All of a sudden the numbers were better
She got initially better and then she started to get worse, so I guess we just assumed she was going to die at three months, and we sort of changed our outlook. And then it was probably right around six months, we went in for a routine ECHO, and the technician actually thought he had done it wrong and re-did it, because all of a sudden the numbers were better! And he called the doctor in and everybody was really excited. So she incrementally got well enough that they could do open heart surgery when she was eleven months and put in a patch for the VSD.
Mother of Eva, 13
They couldn’t figure out why
Mom: What we didn’t know is, the Treprostinil actually opens up the vessels and allows the blood to pump and lowers the pressure in the heart, but her secondary cause was discovered after transplant because we donated her lungs to science: she had PVOD, pulmonary veno-occlusive disease, and the Treprostinil was actually working with the disease, so it was progressing the disease at a more rapid rate. But at the time we didn’t know what it was, and every time we would try to go up on her Treprostinil, she would have such severe bone aches you couldn’t even touch her. And we couldn’t figure out why!
Jess: It was miserable…I couldn’t breathe. I was on oxygen 24/7, like maybe 6 liters or more. I think when I was in the hospital I reached up to 20 liters on high flow, and that would just keep me in the 90s. I couldn’t, like, chew and eat, because I would run out of energy and my sats would drop.
Mom: She got really severely sick. She was walking like a little old lady. It was like you couldn’t touch her, her clothes hurt her, she wasn’t eating, she couldn’t walk from here to there without her sats dropping to 52, and she was on 6 to10 liters and they couldn’t figure out why.
Jess, 18, and mother
A very hard adjustment
He responded well to that for about maybe a year, to the oral medication. Then he got sick again and was hospitalized for about nine days, and it became clear at that time that the oral medication just was not treating the disease properly. He then needed a central line and to go on IV medication.
It was very hard, very hard for me as a mom to see him like that, and to worry about what it meant for him to go to school and how kids would treat him at school. I had a lot of issues around that, around how he now had limitations and he couldn’t play, he couldn’t run and jump like normal kids could. So it was a very, very hard adjustment.
[Later] He had a hard time with having a bag. Because he was so young at that time, we put his medicine in a little mini backpack so it was on his back. He went through some teasing from kids around, you know, “What’s that on your back?” and “Why is that line there?” and “Why do you have a purse?” You know, a lot of things like that. And he would talk about it when he came home, and the nurse was very helpful in going to the class and talking to them in general about someone being sick and needing medication, it’s not something to make fun of. But it was a tough adjustment period, and he missed a lot of school.
Mother of RJ, 12
Chemotherapy
Hannah had about 4 ½ rounds of chemo (some inpatient, some out), constant monitoring of blood work, and lots of scans – it was monthly scans at that point. She was diagnosed in September, monthly scans until November, and the last scan she had, they realized that the tumor was responding very well to chemo, had shrunk tremendously, but it was shrinking in the wrong direction: instead of shrinking away from one of her major arteries, it was shrinking into her major artery. And so at that point, they determined that they would not be able to resect the tumor with 100% conviction that they would get it all, and so that led to the ultimate decision of a liver transplant.
Mother of Hannah, 4
The Kasai procedure
We met with the liver team, and Dr. Kim gave us the background on the Kasai and what it was. He also told us that 1/3 of the children do really well and never need the transplant, and 1/3 of the children can live for a few years with the Kasai alone, and then for 1/3 it may not work at all. But at that time we had no choice: he was looking sick, and he wasn’t growing and absorbing anything he was eating, so we knew we had to do something.
The procedure itself was fine. We were admitted the night before, and then the next day we met with the doctor. The procedure was about four to six hours long, and then he was transferred to the NICU. We stayed over for about two weeks and then we went home and just waited to see if it would work or not.
It was very scary, because like I said, we didn’t know anybody – we both have big families, and no one had ever gone through anything to this extent, so we had no one to tell us what it was like or that it was going to be okay. We were young, we had no idea what to expect, we were first-time parents, so it was very, very scary.
[Later] The Kasai worked well for a little while, but he got it in November, and probably in January we figured that it didn’t work…We would come once a week to the liver clinic, and they followed up with labs and just overall how he was doing. But Noah was getting very sick; he was throwing up a lot, he was very lethargic, very, very jaundiced. And that’s when they told us they were going to list him.
Mother of Noah, 5
Learning dialysis
I remember the nurse coming up and saying, “Okay, we’re going to go into this room now, we’re going to learn about the bags, and we’re going to inject the bags with the medicine,” and all that. So for someone just off the street, not ever considering going into the medical field – that would be the last thing I would ever do! – and all of a sudden here I am with the mask and gloves and these needles and the syringes and these bags…I was beside myself. I just cried and cried.
I didn’t think that I could ever learn it all. I really didn’t, I thought this was impossible. But going back to God: nothing’s impossible with God, and that was a perfect example, because he helped it not only happen but work to her benefit so well.
Mother of Lydia, 6
Finding the good in it
Her peritoneum was intact, so we were able to utilize PD, peritoneal dialysis. She was on it every night in her crib for something like 13 cycles, which took about 13 hours. So we were very lucky, because we didn’t have to bring her into the hospital two or three times a week for hemodialysis. We were really lucky that that worked for Lydia.
I think it was very difficult for her. There were so many side effects, and just kidney failure alone, the worst part is the nausea. She would throw up at the drop of a hat, and that was really hard. And she had to be in her crib hooked up by like 6:00, and then she had to stay in her crib hooked up until like 7:00 or 8:00 sometimes, so she was really attached to that crib. And all the sterile procedures that are part of it because the risk of peritonitis is really, really high…the dressings on her belly where the catheter was were very sticky, and that hurt! Sometimes it would bleed when you pull it off. You try to be gentle. So it was very hard for her.
But luckily as parents we saw the whole picture, and we were able to find the good in it: the fact that she didn’t have to come into the hospital was definitely a bonus. And she had her little life—I mean, when she got off in the morning, she had her little preschool and she got to run around outside and you would never know, unless you lifted up her shirt and saw all these appliances! So that enabled her to have a life, and that I think was really important after six months of sheer hell for her in the hospital, all of the poking and everything and just the medical trauma of it all. For her to have a little life each day was wonderful.
Mother of Lydia, 6
He had to go on dialysis immediately
The doctor came in and she said his blood levels were too high, he had to go on dialysis immediately. I’d been holding it in all this time, but that was like a slap in my face! Because I knew it was coming, but that was like, a reality – “Oh my God, he’s really sick!” I think I managed to get through those whole 17 years by knowing he’s sick but not seeing. You know, you’re doing the cathing, you’re taking the meds – it was all this preventive medicine, it’s preventing this. But he’s more thinking, “Oh my God, I’m sick..”
He’s grasping it, “I’m sick,” and he’s looking at how it’s affecting him. And I’m more like, “Oh, he’s sick, but we’re doing all the preventive things to keep him healthy!” And then dialysis was like, whoa – it’s up a notch. It’s up a few notches now!
Mother of Isaiah, 17
It was just a matter of time
Mom: She couldn’t get any other nutrition other than IV nutrition, so we were basically giving her IV nutrition, trying to maximize calories and minimize liver damage with fat, and she was on Actigall, a medication to protect her liver. Then when she was 3 months old, her liver had already started showing signs of damage with her labs: her bilirubin had started to increase already. And the doctor said to me at the clinic appointment, “You know, I didn’t know we would even be talking about this, but we should try to reconnect her bowel” – because she had an ostomy at that point – “so that we can give her just a tiny bit of feeds through it to help protect her liver from more damage.” It would only have so much protective effect, but at least it would give her a chance, he said…so that’s what we did. And her bilirubin actually did come down a little after that and was more stable for a time period of several months.
Dad: And then we did look into the small bowel transplant, because she was reconnected…and where our insurance would cover was Pittsburgh Children’s Hospital.
Mom: So we went there for an eval – that was when she was about seven months old, I want to say. And then she was listed a few days after that week of evaluation. And at Pittsburgh they basically said that there wasn’t too much that they would change about what our home hospital was doing…It was just a matter of time.
Dad: Getting a transplant before her liver failed completely.
Parents of Sophia, 4
There may still be hope
Mom: The Thursday before [we were supposed to come up to Boston], Sophia actually had a GI bleed, and we went into the ER and she ended up bleeding pretty severely.
Dad: When your liver starts to shut down you start to bleed.
Mom: You don’t have as many clotting factors. And she started bleeding pretty bad…They were giving her tons of blood products and clotting factors and stuff to try and stop the bleeding. And they did say they could do a scope and find out where she was bleeding, but they would have to put her on the ventilator to do that, and I was afraid she wouldn’t come off. And I said, “If her liver is this bad, then let’s just stop,” and basically we decided to take her home on hospice, because I didn’t want her to suffer more than she needed to. I figured, we’re close to the end now. And we weren’t expecting the first bleeding issue was going to be the last…
Dad: And even the doctors there thought that was probably the best way to go about it. Nobody was saying, wait a minute, let’s stop this. They thought she was pretty sick too, and that she wasn’t going to make it.
Mom: So we went home on hospice on Monday, and when we got home, I emailed our doctor here at Boston because we were supposed to go up that next day, and just said, “She’s in end-stage liver failure. We won’t be coming up to start Omegaven because I think she’s too sick, and we have her on hospice.” And he actually called me back within five minutes! Dad answered the phone, and I hear him saying, “Well, I’ll let you talk to my wife, she knows more details,” and he’s like, “It’s the doctor,” and I was like, “He – he called me back?” That was just amazing. And so I talked to him, and I kind of said at one point, “Do you think we’re being too hasty, letting her go?” And he just said, “Well, I don’t know. I don’t know where she was bleeding from, but there may still be hope, and I’ll still see her if you come up.” So I think we had got home from the hospital, what, 4 or 5 in the afternoon?
Dad: Yeah, we were hardly home, and expecting hospice people to show up, and we were going to let her go…and instead we were packing!
Mom: We hadn’t really unpacked the car, so we kind of left things there, grabbed a couple more things that we needed, and we drove up here, all night! It’s pretty much a six to eight hour drive, so we got here around 2:00 in the morning, to the emergency room.
Parents of Sophia, 4