Visible from the hospital on top of a gas station
We’re waiting on the answer to what seems like every question at the moment, so things have gotten a little tense around here.
Zoe’s blood pressure is up and hasn’t responded as well as we or the doctors would like to her initial medication, so there is a decent probability we will not go home tomorrow. And, we’re still waiting on results from CCH on her genetic testing to determine if the disease is Familial or Acquired, which determines most of the rest of our year for us. Any day now.
Zoe had her first of the 4 weekly spinal taps this morning, and did well for that. She had a blood transfusion yesterday to help adjust her counts a bit due to the medications really building up now and taking an effect on her. She immediately perked up from the transfusion, her color improved and she seemed calmer, but her blood pressure is still causing concern. A Nephrologist was in yesterday to run some tests to ensure proper kidney function and isolate the cause of her high blood pressure to the medications rather than some other underlying cause, and the possibility of that underlying cause has us tied in knots a bit. She doesn’t need any more problems, nor does she need more holes poked or medications dripped. Poor thing.
Her BP is currently 157/89, which is really high, even for an adult. I don’t recall for myself but Michelle says she’s never even seen her BP that high. In recent days it’s been holding between 116/70-ish and 144/93-ish (when she’s really wound up/fussy), but this number today is her highest we’ve seen since the start of the treatment for her in a calm state. Possible outcomes are: she needs a higher dose of her current med, she needs a different med (if she’s not responding properly), or there is another cause of the problem. She’s to have an Echo cardiogram shortly to ensure it’s not her heart, and as mentioned there are tests out to determine kidney function.
Additionally she had an EKG taken yesterday to obtain a baseline reading — results so far on that are that test there is nothing to be concerned about.
Side note: the Internet connection here has been spotty the past couple of days, so it’s possible updates may be out later in the day or the next day depending.
Last week we were provided with a document sometimes called The Road Map, which lays out the frequency and rough dosage levels for the medications Zoe will be taking. It’s based on recommendations from the HLH-2004 treatment protocol I’ve discussed previously.
For the sake of documenting the process as much as we can manage, here is what we know about Zoe’s drug treatment plan from here on out. Please note this is not exact nor is it intended to be a recommendation of any sort, it’s intended only for context for those who might want to know what to expect in terms of treatment for this disease.
Click to enlarge
As you can see Zoe is to remain on Cyclosporine and Etoposide until either she has successfully had a BMT or until the disease has gone into remission and roughly 1 year has passed, or some combination thereof. We’l be administering her Cyclosporine at home, with outpatient visits to have her Etoposide treatments (administered over the course of 3hrs each week). Her Dexamethasone (nurses here call it Decadron(sp?), probably brand related) will taper off in coming weeks. She’ll receive anti fungal and antibiotic medications where necessary as well.
As of today, all of Zoe’s medications are administered orally with the sole exception of the Etoposide. This is good news for us, it means we’re still on track to leave the hospital the end of this week.
Evan has been encouraging me to contribute to this blog my perspective as a mother. During this upside down time of our lives Evan has been coping through researching the illness and treatments and writing about it. As Zoe’s mom, I have been focusing on the caretaking aspects of things, like making sure Zoe is well fed, eating well for her, talking and playing with her and holding her as much as I can.
One of the worst aspects of this experience is knowing the invasive procedures that Zoe has faced. Before my daughter had her central line put in she went through numerous blood sticks in her body and feeling helpless to console her I sang to her while she cried. The blood draws, the spinal tap, the bone marrow biopsy, the operation to insert the central line as well as all the medication she has received in her body somehow Zoe tolerates and comes through the experiences. It is disheartening to realize that she could go through all of these procedures and there are no guarantees with this illness and that it is very serious and life-threatening.
Despite the challenges we face at this time we make sure to savor our moments together. Zoe sure smiles a lot for a baby who has been as ill as she has been and her smiles mean everything. Holding her is healing for both of us.
It is difficult to feel like I cannot be there as I want to for my older daughter, who definitely has had her upsets with this experience and who regularly asks me when I am coming home. It feels awful telling Maya goodbye and that I will see her tomorrow and I feel sad that I am not at home to help her through her bumpy nights.
We make our time together count. I took Maya to a birthday party yesterday and she spent time with one of her favorite friends. We sing to Simon and Garfunkel when we are driving to and from various places. We play on the hospital rooftop playground where a few days ago Maya had her first experience with Red Light Green Light and shrieked with laughter as she ran circles around the older kids playing the game.
Time with Evan is different and confined to brief stints in the hospital room. Our first priority is our children and so we understand that we will be spending less time together than we would want. We look forward to having more quality time together in the near future.
We have received a great deal of support from family and friends. This has been overwhelming and has helped us to process and endure this devastating experience. We are so grateful to have such kind and caring people in our lives
After a short break for Michelle and I over the weekend that included a movie for myself (Shutter Island) and a birthday party and play date for Michelle and Maya, we’re back on schedule at the hospital. It was a really needed break and I’m grateful my mother is here and willing to sit in with Zoe while we try to see what normal life is like for a few hours.
There was a new doctor on over the weekend, somewhat younger and who mentioned she had only ever seen two cases of FHLH in her career. In both cases, they found it much much harder to treat the child than they have in Zoe’s case, lending us new hope that this may not be the Familial form of the disease. The reason they are making that judgement is her age, very young to present the acquired version, but as previously mentioned she was very very ill prior to presenting, which is one way that the acquired form manifests itself. We may have our hopes dashed soon enough when the genetic tests start to come in from Cincinnati Children’s Hospital, but in the mean time it’s what we are holding on to as a way to escape this disease without further harm to Zoe.
An update on Zoe’s numbers:
- Her weight has again dropped a bit (not a bad thing) to 13.5lb
- WBC: 3.2 Sat, 4.3 Mon
- Hemoglobin: 8.8 Sat, 8.9 Mon
- Platelets: 191 over the weekend and then 217 today
- Ferritin: relatively unchanged at 880
In general these are positive or neutral signs, though the WBC count is just under normal. Ferritin is a slow moving indicator, which is why they don’t check more often for it’s level. Platelets are steadily rising, a very good sign, and WBC and other counts are holding around normal range.
Right now we are on track to leave the hospital as of Friday assuming no symptoms reappear. We’ll be in regularly for medications and Zoe is set to begin getting a spinal tap every week for the next four weeks starting this thursday. She has to have general anesthesia each time along with the other inherent risks of surgery, so that’s a little unsettling. Every taste of good news seems to come with a bitter side dish around here.
Having raised Maya to 2.5 now and changed who knows how many diapers, I’ve seen some diaper rash. What Zoe is dealing with at the moment is more akin to the results of a napalm attack on her poor bum, so painful does it appear. It’s happening because of the steroid and etoposide medications, which between them give her diarrhea and make her movements rather toxic to the touch. We have to wear gloves to apply her cream as apparently it can hurt us through contact. Dr. B took a look and is moving her to some sort of hardcore diaper cream. We’ll see how that goes.
Thankfully the rash is the worst of her problems on this particular day:
- Her liver and spleen appear to be back to normal, meaning all swelling is gone and we are again seeing a good response to her chemo.
- Her weight is at13.8, holding steady.
- She’s starting Bactrim today to prevent a specific infection type that’s particularly dangerous to children in her weakened immune state.
- Platelets are at 178k, meaning she’s crossed the threshold into normal now; 3 cheers for that one.
- Hemaglobin is at 9
- WBC count is at 5.1
And as a nice end to the day, she was finally detached from her central line iv, allowing her to be free of everything but the little bit attached to her chest inside her onesie. She’ll be reattached whenever she needs medications, but at least we can say she’s strong enough not to need monitors and support fluids anymore. It’s pretty amazing how much difference it makes getting to hold your child and move freely without tubes and wires, and finally feel like she’s getting back to normal again.