Tonight is the first night in over a year that Judah’s pulse ox and saturations have reflected that he needs to be on oxygen. He is usually around 97% with a heart rate of 130. But tonight as he is falling asleep next to me, his breathing is more labored than usual with a respiratory rate at 50 (usually around 24-32). Of course, it ALWAYS happens that he gets sick after we go to the doctor. We knew it was bound to happen after the week we had. This is a tough reminder that although he might look healthy to most people, NEHI will always be lurking and can bring on/worsen symptoms all of a sudden.
Judah saw a pulmonologist (his normal pulm wasn’t there, so we had to see someone different). Our main issue going into today’s appointment was that off of oxygen Judah’s heart rate while sleeping is higher than his heart rate ON oxygen while sleeping, and when we have him off oxygen long term his behavior seems to suffer. She said since his saturations off of oxygen are good she believes that the cannula has probably become some sort of comfort to him and that allows him to fall into a deeper sleep, which is making his heart rate lower on oxygen. And she said that the small amount of oxygen he is on (1/8 LPM) will not cause any problems and will not cause him to form a dependency on it. She said she is going to leave the nighttime weaning up to us based on signs that we see from him. Now, we have never seen this pulm before, and Chance and I aren’t exactly sure we agree completely with her theory that it is just a sort of comfort for him…we do believe he actually benefits from the small amount of extra oxygen he is getting because we’ve seen others in our group that have also benefitted from keeping their child on oxygen even when saturations fine without it. We will trial both sides over the next 6 months and see how it goes.
Overall, a not-terrible day. We survived it 😉 as you can see from Judah’s picture, he was just over it by that point.
Judah’s appointment with the nutritionist was quick and we were able to come up with a plan to increase his calories. She also helped break down his needs as far as fiber goes and believes that the fiber content of his real food in his button (Nourish) could be causing the diaper rash (we’ve been dealing with rash for about 2 years until I just recently started blending our own tube food and it finally cleared up). Continuing to blend our own tube food will be more expensive and time consuming than using Nourish or Liquid Hope, but it is worth it if it means he won’t have diaper rash.
We finally finished our 2:00 appointment around 5:00 tonight. Doctor Carroll said Judah looks great! He had a chest X-ray (which he completely hated) that was completely clear (which the doctor said is amazing because normally they will show at least some minimal spotting or issues). He is happy with Judah’s weight and development. 🙂 The one issue we had going into the appointment is that when he gets less than 3 hours of sleep on oxygen each night he always seems more tired during the day and has more than just “normal toddler” fits. Judah can maintain his oxygen saturations without being on oxygen, but it’s at the expense of him having to work a little harder to keep up. As far as his behavior when we are having him off of oxygen at night he just said that sometimes they can have fitful sleep if their body is having to work harder to breathe which could be the reason he’s acting moody during the day if he’s not getting enough restful sleep. Dr. Carroll said he doesn’t think it’s time for a sleep study or anything like that but we can just give it a couple months before we try decreasing oxygen anymore. We are fine with that since I’d rather have a happy toddler on oxygen than a miserable toddler without oxygen.
As for Isaiah (little brother), he said he thinks the pediatrician should be at the point where he’s showing more concern as far as the lack of weight gain. I showed him the Isaiah’s growth chart and he immediately said “oh yeah, that’s a problem”. Isaiah has dropped from the 98th percentile at birth to the 3rd percentile and says that if he continues on this pattern, soon he will be completely off the growth chart. Mainly our reasoning for asking this doctor about this issue is because Judah’s initial blood work when he was diagnosed with NEHI showed a mutation of the TTF1 gene which is the thyroid transcription gene which other doctors have related to NEHI and heredity. He says that a simple blood test can indicate thyroid issues so he would think that would be a place to start. Then he suggested getting a nutritionist on board to see how many calories Isaiah should be consuming vs what he actually is. And then if those two don’t help him gain weight or indicate the problem then he suggested we see a GI who could address issues like malabsorption.
Overall it was a good appointment, even though Judah shed some tears during the X-ray and while getting his blood pressure checked (he’s still saying “that so hurts” :P). He was happy when the tech gave him bubbles afterwards though!
It was an easy and quick appointment with the Pulmonologist today. We reevaluated our plan for decreasing his oxygen at night. Since April we’ve been increasing his time by 1 hour per month to sleep on room air before turning the oxygen on. So he is currently at the first 6 hours of sleep on room air per night. The doctor said he feels like we could make the change every 2 weeks or so, opposed to a month, given that Judah handles it fine. If he handles it then we are looking at around 3 more months on oxygen at night. (Amazing and also scary for me at the same time!) Of course, with it being winter a lot of this will depend on how well we can keep him from getting sick because that could change things.
The one concern (which they said isn’t even really a concern…just something to be aware of) is that he is gaining weight pretty slow. He has gained around 2 pounds in 6 months, and they like for it to be 1.5-3.5 pounds per 6 month period (as a general guideline)…so he is still in the safe range, just on the low end. So I will be consulting with his nutritionist to see what we need to do about that.
We talked about our lung family/support group we are a part of and the blog I have kept and how I’ve been able to talk to other parents about what they are going through with their NEHI/chILD kiddos. Dr. Carroll said that I am an “expert from experience” so that made me feel good. I just like to help others who are going through this same journey when I can.
Overall, a good appointment!
Two years ago yesterday Judah had his first pulmonary appointment at Arkansas Children’s Hospital. We were in the clinic for over 8 hours. His oxygen levels were at 93% awake and 88% asleep. His respiratory rate was 65+ breaths per minute, double what it should have been. He went through a sweat test for cystic fibrosis, he had a repeat chest x-ray, he was observed during nursing to check for aspiration, he had blood drawn, we answered a thousand questions and explained his symptoms to so many people, and ultimately he was admitted to the hospital. We were not prepared with clothes or anything. He was put on oxygen for the first time and hooked up to the pulse oximeter. It was probably the longest day of my life that turned into the longest night of my life. Judah was scheduled for a CT scan the next day (a year ago today), under sedation, so he could not be fed anything after midnight. He was also put into a baby crib after being used to sleeping next to me and nursing throughout the night. I couldn’t feed or comfort him the only way he knew how. He was poked and prodded for an IV. He was tangled in tubing and wires. He was a crying mess. And so was I. My heart was just broken. Chance did his best to comfort both of us during the night…none of us got any sleep at all. The next morning he was taken for a repeat echocardiogram (he still hadn’t eaten anything, he wasn’t a very happy baby). Around 12:00 he was finally taken for his CT scan. One of the hardest things in the world is to hand your baby over to a nurse so he can be put to sleep. We were there by his side as he was waking up and I got to hold him and feed him while still in recovery. That night our doctor (Dr. Berlinski, the on-call pulmonologist) came and let us know the results of the CT. Judah was diagnosed with Neuroendocrine Hyperplasia of Infancy. We were told that it was “the best of the worst” but that doesn’t mend a broken heart, and when you’re told your baby has lung disease your heart really does break. We stayed at ACH for 4 more days until we could be set up with our oxygen concentrator and tanks at home. Before Judah was even discharged from the hospital Chance had to leave us to go to Tech School for the next 6 months. Judah came home on 1/2 LPM of oxygen and we quickly had to adjust to what our new normal was going to be.
So, fast forward 2 years….ups and downs, struggles, achievements, illnesses, good days, adjusting oxygen as needed, oral aversion, therapies, failure to thrive, G tube…sometimes it is hard to believe that it has only been 2 years that we have been dealing with this, it feels like a lifetime. Sometimes it’s hard to remember that there was ever some other way of life that we considered “normal”. Other days, it seems like 2 years has flown by. What is even crazier to say is that in those 2 years, Judah has now been off of daytime oxygen (except for during illness or teething) for 10 months! I had to count twice to make sure that was really how long it’s been! And he is up to 5 hours each night of sleep off of oxygen! When we were given his diagnosis we really couldn’t see a light at the end of the tunnel and we didn’t know what this meant for him in the long run. He will always have NEHI, but he is outgrowing the symptoms. It’s possible that he will always have trouble maintaining his oxygen levels when he is sick or going through a growth spurt…we just don’t know. There still has not been enough research to know if it is genetic or not, or what causes this. There are still a lot of unknowns for us.
But, what I do know… This kid is smart! He is funny and entertaining. He is so cute. He is kind. He is courageous. He is pretty amazing! We will probably never know (and have learned to accept that) why this was the path in life that was chosen for him. We are so lucky that things are looking up for him and his lungs are growing and becoming healthier! He maintains oxygen levels at 98%+ while awake and 96%+ while asleep on room air, and his respiratory rate is finally in the normal range. He really is doing great!
I have also come to know so many other “lung families” through our support group, and even though chILD and NEHI is rare, I feel so connected to others who are going through the same thing that we are. I have been able to help mentor other mommies and daddies with their own NEHI babies, and I hope to be an encouragement to them and others who are going through this. AND, I have been mentored by other lung mommas who have been dealing with this far longer than I have. I’ve “met” some really amazing people.
It’s not the life I would have chose for Judah, but we have learned to make the most of it. It is our normal. We will continue to rejoice in the victories and milestones he reaches, and we will push through the hard times. Judah is amazing and I wouldn’t trade him or this life for anything.
Happy 2 year NEHI “anniversary”, Baby Judah!
Judah’s appointment with the pulmonologist went well, and even fairly quick (always a plus since we’ve had 4-8 hour clinic visits before!). He said Judah looks great and can really see how he has just grown and thrived (really just since getting his G tube almost a year ago). His lungs are clear, no crackles or wheezing. Yay! 🙂 I’m thankful for a doctor who will take the time to answer all my questions!
We learned that pink eye and respiratory infections can, in fact, be related so when Judah had pink eye and had to go back on the oxygen back in December it was actually related. He said normally in otherwise healthy kids a respiratory infection that small wouldn’t even really be noticed, but because Judah doesn’t have any “reserve” it impacts him more.
We also confirmed (after learning from other moms in our lung group) that oil diffusing around him is a definite no no, and he also said that we shouldn’t even really be using wax warmers, plug ins, or anything of that nature.
He did agree with what we had learned from our pediatrician a couple months ago that Judah definitely wouldn’t be considered “failure to thrive” anymore. Great news (as a mom, it is heartbreaking to hear that your child is classified as failure to thrive).
He gave me some reading material….medical papers published about NEHI (I am constantly doing research!).
He agreed with our use of a probiotic to get Judah’s Candida in check. He was getting yeast diaper rashes pretty often, a sign of Candida overgrowth.
And finally, we are going to start the process of weaning Judah off of night time oxygen and see how he does! We will begin with an hour at a time and work our way up, but he is pleased to see that Judah has done so well on room air during the day. Judah isn’t quite ready to be off oxygen completely as we’ve had some nights where we actually have to increase from 1/8 to 1/4 LPM when he is in his deepest sleep, but this will start the process for when he is ready. 🙂
(The last picture…when you’re just ready to get home but you have to get your belly full! What do your kids eat in the car? Mine “eats” kale, quinoa, garbanzo beans, peas, carrots, and a whole bunch of other good-for-you food!)
We have had such a busy few days! Appointments in Little Rock on Wednesday and Thursday went well, overall…
G button follow up: The tube site looks good and is free from infection (after a course of antibiotics and special powder to draw out the infection for the past week). We will just continue to keep an eye on it and change the button regularly (every 6 months) which I can do at home.
Dentist: The dentist is not concerned with the two chipped teeth and said they should not be causing him any pain. He did confirm that Judah has a lip tie, but he does not want to put him under anesthesia just to fix that. If Judah ever has to go under anesthesia for any other procedure, then the dentist will fix the lip tie (going under anesthesia could negatively impact his lungs…the benefits do not outweigh the risks). He also confirmed a small tongue tie but not one big enough that would be the reason for Judah to not be able to chew and swallow solid foods. His main concern is that the back of the top four front teeth are demineralizing which he said is due to the acid reflux…stomach acid has just eaten away at them. He said it isn’t bad enough to fix them now but will want to continue to check periodically and may have to put caps on them. This dentist was wonderful in working with Judah…a kid who has an oral aversion and does not even like for me or Daddy to put things to his mouth. The dentist wasn’t phased one bit with the reaction Judah gave and actually sang him songs and comforted him as he was looking in his mouth.
Speech therapy: The therapist gave us lots of new ideas on introducing other textures of foods and talked about some behavior modification practices we can try to teach Judah that it is ok to swallow. This will just be on ongoing process. She also wants us to talk to our pediatrician about having Judah evaluated by an occupational therapist due to some sensory issues she feels he might have.
Pulmonary: This was a good appointment (long as usual…4 and a half hours!). The doctor was able to look at the scan that was taken in the ER last week from Judah’s G tube and see a clear image of the lungs. He said that Judah’s lungs look completely clear, free from the mosaic NEHI pattern that is usually present and no signs of air trapping. Of course that is not to say that he doesn’t have NEHI anymore, and there will be good days and bad, but he is optimistic that Judah has definitely shown improvement over the course of these 14 months since he was diagnosed. My homework assignment is to keep even more detailed notes when I check oxygen saturations, like noting how long of stretches of time Judah can tolerate being on room air before his saturations drop or his work of breathing increases. We are hopeful and thankful for imporvements, but we are also realists and know that there will be ups and downs before it is time to wean completely off the oxygen. He did also reiterate the rule from last year that we are to follow…NO CONTACT with anyone that has been sick within the past 2 weeks! At all! Respiratory illness could impact him enough to set him back to where he was at the beginning of all of this and erase all progress his little lungs have made. That means Judah and I are about to buckle down for the winter just like we did last year…only leaving the house every couple of weeks for appointments. I guess one plus about Chance being gone is that he will not be bringing home germs from work (I try to look on the bright side 😉!).
Thank you to everyone who reached out and offered support and thoughts for him…these multiple days of appointments just wear on us both.