And Now He's Better

Nicholas failed his most recent swallow study. I don't honestly remember if I posted anything about that but he did. A swallow study is exactly as it sounds. Barium is mixed with things that Nicholas eats (a bottle of formula and some purees) and then he swallows under an x-ray while a radiologist and speech therapist watch to see what happens.

It was frustrating for Mike and I because we had very high hopes that he would pass. His speech therapist was sure he was doing fantastic and was surprised also. What we found out was that he's still silently aspirating approximately 50% of the time. So basically, one out of every two swallows he is aspirating at least some of his food. That's a big issue because when you put food into your lungs you increase the likelihood of pneumonia. It was determined he's still not safe to eat by mouth and that we would schedule another study soon. The speech therapist believes he may have aspirated more than he normally does because he's teething right now and has lots of extra drool. Either way, he's still 100% tube dependent.

I will be totally honest and say that I have viewed Nicholas's g-tube as an albatross around his neck. I know that we had it placed in the hospital to get him discharged because the NICU wouldn't discharge him until he was safe to eat, and eating by mouth was not going to happen (he was aspirating approximately 75% of the time on his NICU swallow study - so huge improvements in only 7 months), so we agreed to the g-tube surgery and learned how to tube feed him. I will admit it's been rough on Mike and I (mostly Mike) to take him to speech therapy two times a week, week after week, and to continually hear that he's still NPO (not allowed to eat by mouth). I sigh every month when his box of supplies arrive and we dutifully put away the bags and pack away the extra Mic-key buttons and g-tube extensions. I try very hard to not let this show because in the event that he needs to be tube fed when he's old enough to realize, I don't want to discourage him or make him feel like he's "different" but it's been frustrating.

Today as we were walking home from taekwando, Zach was talking to me about a conversation that he had with his friend at school. He said, "I told her that I used to go to the hostible a lot and then Nicholas got his g-tube and now he's better and I don't have to go to the hostible anymore." It stopped me in my tracks. Because he's right. We used to have to go to the hospital every day. I used to have to go home every night and leave my youngest child sleeping in a hospital bed ten miles away, being cared for by nurses (phenomenal nurses, don't get me wrong, but not being cared for by us), I used to have to check myself out of the unit to use the bathroom and then scrub in and check back in to see him. Then he got his g-tube and he came home. I had let the frustration of him failing his swallow study consume me to the point that I forgot what the g-tube had once represented - freedom to bring our child home. 

And really, if the worst thing that comes from Nicholas's hydrocephalus is a g-tube, I am pretty sure we are among the lucky ones. If there's one thing my kids have taught me over and over is that everything will happen on their timeline and not one minute sooner. So I will give Nicholas his time. If he never is able to eat liquid by mouth then that's that. If he uses a g-tube until hes 5, that's that. I will stay patient and remember this is not my journey to walk and that getting frustrated will do absolutely nothing but make me frustrated. It certainly won't help a 9 month old learn how to not aspirate. 

Never Forget

I will never be able to know how terrifying it must have been to be in New York or Washington DC. I know half a country away it was terrifying, I can't even begin to imagine what it was like to be that close.

By the time I turned on the Today Show that morning to get ready for school (I was in college), the first plane had already hit. I tuned half an ear in while I curled my hair. I watched the second plane hit from the mirror. Then the Pentagon. I remember people being afraid that Colorado was a target. Because of Rocky Flats and NORAD. 

Mostly I remember no planes. I walked into the two classes I had before they closed the campus and nothing was taught that day. We watched the coverage in horror. The sadness we all felt when the towers collapsed. We knew this took so many innocent lives. The fear about what was next. The strange silence in the skies. It was all so surreal. And a day that has been forever burned into the memory of this country. 

Today, fourteen years later I say a prayer. For those who lost their lives, both on that day and in the days after to illnesses they wouldn't have but for being there that day. I pray for the heroes that were lost and that must live every day carrying what they saw and experienced there. For the families that have picked up the pieces and moved forward. And for the country that banded together. 

Hydrocephalus Awareness Month Post 5: It IS Brain Surgery

What happens when a shunt is placed?

Shunt placement surgery IS brain surgery. It is among the most common surgery that neurosurgeons perform - upwards of 40,000 surgeries annually. As I've talked about multiple times on this blog, Nicholas was 2 days old when he had his surgery. Because of his age when the surgery happened it was actually an easier surgery (the only time in his life he's had an easier road!). Typically the neurosurgeon has to drill through the patient's skull in order to place the shunt. Since Nicholas was so young, the neurosurgeon was able to go in through his soft spot and place it. As a result, his recovery was easier and the surgery was shorter than typical. 

Shunt placement surgery is typically around 90 minutes and is performed under general anesthesia. Because the surgery team has to go inside the head, the area where they will be placing the shunt is shaved.

A tiny incision is made in the head (older patients get a hole drilled into their skull at this point also), and a catheter is threaded into the brain. The other end of the catheter is then snaked under the skin around the ear, down the neck, and into the area that it's being placed in (most commonly the abdominal cavity). An incision is also made in the abdomen to guide the catheter to the right place (such as not behind the bowels or taking up space the stomach needs). The neurosurgeon then attaches the pump or valve to the catheter to tell the shunt when to drain the CSF from the ventricles. We were warned that a third incision may be needed behind the ear to help guide the catheter around that curve but Nicholas's surgery team ended up being able to guide it without another incision. 

Once the surgery is done, the patient remains in the hospital for recovery. Children are usually required to lay flat for 24 hours after the surgery. Older children and adults may be ready for discharge as early as the day after surgery if there are no complications. Nicholas's neurosurgeon felt comfortable with him being cleared from a neurosurgery standpoint 7 days after his surgery. His extended NICU stint was a result of his feeding issues, not his brain surgery ironically. 

Because the shunt is a mechanical object it is not only common for it to fail, it is pretty much expected to. We were warned of symptoms of shunt failure because leaving a shunt failure untreated can lead to brain damage and even death. Shunts fail more frequently in infants and children than adults. The current average lifespan for an infant's shunt is two years. Older children and adults have a current average lifespan of 8 years. That's a new brain surgery called a revision. Every time a shunt fails.

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?