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.
One year ago we received Judah’s NEHI diagnosis. We didn’t know much about it…as a matter of fact we didn’t know anything at all. We were told it was the “best of the worst” but for us it was still the worst. Our baby’s lungs aren’t healthy. There is still no answer as to whether this disease is genetic, and we are still learning the ins and outs of what it means to live with lung disease on a daily basis. We have gone through so much and continue to face new challenges.
There have been times that I personally struggle because, as a Mom, of course I would give anything for my baby to be healthy. I don’t air a lot of my own internal struggles because I feel like I have to be the strong one, Judah’s advocate. And most days that’s exactly what I am. Other days I get so upset that this is what we were given and that many others don’t understand what our life is like. I find myself getting sad or jealous or even angry over other mom’s Facebook posts who complain about things that seem so miniscule compared to what I feel like we go through…and even times when they are rejoicing over things like their little ones eating, because that has been such a hurdle for us. I know those feelings are something I need to overcome…I am working on it. I am thankful to have found the group of Lung Moms I have been able to connect with who do know these struggles because they have faced the exact same things themselves; they can encourage and offer guidance in times like this. Also, I’m thankful for a best friend who doesn’t judge me on these bad days, and most of all for a husband who can always stay (sometimes annoyingly) positive when I’m having a hard time.
Yesterday and today have been very emotional days for me. I feel like this anniversary is almost like another birthday of sorts for Judah. August 22, 2014 will always be a day that I will remember. I’ve been told that it gets easier, thank goodness.
The one thing that I can say without a doubt, is that I love Judah with all my heart and I know I have been beyond blessed by God to be able to be his Mommy. I questioned God’s will and felt abandoned by Him in the lowest times, when really I should have leaned more on Him. I may not know why this is the life we were given, but I do know that it was in God’s plan even before I was born that one day I would be given this son who would teach me so much. Judah truly is the happiest, strongest, most courageous baby. I know he will go on to do wonderful and amazing things in life. So for this one moment, I will let myself grieve over the life I had envisioned, for all that has happened in the last year…but then I will put on my big girl pants and continue to fight for his care, teach him new things, smile, and be the Momma God created me to be. And I will know Judah is being exactly who God wanted him to be!
Now let’s eat some cake and celebrate overcoming a year of struggles… Happy 1 Year NEHI Anniversary, Baby Judah!
This week marks the anniversary of Judah’s diagnosis with NEHI. These are the original posts we made from the hospital…
August 21, 2014:
Judah has been admitted to AR Children’s Hospital for the night based on his oxygen levels today. He has been on a monitor all day and awake he is measuring around 93%, asleep around 88% which means he is not getting enough oxygen. They took another chest x-ray today that still shows fluid on his little lungs. His breaths per minute are around 64 which is nearly double what it should be. He was given a sweat test to rule out cystic fibrosis which he did pass. All of his blood work came back fine which rules out any infection (they had thought possibly he had pneumonia) and they said they did not feel like it was blood accumulating in the lungs because he is not anemic. He was monitored by a speech therapist while he ate to see if it might be that he is aspirating milk as he drank but she did not see anything that would make it seem to be that. So, he is being monitored tonight and is on oxygen. Tomorrow morning he will have another echocardiogram and he will be under sedation for a CT scan. So until then we will just keep him occupied and comfortable and will hopefully get more answers tomorrow. He is such a little trooper….keep praying, Prayer Warriors. We need this baby, the cutest, most loving and happy and amazing baby, to be healthy. Thank you to everyone who has sent messages today. We appreciate all of your thoughts and the prayers you have said for us already.
August 22, 2014
It was a rough night for little man last night…was given an IV around 11 PM after being stuck 3 times for it. He has not been able to eat since midnight due to going under sedation for the CT scan which we are scheduled to go to at 11:00. He already had his echo this morning and it came back clear with his healthy heart which is still good news. We will not know anything else until the results of the CT come back and the doctors review it. It is a good possibility that we will be staying tonight also so they can monitor his oxygen levels without him being hooked up to the oxygen. So next we pray that he does well with the sedation and breathing tube for his CT scan. I know he is ready to eat; he should be able to right after the scan is finished. Even as hard as the night has been on him he still manages to share his sweet smile with new people that come into his room…he has an amazing little spirit. It was probably harder on me (and Chance) to hear his cries and not be able to do anything for him. Praying for some answers today.
Judah is in the CT now. We will not be able to go back to his recovery room until he is fully awake from the sedation. This is breaking my heart 😦
This little sweetheart was a champ today for his CT. Now we have to wait until tomorrow to know those results. We will stay tonight so they can monitor Judah’s breathing pattern without wearing the oxygen. Should know more tomorrow morning.
Judah has received his final diagnosis tonight…luckily the doctor was nice enough to come tell us tonight instead of making us wait until the morning. SO… Judah has been diagnosed with Neuroendocrine Hyperplasia of Infancy (NEHI) which is a form of Child Interstitial Lung Disease. The good news is that of all the options of lung problems that the doctors had in mind, this is the best. It is something that he could grow out of but for now he is going to be on an oxygen machine all the time and a pulse-ox monitor at night. There is no telling how long he will be on the oxygen; he will have to be on it until his lungs grow and develop enough to be able to absorb the oxygen at 100%. It could be months or years, there is no telling. He can not be exposed to large crowds and abundance of germs as he is more susceptible to respiratory infections and can not be around anyone who is sick. We praise God that it is not worse, but still ask for prayer for the healing of Judah’s lungs, for myself as I will become primary caregiver and learn how to do all of this on a regular basis since Chance will still have to leave to go to Tech School in Texas on Monday, and of course for Chance since he has to be away from us for the next 5 months. Thank you to everyone for your prayers and support through this and thank you for your continued prayers. For Chance and myself this baby boy is our whole world and we want nothing more than for him to lead a normal childhood, so we will pray for healing every day and learn that God’s plan is bigger than ours. We will not be discharged from AR Children’s Hospital until Monday when they can get us set up with an oxygen machine.
More info on NEHI:
We had a good quick visit today (August 17th) to the surgery clinic for Judah to get his first button changed. And guess what?! This Momma changed it! Yup…I learned how to take out the old button and put the new one in myself!! This smiling baby picture was right after I did it so I guess I did a pretty good job 😉 he still got to pick out a new Minnie Mouse to go along with his Mickey that we got during his hospital stay. And bonus…we got a whole box of slip tip syringes…it’s like Christmas! 😛 That’s one thing I can say for sure…I’ve learned more medical stuff since having Judah than I ever thought I would in my life!
Thank goodness….another good appointment today (August 18) with the GI doctor and a new nutritionist, and they are completely on our side about getting Judah on a real food diet!!! They agree that it’s important for his body to be getting real food through the tube opposed to formula and also agree that our ultimate goal is to get him eating by mouth so he doesn’t have to fully rely on the tube for nutrition. Now we just have to work with our DME provider to get insurance to cover the Liquid Hope (real food for G tubes)…fingers crossed it goes smoothly. We also talked about making slow transitions to get Judah off of the Prevacid and test out his tolerance for dairy. I’m excited that all our research has paid off, that we have a new GI doctor and nutritionist who don’t just want to push man-made formula and listened to us completely, and a renewed sense that we are doing our best as parents to provide the best care to our little guy!