the general education teacher brings samples of the student's work as well as supportive data such as reading scores and math scores, behavioral charts, and writing samples. From this data, the team can decide whether the student would benefit from an evaluation, or if they should continue without special education services.

 If the suspected cause of the problem is the result of a learning or behavioral impairment, the SST can work together to determine what tests need to be done and what data needs to be gathered. This can include a variety of performance-based tests such as Woodcock Johnson, Third Edition (WJIII), or the Wechsler Individual Achievement Test (WIAT). In addition, they might perform cognitive function and behavioral function testing.

Following this testing (and gathering of existing information), the evaluation team will meet again to discuss the results of the tests. At this time, if a decision is made regarding evaluation, the student's parent(s) are invited to participate.

I would be amiss if I did not include the caveat that there are certain diagnosis that neither teachers or school psychologist are not qualified to make. These include Attention Deficit Hyperactivity Disorder (ADHD), Autism (or Autism Spectrum Disorder), as well as most physical and developmental delays require a medical diagnosis. If you are an educator and suspect that you have a student affected by one of these conditions, it is imperative to include a medical professional in the evaluation process.

To sum up this portion of the IEP process, in each category of disability, there must be an impact on the student's education that is directly attributable to the disability and they must be deemed in need of specially designed instruction. After all, not all children with a disability require special education services.

For additional information: Individualized education program (IEP), Summary of PL 94-142

What is slit ventricle syndrome (SVS)?

I. Understanding our ventricular system

brain parenchyma

One error that we make in considering the ventricular system of the human brain is that it is static and that the ventricles do not modify over time. This is not true and evidence of this can be seen even before birth by viewing a prenatal ultrasound where they are relatively small. What is the ventricular system? Simply put, it is a communicating network of cavities filled with cerebrospinal fluid (CSF) located within the brain parenchyma (pictured at right). It is made up of two (2) lateral ventricles, the third ventricle (the site used for an endoscopic third ventriculostomy or ETV), the cerebral aqueduct, and the fourth ventricle. Additionally, the choroid plexuses - responsible for the production of CSF - are located within the ventricular system.

The the downside of this dynamic ability is the fact that the ventricles tend to grow larger as we age because of a decrease in the number of brain cells. The good news about this increase in size (except in extreme cases) is that the actual size of the ventricular system appears to have little affect on the function of the brain.

One interesting fact that I wasn't aware of until I began reading for this blog is the fact that each time our heart beats a shock wave is produced that changes both the shape and size of the ventricles. This dynamic change can be monitored using magnetic resonance imaging (MRI) which pinpoints a specific sequence.

II. What are slit ventricles

Simply put, they are small ventricles (often so small that they are barely visible on either a computerized-axial tomography (CT) scan (pictured at left) or an MRI. They can occur following a severe head injury or a viral infection of the brain. In either case, the brain becomes so swollen that fluid is literally pushed from them.

Another phenomena that is of interest to the medical community is the fact that they are often seen following cerebrospinal fluid (CSF) diversion such as accomplished by a implant of a shunt. Interestingly that condition (decompression of the ventricular system) is one of the benchmarks of a properly functioning shunt system and is confirmed by comparing a post-implant CT or MRI with one done prior to the implant. In some cases, this decrease in the size of the ventricular system results in a "siphoning" effect. How the brain physically reacts to this siphoning is largely dependent on the patient's age. In young children, the brain is still very watery and easily changes shape. Unfortunately this effect is very unpredictable and and very little can be done to affect it one way or the other.

It is important to understand that small / slit ventricles do not always cause symptoms -- some patients are asymptomatic (showing none of the usual symptoms) and go for long periods never knowing they have a problem. Typical symptoms associated with small / slit ventricles include: 1) Headache; 2) Lethargy that can vary in intensity from mild to debilitating; 3) Nausea and vomiting. These symptoms can be intermittent and the headache is often relieved by laying down.

III. What is slit ventricle syndrome

Slit ventricle syndrome (SVS) is a grouping of symptoms which, for example, can occur in a patient in a patient with a functional shunt, but whose brain has lost some of its elasticity. As I mentioned above, they might experience headache, vomiting, and drowsiness / lethargy which, interestingly enough, are the same symptoms experienced when a person with hydrocephalus is having a shunt malfunction. One thing that makes SVS symptoms slightly different is the fact that they are VERY cyclical in nature often with like clockwork. As an example, the patient can be perfectly fine for three (3) weeks and then become violently ill, sleep for 24-hours, and then be "normal" again. Generally, there is no cause for the symptoms experienced, although a minor viral infection has been known to act as a triggering mechanism.

Under normal circumstances, the shunt drains the CSF from the ventricles to another part of the body - such as the peritoneal cavity - for reabsorption. In certain patients, however, this causes the ventricle(s) to collapse. In a person who is shunted this cause the ventricle to collapse on the ventricular catheter blocking it off and preventing the outflow of CSF and causes the appearance of symptoms. Due to the fact that the brain has lost some of it's elasticity (due to the original condition that caused the hydrocephalus) the ventricles are slow to return to their normal size causing symptoms to persist.

IV. How is SVS treated

Once symptoms have manifested themselves, the most important thing to determine is whether the shunt is functioning properly and not having intermittent blockages due to a malfunction within the system. In many cases, this can be as easy as measuring the intracranial pressure (ICP) within the skull. Once the functionality of the shunt is assessed, the next step is to consider a volume expansion procedure such as a subtemporal decompression. This procedure involves removing a small section of bone from the skull which allows the ventricle - and the catheter - to expand and relieves the excess pressure.

Hydrocephalus and college: dreaming the possible dream (Part 2 of 2)

III. Who needs to know (about your hydrocephalus)

First and foremost, you should tell your new roommate(s) about your hydrocephalus simply because they are the one person you will be around the most during your academic career. It's best to inform them about it within the first few days after classes begin so that they will know what to expect and, more importantly, how they can help you in the event a situation arises where you need their assistance. It is also a good rule of thumb to let your resident advisor or guidance counselor about your hydrocephalus because they can guide you about who - if anyone - needs to be aware of your hydrocephalus. As you explain your hydrocephalus to your roommate and/or RA/counselor, make them aware of the signs and symptoms of a shunt malfunction so that they can assess your condition if you're unable to do it for yourself. It is also a good idea to make them aware of where you keep the file folder/notebook with information relevant to your condition. Lastly, ask your roommate or RA/counselor if they would be comfortable accompanying you to the hospital in the event a problem occurs. This will facilitate notification of your family and assure treatment is rendered in a timely manner.

Whether or not you inform your professor(s) about your hydrocephalus is a decision best made once made in the first few days of a new quarter / semester. Even if you have previously registered with your school's disability resource center (DRC), your instructors aren't made aware of your condition unless you decide to tell them.

IV. Tips and advice

Academics


I could write an entire blog about academics and those of us with hydrocephalus but one of the important thing to remember is - while researching schools you wish to attend - is to determine what services they offer for students with a learning disability. If you had an Individualized Education Program (IEP) in place during high school, your team should be meeting with you to assist in negotiating your transition.

Medical experts recommend that the beginning of your college years might also be a good time to schedule a neuropsychological examination. such an exam can pinpoint your learning strengths (and weaknesses) as well as guide you on what classes might be the best tailored to your learning style and ability.

As I stated above, make sure to contact your school's DRC  and let them know that you have hydrocephalus.  The staff in the DRC can assist you with any accommodations you might need such as extra time to complete exams or tutoring services.

Broken tubing in a
ventriculo-peritoneal
shunt

Alcohol

Let's be real college students (for the most part) experiment with alcohol. Although there is no known correlation between being shunted and experiencing an adverse reaction to alcohol, studies have proven that drinking in excess is not good for anyone. This is especially true for those of us with hydrocephalus (and particularly if you are shunted). I say that because the symptoms associated with a hangover (headache, lethargy, nausea, and vomiting) are also seen in the event of a shunt failure. The key difference being the symptoms of a hangover normally subside after several hours; if they persist - and you have a shunt - you should seek medical attention to prevent a potentially life-threatening situation.

Consumption of alcohol can also adversely affect your medications. While some simply loose their effectiveness, others can result in extreme drowsiness and other potentially dangerous side effects. The best rule of thumb is: if you take medication(s), don't drink alcohol.

Headaches

As strange as it sounds, headaches are often times a fact of life for many young people. The combination of deadlines, finals, too much junk food, and irregular sleep patterns can trigger a headache, however, if you are hydrocephalic, such an event could potentially be life-threatening. Even for the most knowledgeable person with hydrocephalus, it can be difficult to differentiate between a regular headache and a headache resulting from a shunt malfunction and/or failure. As a general rule, if the headache improves after taking a couple of Tylenol and resting for a short time, it is not coming from your shunt or as a result of your hydrocephalus. If a headache persist (or you have that "gut feeling" that something is not right) seek medical attention because it's better to be safe than to be sorry.

Additional information: College and hydrocephalus

Hydrocephalus and college: dreaming the possible dream (Part 1 of 2)

Four (4) years ago I embarked on this dream journey and it has been an experience! As a favorite saying of mine goes, I "went into it with eyes open" and knew that it would be rough at times. Fortunately I had what I think are the two critical components to my success: 1) The personal chutzpah that said I would succeed (and that failure IS NOT an option); and 2) The strong support network that swoops in and keeps me encouraged when I am tired and feel like I lack the endurance to keep on. Oh, and one more thing, when I graduated from high school thirty-four (34) years ago I received an award for my combination of academic excellence and personality. When I start to tire, I just pull out the remarks written by my guidance counselor and that re-ignites the flame that pushes me forward.

I. Setting the stage: before you go

When you begin considering an academic career, there are several factors that usually come into play. Here are some issues to consider before getting too deep in the application process:

Location is an important consideration because, at least for some, staying closer to home (and family) is the safer, more comfortable option. For others, going away to college in another city - or state - is an important step on the path toward independence. When that is the case, creating a balance between your desires and your parent's worries is crucial. The key to creating this balance can be achieved by a frank and open dialogue that addresses both.

Special services are an important consideration as you are investigating which college you would prefer to attend. Going in with a knowledge of the services available by the college or university of your choice can prove invaluable later down the road.

II. Medical considerations

Wherever you choose to go to college, it is important to have all of your medical information close at hand and in a safe location be it a file folder, notebook, or a binder. This is imperative because it is one of your first steps toward complete independence. This information should include: 1) Contact information for your all of your doctors (it should include their phone number(s), address, and e-mail address); 2) A copy of your most recent CT scan or MRI; 3) Operative notes as relevant (these will provide insight into the type of shunt you have and its settings as well as if it's programmable or adjustable); 4) Complete insurance information including a photocopy of both the front and back of your insurance card; 5) A listing of all medications (both prescription and non-prescription) that you are currently taking; and 6) Information on all known allergies.

Every young adult - or anyone with hydrocephalus for that matter - should wear either a medical alert bracelet or necklace that clearly states that they have shunted hydrocephalus or hydrocephalus that is treated by an endoscopic third-ventriculostomy (ETV).

For students who opt to attend college away from home, they need to put into place a plan for local care in the event of a emergency. The first step in this process is to have your regular neurosurgeon recommend a neurosurgeon near the college you plan to attend. At the beginning of the school year, arrange for a visit with your newly selected neurosurgeon and make sure he/she has received a copy of your medical records.

Additional information:College and hydrocephalus

What is endoscopic third ventriculostomy (ETV)? (Part 2 of 2)

View of the interior of the brain from the right lateral ventricle

In the first part of this blog, I discussed exactly what an endoscopic third ventriculostomy is, what has brought a renewed interest in the procedure, and who makes a good candidate for it. Today I will look at potential complications as well as how success is measured in patients who undergo an ETV.

IV. What are the potential complications of an ETV

Stereotactically guided endoscopy

Thanks to new technology - such as Magnetic Resonance Imaging (MRI), Stereotactically guided endoscopy (pictured at right), flexible fiber optic scopes, and improved tools for manipulation and hemostasis (stoppage of blood flow) the risks associated with an ETV have been minimized. The advent of high-resolution MRI images now allows the neurosurgeon to clearly perceive the absence of CSF through a stenosed or occluded aqueduct, while neuroendoscopic offer unprecedented views from within the ventricular system itself.

The most common complications associated with an ETV are post-procedure fever and bleeding. The fever is the result of a combination of (a) cold light source and monopolar coagulation in the confined volume of the third ventricle which elevates the temperature of the CSF and can cause a fever. Additionally, attempts to perforate the ventricular floor can lead to bleeding, as can damage to the ventricular wall or perforation of the basilar artery. Large bleeds under the third ventricle can be catastrophic, but are rare.

Short-term memory loss is yet another potential complication associated with ETV. This is due to the fact that the procedure can affect both the hypothalamus as well as areas of the mamillary body which is responsible for memory. Over time, however, the individual usually recovers from any short-term memory loss

V. Measuring success of an ETV

"Success" of an ETV is usually considered (by both patients and doctors alike) to be the avoidance of placement of a shunt in a patient who would have otherwise required one. Medical professionals would qualify the procedure as a "success" if their patient exhibits clinical evidence of normal intracranial pressure (ICP) AND structural evidence of stable or decreased ventricular size. (Emphasis added) In the case of a patient who has previously been shunted, the shunted must have either been removed or proved to be non-functional to demonstrate success.

Conversely, most doctors would consider an ETV as a failure if the patient exhibits no change in their clinical symptoms (or ventricular size) or requires the placement of a shunt within days or months of the ETV placement.

Additional information: Endoscopic third ventriculostomy

What is endoscopic third ventriculostomy (ETV)? (Part 1 of 2)

I. What is an endoscopic third ventriculostomy?

In performing an endoscopic third ventriculostomy or ETV, a neurosurgeon makes a small perforation in the thinned floor of the third ventricle thereby allowing the drainage of cerebrospinal fluid (CSF) out of the blocked ventricular system and into the interpenducular cistern (which is a normal CSF space). This allows CSF within the ventricle in an attempt to bypass an obstruction in the
aqueduct of Sylvius, thereby relieving pressure. The objective of this procedure - technically known as a "Intracranial CSF Diversion" is to normalize pressure on the brain without implanting a shunt. It should be emphasized, however, an ETV is not a cure for hydrocephalus, but, rather, an alternate treatment.

Although open ventriculostomies were performed as early as 1922, they become less common in the

1960's with the advent of shunt systems. Despite the recent improvements in shunting technology and surgical techniques, their use in certain cases remains inadequate. This is due to complications such as blockage, infection, and over-drainage which often require repeated surgeries to permanently correct them. Because of these factors, many neurosurgeons are again recommending an ETV instead of implanting a shunt.

II. New technologies renew interest in ETV

This renewed interest in the use of ETV as an alternative treatment for hydrocephalus is due in a large part to the development of a technology known as neuroendoscopy which allows a neurosurgeon access to areas of the brain inaccessible with traditional surgical techniques. It involves passing a tiny viewing scope into the third ventricle of the brain allowing images of the ventricle to be projected onto a screen located in the operating room.

Typically, the endoscopic catheter is passed through a small hole burred (drilled) in the skull. In some patients (who are already shunted) the neurosurgeon may be able to use the original bone defect made when the shunt was initially placed.

III. Who is a candidate for ETV?

Most physicians seem to agree that there are three (3) factors that lead to a successful ventriculostomy: 1) Patient's age (it is recommended they be over age six (6) years); 2) Prior placement of a shunt; and 3) A diagnosis of non-communicating (obstructed ventricular pathways). Additionally, some doctors have noted a higher success rate in patients with aqueductal stenosis which is the most common cause of congenital hydrocephalus. Aqueductal stenosis, is a result of the long, narrow passageway between the third and fourth ventricles. The end result is an accumulation of fluid upstream from the blockage.

Doctors hypothesize that previous shunt presence possibly allows development of the subarachnoid space and the presence of a functional shunt buys time for the patient while he/she develops absorption abilities. It should be noted, however, that in patients that have been shunted for a number of years, it is often difficult to determine whether or not the hydrocephalus is communicating or non-communicating without undergoing invasive testing.


Additional information: Endoscopic third ventriculostomy

Social skills development in children with hydrocephalus

I. Overview

As I read the information about social skills development, I suddenly realized that social skills are an area I had never considered my hydrocephalus affecting because I have always been very out-going and made friends easily. That being said, the Hydrocephalus Association (HA) brings up an interesting point "As the first generation of children shunted for hydrocephalus mature into adulthood, it has become apparent that some of them will have difficulty developing their social skills. Lets look at why this is an issue and what can be done to correct it.

For most of us, happiness means friends, meaning we are able to move from isolation to inclusion. Additionally, it means becoming comfortable with social interaction which causes other people to actively seek us out or, at the very least, not ignore us. In order to be involved in social interaction, the acquisition of social skills is a must. In teaching a child (with hydrocephalus) about social skills, we must remember that they can be broken down into component parts and taught in much the same fashion as other skills. According to a study conducted by the National Institutes of Health (NIH), the greatest area(s) of concern for parent's of special needs children - including those with hydrocephalus - are: 1) Talking over differences without getting angry; 2) Persistence when faced with frustration; 3) Learning to politely refuse requests; 4) Learning to take turns when speaking with their peers or other people; 5) Understanding rules; 6) Following directions; and 7) Learning to wait when necessary.

It should be said, however, that these problems are not just limited to children with special needs. For most of us, learning the social skills to handle these situations is automatic. But, for children with learning special needs, often this is not the case.

II. What happens

For the most part our social learning is done automatically through a combination of seeing, copying, and conditioning. By that, I mean that social skills are learned incidentally and without formal instruction. Research has shown that children born with hydrocephalus often have associated learning disabilities which makes it difficult - if not impossible in certain cases - for them to pick up on both verbal and non-verbal clues necessary to learn proper social skills.

Difficulty in picking up on these non-verbal cues can result in serious (emphasis added) social problems for children with hydrocephalus. One example of this is seen in how they often mis-estimate distance and spatial relationships. As such, they tend to either get too close to other people OR stay too far back. When this occurs, the person whose space they have "invaded" normally has the tendency to back off and find a reason to remove themselves from the situation. On the other hand, if they stay too far back, eye contact is difficult and other children are more likely to ignore them.

Research has shown that children may also have difficulty with non-verbal cues might also have difficulties perceiving intonation (the way in which the speaking voice emphasizes words). As an example, consider the child that hears that a party is being planned and then goes up to the "boss kid" inquiring if they can come. The 'boss' responds "Yeah, sure, I REALLY want YOU at my party" Sadly, when the child arrives at the party, it is most likely going to be a heart-breaking experience because the child heard the WORDS ("I want you") but not the TONE ("I would rather hang by my thumbs than have you at my party"). Instances such as this can be excruciatingly painful for children not attuned to such nuances as tone, rhythm, or pitch.

Other important non-verbal cues can be found in posture and facial expression. If a child isn't proficient in reading the facial expressions of others, he/she will likely misinterpret the other person's non-verbal cues. As a general rule, children with hydrocephalus are able to perceive only two types of facial expressions: 'happy' and 'mad' (and, occasionally, 'sad'). Unfortunately, this is not enough to for them to get along in today's world. In addition to those noted above, they need to be able to perceive (and understand) such subtleties as 'quizzical' and 'reflective' and are expected to learn them incidentally.

III. What can be done

It is a known fact that children with hydrocephalus tend to be slower in acquiring skills such as walking, talking, and hand-eye coordination. With a combination of time, remediation, and early intervention, these skills can be obtained, however, is the same true with social skills? Many neuropsychologist (particularly those who have done extensive work with hydrocephalic children) believe that intervention is vital. The reason behind this is, even if the child does manage to catch up on their own, some necessary skills will be missing.  In those rare cases where undeveloped skills do come later in life, the child may have become exhausted, reclusive, or self-defeating in their interaction with each other.

Parents need not wait for this self-defeating behavior to begin if they realize that many of these social skills being discussed can by taught. The first step in this process is to have the child evaluated by a neuropsychologist which can pinpoint areas of deficiency allowing the most effective way to teach the child the necessary social skills to be undertaken. It is worthy to note that, even though schools are now under mandate to provide many types of evaluations, often the in-depth testing and remediation plan(s) necessary to pinpoint areas of deficiency are best carried out by an experienced professional. 

For additional information: Social skill development in children with hydrocephalus

Hydrocephalus and hospitalization (Part 2 of 2)

In part one of "Hydrocephalus and hospitalization" I addressed how the repeated hospitalizations of a person with hydrocephalus - particularly young children - takes a toll not only on them, but on the entire family. Today I will conclude looking at ways to make these visits more pleasant for school-aged children (ages 7 - 12), adolescents (ages 13 - 18), and adults.

For parents of school-aged children (ages 7 - 12)

• Remind the child that it is perfectly okay to express their emotions while they are hospitalized. It is not uncommon for children in this age range to be hesitant to tell their parent’s exactly how the feel in an effort to protect their parent’s from additional emotional stress.

• Be truthful with your child about their condition. I can remember at that age being scared when I would hear unlearned adults saying “Be careful. His head can burst”. In the hospital setting, reach out to your child’s doctors and nurses and, if one is available, a Child Life Specialist.

• Encourage your child to draw pictures or write about how he/she feels while they are hospitalized.

For adolescents

• With an adolescent, they can begin to be their own advocate and ascertain information about their health and what treatments, medications, etc. will be administered.

• As with other ages groups, they should understand that there is nothing wrong with the emotions they are experiencing. Encourage them not to keep these bottled up or act like a brave soldier if they are really scared or nervous.

• They should see if the hospital has an adolescent support group for “tweens” where they can express their hospital experience. An excellent resource to locate such groups is the Hydrocephalus Association website.

• Adolescents need to realize that it is often difficult for parent’s to express their feelings when it comes to both their “baby” being hospitalized as well as the hydrocephalus itself. Give them (parents) time to come to terms with what they are feeling. (I wish I had been given this advice as I was growing up.)

Adults

• Ask questions, questions, and MORE questions about your personal health records. You have the right to know what tests will be done as well as what to expect during your hospital stay.

• It can’t be stated enough, share your thoughts (and feelings) about your hospitalization with your spouse, a trusted family member, or friend. Encourage them to reciprocate and share their feelings with you as well.

Conclusion

As I indicated in part one of this blog, I am not shunted, but I am familiar with repeated hospitalizations due to Coronary artery disease (CAD) and the tips I have listed here are the very same ones I employ when I have to be admitted. I remember in October of last year (2016) I had to be admitted due to irregular (fast) heartbeat and chest pain. I was kept overnight and, around mid-afternoon the next day, a nurse practitioner came in and announced that “you’re being sent home”. Needless to say, I advocated for myself and explained that I refused to be released until they addressed the issue that was causing my problem -- a blocked artery in the “lower 40” of my heart. The next morning, my third stint was implanted, I was happy, and, best of all, my chest pain stopped.

Hydrocephalus and hospitalization (Part 1 of 2)

The ambulance bay at Southern Regional
Hospital.

Today I decided to broach a subject that I don't have much experience with since I am not shunted and that is hospitalization and hydrocephalus. I went to Hydrocephalus Association (HA) website and downloaded their fact sheet on the subject. As I was reading the information and deciding what information to include, I was struck by a profound statement made by fellow hydrocephalus patient Amy Herrington: "shunts are not capable of paying attention to a family's social calendar, or a child's school calendar". I sat here for a couple of minutes letting that soak in and was again reminded how blessed I am that my dad opted to not have me shunted. Make no mistake, shunts are a good thing because they can prevent an enlarged head such as mine, but with a BIG price including the risk of infection and the need for periodic revisions.

Amy hit it squarely on the head when she said "no matter whether you're a parent, child, or adult dealing with hydrocephalus and the possible need for shunt revisions, the common threat of hospitalization looms in the midst". Although I have never experienced it, I can only imagine what a family goes through when they face the possibility of a loved one having to endure yet another surgery and the range of emotions they will face. I have always heard that "growing old isn't for sissy's" well, I have news for you, hydrocephalus isn't either. Here are some tips that Amy offers for dealing with those hospitalizations:

General suggestions for all patients and families

• You have the right to review your own medical records (or those of your minor child) as well as to know what procedures you (or your child) will receive while in the hospital. (Health Insurance Portability and Accountability Act (HIPAA)

• Bring familiar items from home to make your hospital room look more inviting.

• Keep a written or tape-recorded journal while you hospitalized. Concentrate specifically on your hopes, dream, fears, or anxieties. Doing so is an important part of the emotional healing process.

For parents of infant children

• Remember that you are the advocate for your infant child. You have the right to ask questions (and receive proper answers) regarding your baby’s health. Become educated on what YOU can do to help your baby.

• If possible, hold your baby. Touch is crucial to their development. If it is not possible to hold your baby, talk quietly to him or her. Your voice is literally music to their ears.

For parents of a young child (ages 2 - 6)

• As with an infant, you are still the advocate for your young child. Inquire about your child’s health and learn what their needs are.

• Draw a map with your child that depicts the distance between the hospital and home. This will help your child understand where he/she is.

• Some children feel they have to be brave in the hospital -- especially around parents who care about them. Remind them that it is perfectly acceptable to express feelings of anger, sadness, or frustration in the hospital. Have them draw pictures of how they feel both physically and emotionally while they are hospitalized. This serves as an outlet to relieve tension.

For additional information: Hydrocephalus and hospitalization

Did you know? Some interesting information you might not be aware of regarding shunts and hydrocephalus

I. Highs and lows

A fellow hydrocephalic posted on Facebook that if there is a storm approaching, she tends to become very lethargic. That made me curious, so I did some research and hydrocephalus (particularly if the person is shunted) tends to be effected by changes in the weather. According to published research, fluctuations in barometric pressure can affect people who suffer from headaches such as those of us with hydrocephalus. It is interesting to note that the study particularly addressed this phenomenon as it applies to migraine sufferers, however, it stands to reason that if it affects someone who suffers from migraines, it would also affect someone with hydrocephalus. According to the National Hydrocephalus Foundation, the majority of persons with hydrocephalus - and who are shunted - will be affected periodically by an approaching weather system (particularly if it's an area of low pressure). Symptoms accompanying this "feeling out of sorts" include having no energy as well as being unable to concentrate. If it develops into a full-blown headache, the best remedy is to find a dark, comfortable place and ride out the storm. The good news is, once the area of low-pressure causing your distress is directly overhead, symptoms will cease almost immediately.

II. Recovery from surgery

People who have had an endoscopic third ventriculostomy (ETV) or a shunt implant usually have a four (4) to six (6) week recovery period before they begin to feel like themselves again. The biggest thing patients experience is getting their strength back and the best way to speed up is to take short walks - sound down the block and back - and gradually increase your distance as your stamina improves. Perhaps equally as important as exercising your body post surgery, is to exercise your mind. For those who enjoy playing cards, medical experts say that a game of solitaire, bridge, or even poker will do wonders to improve short-term memory.



For additional information: Patient-to-patient: tricks of the trade 

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

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

V. What treatment options are available?

Just like its counterparts acquired hydrocephalus and congenital hydrocephalus,the only available treatment is surgical implant of a shunt (pictured at right). Most shunt systems consist of three (3) components: 1) A collection catheter (the ventricular catheter in the picture at right); 2) A valve mechanism that controls how much CSF flows through the system; and 3) An exit catheter (the peritoneal catheter in the picture at right) to drain the CSF to another part of the body where it can be absorbed -- in this instance, the peritoneal cavity in the abdomen. In most cases of NPH, the most common shunt implant is a peritoneal shunt and the most commonly used system is the ventriculoperitoneal system which goes from the cerebral ventricle to the peritoneum. Alternatively, the drainage catheter can also be placed in a vein that leads to the heart known as a ventriculoatrial or VA shunt. With either system, once the implant surgery is completed, all components of the shunt system are entirely under the skin.

The shunt valve is a critical component of the system and the design that has been in use is the differential pressure (DP) valve. It operates by the valve opening when the (fluid) pressure at the inlet of the valve exceeds the (fluid) pressure at the outlet by a preset amount.  In adults with hydrocephalus, the valve is sometimes overwhelmed by the effects of gravity, leading to the drainage of too much CSF. The common name for this over-draining is siphoning. In some cases the patient is asymptomatic while others experience headache or nausea while in an upright position. In extreme cases it can also result in so much CSF being drained that it causes subdural hematoma. To counteract this problem, valve mechanisms have been designed that incorporate both anti-siphoning and gravity-compensating mechanisms. The anti-siphoning mechanism component is triggered by a change in the CSF pressure while the gravity-compensating mechanism is triggered by postural changes.

For additional information:

NPH booklet

When shunts go bad: complications experienced with shunt systems

X-ray images of a 20-month old hydrocephalus patient
experiencing shunt malfunction. As a result, the child
experienced sutural diastasis.

Hydrocephalus can be treated with a shunt system, however, this treatment often results in complications. Research has shown that an estimated fifty percent (50%) of shunts in pediatric patients fail within two (2) years often requiring repeated neurosurgery. The most commonly seen of these complications are: infection and malfunction (emphasis added).

I. Common complications

Shunt malfunction

A shunt malfunction involves either a partial or complete blockage (of the tubing) causing the shunt to function intermittently or not at all. As a result of the blockage cerebrospinal fluid (CF) accumulates and, as illustrated in the image at right, can result in diastasis where a fracture line traverses one (or more) sutures of the skull resulting in a widening of the suture.

The blockage can result from blood cells, tissue, or bacteria and occur in any part of the shunt. Both the ventricular catheter (the portion of the system placed in the brain) and the distal catheter (the portion of the system that drains CSF to other part of the body) can become blocked by tissue from the choroid plexus (which produces CSF) or ventricles. In adults, the blockage occurs most commonly in the distal portion of the shunt system.

Shunts - by their very nature - are very durable, but their components can become disengaged or fractured as a result of wear or as a child grows. In rare cases valve failure can occur due to a mechanical malfunction.

Shunt infection

Typically a shunt infection occurs because of the person's own bacteria and isn't acquired from someone else who is ill. The most common infection to occur in a shunt system is Staphylococcus Epidermidis which is know for attacking indwelling medical devices such as a shunt. which is normally found both on the surface of a person's skin as well as in the sweat glands. Normally a shunt infection occurs with a one (1) to three (3) month window following shunt implant surgery, but has been known to occur up to six (6) months following the surgery. Research has shown that patients receiving a ventriculoperitoneal (VP) shunt are statistically at the highest risk for developing a shunt infection secondary to abdominal infection.

II. Less common complications

Over drainage

CT scan shows hydroceplaus
patient with SVS.

This complication causes ventricles to decrease in size causing slit-like ventricles due to the brain and its meninges pulling away from the skull. Known as slit-ventricle syndrome or SVS for short (pictured at right) , it is most commonly seen in young adults (ages 20 - 39) who were shunted as a child. One symptom that is unique to SVS is the severe intermittent headache that can be relieved by lying down. In order to confirm a diagnosis of SVS, imaging must be completed that shows the smaller size ventricles.

Under drainage

Results in the exact opposite problem as over drainage and that is an increase in the size of ventricles as well as the inability to relieve the symptoms associated with the hydrocephalus. In some cases, to restore a balanced flow of CSF, it might be necessary to implant a new shunt with a more accurate pressure valve. In patient's with a programmable shunt, it is possible to restore a balanced flow by simply re-setting the opening pressure.

Subdural hematoma

Control unit of a programmable shunt.

This is generally seen in older adults (ages 60 - 80) and is caused when blood from a broken vessel in the meninges becomes trapped between the skull and the brain. Surgical intervention is required to correct this complication.

Multiloculated hydrocephalus

This condition occurs when a located (isolated) compartment in the ventricular system becomes enlarged and not in communication with the normal ventricle. There a numerous documented causes including: neonatal intraventricular hemorrhage, trauma that occurs at birth, ventriculitis (inflammation of the ventricles), or shunt-related infection. Typically it can be difficult to identify due to the fact that it is most often seen in infants and children who might be neurologically compromised.

 
For additional information: Complications of shunt systems