All cried out

What a long day! I figured I'd finish it off with updating my blog since so many of you are anticipating the outcome of our meetings today. Matt was post call today and didn't get much sleep last night. Call really means "30 hour inpatient shift". These are long days for Matt. Matt was a good sport to agree to come to the hospital and participate in such deep and emotional conversations when he was going into them feeling worn down. Our meetings started at 2:30 with the palliative care team here at Primary Children's hospital. The last hour of our meeting the transplant team joined us. We talked to everybody for 2 1/2 hours. Transplant told us that after a long review of the literature, the CV surgeon and the rest of the team felt that Matthew would not be a candidate for either the LVAD or ECMO as a bridge to transplant. The risks far outweigh the benefits and they feel like the outcome would not end well for Matthew based on past literature (which is limited anyway because there aren't too many children needing LVAD or ECMO when they are less then two years out from transplant needing another heart). Furthermore, transplant told us that we've reached the end of the line in terms of treatment options available to Matthew. We could consider increasing Matthew's dose of Milrinone one last time in the future, but the team felt like the effects of the drug should be seen by now. Increasing Matthew's dose would also require a full time CICU stay. Furthermore, Matthew is too sick to come home. This may change in the future, but we are not anticipating his congestive heart failure getting better rather it will continue to get worse with time. We will most likely be inpatient until transplant. For now, Matt and I have decided to stay the course hoping and praying that, if it's the Lord's will, a heart will come for Matthew. He is happy and playful for the most part. You certainly would never guess that he was in end stage heart failure. If we start seeing other systems begin to fail, we want to take him home on hospice. There have been evidences of some organ damage as a result of his heart failure with his bowel and his gallbladder. So far his liver and kidneys (failure of these organs would require Matthew to be taken off the list) are doing okay. I am nervous because his kidneys are working extra hard as we are giving Matthew 3 dieretics 5 times a day to help remove the extra fluid his heart cannot get rid of on it's own. Matthew has lost over two pounds of fluid since we were admitted 10 days ago. I just wonder how much more his poor kidneys can handle. Transplant admitted that finding a good match for Matthew will be difficult because of his antibodies and his O blood type- he has to take an O heart. Matthew was listed as a status 2 on January 31st and then bumped up to a status 1A on March 15th. Transplant has only had a few offers which ended up not working out because of additional antibodies not listed in the system. This is discouraging! The rest of the conversations were based on Matthew's death. What we wanted, different scenarios of his death, what treatments we could withdraw from and when, DNR at home or at the hospital. On and on and on..... I literally think my tear ducts dried out from the tears I shed in that room! Sorry to be Debbie Downer, but that is the reality of our day. I really appreciate the love, support, and prayers from so many of you that have literally carried us through this day. Thank you!

And, we're back

Matthew was discharged from the hospital last Friday night. By Saturday I noticed increased nausea and vomiting. The little voice in the back of my head told me I needed to call transplant, but my mind got the better of me. I knew we had been in the hospital the night before and chalked up his increase of symptoms to fatigue from his previous hospitalization. Sunday was a big day. Matt was getting back into town Sunday night from a conference he had attended the week before in Arizona. Joshua was being presented in sacrament meeting as a graduate from primary and was going to recite an article of faith and bear his testimony. I was also asked to go to primary on Josh's last day to talk about how "The faith in God" program was a blessing in our family. My great friend and visiting teacher, Kathryn Hart, agreed to come and sit with Matthew while I went to Primary but I just couldn't miss supporting Josh for sacrament meeting. I never feel good about asking somebody else to miss sacrament meeting so that I can attend- so I came up with a plan. Matthew was coming with me- I'd sneak in the very back and then leave right after the sacrament. It seemed to work, but when I got home I realized that the 20 minutes we were gone completely wore Matthew out. He perked up for Kathryn when I went to Primary to support Josh on his last day. When things settled down a bit I realized that Matthew was the same as he had been the day before. I paged the transplant coordinator who was on call on Sunday and she told me she thought we should come in first thing the next day to be seen.

On Monday morning we did bloodwork, an echo, and had a visit with Dr Tani. Based on Matthew's labs and his clinical findings Dr Tani felt Matthew's heart failure had gotten worse. Matthew's BNP went from 600 to 1500 in a matter of days. We were admitted to the CICU for an increase of Milrinone. Matthew is super fluid over loaded so they increased Lasix to three times a day and started Diuril and Aldactone to better dierese his extra fluid. Because his gut cannot even handle Neocate at a small rate through his G-tube (and he wasn't meeting his daily caloric needs anyway) they started TPN along with some additional lipids.

They decided to transfer Matthew out of the ICU tonight- so we are on the floor now. Matthew is technically on a dose of Milrinone that should keep him in the hospital to be monitored. To be honest, I feel like Matthew's care is over my head at this point. He is so sick already and his symptom changes indicating problems are so subtle that I'm afraid I'll miss something if he comes home.

Matt and I have an appointment with the CV surgeon, the transplant cardiologists, palliative care, the social worker, and GI to discuss the progression of his multi systems beginning to fail and the possibility of using an LVAD (left ventricular assist device) as a bridge to transplant. This meeting will take place next Wednesday when the transplant team gets back from a conference they've been attending this week. I'm not sure how Matt and I feel about the LVAD. The placement of this pump would require open heart surgery and a full time CICU stay until transplant which could take awhile still. I feel like things could get real ugly real quick and I'm scared!!

I'll keep you updated after we know more. As you know this whole process is a day by day process. Things change so quickly. Please pray for Matthew!!

30 days....and counting

Happy Spring everybody! I have loved seeing the flowers bloom and the grass green over the past few weeks. There's something about spring that rejuvenates my soul and gives me renewed energy.

Easter was amazing! I had a really spiritual experience on Easter morning. The kids woke up happy to start looking for their Easter baskets. Matthew had thrown up first thing when he woke up and wasn't feeling great, but he was still determined to get going. Seeing Matthew with little energy hooked up to his backpack of Milrinone and connected to his IV pole with his feeds while looking around the house for his Easter basket was a humbling sight. I got tears in my eyes as I watched him and contemplated what we were celebrating Easter morning. I realized at that moment stronger than I ever had that Matthew would be healed from his physically imperfect body one day. I felt so strongly that we would all be reunited one day together as families on the other side of the veil. Of course, I've known these things for a very long time. For some reason, on Easter morning, these truths were seared into my soul.I think this was Heavenly Father's Easter gift to me.

I have to apologize for being an inconsistent blogger. I must admit that sometimes I try to wait to blog until I have something positive to blog about. It seems like lately the good news is few and far between :( Nonetheless, here's the Matthew update:

Matthew's red and white blood count have been low for many months. Because he has spent so much time in the hospital, the medical teams were attributing the lower red blood count to the frequent inpatient blood draws and the white blood count to the chemotherapy drug and other immune suppressant medications Matthew was on. As we spent more and more time away from the hospital and got further out from taking the Bordezomib, we did not see a huge change in his red or white blood count production. While he was inpatient one of the times these past few months, Matthew had blood in his stool. The theory was that his RBC was lower possibly because of a GI bleed or several viruses he could have that can lower the production of RBC. And so the testing began. They took a ton of blood (ironic right) and scheduled him for an endoscopy and colonoscopy. The plan was for Matthew to stay for a 23 hour observation after the procedures and get an IVIG infusion while he was there. Matthew tolerated the procedures well and we now have many internal pictures of Matthew's colon and intestines to put in his scrapbook :) GI did not find an active bleed, but we gave him some because they took biopsies :-/ There were no ulcers either. What they did see is some evidences of ischemia in his gut but only in sections- not the entire gut- the pathology also confirmed these findings. I tell Matt that as soon as Matthew is admitted it's like being in a vortex. I feel like with each day we get sucked in deeper and deeper and there's no way we'll ever go home! 23 hours came and went. More tests were ordered and a CT angiogram was scheduled. The results from the angiogram told us that there was no blood clot or any other blockage leading to his GI system that would explain the ischemia. We also saw on CT that his liver and gallbladder are enlarged...probably from Matthew's heart failure. So, Matthew was admitted being in heart failure...we spent three days in the hospital and spent thousands of dollars on tests only to find out......Matthew's in heart failure. Sometimes I get frustrated! We couldn't proceed with another heart transplant though if he did have a GI bleed somewhere or if he had a clot somewhere. I guess being able to proceed with our next transplant still is the good news to report here. The bad news is that his heart failure is affecting other organs and I'm not sure how long they can hold out before permanent damage is done. Matthew has been on the list as a 1A for 30 days today. The average wait according to UNOS as a 1A is 38 days. I feel like we are racing against time and I'm not sure what will come first. I hope and pray Matthew can receive a heart quickly and we can move on from this nightmare! until then, I try to find peace in the spirit of Easter which I felt so strongly this week.

Home sweet home

Matthew was discharged from the hospital last Tuesday. Once again we are home as a family and I LOVE it!! I got so sick of packing and unpacking that I packed a permanent bag that stays in the van until it's needed next. Hopefully the next time I use that suitcase will be when Matthew gets a new heart!

Matthew came home with a PICC line and 24/7 Milrinone. He is the cutest boy you've ever seen carrying his back pack around with his medicine and his medicine pump. Nothing slows him down or ruins his mood... not even heart failure and a 4 pound bag he has to carry with him everywhere he goes.

Matthew is MUCH better than he was prior to this last hospital admission. They call Milrinone the "wonder" drug. I would have to say that I agree! He is playful and happy. He does not lay on the bed and moan all day long. I am so glad to see such a great improvement in him already. As for his peripheral neuropathy, it is better too! The neurontin seems to be helping him quite a bit. We have begun the weaning process of his Neurontin to see if he still has symptoms without the drug or if his neuropathy was a temporary side effect of the Bordezomib. Please pray that these symptoms will not reappear with the weaning of the drug.

Cardiology has not given us a time frame at all for how long they are predicting it will be before Matthew gets another heart. Matthew's cardiologist said that "only God knows when that will be." We are not only matching his O blood type, we are also matching his antibodies. Last night Matt and I could not help ourselves and we got onto the UNOS website. From there we clicked on a link that posted all the information about who is currently listed and waiting for a heart. The website is really helpful because you can break it down to region or state, blood type, status, pediatric or adult, etc. Matthew is the only status 1A in Utah but there are currently 5 status 1A's with an O blood type waiting for a heart in region 5. Of those, Matthew is the only one who is waiting for his second heart. Dr. Everitt's prediction that only God will know when Matthew gets a heart is completely right on. This transplant is a lot more complicated than the first. We are matching so many antibodies along with his blood type that we were not matching the first time around. Sometimes I wonder if a match will ever really come along. Since Matthew has been sick I wonder if Matthew can hold out long enough to get "the perfect" match. Matthew's white count has also been really low. This really scares me because he now has a PICC line... and he's 4!!! Little 4 year old boys like to get dirty! I feel like I follow him around all day with alcohol swabs cleaning his tubing and as much of his line as I can to avoid an infection.

I also continue to struggle with the concept of transplant. I know what it will take for Matthew to receive a heart. It will require heart ache and loss from another mother. This is hard. As I pray, I find myself asking not for a tragedy to occur but for hearts to be softened in the event of a tragedy so that donors can be available. It took me a long time after Matthew's first transplant to get over the guilt of feeling like I caused a sweet little boy to die in order for Matthew to receive a heart. It was a conversation I had with my mother that made me realize that I had not caused this event. What happens will happen whether Matthew is on the transplant list or not. Only God knows what will happen to all of us in this life. Only He knows when our eartly mission is complete. So I can't feel guilt. I can only pray for comfort for so many of us in so many different but difficult situations. Then I wait, and have faith. Faith in God's plan for Matthew, whatever that plan is. Faith that everything is going to be okay, no matter how things turn out.

Through it all we continue to recognize that we have been so very blessed and that so many miracles have taken place in Matthew's lifetime. We truly are humbled and grateful for the many blessings which we have received.

Heart failure and a 1A status

What a week!! Whenever I feel like things can't possibly get worse, they do! I'm exhausted so I'm going to write a quick update. Last Tuesday we were scheduled to get a chemotherapy infusion. We had done labs in the morning and were waiting in Oncology's infusion center when Dr E came in to review the labs. Although Matthew had been seen by cardiology twice the previous week, Dr E had not seen him for ten days so she wanted to check in on him real quick. She took one look at him and said "He's not getting his infusion today!" She ordered an echo and an ECG and started his paperwork for a direct admission to the CICU. Matthew's echo looked fine, but once we got to the ICU his heart looked huge on chest X-ray and his lungs showed pulmonary edema. They started Matthew on a milrinone drip and watched him in the CICU for three days. Now we are on the floor. Matthew got a PICC line placed and we are hoping to get out of prison soon. Matthew's hemoglobin and hematocrit have been low and his BNP is really high. Matthew's limbs have been "hot" and he feels like "needles" are stuck in his fingers. As it turns out, he's having a lot of non-specific and very specific pain related to peripheral neuropathy which he developed as a side effect from the Bordezomib. It was a known risk, but we really had no other choice in order for us to get on top of Matthew's rejection. I hope and pray that this is something that will go away!!! He is now on neurontin (a seizure medication- why not? That makes the grand daily med total for Matthew 13!!!) We still have some issues to work out before being discharged, but our goal is to get Matthew home on 24/7 milrinone. The bad news....Matthew is in heart failure. The good news....Matthew's heart transplant status has been changed to a 1A from a 2.
My head is still reeling. I think I may be in a little shock. Things in the "heart" world always happen much differently than expected.
Thank you all for your love and support. We could NEVER do this without you!! Our ward and neighbors have been awesome! My parents and Matt's parents have been helping non stop. We have been blessed and we are grateful!!

Home again

We've been home from the hospital now for a week. Matthew is a superstar! The only glitch in the hospital was that after his dialysis catheter was placed and they started the first plasmpheresis, we realized that the catheter was not working as it should. We had to go back to interventional radiology the next morning to replace the catheter. Because interventional radiology uses nurses not anesthesologists to administer anesthesia, they were not able to use Matthew's "anesthesia cocktail" which has been working for him with minimal side effects. The radiologist assured me that Matthew would be fine waking up from the anesthesia they were using as long as he was not woken prematurely. This was far from the case. Just like in times past, Matthew woke up thrashing with his post anesthesia psychosis and was like that for the remainder of the day. I absolutely was not going to allow them to use the same anesthesia they had used the day previous to re-do the catheter the next morning. Everybody in the CICU was completely on board with me after seeing him that first day. So, the CICU organized their own team to come down with us to radiology so that they could give him his propaphol and phentanyl- and guess what?? He woke up great with no side effects!! Matthew spent three days in the ICU and then moved to the floor for the remaining 9 days. He tolerated his treatments well. In typical Matthew fashion he won the hearts of all the hospital staff who dealt with him.

On our last day in the hospital, Matthew got an IV/IG infusion and then got his first dose of Bordezomib. Plasmapheresis is an acute way to remove the antibodies from Matthew's blood. Bordezomib is used to stop the production of memory cells that are made which are the culprit for antibody mediated rejection. Matthew did two doses of the four doses of Bordezomib before plasmapheresis, but when his heart function was declining Matthew's doctors decided that we needed to get a better handle on his rejection episode. This was why we proceeded with plasmapheresis. Unfortunately for Matthew, we had to start from the beginning with the Bordezomib infusions. Bordezomib is a chemotherapy drug used to treat multiple myeloma. They are just starting to use it in cases like Matthew's. Primary Children's hospital does not even have the drug in stock and they have to order it from the Huntsman Cancer Institute every time Matthew uses it. In fact, the nurses in the Hematology/ Oncology clinic have never heard of it. Bordezomib makes Matthew really sick!! Last Sunday, he was throwing up everything we tried to feed him through his G-tube no matter how slow the rate. He could not tolerate anything. He was admitted to the Rapid treatment unit for the day to get labs drawn and to get a saline infusion. They started Zofran which seems to have helped him quite a bit. We have been giving him that consistantly since then. We have gradually been increasing his rate and he seems to be tolerating his Nutren jr. a little better now. He's still behind on his fluids, but we are doing the best we can to give him the nutrition and calories that he needs. He will not eat anything by mouth and pretty much lays on the couch or a bed all day watching tv or playing games. He does not have energy to do anything more than this each day. It breaks my heart to see Matthew this way. He reminds me of what I would have imagined a little boy on chemotherapy to look and act like (minus the bald head of course). We are half way done with infusions!! I can't wait to be done. You have no idea how hard it is for me to drive him to his infusions knowing what the drug will do to him. I always drive there tearfully wondering if what I'm doing is really the best thing for him. I pray that these next two doses can go well and that we can be done with Bordezomib forever!

When I was in the hospital I met with "Rainbow Kids" which is Primary Children's palliative care team. There were 8 members on the team and one of me (Matt had to work). The Rainbow kids team was so wonderful! It was a hard meeting, nonetheless. We talked about what life sustaining treatment we wanted for Matthew and what would we want if Matthew were to code at home and the paramedics showed up, etc. This was not an easy discussion. I know Matthew is sick. I know that these are discussions Matt and I have had in the past. Talking about what decisions YOU as a mother would make if you had the choice in deciding if Matthew would live or if you would let him die is incredibly emotional and difficult. I know we are doing all we can for Matthew, but part of me is still holding onto the hope that he can live a somewhat healthy and normal life after his next transplant. Maybe my hopes for him are unrealistic, but I'm his mother and I just can't imagine the "other" scenario yet. I certainly hope and pray that it is a decision that I will never have to make for him. Seeing him as sick as he's been this last week also makes me realize that I don't want him to suffer like this anymore. He has fought so hard for four years and he's done it with a happy, sweet temperment. He's been through a lot these past six months especially and I see how it is getting old for him. I love this little boy with all my heart. I only want what is best for him. Usually he is so happy and full of life that it is impossible for me to give up on him. I pray that I can have the strength and inspiration I will need to make difficult decisions in the future.

So what's next? Matthew finishes his infusions, he will continue to be followed by cardiology/ hematology twice a week and then he will have a heart cath on March 26th. He's still on the heart transplant list. As far as I know, he could still get a heart whenever a match comes available. I know that this will take a long time and it's the last thing on my mind in regards to Matthew right now. I feel like I'm focused on the current hurdle and then we'll go from there.

I try to enjoy each day, each experience I have with Matthew. He is a special boy. Joshua had the opportunity to bear his testimony at our stake conference last week. He said that whether Matthew is on earth or in heaven he will go where he is needed to build Heavenly Father's kingdom. I know that Joshua is right, I just wish I knew when and where he was needed. Maybe then I could deal with things easier.... maybe!!

Napoleon Part-deux

It has been a while since I have had the opportunity to comment on the blog. Mostly because my poor wife has had the task of taking care of Matthew's hospitalizations and doctor appointments. It was through the grace of one of my partners that I am even here today as we start another round of plasmapheresis in attempts to slow down the rejection that is sucking the life out of Matthew's heart. It is good for me to be here. There are two sides to medicine: the receiving end and the giving end. I spend most of my time on the latter or giving end. I prefer it that way. Its easier. I'm in the know and in control. No one stops me when I come into the icu, I don't get in trouble for using my cell phone, and don't have to bend over and pretend that I am happy when inside I am a wreck.

Back in October when they first diagnosed matthew's coronary artery disease one of the physicians pulled chrissy and I aside and told us that he would likely need a new heart within 18 months. We are starting to really see the reality of that statement. Since coming home from our make a wish trip it has been a steady down hill progression. We even see it at home with more cramps, vague aches and pains, fatigue, and failure to thrive. Don't get me wrong he is still happy and tries his best to be a four year old...his body just can't keep up with his mind. We don't need any echos to see it.

The bortezomib isn't working fast enough so in order to stave off yet another attack of rejection they have invited us back to the hospital for another ten days of sleepless nights, foreign beeps, and stress. We are honestly happy to do it if it means Matthew stays healthy for a bit longer but it isn't easy. Especially for my wife. I would like to think that it is easier for her because she doesn't spend every day in the icu like I do but I know that it isn't. When your son is the object of scrutiny it drains you of spirit, strength, and stamina.

We started at nine this morning with the placement of another dialysis line. He handled the line okay it's just the waking up part that continues to be a problem. He was so dystonic and agitated that in order for them to do the plasmapheresis they just ended up giving him more sedation.... He is still sleeping as I write. Let's pray that he wakes up more sanely this time and not like a cocaine junky looking for his next hit. Furthermore, his line isn't working very well which means more of the same for tomorrow as they exchange it for a larger one. I'm going to need some Ativan if this continues.

Matthew is a trooper though. He has so many friends here at the hospital it makes it almost bearable. We again can't thank our families, parents, friends, and doctors enough. A special thanks to my parents today who on their birthday are celebrating by bringing us lunch and watching Joshua and Luke.

I wish that I had the answers to all of this. I have prayed that our Heavenly father's will would be done but mostly I pray that Matthew won't have to suffer. I have seen too much of that already. Hope is not lost though and we continue to fight. I wish that I was independently wealthy and that i could spend all of my time making sure that he was well taken care of and happy. I know that he is surrounded by guardian angels and his time is not now. For that I am thankful.