Four Years!

Aidyn just turned four! 

The first decorated cake I made for Aidyn

On April 14^th we celebrated among family and enjoyed this special day. He has come so far! I am so glad we listened to our hearts and not what may have seemed logical to the doctors in Aidyn’s first days. 

Aidyn is a happy, bright preschooler who loves to play, learn and make new friends. He is very outgoing and greets just about anyone he passes by. The teachers and his peers really love him and miss him when he is sick at home. He makes doctor visits easy. He knows those visits are for his well being. He cooperates every time for the nurses when they need to take his vitals and measurements. The doctor’s love how easy it is for them to check his eyes, ears, nose and tummy. He says “ahh” before they ask and lifts his shirt for the exam. He stays still when they check his breathing and when they are listening to his heart. He answers all of their questions.  He is a model patient. Even when it time for labs, he barely moves when his arm is stuck with the needle. He watches closely as the blood moves from his arm to the tubes. When it’s all done he says, “See it didn't hurt, I didn't cry,” leaving the phlebotomists in awe and a smile, as he chatters away. One technician said he handles it a lot better than most adults. 

This is not the vision I had of Aidyn’s future four years ago. Because of the poor prognosis he was given and poor outlook on his life, I was expecting a child who would be dependent on machines and severely underdeveloped. Cognitively, Aidyn is probably beyond his years. He is in speech therapy for articulation issues, but he has come a long way since he’s been in school. He loves to talk. He is also in physical therapy once a week to make sure he is developed in all physical areas. He’s a tad behind because of his abdominal surgeries, but he is determined to do things on his own.

If this is your first time learning about Aidyn, for perspective, you could read about the details of Aidyn’s arrival and lack of faith all around us at the hospital when he fell ill there. In a nutshell, Aidyn was given a small percentage of hope to survive when he was just 4 days old. He had 90% - 95% of his small intestines removed and given a central line to nourish him directly through his veins using TPN. 

Five days old, one day after surgery, resting in the NICU.

We were given the option several times to let him go. It was not a possibility for us. And when we made it clear we wanted to keep the machines on, we were told Aidyn would have a low quality of life if he survived. Aidyn proved his initial doctors wrong and survived. He surpassed even our expectations, as we thought he would need a small intestine and liver transplant, but he has survived without it. Aidyn has been able to live a good quality life. He has his ups and downs, but he is a fighter and does it all. He has not been dependent on machines to nourish him since the age of two and he eats without a feeding tube (never had one, either). He defied all odds! I love saying that. He is our hero and teacher. We love him more each day.

Another blog entry coming very soon!

Summer in Fall

In Arizona it’s hard to believe Fall has started. Temperatures still top the high 90’s, and sometimes 100’s. Fortunately, the evenings and mornings are cooler. Aidyn has done very well keeping hydrated during the summer. I think what has been helpful is that I have weaned Aidyn off the antibiotics for bacterial overgrowth. It always gave him runny/watery stools the first half of the week long antibiotic cycle. This is how I weaned him: In June, I started to space out the antibiotics by adding a week to the time Aidyn had off of Flagyl (initially only one week). By August he was able to go three weeks off the Flagyl. He’s currently on his fourth week off. 

First ice cream!

Two weeks ago I started adding ¼ teaspoon of baking soda to every 20 oz of water Aidyn drinks. Any extra gas build up he usually got seemed to disappear. Aidyn now consistently has soft-formed stools. They are smelly, but without tons of gas and liquid. I find if I give a drop of oil of oregano for a couple days, it will kill the bacteria that the probiotics missed and the smell will lessen.

Big Boy bed!!

Aidyn received his first dose of B12 shots the beginning of September. He will continue to get them, with labs done every three months. His weight was checked and I was surprised Aidyn didn't gain weight. He has been hanging around 26 ½ lbs since the beginning of summer. I am hoping Aidyn will have gained some weight by Friday, the next GI appointment.

Much needed haircut done by Mama.

I have some exciting news! Aidyn has started Preschool! He goes half a day, four days a week. It has been over six weeks and it is going smoothly. He did catch a cold three weeks into school, but he recovered quickly. His speech has improved and it is probably the combination of Speech Therapy at school, his peers and just plain time. So far there have been no potty accidents at school. He has good control of his bowels and is pretty regular. Despite that, potty training is very slow. He just doesn't want to take the time to go to the bathroom. I am hoping by the time he is four he will be potty trained AND off the bottle. Drinking Elecare formula out of anything else just won’t do. I shouldn't complain because at least he will still drink it and he has no feeding tubes.

Ready for school.

Finger paint!

Recap on Aidyn: He has only 5 – 10% of his small intestines left, no ileocecal valve, no appendix and 50 – 75% of large intestines left as a result of NEC. He has been off TPN/Omegaven( IV nutrition) for 21 months and drinks Elecare for half his calories. He eats what he can tolerate which is most meats, gluten-free starches and is limited to carrots, apples, tomatoes, and strawberries for fruits/veggies. He is allergic to wheat, cow’s milk, beef and bananas. Each day he drinks about 24 oz of Elecare at 36 cal/oz and another 20 – 24 oz of water with electrolytes and sodium bicarbonate. Along with liquid vitamins, he takes 16000 units of Vitamin D3, 2800 mg of fish oil (when I remember), almost half a sachet of VSL#3, and Ursodiol every day. I give oil of oregano as needed. He recently weaned off Flagyl. He has 2 -3 bowel movements a day, soft formed. That’s it!

I always pray for the miracle that he wakes up one day with everything intact inside. That’s the kind of faith I have and it has gotten me through it all.

Timeline

As usual I am behind with updates, but will have one soon. In the meantime I have a snapshot of Aidyn life while he had a central line. I did this because a parent from a short bowel syndrome support group asked what the timeline looked like weaning off TPN. This is pretty detailed.


Aidyn was born at 34 weeks (4 lbs 12 oz) and got NEC at 3 days old.
* 3 days, 6 days old - 2 resections, jejunostomy, broviac, left with 15 - 18 cm small bowel and 50-75% of large bowel, no icv, no appendix
* 3 days old - TPN and Intralipids.
* 3 weeks old - started Lovenox because of a clot at tip of broviac
* 6 weeks old - went to Boston for eval, started intestinal rehab, switched to Omegaven (his liver was already bad by then), restarted feeds orally with breast milk (not put on transplant list to our surprise)
* 2 months old - Tpn reduced from 24 hr/day to 22 hr/day
* 3 months old - line replacement
* 5 months old - ostomy take down, successful reconnection, liver healing, started physical therapy 3 weeks after reconnection
* 6 months old - started Elecare (couldn't keep up with pumping)
* 7 months old - introduced solids, Cycled from 7 to 6 days/wk TPN/ 18 hr/day and hydration (saline) 1day/wk
* 8-10 months old ?? - Cycled from 6 to 5 days/wk TPN and 2 day/wk hydration 16 hr/day
* 9 months old - surgery to remove granulation from CVL site, 1st line infection/sepsis (Staphylococcus aureus)
* 11 months old - TPN/Omegaven/Saline reduced to 12 hr/day, 20 oz/day orally Elecare (20 al/oz)
* 13 months old - TPN/Omegaven down to 4 nights/wk, hydration(saline) 3 nights/wk, lots of solids and more oral fluids
* 15 months old - TPN/Omegaven down to 3 nights/wk, hydration 4 nights/wk, surgery for broviac replacement due to 8 month long granulation tissue problem, 2nd line infection/sepsis (staphylococcus aureus in new line!), hospitalized again for low creatinine levels most likely a reaction from Gentamicin and Unasyn combination, hospitalized a 3rd time in 3 weeks for another line infection (different bacteria)
* 18 months old - TPN/Omegaven the same, 2 nights hydration, 2 nights completely off, Elecare 30 cal/oz (from 27)
* 20 months old - blood in stool, allergy testing (allergic to wheat, cow's milk, beef), 4 nights off, 3 nights TPN/Omegaven, tested negative for cystic fibrosis but given pancreatic enzymes (Creon 6000) to absorb fat better
* 22 months old - TPN/Omegaven stopped, hydration as needed, peaches and bananas restricted (blood allergy test)
*23 months old - granulation around broviac again
*24 months old - Broviac removed!! 750 mls of Elecare a day. In addition, another 260 – 300 mls of fluids from ORS and non-dairy milks, Lovenox stopped

Aidyn was blessed enough to not have issues with vomiting, reflux or oral aversions. He also never did tube feedings, which is unusual for short bowel syndrome.

Two Years Old!

Here we are, two years later. We celebrated Aidyn's second birthday with a small family party on Thursday. Aidyn has been a delightful addition to our family the last two years and we all adore him. He tried cake for the first time. I scraped off the frosting from the gluten-free cake and he wasn’t sure what to think. After a taste, he decided it was alright. He was in Heaven after opening gifts. Trucks and cars, what more can a boy want?

Aidyn has only a few clear words we understand, but a lot of his own style. He does say “no, go, thank you, all done, where’d it go, Dada, car.” I’m still waiting for “Mommy.” He is expressive and we cannot wait until those mashed up sounds turn into words we understand. Not just because we are waiting for that developmental milestone, but because he so smart. He contemplates and studies quite a bit, so hearing his thoughts on this and that will be a trip. Alyssa thinks he looks like Einstein with his crazy hair, but maybe he's has the same intellect, too.

I can honestly say this past year has been easier to swallow than the first. Although Aidyn had been hospitalized six times, two of which were surgeries, we have come into our "normal" family life. Next week we have another Boston trip planned. We are hoping and praying the central line will be removed. Aidyn did have a dip in weight from February to March, but he did deal with severe diarrhea and dehydration after a bout of antibiotics. He bounced back fairly shortly after a few days of IV hydration supplementation (at home) and has been TPN free for two months. Aidyn has a tentative OR date with Dr. Puder, pending his weight has increased. His weight was 10.285 kg Feb 22nd but 10.075 kg on March 31st. I really feel the line can be taken out despite the weight loss. I think he just needs a chance to show he can gain weight without TPN, maybe I’m too optimistic. He is a bit skinny, but so was Alyssa (and she still is). He seems healthy by way of looks and his activity levels. I am working on ways to add fats and protein. Without dairy, it’s a challenge.

We did vitamin labs Friday to see if there may be something missing, causing the lack of weight gain. The results most likely won't be in by the time we are in Boston's clinic next week. There haven’t been labs done since February when we went to Boston, so hopefully we haven’t missed anything. There haven’t been any blood in recent stool tests and we've been staying away from beef, dairy, gluten, bananas and peaches (well, most fruits). Aidyn still eats a bunch and drinks up to 750 mls of Elecare a day. In addition, he drinks another 260 – 300 mls of fluids from ORS and dairy milks. 

I am so glad we have Children’s Hospital Boston to follow Aidyn. He was supposed to have an appointment Monday with the local GI doctor, but I was called yesterday to reschedule. It would have been nice to have the call earlier in the week, because maybe we would have got in later this week. She hasn’t seen Aidyn since February. I am the one who scheduled a weight check in March since Dr. Ursea had no clinic appointment set up with Aidyn. I just think if you have a patient who has just come off TPN, there would be an interest in how progress is being made. Even if Aidyn has the line come out and we visit Boston less, much less, I think they will still be the ones we will take advice from.

I will update after next week’s Boston visit. Happy Easter!

A Late Announcement

The wait is over. Aidyn is off TPN and Omegaven! We had our CAIR clinic appointment on February 10th and we were given the go ahead to stop all IV fluids. I was shocked! I didn’t realize it would be cold turkey. IV Hydration is only as needed. Aidyn has been doing so well and it’s been almost three weeks. Last weekend he needed fluids, but that’s for another post (explained below). His weight probably hasn’t changed much since his weigh-in in Boston. It was 10.42 kg, or 22.9 lbs. We actually had to wait an extra month to find out Aidyn would be off TPN. The original Boston appointment was set for January 13th, but all flights were cancelled due to the Nor’easter. We scheduled for two weeks later, but after a lot of thought, we cancelled so we wouldn’t end up being stuck in Boston. Finally, another two weeks later, the appointment was set. Boy was it cold! Aidyn got to see the snow. He loves the outdoors, even when it’s full of snow and cold. If you can believe it, the next week, while we were back home and attending the appointment with the local GI doctor, Boston was hit again with more snow.

Dr. Ursea expressed her happiness on how far Aidyn has come during our local appointment. She reminded me that Aidyn’s weight gain would be slow for now. The nutritionist said he only has to make up an extra 200 calories per day with the loss of TPN. Sounds easy, but when you have a toddler whose eating all day anyway, with a threshold for volume, it can be tricky. I have realized this new, wonderful change, this new leaf means another adventure. The learning and researching continues. This turn in the journey with Aidyn has made us happy and excited, yet it is a little scary. We will see how he does over the next month and if all goes well, Aidyn’s line will be pulled by Dr. Puder during our April visit! He’ll be two years old by then and ready for a new chapter.

Valentine's Day - The last day of TPN.

Long overdue update…June and July - edited: Now with pictures!

It seems we’ve been missing in action. I had this idea I would update this blog at least once a month. Trying to put that idea into fruition has been a challenge. Almost five months have passed which means there have been many new accomplishments, but not without a few setbacks.

The summer was busy. My older sister, Cheryl, and her family flew from Maryland and spent two weeks in Arizona vacationing and visiting family from Sierra Vista to Tucson to Chandler. It was a great visit. Everyone finally got to meet Aidyn. He charmed them all. At one point, my house was full of Aunt’s with their families and grandparents from both sides.

With Aunt Cheryl.

Playing with Aunt Jen.

Opening new toys with Grandpa.

By the end of June, Aidyn was walking. He thrives well on praise, so it didn’t take him long to master his new skill. If you can recall, Aidyn had an ostomy up until he was 5 months old. As an infant, Aidyn loved sleeping on his stomach. I was so fearful his ostomy bag would leak and get all over his central line dressing. I didn’t allow him to do tummy time and it resulted in a weak upper tone. He started receiving weekly physical therapy sessions in the home last November. At that time, he was 7 months old and still could not yet roll over to one side, another result of no tummy time. I wasn’t sure how physical therapy would help back then, but the therapist would note how tight his abdominal muscles were from his multiple surgeries and worked him through it. Now, he’s a fierce 18 month old toddler. Pushing anything he can get his hands on… dining chairs, boxes, toy trucks, laundry baskets…and his cherry on top is to ram any of those things in to the poor old dog (who is going deaf). The physical therapist had always been impressed with the quality of Aidyn’s developmental progressions. She could predict when he was about to hit a milestone and eventually he would actually achieve them sooner. He has pretty much caught up developmentally and that’s not calculating in his prematurity. He’s a force to be reckoned with, constantly on the move until nap or bedtime. He contemplates, studies, and plays hard; then he sleeps hard. Knowing that it can be difficult to keep up with him means he’s healthy and thriving well.

The month of July was a flash of activity. Some were good, but mostly tiring and stressful. My mother-in-law and daughter accompanied me and Aidyn to our trip to Boston. The CAIR appointment went very well. Aidyn was able to have another night of TPN reduced, leaving him with only three nights of TPN a week and the rest getting IV hydration. The next day, while I took Aidyn to surgery at Children’s Hospital to have his central line replaced, Alyssa and Eileen enjoyed a day at the Aquarium. They braved the mass transit system alone and did a fine job navigating Boston.

Aidyn had a successful surgery, and what was to be an overnight hospital stay turned out to be a day surgery. That evening you wouldn’t have guessed he’d had surgery. He was busying himself with Alyssa and the toys made available at the home of the host family where we stayed at.

Pre-surgery

Post-surgery

A little more playtime before leaving for the airport home.

Watching the planes before boarding.

The day after coming back home to Arizona, Aidyn was not acting himself. That night he was admitted into the hospital with a fever, was started on antibiotics and was diagnosed with having sepsis a couple days later. He had staphylococcus aureus in his blood stream and his new line! I was worried we would have to replace the line, but thankfully the cocktail of antibiotics given on the first night contained the right drug to beat the bacteria. After four days Aidyn was discharged and went home on 10 days of IV antibiotics, Unasyn to be exact. I suspect the granulation that grew out of control may have been the cause of the infection. Having surgery to replace the line could have made Aidyn vulnerable.

Phoenix Children's Hospital

Toward the end of Aidyn’s Unasyn dose, Aidyn started having new, strange symptoms. He was walking around like a drunken toddler. After talking to the nurse in Boston, we decided to have blood work done. Initially it was thought it could be unbalanced electrolytes, lactic acidosis or a reaction to meds, but instead his creatinine levels were abnormally elevated, indicating possible poor kidney function. Labs were repeated a couple days later and the levels remained elevated.

Waiting to be called back for labs.

Antibiotics were stopped and the local GI requested Aidyn be admitted into the hospital for a 24 hour evaluation. I was not pleased going back to the hospital one week after discharge. After a fiasco in trying to get tests done with uniformed ER doctors, Aidyn was discharged the next day and a follow up for the next week was made with the Nephrologist (kidney specialist). His conclusion was the two antibiotics given two weeks before may have caused Aidyn’s kidneys to sort of have an allergic reaction. So, in the future, we will not ever allow Aidyn to be given Gentamicin and Unasyn together. His creatinine levels did go back to normal. Don’t you love medicine and its side effects?

Just when we thought the drama was over, Aidyn spiked a fever later that night after seeing the Nephrologist earlier in the day. How…why…? It wasn’t expected at all. As a matter of fact, Aidyn was fine all day. He was left with the respite nurse that evening so Joseph and I could attend Alyssa’s meet the teacher night. We came home, relieved the nurse and finished putting Aidyn to bed. As I was finishing up in Aidyn’s room, I noticed he was grunting. When I picked him up he was so stiff, but curled up in a fetal position. He started shaking a bit and I thought he was having a seizure. He was able to make eye contact so we were very puzzled. After about ten minutes, Aidyn relaxed and fell asleep, but then, he suddenly spiked a 102 degree fever. As we all know, we prepared for a trip to the ER. By the time we got to the ER (a 25 minute drive, by the way) Aidyn had no fever. He was acting fine. I hesitated the use of antibiotics, fearing his creatinine levels would go up again and a very possible bacterial overgrowth situation that may result from his good bacteria being wiped out. Well, it turned out, Aidyn had another line infection. It was a different bacteria. We stayed in the hospital eight days this time. You can imagine the nurses were floored to see us a third time in three weeks.

At Phoenix Children's again, a visit with Dad.

Taking a stroll on hospital grounds, hoping to go home soon.

It is believed Aidyn may have been “showered” with bacteria in his blood at the start of is TPN infusion. It may have been hanging out in his line or the line may have been contaminated prior to the hookup of his infusion. We will never really know, but the respite nurse felt terrible. Although we feel we handle Aidyn’s line with great care, we work harder to keep it clean.

One Year Old!

Aidyn is officially a toddler! He turned one year old on April 14th. We had a little family party for him. He was a very good sport with the party hat. A couple of weeks later, we celebrated again with the grandparents, uncle and cousins.

According to his NICU doctors, Aidyn either would have died by now or would not be living a good quality of life. They couldn’t be more wrong. Aidyn has been a survivor from the beginning and has the quality of life any child could have under normal circumstances. It is most likely he will never need a small bowel transplant; he is just doing extremely well! Aidyn is cruising all over, gets into everything, and is fully interested in food and eating. He loves music and sways to the beat. Baby talk and singing are heard throughout the house. He is full of energy and is just a happy child. One day, this life of tubes, dressing changes and Lovenox injections will be a distant memory.

Being mischievous.

Wearing a buggy mask.

Silly.

Books are a big hit!

First Haircut by Mom. Not bad.

Enjoying a snack. Mmmmmm.

At Aidyn’s appointment with his AZ G.I. doctor last month, it was decided we keep Aidyn’s TPN the same so he could get over what had been bothering him. The Dr. finally diagnosed Aidyn’s tummy troubles (although we had been waiting to hear back on some stool studies done a couple weeks before). Just as I suspected, he had bacterial overgrowth. It’s possible picked up a virus while traveling to Boston the end of March. The signs: smelly and loud gas, gurgling stomach noises, weird breath, more loose dirty diapers and a dip in appetite. Bacterial overgrowth is common among people with short bowel syndrome especially those who have no ileocecal valve, like Aidyn. This valve is between the small and large intestines. It keeps the bacteria from the large intestine from backing up into the small intestine, where the body’s immune system is. In the case of bacterial overgrowth, it overtakes the beneficial bacteria in the small intestine. Typically, for a person with a normal gut, it can be kept it in check by adding probiotics to the diet, but it is recommended we not use probiotics for Aidyn until he has his central line removed. Even the good bacteria may possibly migrate into the blood stream, potentially causing a line infection.

Aidyn was prescribed the antibiotic Flagyl and symptoms improved right away. He did have a side effect, watery stools. After 4 days I stopped the antibiotic. Aidyn was clearly getting dehydrated. I called Dr. Ursea’s office and left a message for the nurse and in the meantime, I called Danielle, Dr. Puder’s nurse in Boston. She offered to help get more saline hydration bags sent for Aidyn but wanted to wait until Dr. Ursea’s office responded. The office did respond and agreed to stop the antibiotics, but didn’t address the hydration. During the call, I explained to the nurse my concern; I expected another call back about arranging for more saline. No call. I ended up emailing Danielle at 3am after Aidyn still had a dry diaper (this, after his 7th hour of TPN infusion). Danielle called first thing in the morning and told me she arranged for hydration bags to be sent with the home supply company. We all were definitely disappointed that the local G.I. office failed to help. After Joseph called Dr. Ursea’s office, we figured out there was a breakdown in communication between the staff and doctors. Dr. Ursea called me personally from her cell phone after Joseph called. She apologized and said she would do what she could to improve the way things were handled. Thank God for Children’s Hospital Boston. They have always been there for us and always come through for Aidyn.

A week from last Friday we returned from our CAIR appointment in Boston. As usual, Aidyn made the staff happy with his good weight gain, overall health and activity levels. He weighed 19 lbs 13 ounces and measured 72.6 cm. We talked about how Aidyn doesn’t like baby foods as much or baby cereals at all. We will continue to add more foods to his diet and also introducing milk solids to see if he tolerates it. They recommended Aidyn reduce his TPN to 4 nights a week at the same volume of 625ml 12 hours a night and Omegaven 4 nights a week increased to 90 mls. His IV fluids will be 3 days a week, lowered to 500 mls a night. The team feel he should consume more fluids with the drop in IV fluid volume and also eat more to make up for the drop in TPN.

Taking the train and a walk to Children's Hospital Boston.

As I said, Aidyn is very interested in what we eat. He’s eating diced pears, peas, diced carrots and green beans, rice bread, rice crackers, chicken, sweet potatoes, potatoes, rice and avocado. He loves banana, but doesn’t tolerate it well. Now that summer is around the corner, we have introduced pedialyte throughout the day. He still drinks from a bottle. Getting him to transition to a cup is challenging. We are trying sippy cups, but all he wants to do is bite them, as he is still teething. He is getting a top “molar” in, so there’s tons of gnawing and drooling.

Loving food.

We did have another visit with Dr. Ursea this past Tuesday. She was overly attentive, probably because of the communication mishap earlier in this month. The nutritionist and the doctor were hesitant to reduce Aidyn’s TPN since his weight gain was slightly under the goal, but they know Boston is managing Aidyn’s care and wrote the new orders anyway. I am glad they complied. If it appears Aidyn isn’t gaining enough weight, it’s easy to add the TPN night back in. Joseph and I are willing to go for it. The doctors here in AZ and in Boston wean TPN in different ways. In AZ, volume is reduced first, and then they remove TPN nights. In Boston, they remove TPN nights and replace with IV fluids, then wean totally from IV fluids. The goal for complete weaning of fluids is still set for the end of this fall. We’re staying positive.