Life with a boy and his Hypoplastic Left heart Syndrome, now Heart transplant and partial Liver from Aspergillius. Also, a girl and her Tethered Spinal Cord Syndrome, Neuro Endocrine Cell Hypoplasia, Lumbar Hemangioma, asymmetrical gluteal cleft, Pectus Carinatum and two Super Sisters and one yummy baby brother.
So there was a couple of reasons to break down and get family photos... 1. It's been way to long since I have had them done. 2. After Jenivee(my little niece) passed away, I was so sad to hear that there wasn't any pictures of their little family together other than when she was very sick... Time just wasn't my friend in that situation. We have many wonderful pictures of her and are happy for those. But it did make me want to make sure I have my kids in pictures with me. It's tangible that way for me. 3. Jeff needed something nice for his new office. I purchased a photo package at our youth auction for a steal of a deal. And here are the results...
These were taken at the SL library!
I couldn't find what I wanted for the little girls, so I scoured Pintrest till I came close and sewed these up!
There really isn't much NEHI information. Or many blogs that are recent. Most of the blogs are 2-3 years old. Rare lung disease... Crazy hemangioma... Tethered Spinal cord... Yet this almost nine month old is rocking it. There are some pretty upsetting times, like when the connectors wedge underneath the piano or the couch and her cheeks are pulled back like she has her head out the window and the wind is blowing like crazy... She doesn't think that is funny at all. Or when her brother/sisters don't watch very good and trip on her tubes. Or when the random lady at the store notices her canula is out of her nose and try's to help me put it back, but doesn't know we need to do it a special way. Those are few and far between. The rest of the time we play on the grass, discovering leafs, tasting leafs, getting tickled with things and learning the signs for tree, airplane, grass, and bird.
I started thinking of ways to keep her healthy... I may even break down and buy an oil defuser for the house. I really should buy three.. One for each of Alex's classrooms and one for home. We are headed to get our flu shots this week. I am stocking up on Clorox wipes.. I really want those Norwich clothes... I might add them to my Christmas list:) I also need to find a pattern for a cart cover..
We can do this!!! She will most likely recieve Synigis. Those are a pretty penny. But worth it. Now, if we can just keep her untangled for more than a few minutes.
We got up bright and early for the 5k fundraiser for school. I was only planning on doing one mile but Gator was my half heart runner today and he wanted to do the whole thing. Granted, he also rode on backs, took lots of breaks, and rode in the stroller a little bit. But, he made it. He ran a good portion too. I never thought we would see a day when his heart would let him do things like this. I am grateful everyday. It is amazing to me that he has so much life inside him and goes until he can't. Then takes a break... Then takes off again... He might need a nap later. Munchie and the Squishster rode in the stroller and we were really close to being the last... But we made it:)
Daddy had to run and carry Gator part way. Then swap and take a turn with the girls.. And he encouraged Ry who was off with friends so I couldn't get a photo of her.
I googled three things today.. Fruit leather, More NEHI stuff and Doterra information. I am a nut about trying to do things myself and learn about my kids and how to best help them. I also get to FB, email, and play games.. Internet is so awesome!
Hand me downs-
So after going through all of the kids clothes I realized something.. Eden had very little to wear and May is opposite seasons. So today I went through the hand me downs to see if there was much the little girls could fit into. It turns out that because of other friends generosity both girls have quite a bit. We will need to fill the cracks here and there but at least I don't have to purchase wardrobes. I love hand me downs. If and when we can close the baby chapter in our lives... I have so many baby clothes to pass on...
I am grateful for people who will take the time to teach something to my kids. This is kind of hard to be pulled in so many directions each night, but we are loving the workouts. We are loving the chance to try new things. It is challenging some nights. Tonight Ry got emotional about one of her piano songs. But she worked it out. There will certainly be challenges. It won't always be easy.
Saturday was Gators first practice.. Daddy was going for a ride but felt like he needed to come with us. And a good thing because the battery died in my van at the gas station. I really needed his muscles and car skills.. We got it switched and then headed over to practice. Gator has an awesome little arm. We figure if he loves this then we will find a way for him to participate with his health issues. So he looks like a normal little boy playing sports. Way to go buddy!!!
My little girl is changing into a beautiful young lady. She doesn't want to be lumped with littles anymore she wants to be a tween... Aahhhh that means friend stress and ideas about how things should go. She is managing it all so great. Her big day landed on a Sunday. It is also a family year so we didn't need to worry about coordinating a party. Just spending the day with her.
She got roller blades, clothes and pretty much spoiled by everyone.
She is the new roller derby queen.
We went to gramma and papa's for dinner..
She wanted high heal cupcakes..
She loved every minute of her day and wished it could go on and on.
MayLee has been on continuous oxygen since her cord release and it has stumped us so far. After doing the echo, swallow study, and Zpac with no changes, we finally felt like we needed to go ahead with a CT scan. It was surprising how quickly things were put together and ready. I talked with Doc U on Friday and had it all set up for Monday. He was wonderful to work with and doesn't do the usual hurry up to wait. We are really blessed because our Pediatrician Doc Mashkuri, cardiologist- Doc Su, and now Pulmonologist Doc Uchida are pretty much the most amazing doctors who work well with me and understand when to be delicate and when to be straight. I really really like them. They are on my Christmas card list.
So Monday came and bright and early we headed up to the hotel on the hill (PCMC main campus). We checked in, then played while we waited. This was a conscious sedation so three pokes for an IV and a little Versed later and we were ready. The whole procedure took like ten minutes or less. We went over to recovery. I forgot my ped wanted a set of labs so I had to wait around a little for orders to be faxed in and found. We always get the nicest nurses and they took care of us while we waited.
After that I ran up to see a cute little heart friend waiting for an angel heart. Then went the the RMHFR to nurse May.
I ran to daddies shop to pick up Eden who spent the morning with dad.. What a treat!!!!!
We heard later that night from Dr. M. She explained what she suspected but didn't want to over step. She deals with the child as a whole. So she wanted to let the specialist explain things in depth. While it stressed me out a little I didn't run to google. She advised against that. She did tell me that we need to run a few more labs that were missed. Also May is slightly anemic. A little iron supplement should fix that.
This morning Dr. U called and I missed it. He called back when I was getting Gators meds at Target. He explained that May has a rare lung disorder called Neuro endocrine cell Hypoplasia in infancy... Ready for the acronym.... NEHI.... New to the medical world in the last 7 years... Basically it's a name for what they see... And because its new and rather rare, there are many unknowns... Like is it congenital or genetic. How does it come about.. Long term outcomes of patients with NEHI...
Armed with a little more info I now looked it up... Here is the best article.... Background NEHI is a disorder of the lungs in children and is classified within the group of children’s interstitial lung diseases (chILD). This disorder is relatively rare and was classified and described in 2005. As a result, it is difficult to estimate the number of children with this disorder in the US. Symptoms Children with NEHI often have rapid and difficult breathing, low levels of oxygen in the blood (hypoxemia), and crackles are heard on examination with a stethoscope. Wheezing, although not characteristic, may also occur. Children may be initially diagnosed with asthma or prolonged respiratory infections as the symptoms may overlap with these. However, children with NEHI generally do not respond to asthma treatments and corticosteroids. What causes NEHI? The cause of NEHI is poorly understood at this point in time. Although we recognize the increased number of PNECs in NEHI, the mechanism by which they are involved remains unclear. NEHI has been found to run in some families so it suggests there is some genetic basis for this disorder. However, a gene abnormality has not been identified. Environmental causes may also influence the development of NEHI, but much more research must be done to answer these questions. Diagnosis When any form of chILD is suspected, there are several tests that are commonly done to help with the diagnosis. Lab work to rule out other causes of these symptoms, such as cystic fibrosis or immunodeficiency, is often performed. A bronchoscopy with bronchoalveolar lavage (BAL) is often performed which can look for infection, inflammation, and signs of aspiration into the lungs.A high-resolution computed tomography (CT) scan of the lungs is often useful in the diagnosis of NEHI, showing a characteristic pattern called ground glass opacities. The lungs also show areas that are inflated to different extents, with some areas being overinflated and some underinflated, creating a mosaic pat- tern on CT. An Infant Pulmonary Function Test (infant PFT) has become more important in diagnosing NEHI. This test is not always used, because it takes specialized equipment that is not always available to the clinician. These usually show trapping of air in the lungs in NEHI patients. If all of these tests are characteristic of NEHI, the child may receive a diagnosis of NEHI Syndrome without a biopsy. However, if any of the results or symptoms are not typical, the only way to conclusively confirm the NEHI diagonosis is through a lung biopsy. The biopsy tissue typically has little or no inflammation and when stained with a particular bombesin stain, demonstrates an abnormally increased number of pulmonary endocrine cells (PNECs) within the small airways. PNECs are cells that are usually present in the lining of the airways, alone or in clusters called neuroepithelial bodies (NEBs), and are thought to be involved in lung development. In NEHI, the number of PNECs and NEBs in the airways is always significantly increased. Treatment The treatment for NEHI is mainly supportive. NEHI children often have labored breathing, which uses more calories than normal and can be accompanied by gastroesophageal reflux (GERD). As a result, poor weight gain (failure to thrive) is often seen with NEHI. Optimizing the child’s nutritional status to promote adequate growth is important for overall health. Oxygen supplementation may also be required. The amount of oxygen needed varies in NEHI patients. Some need oxygen 24 hours a day, while others will only wear it at night and during illness, while some do not require it at all. Most NEHI patients decrease their need for oxygen over time and most eventually grow out of the need for supplementation. Common colds and flu can be more severe in NEHI patients, so limiting exposure to respiratory infections is also important. Seasonal flu shots and prevention of Respiratory Syncytial Virus (RSV) is recommended. Oral corticosteroids, which are used to decrease inflammation in other lung disorders, have not been shown to be helpful with the symptoms in most NEHI patients. This is consistent with the limited inflammation seen on lung biopsy. Prognosis There is currently limited information on the long-term prognosis, as NEHI has only been recently recognized. However, the symptoms of NEHI usually decrease over time and are not progressive in nature. No death secondary to NEHI has been reported so far.- See more at: http://www.child-foundation.com/education/NEHI#sthash.Xe2GDifM.dpuf Now, are you wondering if this related to her Tethered Cord syndrome or her Hemangioma??? Probably not. Since this has nothing to with anything skin or spine.... Yeah she just has a jumble of unrelated issues. There is probably another case of the three together out there somewhere... Maybe she is the only one and they will name it something like... LTH or Mays syndrome or something like that....
Are you also wondering what it will be like for us with this news???? Well, it means we don't get to ditch the half child all that soon. And we most likely will need a couple more tests. Doc U is sending her CT readings to his buddies in Denver. They will help us determine without an evasive lung biopsy what we suspect. She will need a sweat test to rule out Cystic Fibrosis. But, nothing we can't manage. My eyes leaked a little in front of the Target pharmacist. Not my best moment... But, I hiked up my big girl panties and took a deep breath and it's really going to be okay.
Mostly I know Heavenly Father comforts us all and The Still Small Voice calmed my fears and I am able to see the bright side of this. Hey I get to have super strong muscles.... Right??? Right??? I get to expand on my ever growing understanding of our amazing bodies and not take things for granted. Our insides really are amazing. Everything is perfectly wired, and has big jobs to do. Down to the smallest cells. WOW!!!! We really put emphasis on the outside... but the inside is way better.
Don't feel bad for us or think I am something super... (remember eyes leaking at store) I am just a lady, who happens to be a momma of some kiddles who need a little extra medical support. I don't have any special training except I know a trick. The creator of us all knows. If I need help, there is answers... What I lack in faith, I borrow. So thanks... Besides... I will do anything I need to for my babies and you would too.
In Other News....
She stands.... Still on her toes most of the time, but pulling to a stand is huge.
Naughty cause she isn't that strong...
To end we just want to say... When life gives you Lemons... Wear this dang cute shirt and be happy happy happy!
Check out the shape of Gators scab.. Did that on his bike... Crazy huh???
My brothers will die of embarrassment because I am going to talk about how awesome they are and they just like to do what my mom calls a "love tap" and give a slug in the arm. My sisters should be okay with me talking about them in a lovey way. I think I am spoiled because family is pretty amazing and I married into a pretty amazing family too. Each of the sibs and in-laws are so kind to me and I am thankful for them. They put up with my crazy... They are such great husbands, wives, missionaries, parents, and helpers. My grandparents, aunties, uncles and cousins rock too.
I have another couple of families I belong to.
My ward family is so amazing. They take care of us when immediate family can't...they stand by us in our day to day lives and they are those who are my village with my kiddles. Every need is filled, every question (even at 3 in the morning... Right Gee's?) answered!!! Every other issue taken care of. Even if they now live forever away. (Missing the Englands)
My heart family-
It is an honor to stand with the families whose kids have heart defects. To have these people in my life is such a blessing. I am one of the luckiest girls in the world to know individuals who have seen hardship yet choose joy and offer to lift others. I am so grateful for them all. This group has brought me lasting friendships with people who I consider my extended family.
Man I am a slacker... I should be up to at least 14 grateful posts by now... I guess if you take into account that I put more than one thing on each of the other posts I might be somewhat current.
So very far from what I think it should be. I am so very thankful for this gift. I am thankful it has given me Eight pregnancies, four of which resulted in my little herd. I am thankful for my wacky curly hair, it is fun to have something bouncy. I am thankful for good eyes and teeth. I am thankful for strong arms to carry my babies, oxygen, and a car load of groceries. I am thankful for my feet, my toes make me happy cause they look nice all sparkled up and they are kinda square like Flinstones feet. I am thankful for all the "extras" because that means my body grew humans and nourished them and that is a big deal. I am so thankful for things inside me... Man, I thought I knew something about my insides... I have learned more medical jargon and anatomy in the past 6 years than I ever did in my classes in high school. So my heart and spine are obvious... Now my lungs too... So amazing the jobs these things do. I am thankful for my voice.. I am a shower diva, I love to sing.. If I have to do it in public I throw up. I have the worst stage fright ever. I am thankful for it just the same because jamming out with my kids is just as cool as singing in front of a million people. One day I might get over it.(probably not) it does make me grateful for others voices!!! I love to be sung to! I am thankful my mouth in general. All I can say is "pathway to my tummy", and best smoocher reciever from my boyfriend/hubby and kiddles!!! Like I said before, I know that there are areas of improvement. But I am so grateful for this one I got!
For Labor day we decided a little family/friend hike was in order....
It was a bit of a trick as always to haul the oxygen. Daddy made it look easy... We even ran into another baby on O's up there.
By the way.... The description online said this was more of a stroll. pffff.... We even had one of our party fall and need stitches... It was more than a stroll. There was also a momma moose and her calf up there. A beautiful sight for sure.
This is quite the load....
We had some great friends that came. They helped with all the little people...
Hiking can sure wear a person out...
Ry made Repunzel hair.
This awesome selfie is proof that I was there.
Daddy is good looking!!! Hey you tall drink of water... Wanna stand by me????