The Week of Misery & what became of it

 After the week we've had, and the week we're about to have, it was time for an update.

Ally recovered well from her shunt revision in March.  Dr. Apfel took her out of school for 2 weeks, but by the end of week one she was going crazy.  She would email her teachers, who would let her know what work she needed to do to stay caught up.  Sometimes Ben brought things home to her and delivered work back to the school.  The last few days of her second week out were during spring break.  On that Wednesday we had her 2 week follow up appointment with Dr. Apfel in clinic.  She had had an MRI done the previous week to be able to make sure everything looked okay from the surgery.  Dr. Apfel said that everything looked fine, including both incision sites, but her ventricles were measuring small.  She wanted to adjust the shunt setting from a 4 to a 5 to allow it to drain less and increase the size of the ventricles.  So we did, and we left.

Ally went back to school the following week.  She did fine and had no issues.  On April 8th she was cleared to go back to sports and gym class, which she also did without any issues.

I was getting ready to make a new blog post about her recovery, but wanted to wait for one last appointment.  Since they adjusted the shunt setting, Dr. Apfel ordered another MRI to get images of what the brain looked like on the higher setting.  We did this on Wednesday April 29th, saw Dr. Apfel in clinic afterward, and went on to live happily ever after.

Until Sunday at 3 AM.

I woke up hearing Ally wimpering in her room.  I went in to ask what was wrong and she said her head was hurting.  I gave her tylenol and laid in bed with her, but over the next hour her pain increased and got more and more intense.  I finally asked her what she wanted to do, and she wanted to go to the ER.  So off we went.

We got to the ER a little after 4 AM.  We were taken back to the pediatric unit and went through all the usual stuff- seeing the ER doctor, then they consult with neurosurgery while giving her something for the pain, and then getting an MRI and shunt series X-rays.

We were there for 8 hours doing all of this.  As we were there, her headache got better, so with nothing showing up on the imaging we were sent home.  I told Ally that next time, we would just stay home and ride out the headache, as there was an eventual end to it.

We didn't have to wait long.

The next night I woke up the same way around 2 AM.  I spent the next 5 hours trying to help her manage the pain.  Honestly, I felt like I was coaching her through labor.  Her pain would ramp up and she would want to cry and yell, which I would remind her to breathe instead, and then it would die off, only to start again 20 seconds later.  She also vomited a number of times through the night.  By morning, we were both exhausted.  I got a sub, got the boys up, had Luke drive himself and Ben to school, and went off to school myself to get my sub plans together.  By the time I got home around 7:30 Ally was asleep.  

At 8 I called the neurosurgery clinic and explained what had happened.  I asked if there was any way we could come in today to get her shunt adjusted back down to a 4.  My theory was that moving the shunt up to a 5 to drain less was causing the pressure to build up quickly, causing the intense pain, and then taper off when the valve released the fluid into the tube.  I hoped moving it back to a 4 would solve the issue.

Dr. Apfel wasn't in office that day or the next, however, the nurses and staff there recognized the urgency (it didn't hurt that I called several times) and got in touch with her at home.  She agreed that adjusting the setting should happen, and we were sent to the clinic to see a nurse practitioner in neurosurgery to adjust the shunt back down to a 4.  She felt some relief right away, though still had an underlying headache but nothing like she'd been having.

She slept through the night Monday night, so the next day I got her up to go to school.  However, she did complain that she still had some pain.  Since she was scheduled to take an SOL that day, I decided to leave her home in order for the pain to subside completely before heading back in.  Luke did not have to be at school until a little after 11, and Russell's parents came over for the rest of the time to be with her.

During the day she had pain off and on, but did ok.  We had an appointment scheduled with Dr. Apfel the next afternoon since she did not do the shunt adjustment, just so she could see everything for herself in person.

The night was ok, Ally got us up a few times with pain but we were able to give her tylenol or ibuprofen and she would go back to sleep for awhile.

The next day, since she still had pain and had the afternoon appointment anyway, Ally stayed home again with Luke, and then I took a half day and came home to take her to the appointment.  When we met with Dr. Apfel, we told her that she was still having pain and nausea.  Dr. Apfel said that she wanted to try to control symptoms, like nausea, dehydration, etc. because that may be what was making her headaches so bad.  We got Zofran for her (she actually took it in the ER Sunday morning, so I thought there was some merit to this idea because that headache did go away eventually) and I was determined for her to stay hydrated and try to eat some and get rid of the nausea with the Zofran.

The night was not great, so she stayed home yet again.  It was my day to travel between my 2 high schools, so I stopped at home to check on her.  When I got there Russell and his parents were all there with her, and she was in pain yet again.  I left to go back to school but called neurosurgery, asking them yet again to let her come in and to move her shunt down again to a 3, as that was the only thing I could think that might help.

Russell took her to the appointment.  While there Dr. Apfel not only moved it back to a 3, but tapped the shunt as well.  Tapping the shunt can do 2 things- 1. It can help you test the pressure in the head and determine if the shunt is working- it was; and 2. Pull out fluid to be sent to the lab.  Dr. Apfel sent some fluid to the lab to test for infection that she was suspicious might be present and said she would let us know the results hopefully that evening.

Right after school I had an event to go to.  I was gone from 4 until about 9.  During that time Russell texted me that Ally was warm, and when he took her temperature she had a fever of 101.  Toward the end of my evening, he texted that she was asking for me.  I was leaving anyway, so I went straight home.  

After being home for about 20 minutes, I got a call from Dr. Apfel that the fluid definitely showed infection, though the cultures had not come back yet so they weren't sure what exactly the strain of bacteria was.  She wanted her to start antibiotics right away.  Being 9:30 at night, we had to have them called into the hospital pharmacy since no retail pharmacies are open that late.  I went and brought them home and started her on them right away.

She had an okay night, woke up a few times with pain but nothing too crazy, so I was hopeful that the antibiotics, once given some time, would clear everything up and she'd be good to go.  She could get through 4 out of 10 days of the meds by Monday morning and be ready to join the world.

Saturday morning at 4 AM she woke us with headaches and nausea again.  While she was with Russell and I in our room all of a sudden she kind of stared off into space with a funny look on her face.  When I asked her what was wrong, she said things looked 'funny'.  I said was it blurry, and she said no, it was kind of foggy, but not foggy, just really weird.  I knew right then that we had to get back to the ER pronto.  Vision changes are a big sign of shunt issues, and she had never experienced this symptom before.

We got to the ER around 5 AM, went through triage and back into the familiar pediatric ER unit. (4th visit in about 5 months!)  It took awhile for them to get over to us, while she laid in the bed moaning and groaning.  I went out twice asking if/when someone was coming to see us, and finally the doctor came in, apologizing because he was getting caught up on her history/charts. (I mean, we went to neurosurgery 3 times that week and had been in the ER not even a week ago, not to mention the revision in March, so...yeah.  Fair.)  He asked questions and did an exam and told us that neurosurgery was aware we were here and were on their way down.

After a bit, a resident came in and spoke with us.  Dr. Apfel was not on call that day, it was her partner Dr. Kline.  From everything they had done for her over the past week, it appeared to them that the infection had really taken over.  The shunt was infected and therefore had to be removed.  

We met Dr. Kline as well.  I can't remember which doctor told us what was going to happen, but it was extremely unexpected to me.  All surgeries have a risk of infection- as much as they try to make ORs sterile, bacteria and germs are just everywhere.  For whatever reason, shunts have one of the highest rates of infection of all surgeries- 7%.  Ally rolled the lucky dice on this one and her shunt became infected.  Not only that, the fact that the cultures took so long to grow suggests that it's a very slow moving bacteria- 'smoldering' as Dr. Kline put it- no obvious signs like pus drainage, redness at the incisions, etc.  However, the infection had become bad enough that the shunt would need to come out in order to get it under control.

However, you can't just remove it and put in the new one right then and there- that would be pointless, as it would immediately become infected as well.  So, we are in a 3 part series to wellness at this point.

1. Remove the infected shunt.

2. Put in an external brain drain (I'm sure there's an official medical term for this, but this is what I'm calling it) while antibiotics are given and the infection clears.

3. Put in a new shunt (and hope this one does not become infected).

Stage 1 was completed early this afternoon.  Dr. Kline did the surgery to remove the shunt from March- all 3 pieces, the catheter into her brain, the valve outside her skull, and the tubing going down her head and neck and into her abdomen.  She did great as usual, and this surgery did not take long at all (about an hour).  They did not have to make an incision in her abdomen, just her head since they were simply removing all the pieces.

Stage 3 will be completed in a week or so, once the infection is clear with antibiotics.

This means we are now in Stage 2, which honestly is probably the worst of all (though the other 2 are certainly not fun).  Having an external brain drain means that she cannot leave the hospital- it requires specialized nursing care.  It has to be monitored at all times and she has to be very careful when changing positions and checked each time to be sure the pressure is still ok (though she is allowed to get up and sit or walk, she just has to be helped by a nurse).  The fluid has to be collected each day and tested, and when it is clear of infection she will have the new shunt placed.  Also, being in the hospital allows her to have IV antibiotics, which will work much faster that taking them orally.

So, here we are, in the PICU.  She will be here for a week give or take, when she's cleared for surgery to have the shunt put back in and she's not wheeling a drain around everywhere she goes like an IV pole.  She is truly a trooper and is doing so great.  She was able to eat some dinner tonight- almost a whole hotdog and a slice of red velvet cake (she has not been able to eat much all week with the nausea).  She's been joking with the family and the nurses some as well.  She really tries not to complain too much and is always thanking the staff who help her and bring her things.  We only have 13 days of school left, and with a week in the hospital and then 2 weeks of recovery after shunt placement surgery she won't get to go back.  She's incredibly sad about not seeing her teachers and friends, as well as missing out on some fun end of the year activities, like an ice cream party in math and choir Olympics (which she was apparently a team captain) and end of the year banquet for both softball and choir. 

I will definitely be putting up a new update as we move through stage 2- so stay tuned, and thanks to all the family and friends who have reached out and are praying for her.  We are truly thankful.

The Revision

 Well, it's been 7 years...let me catch you up!

Ally is doing amazing!  She just turned 12 and has started middle school.  She loves school and is doing super well!  She joined choir for her elective, which she has enjoyed immensely.  She also loves softball- she started playing baseball around age 4 and switched to softball around 9 years old.  She plays for her school this year and also has been playing travel for 2 years.  She joined Girl Scouts in 2nd grade and has really enjoyed girl scout activities and summer camps.  The past 4 years she has sold over 1000 boxes of girl scout cookies for her troop, earning her pearl status!  She loves to talk and be social, she has lots of friends and has never met a stranger.

Dandy-Walker & hydrocephalus-wise has been fairly uneventful the last 7 years.  At this point, Dandy-Walker has become almost non-existent in her life.  She doesn't have any developmental delays or anything else as a result of Dandy-Walker.  In fact, the only thing Dandy-Walker has done for her at this point is caused her hydrocephalus.

Ally has had the same shunt since the original placed in June of 2014.  She's had several x-rays and MRI's over the years.  We stopped doing annual checks with neurosurgery, since there was never much to discuss- she was doing fine, so it just seemed a waste of time and money.  We were told if she was having any immediate issues to go to the ER.

We had one scare in October of 2022.  Ally would wake up several days complaining about really bad headaches.  Finally one morning she complained so much I decided it might be time to get checked out.  Being only 8, I asked her if she really felt this was bad enough to need to go to the hospital.  I tole her it's okay if it was, but that this was serious and she needed to tell the truth.  She said it was so off we went.  She got an x-ray and MRI, but even as we were in the hospital she said the headache was going away.  This time turned into a false alarm.

This January, Ally started complaining about headaches again over several days.  Finally, one Thursday night she said it felt like pressure behind her eyes.  That was it- I took her to the ER. 

We did the usual, x-rays, MRI, etc. and they still didn't see any swelling of the ventricles.  The only thing the pictures showed was that the tubing from her shunt had come apart right around her collarbone.  Apparently this is very common.  Shunts and their tubing can have scar tissue form around it the longer they are in there, and as they grow it can pull and then disconnect.  She even had a shunt tap, where they put a needle into her shunt and try to measure the pressure and extract some fluid.  They weren't able to get much fluid at all.  Ally's original neurosurgeon is retired, so the new one, Dr. Apfel, said she didn't want to do unnecessary surgery and so we ended up leaving around noon the next day.  We made a follow up appointment with her one week later.

At the follow up, Dr. Apfel said there was a test we could do to see how/if the shunt was working,  They would inject a dye into the shunt, then wait a bit and take images to see if the shunt was flowing or not.  She said there was a chance she could be shunt independent, meaning her body had adapted and she no longer needed the shunt.  This would show if the dye did not flow through the shunt.  However, if it flowed, she said she would want to do a shunt revision since the tubing was disconnected.  I left the decision up to Ally, and she opted to do it, even if it meant surgery.  So I scheduled it for March 18th, because that was an easy day for me to take off work.

On March 11th I dropped off the kids at school like normal and went to school myself.  Partway through my first class I got an email from Russell asking me to call him.  He told me that he got a call from the school nurse at Ally's school, that she was in her office and was inconsolable, complaining of a headache.  When I called and asked to speak with her, she couldn't even talk to me, she was crying and yelling so much.  I knew I needed to come get her.  I arranged to leave school and went to get her.

When I got there, she was on the floor of the nurse's office, somewhat calmer and with a trash can in front of her.  The nurse said she felt nauseated.  As I got her out of the school and walking to the car she did get sick.  I knew right then that she was in shunt failure.  Vomiting for no other reason is a sure sign of shunt failure, and she'd never had that before.

We went straight to the ER.  I was worried about how long it would take (in January everything took a very long time) but we got through relatively quickly.  The hospital had been undergoing construction, so even in the 2 months since our last visit things had changed and there was now a nice drop off area for the emergency room instead of parking in the special underground garage first and going up to the ER.  I let Ally out, clearly in pain (she moaned and groaned the whole time) and the staff that met us parked my car for me so I could escort her in since it was just the two of us.  She found me in triage and gave me my keys and a parking pass with my car's location on it.  We got through triage relatively quickly this time, and got taken back to the pediatric area.  There they put in an IV and she got a dose of fentanyl (which even in her state of pain she recognized and questioned, but the nurse assured her it was safe).  I don't think the fentanyl did much for her pain, but she did get a little loopy.

Of course, the doctor's couldn't take my word for it- they needed images to be sure.  X-ray was no issue, because now they have those portable machines that can come to the rooms to do x-rays.  This was done relatively quickly.  However, we were waiting around forever for an MRI.  While waiting, a member of neurosurgery came to see us.  I explained what had happened, and he said it wasn't likely shunt failure, it doesn't usually come on so quickly.  I wanted to put my foot up his ____, because there was no other explanation- I knew my kid and I knew what this was.

MRI was taking so long, so we ended up with a CT scan instead.  It's not as good, but given her pain and all we just needed to get the images to see her ventricles.  That was done fairly quickly once we got there.  When he came back to give me the results, he said the ventricles did appear enlarged from last time (Shocker, right?) and they were recommending a shunt revision.  I signed the papers and they started getting the ball rolling with that.  I knew Dr. Apfel was in the hospital because on my way to the school to pick her up I called neurosurgery to ask if we could come to them instead of the hospital and they told me she wasn't there, she was in the trauma center.  While we were waiting, this neurosurgeon did another tap of her shunt.  This time he was able to measure the pressure (11- whatever that means) and extract 15 ccs of fluid.  As he did so, the pressure dropped, and her pain drastically decreased, so she was able to nap.

We were finally taken to pre-op, where things once again moved slowly.  The fluid was starting to build back up because Ally's pain got worse.  By the time they were ready for her in the OR, she was begging to go get the surgery done, she was in so much pain.  I kissed her goodbye and she flashed "I love you" in sign language as they rolled her away.  (Side note- March 11th, 2014 was the day I took her home from the NICU- she decided to commemorate 12 years home by revisiting for a shunt revision!)

They sent me to the pediatric waiting room, which was deserted.  However, I had just entered when a friend from college walked in!  I had forgotten she went back to nursing school and was working on the ward I had accidentally wandered into when looking for the waiting room.  I hadn't seen her in several years, so we were able to spend a few minutes catching up.  She left to go back to her station and I waited.  She came back some time later to tell me she looked Ally up and they were finishing up with her.  A bit later a nurse came to get me and take me to her in recovery.

She was awake and reached for me when I got there.  She was able to sip some water (she hadn't had anything to eat since breakfast, and by now it was around 6 PM- we wouldn't let her have water either since it looked like she was headed for surgery).  She talked A LOT.  She entertained everyone around.  When Dr. Apfel came in she asked if she saw how smart she was.  When Dr. Apfel said yes, she responded with a somewhat slurred "I get all As and Bs."  (Which is true.)  It took a really long time for us to leave recovery and get to her room on the pediatric ward.  When we finally got there, we were sharing a room with another girl.  We found out she was from the Harrisonburg area and had her appendix out.  She was close to the same age as Ally.  They were a super nice family and Ally spoke to her quite a bit through the curtain separating the room.

That night Russell's parents came with dinner for me (I hadn't eaten since breakfast either) and Russell and the boys came too.  They visited for about an hour and were thoroughly entertained by Ally's drug-induced loopiness.

We were able to sleep most of the night.  In the morning they brought breakfast.  She was able to eat the cereal but didn't want the pancakes and sausage, so I ate them.  Neurosurgery came in and said she could go home that day, but they wanted to get an MRI to get follow up pictures of the brain/ventricles, so after that, we could be discharged.  So we waited...and waited...and waited...

I tried to help Ally 'shower'.  We just basically rinsed her off and rubbed her down with a washcloth in the shower area of the bathroom on her back and down her legs- she has stitches on her abdomen we didn't want to get wet.  She's very orange, especially on her chest/abdomen from the antiseptic they use in surgery- she said she doesn't work for Willy Wonka, so why was she so orange?  (I'm telling you, she was hilarious.  Getting her wisdom teeth out is going to be very interesting someday!)

Lunch came, which Ally didn't want.  Russell brought her a chicken sandwich from McDonalds, which she ate most of, while I ate her lunch.  And we waited...and waited...(we watched a bunch of movies during all the waiting!)

A friend from our church walked in!  She was a volunteer with the hospital and we were both surprised to see each other.  She had a snack cart where we took a few snacks, and she had a bear for Ally and a small pillow she'd made- cow print on one side, pink minky fabric on the other- like udders (lol!).  Both were very soft and comforting for her.  It was so good to see her, and she prayed with me too.

Finally they told us that MRI was backed up and they would discharge us and we would make an appointment in a week or so to get the MRI done outpatient.  So after a lot more waiting, I was able to get her dressed and go down, find my car, and take our girl home!

She's still moving slow and complaining about pain.  Interestingly, most of her pain she complains about is in her neck, where there is no incision.  I think this comes from putting the tube down her neck- I remember her first neurosurgeon saying that in the OR it can look kind of brutal getting it strung through.  Her second place she complains about is her stomach, where she does have an incision about an inch and a half long.  She may also still have some trapped gasses, which can be uncomfortable and painful as well.  The only pain relief we are using is over the counter tylenol, which to me is very impressive as to her pain tolerance.

They did not need to shave any hair to get to her shunt!  Her head incision is closed with staples, so we go back in a couple weeks for follow up as well as to have those removed.  Her abdominal incision is closed with dissolvable stitches.  

The most disappointing thing I think to her is she is out of softball and gym for a month.  She'll miss close to half of the school season, but with her travel team she plays all through the summer so there's still lots of opportunities.  She also needs to be careful lifting things for a while as well.

For our hydrocephalus families who have never experienced shunt failure, I wanted to post a description so you could have our experience.  Everyone is different, and I've heard several different experiences.  I've never experienced one like this before.  I honestly feel like she's been in failure- or close to it- since that first ER visit in January, and then we had a somewhat catastrophic event that moved everything super fast.  Ally kept saying "why me?" during the pain, and I don't have an answer exactly, except to help other families also dealing with this.

Thank you to our families for helping and calling, our friends (and hers) for praying, and the great staff at Carilion.  There were so many great nurses and people we interacted with while there who took such great care of us.  We'll keep everyone updated on her progress back to normal.