So….it feels like a lot has happened since Thanksgiving and we are still adjusting and attempting to wrap our heads around it….so here goes!
As we entered the month of December, Poppy continued to battle the Norovirus…becoming totally depleted, despite our efforts of tube feeding pediayte and pediasure, and pushing food. She had also completely transitioned to the Sirolimus (new anti-rejection drug) replacing the Cyclosporine she has always been on, in hopes of protecting her kidneys and lessening her chances of developing Coronary Artery Disease (super common years post heart transplant). Things seemed to be going well overall with the med switch, but something was taking her down..
While she had many moments of being her happy sweet self, Poppy also experienced lot of fatigue as well as pain. Poppy has had many terrible viruses, and though she’s had every reason to be in pain in the past, she really typically powers right through illnesses… She has been through so much, and poked so often, her pain tolerance is really quite high…anxiety aside, she really is tough. For her to scream and sob in obvious pain was heart breaking and super concerning. We had also noticed that her low tone was gradually becoming more and more tight. Because of her hypotonia, holding her is like holding a 25lb. limp noodle. When we would pick her up, she had started bringing her knees to her chest and the stiffness seemed very challenging and painful to release 😦 Her muscles seemed so tight so fast…she would scream when we brought out the walker to practice 😦 Things deteriorated so fast 😦
At the same time….because of just transitioning to the Sirolimus, she had weekly labs checking drug levels and other markers to ensure the transition was going smoothly. The beginning of November, we started noticing things trending downward, which was expected and considered normal for what can be expected from immunosuppressants. By the last week of November, even more so…beginning of December both her red and white blood cell counts were extremely low…so low that the transplant team decided to hold her second anti-rejection medication, Azathioprine/Imuran, until her white count recovered. We were told if she developed a temperature at all, that we should get her to the ER immediately, as her ANC was 270, as she just didn’t have any reserve left to fight infection….
Well it happened…neutropenic with a fever. She was admitted for a few days for a few doses of IV antibiotics…meanwhile, her red and white counts continued to drop..her hemoglobin was most concerning, as it dropped rapidly. At this point, the Hematology/Oncology team was consulted and added to her specialties. She was discharged on a Friday afternoon, with the understanding that if Sunday morning labs were not showing improvement, the plan was to do a bone marrow biopsy to see what exactly is going on with her bone marrow.
Sunday morning came, and her labs were worse. They had no choice but to rule out leukemia with a bone marrow biopsy. Later on Sunday, she was re-admitted to get a blood transfusion and prep for her biopsy Monday morning. It took multiple tries with flight nurses and finally anesthesia was able to place an IV just before her biopsy. She has always been a hard IV and lab draw….she just has so much scar tissue..she’s been accessed so much.. 😦 She went in to her biopsy Monday morning and ended up getting blood Monday afternoon and evening. While getting the biopsy, labs were drawn. Her hemoglobin was 5.4 (normal range 11.8-14.7) , hematocrit 16 (normal range 35-44), platelets 41 (normal range 150-450), neutrophils still extremely low….they wanted to run a test to see how her body was metabolizing the azathioprine (it had been stopped for a week at this point), but her red blood cell count was too low to complete the test.
This all happened so quickly, and was so concerning…people who take immune suppressant medications are at greater risk of developing cancer…that, combined with the crazy amount of radiation she’s been exposed to with all the CT scans and x-rays to check her shunts for hydrocephalus…the chances of it being leukemia weren’t far fetched in our minds…we felt the Lord’s arms around us as many reached out to help and prayed for us…we just kept thinking back to the weeks prior as she was started on the Sirolimus and came down with Norovirus…she’s had so many terrible viruses before, but they have never taken her down like this. She wanted to lay around all day, she was in excruciating pain just picking her up to hold her, she wouldn’t eat but a few bites every meal… She’s so tough, this really took her down…fast. You could say we were/are stunned…hearts sank…weary..trying not to let worry and anxiety rule us..on our knees in prayer..she’d been doing so well…
The results came back negative for leukemia, but it was found that Poppy has Aplastic Anemia aka bone marrow failure. The Hematology/Oncology doctor explained that as young children, our bone marrow is made up of 10-15%fat, and 85-90% cellularity, and as we age, the ratio typically becomes more equal…so a person in their 50’s might have 50% fat and 50% cellularity. Poppy’s bone marrow showed 90% fat and 10% cellularity…very poor… The thought is that it’s due to the medications she must be on in combination with the Norovirus. There is also a chance that those aren’t what caused it, as many times it can develop without a known cause. In cases where medications have caused it, getting off of those medications can turn it around, but not always. Typically it means that patients can become dependent on blood transfusions, and in severe cases, treatments can include a bone marrow or stem cell transplant. We were and are so thankful that no Leukemia was found, however the more we read research and ask questions, we are understanding that Aplastic Anemia can be quite serious…
Poppy was taking 3 medications that can cause Aplastic Anemia, and so the process of stopping them has begun. Right now, the most likely cause seems to be from medications, so this is where we start. She was on Sirolimus (only on it for about 6 weeks), Azathioprine (on it for 18mos or more), and Keppra (on it since she was 10 days old). She has so far transitioned off of the Sirolimus and put back on the Cyclosporine. The Azathioprine is still stopped and will be until further notice. It’s really the best option to use with the Cyclosporine for immune suppression, so we’re praying she can tolerate it again some day. She’s been on the MMF/Cellcept which is the other option, and had constant vomiting and diarrhea on it, so we don’t feel that is the answer either. Right now, since the Cyclosporine is the only anti-rejection med she is on, she has to be on Prednisone, to be sure she has coverage against rejection while this is all figured out. Obviously long-term steroid use is not ideal, so we’re praying this is short lived, and she can be put back on the Azathioprine safely. The Keppra she is on for seizures, and has always been on. This is really a tough one for us..it’s always been so good to her and with all that’s happening, the last thing we wanted to do was to go messing with her neuro status by switching up her seizure meds. But after visiting at length with her Neurologist and Heme/Onc doctors, it seems it is the safest choice to give her bone marrow a chance at recovering. We are starting the transition to Vimpat…She has tried this one before in combination with the Keppra, and it didn’t seem to help, but because of the seriousness of Aplastic Anemia and the limited seizure med options she has because of the immune suppression meds she has to be on, it doesn’t seem we have a choice but to give it a whirl…praying that the Vimpat and Lyrica combination will work well together, and the transition off of Keppra is safe and seizure free…Ahh..those darn medications…can’t live with them..can’t live without them…
After Poppy’s bone marrow biopsy, she stayed in the hospital overnight and received more blood. She was discharged with a new medication called Neupogen, which is a shot that helps the bone marrow produce white cells.
The weeks following have been filled with lots of labs and follow up appointments. So far, her labs have improved enough to discontinue the Neupogen and space out lab draws to 2 weeks! Everyone is very encouraged as it seems that things are starting to turn around. Her muscles and energy level have definitely improved as her labs have shown improvement. She is even excited to work on walking with her walker again and has definitely gotten her appetite back!!! Hallelujah!!
We have truly felt the Lord’s arms around us as we have found ourselves very blessed by the people who care about us, near and far during this time of this new diagnosis and figuring out what this all means for Poppy and how we care for her from here on out. It feels like it’s been weeks, but it’s only been 2 weeks since she was in the hospital, at her worst..it’s all so new to us and still, in many ways, beyond our understanding …God’s grace is abundant and sufficient…we can feel your prayers. God is so good.
Because of the condition of her bone marrow, the docs would like to see her off of the medications that can cause Aplastic Anemia for a few months and then repeat the bone marrow biopsy at some point to see how things are looking. They have also advised us to keep her home for her therapies and school, as coming down with a virus with fragile bone marrow could land her in the hospital pretty easily. We are working with therapy to have homebound therapies which will start immediately after Christmas. She will also have home visits from her preschool teacher to help us keep her learning and developmental goals on track at home.
Praying that Poppy will continue to improve and be able to enjoy life out and about this spring/summer! Only time will tell how all of this goes…we so appreciate your prayers!! We know the Lord has all of this in His perfect hands…We are soo thankful to be close to such great medical care..I can’t imagine doing this in MT with life flights and a new baby, living away from home, and this new diagnosis…we still feel it’s clear the Lord has lead us here- her care and communication between specialties really has been amazing… and at the same time, we miss Montana and everyone in it, as well as the place and people we called our home away from home for 3 years, Seattle Children’s…wishing we could make it home for Christmas, but maybe next year. The Lord continues to guide, provide, and surround us with some pretty amazing people and so much prayer..it really is humbling…thank You Lord!!