Omar Abou Mrad

All about Yuri's cancer

How it started

We noticed a little tiny bump on his wrist, no more than your usual mosquito bite. There was no irritation and no reddish skin; Yuri felt no pain, nor the urge to scratch it. It was far too small to worry about despite us being quick to call the doctor, as all parents usually do. Weeks went by and that small tiny bump increase a little bit in size; Naturally, we booked an appointment with the pediatrician and after inspection, he informed us that this is most likely a Ganglion Cyst, quite common and it may eventually go away. Weeks went by, the cyst just kept on growing and despite it not being painful, it was simply far too big to ignore.

Yuri's Wrist Bump

We contacted the pediatrician yet again and he asked us to consult a Pediatric Orthopaedic Surgeon. We booked a next day appointment and immediately upon examination, the doctor said it was a Ganglion Cyst. He was a bit surprised about it's location given it's on the side of the wrist and not top or bottom and its size given Yuri's age. Being quite inquisitive, we naturally had a set of questions that needed to be answered, which were prepared before hand:

Questions we asked the Surgeon

- What is that thing on Yuri's hand?
- What is the cause of it?
- How critical is it?
- Is it possible for it to go away by itself?
- How long does it need to go away?
- Is it possible for it to burst?
- How risky would it be if it bursts?
- How can we remove it?
- If we remove it, is it possible to come back?
- Does it come back in the same location?
- What type of anesthesia is needed in the operation? (local/etc)
- How long does the operation usually take?
- Does he need to be admitted in the hospital or is it an OUT procedure?
- Does insurance usually cover this?
- When is the soonest we can remove it?
- How long does the wound (after the operation) usually take to heal?
- Can he still do sports activities after the operation?

He gave me a full brief on the procedures and the chances of the cyst reappearing with every option. Considering a 6 year old child would not hold still during extraction, he needs to go under (anesthesia) either way, so the choice of doing an incision was the right one.

Yuri had the operation on September 15th 2019 for what seemed to be a Ganglion Cyst on his wrist. The operation took less than 30 minutes and the excision was a success. It was so simple that we did not stay the night in the hospital; In fact, Yuri was fully energetic on the same day, playing as if he had not gone through surgery that day.

WARNING - The following link contains picture of the tumor that was removed from Yuri's wrist. If you are sensitive to such imagery, please do not open it. Picture - Yuri's Tumor

it went downhill, real fast

We sent the removed body (1.5 x 1.2 cm) for a pathology test, a common policy when extracting anything from the body is to run a biopsy. We did not give it a shred of thought, I mean, everything has been successful thus far and the doctor was very optimistic. However, after two weeks we were notified that the diagnosis was incomplete highlighting that it's a tumoral proliferation and requires an immunology test. It's notable that the excision of the tumor was complete based on the observation of the pathologist.

Yuri's Initial Biopsy Result

GROSS DESCRIPTION

Number of containers: 1
One nodular fragment measuring 1,5x1,2cm PIT,1block.

MICROSCOPY

Sections reveal a more or less defined and encapsulated tumoral proliferation made up by round small monorphous cells with scanty basophilic cytoplasm and finely nucleated hyperchromatic nuclei. These cells are arranged along sheets with accentuation around vessels. Some mitosis are seen and tiny foci of necrosis are observed.The excision is complete

DIAGNOSIS:
Synovial mass, unspecified site, excision:
- Tumoral proliferation made of round cells, necessitating an immunohistochemical study for definitive diagnosis.

The immunology test required an additional two weeks wait. It became clear that there's something wrong and the pathology test result really gave no hint as to what this was. It was almost impossible to get in touch with the Pathologist that ran the tests; The policy is that you need to refer to the surgeon for the results and explanation. Nevertheless, after two weeks wait, we received a dreadful call from the surgeon that immunology test shows a very high chance of the tumor being malignant. The diagnosis thus far was that Yuri either has:

A chromosome translocation test was needed to get a conclusive answer on what type of tumor this is. The pathologist also highlighted that the specific type of translocation (22 <-> 11) was not doable in Lebanon. We needed to send slides to a laboratory in France.

Yuri's Initial Immunology Result

The immunohistochemical study done on paraffin block P19-41689 shows a strong diffuse positivity of tumoral cells with the anti-MCK, Vimentine and CD99 and a focal lesser positivity with the anti-EMA, SMA and CD34.
Kl67 exhibits a proliferation index of 25% approximatively.
The anti-CD31 is negative.

CONCLUSION:

This immunohistochemical profile in correlation with the cytological features is not conclusive. However it might be suggestive of a potentially malignant round cell tumor necessitating thereby a molecular cytogenetic study of the translocations for final diagnosis (to R/O Ewing sarcoma / PNET, round cell synovial sarcoma among others atypical non malignant round cell tumor particularly that the tumor is well circumscribed and CD34 and SMA are focally expressed).

At this stage, we had jumped from [1] this being nothing to [2] this being possibly something [3] to this being almost positively cancer. You can only imagine how dreadful it was to hear this news. Nevertheless, there was still a chance of this ending up none-malignant, so it was important to get more clarity. The surgeon put me in touch with both the Pathologist who agreed to meet and explain her findings and a Pediatric Oncologist who met with us on the same night. Considering the amount of questions that we had unanswered, we decided to prepare yet another list of questions for both the oncologist and pathologist separately.

Questions we asked the Oncologist

We met with the Oncologist that very same night and we asked the following questions:

- What report did you get from the Surgeon?
- What report did you get from the Pediatrician?
- What report did you get from the Pathologist?
- What type of malignancy did she observe? Is it Sarcoma? Is it confirmed?
- What are the positives that you can see in her report?
- What are the negatives that you can see in her report?
- How many cases of this malignancy happens in Lebanon?
- How many cases of this malignancy have you seen first hand?
- What is the worst case scenario that you have observed for this type of cancer?
- What is the best case scenario that you have observed for this type of cancer?
- What are the procedures that Yuri has to go through?
    - Will they be painful?
    - What type of anesthesia will he undergo?
    - Considering he just went under around a month ago, does that affect him?
- What are the dates that he has to go through them?
- How accurate will the tests be in terms of results?
    - Is it possible for a spread to exist without the test catching it?
    - Is it possible for the test to show complete none-existence of the tumor?
- How will we know what stage of cancer we're at?
- If we notice a spread in the tests, will we have to start the therapy even before the genetic test results?
- Is it possible to do the genetic test in Lebanon?
- What are the treatments that my son has to go through assuming this is fully diagnosed as cancer?
    - What is the frequency of the treatment?
    - What is the approximate success rate of the treatments?
    - How will it affect his day to day life?
- Who is the doctor that i should be following up with constantly?
- Is there anyone that is diagnosed with Sarcoma, or has gone through the treatments that we can talk to?

The oncologist answered all our questions and briefed us on the next steps. Yuri would undergo a PET Scan to rule out the spread and afterwards will undergo a bone marrow extraction to rule out spread to the bones. If any of the two tests are positives, he will have to undergo immediate treatment. If both tests yield negative results, we would have to wait for the translocation test. Assuming the translocation test is negative, then the diagnosis will be of an atypical none-malignant tumor and no treatment would be needed.

Questions we asked the Pathologist

The very next day we met with the Pathologist. A kind lady that was very understanding and felt the need to answer any and all question we have concerning her findings. We prepared a list of questions to ask her as well. On a side note, the exercise of sitting down and thinking of the questions to be asked, help us formulate our thoughts better and allowed to us to properly understand the situation and eventually explain it better to our friends and family:

- Are we correct in the following understanding of the conclusion?
    - #1 This will end up being either Ewing sarcoma / PNET
    - #2 Round cell synovial sarcoma
        - Can you explain the difference?
    - #3 Atypical none malignant round cell tumor
- Could it end up being anything other than the three above?
- Do cells in none malignant tumors generally keep proliferating or do they stop?
- So what differentiates a none-malignant cell-always-proliferating tumor from the malignant ones?
- What are CD34 and SMA?
- We understand that the tumor being circumscribed is a good thing, does that mean there's absolutely no "leak" ?
- Apart from the above mentioned positives, are there any other indications that this may not be malignant.
- How many times do you run the same test on the same sample before you reach a conclusion?
- Will the molecular study definitively identify the type? #1, #2 or #3? or is it #1/#2 or #3?
- If the translocation happened once, do we assume that it can happen again and not necessarily in the same location?
    - Is it more likely to happen?
    - Does everyone of us have the exact same probability of the exact same translocation to happen?
- We are due for a Pet Scan and a Bone Marrow extraction tomorrow
    - Scenario #1 - assuming these are negative and the translocation test is negative - we conclude that it's Case #3
    - Scenario #2 - assuming these are negative and the translocation test is positive - we conclude that it's Case #1/#2 but what does this mean for us?
- Concerning the test process that will happen
    - How long does it usually take the tumor/samples/etc to arrive in France?
    - How long will it take the test to complete?
    - Will they notify you of the result instantly?
    - Will they return the tumor/sample/etc that we sent?
    - Will they rerun the tests that you've already done? Patho/Immuno/etc ?

The pathologist explained all her findings and the rarity of Yuri's case, which stemmed mostly from the presentation of the cancer (it's location, the tumor mobility, etc...). While the case is rare, there are large positive indications that are in our favor. Nevertheless, we prepared and sent the samples to a laboratory in France for the molecular test and requested another pathology and immunology test, providing us with a second opinion from a completely separate entity.

The first round of tests

We had scheduled both the PET Scan and the Bone Marrow surgery on the same day. We assumed Yuri would not hold still during the PET Scan so we decided to use the same anesthesia for both tests. Luckily, Yuri was not afraid of the machine and was awake during the PET Scan. Except for a small activity in yuri's wrist (a wound being healed) there was a small ganglion underneath his left arm pit that is less than 5mm of size. The ganglion did not light up in the pet scan which hints that it could be due to the inflammation in yuri's wrist (or other). Nevertheless, a CT Scan will followup in the next couple of months to ensure the validity of the assumption.

Yuri's PET Scan Test Results

(Excerpt)
IMPRESSION

1. Status post recent prior resection of left write tissue tumour, with faintly FDG avid postsurgical changes.
2. Faintly FDG avid 5-mm left axillary lymph node, likely reactive.
3. No FDG avid distant metastases elsewhere.

Additionally, there were no light ups in the bones during the pet scan which further indicates that the bones are most likely intact. Nevertheless, since we cannot take any risks, Yuri underwent a Bone Marrow extraction operation. The Bone Marrow results came out a week later and were negative.

Yuri's Bone Marrow Test Results

MACROSCOPIC DESCRIPTION

1- Right anterior: Two cores of bonny tissues 2.2x0.1cm each associated with a blood clot 0.2cm in greatest dimension (PIT/1A,1B).
2- Left anterior: A core of bonny tissue 1.5x0.1cm (PIT/2A)

MICROSCOPIC DESCRIPTION

1&2-Sections from either specimen show bone marrow biopsy exhibiting normal cellularity, representation and maturation of three major haematopoietic cell elements and absence of reticulin fibrosis. No evidence of granulomas or abnormal infiltrate. Immunohistochemical stains with antibodies against CD99 does not highlight any tumor cell clusters

DIAGNOSIS

1&2-Bilateral Bone marrow trephine biopsies:
- No significant pathological abnormality.

Great news but we still needed to wait for the translocation test.

All this time, the wound was not healing

The initial operation we had done was quite clean, however, throughout the weeks it simply became much worse and the wound started spitting out the internal stitches and it was just not healing properly despite our constant care. Here's a list of images showing the progression of the wound:

2 Weeks 3 Weeks 4 Weeks 5 Weeks
Wound Picture 1 Wound Picture 2 Wound Picture 3 Wound Picture 4

We simply did not know what was causing this, despite our constant visits to the Surgeon's clinic.

More info about this comes later in We also have to care for the wrist wound section.

The translocation results came in

On a Wednesday mid-day, we received the translocation test result from France which confirms the translocation of chromosomes 11 and 22 finally confirming that Yuri has Ewing's Sarcoma.

Translocation Test Result

Material: Lames

Sondes utilisees:

Vysis SS18 Break Apart FISH Probe kit (CE)
Vysis EWSR1 Break Apart FISH Probe kit (CE)

Conclusion:

L'hybridation in situ flurescente realise a l'aide de la sonde EWSR montre un rearrangement du gene EWSR dans 100% des noyaux analyses (100 noyaux analyses). Ce resultat FISH conforte le diagnostic de tumeur a cellules rondes (PNET/Ewing?)

L'hybridation in situ fluorescente realisee a l'aide de la sonde SYT-SS18 ne montre pas de rearrangement du gene SYT-SS18 dans 100% des noyax analyses (100 noyaux analyses).

The nightmare protocol

We met with the Oncologist on the same day and he briefed us of the next steps. It was very overwhelming and difficult to accept the protocol. Yuri has to undergo a heavy chemotherapy treatment plan as follows:

Yuri would also need to have a portacath installed in his chest that facilitates the chemotherapy.

Cycle Week Due Treatment
1 1 04/11/2019 VDC
2 3 18/11/2019 IE
3 5 02/12/2019 VDC
4 7 16/12/2019 IE
5 9 30/12/2019 VDC
6 11 13/01/2020 IE
  13 27/01/2020 Surgery / Radiation
7 15 TBD VDC
8 17 TBD IE
9 19 TBD VC
10 21 TBD IE
11 23 TBD VC
12 25 TBD IE
13 27 TBD VDC
14 29 TBD IE

Protocol - Page 1 | Page 2

Fast forward two months, we have undergone four cycles already. Here's a projection timeline assuming nothing changes:

Cycle 1 - VDC

Yuri was admitted to the hospital on a Monday. He went through different rounds of tests before they operated and installed the port-a-cath over his chest. The operation was very successful and within the week it healed magnificently.

Portacath

They started the chemotherapy on the same day (sometime around 5PM) and the cycle ended on Wednesday at around 12:00PM when we were released from the hospital. The hospital stay was acceptable, Yuri was mostly nauseated which was constantly controlled with Zofran. Once we got home, we assumed we were safe from side effects; Turns out it was completely the opposite. Yuri had severe jaw and throat pain that would not allow him to eat, drink or even sleep; It was hell for about 4 days. We assumed it was the Neupogen shots he's been taking daily but even after stopping them we problem remained just as severe. Nevertheless, his pain settled at the beginning of the week after just to convert to severe stomach pain, that lasted for over 4 days. We took him to the emergency room and it turns out he had severe constipation, which after a couple of Enemas, rounds of laxatives, ended up clearing one day before we were admitted for Cycle #2. It turns out, Vincristine is quite the mean ass chem.

The poor thing literally had one, single, night's rest.

Cycle 2 - IE

The second cycle was for five days but it was far more tolerable. Yuri was not complaining from any pain beyond the mere nausea and he was eating properly throughout the treatment that week. We went home on a Saturday for a week's rest and we were very much in control of the side effects. Yuri still had the occasional vomit, but we noticed that it was mostly due to the medicine he has to take to control ulcer and constipation.

We finally had a good relaxing week. It's worthwhile noting that during this cycle, Yuri had lost almost 95% of his hair, he lost the remaining 5% the following resting week.

Cycle 3 - VDC

The third cycle was the dreaded one. Considering we knew what to expect, we were scared shitless of the Vincristine cycle again. Yuri was fantastic throughout the period and was eating properly. The biggest problem we faced was vomit which was caused by the smell and taste of the laxative he had to take while nauseated. The doctor boosted the frequency of Zofran and decided to swap out the laxative he was taking from Forlax to Transilax.

We got rid of both causes of vomit! We got home on Wednesday dreading the side effects of vincristine but none were glaring and Yuri was having calm days and sleepy nights for the first 4 days. On the fifth day [monday] Yuri no longer slept properly and started randomly moving around while sleeping and waking up. By the 7th day he had major pain in his mouth and had not pooped for 3 days. Given his platelets were around 120, we gave him enema and cleared his constipation, however he was still complaining about pain in his mouth and had not eaten for a day. Apparently we did not escape the horrors of VDC. Yuri has a lot of mouth sores despite all the medication he's taking. The doctor swapped out Medistan temporarily to Fluconazole but it was to no avail. Yuri's pain kept intensifying to a point where we took him to the E/R one day before his 4th cycle where he was given Fluconazole intervenously as well as pain medication.

Or so they say, Paracetamol does nothing for such intense pain.

The pain we were previously attributing to Vincristine, may have been misplaced. Mouth sores seem to be a common side effect of Doxorubicin as mentioned here

Whether you experience mouth sores while undergoing chemotherapy depends on the type and dose of medication you receive, as well as how often you receive your treatment. The chemotherapy drugs most likely to cause mouth sores include:

Capecitabine (Xeloda)
Cisplatin
Cytarabine (Depocyt)
=> Doxorubicin (Doxil) <=
=> Etoposide (Etopophos) <=
Fluorouracil
Methotrexate (Trexall)

Mouth sores caused by chemotherapy treatment usually develop a few days after treatment begins and go away within two or three weeks after stopping chemotherapy. The mouth sores usually reach their peak around the seventh day after chemotherapy treatment ends.

While Etoposide is in the list, We witnessed no mouth sores during the Ifosfamide and Etoposide cycle (so far). Perhaps the cleansing process in the IE cycle plays a role?

We also have to care for the wrist wound

The wound story did not end, but it's not suitable to discuss the whole progress in the earlier section given the time it's taking to heal. When Yuri was sent in to install the Portacath, we asked the surgeon to fix his wrist wound. The surgeon explained to us that had Yuri not been going under for a Portacath, he would not accept to stitch the wound granted chemotherapy will interfere with the wound healing. He gave us a headsup that it's quite possible for the wound to open up again during the first two months of chemotherapy. He also gave us specific instruction on how to care for the woundby disinfecting it once every 3 days and applying a cream called MediHoney, a rather expensive cream that truly did wonders. The wound spat out 2 stitches so far and this is the progress 1.5 months after the operation:

Wound MediHoney

How we're managing everything

It's quite difficult to say the least, not knowing what to expect, being able to manage the entire ordeal with no hiccup. We have incrementally built a process that would be suitable for the present and we're still improving it incrementally.

Yuri's school year

They are being quite supportive and they're sending a daily sheet with his twin brother containing everything they have learned throughout the day. After we've met with them in person, they've assured us that the school is behind us in all aspects. Nevertheless, the treatment takes a huge toll on the body and while we are currently homeschooling Yuri, he does get tired relatively quickly. Nevertheless, he's been fantastic catching up with the work being done in school.

He's eager to see his friends at school, so we're waiting for an opening where he has no treatment and his immunity his high, that's when we could probably send him (supervised) to school to see his friends.

Dealing with our daily job

My wife was freelancing for the most part, so she took a break in order to focus on Yuri. From my part, the company I work for has been extremely supportive, knowing how difficult this process can be, they gave me the green light to work when I can from anywhere I can. The entire process requires me to be almost everywhere so being able to freely move around without worry makes things a lot easier. I'm ensuring the utmost transparency with the company, giving them the full week's expected availability or unavailability (if any) before hand.

Dealing with the hospital schedule

Considering the amount of time we spend in the hospital, we are taking turns caring for Yuri and the kids.

Hospital Week (3 Days) Day 08:30 09:30 13:30 17:30
Omar Monday Admission Office Home
Tuesday Hospital
Wednesday Office Release Office
Roula Monday Hospital
Tuesday Home
Wednesday Hospital Release Home

The five days (or 6 nights) stay in the hospital is pretty much the same, except we're there for more.

Hospital Week (5 Days) Day 08:30 09:30 13:30 17:30
Omar Monday Admission Office Home
Tuesday Hospital
Wednesday Office Home
Thursday Hospital
Friday Office Home
Saturday Hospital Release Home
Roula Monday Hospital
Tuesday Home
Wednesday Hospital
Thursday Home
Friday Hospital
Saturday Hospital Release Home

It's worthwhile noting that during a hospital week, we're never home at the same time with the kids.

Coping with the long hospital stay

We follow a checklist that allows us to properly prepare for the hospital stay

As a bonus, here's what Yuri's hospital room normally looks like

Yuri's Room

Every Sunday before a hospital week, we visit the closest printshop and print out any image he wants to hang in his room. This completely brightens up the room.

Keeping track of things

Being systematic helps a lot with the process especially when it comes to tracking things. There are numerous papers that we have to keep with us, numerous approvals and signatures that we need to get, and the oh so many prescriptions, force us to be quite methodical. We're maintaining a physical document file where we stored everything we need. We take notes all the time and keep track of the admission, release, immunity shots, blood tests and so on.

Tracking Calendar

C = Chemo,
B = Blood Test,
N = Immunity Shots,
T = Blood Transfusion,
I = Infection Treatment

The calendar has 15 months in total which are hopefully enough to cover the entire treatment schedule.

You can also download the calendar here

What exactly happens in the hospital

The hospital stay is very calm and goes smoothly, there's really nothing to it beyond the standard process that an individual has to go through. Here's a run down of an full treatment week (roughly).

Discharge Page 1 Discharge Page 2 Discharge Page 3
Discharge Page 1 Discharge Page 2 Discharge Page 3
Bill Page 1 Bill Page 2 Bill Page 3
Bill Page 1 Bill Page 2 Bill Page 3

What happens at home

As long as there's no pain involved, there's nothing different than any other day at home beyond taking extra care of what he's touching and what he's eating. Yuri also has to go through a number of medication and shots:

We've yet to get a week where Yuri's immunity is high enough for him to go to school and play with his friends. Hopefully, this changes soon.

Blood tests consolidation

This section aims to consolidate all the blood tests done, both during the hospital stay and as post-cycle checkup.

You can also download the blood tests here

In this sheet you'll find the following columns:

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