Hydrocephalus Awareness Month Post 4: What does a shunt feel like?

The other day I read a post in one of my hydrocephalus Facebook groups that made me sad and also made me wonder. It was posted, "Does anyone wonder what it's like to not have a device in your head and tubing running down your neck and into your abdomen?" It made me sad because it makes me wonder if Nicholas will feel that way one day (maybe a cure will come in his lifetime!), but it also made me wonder what it actually feels like to have a shunt.

Obviously I can't ask Nicholas yet what it feels like to live with a shunt on a daily basis so I turned to the hydrocephalus community to ask them what it's like to have a shunt. I have a small community of people I know in real life with shunts but am a member of several hydrocephalus groups on Facebook and on Babycenter and they provided me with some insight.

The first thing I was told was that the side effects of having a shunt were far preferable to living with the pressure in your head from having untreated hydrocephalus. That having excess CSF was painful, caused nausea and also made you dizzy. That you experienced things like double vision and just overall felt terrible. Obviously left untreated, hydrocephalus can also lead to brain damage and death, so having a shunt is preferable to that.

Also, I heard very different things from people I asked. Some experienced nothing out of the ordinary that they were aware of and some experienced all of these things - so like hydrocephalus itself, having a shunt feels different for each individual person who has one.

The main things I heard were being able to "hear" the shunt. When a shunt turns on, most people say that they are aware. They can hear it buzzing, clicking, popping, or gurgling inside their heads. This depends on the placement of the shunt (the closer it is to the ear canal the easier it is to hear) and it appears to also depend on the type of shunt (programmable, non-programmable, maker, etc.). Shunts are not always on, they turn on when the pressure in the head indicates they should be. The newer
programmable shunts detect the level of pressure in the head and turn on when the pressure exceeds what the shunt has been programmed to maintain. Older, non-programmable shunts, were on levels of high, medium, or low pressure and would detect when the brain reached the threshold that each shunt was set for. The bad news with non-programmable shunts is that if the shunt is over or under draining, the shunt must be replaced with surgery. Nicholas has a programmable shunt which means if we determine (and we have, several times) that his shunt is under draining, it's an office visit and a strong magnet is used to reset the shunt to a better level.

Another complaint is that when the shunt is draining, if the tubing is placed close enough to the ear, it can mess with a person's equilibrium. Because there is fluid running through a tube near the eustachian tube it can make people feel dizzy. This doesn't appear to be a common side effect, but has been mentioned in the community.

Most neurosurgeons leave a large amount of tubing curled up in the abdomen when treating children (Nicholas's did for this exact reason) so that as the child grows, the tubing can stretch and revisions (more brain surgery) are not necessary as long as the shunt continues to function properly. I have been told that as the child grows, it is typical to feel some tightening or tugging as the shunt tubing breaks loose of adhesions under the skin that were formerly created and lengthen under the skin. I imagine that must be fairly uncomfortable.

Weather is a big one. Most surgeons will tell you that weather has very little to do with the pressure inside the head, but almost every single person I've talked to who has hydrocephalus and is treated with a shunt has said that when the weather changes, they get headaches. It's apparently a big debate within the hydrocephalus community. We have seen it first hand with Nicholas. If a big storm is rolling in, he becomes very uncomfortable and cries. If we give him Tylenol, it seems to relieve and relax him. I've been told by my co-worker with hydro that if it's very hot, she gets headaches. She has found that wearing hats helps to reduce the headaches.


Abdominal discomfort is another common complaint. It seems that the CSF draining into the abdominal cavity creates acid reflux in a significant number of hydrocephalus patients.

While not every person who has a shunt experiences all of these, these complaints come up time and again. And again, every person I talked to said that this is clearly preferable to living with hydrocephalus untreated and obviously better than facing death so they take their side effects with a grain of salt, some ibuprofen, and maybe some Tums.

Hydrocephalus Awareness Month Post 3: Side Effects

The problem with hydrocephalus is that it affects people on such an individual level that there's no true list of side effects from it. There are things that are common amongst individuals affected with hydrocephalus, but people can range from completely high functioning (you'd never know they had hydrocephalus) to total brain damage. It all depends on so many factors, including how early the hydrocephalus was detected, what type of hydrocephalus they have, and what the body did before treatment intervened.

Some of the more common side effects are:

Vision problems, headaches, precocious (or early) puberty, seizures, poor hand/eye coordination, learning disabilities including nonverbal learning disabilities, difficulty understanding complex and abstract concepts, difficulties retrieving stored information, and spatial/perceptual disorders.

Because there's such a large range of side effects, hydrocephalus affects individuals all in a completely different way and on a totally individual level. Since there is no way to talk about all the different ways that hydrocephalus affects every person, I am going to tell you how hydrocephalus affects Nicholas.

The first reassuring thing we were ever told about Nicholas was when we met with his neurosurgeon while I was still pregnant with him. Being told that there's something wrong with your child's brain is terrifying. Meeting with a doctor who will do brain surgery on your child within days of being born? Even scarier. We literally had to trust this doctor with our child's life and we hadn't even met this child yet. Our heads were still spinning as we tried to research what hydrocephalus even was and here we were meeting with a man who would go inside this baby's head. He looked at the MRI and ultrasounds and told us that he was pleased to see something. Nicholas had a giant head. We were so confused. That was a good thing? The doctor told us it was a great thing. Because Nicholas's head was so large, it meant that his brain was growing along with his ventricles. When the head was developmentally normal and the ventricles are large, it means the ventricles are growing into the areas where the brain is and that typically means more brain damage than we would likely see in Nicholas.

I can't tell you how lucky we were that his head expanded. He has some issues because of the hydro, I'm not going to lie. But he's one of the lucky ones. The further we venture into the hydrocephalus community, the more we realize just how lucky this kid is. Obviously we don't know yet if he will have a learning disability or some sort of social disorder due to the hydrocephalus, but as of this moment, his issues are small and manageable.

Nicholas's side effects include a g-tube because of a weak swallow reflex. Weak. Not non-existent. That is a big distinction. He's already showing improvement with lots of speech therapy. The neurosurgery and pediatric team believe the weak swallow reflex is a result of some brain damage due to the hydrocephalus. That was so hard to hear. But he's improving. He had a swallow study on Monday and while he didn't "pass" (there's not really a pass/fail according to the speech therapist that administered it), we were told it was his strongest swallow study yet. That's in only 8 months of having a g-tube and therapy!

Nicholas is being stimulated by electric pulses (it's not painful - he actually falls asleep to this!) to help make his muscles in his neck stronger. This will hopefully strengthen his swallow.

Nicholas is also physically delayed. He has been rolling from back to tummy for about a month and just recently gained enough strength to roll from tummy to back. He will scoot a little bit while he's on his tummy using his legs to push. He just started to push his legs up when you hold him upright with his legs on the floor (i.e. he's starting to try to "stand" while you hold him) and he can hold his head up but not for extended periods of time. All of this is being addressed in occupational and physical therapy. We were told by our neurosurgeon at one time that it's not like it will be on his college application when he walked, and we agree with that. If he doesn't start walking until he's 2, he doesn't. It's not the end of the world.

Occupational therapy = playtime! Fun!

Cognitively, Nicholas appears to be on track, if not slightly advanced in some areas. He tracks with his eyes when you put something of interest in front of him. He reaches for toys and passes them back and forth between his hands. He rolls to his side to grab toys. He babbles and laughs (most often at something Zachary does - this kid sure adores his big brother) and smiles. He's a complete sweetheart and everyone who meets him falls in love.

Hydrocephalus Awareness Month Post 2: What is a shunt? Is it really brain surgery?

Once we've explained what hydrocephalus is (and we are more than happy to do so - we like getting the knowledge and awareness out there!), the next question is, if they can't do surgery to remove the blockage, what is the treatment?

There are three different treatments for hydrocephalus, a shunt, an endoscopic third ventriculostomy (ETV), and an endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC). ETV/CPC is only available to infants. The decision to treat the hydrocephalus with any of these methods is determined on a case by case basis, and also within a timeline of a patient's life. It is perfectly acceptable to be treated with one method and later in life attempt the other.  

The ETV requires no shunting so some people prefer this method as there is less likelihood of mechanical failure, but if this method fails a shunt is required. The success rate depends on a lot of

individual factors including age of the patient, cause of hydrocephalus, and amount of scar tissue in the third ventricle. It is not recommended typically for infants because their bodies are so dynamic and quickly changing that it frequently fails. Basically, a neurosurgeon goes into the brain and makes a puncture in the floor of the third ventricle so that CSF is allowed to flow through. It effectively bypasses the blockage if there is one. The third treatment option is very similar to this except it includes cauterizing the choroid plexus which reduces the amount of CSF being generated. There are complications to this method including closure and/or infection of the surgically created pathway, short term memory loss as this procedure may affect the hypothalamus, and potentially endocrinologic irregularities since the third ventricle is responsible for some hormonal function. For individuals that this works for, however, the success rate can be up to 90%.

The shunt method involves a surgical implantation of a shunt. A shunt is a tube that diverts CSF into another region of the body, most typically the abdominal cavity, where it is absorbed and removed. A valve inside the shunt helps maintain normal pressures within the ventricles. The shunt that drains into the abdominal cavity is called a VP shunt (ventriculoperitoneal). The other type of shunt is a VA (ventriculoatrial) shunt which drains into the right atrium of the heart. This is not a preferred placement and is only tried after there have been several malfunctions of a VP shunt. There are

several complications with a shunt system, the most frequent being shunt malfunction. Shunt malfunction is most often caused by a blockage that stops the shunt from being able to function properly. Blood cells, tissue, or bacteria can all create a blockage. Shunts can also malfunction because it is a mechanical device or because it becomes dislodged from its original placement. Shunts can become infected, though this typically occurs within 6 months of the placement. In any of these instances, the patient must have another brain surgery to get a new shunt placed. This is called a shunt revision.

Other shunt complications include under or over draining. This can usually be treated by changing the pressure on the shunt. Abdominal and/or heart complications can also occur depending on where the shunt drains into. Obviously abdominal complications are less severe than heart complications.

Nicholas had a shunt placed on the second day of his life. His neurosurgeon prefers to shunt children

and considering he has the highest success rate of hydrocephalus treatment in the southeast, we trusted his judgement. His goal is to place a shunt once in a child's life and have it last until the child is at least 18. He is successful in that goal in 40% of his patients. Nicholas's surgery was fairly uncomplicated and quick. The neurosurgeon told us that it's easier in babies in that they have a soft spot to go in through, whereas in adults he has to drill through the skull. Nicholas has a scar on his head where the shunt was placed and then also a scar in his belly where the end of the shunt was placed. Most people think that his shunt drains into his stomach, but it doesn't. It drains into the area around his stomach and is absorbed and removed by his intestines. Nicholas has not yet (knocking on wood) had a shunt malfunction but he has had some abdominal complications that involved a hospital stay and several x-rays and ultrasounds. He has had the pressure reset multiple times on his shunt as his neurosurgeon tried to drain the massive amount of fluid he was born with and then to normalize the pressure in his head to remove the optimal amount of CSF.

It is not uncommon for patients to have multiple surgeries with hydrocephalus. There are people who have had hundreds of surgeries and then there are some who have lived with the same shunt for decades. There is no rhyme or reason to why shunts fail multiple times in some people and work like a dream for others. We hope that Nicholas is in the latter group, but the honest answer is we just don't know. We are asked frequently if he is done having surgery and we just don't know. We hope so, but the likelihood is that he will have brain surgery again at some point in his life. Maybe more than once.

Hydrocephalus Awareness Month Post 1: What Is Hydrocephalus?

The first in a month long educational series for Hydrocephalus Awareness Month.

I have answered this question a few times on this blog, but probably the biggest thing I get asked is "What is hydrocephalus?" The word hydrocephalus comes from the Greek hydro (water) and cephalus (head), thus water on the brain. In a person not affected with hydrocephalus, the brain fluid (cerebral spinal fluid) is produced within the ventricles in the brain and in the choroid plexus. It circulates through the ventricular system within the brain, and then is absorbed into the bloodstream. The fluid is constantly in motion and has several purposes, including to cushion the brain to act as a protector against injury, to provide nutrients and proteins to the brain and to carry waste away from the brain. In most brains this system works well and the ventricles remain within a normal range which keeps the pressure inside the brain at the appropriate levels.

In a brain affected with hydrocephalus, for some reason (and there are several), the cerebral spinal fluid is not circulated out of the brain into the bloodstream, or CSF is developed at too quickly a rate for the brain to effectively remove it. This causes the ventricles to enlarge and increase pressure inside the head. In worst case scenarios, the result of the increased pressure is brain damage.

Hydrocephalus does not differentiate between race, sex, or age. It affects infants, children, young adults, and the elderly. Over 1,000,000 people in the United States currently live with hydrocephalus and for every 1,000 babies born in the US, 1 to 2 will be born with hydrocephalus. Hydrocephalus is the most common reason for brain surgery in children.

Normal brain CT scan - dark areas are the fluid in the ventricles

Brain with hydrocephalus - notice the enlarged ventricles in the shape of a butterfly.

There are several different classifications of hydrocephalus:

Congenital Hydrocephalus: This is the type that Nicholas is affected with - it is present at birth and is typically caused by a combination of genetic and environmental factors during fetal development. It is typically diagnosed before birth with ultrasound and fetal MRI (Nicholas was officially diagnosed at 21 weeks gestation but our attention was brought to the possibility at around 13 weeks gestation when he had ventricles on the high end of normal during the NT scan).

Compensated Hydrocephalus: This is diagnosed in adulthood but may have been congenital.

Acquired Hydrocephalus: This develops after birth as a result of head trauma, a brain tumor, cysts, brain bleed, etc.

Normal Pressure Hydrocephalus: This occurs in older adults when the ventricles increase but the pressure does not increase within the brain. The cause of this is typically unknown and it is often - upwards of 20% of the time - misdiagnosed as Alzheimer's. You may have seen a recent Grey's Anatomy about this type of hydrocephalus.

There are several causes of congenital hydrocephalus and the cause of Nicholas's is the most frequently occurring cause: Aqueductal stenosis. There is a passageway between the third and fourth ventricles in the brain (in the back of your head) called the aqueduct of Sylvius. Aqueductal stenosis occurs when this is either narrowed to the point where CSF cannot be effectively removed or there is a complete blockage. We believe Nicholas has a complete blockage based on the CT scans of his brain. I've frequently been asked if we could just have surgery to go in and remove the blockage and at this time, that technology does not exist.

When Nicholas was officially diagnosed with hydrocephalus (he didn't even have a name yet at that time!), his ventricles measured 13 mm. Ventricles that are within normal ranges are expected to be  below 10 mm. For comparison, in the same MRI that officially diagnosed Nicholas, Emily's ventricles were 4.5 mm. At the last ultrasound that I remember his ventricles being measured (I had one ultrasound per month due to my "high risk" pregnancy with twins and ahem advanced maternal age), his ventricles were measuring at around 55 mm. He was born with a head circumference of a typical 18 month old.

Stay tuned tomorrow for more about hydrocephalus. What is a shunt and is it really brain surgery?

Hydrocephalus Awareness Month

September is Hydrocephalus Awareness Month. We knew absolutely nothing when we received Nicholas's in utero diagnosis. I will be giving information throughout the month of September on hydrocephalus right here on our family blog and if we can help one person's journey then it will have been worth it. 

I know we have gotten as far as we have because of the help of people who have journeyed down this road before us and shared their knowledge, so I'll use Hydrocephalus Awareness Month to hopefully educate people and help. 

Along those lines, still working on getting the Hydrocephalus Association WALK in central Florida in 2016!

Cranial Band Therapy: The Beginning

We always knew cranial band therapy (or helmet therapy) was a possibility for Nicholas. Because he was born with such a large head it was incredibly difficult for him to move it and so he spent a vast majority of the first several months of his life on his back with his head in the same spot. This created a pretty significant flat spot on the back of his head. Beyond that, because he had so much fluid inside his head and it's less dense than brain matter, his skull bones that are not fused yet were able to be moved around more easily based on where his fluid was. Once the brain matter started to push in and take over the areas the ventricles had previously occupied when the shunt started to significantly drain the fluid, the fluid that remained had to go somewhere and began to push out of the back soft spot. This created a point at the top of the back of his head. The shunt was turned down (which actually increases the amount of fluid it drains) and most of that fluid has been successfully drained out of his head. The point, however, remains because the bones have been pushed into that position.

It was a fight to even get the cranial band on Nicholas's head. We started with a fight with the PA at the neurosurgeon's office, then a fight with the insurance. We pushed on both fronts and won. The cranial band therapy started with getting a mold of Nicholas's head so that the band would be perfectly fitted to his head. He wasn't terribly appreciative of this process but it was a necessary part. The mold was then left to sit for 24 hours to dry and then sent to Arizona where the actual band would be made. We were told it would be ready for fitting in 2 weeks. We ended up not being able to get the fitting completed until three weeks after the mold due to our travel.

Nicholas was brought into the orthotics/prosthetics office and the band was placed on his head to insure it fit. What surprised us was that the band was not fitted perfectly tight against his head. It's loose and sometimes moves around. This makes sense because we're trying to change the shape of his head so his head needs some space to grow into. The first week there was no padding added, the fit was just checked and we were given a schedule to ease him into wearing the band full time. The first day he wore it for an hour and had it off for an hour. Second day, on for two hours, off for one hour. So on and so forth until he was wearing the band 23 hours a day by day seven. Yes, he even sleeps in the band. He gets an hour off to take a bath and for us to check for pressure sores. Poor dude is so sweaty when we take the band off. And stinky. We have to check his entire head for red spots and monitor them during the hour he has the band off. If the spots don't fade within the hour the band is off, we are to not put it back on and to contact the office the next morning for re-fitting. We don't want him to get any pressure sores from something that's supposed to be helping him!

The second appointment was a week after the fitting. The specialist looks at Nicholas's head and determines where the growth should be limited and where we want the growth to happen more. He does this by placing pads in specific places inside the band. This basically stops the head from growing more in the area where the pads are and promotes growth into the open spaces in the band. By doing this, we are hopefully encouraging growth from the flat spot in the back of his head that will help round out the head. Nicholas continues to go back and pads will continue to be placed.

True to his character, Nicholas has accepted the band with little fuss. He was uncomfortable on the first day but now that he's wearing it 23 hours a day, it's just another part of him that is helping him - much like his Mic-key button for feeding. He remains a happy baby who loves laughing at his big brother, chatters whenever he's awake, and smiles at anyone he meets.

The Kindness of Strangers

Last week we went on a trip to my home state for my sister's wedding (eek can't believe she's married!). We were understandably nervous about the air travel for said trip - it was the first time for the twins on a plane and we were not sure what the pressure changes would do for Nicholas's head and shunt. We were also traveling to a higher altitude than we normally are at. We consulted with Nicholas's neurosurgeon who told us that because we control the pressure in Nicholas's head with a mechanical tool, it was highly likely that he would fare better than the rest of us as we acclimated to the different pressures. That was a relief! Mike collected physician notes from Nicholas's pediatrician and neurosurgeon along with copies of his latest CT scan and shunt series and the card that indicates the current pressure his shunt is set at so we could carry it all with us in the event of an emergency trip to the children's hospital that didn't know us. We hoped against hope we wouldn't have to use it (and we didn't end up having to!) but figured better prepared than not.

Mike called TSA to ask for advice on what to do with all the bottles of water, powdered formula, Nicholas's feeding pump, and the medicine that we needed to carry on with us. The TSA agent told Mike to ask for a supervisor upon arrival to security check and that's what we did. A PSS (passenger support specialist) walked through line with us, helped us get everything on the conveyer belt (the stroller didn't fit, so that was walked through and hand checked), walk all the kids through, check out all our "special" stuff and got us on our way. We were through security in less than 15 minutes. It was incredible.

The flight out was running on time and when Mike went up to get the tags to gate check the stroller, it was suggested that we pre-board so that we had more time to get everything set up. We were not allowed to all sit in the same row since we had two lap infants - there are only four oxygen masks per row so someone wouldn't get one. I sat in one row with Emily and Zachary and Mike sat across the aisle holding Nicholas. Emily was fascinated. She watched people board, smiled and flirted at them, and was very calm. Nicholas, on the other hand, was less than thrilled. He ended up fussing most of the trip there. Luckily we were surrounded by parents and they were all very understanding. There was a girl who sat next to Zachary in the window seat and she entertained him the entire trip. She played games on the iPad with him, listened to his stories about a million times, and helped him get his movie set up when he wanted some quiet time. I was amazed. I thanked her profusely and she revealed that she works with children. She is clearly in the right field.

On the way home, we ended up getting to the airport early. We asked at security for a PSS and they looked at us like we had asked for a three headed dog. We explained what had been told to us at our home airport and they said there was no one there like that. We asked for a supervisor and they told us to speak to the gentleman helping the passengers through line. He didn't really understand what our needs were either, but he tried very hard to help. Our things went through, a TSA agent took the bottles filled with water to be checked and when he was done, he just walked away from them while grunting "it's good." We took that to mean we could pack everything back up and nobody stopped us, so I guess we were right. We were still through security fairly quickly but it was a less pleasant and helpful experience.

This flight appeared to be running on time as well, and when we got the tags to gate check the stroller we were again given pre-board access. Zachary had a meltdown when we boarded this time because he wanted to sit by me and we had decided on the way home he would sit by Mike. A gentleman was sitting across the aisle from Mike so I sat in the row behind him with Emily. When both Zachary and Nicholas were crying, the man jumped up from his seat and ran to the back of the plane. Nicholas cried during pretty much the whole boarding process which ended up working out very well for us because nobody sat in their row. I had a woman sit in the window seat, but the middle seat stayed open. The woman winked and said she always sat next to lap infants because the row almost never filled up. She was also incredibly nice and held Emily for me while I strapped in. She talked to Zachary through the break between the seats when he turned around and said hi. She told me that parents get so stressed out when their babies cry but that most people don't care and understand that babies will be babies.

Nicholas ended up calming down once we took flight and actually slept through the entire flight. Emily cuddled up and fell asleep too, and Zachary watched movies and played games through the whole flight. So, the family that boarded like lions ended up having the quietest children on the flight.

All in all it was a very smooth process because of the helpful staff through most of the journey and kind people on the flights. Ironically, we met twins almost everywhere we went. On the flight out, the woman sitting next to Mike had three year old twins, we met a man in the airport with six month old twins, one of the servers at the wedding reception was a twin, and on the way back the woman who sat next to me was a twin. It seems we've joined a very close knit community.