525,600 Minutes

In one week the twins will turn one. When I think back over this whirlwind year, I can't help but feel like we are so incredibly lucky. 

The first month of the twins lives was hectic and scary. Nicholas underwent two major surgeries and spent 21 days in the NICU. I've learned that's a heartbeat in the NICU. He was a short timer. But for our family that was an eternity. Zach befriended child life, figured out where the playroom in the children's hospital was, learned where to touch on the magic wall in the lobby to make music. He quickly learned how to pull out the blanket child life left for him and play with the toys in "his" bin. And what day there was good stuff in the parent lounge fridge. 

Emily slept in the arms of nurses, doctors, therapists, aunts, grandma and parents as we sat for hours next to Nicholas's bedside. 

Mike and I found the healthier cafe in the hospital, learned how to scrub in properly, quickly learned medical terminology, learned how to navigate a "normal" life as we carried around a feeding pump, feeding bags, extensions. We learned how to replace a button in our son's stomach so he could always eat. We watched therapists work to increase Nicholas's strength and learned what they were doing so we could do it at home. 

We enrolled Zach in taekwando so he would have something for himself and we have watched him blossom into a remarkable child. He is a fiercely protective older brother, a patient playmate, and has most recently become a victim of his sister's experiments with biting. He is independent and excelling in school even though he's one of the youngest in his class. 

Emily is growing into a stubborn and independent toddler. She is crawling everywhere and getting into everything. If there is something she shouldn't be putting in her mouth anywhere around her she is guaranteed to find it and put it in her mouth before anybody realizes. She is friendly and likes people. She spends a lot of her time with her brother at therapy and benefits from playing with the toys there. 

Nicholas has had the hardest year of any of us (and it isn't over yet as he has a minor surgery scheduled December 31st to remove a cyst from his nose) and still remains the happiest, sweetest child. He willingly smiles at everyone, he laughs at pretty much anything his big brother does, loves to pet our dog, and is getting around by rolling all over the place. He is currently anywhere between 3-5 months delayed but is making incredible strides every day. His therapists are amazed at his successes. 

It was probably one of the hardest years of our lives for Mike and I. Between working opposite shifts, planning around hospital stays, fighting insurance, and buying a home, we've spent a significant portion of the year at stress level maximum. But we are so very lucky. We get these amazing examples of tiny people to love and raise. It's not an easy job, and with the independent streak running through all three of them, it will probably get harder but it's a pretty fantastic life. 

And I can't believe we will have TODDLERS in a week. 

The Night Before

Tomorrow I will go to my first Hydrocephalus Association WALK. This WALK is in South Florida and has been running for a significant amount of time and is a largely successful WALK (at this time of writing they are hovering near the $50,000 donation mark). 

A year ago we were about to meet the members of our family that would complete it. A year ago hydrocephalus was an abstract term to us. We knew our son had it but we didn't know what that meant. Would our son be able to function? Would he have any brain damage? What kind of future would he be looking at?

A year later hydrocephalus is integrated into our lives. When I initially reached out to the HA about a WALK in central Florida, I didn't really think about the logistics of actually running one and here I sit in south Florida getting ready to embark on this journey. It's an exciting precipice to stand on. This time next year I will very likely have my first WALK under my belt. What a difference a year makes. 

Pregnancy and Infant Loss Awareness Month

October has been designated as pregnancy and infant loss awareness month. One in four women will or has suffered a loss of some sort - whether that's a miscarriage, a baby born sleeping, or the loss of a child. Chances are that you or someone you know closely has gone through this. 

Achieving our family was not easy. It took six months of actively trying to get pregnant with our first child. And it took a month to lose that pregnancy. We were so innocent. We had no idea that when you get pregnant it doesn't automatically mean you take a baby home. I started bleeding very early into the pregnancy. The first ultrasound there was a heartbeat. That reassured us. We could sleep easy. When the bleeding got worse and I went back for another ultrasound the heartbeat was gone. We watched the ultrasound technician try several times to find the heartbeat. We saw our gummy bear shaped baby on the screen. And we never actually saw or heard the heartbeat. At the time I didn't think to ask for the ultrasound pictures. So the memory of that tiny gummy bear is all we have. 

I never really understood just how hard a miscarriage was until I had one. From the moment I saw those two lines on the pregnancy test, I was a mommy. So the weekend where I finally lost the pregnancy became the weekend where all those dreams of birth, holding our child in our arms, watching our child grown and learn and play and become an adult, those dreams were lost. 

We wouldn't have Zach now if that pregnancy would have gone to term. He's a blessing and he is supposed to be here. But it never stops you from wondering what might have been. 

Time has dulled the ache. But every August I stop and remember that there was a tiny being who might have been if a different story would have been told. 

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?

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.

A Helmet for a Warrior

After we won the fight with the insurance company, the prosthetic company quickly scheduled an appointment for Nicholas to get fitted with his helmet. The longer the wait to get the helmet on his head, the longer he has to wear it because head growth does slow down after a certain age and so the helmet needs to be on longer in order to be effective. His appointment was scheduled for a Tuesday afternoon and we were asked to have his hair cut before the appointment. Because his hair is so long, they were worried that the plaster would stick in his hair and create some issues.

The Saturday before his appointment, we took Nicholas to the barber that Mike and Zachary use. Ironically, the barber is named Nick. He is amazing and we are very excited that we found him. He's originally from New York and his father moved here to retire and opened the shop. Nick took over the shop and according to Mike, it is exactly like a barber shop you'd find in NYC. Nick exclusively cuts Zachary's hair and Zachary asks constantly if it's time to go see Nick and get his hair cut. When we told Nick that we were there for Nicholas's first haircut in order to prepare him for his helmet, Nick smiled and cut Nicholas's hair with buzzers. He saved Nicholas's first curls for me and refused to charge us for the cut. It was hard to see Nicholas's adorable hair go away, but if it would assist in any way in helping him move forward with treatment, it was worth it.

The following Tuesday, Mike packed up all the kids while I went to work and off they went to the prosthetic office. Nicholas's head was covered with plaster (he wasn't very happy) and his helmet was sized. The plaster mold needed to sit for 24 hours and set and then was shipped off to be made. It takes two weeks for the helmet to be made and sent back to the local office, so it should be back soon.

The helmet works by creating space where they want the head to fill and a solid place where they either want the head to stay the same or change shape. He is checked by the prosthetic company on a fairly regular basis and they shave off parts of the helmet to encourage growth. We aren't sure how long he'll be required to wear the helmet, but we're guessing it will be several months. We also aren't exactly positive that this will work - that was part of the fight we had getting the neurosurgeon's office to prescribe the helmet, but we are hopeful for positive results.

We also found several companies that make decals for the helmets to make them cute and decorative. Zachary helped pick some and soon Nicholas will look like R2D2 thanks to his big brother.

Learning How To Be Sick

Last week Zachary caught a fairly nasty stomach bug. It started with diarrhea, progressed into a high fever and then ended with throwing up. He missed a taekwondo class (he would have been miserable and we really don't want to infect all those other kids) but was feeling well enough to attend an XMA (extreme martial arts) class on Friday afternoon.

We often tell Zachary that we share in this house. As any parent knows, something kids share very well and very often are germs. Once one is sick you can almost guarantee the others will be shortly also. And probably mom and dad at some point. All day Sunday, Emily was fairly cranky and clingy. She had a low grade fever around 100 but she's also getting two teeth so we decided that was likely what was causing her neediness and fever. She sat in her high chair next to us at dinner and played with toys while we ate and didn't seem to be uncomfortable, so when Mike picked her up and took her to her room to put her to bed when she started to slump and her eyes started to droop, it was a surprise to both of us when her low grade fever had gone up slightly. It was still not terribly high at just over 101 but higher than teething probably warranted. We gave her some Tylenol and put her to bed. I went in about ten minutes later to check on her and found her shaking (not like a seizure, but more like she was cold), breathing fast, and hands and feet that were purple. I was worried she was having low oxygen saturation and Mike ended up taking her to urgent care to be sure she was okay. The urgent care couldn't find anything out of the ordinary and told us it was likely a virus but that if she displayed those same symptoms again to take her to the emergency room.

At around 3 a.m. I was awakened by Mike telling me that Emily had a fever of 103 and he was getting ready to put her in the bath to try and cool her. This was only a few hours after her last dose of medicine and so we decided since she is so young and her fever was so high to go ahead and take her to the emergency room to be sure she was okay. Zach woke up during all the commotion and ended up cuddling up in bed with me while I waited to hear from Mike. The hospital tested Emily for a UTI and other illnesses and also determined that she had a nasty virus that has been traveling around town apparently and if we couldn't control her fever again, bring her back.

We started her on alternating meds - Tyelnol and ibuprofen - every three hours and seemed to have a handle on it. We were waiting for her to start throwing up or having diarrhea like Zach but she never ended up getting it. Monday afternoon, I had an appointment that I had to take Nicholas and Zachary to. Nicholas was fussy the entire time, but we were out for over 4 hours and I figured he was just sick of being in his car seat. When I would hold him, he would calm down. When we got home, I pulled him out of his car seat and his body was hot to the touch. Took his temperature and got 102.7. Gave him medication to help reduce the fever and started the watch on him as well.

Luckily when Zach was sick, I had reached out to my g-tube group on Facebook and asked how they handle a sick child. Nicholas had the nissen surgery, remember, and what that surgery does is effectively cuts off an upward path out of the stomach. Great for keeping his reflux in check so he doesn't aspirate on it. Terrible for throwing up when he's sick. The group told me that they would connect his extension and tape a diaper around the end so that anything he was trying to throw up could leave his stomach via an open pathway. After holding him and rocking him and trying to soothe him for an hour, I realized he may be sick and ended up trying the trick that the g-tube group had told me. As soon as I connected the extension and opened it, stuff began coming out of his stomach and he immediately stopped crying. In a typical turn of events, the kid who physically cannot throw up got the stomach virus. He "threw up" for a few minutes the first time and then I connected him again about an hour later and he "threw up" for a while the next time. After those two times, he seemed much better. His fever stayed in the 101-102 range overnight and then the next morning seemed to disappear - probably because the medication was steady and helping. We fed him Pedialyte for the next 24 hours to give his stomach time to heal from it's illness and he seems back to his happy little self again.

The Fight

After the prescription for the cranial band was written, I called Cigna to make sure that the equipment would be covered under our plan. Cigna has been overall fantastic during this entire process. They didn't blink at paying for a twin birth, never once questioned paying for Nicholas's NICU stay or surgeries. They set us up with a nurse case manager who was there to stand in and help us with any hospital procedures that we had. He can interpret what we're being told by hospital staff or pediatricians. He helps answer any questions we have. He is a liason between us and Cigna. After every hospital stay, we can expect a call from Ray.

When I called Cigna, the customer service rep informed me that she would contact the prescribing physician and determine the procedure and diagnosis codes to make sure we were covered. Since I called her at 5:30 p.m., she told me she would call when she got in the next day and call me back around 1 p.m. She called at 2 the next day with good news that the procedure and diagnosis codes made the equipment medically necessary and directed me where to go that was in network. We made the appointment I blogged about previously and started the process. The prosthetics company made the authorization request and we were told it could take up to 30 days. Ray happened to call us and told us he would try and expidite the process so Nicholas could get started on his treatment ASAP.

About 2 weeks went by and we received a call from the prosthetics company. Our authorization was denied. The reason was that the procedure code for the band was excluded from Mike's company's plan. They had a loophole - if Nicholas had skull reconstruction surgery they would cover it as a recovery item. This made little sense to us - we could potentially fix his issue with a $1500 piece of equipment when he was young enough that he wouldn't even remember it. Or...we could wait until he was over 2, continuing to hold up his development as he's having a hard time rolling over and holding his head up because the weight is not evenly distributed, have a traumatic and expensive surgery and THEN use the $1500 piece of equipment?

We went into fight mode. The first thing that happened was that we escalated the request to the "mis-quote department." Since we were originally told the equipment was covered, there was a possibility that they would cover it because they told us incorrect information. That would take 10-15 business days to be reviewed. While that was happening, we were requesting letters from all of Nicholas's providers - his neurosurgeon, pediatrician, speech therapist, occupational therapist, and physical therapist. Mike researched and found several journal articles published in scholarly journals proving that cranial band therapy is not only effective but among the best treatment for plagiocephaly. Mike also called his corporate HR office and sought their help. They began their research and promised to get back to us. I began work on a letter and we prepared to use their surgery loophole against them since Nicholas had already had brain surgery and this was actually partially the cause that created the need for the band. We commissioned Ray to go to the medical director at Cigna again to pressure an in house appeal.

Insurance in this country is fundamentally broken. I have seen it in my career - watching physicians have to scramble to prove their prescription is medically necessary to payers such as Medicare and Medicaid. I have experienced it now in my own life, having to fight to get a medically necessary procedure to help my son. It is a sad commentary on insurance coverage when a physician prescribes something and the insurance company gets to decide whether it is medically necessary for the patient. I agree completely that insurance should not have to cover something that is a convenience item or if the patient chooses to upgrade an item, but if a physician deems something medically necessary, that should be the only requirement. Insurance must be fixed. I'm not commenting on if I feel the ACA addresses this or not, because this isn't about a political debate here, but insurance needs an overhaul. It's not good for patients. And I say that with a typically amazing insurance in my pocket.

With all these irons in the fire, we were preparing to send in our appeal, which would go to a third party impartial physician. We were certain that the appeal would be granted or we would fight harder. The prosthetic company was amazing and told us that should the insurance company fail to cover the equipment, they would provide it to us at the price they would charge Cigna and that we could make payments. We kept this in the back of our mind in the event that Cigna was going to take too long and we would need to pay up front and seek reimbursement. Last Friday Mike got a call from his HR representative. His amazing company agreed with us and planned on contacting Cigna and telling them to move forward with authorization for the cranial band. She said she assumed we should receive a call within the next business day. Meanwhile, Cigna had taken a look at the policy they were denying the authorization on and discovered that no such policy existed in the plan Mike's company paid for. Under the original policy that we (and the original agent I had spoken with) had researched, Nicholas qualified and Cigna should pay. The prosthetics company called with an approved authorization and congratulations and Nicholas was scheduled for his fitting. Mid-July he will have his fitting and hopefully have the band soon after. 

Hydrocephalus Association WALK

Hydrocephalus is not a well known condition. Unless you've met someone who was willing to talk about it or you know someone who has been diagnosed with it, the likelihood that you're aware of it is slim. Certainly Mike and I along with our families (with the exception of my nurse sister) had not heard of it until halfway through my pregnancy when Nicholas was diagnosed. 

After he was officially diagnosed, the first thing I did was try to reach out to a community. A group that knew what this was, what the prognosis could be, people who had walked in these shoes before. I was disappointed to find that there wasn't really a community local to central Florida. I found groups on Facebook that have been helpful, albeit faceless. I wanted a support group, to look someone in the face and see my potential future. To hear the best and the worst of this world I was now facing. We have had tremendous support from our families and friends. We have prayers from around the globe. When Nicholas winds up in the hospital we get messages and texts and phone calls and offers of help. And each and every one of those is felt and appreciated and held close to our hearts. It would be helpful to also have a place to sit down with other parents or people with hydrocephalus and say what we see and hear what's worked for others. To have someone know that while in most babies crankiness and refusal to eat probably indicates something benign like teething or maybe an ear infection, your heart is terrified that in your hydro baby it means a shunt failure and your child may be in brain surgery tomorrow. 

In my research I ended up finding the Hydrocephalus Association. I devoured their educational material. I read each and every success story. I sobbed watching the video about the woman who has had 20 revisions in her 25 years of life (that's 20 brain surgeries!) and whose fiancé stood right by her side as she underwent each of them. And I learned about the Hydrocephalus Association WALK. I became excited because what better place to meet the hydrocephalus community than at a walk! I searched several times and didn't find a walk scheduled in central Florida. I ended up sending an email to the address listed on the page asking when (or if) a walk would be scheduled in central Florida. A few days went by and I finally received a response that a WALK hadn't yet been started here but if I were interested in volunteering to start and chair one they would love to talk to me.

At first I laughed. Me. A full time job, a pre-schooler, and twin babies, one of whom has medical complexities. But the more I thought about it, the more I thought why not me? Often a change starts with a single voice or a single step. This is a major metropolitan area and happens to have one of the best neurosurgeons in the nation. He does over 200 shunt placements a year. Clearly there is a community here of hydrocephalus warriors, why couldn't I be the first person to take a step towards uniting us? 

When the event chair from HA called me, I listened to her closely. She explained that of the 35 WALKs nationwide, 100% of them are completely volunteer driven. The WALKs bring in approximately 50% of the Association's annual revenue which goes towards funding research for better and more effective treatments and hopefully eventually a cure. The HA provides training and helps you along the way, but the volunteers bring the walk to life and keep it running. The HA has long wanted to bring a WALK to our area but since it's volunteer driven they needed someone to commit to bringing it in and they haven't had that yet. She told me that if I was interested in getting more information after our conversation she would email me a more detailed description of everything the chair would require. She warned me it would likely be overwhelming and it was designed to come across that way because, though it was very doable, it was a commitment and they wanted us to know that. I agreed to receive the information and look it over. As I read it, rather than become overwhelmed, I felt empowered in the face of a condition that has left Mike and I feeling powerless at times. If I could help just take that first step towards getting this going, I could be part of a force that may eventually make it so no other parent had to deal with this, or no other child had to watch a parent develop this. 

Beyond just that, what a fantastic way to help bring together the hydrocephalus community! Perhaps there could be support groups offered, help with understanding hospital bills, a volunteer translator who goes along to doctor appointments and makes sure the parents or patients understand. The possibilities are endless. 

We have met several people who have hydrocephalus or are parents of children who have hydrocephalus since Nicholas was born. These are all people yearning to meet others in our shoes. 

Provided everything goes smoothly, the HA is shooting for a fall 2016 WALK in central Florida. Keep an eye out for updates!

Noggin Update and a Special Brother

Nicholas had his appointment with the orthotics company that will be making his cranial band yesterday. It was more of an introductory appointment in order for them to determine just how great his need was and also to gather all of the information they will need to get the pre-authorization from our insurance company. Mike said it was a lot of measuring and pictures and a questionnaire for parents to fill out. One of the questions that the parents were asked to answer was on a scale of zero to five how great do you believe the severity of deformity does your child have - where zero is no deformity at all and five is the most severe. Mike answered four to that question. After he was done measuring and taking pictures, Mike asked the expert what he would have answered on that question for Nicholas. The answer was that Nicholas was definitely a five. And that he was the worst head deformity the expert had ever seen. For someone in the business and who makes cranial bands all the time that was a slightly disheartening piece of knowledge. 

The good news is that we don't know just how successful this will be. We were told the band should be able to help with the flatness in the back of his head but they aren't sure how successful it will be in removing the cone shape at the back. Hope for the best and prepare for the worst seems to be the motto we live with Nicholas and he honestly has surprised us most of the time by landing on the better side of the equation so we will just step back right now and hope that this helps him. 

They have also asked us to cut his hair since he has so much of it. I'm a little sad that his first haircut will be at 6 months old but if that's what he needs to be better then that's something I'm willing to do. 

I recently took Zach out to a fun place to play since he doesn't always get to have so much fun between therapy and doctor appointments, hospital stays, and other things that revolve around having a child with high medical needs. When we got there, he was the only child (besides his siblings) there to play. About 20 minutes in, another child and his mother came in. I was playing with the twins in the baby area and Zach immediately approached the other child to play. They engaged in games and both had no problem running with the other's imagination. They were dinosaurs, and race car drivers, and train engineers, and running from snakes. After they had been playing for awhile the other child's mother approached me and asked if Zach had other friends or someone in his life with autism. I answered that he did not (to my knowledge). She looked shocked. She said he's so good with my son. She told me that Zach was the first child he had actually played with. She explained that he usually played next to other children but she had never seen him engage with another child the way he was doing with Zach. I'm not sure if he's just more sensitive, if he is meant to work with special needs children, or if maybe he just clicked with this child but Zach seems to have an ability to see beyond a "disability" and just see the person. Again I hope that's a kindness and a skill that he carries far into adulthood. 

 

Under Construction

Nicholas has had a lot of focus on his head his whole life. The reason is obvious, he has a condition that requires a little more maintenance than the average noggin. As his ventricles have been draining since the shunt was placed his second day of life, we have watched his head begin to take a concerning shape. While the size has reduced drastically, he has taken on a cone shape. 

The first time we discussed the shape with the neurosurgeon, it was determined that his back ventricles still had extra fluid and his brain was growing so there was nowhere for the fluid to go but push out against the back of the skull. This began the frustrating Easter hospital visit when the shunt was turned down drastically and Nicholas had a poor reaction. 

Nicholas's pediatrician and his therapists had all mentioned to us recently that they thought it was time to start considering helmet therapy. His therapists have mentioned that they believe that he is having trouble holding his head up in part due to the shape of his head since it is off balance. Certainly the size has a lot to do with it as well, but they feel that it doesn't help to have it be so misshapen. He is also having difficulty rolling over, though he's trying very hard, again due to the cone shape. 

Last week after a discussion with his neurosurgeon's office to get on the same page, it was decided that we would move forward with putting a helmet on Nicholas. The official term is cranial band therapy. We were preparing for a fight with our insurance and were pleasantly surprised to not find one and he is scheduled for his measurements on the 15th. 

We have been warned that there has been very little research done on putting helmets on hydrocephalus patients so the neurosurgeon's office is not sure that the helmet will be successful in reshaping Nicholas's head satisfactorily but we decided we would rather try and fail than not try at all and either always wonder if it would have helped or end up having to make him go through another surgery at some point in the future. That's not outside of the realm of possibility of course, but hopefully this can help us avoid it. 

Here We Go Again

When Nicholas had his hospital stay a month ago, we had been asked if we had ever noticed his pupil sizes were different. We really hadn't, and at the time figured that, with as many medical professionals as he had involved in his care, someone would have noticed that before a hospital stay. It was eventually decided that it was likely a side effect of the pressure changes from the big change in the shunt and that it should resolve itself.

Saturday morning, I was rocking Nicholas back and forth in my arms because he had been very fussy the whole morning and he was staring up at the ceiling. I noticed that he was tracking something on the ceiling with his eyes but that when I moved him to a certain spot, his eyes would sort of jerk back to the center. It was a very rough movement and I was a little concerned and decided to watch it. We ended up going to lunch and Mike noticed while we were there that his pupils were still different sizes (they've not been the same size that we've noticed since the last hospital stay) but that the sizes were very pronounced. One was very small and the other was very large. We googled (you should really never do that!) and found that one of the main symptoms of a brain bleed is different sized pupils. That combined with the fussiness got us a little worried.

We called the on-call pediatrician who asked us to call the on-call neurosurgeon (NSG). The NSG who had placed Nicholas's shunt actually ended up being the on-call this weekend in a stroke of luck. He told us that if our guts were saying to get him checked out, he agreed with us and suggested we head to the emergency department.

We got to the ED and the doctor came into our room almost immediately to check his eyes. She stated that one of his eyes was reacting sluggishly and was slow to constrict under light. She asked if he would need sedation for a CT, we confirmed he did not, and the nurse whisked him and Mike off to Radiology. About an hour and a half after he returned, the new doctor (somehow we always show up during shift change!) told us that while they hadn't seen anything worrisome, they wanted to admit him overnight for observation. They brought us upstairs to the Pediatric Intensive Care Unit (PICU). We asked why he was being admitted to intensive care and were told that because things can go from fine to terrible in a very short time due to his medical complexities, they wanted him under the care of a higher level unit just in case. Made sense to us.

Early Sunday morning, the NSG came in to see Nicholas before I got there (Mike was there having spent the night and getting almost no sleep). He confirmed that the shunt was working perfectly as far as he could tell but that he was wondering if Nicholas was having some seizure activity. We had been warned even before Nicholas was born that people with hydrocephalus are at higher risk for seizures and so we were not surprised by this. Worried, of course, but not surprised. The NSG suggested to the floor PICU doctor that they consult in a neurologist and order an EEG to see what was happening.

The neurologist ordered a standard EEG. I had never seen an EEG before, but it was a little unnerving to watch. First, they measure his head to determine where to place the leads. Then many leads are "glued" to his head and once those are all placed, gauze is wrapped around the leads to make sure that nothing moves. The first part of the EEG involves placing a strobe light very close to his face and strobing at varying speeds. Essentially, they are trying to force a seizure to happen so they can catch it on the monitors and the neurologist can determine the best method of treatment. Nicholas actually loved the strobe lights. He was very upset when he started the test but every time they turned on the strobe lights, he stopped crying and just stared at the lights.

We had to wait for the neurologist to read the results. Once he read the results he came and met with us. He ended up meeting with Mike because I wasn't there, but we found out that there had been no seizure activity whatsoever on the EEG! Fantastic news! Mike asked why the pupils were different sizes and the neurologist said that's actually a variation of normal and that sometimes you can see it more prevalantly from a certain angle or in a different light. We asked why the pupil had been sluggish and the neurologist said that he couldn't answer with 100% certainty but that he wasn't concerned because since being admitted, his pupils had been equally reactive, so they were unable to duplicate what the ED saw. The theory he came up with was that he believed Nicholas's shunt may have turned on and that's why the size difference was more noticeable and likely also caused the sluggishness.

We also asked about looking for seizure activity in the future. The neurologist stated that in the vast majority of hydrocephalus cases that he has observed, if the patient was going to be prone to seizures, it would happen almost immediately after the shunt placement. Since Nicholas has had the shunt for almost 5 months and since they did everything they could to try and force a seizure and he hadn't had one, he felt like we were in the clear for seizure activity in the future. Good news for once!

With both the NSG and the neurologist deciding that Nicholas had no issues that required intense follow up care, Nicholas was discharged. We were told by his nurses that they loved him but they really didn't want to see us again and we echoed the sentiment.