Progress

We are still here. Phoebe has had some very good days. Great days in fact. She has started walking, riding her bike, and playing with Mae. The way they interact reminds me of how they played before all of this happened - before the stroke and the seizures, the fungal infections and brain lesions. It is a beautiful sight. Phoebe will say; "Mae, will you play with me?" and Mae's face will light up with a big "yes!". And then they will get right to it. I think Mae needs this as much as Phoebe and it does my heart good to see them together and happy. 

Yesterday, Phoebe had a CT scan of her lungs and thankfully things look good. The fungal lesions that we have been watching since October are smaller and overall, her lungs look better. We are making progress. 

This progress is a result of a lot of time spent at the hospital and endless dedication by staff throughout St.Jude. Phoebe is a busy girl and we are so thankful for the army of people who take care of her. Sometimes it seems that every department of the hospital plays a role in helping Phoebe get well. From oncology to transplant, the blood donor centre, the blood bank, pathology, radiology, child life, infectious disease, wound care, occupational therapy, quality of life, music therapy, psychology, nutrition, neurology, nursing, and the food services staff who have gone grocery shopping so Phoebe has her favourites when we are in patient - this hospital is a beautiful place and we are so grateful for the care Phoebe receives and the people who care for her and about her while also looking after sweet Mae and our whole family. 

We are at the hospital every day, sometimes two or three times a day if the day includes labs and doctors visits in addition to the daily two hour infusion of anti-fungal medicine. This medicine can cause a drop in potassium, so each morning we arrive to check Phoebe's potassium and then return hours later for the medicine. We try to time this around meal times and quality time so that it doesn't feel like we are always at the hospital. The days that include infusions of granulocytes are especially busy and include an overnight stay at the hospital. The granulocytes need to be given 12 hours after the antifungal medicine, so on these days, Jon brings Phoebe in to the medicine room at 4 am and I arrive at the hospital at 8:30am to donate granulocytes. The donation process takes about two and a half hours and the granulocytes are started once we are admitted to the in patient unit in the early evening. It's busy. Right now we are giving granulocytes once a week on Fridays and I'm hoping that I can continue to donate these cells to Phoebe although my hemoglobin has been slowly and steadily dropping. Phoebe is still neutropenic and she needs granulocytes to fight the infection, so in case I am unable to give, the hospital is testing a few family members to see if they are an HLA match to Phoebe, so they may be able to give too. Another reason St.Jude is great.

The plan for now is to continue with the granulocytes and the anti-fungal medicines that Phoebe is receiving and hope and pray that this progress continues and brings Phoebe to a point where curative leukemia therapy is possible. Next week brings an MRI of Phoebe's brain and a bone marrow aspirate. Phoebe is still neutropenic, but she is needing fewer transfusions and has some signs of count recovery, so we are anxious to see what is going on in her bone marrow. We need good news of healthy recovering cells and no leukemia as to effectively fight this fungal infection and heal long term, Phoebe needs her own immune system to recover. 

Phoebe has already greatly surpassed expectations and overcome so many odds - so much so that her doctors have started to refer to her as a miracle. If the shoe fits ... 

Go Phoebe go ... 

"There are only two ways to live your life. One is as though nothing is a miracle. The other is as though everything is a miracle." Albert Einstein 

The makings of a miracle ...

We are back at the Target House, returning to St.Jude daily for labs and an antifungal medicine that can only be given in the hospital. We are busy - caring for Phoebe is a full-time job, but we get to sleep in our own beds at night. It's the little things. 

Against all odds, Phoebe has been doing well. She is getting stronger, and although she has a long way to go, things are improving. She is still weak, but she surprised us by riding her bike the first day we were home, and today she walked for the first time in three weeks. The fungal lesions are very slowly healing. It is a painful and slow process but we are starting to see some improvements.

Phoebe's bone marrow results show a very low level of disease (less than 1%) and an improved chimerism. I like to think the leukemia is "stable" and we are relieved that the clinical trial was successful. There are some signs of count recovery - Phoebe is maintaining a very low ANC and needing fewer platelet and blood transfusions. She will receive another unit of my granulocytes on Friday and after that ... decisions and plans are made one day at a time. 

We are hanging on, grateful for progress and hopeful for continued miracles. 

And now from Phoebe's Daddy who can tell it like it is far better than I can ... 

"I couldn't hold back the tears today. 3 weeks ago Phoebe was diagnosed with a very serious systemic fungal infection. Fusarium fungemia. If you google it you'll find that in someone like Phoebe who has no immune system there is a 100% death rate. The first thing the doctors did was to call in the quality of life team to ask us our expectations of the hospital when Phoebe's life was to come to an end. Instead, we fought for treatment using Jenny's white cells and 4 anti fungal antibiotics to build a bridge so Phoebe could gain some time for her own immune system to have a chance to return as we get further out from the last experimental trial which she responded well to. We fought for life. Today I cried. I cried because the bridge we built is holding. Phoebe's blood work today shows her immune system is in recovery and with it we will defeat this horrendous fungus. I absolutely love how Phoebe continues to put doctors on the side of hope. I believe".

Go Phoebe go

Phoebe is out of the ICU. It was a short stay - just twelve hours until we were back on the regular floor. In typical Phoebe style, she had a room full of very worried doctors, but she appeared to improve without any intervention as soon as we entered the ICU. In fact, as we wheeled her in her bed through the halls towards the ICU she perked up and pointed out the murals and pictures on the walls. It was and is very serious, but in that moment, it seemed that Phoebe just wanted to take a ride outside of her little room. 

We are now back on the regular ward and things are slowly improving. We are cautiously optimistic. Terrified but always hopeful. Phoebe is fighting and so we are too. 

Here is what we know ...

What we once called a rash is fungal

lesions that cover Phoebe's face, legs and arms. Most have turned black and are angry and painful. It is as awful as it sounds, but they are not as painful as they once were and there are no new lesions which is a good sign. They do have a lot of healing to do and they make Phoebe sad. When we ask her if she is feeling better, she says "no, because these spots are just not going away". 

The granulocytes are helping to temporarily boost Phoebe's immune system which together with the many medicines she receives is helping to clear the infection, but she still has very few signs of count recovery. We need count recovery and in the absence of this, Phoebe will probably continue to receive granulocytes. The plan is very much one day at a time. 

The infection/inflammation marker in Phoebe's blood is coming down and took another big jump in the right direction today. 

The CT scan of Phoebe's lungs shows the same small lesions from the previous infection and some other "gunk". The granulocytes were given right before the scan so things may look worse as they cause inflammation. 

We finally have negative blood cultures. 

Phoebe is still needing blow by oxygen, mostly just when she's sleeping and her breathing has improved. 

Today, our infectious disease doctor told Phoebe that she was their "most improved patient". Go Phoebe go. 

Today, Phoebe colored a picture and wrote her name for the first time in weeks. 

We love her so much. 

We are following Phoebe's lead and hopeful that she will be able to leave the hospital soon. We are still isolated, so when Phoebe is in patient she spends every day in a little room. It's hard and she usually shuts down and refuses to leave her bed. She is depressed and some time outside of these walls would do a world of good.

Mae is busy making friends and dates to colour with many of the nurses. She is surrounded by love, support and friendship and for that I am so grateful. Phoebe's nurses are beautiful kind souls who recognize her for the unique little person that she is and care for her with incredible patience and understanding while always making time for Mae. 

Thank-you all for continuing to think of Phoebe and our family. Your words and kindness help us through. 

ICU

Today Phoebe was transferred to the ICU. She received granulocytes last night and early this morning and although things seemed to go smoothly, by this afternoon, she was breathing very fast. She was struggling to breathe, needing more oxygen and just working too hard. Our room was full of people - nurses, a nurse practitioner, a respiratory therapist, fellows, the attending physician for the leukemia floor, and the ICU attending. Thankfully, Mae was being entertained and kept busy with some wonderful nurses and volunteers. Phoebe had an X-ray and more blood tests to check blood gases both of which looked okay, but within minutes of the ICU attending laying eyes on her, we were on our way to the ICU. 

As I write this, Phoebe is settled in the ICU. She is still on blow by oxygen - mostly because she refused the nasal cannula - and the amount of oxygen is greater. She is still breathing quickly and we are worried. So very worried. 

We are not sure what is causing her breathing difficulties but we are hoping it is "just" the granulocytes going to work on this horrible infection and things will calm down soon. 

Please keep our sweet and feisty Phoebe in your thoughts and prayers. 

Hospital life ...

It has been a long and busy two days. Some mistakes were made with Phoebe's care and we are exhausted. 

Yesterday morning I went to the blood donor room to give my granulocytes. Phoebe's labs were drawn at 4am and her platelets and hemoglobin were low - her platelets were critically low. Before I left her room just before 9, they had been ordered and all was well.

Jon stayed with Phoebe and Mae and when I got back after noon the blood was just about to start - a blood transfusion in someone Phoebe's size can take up to three hours, but platelets are much faster. The platelets should have been given first. By the time the blood started, Phoebe's nose was bleeding, her poop was dark and tested positive for blood, and she had a huge bruise on her eye lid. Her blood wasn't clotting and so she was bleeding. By 5pm there were still no platelets and even Phoebe started asking where they were. I write asking, but she was actually shouting "I need platelets!! Where are my platelets!!", as she wiped blood from her face. Even at four years old, she knows better. 

The platelets were finally started at 5:30 pm - over 12 hours after they were found to be critically low. Phoebe spiked a fever soon after they were started which was  probably because she wasn't getting the Tylenol that was scheduled every four hours to keep ahead of the fevers (we had three different nurses in one shift yesterday and it was pure chaos). Because of the timing of the fever, it was documented as a transfusion reaction and the platelets had to be stopped. Despite the fact that Phoebe desperately needed them, they were stopped, sent back to the lab, and another unit was sent for. All of this and Phoebe still needed the granulocytes - and they expire. 

The first unit of granulocytes was started in the late evening and finished in the very early morning with no problems. The next unit was supposed to be given shortly after, so we finally went to sleep - Jon with Phoebe, and Mae and I in the parent room connected to Phoebe's room. 

After very little sleep (beeping pumps, pull up changes, watching and worrying about Phoebe) Jon woke up early this morning and noticed the granulocytes were missing from Phoebe's pump. He quickly went to get the nurse to tell her that they would expire and to ask where they were. Despite his efforts, they didn't arrive in time. I woke up to Jon telling me that I needed to come into Phoebe's room because the second unit of granulocytes had expired. The granulocytes that I spent 3 hours donating for Phoebe - the cells that I am only allowed to give six times in six months. These precious cells that Phoebe isn't making on her own but desperately needs to fight this fungal infection were wasted. 

I can't put into words the sadness and just pure frustration I felt this morning. Everyone was frustrated but no one seemed to know how the mistake was made. After the day we had yesterday - a day filled with too many careless mistakes and chaos, I am not surprised that this bigger mistake was made and that was the consensus at the end of the day - the chaos of yesterday led to mistakes today. 

Phoebe's situation is so fragile and precarious, she just can't afford any mistakes. 

Today, however, is a new day and we are moving forward. As the doctor said this weekend - the good thing is that Phoebe is stable. She is, and she did receive some granulocytes so all is not lost. The inflammation marker in her blood is slightly lower, she is not requiring any oxygen and there were some signs of count recovery, but the latest blood cultures were still positive for fungus so there are also a lot of reasons to worry. The doctors have made some small changes to Phoebe's antifungal medicines and the plan is to continue the granulocytes next week. 

I very rarely "vent" or write about mistakes and frustrations that happen in our day to day hospital life, and I think it's because we are usually just so grateful for the many amazing people who care for Phoebe - they are incredible and we are grateful - we always are - but today after all that has happened I just needed to vent a little. 

We are holding on to hope and grateful for any progress. Last night despite the chaos and stress that Jon and I felt, Phoebe was up and asking to play and watch movies. She even had some snacks. She is still having high fevers and she has a very long way to go, but we are taking things one day at a time and always hopeful.