About James

 

Our Journey with James 

It has been a long and incredible year. You could go as far as to say that what we thought was the most stressful and horrible year of our life actually turned into the most magical and rewarding year we could have ever imagined. We brought an amazing little boy into the world who was able to change many lives without even trying. Our family was blessed with James. He beat the odds, and went further than anyone could have ever imagined. We have been very private regarding James and his medical care, but we are going to share his full story. Our sweet boy passed Saturday afternoon after a very long fight with congenital birth defects and he deserves for everyone to know how unbelievable he was.

 

In October 2015 Whitney was at a high risk OB office for a second opinion due to bad angles and lots of movement from our very active baby. The tech sped through the entire ultrasound and revealed all of his normal anatomy until she got to his heart. She lingered on every view for an uncomfortable amount of time trying to identify the different structures. She squinted at the screen and turned her head all angles trying to make sense of what she was seeing. After ultrasounding the heart for no less than an hour, she called the doctor to discuss the results with her. It is the type of diagnosis where they use soft voices and sit you down in a special room that is already filled with tissues. James was diagnosed with Hypoplastic Left Heart Syndrome.

 

We were given our options: keeping our baby comfortable after birth until his inevitable demise or undergoing three palliative surgeries as an attempt to optimize his very abnormal anatomy. Mourning the idea of your perfect baby is like having a funeral for a living person. Staying in the womb provided safety and security because Whitney’s circulation would be able to support him, and this made the idea of delivery an especially terrifying ordeal. We were lucky enough to be able to take the week to grieve, discuss goals and expectations, and research which facility would give us the best chance of bringing our boy home. We decided that Boston Children’s Hospital (BCH) would give James the best hope for a future.

 

In mid-December we flew to Boston to meet with Dr. Kevin Friedman to figure out our best plan of attack. We had no idea of the important role that this man would play in our lives, but we are forever grateful for the outstanding care he gave James and the friendship that he gave us. The option of comfort care until James passed was taken off the table. Boston was going to attack James’ condition and do everything to give him a full life. It was this day that our fear turned to hope. That was an exciting and unexplainable feeling.

 

James was born early in the morning on February 2nd. He weighed 8 pounds and was 21 inches long. It took careful orchestration to ensure that all of the timing was perfectly lined up and the delivery room was packed with medical professionals from both hospitals. He was pink and crying, so we were able to spend a few moments with him before he was whisked away to the NICU for his treatments to start. They placed a catheter that would infuse a drug to keep his PDA open until his first surgery and was then transferred to BCH. It was so hard to accept that he was sick because he looked so healthy. The doctors noticed that James had a birthmark above his left eye that extended to his scalp. The dermatology service was called to evaluate it and decided that it was a port wine stain, which is a venous malformation (or abnormal blood vessel) that is located directly beneath the skin. We were told that this could strictly be a birthmark or it could be associated with Sturge-Weber Syndrome (SWS). The doctors said that it was highly unlikely and that both HLHS and SWS are rare and are not associated with each other. The neurology service ordered a brain MRI to look for evidence of abnormal vessels and we were ecstatic when we found that the preliminary reading was normal.

 

On February 4th James was scheduled to have his first surgery. The doctors talked to us that morning and told us that there may have been some very mild abnormalities noted on the MRI, but that they wouldn’t cause him issues and weren’t a reason not the proceed with the surgery. We walked our baby down to the OR for the first time and left his life in the hands of strangers. We waited for hours and cried in Whitney’s hospital bed while we waited for updates. After 6-7 hours we received the call that James did well and was out of surgery. We were overjoyed that he made it through his Norwood, the first of his three open heart surgeries, with flying colors.

 

While we were waiting to see him, we were taken into a conference room to discuss James’ port wine stain with a neurologist. The MRI images were pulled up on a projector and our hearts sank. The MRI was extremely abnormal with little symmetry between the left and right sides. James was officially diagnosed with SWS and his brain lesions were extensive. The neurologist told us that James would have significant developmental delays and would likely succumb to a stroke-like episode. He would have severe epilepsy that would start before his first birthday and may require a hemispherectomy in the future. He could possibly be blind or wheelchair bound. We asked what this would mean for his second surgery, and the cardiologist told us that they didn't know if he would even be a candidate for the second one. This was the first of his many hurdles in life and no one knew what to expect or how he would fare. HLHS would require at least 2 more surgeries to improve his vascular efficiency, but this would potentially cause problems with his SWS. James was the only documented person to have both HLHS and SWS, so we were flying blind.

 

We left the room terrified and angry and went to break the news to our family. Everyone was crushed. Our dreams of a future were shattered and we had to grieve not having a “normal” baby all over again. We tried to live in the present and celebrate the fact that he did so well during his major surgery. We also opted to not tell anyone outside of our immediate family because we didn’t want people to have another reason to pity him. SWS makes a person prone to seizures and learning, developmental, and physical disabilities. The SWS specialist from BCH met with us the following day and gave us a new sense of hope. Dr. Pinto explained that the patients with this disease present on a spectrum and that the brain lesions aren’t necessarily reflective of the symptoms you can expect to see. James had the most severe form where it involved the entire left half of his brain as well as the back of the right. The doctor told us that she couldn’t make any promises about what the future would hold, but that we should focus on James as a person and not his diagnosis. This solidified our decision to keep the SWS a secret as long as James was developing appropriately and his seizures were controlled. We were going to let James decide whether or not he wanted to share his full story. Anyone who met him when he had a shirt on would have never suspected that he had either condition.

 

James started having seizures a couple of days after his operation, but they were easily controlled by medication. They did a head ultrasound and it revealed a small stroke. His physical exams remained normal and the neurologists continued to closely monitor the bleed. It began to look like James’ ventricles of his brain were enlarging on ultrasound, and the doctors were concerned with hydrocephaly. They did a CT of James’ head and it revealed what they thought there two large strokes. They were not sure of the severity, but were pretty certain that we would see further developmental issues. His daily physical exams remained consistent and reassuring, his heart was recovering unbelievably well, and he was on track to reach the recovery floor in less than 2 weeks (which is pretty impressive).

 

On the day he was supposed to move, James aspirated while receiving food and got pneumonia. After two days of grasping for air and losing all energy he had gone septic and had no white blood cells left to help him fight the infection. A breathing tube was placed and we were told that we would just have to wait and see if James had enough energy to keep fighting. After several days of fighting, James could no longer lift his arms or legs or produce sound when he cried. We were presented with a bleak outcome and had began to make funeral preparations. The next morning, in true James fashion, he began to show signs of improvement and made a rapid recovery. Luckily he was born weighing 8 lbs because by this time he likely weighed about 5. This was when we changed all of our goals for James. We quit thinking about taking him to feed ducks, going on family vacations, and dancing with him at his wedding. The only goal that we had for James was to make sure that he had fun every single day and that not a single second was wasted. The neurologists were finally able to get another head MRI and it revealed that not only was James not hydrocephalic, but he also had not actually had two strokes. The doctor walked in and said, "We were wrong! Isn't that great news!" Our luck was turning around. We finally made it to the recovery floor and James had a G-tube placed to ensure that he received adequate nutrition and would receive his many medications with no issues. On March 16th we were discharged and were back home on the 18th.

 

We spent the next 3 months at home and it was incredible. James would have weekly doctor and therapy appointments with his team in Vanderbilt, and luckily he loved all of the road trips. Dr. David Parra was wonderful and welcomed James as a patient even though he had received his previous care in Boston. He made sure that James continued to thrive while we waited for him to be big and strong enough for his second surgery. We did all of the regular baby stuff plus a few extras and every second was a pleasure. He was a wonderful baby who slept through the night and never cried. James loved to play with Whitney and be “big spoon” when they napped. He was always excited to see Bryon after work and loved when he would play his guitar for him after bath time. James loved to lay on his changing table and play with the cats. He even began to give us his beautiful smile. Every morning started with smiles and every night ended the same. James was developing so well that no one could believe that his first month of life was spent on his back in a hospital bed. James looked incredible and his second surgery was scheduled for mid-June.

 

Prior to the Glenn procedure the doctors would need a better look into James’ anatomy, so he was sent to a cath lab to have any possible issues addressed. His body had developed abnormal blood vessels, known as collaterals, to help meet his body’s oxygen requirements These extra vessels would cause a great deal of stress after his next surgery, so the doctors coiled (cut off the blood flow) to these vessels. These coils would take time for the body to cope with, so fevers were to be expected. On top of that, different parts of James’ body would lose the blood flow they were used to having. These stress factors pushed James to have very severe seizures which resulted in him having to have a breathing tube placed and be moved to the cardiac ICU. The seizures continued off and on for 4 days until he finally made it 24 hours without having them on Whitney’s birthday. After that, we were told that James probably would be completely different and possibly have altered brain function. We decided that we had nothing to lose from attempting the operation, and once again, a funeral was being planned.

 

James underwent his second surgery on June 24th. He began having seizures postoperatively, but they were quickly controlled. As he had done in the first round, James bounced back beautifully. He would have to cope with the headaches that come after the Glenn and some new teeth, but we were discharged from the hospital on July 2nd, watched the fireworks in Boston on the 4th, and flew home on the 5th.

 

There was a clear change having James home this time. He had trouble eating and holding down his medicine. He was always tired and as the days passed he only got more exhausted. He clearly felt bad, but all of these symptoms coincided with different things that had occurred with his health. By the final week of July, James was vomiting daily and sleeping most of the time. His heart function had decreased, but the doctor was optimistic that starting a new drug would improve his function. We were given several reasons from pediatricians not to worry, but things didn’t feel right. James developed a cough on his last night home and we went to Vanderbilt the next morning.

 

An echo was performed and James’ heart function was markedly depressed. He was admitted to the cardiac ICU on August 4th. They were quickly able to stabilize him with a constant infusion of a drug that would help improve his function. The week after James was admitted to Vanderbilt, he had clinically began to respond to his heart failure therapies. We were blown out of the water by how good our baby felt. Nobody on the ICU floor even knew why he was in the ICU. Watching someone who had never really felt good go straight to someone feeling great is a gift not many people receive. James drank bottles on his own, he ate applesauce and sweet potatoes, and he didn’t take naps because he was too busy playing! His new burst of energy was exhausting, but it was incredible to see him so happy. We had to put signs on his door because too many hospital employees were stopping by to play. We also found out that James was a complete flirt with nurses. He had developed a fake cough to keep the pretty ones in his room.

 

On August 23rd, Bryon noticed that James’ right arm and leg were not moving while James continued playing with his left side. It was discovered that sometime that morning James had suffered a stroke. We were devastated, but James’ neurologist felt, with the right medication and stimulation, James would recover completely. We were shocked that in as little as 2 weeks James had fully recovered.

 

While his activity level had improved significantly, the pumping ability of his heart was very weak and it showed very little improvement. At that point we were told that James’ heart was unlikely to return to normal and that he would require a transplant. After a short evaluation, he was rejected from their transplant program. This was due to their unfamiliarity with James, him being highly sensitive due to previous exposures, and his abnormal anatomy. After a month in Vandy’s ICU, James began to rapidly decline. The doctors discussed intubating him and placing him on ECMO, but we refused. This would not be a path that would get us a new heart. We re-evaluated his current treatment plan and it bought us enough time for a bed to open in the BCH CICU. James was life flighted to Boston through hurricane winds on September 5th. We were lucky enough to have Dr. Andrew Smith ride with us to make sure he arrived safely.

 

We were very happy to be greeted by Dr. Sarah Teele who already had a plan in motion that would stabilize our sweet boy. She had followed him closely during his previous stays and seeing her was always a breath of relief. In the following days we met all of Children’s outstanding heart failure team, including Dr. Kevin Daly who helped us get the go ahead to fly up for re-evaluation. On September 15th Boston’s heart failure team was ready to take on the challenge and he was accepted on the transplant list. After a week and a half at Boston, James had regained his energy and was stable and playful. Once again, we had to keep track of all his visitors and friends, not only because we needed him to not be worn out, but also because some employees had a bad habit of staying too long and forgetting their other responsibilities. A few people even referred to James as the Mayor of 8 South (the cardiac ICU). He was easily the most popular person on the floor and everyone loved him. He probably had 5 or 6 girlfriends that were getting pretty serious.

 

We spent our days receiving music therapy with his friend Big James and pet therapy. James was progressing beautifully with his physical, occupational, and speech therapies. His fine motor skills were so good that the therapists wouldn’t even believe what he was doing. We read many books and played in his exersaucer. He would go on strolls to visit his friends around the unit. The good days drastically outnumbered the bad ones, and we made the most of every one.

 

On November 12th Whitney received an early morning phone call. James had been having abnormal amounts of liquid from his G tube and had been vomiting. That morning she was suspicious of a bowel obstruction, and, unfortunately, those suspicions proved to be true. James had likely been obstructed for almost 24 hours, and the surgeons were unsure of what they would find with their exploratory. We were incredibly lucky that he was able to reduce the torsion laparoscopically and, after a few complications with his heart, James felt better than ever. The next 3 weeks were the most wonderful weeks of our lives. Bryon was able to stay up here with us and James’ personality was really starting to shine. He would just lay in his bed and giggle as he tickled his feet. He would lure people into his room with his disproportionately long lashes and his piano. Many people would come to see him just to brighten their day.

 

December 9th was the day that we were informed that James had an organ donor and we were unbelievably excited. That day we had a crazy amount of fun and James was feeling the best he had ever felt. We laughed, played, and sang and his surgeon was ecstatic to have a happy, healthy baby going to transplant. We hugged him and kissed him to the point of getting on his nerves and literally took 100s of pictures. We walked him down to the OR and told him how brave he was. He rubbed Bryon’s cheek, gave us one of his sweet smiles, and headed back for his new heart.

 

December 10th, James’ new heart was successfully in place. Three hours after surgery he was placed on ECMO because his body was going into shock. ECMO is a life support machine that would give his new heart a chance to rest after surgery. He quickly stabilized and everyone was optimistic that his heart would recover nicely. After 48 hours, he was taken off ECMO and a few days later his chest was closed. One week after surgery, James’ kidneys began to acutely fail. We had to make the decision to put James back on ECMO to balance the needs of his heart and his other organs. He was also taken to the cath lab to see if his hemodynamics could be improved. His new heart was not producing the results we were hoping for and we hoped that a trip to the cath lab would explain why.

 

While at cath, James had 16 collaterals coiled, a stint place in his right pulmonary artery, and his aortic arch ballooned. These fixes were not expected to yield big results, so our doctors got emergency FDA approval to use a drug that is not yet legal in the United States. This drug would help his heart to pump without increasing the work it would have to put into it. We weren’t given high odds to see many changes, but on Christmas morning we and the doctors were shocked to see that James had responded amazingly to his therapies. His pressures had completely normalized and we were sure that this was what he needed to thrive. James came off ECMO once again and held his own for a couple of days before his pressures started getting hard to control. By December 28th it was clear that he was struggling and our team was trying every treatment they could think of. James had always turned around and recovered from these situations, so we were still very hopeful.

 

The night of the 29th James was placed on ECMO for a third time. This carried an extremely poor prognosis, and the decision was not made lightly. The incredible heart failure and cardiac ICU teams were racking their brains to ensure that no stone was left unturned. We are forever grateful for the time and effort they put into giving James every chance. There was never an obvious cause for James’ worsening condition, and we had discussed in the past that James was put on this earth to live and not just be kept alive. His quality of life was our top priority and that was slipping away. The next morning, we received a call very early that James’ new heart had developed an abnormal rhythm and needed to be corrected. We received another call shortly after that and learned that his heart had stopped and James was being completely supported by ECMO. We called our immediate family, gave them the heartbreaking news, and asked everyone to pray for a miracle.

 

Our incredible family members were all in Boston by the end of the day. We, as a family, got to have a very powerful experience together as we laughed and cried and talked about the amazing year we had with our incredible little man. Nurses and doctors all came by to join us and helped us realize how much of a blessing our time together was. At 1:15 pm on December 31st, James was able to stop being a brave fighter, and he got to rest in our arms. Many hearts were broken, but we all found comfort in knowing that James truly got to live. And, at this very moment, he is assisting with congenital heart disease, heart transplant, and SWS research with some of the brightest minds in the world.



James was a beautiful, intelligent, and, most importantly, silly little soul. His favorite things were playing with his feet (he was extremely flexible), flirting with girls, spooning his stuffed animals, dancing, and smiling. He was wise beyond his years and took a genuine interest in everyone he met. He comforted us on his bad days and taught us to cherish every moment. This year took a lot of work, but we would do it all over again. We want everyone to see James’ trials not to feel sorrow or pity, but to understand how tough and incredible he was. We want everyone to learn a new level of appreciation for being healthy and to enjoy every single day. There are no words that can express the pain that comes with him being gone or the desire we have to give him one more hug, but heaven just got a lot brighter (and 100% cuter). We can’t wait to see him again and celebrate our reunion. James touched hundreds of lives in 11 months on earth. We are truly inspired and beaming with pride that he was ours and that we were able to share him with so many people. He knew nothing but love, and we will be forever grateful to the exceptional doctors, nurses, and staff who became a part of our family. James Solheim Bruce truly made everything and everyone better.