Here we go... Kira's story

[Chris H]

Our family’s journey into/with Aplastic Anemia started in July 2011 when our daughter Kira (11 yrs old) developed a ‘wicked bruise’ after bumping her hip on the underside of a trampoline. We look back now and are very thankful she wasn’t jumping ON the trampoline!!

We took Kira in to see her doctor on July 11th; we had been noticing small red dots on her skin (petechiae) going back as far as April and now with the nasty bruise it was adding up to be more than just ‘sensitive’ skin. Kira’s doctor sent her for blood work and on July 14th we were contacted to come back in for another blood test to confirm the first results. We were also told that Kira was being referred to a pediatric hematologist as well. This was when we realized that there was more to the story than a few bruises and red spots.

Kira had her first appointment with Dr. Geoff Cuvelier at Cancer Care Manitoba on July 20th. It was apparent right from the start that Dr. Cuvelier was awesome with Kira. He took time to get to know her, explained a bit about what was going on with her blood and how they were going to find out what was up, and even stopped to make sure she was alright when he saw her putting her head in her hands out of the corner of his eye. Terry (her dad) and I were confident that we were in good hands!

Kira’s initial blood work was as follows;
WBC 4.8
RBC 2.97
HGB 101
MCV 100.7
PLT 25
Neut 0.92

Needless to say her Dad and I were in a complete state of shock. Other than some bruising there was NOTHING wrong with Kira, in fact she rarely even gets colds or flu’s. She had always been a little less active than her brother Ethan (9 yrs old), she was the one ‘butterfly watching’ on the soccer fields while the team ran around her, but put her in water and she went nonstop so we just figured team sports weren’t her thing.

We spent the next three and a half months visiting the clinic at Cancer Care every other week, or sometimes every week, to get blood work done as Kira started to develop nose bleeds on a regular basis. She went for her first Bone Marrow biopsy on July 27th and her second on August 31st. Both seemed to be inconclusive for diagnosis. Idiopathic thrombocytopenic purpura (ITP) and Thrombotic thrombocytopenic purpura (TTP) were in our genetic background but were both ruled out early on. Kira was tested for Fanconi's anemia (FA) and Shwachman-Diamond Syndrome (SDS), both of which came back negative. The doctor also felt Myelodysplastic Syndrome (MDS) and Dyskeratosis congenita were not indicated. Kira seemed to be proving to be a bit of a mystery. Of course all of these were foreign to us so off I went to research all I could about blood and bone marrow issues despite being advised that there is a ton of ‘scary’ information out there that might be less than helpful to read. In my mind the not knowing was worse; I had always maintained an interest in the science/biological field, so I promised myself to take everything with ‘a grain of salt’ and to wait for Dr. Cuvelier to tell us definitive answers.

Kira continued to go to school and carry on as before with some adjustments to her schedule. She was excused from Phys. Ed. and any activity that might cause ‘trauma’ to her system. We also made her aware that she had to be careful of friends that were really sick with colds or flu’s as she would be more susceptible. Kira was told to avoid head trauma, so… of course the very next thing to happen… she swam into the dock ladder at the lake right at her forehead. Nice big squishy purple ‘egg’ on her head for a couple of days. Then she was told to avoid activities that might cause broken bones or deep cuts. You guessed it… she tripped on a piece of rope and shredded the skin on both knees and her palms on the cement. Needless to say we quit telling her ‘specific’ things to avoid! She was ecstatic the day she received her own cell phone so that she could reach us in the event of a concern or emergency. And so the texting began… groan.

We brought Ethan in to meet Dr. Cuvelier at one point as he wanted to check his blood as well to make sure there wasn’t anything going on there. Ethan was very upset the night before and told us he didn’t want a blood problem as he already had asthma and it wasn’t fair. We had a good chuckle at that and explained – to a very confused looking boy – that we were not taking him in to ‘give’ him a problem just to check and make sure his blood was okay. He was a little relieved after we explained that he was not likely to have anything wrong with his blood at all but the doctor wanted to compare it to Kira’s. They also took blood to screen him for bone marrow compatibility to Kira as it was felt a transplant might be necessary eventually. Results came back and Ethan is a perfect match for his sister!

Then on November 20th we took Kira in to Emergency at Children’s Hospital with signs of an appendix issue. She had been complaining of stomach pain for three days already but that has been Kira’s complaint since she was little so we had chalked it up to nerves. However on the 20th she couldn’t laugh at the supper table without saying ‘Ouch’ so off we went. On the morning of Nov 21st Kira had her appendix removed and was in a recovery room by just after noon. We settled in for our two day stay and started laughing about how we thought when we came in that we’d just be sent home with something for a ‘tummy ache’.

It was truly unfortunate, and almost more than I could believe, when Kira started to show signs of complications. First a fever that they attributed to a little infection around her belly button (the appendix had split when it was being pulled out and they thought maybe some got onto Kira’s wound) and then no signs of bowel movement and new stomach pains.

Kira went in for bowel surgery to fix a kinked and fused bowel and abdominal abscess on November 29th. Her recovery was excruciatingly slow as the surgical team had to work with and around her fluctuating blood issues, numerous nosebleeds, stomach pump, TPN nutrition, etc. She was on three medications for nausea that was being caused by the three types of antibiotics being used to reduce the infection in her abdomen and the new one in her PIC line. The three weeks in hospital were almost as hard on her brother Ethan as he was at home without his sister AND Mom. He would visit as often as possible and I would try to go home to spend time with him but he still felt very lonely. We live in the country so he’s not able to just venture next door to find a friend, his closest friend is his sister.

Kira was finally released on December 20th and I will admit I’ve never had a better Christmas present than having our family all together again at home!

(continued in next post)

[Chris H]

(continued from previous)

January 18th Kira went for her third bone marrow biopsy as well as a skin test for Mosaic Fanconi Anemia. On January 24th, 2012 our whole family went in to Clinic to hear the results of this biopsy. Dr. Cuvelier said he was finally able to get a satisfactory sample and it indicated that Kira had less than 10% cellularity in her bone marrow – diagnosis Severe Aplastic Anemia.

Dr. Cuvelier explained that Kira had presented as a bit of a mystery at first and he figured that it was because she hadn’t fully progressed into the condition. If the bone marrow biopsy done on Jan 18th had been the first they would have reached a diagnosis immediately. HOWEVER… as they had taken the skin sample as well on the 18th and were currently culturing it for the Mosaic Fanconi test the team had agreed that if Kira remained stable at the present levels they should wait for the results of that skin test to come back. The treatment for Aplastic Anemia is not the same as for Mosaic Fanconi’s and although the team feels it is SAA they do not want to take a chance with her well being and are waiting for a definitive negative on the Mosaic test before they proceed.

Terry and I feel this is the best course as her levels remain stable and she is well (rapidly knocking on wood as I type) so this gives us all time to prepare and adjust to the idea of what happens next.

Kira’s levels now:
WBC 3.73
RBC 2.65
HGB 91
MCV 104.2
PLT 30
Neut 0.69

We have a rough estimate of late April for Kira’s transplant with her brother as the donor. Ethan is already stressing about the time away from his sister and I as the last hospital adventure is still fresh in his mind. He is also worried about donating but I think Dr. Cuvelier helped him to see that it’s not as big a ‘scary’ thing as his mind had grown it into. The nurses we met that will be Kira’s team are also very helpful in making Ethan feel a willing partner in this process too. We are very fortunate to have a group of awesome people looking after our family!

As an aside note before I end Terry and I wonder if there is more to Kira’s condition than just “unknown cause”. Kira’s follow up appointment with her appendix surgeon included a pathology report that indicated that Kira’s appendix showed not only new inflammation but also chronic inflammation. Kira has been dealing with stomach issues since she was very young. As early as Kindergarten she would end up staying home with tummy aches, sometimes getting sick to her stomach sometimes not. As the years went by and they never seemed to get any worse or better and the doctor had no answers (ultrasound in 2009? proved nothing) we assumed it was nerves. Looking back now we wonder if she has had very tiny flares from her appendix all her life. We also wonder if this may have been the trigger that sent her immune system into overdrive thereby attacking her own bone marrow… We may never know but I am just putting it out there as a possibility/point of discussion.

So… after babbling on forever all that seems left to say is “Here we go into the rabbit hole…” (Alice in Wonderland)

[Chris H]

We have a date...

March 21st Kira goes in for the conditioning and March 26th will be transplant date.

Tidying up all the last minute things and away we go.

Excited, nervous, scared and yet calm and ready to 'Get this done' (as Kira put it)

[evansmom]

Great news Chris, wishing Kira all the best.

In your previous post, you wondered if the cause of Kira's AA had anything to do with her chronic inflammation etc.

I'm not sure if I've ever posted my thoughts on this specific subject or not but sometimes I wonder as well if something like that caused Evan's AA. Even though he tested negative for all of the viruses (eg Parvo) that have been known to cause AA when his bone marrow failed, it still doesn't remove the fact that for many months (if not a year or more) before we knew he was sick, he experienced drenching night sweats on and off. He never felt ill with them and never registered a fever any time we would check it. I would tell his doctor "this is not normal", my daughter never once had a night sweat. But everything always checked out fine, not even a swollen lymph node to be found.

We learned in transplant that Evan had cysts in his right kidney. To this day they remain and are considered simple and no cause for concern. As well, to this day, Evan experiences drenching night sweats on occasion, maybe 2-3 nights per month (as far as I know).

There are times I think I'd like those cysts biopsied for my own piece of mind but so far, I have just kept these thoughts tucked in the back of my mind.

For all of these idiopathic cases of AA, I believe with all my heart that there is a trigger for each and every one.

[Ellensmom]

Thank you for telling us Kieras story and congratulations on the date for your daughters BMT.
We were also nervous, exited and scared going into our daughter Ellen’s BMT, but now 14 days in things could not be going better and we are more confident each day that she will beat her disease – MDS.
I have answered your post in the paediatric forum – and hope you might find my suggestions useful.

Like you and Evans mom I cant stop wondering, what could have triggered Ellen’s MDS. And I was a bit chocked to read about Evans night sweats. About a year prior to Ellen’s diagnosis, she began sweating through nightgowns and sheets. This happens a couple of times each month. Our doctors have not offered any explanation, however a seasoned nurse told us she had met several MDS and AA kids who had the same experience…

All the best
Freja

[Chris H]

Thanks for all the support and glad to know I'm not the only one wondering what 'other' things could be causing aplastic anemia and other bone marrow failures to occur.

Started a CaringBridge website for Kira (thanks evansmom for the idea) and now our whole network of family and friends can get the daily scoop from one place. No more phone calls, txts, and emails sent to 100 addresses, etc. If someone has questions they get ahold of us and it seems to work well!

Off to finish up the last few appointments this week before the ball is fully rolling.

Best wishes to you all!!