Summer in Boston

Well, I thought I posted this entry, turns out it was waiting for pictures to be added. Here it is:

June 26th marked the 15^th time Aidyn had flown to Boston. He knows about everything when it comes to flying on a plane. He has traveling by air since he was 6 weeks old. Our family of four headed to Boston for another clinic appointment at Children's Hospital Boston. I am still very cautious about germs from the general public even though Aidyn doesn't have his central  line anymore. Each time we board a flight, I whip out my handy antibacterial wipes and wipe anything within arm’s reach.  Using the plane restroom is a task in itself for the average person, imagine bringing in your baby or toddler in, instructing him not to touch a thing so you can wipe all the surfaces before laying him down for a diaper change, or in the case now, getting him to hold on to you while he sits to do his thing during turbulence. Speaking of that, Aidyn did an impressive job at using the commode on the plane, TWICE on the way to Boston and once on the way back. He had dry pull-ups after each landing. 

This time around, we wanted to make a vacation out of the trip. We took advantage of all the miles we had and were able to pay for two tickets at $99 round trip each, one for free and the other at full price. We stayed in Burlington, a suburb of Boston and enjoyed the cooler weather Massachusetts had. It was nearly 100 degrees in Phoenix when we left and a nice, cool 67 degrees when we landed in Boston. It averaged about 85 degrees during the day, perfect. We arrived a couple nights before Aidyn’s appointment to relax and recover from the three hour jet lag. After a day at the Burlington Mall (where "Paul Blart: Mall Cop" was filmed), Aidyn had his first dip in a public pool, the hotel pool. He had fun going in and out of the cold pool, which surprised me because he never likes playing in the splash pads back at home .

Although long, Aidyn’s appointment with the CAIR team the next day went very well. He happily picked out a toy from the gift shop for having had to wait so long and for giving what a lot of blood for lab work. Everyone, the surgeon, nutritionist, GI specialist and nurse practitioner, all agreed that Aidyn was doing quite good. His weight was 27 lbs 12 oz (maybe a little inflated due to him having his clothes on) and it calculated just right for his height. They felt he was actually taking in enough calories for his growth and encourage us to keep doing what we are doing. We found out his Vitamin D results were 26.1 ng/mL and his B12 was 169 pg/mL. Both a bit low. We will continue to give the over the counter Vitamin D3 at 8000 iu/day and retest in a couple months. As for the B12, Aidyn will start B12 injections again.

Dr. Puder's Omegaven nurse came to see us while we waited for the CAIR team. Alexis chatted with us and with a sense of accomplishment she reminded me that Aidyn was one of the youngest babies to receive Omegaven there at Children’s Hospital. You can see the happiness in her face when she looked at Aidyn, seeing how big and healthy he got was remarkable. She let us know that Dr. Puder would love to see Aidyn and had him paged. We just love that man! He looks at Aidyn and without a second thought says he’s doing just fine. He chats with us about this and that, and then tells us of a conference in Phoenix that he will be attending early next year. He was hoping he could see us and we said we would definitely make it work. By then Aidyn will be almost 4 years old and in preschool.

Before coming back home to AZ, we spent a few days relaxing, going to the Museum of Science (which is amazing) and spent an afternoon at Revere Beach. 

It was a very nice trip to Massachusetts this summer. We got to spend a wonderful time together and came back with great reports for Aidyn. Aidyn is due back in another 6 months.

Summer is here!

I can’t believe summer is here already! It has started off very hot, so playtime outside is limited due to the high temperatures. By 10 am it is already 90 – 95 degrees. Aidyn had been increasing his fluid intake over the last few months.

Well, on April 14^th Aidyn turned three years old. He has come a long way in the last year. Shortly after his second birthday last year, he had his central line surgically removed by Dr. Puder a couple of hours before his CAIR appointment at Children’s Hospital Boston. I can’t tell you how happy we were to have that line removed. For the next 6 months or so, I kept hoping and praying Aidyn would not have to have one put back in. I am glad to say he is still line free. At the end of May Aidyn saw the GI. Aidyn weighed 12.2 kg, or 26.8 pounds, so he has gained four to five pounds since his line removal, but not without weight fluctuations. I worked very hard in finding the right balance of food and fluid intake to ensure he wouldn’t dump. In my mind, there was no point in allowing Aidyn to eat and drink absolutely any time he wanted if that meant he would lose possible nutrition in a dumping episode. Over time, Aidyn did tolerate feedings and fluids closer together. These days, I do allow him to eat and drink almost anytime he wants. He does haves a tendency to chug down all his fluids, so I control his drinking during meals, allowing ½ to 1 ounce increments between a few bites. It sort of forces him to sip. He still has popsicles that I make from the Ultima Replenisher electrolyte powder to help control his flow of fluid intake, too.

A trip to Sedona in January.

In the last few months Aidyn gained very little weight and it’s difficult to tell why. It could be because the Periactin he was taking was stopped and his appetite went down. Or, maybe he is burning more calories with the warmer weather. Or, it could be that I messed up his system when I didn’t give him his rotated antibiotics on time (a few days off), causing more liquid stools. The increase in drinking water? Maybe it is all of the above. Despite the lack of weight gain Aidyn has stayed healthy, maintained his weight and has grown in height. In January, he did catch a nasty cold after going to a preschool vision and hearing test. There were little children in and out all morning. He had fevers for a couple days and we managed it all at home without a doctor’s visit. He bounced back fairly well. His sister, Alyssa, and I ended up with the same thing, but we didn't recover as nicely as Aidyn did. 

Aidyn had been dealing with low Vitamin D levels for a year, and low B12 levels the past 6 months. The week of Thanksgiving Aidyn went from supplementing with 2000 units to 8000 units of Vitamin D2 a day. Levels were rechecked in January, but it went down to 17 from 21. The GI referred Aidyn to an Endocrinologist and it was suggested we try getting in at 10 – 15 minutes of sun three to four times a week. Labs at the beginning of April showed Aidyn’s Vitamin D went down to 14! It seemed obvious to the doctor Aidyn was just not absorbing the supplement. He suggested we increase to 16000 units a day. Labs at the end of May revealed Aidyn’s level went up to 28, still a little low, but much better. I believe the increase in Vitamin D supplementation along with weekly sun helped. We switched from D2 to D3 about a month ago. The insurance doesn’t cover the prescribed Vitamin D2 and it is very pricey. We will see what the over the counter D3 will do for Aidyn. 

Apples are Aidyn's favorite, but too much isn't good.

As for Vitamin B12, the Boston team started Aidyn on a series of three B12 injections (January, February and then March). Labs in April showed he was no longer low and the GI here in Phoenix decided to stop the treatment. Children’s Hospital Boston, on the other hand, felt it would be better to have Aidyn on a maintenance dose because of the portion of intestines Aidyn lost. It turned out a maintenance dose should have been in place as he is low again. The local GI wants to see what Boston thinks would be a good regimen once we go to the CAIR appointment the end of June.

 

I am happy to annouce that Aidyn will be attending preschool this fall! He aged out of the Early Intervention program and was evaluated for developmental preschool since he was premature and still has speech issues. He qualified for the preschool program based on speech alone. His articulation is still an issue and it was agreed that a school setting would be best for him. I can't wait to see the progress he will make once among his peers. He is such a bright child and always has a lot to say. It will be wonderful for him to speak where we all can completely understand him, or with out Mommy translating for him. I know it will make him feel better when he is better understood. Let's hope his immune system will be able to handle all the icky colds and such that he will be exposed to.

Oh, one last thing, my in-laws, Mike and Eileen Stephany sold their home over 200 miles away to be closer to us! We now have family close by and the kids will be able to see their grandparents a whole lot more. It really is nice having family just minutes away. In the couple of months they have been here, I have been able to catch up on three years of medical, dental and vet appointments that have been long over due. We are so grateful they can be of help to watch the kids, especially Aidyn.

Post Line Removal

A lot has happened over the last 5 1/2 months. Aidyn had his central line removed, lost nursing through the state, he had his first real cold, got a stomach bug, was hospitalized for dehydration, and started battling with weight fluctuations. It has been a rollercoaster ride, for sure.
Towards the end of April the whole family flew to Boston for Aidyn’s CAIR appointment. It was an extra special trip because it would be a turning point in Aidyn's care. It was the first time all four of us have traveled to Boston together. We stayed six nights this visit, five of which was with the wonderful family in Brookline. They always make us feel at home.


Flying to Boston

 Aidyn had a consult appointment with Dr. Puder the day after we arrived. Dr. Puder had not seen Aidyn since last September, so he was thrilled to see an active, happy boy playing in the hall upon his arrival. He took the time to go over Aidyn’s progress, weight fluctuations, future cares and of course, down to earth chat. Dr. Puder was happy to make the suggestion to have the central line removed. He checked out the granulation tissue growing out of the CVL site and told us the surgery would be easy. Dr. Puder was just as excited as we were to have the CVL removed. He said that typically, in a case like Aidyn’s, TPN would most likely continue for another year or two. It was a very enjoyable appointment.

We reminded Dr. Puder how grateful we were to have him in our lives. He remembered what we told him when we first contacted him over two years ago by recalling our story. How Aidyn’s initial doctors told us he most likely wouldn’t live past the first surgeries and if he did, that his quality of life would be poor. How transplant was the only option for survival. We were told several times we could remove life support and let Aidyn pass away in our arms. And when Aidyn fought hard and was nearing discharge, we were encouraged to put Aidyn in a hospice home for terminally ill children. Instead, after discovering Omegaven, we took Aidyn home and boarded a commercial flight to Boston five days later. And the rest is history. We were probably the most troublesome parents for those doctors where he was born; I hope they saw that we were the best patient advocates they had ever come across. And one day, hopefully soon, I will send an update letter to that hospital, directed to the NICU. They must know that each patient deserves any and all treatments that are available out there before giving up.

At the time of clinic in April, Aidyn weighed 10.2 kg, or 22.4 lbs. But before clinic, Dr. Puder removed the CVL and granulation tissue in surgery.

Pre-surgery, still a little tired, especially with the three hour time difference.

 We were excited that the time was near to be able to give Aidyn a real bath! Aidyn did well during and after surgery. Within 30 minutes of waking up we left the hospital for lunch across the street and headed to clinic after that. The team was happy to see how far Aidyn had come. There were smiles all around. The plan was to increase food and fluid intake and come back in two months to be sure Aidyn was progressing.

The next few days were spent going to the Children’s Museum, the Aquarium and and spending time with my brother and sister, and her family.

Children's Museum

The Aquarium

The day before leaving Boston was Easter Sunday. Our host family invited us to Easter service and Easter dinner. Their three children, two teenagers and preteen, even set up an Easter egg hunt for Alyssa and Aidyn. They had so much fun, especially Alyssa. The home we stayed in was built in the 1890’s and is three floors with a finished basement and finished attic. Alyssa loved going up stairs from the main floor to continue the egg hunt.

Easter with the Mills.

Our trip to Boston in April was an enjoyable one. It was a start of a new life without a central line for Aidyn, for all of us.

It was a beautiful Easter Sunday. We enjoyed a walk and nearby park.

The Revolving Doctor's Door

Within days of coming home from Boston in April, Aidyn’s nursing from the state was dropped. I didn’t even get a phone call, I found out from the nurse when I called to set up the next visit. She thought I had been notified and felt terrible. I thought we would get at least a couple weeks to adjust, but not so. It was only 16 hours a week, but it sure was a great help; I could run errands or just catch up on laundry. Then, a few days later, Aidyn spiked a fever. I didn’t know if it was related to having surgery or being exposed to everyone in the hospital or the airplane. His fever topped at 103 by the time we got to the doctor’s office. Since there were no symptoms at the time, we were instructed to use Motrin and Tylenol up to four days as long as there were no other major symptoms. By the weekend, he was having a runny nose and cough. I almost took him in to the hospital for dehydration, but he seemed to do better after some rest. Hydrating Aidyn was tricky, he wanted more than he should drink at one time, causing more stools. I started giving Aidyn electrolyte pops to slow down his intake, and it helped. We avoided the hospital. Seems Aidyn just had a cold. I quickly learned fevers can’t be taken lightly without having the option of giving IV fluids, so I have to be sure to give extra fluids at the first sign of them.  



 Five days after line removal.                         Getting over his cold.    

Aidyn saw his hematologist May 3rd and we got the go ahead to stop giving him Lovenox injections. He was on Lovenox because of a blood clot he had a couple weeks after birth and that could have posed a problem with his PFO, a hole between the two chambers of his heart. It usually closes before the baby is born. I had given him over 1000 Lovenox injections already and was so happy to stop giving them. He still has bruised thighs. She advised us to check with the cardiologist to see if he still has a PFO. I have yet to set up the appointment with the cardiologist. It will happen but I would like to take a break from seeing more doctors right now.

First real bath. He wasn't sure about the water being poured down his back for the first time.
Much different than a sponge bath. Bath time is so fun for him now!

Aidyn’s appointment with his local GI was set for the next day, but unfortunately we didn’t get to see her. While I thought we arrived on time, we were a half hour late. I'm not sure whose mistake it was, but I thought the doctor would have worked Aidyn in since she had not seen him in three months and he had many changes to his care. We contemplated on finding another GI as it wasn’t the first time we felt let down. In the end we opted to stick it out with her because she has all his history and has shown her cooperation with Boston.

Backyard fun, a walk, Mexican food and a snuggle for Mother's Day.

Aidyn finally had his two year check up with the pediatrician the second week of May. His weight dipped to 10.02 kg, or 22.09 lbs. According to the NP in Boston, it plotted him below the 3rd percentile. It was most likely from the extra stooling the week before. He caught up on his vaccines, except chicken pox. Two days after getting his vaccines, Aidyn started with low grade fevers and then major stooling. I made sure to give extra fluids, but it was not working. He became more focused on drinking than eating, he looked tired, his mouth was dry and he looked thin. Four days after the vaccines I decided to bring him to the nearest ER at John C. Lincoln. Normally we would have taken him to Phoenix Children's Hospital, which is a 40 - 45 minute drive, but this hospital was only 15 minutes away. I was nervous because they had no history on Aidyn. They did a wonderful job and made contact with Boston so everyone could be on the same page. We had never been in a hospital where all the rooms were made for one. It was quiet and with no residents. It's too bad they don't have a specialty in pediatric gastroenterology. If Aidyn still had his line we would have went to Phoenix Children's and never found out what a nice hospital John C. Lincoln was.

 

Getting much needed rest before being discharged home.



Looking thin.

Anyway, Aidyn's CO2 was at 12 and his weight went down again to 9.6 kg, just over 21 lbs. He was admitted for dehydration and spent the night getting fluids and resting his gut. He had a rough night and was very agitated. Eventually he fell asleep. The next day we spent most of my birthday waiting to see if Aidyn would be able to go home. Labs revealed his CO2 went up to 19, his BUN decreased to 6 from 30 (a high number indicates dehydration) and he was negative for the Rotavirus that was going around. He started feeds again and was discharged so he could continue getting better at home. His weight at discharge was 10 kg, 22 lbs. A few days later, stool cultures resulted in nothing. It’s assumed he caught some sort of virus.  Aidyn finally saw the GI later in the week and his weight went down to 9.85 kg, 21.7 lbs. His stooling wasn’t under control yet, so in an effort to help, she recommended Aidyn take Imodium AD. We talked about starting Flagyl again to help with what may be bacterial overgrowth, as well as start probiotics. We did the Imodium AD for just a couple days and Aidyn’s bowel movements went back to three to four a day. The doctor gave us samples of Florastor, a yeast based probiotic, to try. It seemed to help for a while, but I stopped giving it to Aidyn about a month ago, as it looked like he may have been getting thrush from it. He has since started Culturelle. We will most likely cycle Culturelle and Florastor every few months.

 

Two weeks after the appointment, Aidyn started increasing weight, again. He was 10.17 kg, 22.4 lbs, the first week of June. We continued to check his weight each week.

 

 

Back to his eating antics.

During the month of June Aidyn’s weight slowly increased. By the time we went to CAIR at the end of June, Aidyn weighed 10.4 kg, 22.9 lbs. Recommendations were made to start Flagyl based on gassy symptoms and an abdominal x-ray he had in Boston. It showed dilation in his small intestine and colon. It indicated colonized bacteria. We also stopped goat’s products since he has a milk allergy. His Elecare increased to 36 calories per ounce, up from 30 calories per ounce. I started using a sugar-free powdered electrolyte drink called Ultima Replenisher for Aidyn for hydration. I thought the sugar in the Pedialyte and even the home made ORS was not being well tolerated by him. Sugar and salt are needed to absorb electrolytes, but it in my mind the sugar was making Aidyn have looser stools. The dietician said it should be okay for him to use this product since Aidyn has most of his colon. It uses a non-GMO maltodextrin to help the electrolytes absorb, and it’s sweetened with Stevia leaf and Lo Han Guo fruit extracts. We tried Ceralyte and it works great since it is rice-based, but it’s expensive. Also, it doesn’t make good popsicles. With the Ultima I can make popsicles to slow down Aidyn’s intake. It doesn’t taste bad, especially cold. Overall, the trip to Boston was helpful. The next appointment didn’t have to be scheduled for another four to six months! We go again November 3rd.

I’ll end this post with more pictures!

 

 

A much needed haircut, very handsome.

More updates to come soon!


 

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.