A Miracle Boy's High Risk Transplant

Today we met with the Transplant Team to discuss Evan's upcoming kidney transplant. Our Transplant Surgeon, was ironically in the OR transplanting a kidney when we first arrived. We spoke with the Coordinators, the Social Worker, The Nephrology Fellow and the Attending Neprhologist; the usual suspects. We knew coming in of certain obstacles and spoke superficially of the risks, the plans and the timeline. This was the conversation we've grown accustomed to and we were ready to leave, positively anxious to finally go through with our plan.

Cue the Surgeon, a little winded with sweat on his brow; clearly he had hustled over to see us. He walked into the room with a bright smile, told Evan he was the cutest little boy he's ever seen and he took the time to exchange pleasantries with us; a rarity in Surgeon to Patient communication. We could see he felt for us as soon as he started to speak of Evan's situation.

Unabridged, somewhat toned down, medical version [in brackets]:

[We learned that Evan's heart is actually backwards. In a "normally" positioned heart the aorta comes up and horseshoes over the heart falling to the left side of the body. In Evan's case it falls over to the right side of the body. Normally the aorta will come all the way down the middle of the trunk and split at the base into two vessels, one for each leg. Evan's, because of the "backwards" heart, splits higher up. Normally, a kidney would be hooked up lower in the body near the bladder. Evan's transplanted kidney would have to be hooked up higher because of the location of the split in the vessel. Luckily, or not depending on one's perspective, Evan doesn't have a bladder so the kidney being higher won't cause problems in regards to the kidney to bladder hookup.

Not having a bladder brings all whole new set of complications though. Where will his urine drain? For now they'll just move the ureter to the skin and the urine will drain into a bag or a diaper. Two complications stem from this: (1) bacteria loves a moist, warm environment and infection is a very real threat. (2) the body has a tendency to close holes as part of the normal healing process. Normally they like to use a piece of the colon to exit the skin so it's harder for the body to close over the larger opening. We don't have that luxury and have to hope Evan's body doesn't clot off the tiny opening the ureter provides.

Let's go back to the kidney needing to sit higher up. In most cases the kidney can be tucked underneath the liver. In Evan's case the liver must be moved out of the way; either pushed up, rolled over or flat out held in a Surgeon's hands. Surely not an ideal situation; no one wants to touch the liver if they don't have to and major consequences can come of this. A Liver Transplant Surgeon will be consulted beforehand and will be present in the OR for Evan's surgery.

Evan's vessels are tiny, he's a small guy, and his abdomen will just barely fit mom's (my) kidney. The Surgeon spoke of the need for having spare cadaver vessels at the ready in case he needs to make Evan's vessels longer, like an extension cord. If none are available they may need to explore artificial vessel construction, but Evan has a history of clotting anything artificial so that would be a worst case scenario. The addition of a cadaver vessel adds more complications; a chance of infection and finding one compatible that won't interfere with the ABO incompatible transplant. Still following?

I spoke of the ABO incompatible transplant in an earlier post; another added level of complication. Since our blood types don't match, putting in a random donor's blood vessel has added complications of rejection, or kidney rejection, in addtion to normal clotting risks In the same vein, some pun intended, if Evan needs multiple blood transfusions, which he likely will, they'll need to be careful to find the least reactive blood to prevent rejection. If he needs plasma the same applies. The plasma they'll have ready for him will be AB. The blood A.

If you're still with me I think I've covered all complications, well most. Evan's a blood type A; I am blood type AB. Evan's unique anatomy presents rare circumstances never seen by this team before. Evan's liver will have to be mobilized (moved). Evan's history proves that he is a bleeder and a clotter so they must be ready for either response.]

We were told that Evan's upcoming transplant is extremely high risk. This Surgeon, who is one of the best in the field, has never had a case as perplex at this. The Surgeon said it's flat out scary. He's apprehensive, but believes there's no other alternative. "He's two infections away from not having a way to dialyse (sustain) him," were his exact words. He also believes that this is our only shot as far as transplanting a kidney goes. All or nothing, do or die, all our eggs in one basket; these aren't cliches one wants to hear when it comes to their child's life.

Due to the extreme nature of Evan's surgery we have the best of the best in place. We've been told that when Evan goes in for his procedure the entire hospital will be on alert: the OR, Urology, the Liver Transplant Team, Hematology, Blood Group Incompatible team, the blood bank, the PICU... All needed resources will be available to him. They've covered all bases and aligned their best tools and minds. We really couldn't ask for more. If we only have one shot, this will certainly be the epitome of the good old college try.

We're certainly faced with the brutal realization that we need another miracle for our boy to survive. It's devastating, but not something we haven't dealt with before. Deep down there's a calm; I know everything will be fine. I felt the same when I was pregnant and they told me not even a miracle could save my child: here we are two and a half years later. We need a miracle. We need our prayer warriors again. We need you to tell anyone you know who can pray for our boy to do so. We need love and light around the world pulling for him. We can do this. He can do this.

We have to meet with the Liver Transplant Surgeon for a consult. Afterward, we'll meet one last time with the team and cross match our blood. Then the surgery will be scheduled. It's looking like this will all happen in January. Until then we'll pray diligently; work with Eastern Medicine via a Qi Gong Master; love and snuggle the heck out of our boy; and enjoy our time together in this calm before the storm. We will be positive. We will be productive. It's the best we can do. We owe it to our boy to give him the best of us when we're asking for so much more of him.

It's Go Time! ...Almost.

Evan's just over two year old. His entire life we've been fighting to get him to 10 kilos, 22 pounds, so that he may receive a kidney transplant. It's been long. There have been many ups, many downs and many times we just didn't think he was going to make it. These days you'd never know that he's spent a year and a half of his short life in hospitals. He's intelligent, social, always happy, and continuously thriving. My little man has a smile that could light up a building.

Currently Evan is 9.8 kilos. He was put on the list when he reached 10 kilos, but then had some GI issues and lost a hard earned kilo in a month. Well GI is resolved and he's back in action! Up next, surgery #15, an incontinent bladder construction. His Urologist will take a piece of his colon, create a tube, and connect it to an ostomy bag where his urine will drain. 2-3 months later he will receive his kidney transplant.

As of now, I, Evan's proud mama, will be the kidney donor. My blood type is AB+, Evan, A-. Years ago this would be enough to disqualify me, but this is 2011; these days transplant centers can do what they call an ABO Incompatible Transplant. By using a technique called plasmapheresis they can physically remove Evan's B antibodies thus eliminating the blood type rejection. UCLA has done 30 ABO incompatible transplants, but they have never done this to a person with A blood typing. Evan would be their guinea pig. As a mom this isn't the most comforting news to receive. Why would they consider to venture into the unknown with a two year old boy? My boy?

While I am not an ABO match I am a perfect tissue type and I match 4 of the six antigens in the crossmatch testing. In short, Evan's body would be more apt to accept my kidney, not needing nearly as many meds to help with regection. It's such a sound match that my transplant team believes the kidney could last for up to 30 years in Evan. Most successful tranplants last for 7-10 years and that's considered a good run. 30 years? Well that certainly is a beautiful rarity.

If you know anything of Evan's past, regardless of what he looks like on the outside, he is certainly fragile. While the above looks all good and well it is surely not ideal to put a high risk case through something UCLA has never done, unless the reward far outweighs the risk. His team wants to consider the National Kidney Registry to see if there is a better kidney out there for Evan before making any decision. In this program we can participate in a paired kidney exchange. Simply put, someone may want to donate to their loved one, but aren't a match so both enter this program to find others like them and a more suitable kidney for all. The likelihood of Evan finding a more compatible kidney than mine is not very probable, but it may be out there and they'll be sure to know the best option for Evan before we proceed.

To say we feel blessed yet again is a complete understatement. With all that Evan has been through nothing has been routine or easy, but everything had been divinely inspired. The coincidences we've come across just don't exist if not for someone, something else looking out for our little guy. Deep down I rest comfortably in the awareness that all will be well and I am grateful. That's not to say that normal feelings of fear and anxiety don't creep up. Everything we've done his entire life has been for this. All our eggs are in this basket. It's normal to understand the entire picture and I am well aware of both ends of this situation. Every day as we get closer I feel the pressure, and understand how scary what we're going through is. I also know the track record. We've been to hell and back and he's prevailed. Evan's a brilliant warrior. I find peace in knowing there is nothing I can do to avoid what we have to go through.

Daily, sometimes more, I acknowledge the "normal" feelings; am thankful for them; and I let them go. They serve me not. The only control I have is of my outlook. I can be the best advocate and the biggest cheerleader for my son. I can pretend, when we go back into battle, this is how everyone's life is. He doesn't know any different and I will not be the one to open his eyes to it. I will tell him how strong he is and how proud of him I am. I will thank God for Evan and for the opportunity to "go under" that day with him, in solidarity; to give him a real piece of me that he can carry with him. It's the ultimate gift for me, to give to the boy who already has all of my heart and every bit of my soul. I prayed for this day. I thank God for this moment. I thank everyone that has supported us along the way. God bless you all. God bless my beautiful boy.

Spica be Gone

Tomorrow morning, after 15 weeks, Evan will be without a Spica, full body, cast. He'll be fitted and placed in a brace for a month and after that he's free to learn to sit, crawl and walk. It's a good feeling to be on the opposite end of this journey; to know that he's almost free to develop like any other little one. In the final moments of the Spica I can't help, but look back on this experience with complete gratitude and a full heart.

Not long ago a boy born with a malformed hip would be forced to walk with a gentle limp; doomed to have severe arthritis; and eventually need a hip replacement by 30. His quality of life would be altered. Sports would definitely be out of the question. He would always be aware of his hip. Fortunately today that same boy can undergo a surgery, or two; two casts over a period of 15 weeks; a brace for a month and rigorous Physical Therapy. He can battle through all that before his 2nd birthday and be free from pain, limp, arthritis and any limitations. How lucky we must be!

A toddler in a full body cast, particularly during the spring and summer months, is no easy feat. There are many issues to address: his emotional well- being; his comfort; keeping him stimulated; providing him a sense of freedom; transporting him; and positioning to allow him to sit, sleep and play.

Emotionally I couldn't prepare Evan for this journey. He was standing and crawling one day and in a ton of pain with a cast from nipples to ankles the next. His reaction was intense, but it only lasted two days. He just accepted the situation and smiled through the entire process. To say we're blessed is an understatement. Keeping a growing boy stimulated and happy meant sacrificing some time. So the house was a little messier, or dinner wasn't on the table when his Daddy arrived home from work; those sacrifices were well worth the reward. The extra time, extra smiles, and all the kisses and loves I shared with Evan were some of the greatest moments of my life.

Making him comfortable throughout the day and while he slept consisted of many pillows, blankets, belts and even exercise bands. Creativity was key in this situation! Transportation was settled by a purchase of a new car seat that could accommodate the cast. Luckily insurance, after some prodding, paid for our seat. This seat, a Britax Hippo, was an incredible asset and we highly suggest this to others in our position. Another valuable asset, that is if you want to grocery shop; walk your dog; or go anywhere at length, is an infant carrier. Ours is a gently used Baby Bjorn that we picked up at a Children's Orchard. I used this carrier multiple times a week and really feel it gave Evan and I a sense of normalcy. We enjoyed many wonderful moments with our Baby Bjorn; it truly fueled our freedom.

Our biggest challenge with the Spica was cleanliness and keeping Evan comfortably cool. Anyone with a little one will understand that poo is often explosive and vomit can sometimes run a plenty. These things happen. Unfortunately they soil the padding and create an unsafe, uncomfortable and smelly situation. On average I was pulling out all the padding I could and replacing it 2-3 times a week. This process takes about an hour and is absolutely not fun for the little guy in the cast. Afterward though I could always tell he felt better. Luckily we had all the supplies we needed: moleskin for the edges, Microfoam for the diaper area, and eventually both to cover the inside and act as padding. The biggest asset was something we purchased online, the Cast Cooler. For about 30 bucks, I believe, we gained a world of comfort for Evan. It dried the cast in ten minutes and kept a steady flow of cooling air underneath. On the hottest days I'd hook this up and watch Evan fall asleep almost immediately. You can't put a price on that kind of comfort.

The past 15 weeks has truly been an adventure. It was challenging, but it's been rewarding. There were times when I thought I'd love to burn the cast as soon as it got cut off, but honestly as I sit here now I feel quite fondly for the thing. Like a good friend, it was there to support Evan when he needed it most; it's contribution, irreplaceable. The cast, the surgery, this experience, is one more gift in a long line of blessings Evan and our family have received. I am overwhelmed with love and gratitude for this moment. I am excited for the next chapter.

The Future's So Bright

Evan Today

Evan is currently in a spica, full body cast, after a right hip reconstruction; one of the anomalies on his right side. The course is 15 weeks in a cast, one month in a brace, then rigorous physical therapy so he can learn to sit and crawl again then he’ll learn to walk! The cast comes off July 6th. This was a planned surgery which is always a good thing; another check off the list.

Evan has reached the minimum weight and height requirements to be eligible to receive a kidney transplant. At 21 months he is now 22 lbs. He was added to the UNOS Kidney Transplant List on June 3, 2011. This was a big check off the list!

He has an appointment with his transplant team to discuss the next steps. The biggest step is the construction of an incontinent bladder. He needs a functioning bladder before he can receive a kidney. The timing of both must be precise so a living kidney donor is ideal. His dad is a match and almost finished his donor work up. We remain positive that he’ll qualify as Evan’s donor, but multiple plans are in place in case needed.

Additionally, Evan was recently prescribed eye glasses for farsightedness. His eyes, particularly the right are crossed. The Dr. is hoping his eyes will relax with the glasses and his vision will get worse. In three months he’ll be re-evaluated. If all goes well he’ll get a different prescription and be done. If his vision remains the same he’ll require surgical intervention to detach the muscle and reattach it further back. His follow-up is at the end of August.

Developmentally Evan is behind, but he is well advanced considering the time he’s spent in the hospital. He speaks at least 35 words, uses sign language and even knows a few words in Español. His hobbies include: pointing at things and asking, “that?!”; blowing kisses; turning pages in books; playing, listening and dancing to music; throwing things on the ground; laughing; repeating what we say; meeting new people; and talking on the phone.

Warrior in Action

Dialysis Complications

Evan has endured 17 surgeries. Most attributed to complications of dialysis, a necessary treatment to sustain Evan’s life until he’s ready for a kidney transplant.

There are two types of dialysis:

1) Peritoneal Dialysis (PD) - basically places a sugar water solution into the cavity that houses your organs. The solution attracts fluid from the body and the lining of the cavity acts as a natural filter. It's a super gentle method where you put the solution in via a catheter, allow it to sit for a while, drain it out and repeat. You continue to do this for however long is needed; usually anywhere from 8-14 hours. It's done everyday and can be done at home. The downside is that body cavity has no antibodies, so you can't get dust, dirt or anything in there or you'll get violently ill. It's a clean technique, as close to sterile as possible.

2) Homodialysis (HD) - this is done at a center, for the most part. For babies it's rare to find, but certain hospitals will do it. There are two in the LA area that provide outpatient HD. HD takes the blood out of your body, runs it through a machine to clean it and returns it back to you. A treatment runs usually from 3-4 hours. People are usually dialysed 3 times a week. This treatment is exhausting, harsh on your body and drastic changes can happen. One can experience cramping, the feeling of falling, and their blood pressure could drop so low they go comatose.

Our first choice for Evan is PD. Unfortunately, Evan’s body attacks peritoneal dialysis catheters; he’s had 4 fail, endured multiple revision surgeries and numerous complications. PD just doesn't work for Evan; fortunately HD, does.

Evan received his first HD catheter on January 21st, 2010. The next day he was transferred to the PICU of another hospital, the “Best in the West” for Pediatric Nephrology. He participates in 3 hour treatments 4x a week. Babies are fragile, especially when it comes to HD. I've seen him cramp, shake and scream in pain. I've watched him go limp when is blood pressure's bottomed out. He's lost his internal right jugular vein due to scar tissue from two HD catheters and is on his 3rd catheter, threaded through the left internal jugular.

His care is complicated and he is fragile, but he’s continued to thrive. In spite of everything, his life is full of love and positivity. He doesn't understand that what he goes through is any different than what we endure. His trips to dialysis for all he knows are equivalent to going to Grandma's. The staff treats us like family and really makes the best of his situation. He's their baby. We're truly blessed to have the resources and team that we've been given.

And the end is in sight. Evan has reached the weight necessary for transplant. He's been placed on the transplant list and undergoing his work-up.

Little Love

The Beginning

Evan's story as featured on www.prayersforevan.org:

When Evan was 21 weeks in the womb Evan's parents were informed he had kidney failure, causing low amniotic fluid levels, making it impossible for him to form lungs. They had three weeks to decide whether to terminate the pregnancy. They saw five High Risk OBs and all had the same prognosis. One simply stated, "don't even pray for a miracle." At the time Evan had begun kicking actively and his parents-to-be decided to celebrate their child's life while he was still present rather than morn the loss of their unborn child. If they only had him in the womb they would cherish the pregnancy and as long as their unborn child fought to live they would support him. They started to work with Eastern healers: a Qi Gong Practitioner, Acupuncturist and Herbalist. Most importantly they turned it over to the care of God, meditated, prayed and asked for prayer from others.

On August 31, 2009, at 32 gestational weeks, Evan was born via c-section as the umbilical cord wrapped around his neck three times. Weighing in at 3lbs 10oz, Evan was born with a malformed right lung and collapsed left lung. At the time he was on pressurized oxygen heading to a ventilator. His parents were told he wouldn't last 24 hours. The Drs explained there was nothing they could do and offered their condolences. That night, without any medical intervention, his collapsed lung healed itself. Three days later he was breathing room air on his own. To this day the Doctors can offer no explanation.

Evan was born with a VACTERL association which includes radial aplasia (his right arm is missing the radius bone) and a malformed urinary tract (he has a small, non-functioning bladder; a missing right kidney; a multi-cystic dysplastic left kidney that has failed; among other urological defects). In general, the right side of his body never formed properly and he has multiple anomalies; some requiring surgical intervention. The Genetics teams at two accredited hospitals attribute the above-mentioned to a spontaneous genetic mutation, “just a fluke”.

Evan spent the first 168 days of his life between two hospitals; enduring 10 surgeries and multiple complications. All but two weeks of his hospitalization was in an ICU.

On February 14th 2010 Evan was discharged from the hospital and able to come home for the first time.