The Gift of Life

Today was preparation day for the surgery. Not much was required except one final check-up for Aaron, who started the morning with a little cough. The doctors at SCCA weren't concerned by what they saw so they gave him the green light to donate his marrow tomorrow.

This means that at around 6:30am tomorrow with hundreds of people fasting and praying for both our little boys, Aaron will be the first one to enter the surgery center where we get to hold his hand until the anesthesia  kicks in and he falls into an induced sleep.  I hope he dreams of family and all those who love and pray for him.  Even with little risk, it is scary to think of our 4-year-old going into any surgery and we hope to have no surprises.  

After the extraction, the marrow and blood from Aaron will be processed at some level and several hours later Jordan will receive this infusion.  It should all be over by around 1 or 2pm.  And then the waiting begins.  Then we get to watch how Jordan's body responds.

Today Jordan started out with a little deeper breathing and a slightly larger stomach due to a growing liver.  The RT team increased the pressure of the air going into his lungs just a little.  This won't impact the infusion tomorrow but it does mean the virus is still moving forward.  Everyone is doing their best to keep smiling when they talk to us and to remind us to take this a day at a time; I continue to feel that we will be okay.  

For Aaron's part, he only understands that he has the "toughest" cells in his body and that he gets to give some of these to Jordan. He asked tonight if he would have any T cells left after his surgery to fight the "bad guys" in his body and we assured him he would.  He smiled and went to sleep.  

Some day in the future, Aaron will recognize that no matter what the Lord has in store for little Jordan, Aaron gave Jordan the best chance at life possible.  What a gift for both our boys.  

A House of Faith

A week ago today I brought my children up to Seattle. For the first week she was here, Heidi slept in Jordan's hospital room.  The following week, we were approved--based on the severity of Jordan's illness and the likelihood of an extended time in the hospital--to stay in a really incredible place called the Ronald McDonald House.  This is a residence that has rooms like a hotel, shared areas where children can get distracted from their worries and illnesses, and a feeling like a temple.  

On the main floor they have several play areas for younger kids, a movie area, a computer room for kids 11 and older, a video game area and an outdoor play structure that is fenced in; one level down is a big kitchen where each family has a small personal fridge, a shared freezer, a personal cupboard and shared plates, cups, bowls, dishwashers, stoves and ovens, and everything you need to cook and clean; down another level is a bigger play area with ping pong, Foosball,  air hockey, old-fashioned arcade games, a teenager room with more video games and televisions for movies, and a padded area for small kids with little houses and slides and "enchanted" trees.  The top two floors are residences where we have room for 6 and just across the hall is a study where the kids can sit and read or play a game together.

Having a place for kids to play and parents to stay that is just blocks from the hospital is wonderful.  What is REALLY incredible though is that it is paid for not by hospital money but by volunteer time, by parent involvement, and by donations from individuals and corporations.  As a result, families that don't know how long they are here (such as us), or families that can't afford to be anywhere else can stay here at little or no cost.

In addition, volunteer groups prepare and share meals 5 nights a week and for those days and nights where meals aren't prepared by volunteers, there is a large pantry full of donated food that families can use with staples such as flour, rice, cereal, tunafish, etc.  Several nights ago we had a biker "gang" in all leathers bring dinner for everyone.  Tonight dinner was brought in by a company and along with dinner, the acclaimed children's band Recess Monkey played for the kids, while other volunteers set up sugar cookies and frosting for the kids to eat on all the tables.

Sometimes it can feel like Willy Wonka's Chocolate Factory (there is even a fridge stocked with chocolate milk), except that for all the fun the kids are having and all the joy seen on their faces, every family is here because one of their children has a life-threatening illness.  That makes this place and the volunteers and staff that serve in it very special.

When I see a family from east Seattle with a 10 year old daughter that is fighting cancer, or see a 5 year old pushing her 6 year old sister down the hall in a wheelchair, or a young girl not more than 7 with tubes coming out of her nose as she smiles at everyone who passes, or see a 13 year old girl who is also a cancer patient wheeling herself down the hall, I recognize that this is not just a place to rest, it is a house of faith; and it is a sacred place.  

Every day and night parents pray for the lives of the children they have here and weep for the same helplessness we feel for Jordan.  And we share in these prayers and hopes, not just for our children but for those children and families we see around us.  And I know with absolute certainly that the prayers of these parents and the challenges of all the children and families here don't go unnoticed and that this house of faith is also a house where angels dwell.

Tonight I participated with my brother-in-law in giving a priesthood blessing to my 4 year old son and to Jordan as they prepare for surgery tomorrow.  And in this house of prayer and in the children's hospital which is also a sacred place, I felt the presence of those angels.  And I am again grateful I can feel the joy these children feel that comes from the love of others and the watchful care of a faithful Father in Heaven.

When my family is through this, one of our commitments is to serve others in the Ronald McDonald house near St. Vincent's and to try to find other houses of faith like this one, where we can pass on a little of the gift we have been given.

Donor Chosen and Risks Outlined

Meeting day Tuesday wasn’t great.  We heard a lot of what we had heard before, only this time all at once as part of signing consent forms. We consented to let the doctors do whatever they need to for the transplant as well as any emergency that may arise.  We consented to allow several research hospitals to use Jordan’s information and extra specimens for research.  And we consented to all the risks that are inherent in anesthesia, bone marrow transplant, and any surgery including everything from a slight rash to sudden death. 

While sudden death is very unlikely, the doctors informed us that Jordan surviving the next few months is also unlikely.  Since the viruses have spread throughout this liver, lungs, stomach, and bowels, what is likely from a clinical standpoint is that either Jordan’s lungs will inflame so much that they will no longer be able to oxygenate his blood, or his liver will stop functioning.  The lead physician in the meeting made it clear that even with the transplant, it will take a miracle for Jordan to survive long enough for the new T-cells to take effect…; good thing I believe in miracles. And I believe in promises. 

I believe in the Lord’s promise of peace in this world and eternal life (family life) in the world to come.  With literally hundreds of people praying for our family and many fasting as well, we are already feeling a level of peace that is itself a miracle. 

In addition to these meetings, we were informed that Aaron has been selected as the donor.  Michael was originally supposed to be retested yesterday to see if his white blood cell count had increased, but since the doctors didn’t know why it decreased in the first place, they decided that using Michael introduced too much risk of new infection for Jordan.  In our minds we hoped it was Michael but in our hearts we felt it would be Aaron so again this feels right. 

For Aaron’s part, he sailed through another blood draw without a complaint and was elated to get the chance to pee in a cup as Michael had done.  It is never difficult to convince a boy to pee into something new—especially if it is likely too small to actually hit. 

Fortunately, the more we learn about the actual harvesting of the cells from Aaron and infusion into Jordan the less concerned we get about this part of the next several months.  Apparently the harvesting process consists of a general anesthetic for Aaron (that is the scariest part and carries low risk) followed by 2-4 pricks through his skin to reach the bone near the hip on his back side.  The doctors then hit the bone marrow about 20-50 times on each side to get the “juicy” cells they need.  The recovery for Aaron is expected to be 2-3 days of some minor soreness and the procedure itself is only about an hour. 

For Jordan, the infusion process is only about an hour and similar to getting blood so it has a very low risk. The risk for Jordan comes really after the cells are infused and over the next few weeks as follows:

If Jordan gets the T-cells he needs from Aaron that know how to fight the Adenovirus and they choose to aggressively attack the virus, this may be too much for Jordan’s little body to handle and he will likely get much sicker, which could be life threatening.  We would expect to see this in the first 1-2 weeks after the infusion. 

If Jordan doesn’t get the T-cells that know how to fight this virus, Jordan will need to survive another 2-3 months while the T-cells from Aaron mature and train to fight this virus.  The doctors feel it is unlikely (except for that miracle) that Jordan can survive this long without a functional immune system given the fact that his copy numbers (viral particles) with the Adenovirus are already more than 9 million.

If Jordan does get the T-cells he needs and they only mildly attack the virus as we hope and Jordan’s body holds on, he has a 10-15% chance of developing Graft Versus Host Disease (this would be a 30-50% chance if he didn’t have a matched sibling to be the donor so we are very blessed there), which could lead to anything from a rash to death.  The only way to fight Graft Versus Host is to suppress the immune system response through steroids which in Jordan’s case would decrease his new immune system’s ability to fight the viruses. 

If he gets the T-cells he needs and doesn’t develop immediate GVHD, then there is a 50% chance of long term GVHD, which can require 3-4 years of hospitalized therapy (intermittent, not ongoing) and can be fatal. 

SOOO, there is a lot happening that introduces risk, but the doctors are doing all they can to mitigate these risks, and so are we.  For the doctor’s part, to alleviate the likelihood of GVHD since it is especially difficult to fight in Jordan, the doctors will partially suppress his immune system through medication—MMF and Cyclosporine.  These are experimental drugs for this purpose but the other option of killing all T-cells through chemotherapy is too dangerous for Jordan. 

Our part is to continue building a community of faith to strengthen and support our own.   

Thinking time

Post from late day Monday, May 27, 2013

After all that happened today we did not have the barrage of doctors and visitors we have had most of the past few weeks, which is a nice change.  The doctors are very patient and spend as much time as we need answering questions and trying to be clear about where we are headed, but sometimes just a little silence to be with our own thoughts is also nice.  It allows us to gear up for tomorrow when we will be in transplant meetings nearly all day to hear about the risks, sign paperwork, and test Michael and Aaron one more time to see if perhaps Michael's white blood cell count is back up to a safe level.

Thymus

Like most of the things that keep me alive every day, I wasn't even aware a Thymus existed--let alone that one existed inside me.  Apparently, the Thymus educates T-cells on how to work, like a military camp for training soldiers.  Without a Thymus, the only way to defend against disease is to transplant T-cells into a body every time the T-cell count gets too low.

In the past few weeks, one of the fears for Jordan was that along with his SCID diagnosis, he might also have a genetic disorder called Charge syndrome.  This impacts organs and functions in the center of the body such as breathing and swallowing, the kidneys, and in some cases the Thymus.  The concern was that Jordan didn't have a Thymus which means he would have been transferred to either New York or Duke University to get T-cells implanted or to have an experimental surgery for a Thymus implant. In the last 15 years only 60 Thymus implants have been done and certainly not all have been successful.

Beyond the added challenge not having a Thymus would have created for Jordan, it also would have meant we as a family would have been traveling again--this time much further away.  So we fasted and prayed and after getting an MRI, the doctors informed us he does in fact have a Thymus, which is a huge sigh of relief.  We still aren't sure how well it is working, but we know it is there and so we are more hopeful that the bone marrow transplant can be successful.

Miracles, Creation, Love

I have to add to the last post one other miracle from today.  While Heidi took Aaron to get his blood workup done, I took the other three kids to church in Northwest Seattle.  We only made the last hour, which happened to be sacrament meeting (like mass except there is no paid ministry so the speakers are from the congregation) and the speakers were all talking about gratitude and the joy we find in the midst of challenges when we look for ways to be happy.  It was just what I needed and a great blessing to feel the Lord was comforting me as I tried to comfort my wife and children.

When we arrived at church, I asked the three kids with me to have a question in their heart for the Lord and then to listen as the Holy Ghost came during the meeting, for answers since that is the process of revelation.  Michael afterward said his question was whether Jordan would survive and the answer he felt was that Jordan would but that if he didn't it would be okay.  That was quite a teaching and learning moment.

I had a similar question and also found myself considering how while I took part with the Lord in Creating my children, yet unlike anything I have ever created or put together, when my children "break" in some way or something goes wrong with them, I have NO idea how to correct or "fix" it.  I trusted in the Lord and His miracle to bring each of my children into my life and now I have to trust Him again that while I don't know how to heal them, He does.  And that goes for my wife as well, that He knows how to help and heal her in a way I can't; this is important because when I see a mother standing over her child and unable to pick him up for all the wires and tubes running through his failing body, and when I see that little child unable to sit up or speak or even hear his mothers voice staring into her eyes and barely blinking, I recognize that for all the love I have for her, I can't begin to understand what she is going through.  I can't begin to imagine the depth of love and loss she feels every moment Jordan is not healthy and in her arms.  But I know the Savior can; and I know that He can and will heal the brokenhearted--and that is the hope I need.  

A Fighting Spirit

Jordan is a fighter—that much is for sure.  Without a functional immune system and with two nasty viruses in his system, he has managed to grow stronger and fatter.  With one of these, the Adenovirus, spreading in his lungs, he manages to keep breathing on his own.  And with more than half his life in a hospital bed, he manages to keep interacting, smiling, and melting the hearts of the nurses, doctors, and those who love him.    

Today we had to become fighters too.  We were informed this morning that the Adenovirus has spread not only to Jordan's lungs, but to his stomach and his liver.  His liver is expanding and some of the functions the liver provides such as assisting with blood clotting, are starting to weaken.  With the virus in his stomach, Jordan has been throwing up with so much force that blood vessels are exploding in his eyes, leaving him with red eyes and dark rings around the eyes.  To offset this, doctors stopped feeding him through his g-tube and are instead feeding him through an IV for at least the next several days.  Combined with more severe fevers, the doctors expressed worry on their faces that we have not seen before and began discussing the "what if's".  

I remember the "what if" game from grade school.  "What if I had a million dollars?", or "what if I could fly?".  We never practiced the "what if my son was in the hospital fighting a virus and with all the modern medicine and knowledge available there was no way to save him?"  We have to begin this discussion with our children, while at the same time balancing our own hope so as to keep all the positive energy we can.  We certainly have hope in the Lord that the outcome is His will, and we have hope in the future that there is joy there regardless what happens now, but that doesn’t diminish the helplessness we feel.  

For now the doctors are moving ahead with the transplant as quickly as possible without putting Jordan and the donor at any more risk. We have already seen many miracles in this process, including finding out that all 3 of our other sons are a match—not only for the bone marrow, but also blood type.  The statistician in the lab stopped trying to figure out the likelihood of that happening, but needless to say it is extremely unlikely.   For whatever reason having all 3 match was necessary and fit into the plan the Lord has for Jordan and so those prayers were answered and we are very grateful for it.  That means that instead of taking only the match, the doctors are able to test all 3 brothers for the healthiest immune system. 

The other factor in choosing the donor brother is the one that is most likely to have had the Adenovirus since that means the donor brother could provide T-cells that already know how to fight this virus. Jordan needs as much of a head start for these T-cells as possible to give him the best chance to hang on. 

As it turns out, Mikey and Aaron are most likely to have had the Adenovirus and so we figured Michael would be choice; but his white blood count is down due to an unrelated infection he is fighting and that moves Aaron to the front of the line. 

Miracle two is that Aaron agreed to be the donor and go through the blood tests required for donor screening.  As of yesterday, he refused to do another blood test after already giving blood twice in the last week and he threw a major fit the second time.  We talked to him and asked him to help and offered him treats and he said “no, I not doing it” after every attempted bribe.  With all the kids have gone through I put my foot down and told the doctors that we would not force Aaron to go through this because I wasn’t going to hold him down and traumatize another child.  They told us we had 24 hours to decide and that they would use Tyler if needed.  So we prayed that the Lord would teach Aaron and soften his heart so Jordan would have the best chance possible.

This morning I asked Aaron again if he would help and he said, “no”.  Then I got down on a knee and told him, “Jordan needs you.” Aaron looked me in the eye with a somber face and said, “okay Dad, I will do it.” Then he brightened up and added, “I am so excited.”  And he was. He gave blood without complaining and stayed happy about the chance to help his brother.  This truly was a miracle.  (To be fair he was also happy about not going to church which might have helped, since he gets shy about going to a new congregation.)

Now we need just a few more miracles. One to get Jordan through the transplant, one to have his new T-cells fight the viruses without taking too much of a toll on his body, and one to keep his body from rejecting the transplant and causing Graft Versus Host Disease, which can of itself be deadly.  Then perhaps we can consider moving back home to a new “normal”, whatever that will be.  

SCID and Seattle

Post from May 11, 2013

The doctors finally determined what Jordan's issue is, and it isn't great news.  He has Severe Combined Immunodeficiency (SCID), which is what the "boy in the bubble" had in the early 1980's.  Basically, it means the immune system is not functioning properly and Jordan's T and B cells are extremely low.  In fact, he may not have any T cells at all, which would mean he can't fight disease. This is apparently what has been happening--he contracted two nasty viruses and they are taking a toll on his body from day to day. Some days he seems to be doing much better and then the next day he seems to crash because of the viruses.

While I have read that only 1 in 1 million children have this disease, it is visible enough that a lot of genetic research has been done on it, which keeps us hopeful.  The possibility of a future for Jordan, however, is not an easy road. At the very least, the doctors believe he will need a bone marrow transplant, which is why they are sending us up to Seattle Children's Hospital where there is an immunology group.  We have known this for about a week and have been waiting for a space in the ICU at Seattle Children's for several days.  Today we got notified of a space about 10am and we are headed up in just a few hours.

As for the rest of the family, the kids are still in school so I will settle in with Jordan in Seattle and just take it a day at a time. Ryan should be coming up later in the week and bringing the kids the following weekend.  My mother and father are in town as well to help out and everyone in the ward and in our community continues to bless our lives with their kindness and care.

I will add some pictures to this post a little later.

23 Days and Counting Down . . .

This is a post from 4/29 that was in "draft" status so I am going to post it now:

Still looking toward coming home but no sign of when that is.  We learn more every day and the doctors are feeling like he is improving but it seems like every time they okay him coming home, he goes downhill the next day.  We have literally been ready to check out 3 days and at the last minute the doctors tell us they aren't comfortable sending him home.  We don't want to take him until he is ready but this is beginning to get difficult for everyone and we have decided to stop telling the kids he is coming home until he actually arrives.