Henry update…

again, written by our dear friend anna…

I had a chance to talk with Ginger tonight [last night, 14th] and wanted all of you to know how sweet Henry is doing.

He is feeling MUCH better!  He wants to go home, and of course his parents want him to be able to.  However, there are still a few issues that need to be resolved before that is to happen.   Henry tested positive for a virus that causes Roseola, which will generally clear up on its own unless (as in Henry’s case) there are other complications. Here is how we can continue to pray.

Henry’s heart rate, while he sleeps, is unusually low.  Upper 40’s to lower 50’s.  It is rather strange and they don’t seem to have a definite answer for it other than that he is still sick and is recovering.  We can pray that his heart will begin to work as an active child’s should.  Along these same lines, please pray for Joey and Ginger as they take turns sleeping at the hospital.  I think we all know how difficult it is anyway, but combine that with an alarm going off repeatedly throughout the night due to Henry’s low heart rate and you can imagine how worn out they must be.

hanging out with mama and Mousey

Another alarming issue is Henry’s low platelet count.  His body seems to be recovering well at this point, but his platelet count is in the 12,000 range.  An acceptable count would be in the neighborhood of 150,000.  But Ginger told me that the last time they did a blood test on Henry when he was well, his count was between 300,000-400,000.  Hopefully those numbers help you to understand how low his count really is.  Again – it is odd that it should still be so low considering his improvement.  Please pray that this number would dramatically jump in the next 24 hours.  He will not be allowed to go home should it remain this way.

So far, the tests that are being run to rule out HLH have been coming back normal.  There is one test still out that checks the NK (Natural Killer) cells – part of white blood cells.  It is rather important that this test come back with normal results.  Ginger was told today that if the results are abnormal, then hematology will recommend that they proceed with Henry as though he has HLH and begin “treatment”.  What they mean by this is that they would start Henry on chemotherapy and radiation and ultimately, a bone marrow transplant. A very drastic response to one test.

That being said, Ginger and Joey are not convinced that an abnormal test result is a absolute that Henry has HLH.  They would have to consult with many other doctors before that – and Henry is doing so well now, that it seems unlikely that  the test would be anything BUT normal.  But we also know that things have not gone “normally” for these boys from the beginning, so please pray for that normal result!

helping the nurse remove the IV tube

Caid and Ian

Please also remember Caid and Ian at home.  Caid began school this week, so it was a particularly difficult time for the family to be split in so many different ways.  Pray for a spirit of peace upon Caid and Ian as their routine and structure is in a time of upheaval.

Also ask for a huge measure of blessing and grace to be poured out on those that are stepping in to help care for them while Ginger and Joey tend to Henry and work.

Joey and Ginger are concerned that there could be some underlying complication with the boys’ immune systems that has not been found, or cannot be found due to the very little known about HLH.  There seems to be a pattern in their illnesses.  One that would indicate that the same “thing” is being triggered every time, causing the boys to react in the ways that they have every time they become very sick.  If this is the case, and something can be done, please pray for wisdom for these doctors.  That they would be able to see into this disorder, make sense of it, and be able to prevent these situations from becoming what they have.  Each time these boys get sick, it becomes a gut-wrenching guessing game for their parents of what to do, when to do it, am I just being paranoid?  May the Lord give them an abundance of mercy and clarity in these moments.

I know that this is such a well-known and used verse, but each word in it speaks to their trials:

6    Do not be anxious about anything; but in everything, by prayer and petition, with thanksgiving, present your requests to God. 7 And the peace of God, which transcends all understanding, will guard your hearts and your minds in Christ Jesus.  -Phil. 4:6-7

So much anxiety surrounding the well-being of your children, anxiety about whether or not the doctors are doing the right thing, anxiety about whether or not YOU’RE doing the right thing, anxiety about their future.  But by prayer and petition – OUR prayer and petition, on their behalf – with thanksgiving for the lives of these two little boys, we make our request to God.  Knowing that He is able and willing to give a peace that passeth understanding – even when we question what is happening.  Rejoicing that He WILL guard their hearts and minds.

Thank you, to all of you.

In Him,
Anna

Prayers for Henry

I cannot thank you all enough for the prayers on behalf of Ian last month.  As you all know, he is doing very well, receiving continued care and follow-up appointments.

The reason for this post, however, is to let you know that Ian’s twin brother, Henry, has been admitted to the hospital as of last night.  He had been struggling for a couple of days with a fever, vomiting and fatigue that seemed to get worse as time went on.  Joey and Ginger took him in on Sunday evening to be safe.  The doctors are currently running a barrage of tests in hopes to pinpoint if there is anything that they might need to address.

big brother (and mousey) to the rescue

If you are not aware already, Henry has NOT been diagnosed with HLH as Ian was.  They are, however, checking certain “markers” to make sure that what is going on with Henry is not indicative of HLH.  I do know that he was feeling a little bit better this afternoon, but will hopefully know a little bit more about any test results tomorrow.

I know that they count it as precious that each one of you loves them so very much to stand with them in prayer.

~credits to our dear family friend anna for writing this.

a little relief

Once Ian was put on the second ventilator last night, he was able to stabilize, and has since shown some improvement. Small improvement, but we’ll take it!

The doctors are performing the bone marrow aspiration today (which is when they take bone marrow to have it tested for possible problems such as infection).

ian on the second ventilator

I’ll post more as we learn. Thank you all for everything… ~~cnp

urgent prayer needed for Ian

Ian was taken to the ER at CHLA last night for the second time this week. He is still hospitalized and he is very sick. Right now they suspect he could have relapse of HLH (his underlying disease) or rejection of his bone marrow transplant. They may do a bone marrow aspirate today. Doctors round at 9 am and we hope to find out more from them then.

pic from a hospital stay last month

Day +148, update from Ginger!

Time for an update!

leaving CHOC a few weeks ago

We have been going to CHOC every week on Tuesdays recently, as there was concern over Ian’s donor chimerism numbers which continued to drop (chimerism basically means a mix of cells – Ian’s and Caid’s). From the beginning we have been told that the number itself isn’t as important as the trend of the numbers, and a trend downward isn’t good.

Ian’s Chimerism chart

A quick breakdown of where his donor chimerism numbers have been – this percentage represents Caid’s donor cells:

July and August they ranged 88-86%

At the beginning of September it dropped to 80%

Mid September it was 76% and they started seeing us every week again (we had previously been moved to bi-weekly appointments)

9/25/12 –  66%

10/2/12 –  70%

10/9/12 –  73%

feeling good!

We get the results from the previous week each Tuesday, so we just received the 73% news today, and they did a blood draw to find out where the number is now. The stabilization of numbers is VERY good and we pray that it continues to hold steady. If his numbers are dropping, there is risk that HLH could reactivate. So, if they see the numbers falling, they would do a procedure called a Donor Lymphocyte Infusion (DLI) that they can do to “boost” the donor numbers, before risking the HLH flaring again. Caid would have his blood drawn and they would separate out the Lymphocytes, and give Ian an infusion of them.

This procedure can increase the risk of developing Graft vs. Host Disease. The main difficulty with DLIs is that there isn’t a lot of information for when exactly they should be given (in terms of what the chimerism numbers are doing). The is evidence of some kids with HLH who have very low percentages for their chimerism numbers, but as long as they are holding the numbers steady, they do not have HLH flare up and may not need any DLIs.

So, if you would like to pray that his numbers hold steady and we can avoid the whole thing entirely, that would be appreciated!

All three boys got sick soon after the school year started. Gotta love school germs! Caid came home with a cough and that night had a fever, from which he recovered, but not before passing it to Henry. Henry also got a fever, which sent us to the ER at CHLA for blood work. Our Hematologist/Oncologist at CHLA had recommended that I take Henry in for blood work tests anytime he got a fever to rule out HLH, since Henry had gotten so sick with the same virus that triggered Ian’s HLH and had also had some strange blood work during that hospitalization. Thankfully it was all normal blood work and we got to go home diagnosed with an ear infection.

henry and the balloons

Henry had an immune function workup at CHOC, to perhaps give us more information about his risk of having HLH. When we found out we didn’t have any genes identified for the mutation, we knew that we couldn’t have any definitive answer, but these tests were offered as something we could do to get as much information as possible to find out if his immune system is functioning normally. These tests were also done on Caid as the donor, and results came back normal for him. We are still waiting on Henry’s results.

waiting to see the doctor

Ian has been doing extremely well with his physical therapy. He has a physical therapist who comes twice a week to work with him, and he has been making great progress since being out of the hospital. He just got orthodic inserts for his shoes this week (they are the cutest!), which we are told will likely help him with his balance by helping everything be aligned properly. He also has speech therapy once a month, and has been saying more words recently.

Lastly, all our boys are a year older now! We had a small family gathering (to minimize germ interaction) to celebrate Henry and Ian’s 2nd birthday and Caid’s 7th birthday (their birthdays are five days apart)!

birthday cupcakes!

Thanks to everyone for their support and prayers!

improving your Support The Adams experience…

I’ve added some links and additional info for HLH awareness and ways you can help spread the word, and even help find a cure for this disease.

*First of all, Liam’s Lighthouse Foundation is ecstatic to announce the funding of its first research grant to Cincinnati Children’s Hospital Medical Center in the amount of $100,000 over the next two to three years for the research of gene therapies for Hemophagocytic Lymphohistiocytosis (HLH).

*Also take a look at the recently launched Histiocure.org. Their mission statement: “Founded by families fighting histiocytosis. Our goal is simple – to find a cure for this disease by 2021. We are an all-volunteer organization – so that 100% of your donation will go directly to research for a cure.”

I’ve put a few links up at the top of the sidebar for general HLH info.

You will also see a new section a little further down the sidebar we have “Other people you should visit…” one of these is a blog written by the father of  5-year-old boy named Cole who also has HLH and just underwent a BMT.

And lastly, there are still the links to our twitter feed, PO Box for the family, and the Paypal donation link. Thank you for taking the time to read, and for your dedication to this family and their physical, emotional and spiritual support.~~cnp

 

 

Difficult Day

Ginger writes:

Ian just fell asleep now after a pretty miserable day.

He tossed and turned all night last night and I woke up to him wretching at 4 am. He wasn’t throwing up much, since he hadn’t eaten much, but he kept having waves of the nausea. He got back to sleep from 6 until 8 am, but then woke up the same way. We gave him a dose of Anti-nausea medication zofran a little early to help, but it didn’t really work. So next we gave him Ativan, which made him super hyper, but he ate and drank a little. Next they gave him a much stronger anti nausea med which i was told is more specific for chemo patients. He wouldn’t nap until late in the afternoon, but really crashed.

After two hours, I tried to get him to wake up, and he really wouldn’t rouse out of sleep. I waited a half hour and tried again, and called the nurse to ask her opinion and find out if any of the medications would be causing it, which they shouldn’t have been at that point. She checked him and spent a lot of time talking to me and reassuring me that he was probably just really wiped out from the chemo.

I waited another hour and then tried again and then really started to get scared that he was so lethargic. He eventually came around and perked up a bit, but then became really nauseated and inconsolable again. As much as I didn’t want him to have more Ativan, I really felt like it would be better than him being miserable. So we did another dose and he got crazy hyper again (which is actually the opposite of what it is supposed to do.)

We are done with the chemo, today was a rest day and so is tomorrow, but the stuff he had yesterday is really a strong and notorious kind of chemo, plus I have been told that sometimes there is a cumulative effect from having 6 days straight chemo prior.

I tried to think of it today as “working” to get ready for transplant, but it is so hard and exhausting to see Ian suffering and I get so scared now when my kids act super sick. It is especially hard since he is little and can’t tell me what is going on or what he is feeling so I am left to guess whether it is typical BMT related sickness stuff or some scary complication.

I am very thankful for the kind and patient nurse who took my fears seriously and spent a lot of time with us today. More tomorrow…

Cincinnati Trip Recap

Ian & daddy dozing on the flight.

Ginger writes:

We are just getting our feet back under us after our trip to Cincinnati. It was a very long trip there and back, but Ian did great. We were delayed in Chicago for several hours, so our layover there was very long and we didn’t get settled into our hotel until 2:30 am and even then Ian was up throughout the night because of being overtired and not being in his own room, I think.

The next morning we were off to Cincinnati Children’s Hospital for our appointment with Dr. Filipovich. It was another long day, we were at the hospital for 5 hours. Dr. Filipovich really took time to talk with us and asked us a number of questions, in addition to the extensive medical history we had already given to the nurse. She said that she has been treating HLH and doing Bone Marrow Transplants for HLH patients for 35 years. She explained that HLH is a disease that causes a lot of inflammation in the body, and that she believes that this pre-existing inflammation that is smoldering in HLH patients is what causes the increased mortality rates (as high as 30%) for HLH patients who receive the Myeloablative Conditioning.

The drugs used with the Reduced Intensity Conditioning are different and do not seem to have the same effect on HLH patients. She stressed that she was talking specifically about HLH, and not Leukemia, that these results are peculiar to HLH alone. She also explained that Ian being in a stable remission and having a sibling donor who can donate marrow makes him an ideal candidate for the Reduced Intensity Conditioning.

She said that she would repeat some blood tests on Ian to look at immune function and also have these same tests done on Caid, to be sure that he was a good donor. They would expect Ian’s tests to be abnormal and Caid’s to be normal, since Ian has HLH and we hope that Caid does not. In order for the bone marrow transplant to cure Ian, his diseased immune system needs to be replaced by Caid’s healthy system. (CHOC had already run these tests and they have come back with normal function for Caid.)

I also hope that we can do these tests for Henry to give us clues about whether he might be affected by HLH or not. She also spoke well of Dr. Neudorf, the head of Bone Marrow Transplant at CHOC, who she had trained a number of years ago, and from the beginning, we liked that Dr. Neudorf had a professional relationship with her.

more blood!

Dr. Filipovich also asked if we would be willing to give some of Ian’s blood for their research on HLH, since he has HLH, but the mutation is not carried on any of the genes they currently know about.

Although it was a very tiring and long trip, I am so glad that we went because it did give me the peace of mind I wanted. I know that we are doing the right thing for Ian, no matter what comes, and I feel ready to sign the consents next week at CHOC.

Caid & Henry pose for a picture to send to Mom, Dad and Ian.

Moving Forward…

Ginger writes:

Things are starting to move forward quickly.

Caid and Ian will be having their pre-transplant workups at CHOC next week. We asked CHOC to have Dr. F, the foremost HLH doctor, consult on our case and we also have an appointment to see her in person at Cincinnati Children’s Hospital the first week of May. This is mostly to give me the peace of mind and reassurance going into this that we have the opinion of the doctor who knows about the latest advances in HLH and bone marrow transplant for HLH.

When I last spoke with our nurse at CHOC, the plan is to go into the hospital the second week of May to place Ian’s broviac line and begin pre-conditioning treatment, with June 1st being the transplant date. This may change, and the doctors are still finalizing plans for timing of one of the drugs, which will affect things too, but we know it won’t be long now either way.

Update: new answers from CHOC

Ginger Writes:

Last Friday, we met with the doctor who is the head of Bone Marrow Transplant at Children’s Hospital of Orange County (CHOC) and the rest of the BMT team there. We came away with a much better feeling about the plan. We had clearer communication from the doctor and nurse there in answer to our questions and from what we discussed, we feel more certain about moving forward using Reduced Intensity Conditioning.

That is not to say that there are not risks involved with Reduced Intensity Conditioning or the BMT itself, there most definitely are still side effects, both short and long term and risk of death, but with Reduced Intensity Conditioning, the risks associated with the toxicity of the chemo is minimized, since it is not as strong. *With RIC, Ian’s HLH disease will not be as effectively wiped out by the chemo they give prior to the transplant because the chemo isn’t as strong. However, because it isn’t as strong, the risks of complications related to the chemo are minimized.*

In the articles about RIC vs MAC (Myeloablative) the doctors discuss that kids with HLH are seemingly at a higher risk than most BMT patients for developing veno-occlusive disease (VOD) which affects the liver and pneumonitis (non-infectious), which affects the lungs, and these diseases happen from the chemo drugs given to prepare the patient for transplant. This is doctor speak for saying that the liver and lungs are already more susceptible, since HLH attacks and damages these organs when it flares. So with RIC, we minimize these risks, although these complications are still possible. The drawback with RIC is that it does not wipe out the disease as effectively.

With RIC, we are relying (and hoping) that the donor’s system will replace Ian’s diseased blood system, but because Ian’s system is not completely wiped out, we may get something called “mixed chimerism” which means that Ian has blood made up of some of Caid’s cells and some of his cells. I’ll explain more about that later. Basically, we want the Caid cell numbers in Ian to be high or at least the majority and holding. If they begin to drop, then Ian may need something called a Donor Lymphocyte Infusion. This is not unusual with RIC, and this was the main reason that CHLA told us they are not on-board with RIC. A donor lymphocyte infusion would be a blood draw they would take from Caid to get the lymphocytes from his blood, and they would give them to help boost up Ian’s “Caid/donor” numbers. When they do this, they have to pull back on immune suppressive drugs they give Ian to help prevent the dreaded graft versus host disease, another potential complication of transplant.

All this is obviously pretty complicated and scary stuff which is why we were feeling so unsure about how to proceed previously. That said, it seemed to us that a much higher survival rate with the RIC was the most compelling thing. It certainly hasn’t been exhaustively studied, but it seems like that may not be a possibility since HLH is a rare disease. It was important to us was that we get a recommendation on the treatment from a facility that offers BOTH types of preparative regimens, so we know we are getting a recommendation for what is best for Ian and not for the only thing they offer.

The doctors at CHOC said that the evidence for increased survival rate can’t be ignored and that any complications that come with RIC are ones that you deal with, since you are giving your child the best chance for survival, and minimizing toxicity at the same time. Because Ian doesn’t have active HLH or central nervous system involvement (both of which would exclude him from RIC) and has a sibling donor, he is an ideal candidate for the reduced intensity. We feel very hopeful and are glad that we have found a place that we can get reduced intensity that is close to home.