Checking Off Milestones

In all of his recent evaluations, Nicholas has been determined to be about eight months behind in his physical development. He's maintained a steady eight months behind in development level, which is great because it means, while he's behind his "peers", he is staying on his own track and isn't falling behind.

He is a few months behind in cognitive development, and is actually age appropriate, or even advanced, in social skills (which anyone who knows him will tell you - our giant flirt).

So, even though he's currently eighteen months old, he's technically considered seventeen months old developmentally since he was born a month early, and he's measuring at nine to ten months in his motor skills development level.

As I've mentioned before, Nicholas isn't crawling or walking yet, and we honestly don't know when that will happen. We're fairly confident that it will - he's proven himself to be a singularly determined individual, but it will be late. Many people with hydrocephalus, especially at the severity that Nicholas had, will never walk, so we're grateful that he likely will, even if late.

He's been sitting up on his own for some time now, as long as someone helps him into a seated position. His core strength has grown that he now sits for the entirety of a taekwondo class (while playing with the other babies), and it doesn't exhaust him. Early Saturday morning, I was up with all three kids. Zachary was watching cartoons, Emily was playing, and Nicholas was practicing getting into his crawl position. Suddenly Zachary says, "Mom! Look at Nicholas!" I looked over and Nicholas was sitting up next to the entertainment center, trying to get the DVD player. I said, "Oh wow, that was nice of you to sit him up, Zach!" Zachary said, "Mom, I didn't!" I asked him if he was being honest, did he really not help Nicholas at all? Zachary swore that he hadn't helped Nicholas up. Which left the conclusion that Nicholas had gotten himself into a sitting position for the first time ever. We watched him throughout the morning and it never happened again.

Until nap. We went to get him after his nap and he was sitting up in the crib. We knew at that point that he had to have done it himself. That night when we went in to feed him his 9 p.m. feed, we found him sitting up in the crib instead of sleeping, laughing and incredibly proud of himself. Since then, we've found him in a sitting position several times, though we've never actually seen him put himself there.

This little guy continues to amaze us, and we are so proud to be able to watch this journey of his!

Eye See

After several weeks of patching, Nicholas followed up with his ophthalmologist where we learned that his patching regiment had done next to nothing to correct his eye. His left eye was still turned in pretty severely and the ophthalmologist was concerned that if we waited much longer, the possibility that Nicholas could lose his vision was high. Basically, because the signals coming from his left eye did not match with the signals (images) from his right eye, it would be possible for his brain to turn off the left eye to reduce confusion. In that case, the left eye would stop working and he would be blind in that eye. So, the doctor wanted to schedule the surgery.

He wanted to wait to hear from Nicholas's neurosurgeon first, so that we could be certain that the pressure inside Nicholas's head was within normal ranges and that pressure changes weren't causing the issues with the eye, and luckily, we just happened to have the annual follow up/ CT scans/ shunt series scheduled. As I blogged about earlier, that went very well and Nicholas was labeled stable by his neurosurgeon office - unless something happens (everyone knock on wood!), they would see him in a year. With the all clear from neurosurgery, we contacted the surgery scheduler at the eye doctor and Nicholas's fourth surgery was scheduled.

Today was his pre-op appointment and he was deemed fit to move forward with the surgery. Again, we have lucked out by getting matched with one of the best physicians in his field in Orlando. The eye doctor works specifically with children with neurological issues and has been performing this operation for over 20 years.

This is quite a common procedure, and also a fairly easy one (compared to the brain surgery and g-tube placement/nissen surgery he's had previously), but of course, it's still surgery. The ophthalmologist will be making a small incision on either side of his left eye. On the inner part of his eye, where his eye turns in, the muscle will be detached and reattached further back. The muscle on the outer side of his eye will be either folded and stitched to increase tension (the preferred method) or will be shortened and reattached. This will hopefully cause the muscles to pull his eye back into the correct alignment. Many people we know have either had the surgery themselves or have children who have, typically with fairly good results. The surgery has approximately an 80% chance of correcting the alignment issue with no further issues. It's possible to over or under correct, in which case we would try patching again and if that doesn't work, he would need to have surgery again. We're not going to look that far into the future though - we're going to just look at next Friday. He's scheduled to arrive at the surgery center at 6:15 a.m. on Friday, July 1st. The surgery will take around an hour (unless - in true Nicholas fashion - something strange comes up), and he'll be home that night. Everything I've heard, it's likely to be one of the easiest surgeries he'll have.

Thoughts, prayers, good wishes, whatever floats your boat are always appreciated. Send a little thought towards Orlando next Friday morning!

Who's Counting?

During the first few years of a child's life, you spend a LOT of time visiting with their pediatrician. Even completely healthy babies get seen frequently for check-ups, vaccinations, and sick visits (even babies who stay home - have to build that immune system!). You become even closer to your pediatrician if your child has any kind of a medical issue whatsoever.

During each of these visits, as all parents can tell you, the baby is weighed and their length and head circumference is measured. These go on a chart as little dots and you have a brand new thing to stress about. Is my child's weight/height/head circumference normal? Is it too high? Too low? You leave the office in proud possession of their latest percentiles. Percentiles are rarely anything to be concerned about. If your child is in the third percentile, it just means that 97% of babies are bigger than him or her at this specific moment in time. It only becomes concerning when a baby doesn't follow their "curve." Emily, as I'm sure you can guess, tracks very low in her percentiles because she's a tiny little peanut. She is, however, on the full term growth charts as opposed to the preemie chart even though she was technically a preemie, so that's a tidbit to be excited about.

Nicholas tracks along his charts fairly well, with one exception. I'm sure you already know the exception is his head circumference. He was born with a head circumference of 46 cm. The "average" head circumference for a newborn is 36 cm, so you can see just how large his head was when he was born. He went as high as 47 cm while in the NICU, but some of that can be attributed to swelling after surgery along with human error (the nurses don't always place the tape measure in the exact same spot). Nicholas has always had a large head for his age and he's always been so far off the chart that it would be absurd to assign a percentile to his size. He's in the 175th percentile for age??? It's meaningless.

Obviously, unless the circumference shifted dramatically larger, his pediatrician was aware of his head circumference and was not overly concerned. The thought has always been that at some point, his body would catch up with his head and he'd start following along his growth curve.

Recently, he was seen for his 15 month (15 month????) check-up. He has recently made some significant leaps forward in his progress and we were excited to talk to his pediatrician about it. We've started adding real food to his formula diet. We're currently using fruit and vegetable purees and adding them to his formula and feeding it through his g-tube. We're still thinking that he'll eat by mouth one day (we don't know what day, but one day), and we'd like his body to be used to "real" food when it happens. His head size has also gotten smaller as I mentioned in a recent blog post. He's sitting up far more frequently and has started rolling over onto his belly and trying to push himself up into a crawling position.

While at the check-up, the standard measurements were completed. He's staying right on his growth curve for height and weight. And for the first time in his life, he is on the chart in head circumference. His body has finally started catching up with his head size. He's in the 97th percentile, true, but he's on the chart. It was really cool to finally see that dot fall in line with other babies his age. And it happened far sooner than we thought it would - we'd been told previously it would probably happen around age 2 or 3. Clearly this kid is an overachiever.

Changes

When I was in the training for the new WALK chairs in Charlotte recently, a chair of another WALK and I connected. We were sitting next to each other at dinner one night and talking about how hydro affects the lives of our loved ones and she asked me, "Would you change it?"

We're supposed to say no. We're supposed to say that we love every part of our kid and would change nothing. And you know what? Maybe there are parents out there that wouldn't, but for me, I say hell yes, I would change it in a heartbeat if I could. If I could have this same sweet amazing boy that we've got and take away the difficulties that hydrocephalus has brought and will bring to his life? I'd do it. Immediately.

Without hydrocephalus, he would probably be able to eat by mouth. We'd be off formula by now and working on learning how to use a spoon, fork, and sippy cup rather than focusing on how to swallow without flooding his lungs. He would more than likely be sitting on his own, maybe crawling, and probably be trying to keep up with his little sister who decided in basically one day that walking was the way to go. He wouldn't have already had three surgeries in his short 14 months and the likelihood of future brain surgeries (which is almost certain right now) would be slim to none. His biggest worries would be keeping up with a big brother who plays a little rough and a big sister who loves him so much she's constantly hugging and kissing him.

Would I change Nicholas's story so that it was without hydrocephalus? Without a doubt. The truth is, though, I don't focus on that. Because I can't. There's no cure for hydrocephalus and Nicholas was born with it. So we focus on making sure that hydrocephalus doesn't hold him back. That he will hopefully one day be able to safely eat by mouth. By getting him amazing providers who work with him so hard to get him to sit up on his own and one day crawl and hopefully walk. Who are teaching him how to use his mouth to say words. We watch intently for signs of shunt failure so we can get him help the second he needs it. We figure out the best way to store his medical supplies and teach care givers signs of shunt failure to look for and how to hook him up to an extension to feed. We learn the ins and outs of the medical world, and a whole new vocabulary. Revision, Mic-key tube, nissen fundoplication... We love him deeply and try to show him (and all of our children) just how loved he is. 

New Year, New Updates

I've been terrible about updating this blog, but I'll try to be better - especially with all the exciting things going on!

Nicholas is, and has been, very stable. (Knocking on wood right now!). We've had one hospital trip recently and it had nothing to do with his hydrocephalus and everything to do with a user (ahem mom) failure in trying to remove his g-tube button. I couldn't get the syringe inside the spot to release the liquid out of the balloon. I tried several times and since Mike wasn't home to try, I decided to just take it to the experts. The ER had the button out and fixed very quickly.

We have noticed that Nicholas has been having several breakthroughs recently. He's getting much better about sitting up on his own, if we stand him upright he will plant his feet down and straighten his legs until he's standing with assistance, he has started trying out words (ball! mama! dada!), and we've started to feed him baby food along with his formula through the g-tube so he can get nutrients from real food.

I was in Las Vegas for work about a week ago and Mike called me. He had just left the office after Nicholas's appointment with his helmet specialist. When he initially was evaluated, the asymmetry of his head was at 26 mm. At the meeting last week, Nicholas had gotten his measurements done. His asymmetry measured 9 mm. 9. Less than 1 cm of asymmetry. And, his head had gotten smaller than it ever has been before. The helmet specialist told Mike that if this trend kept up, he thinks Nicholas will be discharged from his helmet in April. This is the child the PA in the neurosurgeon's office wanted to not even give a prescription for helmet therapy for! He thought it would be pointless.

Other things -
I went late in January to my Hydrocephalus Awareness WALK chair training. I came back ready and willing to hit the ground running. I'm so excited! I have two fantastic co-chairs who are just as excited as me, we've already started garnering sponsors, and I'll be announcing our location and date within the next couple of days! If you haven't liked our page, please do so ASAP! You can also check out our WALK website or Nicholas's personal fundraising page. We'll be fundraising for the Hydrocephalus Association in his name for the WALK. People can join his team and fundraise for that team as well, create a new team to fundraise in someone else's name (or just a fun team of your making!), or WALK virtually if they can't attend the local WALK. Lots more information to come about helping out in that direction!

As for the other people in our family, Emily started walking about a week and a half ago. She took a few tentative steps and was at that point when I left for Vegas. By the time I got home, she was fully walking. It happened very fast. I was very worried because Zach was an early walker (he was pretty much walking by 10 months), but again I've been taught by my kids that things happen on their own timeline and when they're ready, it's go time. Zach is still doing fantastic at taekwondo, he has a tournament in April and he's going to compete in sparring for the first time ever. His instructor swears he's ready, and I believe him. Zach absolutely loves his sparring class. I have to admit I was really nervous for him to start sparring, it seems so violent for such a little guy. But, it's points sparring, so the idea for the match is to get points, not knock out your competitor. Of course, it's possible that he'll get kicked or punched the wrong way and get hurt, but frankly he could get hurt playing soccer or baseball as well, so we just learn the safe and right way to do things (learning defense is a HUGE part of class) and try to minimize the risk.

Again, I'll try to be better about updating the blog. I know that I've done such a terrible job lately.

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 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 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!

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.

On This Road

There's no way to describe the way you feel when the doctor tells you, "We see something wrong," while looking at an ultrasound. Your heart drops. You grasp your husband's hand, you stare at the grainy images they're showing you, trying to make sense of light and shadows. When those words "We see something wrong" includes the word brain, it gets really scary. A big long name for a condition we had never heard of. Hydrocephalus. Prognosis is typcially good. You cling to the word typically but live in fear of the atypical. Of the outliers. Of the ones who don't have a good prognosis. You vow to help him fight, to help him live the best life he can.

The day he's born. You barely see him before he's whisked off to be cared for by someone else. Your child being placed into someone else's arms. Someone else leaning in close and providing warmth. You're laying in a cold operating room with no answers. Your heart pounding, the worst case scenarios running through your head. Asking the doctors for answers and being told they have none yet. You'll get them they promise but not right now.

Standing in the NICU next to his bassinet and hearing "Not swallowing the way he's supposed to. Swallow reflex not working. Probably some damage to his brain. Hydrocephalus." Suddenly your vision of your future, and his, comes crashing down around you. The future you've always envisioned is raising happy children that leave and live lives with their own families, their own careers. Brain damage may change that future. Certainly you'll love him with everything you have forever, but that future may include him by your side for the rest of it. And what to do when your future ends but his keeps going? What happens then? Your resolve to fight slips a little. It's a scary time to hope, but what choice do you have? You start therapies. You agree to another surgery. You do everything the doctors and nurses tell you because it will help.

It's terrifying to go through. It breaks your heart because all you want is for your child to be happy. All you want your child to know is love and joy. And while you know that's not possible for his whole life, you'd really like to think it is possible in the first three weeks. And you feel guilty that your child has to know pain. And fear. And be poked and prodded. And x-rayed. And have ultrasounds. And have a PICC line placed. And you know that all of this is to help him. But he doesn't know that. He has no clue.

Then one day he does this.

And two days later he does this.

And you compare this.

And you start to feel sparks of true hope. And you start to allow yourself to consider that word typically again. Typically good prognosis. And you realize that what they told you in the NICU, "Nicholas hasn't written his story yet. He's writing it every day. There is nothing set in stone." is being proven to you by leaps and bounds. By a baby who doesn't know any better than to fight. And your resolve to help him fight that you thought you had lost? You realize it has been there the whole time and has become so ingrained that you don't have to think about it anymore. And you realize that nobody's future has been written yet and it is never never wrong to cling to hope.

One Step Forward Two Steps Back

Well, as soon as I hit "Publish" on that last post, things changed. Of course. Because if we've learned anything from these almost 4 months of Nicholas's life, expect the unexpected.

After the shunt was turned down on Wednesday, Nicholas was immediately fussy. He wasn't tolerating his feeds very well. He just wasn't acting like himself. We put a call in to his nurse and she said this was typical behavior for someone who just had the pressure in his head changed. It's a big change and it makes them feel yucky for awhile. Basically, he could be agitated and he probably wasn't tolerating the feeds as well because his belly was full of the cerebral spinal fluid the shunt was rapidly draining from his brain. If it continued past the weekend, let them know.

We had previously noticed a slightly hard spot on Nicholas's belly right between the two incision spots (one from the shunt placement and the other from the g-tube placement and nissen). We had mentioned it to his pediatrician the previous Friday, and they agreed it was slightly abnormal but that they weren't concerned and we would keep an eye on it.

Friday, Nicholas had his speech and occupational therapies. We had noticed the spot on his belly had grown and was now protruding. Mike felt Nicholas's belly after therapy and discovered it was rock hard. Nicholas had been inconsolable all night Thursday night and most of Friday morning. Since the neurosurgeon was in the same office building as the therapy, Mike decided to head up to the fifth floor and see if they could take a brief look at him. Both Nicholas's neurosurgeon and his partner were in surgery, but the medical assistant came out and looked at Nicholas. She became very concerned about his belly and asked Mike to take Nicholas across the street to the emergency department immediately.

Mike got into the ER very quickly (our hospital has a separate children's ER) and I left work to come over as well. A CT scan, shunt series, and abdominal ultrasound was ordered. As soon as I got to the room, Mike and Nicholas left for radiology. I stayed in the room with Zach and Emily. The CT and shunt series came back normal and showed the shunt is continuing to work very well. The abdominal ultrasound showed what appeared to be a cyst in his belly and the decision was made to admit him to the hospital to see if the cyst could be drained. They also ordered a CT scan of his belly to get a better picture of what was going on.

Mike ended up staying Friday night with Nicholas while Emily, Zach, and I went home. Not much happened overnight except that he did not tolerate his feed at all. Mike only got 60 ml (half of his normal feed) in while he screamed. After midnight, he was not allowed to eat anything in preparation for the cyst to be drained in the morning.

This morning, we received a visit from the PA in the neurosurgeon's office. She felt confident that the shunt was not the root cause of the issue and wanted to consult in general surgery to see if they felt the catheter of the shunt that lays in Nicholas's belly needed repositioning or if they felt the problem was something else. After awhile, the surgery resident came in and told us that after further review of the belly CT, they felt it was a pocket of CSF that had gotten stuck and appeared to be subcutaneous and therefore shouldn't be causing pain or the intolerance of feeds. He stated that his gut indicated that it was something else and he wanted to get the surgeon to review the tests and they would be back, hopefully with a plan. We were prepared that he may be scheduled for an exploratory laparascopy.

The surgeon came in and examined Nicholas. He agreed that the spot on his belly was likely not the cause of the pain and feed intolerance and ordered an upper GI scan to make sure that there wasn't a blockage somewhere in his digestive system. Nicholas was whisked away to radiology again. He was given barium with contrast through his g-tube and pictures were taken. Then they waited a half hour to complete some more pictures - they were trying to see how well the contrast moved through his digestive system. The answer came back that he has no obstructions in his digestive system. But...we still don't know what is causing the issue.

When he came back, his nurse came in to complete vitals and asked us if we had ever noticed his pupils were different sizes. We had not, and were pretty sure if they were, some medical professional would have noticed by now, as many as he had seen. The floor attending was called in to look and confirmed that one pupil was significantly larger than the other. The neurosurgeon's office was paged again and he was placed on full monitors. They weren't overly concerned, because he had no other symptoms that indicated potential neurological problems, but they wanted to watch him on full monitors just in case.

We asked about getting him fed, because at this point it had been 18 hours since he last ate. The nurse asked the floor attending who said we had to talk to the neurosurgeon who said that we had to talk to the general surgeon. The general surgeon approved him for feeds but suggested that we slow his feeds down from 4 ounces every four hours to a very slow continuous feed over 24 hours. We said okay, sounds good to us and the nurse laughed. She said that order has to come from his pediatrician so we have to page them. Finally, he was started on a continuous feed of 30 ml/hour for 24 hours.

That's where we stand right now. We don't know what's wrong with him, though there have been plenty of ideas tossed around, from a kink in the shunt catheter to the idea that perhaps the shunt catheter has grown into the scar tissue from the surgeries he's had. The honest answer is that he will likely require surgery again for them to get in there and figure out what's wrong. And that sucks. That sucks so bad for our little guy. It sucks that he's had to endure so much in the short 4 months he's been here. The good news is he's little enough that though the memories of this will be forever burned into our minds, he won't ever remember this. Hopefully we'll have figured out the big issues by the time he is old enough to remember and hospital stays and surgeries and radiology will be something he knows very little about.

I'll update when we know more.

It's Not Brain Surgery

Nicholas has a big head. We've established that. He also recently has a cone shaped head. We've talked to his pediatrician about it and have been trying to figure out what's going on and how to fix it.

Because Nicholas wasn't cleared for tummy time (freshly placed g-tube probably not too comfortable), the thought was that his head was flattening out because he spends a large amount of time lying on his back. Since fluid is easier to move around than brain matter, the thought was that the bones of the skull may have shifted. We were first trying to rectify the situation by positioning his head different ways while he slept or laid on the floor in his play mat or on his pillow on the couch. That didn't seem to help. Then he was cleared for tummy time. He haaaaaaaaaaaaaaaates tummy time. It is like a nuclear war happening here when Nicholas is doing tummy time. The next thought was that with the addition of tummy time and the re-positioning of his head, we will be able to get the head to a "normal" shape. 

None of those ideas worked and his cone head seemed here to stay. At his last pediatrician appointment, the doctor suggested it was time to meet back up with the neurosurgeon who had placed the shunt and see if he wanted to give Nicholas a helmet to help re-shape his head. We had been prepared for this since before birth, so it wasn't really a surprise for us. We had originally been told they would look at putting him in a helmet after six months, so it made us a little nervous that they were looking already, but if it's what is best for Nicholas, we will move forward with it.

The neurosurgeon required a CT scan to see him about the helmet, and since he was going into radiology anyway, the neurosurgeon requested a shunt series as well. He had both the CT scan and the shunt series Monday. Mike took him in at 2 p.m. and the nurse at Nicholas's pediatrician arranged for a child life specialist to go back into radiology with him since I was at work and Mike had to stay in the waiting room with Emily and Zachary. I met Mike at the hospital after I got off work at 3, we traded cars so he could go to work and I have the car with the car seats, and I went back into the radiology department to wait for Nicholas to come back.

Wednesday, Nicholas saw his neurosurgeon. The neurosurgeon told Mike that everything on his CT scan looked fantastic and he doesn't think that Nicholas needs a helmet at this point. The reason that Nicholas's head is "coning out" is that his brain is growing (yay!) and the shunt isn't removing the cerebral spinal fluid fast enough, so the CSF is being pushed out anywhere it can go - and that just happens to be out of the soft spot in the back of Nicholas's head, creating the cone shape. The good news is this is a fairly easy problem to rectify...we hope. We have found that sometimes things that are "fairly easy to rectify" cause Nicholas to have other issues.

The way the shunt works is that it is set to detect the pressure in Nicholas's brain. When the shunt detects that the brain has gone above that set pressure, it turns on and drains fluid until the pressure is back under what the shunt is set to and then the shunt turns off. When the shunt is set, the lower the number on the shunt the more it will drain. Basically, adults typically have a pressure of 100. That was Nicholas's initial setting. They set his shunt very conservatively initially because they wanted to remove CSF but they didn't want to do it too fast and cause the brain to pull away from the skull and cause a brain bleed. After a few weeks of draining at a pressure of 100, they lowered his pressure to 70, which is a typical pressure in infants. His shunt has been at this pressure since. On Wednesday, they turned his shunt down to 50. They warned us he will have so much pee as his shunt drains and also that he may be irritable. Hopefully this will fix the issue of the CSF oversupply.

You're So Strong!

If "You've got your hands full!" is the most common thing we hear, "You're so strong!" or "I don't know how you do it!" are not very far behind. I take a step back and look at my family to see what they see.

The three year old? Certainly a three year old is a force to be reckoned with. You've never understood the term threenager until you've had one. Some days everything is awesome (does anyone else sing that phrase? Thanks Lego Movie!) and some days the world is ending because we used the wrong color plate or his favorite cartoon is on or the milk is too white. Yep. Those days take some inner reserve. 

The twins? Well they can be overwhelming. Who do you comfort first when they're both crying and only one parent is around? What about when they both need a bath? What if you're changing a diaper and the other one starts melting down?

A medically special needs child? Yeah this one can be tough too, though at this point we have everything about his care down to almost a science that it's a little less overwhelming. Except for times that he throws curve balls at us. Like the other night when we were at dinner and Mike was gravity feeding him and Nicholas was NOT happy and was having such a melt down that somehow his milk actually came out his nose. We aren't sure how that happened. His nissen is supposed to prevent that. He must have exerted some serious force to bypass that. 

So yeah. I guess we have three pretty overwhelming situations all squashed together into one family. But...it's our family. And really, what choice to we have BUT to do it? We can't stop caring for them. We can't not provide for their needs. So we just figure out ways that work for us and keep on keeping on. 

I laugh when I hear that. I appreciate the compliment it implies, but then I think about the families I used to think that about. Another family with a toddler and multiples (if only I could have seen the future!). A family with a toddler with cancer fighting for his life. Another family with more kids than I thought I wanted. And now I know. Yes, it takes great inner reserves of strength and patience but when it's your life you don't really notice yourself pulling up your bootstraps, you just DO IT. And I know that, faced with whatever difficulties lie ahead, any of my friends or family would do the very same thing. Just roll with the punches and figure out what to do next. It's what any good parent does.