Hydrocephalus: The Past, the Present, and the Future

Hydrocephalus is a neurological disorder where there is a fluid buildup in the brain. Diagnosis and treatment of hydrocephalus has improved dramatically. This disease can happen to anyone; it is not a genetic disorder, and it can be diagnosed at anytime. Physically, mobility is often a difficulty. The fluid buildup leads to constant headaches, anorexia, lethargy, and a plethora of other health concerns. The resolution to these problems is a shunt. Emotionally, acceptance can be hard for those diagnosed later in life. Depression is also common for some; medications are prescribed to help.

The history of hydrocephalus leads all the way back to 1550 when researchers first introduced noncommunicating hydrocephalus ("Timeline Results for Hydrocephalus History." Web. 19. Apr. 2010). Chronic hydrocephalus was introduced in 1593(Sauvages, The Cyclopedia of Practical Medicine. Google.org). This was also the year that researchers put the abnormal shape of babies’ head and the disease together. In 1891, a spinal tap was used to treat the disease (Aschoff, Kremer, Hashemi, and Kunze, "The Scientific History of Hydrocephalus and its Treatment. Neurosurgical Review). In the 1950’s, John Holter, a researcher, came up with the first effective shunt for his son. A decade later, the shunt procedure was explained. Today the process for treating hydrocephalus is much that same as it was in the 1950’s. However, there are now many different types of shunts.

Centuries ago, children with hydrocephalus weren’t treated. In fact, children with hydrocephalus were seen as aliens or freaks (Najaflan, Alireza. "Severe Hydrocephalus." Goldbaboo.jpg). Before the 1950’s, hydrocephalus was usually fatal. When shunts started to be used, the prognosis was 46% fatality (Fried, A. "Childhood Hydrocephalus: Clinical Features, Treatment and Slit-Ventricle Syndrome." virtualtrials.com). Today, many people with hydrocephalus are successfully treated, although they may have a variety of other issues.

A child may develop hydrocephalus before they are born. This can be detected by the use of an ultrasound. Usually the doctor has to take the baby by caesarian section. These babies generally have a lot more problems because there has been a buildup of cerebral spinal fluid (CSF) ("Symptoms and Diagnostic Tests." hydroassoc.org).

Babies may also develop hydrocephalus when they are born. The two common causes of this are a bleed in the brain or an infection that goes into the brain. Babies that develop hydrocephalus often develop a large head, the soft spot becomes hard and bulges, the veins in the head stand out, and the eyes drop down and you can’t see them much. Also most babies with hydrocephalus become irritable, lethargic, don’t eat, and tend to throw up a lot ("Hydrocephalus." neurosurgerytoday.org. What is Neurosurgery?).

In toddlers and children the symptoms may be the same as in babies. However, additional symptoms may include headaches, fever, personality changes, and trouble falling asleep or not being able to wake up. Physically, they become unstable in their balance, they have delayed progress in walking and talking, poor coordination, and lose much of the ability to do things they were previously able to do ("Hydrocephalus." Neurosurgerytoday.org. What is Neurosurgery?).

Young and middle aged adults who develop hydrocephalus usually have headaches, problems waking up or staying awake, poor coordination or balance, problems controlling their bladder, and impaired vision and cognitive skills. For older adults the symptoms vary a bit. Their coordination or balance becomes poor to the point where they shuffle their feet when they walk. They also have headaches, memory loss, and problems controlling their bladder("Hydrocephalus." Neurosurgerytoday.org What is Neurosurgery?).

There are many different causes of hydrocephalus. For babies and children congenital malformations are a major cause of hydrocephalus(Fried, A. "Childhood Hydrocephalus: Clinical Features, Treatment, and the Slit-Ventricle Syndrome" virtualtrials.com). These malformations don’t allow the CSF to drain from the brain. Instead the CSF stays in the brain, pressing against the brain cells. In a normal brain, 80% of the brain consists of brain cells, 10% is blood, and 10% is CSF. If more than 10% of the brain is made up of CSF, the percentage of brain cells decreases. There are three other common causes of hydrocephalus in babies and children. One is an intraventricular hemorrhage in the brain, another is a tumor, and the third is an injury to the brain ("Hydrocephalus Fact Sheet." National Institute of Neurological Disorders and Strokes. ninds.nih.gov). The most common cause of hydrocephalus in teenagers through middle aged adults is head trauma. For elderly people, hydrocephalus is associated with Alzheimer’s and Parkinson’s diseases.

At first, doctors will diagnose hydrocephalus by monitoring the size of the head growth (in infants), taking a detailed history, and using a CT scan to see if the ventricles are enlarged. Sometimes an MRI may be used as a diagnostic tool. In rare cases doctors may measure the intracranial pressure (ICP) by placing a monitor in the brain to determine the pressure in the brain or by doing a lumbar puncture. Normal ICP is between 1 and 18 mm of mercury("Intracranial Pressure Monitoring. US National Library of Medicine and National Institute of Health. ninds.nih.gov).

The normal course of treatment for hydrocephalus is placing a shunt into the patient. The shunt drains the extra CSF off the brain or spine. Usually, it drains into the abdominal cavity (ventricular peritoneal shunt). However there are other places the CSF can be drained to. This includes the heart (ventricular atrial shunt), lungs (ventricular pleural shunt), ureter, or gallbladder. If they find that a baby has hydrocephalus before being born, a doctor will put a shunt in when the baby is 2 days old ("Hydrocephalus." wikipedia.org).

To place a shunt in a patient, a surgical cut is made through the scalp, behind the ear. There is another incision made in the abdomen (or wherever they place the end of the shunt). The doctor drills a small hole in the skull. There is a small tube that is put through the opening, through the brain, and into the ventricle. A valve is connected to the shunt and placed on the outside of the skull. Another tube is placed in the valve and taken under the skin to the location of the drain. The incisions are closed up and the patient goes to recovery.

There are several different types of shunts ("Cerebral Shunt." wikipedia.org). There is a Delta valve that helps prevent the shunt from taking too much CSF off. If too much CSF is siphoned off, you will end up with severe headaches and you have to stay down. Another shunt is a Medium Pressure Cylindrical valve which may lead to uneven drainage from the ventricles. This would cause you to fluctuate from feeling good to awful (actually horrible).

There is also the Nulsen and Spitz valve. This was the first mass produced valve that they used to treat hydrocephalus (Aschoff, Kremer, Hashemi, and Kunze, "The Scientific History of Hydrocephalus and Its Treatment. Neurosurgical Review). This contains two ball-valve units connected with a spring. It does not have an adjustable pressure setting. Without the adjustable pressure setting more CSF is likely to drain off when you stand or sit up. An Anti-Siphon device can be put in to help so the shunt doesn't take too much CSF off (Fried, A. "Childhood Hydrocephalus:Clinical Features, Treatment, and Slit-Ventricle Syndrome." virtuals.com).

The Sigma valve operates on a flow-control mechanism. This is completely different than how the other valves work, which are pressure-controlled systems. This Sigma valve can be adjusted to change the amount of CSF being drained by use of a magnetic device. This means you wouldn’t need surgery to change the pressure ("Cerebral Shunt" wikipedia.org).

Shunts seen like an easy solution, but they aren’t. There are risks when placing a shunt in a patient. There can be bleeding in the brain or brain swelling. The shunt could get infected. There may be an infection in the brain or damage to the brain tissue. The person may also have seizures. If the shunt is successfully placed, too much CSF can be drained. The most common problem with a shunt is that it can quit working. This means another surgery. Currently, in the United States, there is one shunt surgery every 13 minutes, with a cost of over 1 billion dollars. After having a shunt inserted, there is a 50% chance that a revision will be needed within 2 years ("Cerebral Shunt" wikipedia.org). For some people it can be far more than that.

Even if the hydrocephalus is treated, most individuals have some type of cognitive and/or physical impairment. This is why it is so important to find ways to prevent and better treat hydrocephalus. Hydrocephalus, especially which related to brain injuries, may be preventable. People should always wear a helmet when riding their bikes, using roller blades or when skiing. Also helmets should be worn by motorcyclists. People should always wear a helmet when riding their bikes, using roller blades, or when skiing. Also helmets should be worn by motorcyclists. People in cars should always wear a seat belt to prevent their heads from being bumped. Children and adults should wear helmets with any contact sports. Stop and think. Anytime your head can get bumped, it’s best to protect it.

You or someone you know could become hydrocephalic. The Hydrocephalus Association has a wonderful mission. Their mission is, “To eliminate the challenges of hydrocephalus by stimulating innovative research and providing support, education, and advocacy for individuals, families and professionals dealing with hydrocephalus.” Please join this non-profit organization that works so hard to make the lives of people with hydrocephalus better. Their website is: http://www.hydroassoc.org/.

Research

Medical treatment happens over time. My paper will cover the history of diagnosing and treatment of hydrocephalus.

Aschoff, A., P. Kremer, B. Hashemi, and S. Kunze. "The Scientific History of Hydrocephalus and It's Treatment." Neuosurgical Review Oct. 1999: 67-93. Web. .

This article gives the history of when hydrocephalus was first described and how it was treated.



Hydrocephalus History - Search." Timeline Results for Hydrocephalus History. Web. 29 Apr. 2010. .

This article documents the history of hydrocephalus from 1550 to 2010.





Lifshutz, J., and W. Johnson. "History of Hydrocephalus and Its Treatments." Neurosurgical Focus 11.2 (2001). Web. .

This article is about how treatment of hydrocephalus has changed.



Fried, A., and M. Epstein. Childhood Hydrocephalus: Flinical Features, Treatment, and the Slit-Ventricle Syndrome. The Musella Foundation For Brain Tumor Research & Information, Inc. Web. 28 Apr. 2010. .

This article describes the treatment of hydrocephalus and different types of shunts.



United States. National Institute of Health. Ventriculoperitoneal Shunting. By D. Dugdale and D. Hoch. A.D.A.M., Inc., 21 Dec. 2009. Web. .

This article describes the treatment of hydrocephalus.






Photographs:

Najaflan, Alireza. Severe Hydrocephalus. Photograph. Gold Bamboo. Web. 21 Apr. 2010. .

Hydrocephalus. Photograph. Gold Bamboo. Web. 21 Apr. 2010. .

Hydrocephalus Baby Jpg. Photograph. Gold Bamboo. Web. .

Noguez, L. ""Alien Skeleton" Case Solved." Forgetomori. Web. 21 Apr. 2010.

Goals for Living With Hydrocephalus

I have really enjoyed doing this blog. It has given me a chance to find out a lot more about what kinds of things can happen when people go through things with it. Sometimes I have a hard time trying to get things figured out so I can write it. I am hoping I can keep it up after the class is done.

I would like to get some other pictures in it. I have a lot of different ones that I've been looking at. I have a class that I just finished using pictures. I'm going to keep working on that and try to get some of those on the blog.

I have some other topics that I want to get into my blog. I will keep looking at things to try to get more information on it for different topics. I still want to get more things about hydrocephalus. I would like to show more people the blog after the class is done. I have told people about the blog so I would like to make sure they get to see it.

I want to write a book with everything that I've been through in my life. This class has helped me a lot to get things straight so I can work on that. It has gotten a lot easier for me to get words out the way I want to.

Medical Professionals

There are several medical professionals that people with hydrocephalus have to see. If you are diagnosed with hydrocephalus, you will have to see a pediatrician when you are growing up. The pediatrician can help to find out if you're having any problems. Later on, you would have to visit a general physician when you're older. This person will check everything out to make sure you are healthy and everything is checking out.

A lot of times people with hydrocephalus will see a neurologist. That person will watch to see if you are having seizures. They also will watch to see how the body is working. When the neurologist tests for the seizures, they hook you up to an EEG which will determine how your brain waves are working.

You will akso have a neurosurgeon to watch how the shunt is working. They will do surgery to put a shunt in and any revisions if you start having problems. If you have to have surgery, you will have to meet with an anesthesiologist before the surgery, as well. That person will be in the surgery to keep the person asleep during the surgery. If you have local anesthesia, you are still awake but you are numb.

There are physician's assistants and nurses that can meet with you. They can follow your case so they can get things figured out if something's going on. Generally, the nurses and physician's assistants are very knowledgable on hydrocephalus in the department. I meet with the nurses to discuss my medicine pump and I call them first if I start having any sorts of problems.

Sometimes you will have to see occupational and physical therapists. They will find ways for you to be able to do things by yourself. They will come up with exercises to help get your body to work so you can keep the muscles relaxed. There are rehab doctors that can help watch if your having problems.

As you can see, there are several doctors you have to meet with if you are diagnosed with hydroceohalus. It can be frustrating at times but it is also comforting to know that there are so many people there to help you.

Support for Living with Hydrocephalus

Living with hydrocephalus has been challenging. I’ve had to go through so much when trying to figure out about my shunt and if it’s working or not. For example, when I’m not able to eat and my head hurts, my shunt might not be working. If they had better tools to diagnose it, it would be easier to figure out what’s going on. Right now, they don’t have a lot of good tests to tell if there’s something not working. They won’t see me until it’s really bad and a lot of times I end up in the emergency room. The doctors need to have better training to diagnose shunt failure. There needs to be more doctors that can treat shunt failure.

The government makes it hard to get good medical care if you have to cross state lines. You have to go through all the approvals to go out of state. If it’s an emergency you don’t have time to do that. It would help if families could help more without taking away other insurance. The government needs to have funding to do more research into treating hydrocephalus. They also need to have funding for mental health services. It is a common thing for people with disabilities to be depressed or have other problems with emotions.

Shunts are fairly new, so there haven't been a lot of studies on the effects of having shunts. There has been more information on learning problems that a lot of people with hydrocephalus have. The information has not gotten through to schools, so a lot of students do not get the help they need or are not diagnosed correctly. Most kids with hydrocephalus do not meet the criteria to get into special education.

Shunting

When people are diagnosed with hydrocephalus, they put a tube called a shunt in to drain the extra fluid off. They can be in the brain or in the spine. Usually, it drains into the abdomen. It can also drain into the heart, lungs, ureter, or gallbladder. If they find out about it before the person's born, they put the shunt in when they're 2 days old.

A surgical cut is made behind the ear. There is another one in the abdomen. The doctor drills a small hole in the skull. There is a small tube that is put into the ventricle. The other end of the shunt tube is placed in the abdomen.

There are some different types of shunts. There is a Delta valve that helps prevent the shunt from taking too much off. There is a Medium Pressure Cylindrical valve which may lead to uneven drainage from the ventricles. They also have the Nulsen and Spitz valve. This was the first mass produced valve that they used to treat hydrocephalus. This contains two ball-valve units connected with a spring. It does not have an adjustable pressure setting. They can put an Anti-Siphon device in to help so the shunt doesn't take too much off. The Sigma valve operates on a flow-control mechanism. The other valves have a pressure-control system. This can be changed without being programmed or surgically changed.

There are some risks with having shunts placed. There can be bleeding in the brain or brain swelling. The shunt could get infected. There may be an infection in the brain or damage to the brain tissue. The person may also have seizures. The shunt can take too much off. It can also become blocked on either end.

People should not have anything to eat or drink for 6 hours before the surgery. They can have clear liquids up to 4 hours before the surgery. Babies under one year old can have formula, cereal, or baby food until 6 hours before surgery. They also can have clear liquids up to 4 hours before the surgery.

Diagnosing Problems

There are several different tests that they can do to see if somebody is having problems. CT scans show pictures of the brain so they can see if there is any change in the size of the ventricles.

MRIs use magnets to get a picture of the brain. They can see more details of what is happening in the brain. They put you in a tube that's completely closed in to take the pictures. I can't have MRIs any more. The shunts that I have are magnetic and it will change the pressure on my brain.

Sometimes they monitor the intercranial pressure. When they do it in La Crosse, they put the monitor on the outside of the body. With the shunts they put in when I went to Chicago, there's a piece on the shunt that they use to check the pressure. They have a piece that I carry with me that they can get the information downloaded onto a laptop. I also can rub it over the piece on the shunt if I'm having problems and that will show on the laptop, too.

They can do a radioactive isotope to see if the shunt is draining. They inject it into the shunt valve and watch to see if there's any movement. Sometimes it will go through right away. When I've had these done usually there will be no movement. They have me sit up and it goes down right away. If there is no movement at all they know that the shunt is plugged.

They also do x-rays to see if the shunt tube is broken. The pictures will show if there is a problem.

Sometimes physical therapists can do a different test to see how tight the muscles are. They check on the arms and legs to see how everything's working. They have a scale to rate how tight it is. If they can move it without problems it is rated at zero. It can go up to four if it is really tight.