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.

New Goals

Our goal for Aidyn is to increase his oral fluid intake and calorie consumption. We must make up for what Aidyn would have been getting from TPN and lipids. I am searching for calorie dense foods he can tolerate. As was mentioned in a previous post, Aidyn had both the skin prick and skin patch allergy testing done and was found to be sensitive to beef, cow’s milk and possibly wheat. A recent RAST test was done and I got an unofficial, over the phone result read by the nurse. It appears Aidyn may have sensitivities to peaches and bananas, too. Giving up beef is easy, but finding alternatives to cow’s milk and wheat that contain a lot of calories is difficult. Of course, Aidyn gets most of his calories from Elecare, an elemental formula. He is easily taking 21 – 23 ounces of that daily. Sometimes he may have a few ounces of goat’s milk or coconut milk, too. Here’s an example a typical day of eating, which includes supplements, vitamins and meds:

730 am – 120 - 150 mls (4 -5 oz)of Elecare, fish oil (1g of DHA/EPA), Ursodiol
930 am – breakfast: goat cheese, corn tortilla, avocado, ¼ - ½ tsp coconut oil, vitamins
12:30 pm – 120 mls Elecare
3:30 pm – 120 – 150 mls Elecare, 1 g fish oil
6:00 pm - dinner: rice with Braggs amino acids (like soy sauce), chicken sausage, toddler carrots, maybe fish oil
8:30 pm – 120 mls Elecare, ¼ - ½ tsp coconut oil, Ursodiol, Lovenox injection
11:30 pm – 150 mls Elecare

Aidyn also eats turkey, pork, deli meats, eggs, tomato or spaghetti sauce on pasta or rice and peas. He snacks in between with foods like: pasta, pretzels, potato chips, cookies, goat cheese, bread and tortillas. We stay away from most fruits and veggies because of the sugar and fiber contents. He does have a little applesauce with his enzymes, though. He eats gluten free foods, just like his sister (she was unofficially diagnosed gluten sensitive when she was two). I use Smart Balance original Light “butter” since it’s non-dairy and coconut oil in cooking. Before each Elecare drink, meal or snacks with protein or high fat, he is given Creon 6000, a pancreatic enzyme. He also drinks at least 8 ounces of oral rehydration solutions (ORS) in between. I do not use Pedialyte for ORS because it seems Aidyn doesn’t tolerate it well. I think it may be the sugar. Instead, I make his ORS from a recipe I found on the internet. It uses rice cereal in place of sugar. The recipe is as follows:

• 1/2 cup dry, precooked baby rice cereal
• 2 cups water
• 1/2 teaspoon salt

I combine the ingredients in a blender until well dissolved and smooth. It’s thick, but drinkable. It is refrigerated and thrown out after 24 hours. I think it works better for Aidyn since the rice absorbs slower than straight sugar.

I have been attending a monthly webinar series in nutrition for short bowel syndrome called Maximize Health, through Aidyn’s medical supply company. ThriveRx is great because they aren’t just a pharmacy providing medicines and supplies, but a company that provide educational nutritional support with the help of their pharmacists, nurses and dietitians. The series has been helpful in knowing what percentage of proteins, fats and carbohydrates belong in his diet.

Right now, the challenge is trying to separate Aidyn’s drinking from eating. He tends to want more to drink the later part of the day. I spread Aidyn’s drinking by offering one or two ounces at a time and distracting him with playing, books or walks (or whatever). Of course, that’s not always possible and he will drink a large amount and then “dump” a half hour later. I do offer him drinks when he’s not asking for it in an attempt to hydrate him before he’s thirsty. It will be easier as he gets older to make him understand that it’s best he drinks his fluids more slowly, especially when he can communicate to me better.

This video is from back in November, when he was trying Goat cheese for the first time. He ended up eating quite a bit after this video and it's one of his favorite foods.

A Little Hiccup

A little over a week ago, I brought Aidyn to the surgeon’s office to have the stitches removed that we’re still firmly in place around his broviac. The hefty, knotted end of the stitches was cutting into his skin and the skin started weeping. As a result, granulation tissue started growing around his line. Sounds like a repeat of last year. Anyway, the surgeon prescribed Aidyn Keflex since the site looked infected. He said to use it for two days and call back in three to four days to see if the wound culture sample he took grows anything out. So we started the meds a week from last Wednesday and that Friday. In the meantime, Aidyn started showing signs of bacterial overgrowth over the weekend resulting in a lot of stool volume and getting dehydrated. I had to use IV fluids for the first time in 19 days since being off TPN. Monday, I was instructed to continue using the antibiotic since the wound culture came back positive with some staph cocci gram positive bacteria growing on it. The site looks better now, but the tissue is still growing.

Aidyn really had a hard time since he was stooling so much and then getting lots of gas once we restarted the antibiotics. He lost his appetite for a couple days and he couldn’t take the Elecare full strength at first. I am sure it’s because he was dealing with bacteria die off from the Keflex killing off the overgrowth in his gut. He is back to normal now, but we will see what happens in the next few days since he had his last dose of antibiotics yesterday morning. With this experience, it shows us what we may have to deal with when Aidyn gets sick and looses fluid without a central line. We may just have to hospitalize him for fluids in the future.

Dehydrated and off track.

When we had our Boston visit last month, I asked the resident surgeon on staff that day if he could remove the stitches. At that time, Aidyn had layers of Iodine covering the exit site on the line. Stella, our respite nurse, and I had been slowly getting those layers of Iodine off the line each week during dressing changes using hydrogen peroxide. With doing so the ends of the stitches around the line were able to move more freely.

Taken 1/07/11, before the we dissolved the iodine and stitched came off.

Anyway, the resident surgeon did not feel comfortable removing the stitches, but he did shorten a couple ends. Looking back, I think if the stitches were removed when I requested it, Aidyn probably wouldn’t have had the granulation tissue growing. Here I thought removing the layers of iodine from the broviac site would make it better, but really, it made things worse. I just have to leave those thoughts alone now. There’s a reason it happened this way, I just don’t know yet. Hopefully it will not become a problem anymore and that we can wait until next month when we go back to Boston to deal with the line. Hopefully it will come out.

Back to himself.

Update Part 2, 2010

The second half of August finally greeted us with some relief from prior hospitalizations and antibiotic rituals at home. Surprisingly, Aidyn did not end up with bacterial overgrowth as he did after his bout with antibiotics back in April. I can only credit adding coconut oil to his diet.

You see, after Aidyn’s treatment for his line infection in July, I became suspicious about possible bacterial overgrowth symptoms. There were increased stools, frothy at times and very bad gas. I didn’t want to put him on more antibiotics. I figured he would have to be cycled on and off once he was started on antibiotics again. We didn’t want that, so I did a little research. I found several natural things that have claimed to help with bacteria, such as grapefruit seed extract (GSE), garlic and coconut oil. The GSE could cause bleeding in those taking Warfarin, a blood thinner. Aidyn isn’t on that, but is on another blood thinner medication, Lovenox. I decided we should stay away from GSE. I tried garlic drops for a couple days and it seemed to help, but I read it may possibly wipe out all the beneficial bacteria, too. I stopped the garlic and started adding coconut oil to Aidyn’s diet. It appeared to stop all overgrowth symptoms. I was very amazed. So, since July, Aidyn has been taking about ¼ to½ teaspoon of coconut oil a day. There has not been one episode of bacterial overgrowth. Since we all like the taste of coconut, I have been baking with it. It’s great in pancakes, cakes, brownies and such. I buy unrefined extra virgin coconut oil. I do use it in regular cooking, but using the refined oil is better tasting since the coconut flavor is mild to none. The dietician and nurse practitioner in Boston are very interested in Aidyn’s use of coconut oil. They have even asked what brand we use and where I buy it.

In September we traveled to Boston for our routine visit. Aidyn gained some weight and maintained it. The team was happy with that since he had been in and out of the hospital in July and August. But since he didn’t gain tremendous weight, the team thought it was best to keep the TPN at 3 nights per week and try for two nights off everything. That was great news. Also, his Elecare concentration went from 27 calories per ounce to 30 calories per ounce. It took him a couple of weeks to adjust. His appetite increased and he started eating more frequently, but taking in less formula volume than before. He always knows what his limits are and I try to respect that.

It was a long day at clinic.

Boston is beautiful in the Fall.

Right after returning from our September visit in Boston, we moved. Finally, we headed north of Phoenix to ease Joseph’s commute. He went from driving 1 – 1 ½ hours each way (6 days a week) to 15 minutes each way. We love our new neighborhood and Alyssa loves her new school. The transition was great for all. Alyssa and Aidyn go to the park around the corner almost daily and Joseph can spend more time at home with us, not to mention if Aidyn funs a fever, Joseph can be home quickly so we can organize going to the hospital (knock on wood, he hasn’t been there since summer). We were sad to say goodbye to Roxanne, Aidyn's respite nurse, who was like a second grandmother to Aidyn. Luckily, Aidyn took to Stella, his new nurse, very well. She helps me with CVL dressing changes and general work that revolves around Aidyn (washing bottles, preparing formula, laundry, and of course, Aidyn, himself). I really utilize the time to catch up on much needed housework and grocery errands. I still haven’t had the time to volunteer at Alyssa’s school, as I used to do before Aidyn came along. I was hoping the break from Aidyn’s cares would allow me that and maybe it will soon.

Aidyn, 18 months old, Alyssa almost nine, Oct. 2010.

Aidyn and Alyssa enjoyed Halloween this year. It was fun to dress Aidyn up in his skeleton costume and have him join Alyssa trick-or-treating. Alyssa loved having him there. The year before, he was still infusing daily for about 18 hours, so it wasn’t worth having him experience Halloween. He wouldn’t have enjoyed it anyway. Alyssa scored well, especially because she could have all of Aidyn’s loot, at least all the gluten-free ones.

In November, Aidyn saw an allergist to rule out any potential food problems. I had him tested for about 40 foods. I felt terrible seeing him pricked on his entire back, but I wanted to be sure we covered a lot of what he was being offered and what he would potentially try. It turned out he was intolerant to beef and dairy, and slightly to wheat. He had not been eating any gluten, but lots of dairy and beef. We did a patch test for eggs, wheat, goat’s milk, soy, and corn. Wheat was the only one he had a slight reaction to. Aidyn finally had his immunizations updated with his new pediatrician. He was behind quite a bit since we opted not to have them done this summer. We are very happy with the new pediatrician. He is intrigued by Aidyn’s story and progress, and impressed with his well being.

Barely two months in our new home, we were celebrating Thanksgiving with my dad and family, but the week before was another Boston trip. We stayed with the Mills family, our home away from home. They have been wonderful, allowing us to stay with them for each appointment or hospital stay. They look forward to seeing us each time, as we do them. The clinic appointment revealed Aidyn actually flattened out on the growth curve. He had not gained enough weight to wean any TPN. Despite the small weight gain, the team decided it was time to knock off the remaining nights of hydration. I was a little nervous about this new change, but knew I could just give Aidyn supplemental hydration if needed.

Waiting for our cab to go to Children's Hospital Boston.

The GI visit back at home gave me mixed feelings. Aidyn had his stools tested for fat malabsorption and blood. Dr. Ursea wanted to figure out why Aidyn did not gain much weight. Test results showed he was not absorbing his entire fat intake and there were microscopic amounts of blood in his stool. There were two concerns: Aidyn could have cystic fibrosis (the cause of malabsorption) and eosinophilic gastroenteritis (a result of more severe food intolerance, leading to malnutrition if not corrected). A sweat test could diagnose cystic fibrosis and an upper endoscopy with a flexible sigmoidoscopy could diagnose eosinophilic gastroenteritis. Since Aidyn had just stopped eaten the offending foods just about a week prior to Dr. Ursea’s appointment we did not want to have invasive procedures done yet. The team in Boston agreed. We waited to be scheduled for the sweat test and discussed the invasive EGD and flexible sigmoidoscopy at the next appointment. In the meantime, Aidyn was prescribed pancreatic enzymes (Creon 6000) to help with digestion, in case his pancreas was indeed having trouble with digestion. It wouldn’t hurt.

Lovin' the tortilla chips.

It's like playing "Where's Waldo?"

December was super busy, as anyone might imagine. Aidyn started the enzymes and seemed to be eating and drinking a whole lot more, but not before having some gassy symptoms, distended stomach and maybe constipation (how can you get constipated when your poop is like diarrhea most of the time?). I think the introduction of the enzymes caused a slight side effect. It has since resolved. Anyhow, I was sure he gained weight, but a visit to Dr. Ursea’s monthly check up showed he gained hardly any. He was healthy and we just kept the TPN the same and gave the enzymes a chance to start working.

Three generations.

Christmas was a whole new ball game this time. Santa was very generous to Aidyn and Alyssa, and so were a few folks who made sure they would have a great Christmas. We appreciated the extra gifts from friends and family. Joseph’s parents came for a visit Christmas day and my family came up for New Years Eve.

It felt like a much happier holiday this time, as Aidyn was healthier and we were more relaxed. There hadn’t been any hospitalizations since August and we all have been making new friends in our new neighborhood. We will soon be posting more recent news...good news!

Updates?

I know I have not updated in a long time. I promise it will happen very soon. I have actually had it ready for weeks, but I need to simplify the new post. I can say that Aidyn is doing well and we anticipate good news from our upcoming Boston appointment this week!

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.

He walks!

Aidyn has been dabbling in walking for a couple weeks now. But last Saturday, he went for it. It seems his medical issues are no match for his determination and curiosity.