The Bumpy Road to Langerhans Cell Histiocytosis

Julia has just experienced an unexpectedly rough seven weeks. She’s been diagnosed with single-system multi-focal Langerhans Cell Histiocytosis (also known as LCH), present in the spine and skull. Her particular type of LCH is, so far, one of the low-risk presentations which has a very high curability rate and excellent prognosis. LCH is not technically a cancer, depending on who you ask, but it is usually treated with some type of chemotherapy. Julia’s type requires a year-long staged regimen of Vinblastine and Prednisone, which–as chemotherapy goes–is fortunately mild.

The pun in the title of this post had more of a humorous component when I thought of it, before a few of the later events in this story unfolded. I’m keeping it anyway, since it still fits. Here’s the surprising tale of how we got here.

Day 1

On Thursday, July 2nd, we noticed a bump on the back of Julia’s head. It appeared suddenly, without any apparent cause; she hadn’t fallen over backwards or hit her head on anything that we knew of. It also didn’t have any discoloration or bruising, such as what you’d expect from external trauma. We decided to wait a few days to see what would happen to it, and then visit her pediatrician on Monday if it was still around by that time.

Day 5

After the weekend came and went and nothing changed (other than the bump getting ever-so-slightly larger), we called the doctor on Monday morning and took her in a couple of hours later. He and another pediatrician in the office looked at the strange bump and noted that it seemed both smooth and mobile–apparently good signs for a bump like that–but recommended that we get an ultrasound for further analysis. An MRI or CT scan would have provided more helpful images, but those tests would have been overkill based on the information available at the time.

Day 13

On July 14th, we took Julia to the hospital for her ultrasound. When we asked the technician after she acquired the images, she said it looked like it could have some cystic components. The official analysis the following day from the pediatrician was that it definitely looked like a cyst and should probably be removed, since it hadn’t gone away on its own. Given the position of the bump, he recommended consulting a neurosurgeon.

Day 26

On July 27th, we took Julia and the digital ultrasound images to see a neurosurgeon. She confirmed that the bump looked like a cyst that needed to be removed, but requested an MRI to determine the complexity of the surgery necessary to remove it. The occipital bone at the back of the skull is close enough to the brain and spine that any potential nerve or vein involvement could increase the technical difficulty of the removal procedure.

Day 33

Due to the inexplicable rules on medical machine time allotment when scheduling certain types of procedures, the neurosurgeon had to schedule the MRI as an inpatient procedure. Therefore, we headed to the hospital on the evening of August 3rd to settle in for the night, waiting for the procedure itself to be done in the morning. Julia had to be NPO starting at 4am since she had to be completely sedated for the MRI (good luck getting a 7-month-old to hold still for that otherwise), and general anesthetic requires no food or drink to minimize risk of suffocation should something go wrong. Not only could we not feed her, but for ridiculous administrative reasons, we had to stay at the hospital overnight at the same time.

Late the following morning, Julia received the planned sedation, then the MRI. She had no issues with the procedure or test, and recovered well after so many hours without nourishment. She downed about 6 ounces of Pedialyte and nursed for a while, and had some formula. And burped. A lot.

Break for Software Engineering Rant

The kind of workaround they made us do for MRI access is not acceptable. It is inefficiency of the highest form in one of the worst environments possible due to the insane costs of various types of healthcare–regardless of whether my insurance has to pay for it or I do. The neurosurgeon warned us that it might be necessary to do this in order to gain access to the MRI resources she needed in the time frame required, and apologized in advance. The scheduling assistant at the hospital apologized as well. I explicitly told her (as an observation, not that she could do anything about it) that it was truly insane to have to do it this way, and asked if there was any possible way to achieve the same MRI time without physically showing up at the hospital until we truly needed to be there. She said no.

I have built back-end software (from scratch) to manage scheduling of multiple resources among multiple people. I even came up with a computationally efficient way to find availability in a schedule by looking for what isn’t there. This is really not that hard of a problem to solve. Use a priority queue to manage urgent patients and delays. There is no reason why anyone should have to undergo an outpatient procedure as an inpatient because of some administrative rule that says you’re not allowed to access the MRI machine quickly as an outpatient, when they clearly have the resources available for it and for keeping you in the hospital at the same time. It’s mind-boggling.

Separately, there is a communication issue that the administration or doctors or possibly nurses need to fix (though the nurses we had were very good, so I don’t think this is on them). Regardless of how efficient or inefficient your testing and surgery processes are, whether it takes 30 minutes or two hours or two months to get an MRI done, it is paramount to correctly manage expectations concerning what will be happening. We were originally told that Julia’s MRI would be at 8am, and the NPO requirement was based on that. She didn’t have it until about 11am. It’s a big deal to keep a 7-month-old baby from eating or drinking for that many hours in a row, especially when it could have been much shorter if we knew what was actually going to happen.

I understand that urgent and unexpected things come up, especially in a hospital environment. Sometimes the people or machines you planned to use get booked for more time-sensitive cases, and there’s nothing you can do about it. In those instances, it is important to let affected patients know ASAP that their procedure(s) will be delayed due to resource availability issues stemming from unforeseeable circumstances. But that is not what happened to us. There was no advance notice until it was too late to affect Julia’s nourishment, and the notice didn’t come with any explanation for the delay. Why couldn’t they have just scheduled the procedure originally for later in the morning? Maybe there was some reason, but if there was, we never heard it. We sent some pretty specific information stating all of this through the feedback mechanism provided by the in-room TV system.

Carilion: please fix this. Get somebody else to fix it. Get me to fix it; I can write software. Please, just stop doing this to people.

End rant.

Day 36

After a rejuvenating Wednesday at home between hospital visits, we headed back to the hospital at 5am on August 6th for the cyst removal surgery. During the course of the initial discussion of what was to happen that day, we got our first bit of unexpected bad news when the anesthesiologist asked for confirmation of “which ones we were going to be removing during this surgery.”

Which ones? Plural?

The MRI results from two days earlier had come back and shown not just one but three or possibly four lesions in Julia’s skull. None anywhere near as big as the one on the back of her head, fortunately, but still present. Nobody had mentioned that to us before we showed up, so it was a bit of a shock. We only knew about the one we could see. The MRI revealed a small lesion on the upper left part of her mandible, and one in the middle of her sphenoid bone, and one more small anomaly that couldn’t be definitively identified. Due to the unexpected findings, the neurosurgeon also ordered a full skeletal survey (series of X-rays of her whole body) to check for anything wrong elsewhere.

With this news firmly planted in the most worry-prone areas of our minds, we left Julia in the hands of the anesthesiologist and surgeon to work their magic on the one big bump on the back of her head, and returned to the surgery waiting room until they moved us up to a dedicated room in the pediatric ward.

The excellent surgeon performed the cyst removal procedure with perfect results, and Julia handled everything as well as could be expected. She was back with us just a couple of hours later, sans a small bit of hair and with a new gauze patch on the back of her head–much less frightening than we were afraid it might be. The removed mass was sent off to pathology for full analysis, but the initial results suggested that it was indeed exactly what they thought: a benign aneurysmal bone cyst (which sounds awful, but actually isn’t).

Late in the evening, a team of two RNs came to get Julia for the skeletal survey. We were hopeful that we might actually get to go home that day since Julia was nearly back to normal behaviorally speaking, but no such luck. They decided to keep monitoring her, and (per the above rant) keep her as an inpatient to expedite anything more they might need to do based on additional discoveries. This became standard protocol over the next many days, much to our chagrin and consternation.

Day 37

The skeletal survey results that came in on Friday revealed nothing more of interest except for one small abnormality in the T-8 vertebra. The neurosurgeon said that if it weren’t for the unexpected additional lesions in the head, she would have ignored the T-8 anomaly, which was so subtle that it could have been a random shadow on the X-ray. However, she ordered a second MRI to focus on the thoracic vertebrae to get a clearer picture.

The sedation and thoracic spine MRI occurred later that day. That was the 3rd application of general anesthetic to date. (By this point, we’d learned most of the pediatric nurses’ names, and everyone on the floor recognized Julia. We’d also determined that the cafeteria food at Carilion is remarkably good.)

Day 38

On Saturday, August 8th, the results from the second MRI came back and showed some abnormalities that they didn’t see on the X-ray before, but which also did not look like the same bone cyst that they took out of her head. The T-8 vertebra in particular appeared to be somewhat collapsed. The “shadow” from the skeletal survey X-ray was not just a shadow; it had a definite cause.

After this, we spoke to a resident oncologist/hematologist who had been made aware of the findings so far, and she expressed her concern based on the symptoms that Julia might have Langerhans Cell Histiocytosis. She explained some different ways it might present in Julia’s body, and told us that it would probably require chemotherapy if she turned out to be correct. To continue investigating this, she and the neurosurgeon together ordered a bone scan, a full spine MRI, and a biopsy of the T-8 vertebra.

Day 40

After one full “downtime” day in the hospital waiting for the availability of a single anesthesiologist who could meet the planetary-alignment-like requirement of being available for three separate sedated procedures in a row, we got the remaining scheduled tests done on Monday, August 10th. As it happened, the first test (bone scan in Interventional Radiology) did not actually require Julia to be sedated, so they did that one first. The biopsy and MRI happened many hours later, and took about four hours start-to-finish.

The doctor who performed the biopsy elected not to use the T-8 vertebra due to the high-risk nature of the procedure and the fact that the T-12 vertebra provided a much larger and safer target. The T-12 appeared on the MRI with the same “lit-up” properties as T-8, so it would have the same fundamental LCH qualities (if any) as T-8.

We finally returned to our now-familiar room on the pediatric floor, reveling in the fact that Julia would not have to endure any more tests before we could go home–even if that turned out not to happen the same day, which of course it did not.

Day 41

On August 11th, the final results came back from the original pathology of the aneurysmal bone cyst, confirming that it was clean and nothing more than what it appeared to be. The neurosurgeon and oncologist also got the imaging tests done the previous day. The bone scan and MRI showed only a little more of the same things seen from the second MRI, namely that there were a few additional sections of the spine that lit up the same way as the previously identified vertebrae. With a summary discussion with the neurosurgeon concerning the results and what to expect in terms of later analysis and follow-up visits, Julia finally received official permission to go home.

Courtney and I unpacked, showered, and rested (along with Julia) as best as we could, with the help of both of our sets of parents. We also kicked into high-gear “research mode” for anything and everything we could learn about LCH in preparation for the scheduled meeting with the oncologist on Wednesday of the following week, to discuss the results of the T-12 biopsy which would be available by that time.

Day 47

On Monday morning, the oncologist called to let us know that the T-12 biopsy results had come back initially positive for LCH. She recommended additional “stains” to rule out and/or confirm various possibilities, but she wanted to let us know in advance of our appointment, and so that we would be aware of the results and of additional appointments she requested for us in advance. These included an ultrasound of the abdomen to rule out liver, kidney, and spleen involvement, a chest X-ray to rule out lung involvement, and a consultation with a pediatric surgeon who would be responsible for a Broviac line placement for chemotherapy delivery. (Julia’s body is too small for a typical port, and arm-vein-based delivery is not recommended for Vinblastine due to its harsh effect on skin and nearby tissue.)

Day 49

On Wednesday, August 19th–my mom’s birthday, no less–we had the official diagnosis and treatment discussion with Julia’s oncologist. By this time, we already knew the fundamentals of almost everything she told us. The new elements were the specific chemo regimen recommended for treatment, and the official classification as single-system multi-focal LCH (which, given the possibilities, relieved us somewhat because of how much worse it could have been).

Today, we’re pursuing a second opinion from Johns Hopkins up in Baltimore just to get further confirmation and expertise. We anticipate being told the same thing that the local doctor told us, but since JH actually has an LCH-focused group within their oncology department and they work with it more often, we want any additional insights they can provide before starting a chemotherapy regimen.

A Silver Lining

Julia’s LCH diagnosis ultimately came after investigating the bump on the back of her head, which had nothing to do with LCH. It would likely have been another 6-8 months before we would have noticed any symptoms from LCH, during or after she started walking. Despite the fact that she had to endure two ultrasounds, three MRIs, surgery on her head, a skeletal survey, a bone scan, spine biopsy, and endless pokes and prods for lab work and vital checks, I’m grateful that we caught it now rather than a lot later. On top of that, since Julia is still so young, she has no way of knowing yet what lies ahead for her, and so she has no fear or anticipation of any kind. It would be harder for all of us if she did.

Also, Julia has been incredibly resilient through all of this. She is consistently as happy as usual (which is a lot), eating normally, sleeping more or less normally for an 8-month-old, and just generally enjoying life. The surgery on her head is healing well. We are constantly amazed at how well she is doing despite what she has had to go through so far.

The next year will be difficult for all of us, especially Julia, but we have a great support network here with our family and friends. For those of you reading this who have already provided support, prayer, food, cards, and more: thank you. Although the circumstances aren’t what any of us would hope, it brings out amazing acts of kindness.

2 thoughts on “The Bumpy Road to Langerhans Cell Histiocytosis

  1. In the Name of Jesus I lift up precious Julia to u & ask for a complete healing of this disease. Lord we know you are able & trust that ALL will be for ur honor & glory. I lift up Jeff & Courtney to u also asking for ur wisdom as they navigate through these deep waters. AMEN
    We love u & will be praying for you🙏🙏

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