08/22/10 Hematology

This week we were able to get Eli in to do some additional blood work and we had a hematology clinic appointment. The lab work showed Eli's hematocrit had improved a little since our last appointment but it was still a little low and they are going to continue to monitor it. In addition we found out that the size and shape of Eli's blood cells are normal which rules out a lot of bad blood disorders. It looks like Eli's marrow is producing blood cells but the recurring theme was a low ceruloplasmin level. Ceruloplasmin is associated with Menke's disease and we've had Eli tested for the genetic marker and it came back negative. We've also gone round and round with his ceruloplasmin tests. They sent this test off to a special lab at one point to measure how low it really was and the special lab came back with a normal result albeit a little lower than what was measured at the hospital lab. According to the hematologist we meet with the result may be "normal" but it's still lower than it should be and could have some blood impact. They are going to send Eli's blood off to the special lab again to measure the level again and then we're going to look at next steps. We discussed the possibility of doing a bone marrow extraction to make sure his marrow was healthy and producing red blood cells the way it should. This is a bit of a long shot but something we might consider if Eli has to go under general anethesia for something else.

I've attached a graphical representation of Eli's hematocrit level since he was born to give you an idea of what we've had to deal with. Normal hematocrit levels for an adult male range between 39% and 50%. For a 6 month old the level should be somewhere between 33% to 40% on average. Young children have a little lower hematocrit than adults. Hematocrit measures the volume of red blood cells as a percentage of your overall blood.

Eli has been running on the low side of normal for most of his short life. What we don't know for certain is what impact we had from drawing all the blood during his first month of life. There was certainly some impact and we bolstered him with the transfusion but we still don't know if his blood issues are indicative of his overall condition or if they are simply a complication from the hospital stay and trying to find a diagnosis. The only thing we can do now is continue to monitor it and address any issues that come up.

07/30/10 Blood Work

We got a call from the hospital today to discuss Eli's tests and blood work. Eli's hematocrit has dropped down again and the doctors are a little worried about it. They want to do some more testing and more thorough blood work on him. We've asked if we can be referred to a specialist in hematology but Eli's doctor, Dr. Makris, is out of the office this week and the current attending isn't willing to refer us. I'm not sure what the big deal is because we had a hematology consultation when we were in the hospital dealing with this same issue so this isn't uncharted territory. It looks like we'll be waiting until next week to find out if we get a hematology referral.

07/28/10 Results

On Monday Patricia took Eli in for his clinic appointment. Things have been going fairly well at home so we were looking forward to getting some test results back to confirm his progress. Overall, his appointment went well but we have a couple issues to deal with. Specifically, the site where the G-tube comes out of his belly is growing. They call this a granuloma. Eli had an umbilical granuloma which we had treated with silver nitrate. They recommend we have the granuloma site treated to stop the tissue growth. They also think we need to get a button put on the G-tube. Normally this wouldn't be a big deal but we have to put Eli under anesthesia to perform the procedure. He didn't recover very well when we had the original procedure done so we're a little worried about this.

Eli's CO2 levels have been really good lately. He's even dropped down into the high 40's on a few of his tests so it's nice to see a little progress on this issue.

07/25/10 Steady Progress

Eli has continued to show slow but steady progress. He still have pronounced hypotonia but we think we might be making some strides with his apnea.

Over the last month, nights had become increasingly difficult because Eli simply didn't want to keep the BiPAP mask on. We were getting maybe 4 or 5 hours of sleep and Eli was barely tolerating the BiPAP during those hours. He continued to grow more and more restless. We soon got to the point where Eli was getting less than 4 hours of good BiPAP time at night so we had to start adding some BiPAP time during the afternoons. Needless to say this wasn't working very well. The good news is Eli continued to maintain his CO2 levels throughout this entire period so we were starting to wonder if we could reduce or even take him off the BiPAP.

Around the first week of July Patricia called the hospital and scheduled another sleep study for Eli. Eli's pulminologist, Dr. Makris, just about fell out of his chair when he found out. We had several wasted sleep studies while we were in the hospital and we vowed we'd never do another one. Well, times have changed...

A week later Patricia took Eli into the hospital on a Sunday night for the sleep study. The sleep study was done without BiPAP. Eli was on regular nasal cannual oxygen the whole night. Two days later we got the preliminary results. Eli had shown a significant improvement in the apnea and he was able to maintain his CO2 in the low 50's throughout the study and the blood work they did the next morning confirmed it. Dr. Makris agreed that we could take Eli off the BiPAP as long as we continued to monitor his CO2 level closely at home.

That was about two weeks ago and so far so good! Eli has continued to maintain his CO2 levels in the low 50's and he's getting more restful sleep. So are we! The net side benefit of this is we both feel Eli has had more alert and awake periods during the day. This was a very big step for us and we're hoping we can continue on without the BiPAP.

Eli is scheduled for his regular clinic appointment tomorrow so I'll post a little more about the sleep study and how he's doing after we get the results from his check up.

06/27/10 Holding Strong

The last few weeks have gone well. Eli continues to grow stronger every day. Nights are still rough but we're managing. I even got away to Hawaii on a business trip for 4 fabulous nights of uninterrupted sleep!

About 2 weeks ago we took Eli in to see a neurologist that was recommended to us by Dr. Shlafer. We were looking for a couple things. First, did we miss anything with the neurological workup Eli received at Seattle Children's and second, is there anything we can be doing right now to make sure Eli has the best chance to live a normal life as he grows up. There were two poignant moments for me during the visit with the neurologist. The first came when the neurologist was looking over Eli's records from Seattle Children's and noted that we had a very thorough workup and the doctor then mentioned how frustrating it must be to have such a pronounced case of hypotonia and no answers. In cases with pronounced hypotonia at birth there's usually a fairly significant underlying cause. For me, that just underlined the fact that Eli has some significant issues and challenges to overcome. The other moment was when Patricia asked the neurologist if he thought Eli would ever walk. I've never even considered the fact that Eli might not be able to walk but it's a possibility. We simply don't know what to expect at this point. We just have to keep praying and hoping that someday Eli outgrows whatever is wrong with him so he can live a fairly normal life.

Over the last couple weeks we've also had some issues with Eli's G-tube, specifically, the skin underneath and around where the tube comes out of his belly looks inflamed and possibly infected. Patricia has been cleaning it daily but it just doesn't look right. We took Eli in to the pediatrician and he said it looked ok so we're not sure. He told us to put some of the antibiotic rub on it daily but other than that it looked fairly normal. I still think it looks funny so we're going to ask at Eli's next clinic visit which is coming up in a week. In the meanwhile we're watching it close to make sure it doesn't start to look any worse.

On the positive side of things, Eli continues to get bigger and louder. He's making more baby sounds now and he isn't shy about letting us know when he needs food or if he isn't feeling well. He even started arching and trying to roll over when he's on the floor. I think he's really close to rolling over but I haven't seen him make the flop yet. Within the next week or two we expect he'll start doing it regularly so we're very encouraged by this progress. We also had some interaction with the Little Red Schoolhouse program sponsored by Washington State which provides physical therapy to children who are developmentally delayed.

Overall we're still watching and waiting. The boys love hanging out with Eli and Eli is especially responsive to Ian. Ian has such a gentle loving way about him and he really dotes on his littlest brother. Too bad that doesn't hold true for how he treats Obie... Ian loves to get down on the ground with Eli and he spends a lot of time talking to him and checking him out. Eli in turn stares at Ian and more recently Eli has started to babble and coo at him. It's been great to see the progress and the family coming together.

06/06/10 Overdue Update

My intent was to do weekly updates but there hasn't been a lot to report on. Patricia reminded me that we have a lot of people following the blog and even the little things are important so I figured it was time to post something again.

Eli has been home now for a while and he is still growing and doing well. As Eli has grown he has become a little stronger and a little healthier but we still run into issues now and then. For example, Eli was making some very frightening noises over the last couple weeks. When he inhales its sounds like his throat is collapsing and he is trying to suck air through a collapsed airway. The sound is fairly loud and can only be described as a cross between gasping and choking.

It just so happens that we had a swallow study scheduled last week so we decided to have the pulmonologist look at Eli to see if we could determine what was going on with the noises. Patricia took Eli down to the hospital and between the swallow study, the visit with the pulmonologist, and the lab she was at the hospital all day.

The swallow study went well. Eli has been progressing steadily in terms of his ability to swallow and handle fluids. They cleared us to increase his feedings by the bottle and subsequently he has taken a few of his feedings entirely by bottle. This is a big step forward for us. He isn't to the point where he can take all of his feedings during the day by bottle but just the fact that he is taking an entire feed now and then is a big step. We are still feeding Eli every 3 hours. We were also cleared to start him on some rice cereal. We'll start on some solids in the next month or so.

The meeting with the pulmonologist went well too. After examining Eli and listening to the noise he is making the pulmonologist thinks it's simply a matter of Eli getting bigger and having some floppy vocal cords. If you've been around any infants you know they can make all kinds of strange and startling noises but after a while you get used to them. I don't think I'll ever get used to the sounds Eli makes.

Eli's lab work came back relatively clean as well. His CO2 was in the low 50's and his bicarbonate was in the normal to normal high range. Everything looked pretty normal with one exception. Eli had a dangerously high level of potassium in his blood. We were told that this is fairly common if a blood vessel is broken during the blood draw. Basically it's a false positive. They asked us to bring Eli back the next day for another blood draw. The result of that blood draw was perfectly normal so the assumption that the potassium was related to a broken blood vessel must have been correct.

As of this weekend we're still progressing. We've been in contact with some early development intervention professionals. They will come evaluate Eli and work with us to make sure Eli has the support he needs to catch up with his development. This is a state funded program available for children who are deemed to have developmental delays of 20% or greater. Since Eli has a feeding tube and is taking the majority of his feeds through the feeding tube he automatically qualifies. We've also scheduled an appointment with a neurologist outside of the hospital network. He was recommended by our pediatrician and we're going to have him review Eli's case and examine Eli to give us a second opinion. We want to make sure we're not missing something and we want to make sure we're still pushing for a diagnosis.

Finally, as of this weekend, we think Eli is starting to make some normal baby noises like cooing. He has been having more and more alert and awake time and he will sit and stare at his big brothers while they are running around acting crazy. Both Ian and Obie absolutely love their little brother but they are very rambunctious and they need to be reminded to be careful around their little brother.

For all the positive things going on we're still having a lot of issues with sleep. Nights are not the same in our house. Eli continues to struggle with his BiPAP mask and he requires a lot of interaction at night. Patricia has handled almost all of this interaction. Obie has been struggling a little getting used to having a younger sibling in the house and I think that's come out at night. He wants to be snuggled to sleep most nights and almost always by Mom who is busy trying to deal with Eli. Between Eli and Obie and having them wake up in the middle of the night, sometimes several times, we don't get a lot of good sleep. There have been several nights where we've had less than 3 hours of sleep and those are tough especially if it's on a work night. Thankfully Ian has been steady when it comes to sleep. He goes to bed at bed time and he wakes up around the same time every morning. We've had limited issues with Ian related to bed wetting but most of those challenges are behind us now. We're dealing with the lack of sleep and we're taking every day in stride. Overall it's been worth the struggle because Eli is home and progressing.

In a broader sense we're watching Eli grow and we're working to prevent future developmental issues but it's largely a waiting game at this point. We continue to pray for strength, patience, and healing. The love and support everyone has shown us has been absolutely amazing and we want you all to know how much we appreciate it. I couldn't imagine going through this process without all the help, love, and support we've received.

05/23/10 Weekly Update

The last week has gone pretty well in terms of Eli's health. We are very happy to have him home but it has been a lot of work. The nights have been especially rough as we cope with putting Eli on the mask while trying to get him settled down every night.

During the week Eli had a pediatrician's visit and a pulmonary clinic visit down at Seattle Children's. Both appointments went very well. Eli is maintaining his CO2 at home and his bicarbonate levels are holding steady. The only thing to note was a slightly lower hematocrit level. If you've been following the blog you know that we've struggled with some blood issues and we even had Eli transfused at one point to overcome the hematocrit issues. For some reason we just can't seem to put the hematocrit issues behind us. Eli's retic count is up in a solid range so there wasn't a lot of concern at the clinic but it's something that we are going to need to watch for the long term. Maybe the low hematocrit levels have something to do with Eli's overall condition. Since we don't have a diagnosis we're still looking for any possible explanation but so far we haven't been able to nail anything down.

The biggest challenge right now is getting enough sleep to be functional. As any parent knows, there's a period of time after you bring your baby home when you long for a solid night of rest. We pushed that off a little when we had Eli in the hospital because we could head up to the sleeper room to get some uninterrupted sleep. Now that we have Eli home we have been dealing with the issues that lack of sleep brings. Everyone has been a little cranky this last week but we're working through it and we're getting the job done with Eli.

On most days when we take Eli off the BiPAP we take a quick sample of his CO2 level using the end tidal CO2 monitor. I think we're averaging something in the low 50's most of the time and we even had a reading in the high 40's one morning. Eli's next clinic visit is about 3 weeks out so we have some time between the official check points but we feel like things are going well.

I'll try to post another update soon so stay tuned and thanks for your continuing support and prayers.