Summary (cont)

1/16/13 at 1500 hrs - She was awake and alert for the first part of the morning and was resting until 1400 hrs when the trauma burn team came back down to change her dressings. She did not like her dressings changed as you would expect. It was nice to hear her cry even though we knew she was in pain. Her cry is still not very loud yet. The gauze stuck pretty bad still and this time made her bleed which was hard to see.

We found out today that Katelyn has Junctional Epidermolysis Bullosa (EB). The sub-type is Junctional EB with Pyloric Atresia. There are only 50 known cases worldwide.

(From DEBRA website) There are three major sub-types of Junctional EB. Herlitz, non-Herlitz and Junctional EB with associated Pyloric Atresia. Though Junctional EB is considered a non-scarring form of EB, tightening and thinning of the skin does occur. In many instances residual atrophic scarring occurs.

(From epidermolysis-bullosa.com) Junctional Epidermolysis Bullosa is the third most common form of EB, representing 5% of cases. JJEB affects less than 1 person in 1,000,000 in the USA.

Many of the kids with JEB do not survive the first year and with only 50 known cases worldwide there are not many others to compare her to. We will just take it one day at a time and any progress is good in our book. We figured the news would be hard to swallow, but never thought she would have such a rare form of EB.

1/16/13 at 2130 hrs – Katelyn is up to 6.5 CC/ hr now. She is peeing and pooping fine. Her urine was red a day or so ago and that has gone away.

1/17/13 at 1705 hrs - She is up to 8.5 CC/hr of breast milk in her J tube and she is now a whopping 4lbs 9oz. Last day on TPN and soon she will be on only fortified breast milk. She will need more protein and nutrients than a regular baby due to the need to constantly heal and grow. They did an ultrasound of her kidneys and lungs today to check a few things and we are still waiting for our debrief. Her right lung is still not inflating so it could be a diaphragm issue instead of a lung issue. She is still hardly needing the air through her nose. Her mucus has reduced and she only chokes every once and awhile. It seems to happen whenever we come back into the room. She must jump with excitement when she hears our voices.

The doctor just came in and said her diaphragm on the right does not contract as well as it should. We did send some emails to some organizations hoping to get some more information now that we know what form of EB she has. Some of our new EB friends may have also reached out for us as you can see on the posts section. There is an amazing group of dedicated people out there to help families facing EB. We look forward to learning more and meeting more people who share her condition.

1/18/13 at 1530 hrs - Burn came in and changed her dressings to Acticoat dressings so now she can go longer between dressing changes only every 2 hours the dressings need to be moistened to keep them from drying out. She has been increased to 10 CC/ hr of breast milk. They were concerned about the volume and what was coming out of her G tube drainage, but the surgeons took a look this morning and decided it was not an issue. We also met with the geneticists here about finding the exact mutated gene(s) to better treat Katelyn. Her EB is most likely caused by a recessive mutated gene both Katy and I have in common and shared with her and Evan.

1/19/13 at 0815 hrs (14 days old) - We had a busy night last night with my brother, cousin, and step mother in town. Sean was having a blast at the hotel pool and was upset we had to leave. Since Katelyn received a blood infusion yesterday her heart rate is below 160 and she seems more comfortable. The doctor this morning said her lungs and bowels sound the best he has heard. She really hates the nurses putting water on her dressings every two hours, but stops crying shortly after they are done and falls back asleep. It still is a rollercoaster of emotions being here. One minute you imagine taking her home someday soon and the next it seems she will not leave here alive. It was hard to hear her cry every two hours last night and this morning during dressings wetting. Hopefully she will be stable enough to hold soon. Katy and I are dying to hold her instead of patting her head through and isolette hatch. I am sure she would like the cuddling as well even if it is just through a pillow (to keep from messing her skin or dressings up).

One person asked who Evan was in a post. When I wrote the first 12 days of this post I never imagined so many people would read it so here is a little back story. Sean is our oldest boy born in 2009 and completely healthy (might be an EB carrier?). Evan is our second son who we lost at 32 weeks in utero in late 2010. Now that we have Katelyn we realize that Evan also had EB. His elbows and some other areas were missing skin, but not to the extent Katelyn’s legs are. He died of a cord accident at his belly. The last two inches to his belly were completely tight and cut off blood flow. We had never even heard of EB until about 13 days ago. Anyone who has lost a child knows how hard it is to talk about, but we enjoy the short time we had with Evan and the chance we had to hold him even though he had passed. He was and remains a very important part of our lives. We are fortunate to have our very own angel looking out for us and his little sister Katelyn. Sean still does not completely understand about Evan, but he knows he has been a big brother since 2010. I like to say that Katy will be a better RN now that she has personally experienced most every condition and issue you can have during a pregnancy and birth. Most only read about this stuff in a book.

1/19/13 at 1935 hrs - Katelyn had a good two week birthday today. Her heart rate is now holding in the 150 range and she has finally had a deep sleep. The rest of the family took off around noon to try to beat the snow storm home. Her feed increased to 11.5 CC/ hr and she is not off TPN. Tonight is the first night I am thinking of sleeping in an actual bed and away from the hospital. As long as she is okay for the next few hours I might actually get a deep sleep too. One benefit of being down here for our little girl is being able to reconnect with all of the family we always seem too busy to hang out with. Tonight we saw my older cousin Eric I had not seen in some time. He just so happens to work at a daycare near the hospital where Sean might be able to go.

1/20/13 at 1645 hrs -She did not care for the wetting of her bandages most of the night, but was able to rest some. Mommy had to go and stay with Sean last night because he did not want to stay alone at the hotel with Granny. Katelyn had her feeds increased again; they are now 13 CC/ hr now. Katy found a couple of local stores that sell clothing and things made from bamboo that is super soft for Katelyn.

1/21/13 @ 1030 - She is officially off the TPN and is now on momma milk running at 11.5 mL/hr. Her electrolytes (sodium and potassium) were a bit off this morning. They've been using acticoat (a silver infused dressing that needs to be moistened every 2-3 hours), but that has been causing her to get the goosies, so they may have to change the type of dressing, yet again. Our good friends in the EB community have recommended Mepitel and restore contact layer - so I will drop those names to the burn team today when they arrive.

1/22/13 @ 1030 - When they took off her acticoat dressings, they were super dry - and ended up causing more damage yet again. So we are back on the vaseline gauze with baci slathered on, with dressing changes daily. I contacted a couple reps from various dressing suppliers and they were extremely helpful and will be sending us samples so that we can figure out what works best for our girly. Until then, daily changes :( Brad is going to be heading home this afternoon and coming back tomorrow evening.

1/22/13 @ 2015 – Katelyn’s abdomen was distended and she's very lethargic. I came in and they were getting abdominal and chest xrays. They've subsequently diagnosed her with necrotizing enterocolities (NEC). Which occurs when the lining of the intestinal wall dies and the tissue falls off. So they've stopped her breastmilk and will be starting her back up on her TPN tomorrow morning. They are giving her antibiotics (vancomyci & gent) for 10-14 days (so no feeds until her antibiotics are done). They are also doing xrays every 8 hours to keep an eye on the progression of the illness, if it gets worse, she may need to be reintubated and have surgical intervention. She tolerated her dressing change fairly well today.

1/23/13 @ 0030 - Katelyn was reintubated about an hour ago. She looks much more comfortable now that the tube is in and is getting the oxygen she needs. Radiology is going to do another xray at 0400 and if she hasn't shown any improvement they are going to insert a nasogastric (NG) tube down her little nose and into her tummy. That will be hooked up to suction to get the yuckies out of her belly. Tonight is my first sleep over with Katelyn ;) off to bed to try and catch some ZzzZzz's...

1/23/12 @ 0800 - Katelyn is a fighter, that's for sure! Her xrays showed slight improvement this morning, so no NG tube yet. Complications from NEC can include perforation of the bowel, which usually leads to abdominal surgery and placement of a colostomy/stoma, but in Katelyn’s case, a stoma would not heal and we can't place any adhesive appliance on her skin to catch the stool. So the other option would be to continue to keep her comfortable and enjoy the time we can with her. Here's hoping that Brad and I don't have to make that decision any time soon. She is doing well on the intubation, her lung that was slightly collapsed, reinflated, and her diaphragm is moving nicely (it was semi-paralyzed on the right side before). So that's good news, and with us not feeding her breastmilk, gives us time to heal the sores on her bum from all the pooping she