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Hydrocephalus

Hydrocephalus Poster Child
Kate McDonald

Hydrocephalus Poster Child
Tommy Gundlach

Introduction

Hydrocephalus is an accumulation of cerebral spinal fluid (CSF) in the ventricles of the brain, causing them to swell and, in infants and small children, the head to enlarge. Typically, the fluid will flow from one ventricle to the next, out over the outside of the brain and down the spinal cord, and then will be absorbed into the bloodstream. In those with hydrocephalus, there is an imbalance between the amount of CSF that is produced and the rate at which it is absorbed.

In the United States, hydrocephalus occurs in 1 out of 500 live births. The World Health Organization shows that one birth in every 2,000 results in hydrocephalus. It is one of the most common birth defects and afflicts more than 10,000 babies each year.

 

Features and Characteristics

Hydrocephalus can occur due to several reasons:

There are several problems associated with hydrocephalus:

 

Diagnosis

Depending on the age of the individual when hydrocephalus is suspected, several diagnostic tools are available. An ultrasound of the brain is usually performed in children ages 6-12 months. After the skull has fused, a better diagnosis of hydrocephalus can be made with an MRI or other brain imaging technique.

 

Treatment

Hydrocephalus is usually a lifelong condition; the patient is treated rather than cured. The treatment most often used is inserting a shunting device. Although shunting does not cure hydrocephalus, it controls the pressure by draining CSF out of the obstructed pathways and into the bloodstream. The shunt is a system of tubes with the upper end surgically inserted into a ventricle of the brain and the lower end into the heart, outer lining of the lungs, or the abdomen. In most cases, the shunts are required to stay in place for life, although alterations and revisions may be required.

The complications associated with a shunt are often caused by a blockage or infection. If the shunt becomes blocked, the symptoms may develop gradually or may occur suddenly in the form of severe headaches and vomiting. If the shunt causes an infection, there may be accompanying fever, abdominal discomfort or pain (for those draining into the abdomen), skin rashes and joint pain. Various tests can be used to determine if there is an infection or blockage and medical advice should be sought immediately if either are suspected. If there is a blockage, an operation will likely be required to adjust or replace the shunt. If there is an infection, the entire shunt may be removed and a course of antibiotics may be given before a new shunt is inserted. 70% of shunts are said to fail within a 10 year period and a child may need five to six shunts before reaching adulthood. Shunting can often times result in individuals leading full and active lives.

Endoscopic third ventriculostomy is a procedure used to open the floor of the brain, using a laser fibre or other device, to allow the fluid to flow out of the brain via a natural bypass. The risk of the procedure is very low and there are only a few potential side effects. In addition, there is no overdrainage, no blockage, the risk of infection is small, and there is no foreign matter left behind that can cause difficulties later in life. The success of the procedure does depend on the cause of the hydrocephalus, however. In cases caused by brain tumors or from birth, the success rate is 85%. When there has been an infection or brain bleed, the success rate is 50%. The overall success rate of endoscopic ventriculostomy is 66%.

 

What to Expect

Many newborns born with hydrocephalus will have a normal life span and normal or above average intelligence; 75% will have some type of motor disability. Generally speaking, however, those who have had hydrocephalus since birth or childhood have a lower IQ than those in the same age group without hydrocephalus. For some children, special education and physical and occupational therapy may be necessary. With the use of shunts, the outcome for most children with hydrocephalus is optimistic. It’s important for parents to know that the effects of hydrocephalus vary from individual to individual.

 

Personal Stories

My daughter, Kate, has hydrocephalus. She is a surviving twin born at 26 weeks, weighing 850 grams (1 lb. 13 oz.). Kate's first IVH screening had been clear. Her subsequent ultrasound follow-up indicated that there had been a bleed and she was diagnosed with a grade III IVH and followed accordingly. A few weeks later, it became apparent that the bleed was not resolving and had caused hydrocephalus. Neurosurgery was called in and Kate underwent surgery. It was during this surgery that it was discovered that the areas that had appeared on the ultrasound to be blood were actually bacterial abscesses/infection. The abscesses were drained as much as possible by syringe and an external ventricular drain (EVD) was placed in her left ventricle. The EVD remained in for 7 days and was then removed for fear of further infection. Kate was started immediately after surgery on a 6 weeks course of antibiotics. For six weeks her hydrocephalus was untreated and her head circumference grew daily. A CT scan after 5 weeks showed the abscesses were gone. Two weeks later, Kate received her shunt. Her head went down quite a bit and she continues to catch up to it as she grows.

Kate is now one year old and 9 months corrected. She is doing fabulously. We aren't seeing any major limitations at this time, although she continues to use her right arm less than her left and it tends to be tight.

My daughter is the light of my life. She amazes me everyday. I have learned more about strength and love in the last year than I have during the previous 29 years of my life!

My advice to parents going through tough times with their kids... do whatever you need to do to get through the rough spots... you will be rewarded! Keep hope and never give up. Doctors always tell you the worst. Don't despair when you are told your child won't be normal - it seems impossible, but you will eventually find a new definition in your life for what normal is. Lastly, I'd tell parents to reach out and find other parents and resources for special needs kids. It really helps to see you are not alone. - Hilary McDonald

My son, Tommy, was born with hydrocephalus. His shunt was put in two weeks after he was born. His right side was affected by the pressure on the brain. His doctor says that the whole left side was damaged and is not useable. He has adapted somewhat. He drags his right foot when walking and has a hard time using his right hand. He has mild cerebral palsy and walks with a walker. Tom is still in diapers, mostly because he is stubborn. He is 13 years old and attends special classes. Tommy is at the age level of a 5 year old in school. He is learning lots of math problems and is writing much better this year; I owe it all to his teacher. When he was 6 years old, he had his hamstrings and heal cords released. Three weeks after surgery, he was hospitalized with a very bad staph infection. For 2 months off and on, we were in the hospital fighting the infection. The doctor thought it would be best if we just put in a whole new shunt. Knock on wood, we have been hospital free for 7 years, thank God.

Tommy is very smart in his own way. He doesn't forget a place or face. He knows every movie that he has watched and has his own CD player. His favorite music is by The Who. Tommy still watches Barney and Arthur. His hobbies are collecting Hot Wheels and Batman figurines.

Tommy is such a joy for us. He has an older brother Mick, who is almost 16 years old and a younger brother, Tyler, who is 5 years old. We are also expecting another baby in April, 2000. You don't seem to notice all the accomplishments with a healthy child as you do with a handicapped child. Tommy is doing better than what the doctors had given us hope for him to do. He has taught us a lot in parenting. - Sue Gundlach

When I was 19 weeks pregnant, I had an ultrasound that was completely normal. After weeks of hounding my husband to find out the sex of our new child, we went for another ultrasound at our own expense. Thank God we did!! I was 25 weeks pregnant and it showed that our baby was a boy, and his ventricles were 1 ½ centimeters. The next week, I went to see my OB and she sent me to have a level 2 ultrasound, although she told me she hated it when mothers went to other places to have them because they always find something wrong. That was 3 days before Thanksgiving, so you know I couldn't get in to see anyone for 1 ½ weeks (which was pure hell). At the level 2 ultrasound appointment, they confirmed our son had hydrocephalus.

During the next 9 weeks, I went for weekly ultrasounds because his ventricles were swelling so rapidly. Finally, within several hours from the last ultrasound, they decided I had to deliver the next day. I was 34 weeks gestated. The OB said that Andrew’s head didn't seem to be that large, but still required a C-section. When the delivery was over, she pulled the drape down and told me she was wrong, and that Andrew’s head was much larger than anticipated; it was 44 centimeters! During the next 8 days, Andrew needed 4 blood transfusions because my blood and his didn't mix after he was in the real world, and he was on a ventilator. On January 28, 1999, Andrew was successfully shunted. Thank God he still has his original shunt. Although the catheter is floating between his ventricles, it is still working.

Andrew was born with both his eyes facing out, which is kind of rare because most of the kids that have eye problems are born with them in or crossed. He also has a disorder called septo-optic dysplasia. It is a rare mid-line defect that causes problems with the pituitary gland, and is associated with optic nerve hypoplasia, which he also has. Andrew has polydactyly syndrome - 6 digits on each limb.

Because of the hydrocephalus, they took an MRI right away and we were so lucky to discover his other problem. Andrew has severe reflux which warranted a fundoplication. Andrew is also hypoglycemic due to the septo-optic dysplasia, so he gets MCT oil in his formula, which has caused a very rare side effect on his G-tubes. He has gone through 11 G-tubes in 3 months! That amount would usually last a kid 2 years. All of our nurses say that Andrew is the most complex kid they’ve seen who doesn't require machines. Andrew is also blind, but knows his mom’s and dad’s voices as soon as he hears us. He is hypotonic from the waist up and is no longer able to eat by mouth because he just recently started to aspirate. They think he is doing that because he has a very high pallet.

Although I've rambled on about Andrews problems, I have to tell you all how much we LOVE Andrew. He is the miracle baby that took us ten years to have! - Nancy Mendola

Our son, Daniel, was born with an occipital encephalocele and resultant hydrocephalus 11 years ago. He has had 8 shunts and needed to have a posterior fossa decompression, has ADD/ADHD, a seizure disorder, and had eye surgery for muscle weakness. He is mildly mentally retarded.

Danny is a great kid; he loves to joke around and play musical instruments (self created - banging on something with pencils, play guitars, etc.). He loves game shows, computers and riding his bike. Danny cannot, however, read, write well, walk down steps one foot over the other, or tie his shoes.

We love Danny for his great outlook. He had a very difficult beginning, overcoming an infection that went into his cerebrospinal fluid, and the other operations. He is the most loving kid you would want to meet and laughs all the time - everything makes him laugh!

Even though dealing with Danny's behavior problems can be daunting at times, he really is the "light" in our world! - David and Cathy Golder

My son, Michael, was born July 8, 1990. He was diagnosed with aqueductal stenosis at 5 months of age. He was shunted at that time and has had six subsequent revisions to the shunt system in total. Michael was the test case for a programmable shunt; the first patient implanted with it in the United States (February 27, 1992). He has an excellent quality of life, a fantastic memory and sense of humor, a huge appetite, and a positive approach to life. Michael receives occupational therapy within the public school system to help his fine motor skills. He is now in fourth grade. Parents, don't give up hope! - Christine Riccio

My daughter, Emily, is almost 8 years old. She was diagnosed as having hydrocephalus while I was still pregnant. She was two weeks past her due date. During a routine ultrasound, it was noticed that her head seemed a little big. I was sent to our local medical university hospital to have her there so that she would be able to have surgery as soon as necessary. I was very glad that the medical problem was discovered before birth. Had it not been, my daughter would have been transported to the medical university and I would not have been able to see her for a few days (I had a C-section).

Emily was 5 days old when she had the placement of her shunt. It is a little valve that was placed to the side of her head. It looked like a walnut under the skin before her hair grew over it. It is there to take the extra fluid away from the head and recirculate it into the stomach cavity. Cerebral fluid is supposed to flow up and down the spine. Under normal circumstances, the brain is continually "washed" and protected by this circulating fluid. In Emily's case, the fluid was circulating into her head, but not out of it very well.

Emily is a beautiful girl, with a porcelain complexion just starting to freckle and red hair. Her glasses frame her father’s blue eyes and give her an intellectual look (although when she was 3 months old, they made her look more like Mr. Magoo).

Emily can maintain a sitting position, but is not able to sit up on her own. Emily doesn't walk, but can stand while holding on to your hands. Emily can drink independently (sippy cup) and has done some self-feeding with a lot of guidance. (While learning to use a spoon, Emily would throw her spoon or run the spoon over the top of her head. After washing grits out of her hair, it was decided that self-feeding would have to wait.) Emily does not talk, however, she laughs whole-heartedly when something tickles her (usually a sound). She enjoys lights, music, cuddling, movement and children. She has a very special bond with her paternal grandfather. She attends school (the profound class) and enjoys taking the bus.

There were several things that I would do differently if I had the information that I do now:

1. I would find out all that I could about the shunt and the operation involved to place it (I know that this is not always possible and that sometimes it is necessary to act quickly). The placement of the shunt, from what I have read so far, was most likely the best thing we could have done. And in our case, has worked very well for us. However, whenever the brain is cut into, there is the potential for problems. I can't help but wonder if my daughter's vision was affected by this necessary operation. She was later diagnosed with congenital glaucoma and is now considered legally blind.

2. Why was the operation postponed until she was five days old and what affect did this have on my daughter's brain and eyes? I was confused to have the doctors tell me that the operation was not an emergency and could wait. If there was too much fluid on the brain, causing there to be pressure and the head to grow larger, then why wasn't it important to get this problem fixed as soon as possible? Again, I don't know if this was of significance or not. But, I would have insisted on getting an answer had I to do over. Emily was a full term baby without other health issues.

3. I would have gotten a better definition of the word "delayed," as it was often used in regards to my daughter. Anyone who has ever waited for a late train, plane, or bus, knows that if it is delayed that it will eventually get there and you will get where you are going a little (or a lot) late. Delayed does not imply that your train is not coming or that it will drop you off along the way, away from your original destination. I use the train analogy because when my daughter was about 6 years old, another train analogy was said to me and it finally occurred to me that in our case, my daughter was not going to "catch up." I was probably in denial about how things were and it is a thin line between being realistic and pessimistic.

The train analogy that the neurologist told me was this: Your intellectual development is like a train track going from one coast to the other. This trip is made in about 12 years, normally traveling about 1/12 of the distance each year (some years are quicker than others). Now if this intellectual train is allowed to travel at this rate, it reaches its destination on time - age 12 with intellect of 12. However, if it is delayed, then it travels at a much slower rate. So in Emily's case, she is traveling at about 1/4 of what is the average. So, if things continue to progress as they have, by the time she reaches intellectual maturity at age 12 years, her level will be 4 years.

For those of you whose children are not mentally impaired (or who are minimally impaired) and visually impaired, this may not be the case at all. I am told that there are some children with hydrocephalus who are able to lead "normal" lives.

4. No one said anything about seizures or the necessity of replacing the shunt! After my daughter started having seizures, I was told that a large percentage of children with disorders involving the head have seizures. Fortunately, the seizures have stopped. She has been seizure free for about 5 years and medicine free for about 3 years.

When the shunt was placed, we were given a list of things to look for in the event that the shunt should malfunction. But, we were also assured that they usually only malfunction due to an infection, which usually occurs shortly after placement. The doctor went on to say that the type of shunt that Emily got was the type that empties into the stomach cavity. A lot of extra tubing was placed in there to allow her to grow without disconnecting the valve (her x-rays look like she has eaten spaghetti!). In the past, they had shunts that emptied over the heart; they needed to be altered from time to time.

When the nurses used to ask how many revisions Emily's shunt had, I just assumed that they were not aware that she had the type of shunt that didn't require revisions. That was until a friend, that has the same kind of shunt as Emily, had to have her's replaced. That was when I found out that about half of the shunts need to be replaced after about 10 years. And, about half of the ones left may need to be replaced in the next 10 years.

I am sure there are other things that I would like to have done differently. And, there are other things that I may never know, such as what caused Emily to have all these disabilities. But, I have come to realize that you rarely find out those answers and that having those kinds of answers aren't much help.

I did however go through genetic testing to find some of those answers, mostly to give us some piece of mind in planning our family. We do have a beautiful healthy 4 year old daughter. She is a good little (big) sister. - Mimi Morrison

I had many problems throughout my pregnancy (I have endometriosis, which can give you problems in conceiving and carrying a child). We were told to get pregnant right away by my OB-GYN (whom I love dearly). We conceived within two months. The day we found out I was pregnant was August 10, 1998. We were both very excited, but nervous; I had miscarried the previous year. Of course, everything that could go wrong, went wrong.

I started having problems from 3 weeks on. I was constantly in the doctor’s office for ultrasounds to see if the baby was okay. Finally at 18 weeks gestation, I went into the ER with spotting problems. This is where we first found out that we were having a baby girl. We were so excited, yet the ultrasound tech called in Radiology to look at the ultrasound as we waited. They sent me home and told me to see my doctor that week. On Friday of that week, we went in, they gave me another ultrasound, confirmed that she was a girl and sent us into the doctor's office. That was one of the worst day's of my life. They told me that my beautiful, unborn daughter had hydrocephalus. We were stunned; we had no clue as to what hydrocephalus was, which the doctor explained to us, is extra fluid on the brain. He told us that we would need to continue to get ultrasounds at another hospital because they are the best of the best in reading and following things like these. My husband and I both cried the whole way home. We then came home and told our families. They were in shock. How could this have happened, they all asked. Well, my mother and I immediately began researching the Internet for any tools to help us. We found good and bad. We prayed.

After the first ultrasound at the new hospital, the doctors told us I would need an amniocentesis (where they insert the needle into your belly) to check for spina bifida and Down syndrome (which is common with this). The amniocentesis came through with flying colors, although I would never have one done again, as it would not change my mind to keep my unborn child. At this point, we were to continue with an ultrasound every two weeks, to watch for extreme growth in the head that would endanger her. We prayed all the time that a miracle would occur and her head would be fine.

Finally, in February 1999, we met with the neonatologist who would assist with our daughter's birth. They would take her at 33 weeks gestation as long as her head did not grow significantly by then. We also discontinued ultrasounds at the hospital, as we were unhappy with their staff, who were VERY UNSUPPORTIVE and put me in immense pain and tears every time we went there. We continued with ultrasounds at my doctor’s office and I also had steroid treatments to help mature her lungs.

D-Day (Delivery Day) was scheduled. My daughter would be born on March 4, 1999. I did not get a wink of sleep the night before. We were told that she would, with no complications from her shunt, probably only be in the hospital for 1-2 weeks. We were very hopeful that this would be the case. All that I knew, was that I wanted them to help her. We wondered the whole time, if she was in pain. Caitlyn Marie was born at 8:09 a.m. on March 4, 1999. She was the most beautiful thing in the world. All I knew was that I was a mother to a beautiful little girl, whom I already loved with all my heart. Since I had a C-section, I was unable to see her until around 5:00 p.m. She weighed in at 5 lbs. 15 ozs. - a pretty big girl for being only 33 weeks. Little was I to know, that our problems had just begun.

The neurosurgeon came to see us in my room the following night. My little girl had SEVERE hydrocephalus. Basically, they did not know what she would be like or if she would be able to see, and her poor little head was misshapen. Her head was long instead of round from the pressure and her anterior soft spot was pushed out. We cried and cried. The doctor was going to operate the following morning to put in her shunt, and then on Sunday, he would try to remove the damaged skin on the back of her head. Well, guess what?... She pulled through wonderfully.

Caitlyn only stayed on the respirators for a short time and was off the pain medication shortly thereafter. We were finally able to begin feeding her a bottle. Seeing that she was 7 weeks premature, her suck was underdeveloped and she wouldn’t eat hardly anything. She was so tired all the time. After two weeks of fighting this, the neonatologist told us that we could either put in a G-tube or give her another week and he would put it in anyway. Well, we did not want this. Our baby had been through so much already and we did not believe that another surgery would be wise. So again, we prayed. Our Caitlyn (and several NICU nurses - who are simply angels on earth) decided that the tube was not going to happen either. Caitlyn started eating from a bottle that night and kept going strong.

Well enough chattering; you want to know the happy ending, RIGHT?? Caitlyn is now almost seven months old. She can see, is developmentally correct for her age and loves her mommy and daddy. She is still petite for her age, but very beautiful. The shunt is working, even though the bubble on the back of her head has still not closed. They will most likely do plastic surgery on her head, but not for awhile. She is an exception to almost every rule (she was a little slow at baby talking) and our little miracle. I thank God every day for the gift he has given us and for allowing her to develop as she should, and to all the people who prayed day and night for her. I will never let her feel different because of her condition and we will help her in any way to become whatever she wants. Who knows, maybe she’ll be the president someday. - Heather Garant

My daughter, Jennica, was diagnosed as having some form of brain disorder while I was 7 months pregnant. I had gone into pre-term labor, and an ultrasound was performed to help determine the cause. I had an alpha-fetoprotein test earlier in the pregnancy, which was negative for neural tube defects. This was my first ultrasound of the pregnancy, because I had been too sick to hold down enough water to perform the study in earlier months.

The ultrasound showed numerous problems with Jennica, many of which were inaccurate. They did not diagnose hydrocephalus at that time, because her head had not enlarged. They did note that the left hemisphere of the brain was not present and that it appeared to be growing outside of her skull, she appeared paralyzed on the right side, and had a club foot. There was the possibility of cerebral palsy, and she was considered low birth weight.

Ironically, the pre-term labor stopped as mysteriously as it began, and I was able to carry Jennica to the complete term. The moment she was born, we began to learn of the inaccuracies of the ultrasound's predictions. She was not low birth weight - she arrived at 6 pounds. The right side was not paralyzed, there was no club foot, and there were no signs of cerebral palsy. The left hemisphere was present, but compressed due to the enlarged ventricle. An MRI, performed shortly after the birth, revealed that there was no brain matter growing within the encephalocele sac that was present on the back of her head. This sac, in fact, was a natural shunt that was slowly leaking CSF, not allowing her head to increase in size.

The sac was removed when Jennica was 5 days old, and it was at that time that hydrocephalus became the official diagnosis, since the CSF could no longer drain. She received a shunt at 1 month of age, and we attempted to make our way within this new world we had become a part of.

It was in those early days that we had the greatest struggle trying to find direction. Jennica's neurosurgeon did not feel that she needed to be followed developmentally. I was an audiologist and her father had some special education background. The neurosurgeon felt we would know if she was falling behind - "Why spend the extra money on testing?" was his rationale. There were no social workers made available for our questions, and we really had no idea of the resources available to help Jennica keep up with other children, or the problems we might face as a result of the hydrocephalus.

We went for 4 years, only watching the concrete milestones she was meeting so easily (walking, talking, sitting up, etc.), but missed the more subtle developmental problems that were occurring (word finding difficulties, organizational problems, social issues). If we had been followed by a developmental specialist, we would have automatically been included in therapy programs based on her diagnosis. When we finally started seeking help, just before her fourth birthday, she was then at an age where the system required proof of a delay prior to providing services. The professionals who evaluated Jennica kept telling us that her studies came out within the normal range, despite the fact that it may have taken them five sessions to perform a study normally performed in one. They would provide us with some basic therapies to appease us, but Jennica never really got the help she needed until she turned six and entered a marvelous school district.

She was immediately enrolled in an all day learning support kindergarten, and finally began to receive the help that she needed. She went on to an ungraded learning support "first" grade, and this year was placed in a regular second grade class with a full time special education teacher present. I'm so proud of the progress she has made, and so pleased with all the help her teachers have given to her. But I can't help but think of how much better she'd be if we had gotten the help that we so desperately needed early on.

Regarding Jennica's health, I suppose we have been lucky, until recently. We were a nervous wreck when she was a baby. It felt as though we were playing Russian roulette when it came to her shunt. In our minds, it could fail at any time, and there was tremendous stress trying to determine what was normal behavior, and what could be the sign of a shunt failure. We were sent home with a 1 month old infant and were told to be concerned if she seemed cranky, vomited often, or slept a lot. And as with all infants, that's exactly what Jennica did in the beginning. Our prayer was that we would somehow "know" that her shunt was failing, because we could not find any detailed information on the subject. We slowly relaxed, as the years went by, until a month and a half ago, at the age of 7 years, when the rules began to change.

Jennica's shunt did fail, and the signs were quite clear. She awoke with a violent headache and proceeded to spend the next 4 hours vomiting, and then it stopped - just like that. She was fine for the rest of the day, with no signs that she had been so sick that morning. These symptoms were unlike anything she had ever experienced before, and when they occurred again 4 days later, that's when I became sure it was the shunt. After a full day of waiting for studies, it was determined that surgery was required, and a complete revision took place.

As traumatic as the event was, I was so proud at how well this little girl handled it all. And, while she made it clear that she did not like the IV or the new pain she experienced from her incisions, she truly bounced back quite quickly and remarkably. Another chapter closed? Not quite.

Three weeks later the symptoms returned, and she needed another emergency surgery. Twelve days later they returned again, and the neurosurgeons were scratching their heads for what to do. The third surgery was to install a pressure monitor to evaluate what was occurring. It seemed to me that they spent the next several days trying to convince me that the headaches and vomiting had nothing to do with her shunt; that this child, who had been so well for the last seven years, was suffering from these debilitating idiopathic headaches from now on. In the midst of their rationalizations, they discovered that her shunt had indeed failed again, and a fourth emergency surgery took place.

This last surgery took a great toll on us emotionally. Jennica had gone to sleep that night thinking she was fine, only to wake up being wheeled out of the OR. She was hysterical screaming, "Tell me it's a dream!" and I couldn't be with her to comfort her. Shortly after surgery, she went into a twenty minute focal seizure, that I thought would take her life. Thankfully it did not, but my newest chapter of this disorder regards my growing dislike for her current neurosurgeons because they do not feel it is important to discuss details with parents in order for them to become part of the decision making process.

That surgery was just about two weeks ago, so I apologize if my emotions seem a bit raw at the moment. I've spent the last few weeks trying to learn everything I can about this disorder, trying to make my daughter feel safe, trying to restore a sense of calm back into our lives, hoping that I can learn enough before the next surgery is required, and crying a lot when I'm by myself.

I could go on about all of the wonderful things she has brought to my life, but will save those stories for another time. - Tricia Dabrowski

My son, Michael, is 6 years old. He has congenital hydrocephalus, but wasn't diagnosed until 10 and a half months. It was the worst 10 and a half months I have ever experienced. He was miserable, and didn't appear to be a "typical" infant. I was told that he had colic and would eventually settle down. My gut kept saying, No, something must be wrong. No one would ever have a second child if this is what it was really like. But, this was my first child, so what did I really know?

Michael was eventually diagnosed with hydrocephalus and a third ventriculostomy was performed. He did well for 10 months and then began having problems again. He was shunted at 21 months and still has his original shunt.

Michael has a duel diagnosis. Along with hydrocephalus, he has been diagnosed on the autism spectrum. He is very high functioning and to a stranger's eye, he appears very typical. He is very involved with a sensory dysfunction, however, he is very concrete and literal in his thinking. He needs a lot of help with organizing and keeping himself together emotionally.

Michael is very bright, extremely energetic and has more enthusiasm than anyone I know. He makes me laugh, when I don't think I will ever laugh again. He has so much character and continues to march on despite all he has been through.

Once I received the hydrocephalus diagnosis, I was informed that Michael was an "at risk" child. He would probably have some learning disabilities. What I didn't know, was the characteristics that put him on the autism spectrum would be so difficult. He struggles socially and emotionally and it is difficult to watch how hard peer interactions can be for him.

The one piece of advice I can give to parents who are new to this special world is to always trust your instincts. You may not be an M.D. or a Ph.D., but you know your child. If something doesn't seem right, it probably isn't. Also, there are some fantastic, caring professionals out there and I suggest you surround yourself with them, because it is not an easy road. - Sandra Bauer

I would like to tell you about my daughter, Katie. She was born with congenital hydrocephalus on September 24, 1991. I found out when I was 4 months pregnant that she had hydrocephalus. The first thing the doctor talked to me about was aborting her; that was totally out of the question. The odds were stacked against her; with her having this, and me being an insulin dependant diabetic, things certainly did not look good. To make a real long story a little shorter I'll skip to her birth.

She was born 4 weeks early by C-section, was breathing totally on her own, and had the most wonderful cry. I knew she would be okay. The neurologist came to my room that night and told me that my daughter did not have a brain, was probably blind, would not be able to suck, swallow and breathe at the same time, and that we should institutionalize her. Her head circumference was 48 cm. They were going to wait 24 hours before they placed her shunt to see if she could hold her own, which she did, but 12 hours later, she went into surgery and everything has gone up hill since.

She is in the first grade in a public school, she is on a third grade reading level, and she is the most loving child. We have had some problems though. She started having seizures 3 years ago, so she is on medication and so far, she is under pretty good control. She is delayed physically and mentally, however, she is not mentally retarded like I was told she would be. She didn't walk until she was 4 years old, and we're working on potty training. She has overcome a lot of fears that she had (like movement - she wouldn't ride in a stroller or anything that moved other than a vehicle, and she didn't like loud noises or toys that made noise). She loves music and dancing; she loves being a ballerina. She has a memory like you wouldn't believe and she loves Barney, The Rugrats and Blues Clues. She likes to role play; she memorized the movie Toy Story. She has done and is doing everything the doctors said she wouldn't.

As far as medical problems, she really hasn't had many problems other than the seizures. She had strabismus, which was corrected, and she had a problem with ear infections (she had her adenoids taken out in 1993 and hasn't had another one since). She does get physical and occupational therapy once a week, and will be starting speech soon (but only for the language - she was talking and pronouncing her letters correctly when she was 2 years old). - Della Anderson

Jonathan is our youngest of 3... all boys! He was a surprise, but one that we are glad we got. He was born on June 1, 1998 weighing 7 lbs. 14 oz., but only after having a hard go of things. See, we were hit head on by a drunk driver when I was 7 months pregnant. I broke my pelvis in two places and was on a walker for 8 weeks and then a cane until Jonathan was born. The doctors were not sure for the first few days if Jonathan would make it, but he did. We knew then that the Lord had great things in store for him. With the other two boys having Nephrogenic Diabetes Insipidus (NDI), the doctors were much more cautious with Jonathan. His breathing was a little off and he had a larger head than normal (15 inches) so they put him in the NICU to watch him more carefully.

Well, at first the NDI did not show. We expected it would not at first, but the doctors were concerned with something else: his head size. The doctor had a head ultrasound done and the doctors delivered to us heartbreaking news. Jonathan had hydrocephalus, water on the brain, and needed brain surgery right away. I was numb inside. I thought, isn't the NDI going to be enough for the little thing to handle without this? But once again, the Lord provided everything needed.

The day before his surgery, I woke up and told my husband I wanted Jonathan transferred to ACH (that is where his kidney specialists were and they have to be in on the surgery). So, we spoke with the doctors in the NICU and they arranged everything. Angel 1 from ACH came and picked him up and transported him to the NICU at ACH. By this time, his sodium levels were starting to rise. They were in the high 150's, so it was time to get the ball rolling! I was nursing him so they discharged me (after having a C-section... barely 3 days post-op) so that we could follow and be close to him at ACH.

At ACH, Dr. Belsha and Dr. Wells were waiting on Jonathan when he arrived and they wasted no time in getting treatment started for the kidney condition. Later that night, Dr. Teo, the neurosurgeon, came in to see Jonathan and called us down for a meeting. My husband and I were sick to our stomachs. It was midnight and we were expecting to hear the worst, but not this time! Dr. Teo, who is very well-known to be one of the best, told us that Jonathan did not need surgery at this time. He wanted to wait and watch. He did express to us that Jonathan's ventricles were grossly enlarged but in some cases, like Jonathan's, there was no progression and surgery may not be needed. We were so very happy!! Praise be to God for giving us a doctor that was not quick at the knife.

Dr. Wells watched Jonathan for a few more days. He started him on Diuril and potassium chloride. He was discharged under strict doctor's care. At that time, he was nursing every 2 hours for 20 minutes and getting water. It was not long before we noticed that Jonathan had reflux, not as bad as Justin, my other son, but still enough to cause concern. Again, we went to see Dr. Casteel and she ran the test and clearly he had reflux so the medications were started! We weren't sure at that time if it was the NDI or the hydrocephalus causing it. It was very clear to us though that the NDI and hydrocephalus were two diseases that were not going to work good together! One seemed to mask symptoms of the other and vice versa.

We took daily weights and charted his intake/output for the doctors, and made bi-weekly trips to ACH for several months. His sodium levels were running a little higher than normal, but seemed to be staying steady in the 145-147 range. Maybe his levels were just going to run higher than the other boys?! Otherwise, he was doing good. Well, Dr. Teo followed Jonathan for 6 months very closely with head ultrasounds and also just by measuring Jonathan's head. We went in for a CT-scan on December 15, 1998. After Dr. Teo measured his head, he expressed to us that we could wait no longer, and that we had to do something. We discussed the shunt procedure and both Jeff and I were uneasy about it. So, I asked him about the procedure that he was known for, which is called a third ventriculostomy. It is not widely used and mainly is known to be more effective in children over 2 years old. This is due to the fact that the sutures are fused at this age and the cerebral spinal fluid is better absorbed. Dr. Teo explained to us that the chances of this procedure working were only 50/50 and told us about all of the things that "could" happen. We decided it was worth the try because if it did work, it would allow Jonathan to live shunt free but still control the hydrocephalus. So we said go for it!

The surgery was scheduled for December 30th. Normally, for this surgery, the child would come in the morning of the surgery, but not us. We had to involve Nephrology completely. We admitted Jonathan the day before for IV fluids so that he would not become dehydrated during the time he was NPO. Talking with the anesthesiologist was difficult because they just don't seem to get that kids with NDI can't have just any ‘ol IV fluids. But finally, we made everything clear and the plans were made. The next morning they came to get Jonathan to take him and we were told that he would stay one night in ICU and then another 3 days or so on the regular floor. We were prepared for the worst. There was a slim chance that Jonathan may not make it through the surgery or even some chances of more brain damage from the surgery itself, but we had total faith in God and the doctor that He had sent our way.

Jonathan's surgery was about 2 ½ hours (total time) and when we got to see him in recovery, he was waking up so good. He was ready to drink, of course!! He did so well, that he went straight back to his room. His sodium levels stayed in the normal range, and get this... he went home the next afternoon!! There is still a small question of whether or not the surgery was a complete success, but he suffered no other damage and is progressing slowly but surely! So to us, it was a total success!!

He is now 10 months and weighs 19 lbs. 6 ½ oz. with a head circumference of 51.5 cm or 20 ½ inches. We see Dr. Wells every 4 weeks but we don't have to see Dr. Teo until September. It looks like we are on the down-hill side of the mountain now!! Recently, Jonathan has started showing signs that prove the third ventriculostomy to be failing. We had a CT scan done and his new neurosurgeon has scheduled a shunt placement for October 11, 1999. He is still progressing well in his therapy, but his head is starting to grow at a very fast rate. However, shunt or no shunt... we know that God is in control and all will be fine! - Tammie Works

 

Resources

If you are interested in meeting other parents and individuals who are involved in raising a child with hydrocephalus, the following listserv is available:

Hyceph-L email discussion group - To subscribe, send a message to: listserv@listserv.utoronto.ca, leave the subject field blank and type the following in the body of the message: "subscribe HYCEPH-L" (without the quotes) followed by your first and last name.

For more information on hydrocephalus, please visit the following helpful sites:

 

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