This may not be the best organized story. It may not flow well. But our journey hasn't flowed perfectly. It’s been a bumpy road, and continues to be. I will keep this page updated and try over time to smooth out some of the writing. But for now this gives our bumpy journey in some sort of order.
We found out that I was pregnant on April 20, 2009, 1 day before my 30th birthday. It was a complete surprise. At a routine ultrasound on July 28, 2009 we found out that Eli's kidney wasn't quite right. It was enlarged and the doctor told us at this point that it was probably a small blockage that would most likely correct itself. He did decide to do another ultrasound in a few weeks just to check. At the next ultrasound his kidney had become even more enlarged, and Eli wasn't growing as they would normally expect. At this point the doc decided to do routine ultrasounds every 3 weeks. At each ultrasound his kidney got worse and worse, and Eli's growth continued to slow. Right before thanksgiving we had our last ultrasound and found out that yet again Eli's kidney had grown larger, and since the last ultrasound 3 weeks before, he hadn't grown; not 1 inch or gained not even 1 oz. The doctor scheduled an amnio for Dec 1st, and said most likely we would induce. The amnio showed Eli's lungs to be ready, and I was already in labor. Eli was born after 4 hrs of easy labor on Dec 1, 2009 at 4:02pm, weighing 5lbs 11oz and 16 1/4in. Within an hour of his birth we knew there was more to the story than just the kidney.
Eli began having twitches and spasms to the point that you could hardly move him or touch him. They did ultrasounds of his brain to check for bleeds, all were negative. They thought maybe it was just a bit of neurological prematurity and said it would be closely followed. They also found the undescended testicles at this point as well.
We went home from the hospital 2 days later on a Friday. By Sunday Eli had begun spitting up. By Monday he was gagging and choking and he would stop breathing. By Wednesday it was green and yellow bile that he was choking on. He was admitted to St Joe for testing. It took forever. He had so many tests, tests I had never heard of. If he was fed, if he was moved, if nothing at all, he would choke and turn blue. It was so scary during this time. He came home late Wednesday night with tons of information but no real answers. His kidney was huge and squashing his bladder, and he had level 3 reflux. They started him on meds. Thursday we met with the urologist and they decided to do further testing on his kidney. Friday we found out that his kidney was not working at all and was a lot of dead tissue and fluid. He would be referred to a children's hospital in KC for a surgeon.
We woke up on Monday Dec 14th to hell. This was the 1st time we were unsure if Eli would make it thru the day. Eli woke up with a high fever and choking and gagging more than he had been. He couldn't get his breath at all and was choking on such thick mucus. Bob had to rush him to the bathroom and turn the shower on hot and use the steam to try and break it up and be able to suction it out. Eli was blue. He was rushed to the hospital, suctioned the whole way. He was immediately admitted as his oxygen was dropping into the 70's and lower. He had a severe kidney infection, aspiration pneumonia, and a whole list of other things going on. They dropped an NG tube, which is a feeding tube that goes from his nose to his tummy. At this point he did still nurse some and also took some feeds thru the NG. He was so tiny, having lost so much weight at this point. Every feed was followed by throwing up, normally 10 plus times each feed. Every throwing up session, involved him choking and turning blue. Overnight Friday the 19th we had our 1st glimpse into Eli's heart problems, although it wouldn't be officially diagnosed for almost 2 more months. Eli failed his 1st swallow study on Monday, Dec 21st. We begged and pleaded with the doctors to go home for Christmas. Eli was discharged on Wednesday Dec 23rd, late in the evening. He was sent home with an NG and tons of other equipment that we didn't really understand.
We made it thru Christmas, mostly. We were exhausted and lost and had no clue what we were doing. Our oldest 4 kids came home on December 30th, and we hoped to settle into the routine of a family, just the 7 of us.
January 2010 was spent at doctor’s offices and labs. Eli couldn't seem to gain weight. He kept choking and gagging and throwing up. The NG tube was in and out. Nothing stabilized. He struggled continually and we just tried to keep up. There were lots of tests, but no answers. There were specialist appts, there were phone calls. It was a blurry mess.
February 2010 was spent at the hospital. Eli wasn't gaining enough weight, he was so sick. We met with the surgeon. She couldn't believe it. She said Eli was one of the sickest little guys she had seen. He was so tiny and malnourished. We met on Thursday Feb 4th. Surgery was on Monday Feb 8th. This was day 2 of thinking our son wouldn't make it thru the day. I remember it like it was yesterday. The surgeon was not optimistic about how Eli would do in surgery and coming out after surgery since he was so sick; but he had to have this surgery to have a chance of making it. The surgeon came out into the waiting room hours later and we could tell it wasn’t good news. They couldn’t get Eli to wake up and breathe on his own. She told us they were going to rush him to PICU. We headed down to the waiting room. We waited. We heard a noise in the hall and ran out; they were rushing his bed down the hall with nurses all around. They were bagging him. They told us they couldn’t stop and rushed past us. It was the scariest thing I have ever seen. An hour later we were allowed back to see him. He was hooked up to the vent and not doing well.
This is when we began to realize that there were more heart problems. His heart rate was incredibly high and then it would drop really low, his breathing was horrible, and he struggled. He was “awake” and off the vent, on special machines to help him breath. He was in a lot of pain. They did an EKG and an ECHO. He had a hole in his heart, an extra artery, and a heart murmur.
Eli finally came home on Feb 13th. Nursing began on Feb 17th. I was nursing during the day as much as he could tolerate, and pumping, and then he was having continuous overnight feeds with his pump. Eli’s Mickey was placed on Feb 23rd. It was so much easier and we loved it. The rest of Feb was spent at home, figuring out how to live.
Then came March 2010. March was again spent at home, slowly growing. He did grow, but not enough, not fast enough. He wouldn’t throw up any more, but he choked constantly. It was a daily event to see our child turn blue. We never left his side. We were at doctors all the time. Labs, x-rays, tests. It was a constant.
April 2010. The beginning of the month took us to KC to meet the kidney surgeon. He did lots of testing. Eli began getting sick in KC. We came home and Eli was admitted to St Francis on Feb 11th. He was discharged on the 12th. Eli spent most of the month sick with pneumonia. He also had his EEG. The results showed no seizures, although the neurologist is sure there is something going on.
May 2010. Began slowly. Then Eli started getting sick. By May 12th Eli had pneumonia. This was a bad round. He had to go off feeds. Breathing treatments constantly. His heart rate went sky high. He wasn’t peeing. He got antibiotic shots daily and we were able to keep him out of the hospital with this one. He lost a lot of weight and we really struggled getting his feeds back up. Next was his MRI with sedation. All showed ok on this test.
June 2010. Eli’s lungs are just getting worse and worse. He’s choking all the time. His oxygen drops frequently. He has pneumonia almost constantly. His chest x-rays always show fluid. Tuesday the 15th brought Eli’s next swallow study. The radiologist running the test was a mess. She goofed up the test and I knew it. I called and pushed for another test. I knew it was wrong, I knew she was missing something. The director of radiology agreed. The test was redone on Thursday the 17th. I was right, but not in the way I expected. Eli failed. We saw something on the test we had never seen before. They followed the liquid farther down this time and there it was. His swallow wasn’t so much the issue, but his esophagus was. It wasn’t moving. After a few swallows the food had completely stacked up and was then spilling over into his lungs. Everyone went frantic and ran around calling everyone. Eli was put NPO. I have never nursed my son since. He hasn’t had a bottle, a bite of food, not 1 single drink since this day. The next week was spent on the phone and a lot of crying. Phone calls were made to a motility specialist in KC. Appointments and tests were scheduled. Eli cried because he wanted to nurse, I cried because I wanted to nurse him. It was a rough time. Saturday, June 26th Eli was hospitalized with a fever. He came home the next day with another fever of unknown origin.
July 2010 brought a failed sleep study. Eli was put on oxygen at nights and for naps. His oxygen levels were dropping substantially at night time. Tuesday the 4th we headed to KC to meet with the motility specialist. The surgeon said that we needed to schedule some tests and they would call me with all of that information. The end of July took us back to KC to meet with his kidney surgeon for his next round of testing. His kidney tests came back showing his kidney was still very enlarged, but he didn’t want to schedule surgery until Eli was stronger. They also did a swallow study while we were there and Eli failed, and the test was stopped. Eli was immediately sick upon coming home and we were able to talk to his pediatrician by phone to try and figure out what next instead of heading to the ER.
August 2010 was rough. Eli continued to stay sick. He coughed constantly. He choked and gagged. His oxygen would drop and he would get tired so quickly. We were at the cardiologist, the pulmonologist, the pediatrician. It was a rush to figure out why he was getting sicker and sicker. Wed the 11th we found out he had pneumonia. He’s NPO and has a working fundo. Nobody knows why he has pneumonia again. He’s put on O2 constantly at this point. Eli just keeps getting sicker. Friday the 13th he ends up in the ER. They run tons of tests but can’t figure out what is going on. He gets sicker. Eli goes to his pulmonologist on Tuesday the 17th. By Wednesday the 18th we know what is going on. Eli has pertussis, whooping cough. He is on tons of antibiotics, as well as the rest of us. Eli just keeps getting sicker and sicker. He can’t keep his O2 up, his heart rate is so high, and he is choking and gagging. Monday Aug 30th Eli was admitted to the hospital. He was on ½ liter O2 and by the middle of the night we had to kick it up to 4 liters O2. His O2 kept falling. He was again rushed to the hospital. Tons of things happened. It was such a blur. This rushes us right into…
September 2010. They began Eli on the peds floor. I went home to get some clothes and sleep, bob stayed at the hospital. At 4:30am on September 1st my phone rang at home. Eli had a choking spell, his sats dropped; it took them almost 10 minutes to revive him. He was rushed to PICU. Next the doctor called. He wasn’t sure if Eli would make it thru the night and said to get up there now. Everything at this point becomes a blur. My parents come. I drive faster than ever before and get to the hospital. I run all the way to the PICU. They can’t get a line. They can’t get him stable. The PICC team comes but they can’t get a line. The head of PICU tries for a central line and Eli crashes and they have to bag him. He struggles. We are told they don’t know if he will make it and it’s a day by day. Doctors everywhere. We find out he has pulmonary hypertension. His numbers are really high. The doctors are worried. Finally, we went home with around the clock nursing and more equipment than ever on Sept 9th. We were sent home with ambu bags. We were trained on how to do advanced life saving, how to bag our son. He choked constantly; he stopped breathing all the time. He slowly began to recover at home. His heart rate stayed very high and we had to use O2 a lot to try and keep it down. By the 25th Eli was gagging and retching and actually throwing up some. The 28th brought another cardiologist appt and more tests. His pulmonary number has dropped some more, but still very high. We found out as well that the nurse at the motility specialist dropped the ball and lost Eli’s paperwork. She forgot to schedule all of his tests. Now he can’t swallow and can’t have the tests until he can safely swallow some. On the 30th I went to the bedroom to get Eli’s food, when I came out he was silently choking and turning blue. A lot of phone calls to follow. They think it’s a loosened fundo from all of the coughing. Back on O2 and the sat monitor continuous. Eli is placed on continuous feeds instead of his bolus feeds. The goal is to keep his stomach as empty as possible but still give him calories. They are hoping this will get us by.
October 2010 rolls around. Eli has surgery on Mon Oct 4th. They cram tons of stuff into 1 surgery. He has scopes, and they try to pull a testicle down, he has a flexible and rigid bronchoscopy, and a PH probe. They tried for a line. She couldn’t get one. She spent over an hour. She ended up having to slice open his neck and put a line straight into his external jugular. He woke up well but didn’t breathe well. He didn’t pee. They ended up having to cath him. We were right back in PICU. Eli came home on Wed the 6th with tons of nursing and a catheter. His cath came out on Friday the 8th and he was peeing on his own. He was still on continuous feeds at this point. He went to KC for feeding clinic, and to try and work thru his severe oral aversion. Oct 27th brought a sick tiny man. His feeds are stopped and his back on continuous pedialtye. On Friday the 29th Eli had a major choking episode. He couldn’t get any air at all. The surgeon immediately scheduled him to have a GJ placed.
November 1, 2010 brought a hospital trip to have the GJ placed. This brought MORE information. Eli’s stomach isn’t moving properly. He has gastric motility as well now. It was a rough start to get the GJ in place. He began vomiting and having bile spill out everywhere. The surgeons didn’t know what to do. Nobody knew what to do. He was getting so sick. His heart rate was thru the roof, and his lungs sounded horrible. Some tubie moms were able to help and we ended up draining his stomach and trying to slowly feed the J. This seemed to help some. Breathing treatments were increased. Steroids were started. O2 was put on to help with his heart rate as well. Eli started getting sick again. He was coughing a lot, he was choking, and he had a fever. He was hospitalized Saturday the 13th. He again has aspiration pneumonia. Tons of labs, fluids, O2. His heart rate is again super high, his respirations are super fast. He has acidosis. His pertussis again comes back positive. Tons of meds.
The rest of the month is a blur of bad news. We see doctor after doctor. Meds after meds. Coughing, choking, and refluxing.
Eli has his sleep study on Monday night the 22nd. He fails horrible. His O2 drops, he stops breathing. He has central sleep apnea. This means his airway is collapsing and his brain isn’t telling his body to breath.
December 2010. Eli struggles medically. He still isn’t making any sounds. They do his hearing test. He fails. He goes to the ENT. No fluid. The ENT says it’s permanent. He is going to do a special test at the hospital to find out if there is any hearing left. Eli will be in speech and OT to begin learning sign language. He has PT weekly as well. He meets with doctors and surgeons. He has geneticists to try and figure out what is happening. We have a long road ahead. Trying to get strong enough for kidney surgery. We need another surgery to fix his fundo. The testicle that was lowered has gone back up, so both need pulled down in a 2 stage surgery. We need testing for motility. Currently there is no cure for esophageal motility problems or gastric motility issues. His heart is doing a bit better. The hole is smaller, the extra artery gone. His pulmonary hypertension number is at the top end of normal. His lungs are a mess. Permanently damaged, constantly struggling. Neurologically we are unsure. He meets with a new neurologist next month to start all over there. The geneticist is certain there is a problem but can’t find it. The surgeon is at a loss. The GI doc says she has no clue what to do next. The motility specialist is stuck because of Eli’s severe aspiration. He can roll now. He can’t crawl, he can’t walk. His feet are starting to drop from lack of use. He will be fit with hearing aids if there is any hearing left, if none left then it’s simply signing. We have doctors everywhere and are going to be expanding soon to Denver or Dallas. Eli is watched constantly. He is grounded to the house. His cardiologist is concerned for him. If he gets sick or if he needs another central line. His heart keeps making it thru each process put concerned that if he’s sick again we may begin having permanent damage. We all know that we are missing something but we don’t know what. We keep losing more systems. Things that were working are no longer working.
It’s a rush to find out what is going on. It’s a rush to stop it before we lose the next thing. Our order of progression is as follows:
1st kidney, testicles
3rd stomach valve
6th esophagus motility
7th gastric motility
Christmas afternoon eli took his 1st crawl forward. We were beyond excited. By Christmas evening we could tell that Eli was getting sick. We made it t home for the rest of the year.
January 2nd 2011 brought sickness full on. Eli was admitted with gastric and urinary shut down. They got a IV going (barely) and began fluids. He wasn’t able to tolerate ANY feeds. It was decided that a port was necessary to give TPN. Eli went into surgery on January 5th. They were able to get the port placed and get TPN started the next day. Over the next week plus his feeds were very slowly increased. Eli thrived on feeds, TPN, and IV fluids. The TPN and fluids were weaned down. He struggled with energy. Nobody was sure why, but it appeared as though he needed more fluids and protein. He was finally discharged on January 20th. January 21st brought a very tired little man. He hardly wanted to wake up. January 22nd brought a very high fever and high heartrate. Called the oncall doctor and they said take him into the ER. We got to the ER and they said it’s a bug from being hospitalized, go home. They didn’t run a lab test, not 1 single thing. We left to head to KC on January 23rd for a doctors appt. Eli was struggling with his O2 levels. By the time we got there his heartrate was still high and he still required O2. 30 minutes after checking into the Ronald McDonald House Eli crashed. He couldn’t get enough air moving, his entire body was shaking uncontrollably. He turned blue, then gray. We called 911, and he was rushed by ambulance to the Children’s Hospital. He was taken into a trauma room and swarmed by tons of people. He had an infection in his port that went into his blood stream and into his heart, and he was septic. Gram negative seratia sepsis. He was admitted to the PICU. They discovered the next day that he had a blood clot attached to the end of the port in his heart. Surgery was on January 25th to remove the port and the blood clot. The next day they ran another ECHO and his port had been hiding a moderately large sized blood clot right next to the Vena Cava. They also discovered that Eli was anemic and that it appeared to be a long term issue that had been going on for some time. This was by far one of the scariest times we have gone thru in the last 15 months.
This brings us into February 2011. It was a long road getting Eli stable. They put in a PICC line and he was on IV antibiotics as well as blood thinner. 2 weeks in the hospital. He was discharged on February 5th. Eli did another 2 weeks of antibiotics at home. 3 days before ending that his PICC line got a hole in it and had to be removed at our local hospital. We got a call from KC on Monday concerned since an ECHO hadn’t been done before removing. Back to KC we went for another ECHO. The blood clot had gone from 6x6 to 4x7. They decided to extend the blood thinners to 4 months.
On February 26th Eli had a scope run to look at his larynx and voice box and the anatomy of his throat. All looked good with this. He also had an ABR and he again failed. They immediately fitted him for hearing aids.
March 2011 brought an old “situation” back. Eli has begun gagging and retching again. He is mostly just turning red, but once or twice he’s turned blue. We’ve had some issues with elevated heart rates and lower than normal O2 sats. They have checked GJ tube placement and it is fine. He doesn’t have any extra secretions.
We have been in KC each and every week. Eli requires a lot of lab work and follow-up procedures. He has frequent ECHOs to keep track of what is happening with the clot.
He is working with feeding therapy to try and be to the point of safely taking 10 bites of baby food by mouth in under 3 minutes. He needs to be able to pass a swallow study so that he can safely finish up the motility testing. His hearing aids will be in at the end of the month. He has switched over to Children’s Mercy for all of his care, including his primary pediatrician. We have been incredibly impressed with them. They are scheduling a lot of appts and testing coming up over the next few months. He will be meeting with ortho to discuss foot braces, and meeting with seating clinic to look at wheelchairs. He will be meeting with a developmental and behavioral psychologist as well for a full “where are we now” kind of thing.
April 2011.We headed to Atlanta. Eli, Bob, and I flew to Atlanta to meet with the worlds leading Mito specialist. Eli had a muscle biopsy and tons of lab work. They ran over 27 tests. Eli did well on the flight there and even after his surgery. The flight home was a disaster. He couldn't keep his O2 up and ended up needing the airline oxygen on top of his own wide open on the highest flow. The rest of April brought alot of doctors appointments and a lot of testing. Eli started struggling with his secretions more and more. We met with tons of doctors and he endured alot of tests to try and find out what was happening. The choking and gagging was out of control and was exhausting him to the point he slept for 20 hours a day. His heartrate was thru the roof.
May 2011 .Brought 8 new specialists. It was an exhausting time. The neurologist is unsure if Eli will gain the skills that everyone is hoping and pushing for. He is concerned that Eli is functionally deaf. He thinks the mitochondria in the brain are breaking down and making road blocks along the pathways, which is causing sound to not be processed. This would also explain Eli's lack of understanding and sound and so many other things. He says there is sadly nothing we can do but wait and see. The longer it takes Eli to gain a skill the less likely we will ever get that skill. Pulmonology was greatly concerned with how Eli is handling his secretions as well as how he handles his O2 in his carseat. New meds started to lower secretions and now also using catheter suctions to help get some of the secretions farther down. Eli's last ECHO showed that the blood clot has changed into a calcified leason and is now growing. Being that it is inside of his heart that is of concern. The hemoc team has turned it over to his cardio team. His heartrate spent most of the month very high and has now dumped down strangely low. As the weather is warming up Eli is struggling more to keep his body cool, and dehydration has been an issue again. Trying to find the balance so his kidneys don't have to work to hard. Still alot of unanswered questions.
June 2011. We packed up and moved to KC. Eli spent the beginning of the month getting completely situated with his docs and therapy. They discussed adding O2 to his CPAP. They also spent some time discussing his heat sensitivity and hydration needs. June also officially brought Eli's Mito results. It came back showing Complex I and III, problems with OXPHOS, multiple deficiencies and immune system issues. Some more hydration issues as well as some weight loss.
July 2011 Eli ended up admitted on the 4th of July. His blood sugars kept dropping and he displayed symptoms of sugars to low. He was discharged with orders for sugar checks and increased fluids. Eli had testing with Hemoc and it came back showing that he was at increased risk for another blood clot. Eli had alot of sugar, bowel, and hydration/weight issues. Meeting with Eli's GI doc and it was found that he was shedding pieces of his bowel in his stool. Eli ended up admitted mid month to try and find out what was going on with his GI system. He ended up having alot of blood sugar issues overnight. While inpatient hemoc came by and informed us that Eli's blood testing came back showing that he has Factor V Leiden. This is a blood clotting disorder that causes him to clot. During this hospital stay and all of the testing it was determined that Eli also has a Growth Hormone Deficiency and will need to go on growth hormone injections every evening. Eli needed to have an MRI run to make sure that there was no tumor on his pituitary gland that could be causing the issues.
August 2011 Eli struggles alot with heat and fluid retention. Eli struggled this month with only using his left leg and he would simply drag his right leg along behind him. They ended up scheduling Eli for an emergency neuro appt. A few days after Eli had his first 2 noticeable obvious seizures. They scheduled an EEG. He also had an appt with the new Renal Doc. Eli had some big breathing issues while sleeping and the docs believe it was seizures while he slept. They ordered more O2 with his CPAP and ordered an emergency sleep study as well. We met with the new neuro and he said that Eli's EEG showed seizure activity and over all reduced background waves. He decided to place Eli on seizure meds.
September 2011 Eli met with the muscle and nerve clinic and he diagnosed Eli with mitochondrial myopathy. Eli used a gate trainer at PT and took his first steps ever!!! Eli also had his sleep study. Eli continues to have sleeping issues and they decided to switch to BiPAP. We met with eli's pulmonologist and discussed all the results. Eli had central mixed sleep apnea as well as restless leg syndrome. He was also concerned about Eli's secretions and the amount of choking that was again occurring. We ended the month by meeting with Eli's geneticist.
It is now June of 2013. My baby is GONE. Our tiny hero passed away on April 28th 2013 at 4:08am.
I was going to go back and update everything I never got to do.
In a nutshell so many things of Eli declined. He ended up needing a central line for all of his fluids and sugars. He needed more and more meds. He needed more and more supports and help and supplements to keep some semblance of balance. He got sick frequently and we were in and out of doctors and hospitals. This was our life. More medical equipment entered our house constantly.
Also some things improved. He learned to walk and climb on and off the couch. He was trying to learn to run. We finally had communication. Eli could sign. He had just learned to say, Hi and Ma and Da. He loved to play with his siblings. He learned to walk outside. He was smart and beautiful and perfect.
But that all came crashing down on us. We lost our baby. None of the month by month updating seems of any importance when our arms are empty and our world is shattered.
I can't retype about that day. It's to much. Here is the link to read the blog post explain what happened.
Some day I may be able to update and fill in the blanks, but that day isn't now.