Ophthalmology and Endocrinology updates

In the last two weeks, Summer has been seen by Ophthalmology and Endocrinology for follow-ups. Ophthalmology was on the 15th, so I'll start there. I don't know what it is, but there's something about those eye appointments that makes Summer exceptionally uncooperative right from the start, so that's always fun.

We started with a vision check, similar to the letter chart used for adults but with pictures on a TV screen. We were able to get her to cooperate enough to tell that she hasn't gotten any worse from the last time, but we couldn't tell for sure how good her vision actually is as she lost interest really quickly.

Using both eyes, her vision is at least 20/40 which is still within normal for her age.

Her right eye is significantly worse than her left. They can see as much by the coloring of her optic nerves (the nerves for the right eye are paler, indicating damage), and we've only ever gotten measurements as high as 20/100 on her right eye. It might be slightly better, but it's tough to get her to continue the test for very long when we have to hold our hand or a patch over her good eye. I can't really blame her, though - why would you want to have something covering your eye when it immediately makes it so you can't see as well?

We're going to practice at home, try again in August to see if she'll cooperate a little better, and we'll go from there. We talked about possibly having her wear a patch over her good eye from time to time, in an attempt to force the nerves on the right eye to work harder and improve. It doesn't always work, but it would be worth a try. I'm hoping we can sell that to her as "exercises" to make her eye better...we'll see how well that goes over. I'm not holding my breath.

They also tried to get an image of the nerves using a camera set up I'm pretty sure I've described before, although I can't find the post. The short of it is that she cooperated really well, but just isn't quite old enough to follow the very specific instructions (she has to stare straight at a specific dot or line long enough for it to get a picture without looking at anything else), so it didn't work. Maybe next time. That would give us the most accurate way to compare the actual state of her optic nerves from visit to visit.

They also dilated her eyes and looked at the nerves with a magnifying glass. Getting the drops in was a battle, but she did remarkably well with letting Dr Baran look into her eyes once they were dilated, so the appointment ended on an up note.

This morning we saw Dr Pihoker in Endocrinology to check in and review the lab results from the blood they drew back on 6/2 when she had her MRI. Summer was very cooperative and chatty at this appointment. We all really liked the doctor who came in before Dr Pihoker to do an initial check up and collect our questions/concerns. I wish I could remember her name!

Summer's thyroid levels look good, so we're medicating at the right levels at least for now, although that could change as effects from the radiation become apparent.

Her growth hormone factors, however, are low. We knew that one was only a matter of time. Summer now weighs 12kg (26.4lbs) and is 89cm (35") tall, so she has been growing and gaining weight at a reasonable rate, although not quite as quickly as they would probably see in a normal child.

They don't usually do anything about low growth hormone within six months after treatment, especially not when we've only had one low reading. They will test again in three months (during her next MRI in late Aug/early Sept) and assuming the levels are still low we would probably start growth hormone shortly thereafter.

We talked a little bit about what will happen at that time, including additional stimulation testing if required by the insurance company and an x-ray of Summer's hand to look at her bone growth and help calculate her target height. Unfortunately, there's no oral form of the hormone available, so Jason and I will have to be trained on administering daily injections...ugh. We were assured it's a small needle and people do really well with it. But considering that Summer is about as trim as they come, I'm sure it won't be fun for her and we'll have many battles about it for a while. I suppose it will be a good thing to start it while I'm home on maternity leave because it will probably take two of us to get the shot done until she gets used to it.

Results from Thursday's MRI

Summer had an MRI on Thursday morning and, short of everything miraculously disappearing as if it had never been there, we couldn't have hoped for better results. The cyst is smaller than it was at her March 4th scan (stable from a HASTE MRI on 4/18), and the mass has actually shrunk by about 20%!

The doctors kept telling us it might not shrink at all, that if the radiation worked it would (hopefully) just not get any bigger. It's a good sign that everything is headed in the right direction, and that the radiation is working to control at least the mass portion of the tumor. In theory, the less mass there is, the less fluid can be generated for the cyst, but it still might be years before we can say anything even remotely definitive about the effect on the cyst.

As far as the MRI itself, Summer did extremely well with the pre-scan stuff - weight check (11.7kg or 25.74lbs, up 1kg in the last couple of months, after holding her weight at 10.7ish kg for an entire year), blood pressure, temp, etc and even asked for the anesthesiologist right when we got into the MRI triage area so she could tell him she wanted strawberry flavored gas. Jason and I can't help but laugh a little to watch this pint-size kid directing the nurses.

She went under very well, with no fuss about the face mask. Unfortunately, she didn't wake up quite as cheerful as she went down. This post is so late in coming because she was quite the handful for the entire rest of the day. Re-programming her shunt was a struggle, and we didn't get to have much of a conversation with the Heme-Onc nurse practitioner about the results. Luckily they were good results so we didn't have many questions!

Summer's next MRI will be three months out, but we'll have check-ins with ophthalmology and endocrinology in the meantime. I'm not really worried about her vision, given that the tumor and cyst have either been holding stable or getting smaller since her last eye exam. But I am especially curious to find out how her endocrinology blood work (drawn on Thursday) comes back, since we have been told it's only a matter of time before she stops producing growth and thyroid stimulating hormones.

After this clean scan I'm hoping for a bit of breathing room where I am not constantly questioning whether the shunt has failed or the cyst is growing. Last time, we made it about six weeks before I cracked and demanded another scan. I have a tough time trusting that certain changes (bigger tantrums, sometimes stumbling over words, etc.) are part of normal developmental phases and not because of the tumor. Hopefully it will only get easier as we have more good scans under our belt and Summer gets better at articulating what she's feeling.

One year later...

Today is the one year anniversary of Summer’s diagnosis. As my friend Casie said, anniversary is too happy of a word to mark such an event, but I can’t think of anything else to call it.

So much has happened in the last year. Most of the time it seems much longer than that, like we’ve been dealing with this forever. It’s so normal for us now, that I can’t really remember what it was like to not know this brain tumor was a part of our lives. To worry about “little” things like ear infections and colds instead of cyst re-accumulation, tumor growth, hormone deficiency and shunt failure.

I commented to someone the other day that this next baby probably won’t get nearly as much sympathy from us for the minor things. Like shots? I used to get so stressed out about Summer’s vaccinations when she was a baby. Now? Pfft. Big deal. 

Over the past few days, I’ve been reading back through my earliest posts on this blog. It’s amazing how much you can forget in just a year. I’m glad I wrote it all down as it was happening. 

Here’s the roll call of what we’ve been through in one year:

• 5 surgeries total - two craniotomies, one reservoir to VP shunt conversion, port-a-cath placement and port-a-cath removal
• Intracystic Interferon therapy – the chemical therapy injected directly into the cyst
• 28 days of proton radiation
• Countless appointments, blood tests, MRIs, CT scans, VEP and vision tests

It’s a lot! Thankfully, Summer has come through it all with flying colors. 

We have yet to make it six weeks without a doctor’s appointment, and this six week stretch I was getting optimistic about has turned out to be no exception. I forgot she had a dentist appointment yesterday, and we have an appointment at Children’s on Wednesday for a study we’re participating in. 

The study is to try out a new comprehensive care plan, with the goal of better co-ordination and communication between primary care physicians and the specialists at Children’s for patients with “complex medical needs.” When I first got the invitation to participate, I actually chuckled a little bit – it’s strange to think of Summer as someone with “complex medical needs.” 

Of course she’s been through a lot, but on a day to day basis, she’s just a normal kid. We’ve been very lucky in that way. She’s retained every bit of her personality - the smarts, humor, stubbornness and sass we always knew she had. 

What the nurse told us after Summer woke up from her very first surgery is absolutely still true: "this girl does what she wants!" Just ask her ballet teacher. She constantly interrupts class, wagging her finger at the teacher and saying things like “Hold on. I just need to make a phone call.” Or “Wait, I’m going to go eat dinner.”

So what do you do when a year goes by and your kid is doing as well as Summer? You celebrate! I haven’t had the time or energy to plan very well this year (they SAY you’re supposed to have tons of energy in your second trimester, but chasing after Miss Energizer Bunny drains every ounce it). Maybe next year we’ll have a party. 

For tonight, I think the three of us will have to go get ice cream cones at the drive in or a mini cake with lots of frosting from the bakery. Maybe both! 

Great MRI today

Summer had a HASTE MRI this morning. Jason went in with her for the scan, and she didn't enjoy being strapped down to the table but calmed down as soon as the machine started going. The scans at Childrens are SO much faster than the ones in Boston - 10 seconds, vs 5-7 minutes - so it's much easier to get through.

We saw the neurosurgery nurse practitioner immediately afterward to have her shunt magnetically reprogrammed, and she told us the scan looked great.

After picking up the obligatory lollipop at the gift shop, we met with the oncology Nurse Practitioner, Cory, to review the images in detail. The cyst looks nice and small, and her ventricles are now a normal size for her age; they were compressed previously because the cyst was pushing everything around in her head.

I always feel a little awkward asking for scans before they're "due," but Cory was very reassuring. She gets that we've been through one heck of a year and that we're going to be a bit overly cautious until we've had a few of these good ones under our belts. It was such a relief to have a peek at what's going on in there!

Summer will have a full MRI on June 2nd (6 weeks out) to check in again. Assuming no complications before then, that means we have a six week vacation from doctor's visits! That might very well be the longest break we've had in a year.

MRI on Monday

I just realized it's been a month since I posted last, so I thought I'd share a quick update.

Summer has been doing mostly well over the last month. She was sick for about a week, which prompted us to start a stress dose (3x what you would normally need) of hydrocortisone to help her body cope with the illness. It was as a precautionary measure the endocrinologist recommended because her cortisol levels hadn't been tested in a while. If her body wasn't naturally producing the cortisol it needed, this type of flu-like illness could have been very dangerous for her. Luckily, her levels ended up being just fine on their own, and we were able to wean her off the medication last week.

A few days before she got sick, Summer started sleeping poorly, was especially irritable, and not interested in food at all. At the time, we didn't know she was getting sick, so this concerned us a bit. We also hadn't heard back from Dr MacDonald about Summer's last scan (from March 4th), and how it compared to the most recent scans done in Boston (our doctors here at Childrens only had one from early January to compare to, so we were interested to see if it had changed since the mid-February scans from Massachusetts General).

It turned out that the records never made their way to her, so I requested them from Childrens again. We had gone back and forth with Dr Geyer about the time frame for the next scan and were originally going to wait three months unless Dr MacDonald wanted one sooner. Since Dr MacDonald hadn't yet received Summer's most recent scan and we were getting a little worried about the irritability, I decided it was time to push for scheduling her next MRI. I sent an email to our team at Childrens outlining our concerns, and we now have a HASTE MRI on Monday morning (4/18) to check the cyst size.

Since Summer has recovered from being sick and is back off the hydrocortisone, her behavior has mostly returned to normal. We also heard from Dr MacDonald that the overall cyst size was stable, with some minor increases and decreases in various pockets of the cyst. I'm no longer terribly concerned about what the scan will show, but we're keeping the appointment for the peace of mind.

I'll post on Monday to let you all know what we find out.

Catching up

Today Summer had her port-a-cath removed. As a refresher, that's the access port they placed in January for the daily administration of anesthesia while we were in Boston. Since they no longer need it, it was time to have it removed. Jason and I were glad to see it go - it was pretty disconcerting to feel this big lump under her skin every time we picked her up.

The surgery went well and was very quick. It was only about 35 minutes after they took her back before we got the page telling us they were finished and closing up. 30 minutes or so after that, we talked to the surgeon briefly, and we saw Summer about 20 minutes later. She was awake already and NOT happy.

I'm not sure if she was in pain or just having a bad anesthesia wake-up (they can vary widely in reaction), but she was furious. I'm talking all-out screaming, flailing around, temper-tantrum-of-your-nightmares kind of stuff that lasted for a good 30 minutes before she finally calmed down. It was the most intensely angry reaction she's had to anything so far - Jason and I were shocked - but once she calmed down, it was like a switch had been flipped. She was talking normally and politely, asking if she'd get to see the doctor again, like nothing had ever happened.

She hasn't seemed to be in much pain today - the oxycodone is working well for her. She'll be on that for a day or so, but should recover from this pretty quickly and should only have pain at the incision site.

After two weeks, I think we've fully adjusted to being home. We miss our Boston friends, but are glad to be back! We've had a great time catching up with family and friends, and getting back into our normal routine. Summer's been in a "mommy needs to do it, not daddy" mode for the past few days, and I'm guessing that's part of adjusting to me being at work during the day instead of home with her. Otherwise, she's been coping with all the changes amazingly well.

Summer has had one MRI since being back. It showed the cyst being smaller than the last scan Children's had on file, but that scan was outdated so it's hard to say what the real results are. We're waiting to hear from Dr MacDonald in Boston once she is able to review a copy since she has Summer's most recent scan. Assuming there's no change in cyst, it will probably be another 2 1/2 months before her next one.

She's still not exhibiting many side effects from radiation, which we are grateful for. I've heard reports from some of the other parents of major headaches and continued fatigue, but Summer doesn't seem to be experiencing any of that.

She has had some hair loss, which is mostly noticeable on her right temple where her hair is short from her December surgery. I pointed it out to Jason one day, and apparently it really freaked her out - she's been having meltdowns about it from time to time ever since. Jason and I have had to reassure her numerous times that it will grow back. Poor kid - we'll have to watch what we say around her about the medical stuff while she's in this sensitive stage.

One of the questions Jason and I get asked most is: So, did it work?

The answer is: We don't know, and we won't for some time - maybe years. The tumor is not expected to shrink in size at all, so we won't have any indication of the cells dying off. We'd know sooner if it didn't work because they'd see growth in the tumor mass, but Summer's tumor (the mass part) is so slow-growing that it might take a year to tell if there really has been growth or not.

Unfortunately, we won't ever really be certain that the radiation worked on the cyst. We'll know if it didn't work only if her shunt also fails and stops draining the cyst, which would result in it filling up again. But if there's no change in the cyst, it means that either the radiation worked or the shunt is still working.

I think that's about all I have to update you all on now, but I'll post again when we hear back from Boston - hopefully in the next couple of days.

The big day!

Last night, we picked Jason up from the airport. He flew back out to Boston (thanks Uncle Jimmy!) to be here for Summer's last treatment and help us get back home.

Here's Summer patiently waiting for him at baggage claim:

She didn't spot him in the crowd of people, but once I pointed him out, she jumped out of the stroller and ran to meet him.

Today, Summer finished her final radiation treatment. Her last session this morning went well, and she was in good spirits for her bell ringing. I managed to keep the tears in check, which is quite a feat since I could barely keep from crying when complete strangers would ring the bell. It's a very simple, but emotional thing.

Below are some photos from the last day, and a video of the bell ringing toward the end of the post.

Summer's last day happened to also be a Toy Friday. This is the toy cabinet she got to choose a toy from each Friday. Nurse Rachel, who usually sets it all up, was on vacation, so it's a little less organized than usual but Summer didn't care one bit. Today she chose a giant coloring book.

When we walked into the prep/recovery room, there was a surprise waiting for Summer. Her bed was decorated with a congratulations banner and gifts:

 

This lucky girl gets to take home her very own monkey puppet, which was one of her favorite toys to play with each day:

After Summer woke up from treatment, we went out to ring the bell. She was pretty anxious to do it - she's been talking about it a lot since she saw Casey ring the bell on Wednesday.

We'll head back over to the proton center on Monday to say goodbye to Nurse Rachel, but Summer couldn't leave today without saying goodbye to Dr Nick, her favorite anesthesiologist:

 

I don't think it's really hit that she's done, but maybe by the time we get home on Tuesday night. We'll see.