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!