Afton Jane

Life Changing Decisions

We apologize for not keeping up with our blog. Our lives have just been so hectic. So, this update is meant to tell you everything that has and will be going on with Afton. First, we would like to say THANK YOU to everyone for supporting us. The amount of people who have reached out and tried to make our lives a bit easier is just amazing. We really appreciate everything.

Afton is now 5 months old. She is smiley and giggles sometimes. We have updated her photo library, so be sure to check it out.  She is still quite a handful because she is pretty fussy. We sometimes wonder if the fussiness is her personality or her metabolic disease. She often times just looks uncomfortable. So we spend a lot of our time holding and cuddling her. Her nanny, Brittany, is absolutely amazing. Afton loves her. We know how hard it is to care for Afton and we are thankful everyday for having found someone like her.

For the first 3-4 months of her life, Afton was very stable. The metabolic doctors that she sees at the University of Minnesota thought that she was doing wonderfully. She learned to eat all her food orally. In hindsight, this was surprising because kids with these disorders tend to hate food and can develop oral aversions rather quickly. Afton has a certain amount of calories that she needs daily, so we were constantly struggling to reach those goals. Believe us, it is far from easy to get a baby to a food goal if they hate eating. We started to depend on her feeding tube overnight to meet her goals.

Since everything seemed so stable for such a long time, we got a bit confident in her condition. Each trip to the clinic didn’t even include a blood draw to check her levels. We were all just confident that she was fine, since she was usually smiley. Then we got the call one random Wed about a month ago. Afton had been at the clinic and they drew blood for the first time in weeks. Her ammonia was 250, where a normal level is under 30. Now we were always told that when her ammonia goes up that we would know. Usually babies start vomiting, become lethargic, and if it gets high enough they can convulse. Her ammonia was high enough that she should have been showing all of these symptoms. However, Afton just smiled the entire time.

They had us bring her to the ER, where they checked her ammonia again. It was assumed that the test was bad (it’s a very picky test) because she was acting so normal. Unfortunately, at the ER her ammonia came back even higher. So, she was admitted to the hospital, and given IV fluids. Her ammonia continued to rise, so she was transferred to the Intensive Care Unit. They surgically put in a central line in her, which was used to administer stronger medicine to lower her levels. Even the doctor in the PICU was surprised that her ammonia was so high since she was smiling at him. By that time it was over 300. Once the central line was in, her levels came back to normal fast, but it wasn’t easy on her. When her ammonia returned to normal she screamed inconsolably for 30 hours. Nothing seemed to help her. The doctors believe that she may have gotten a really bad headache coming down off the ammonia because it is a poison building up in the body.

Fast forward two weeks later, and Afton had been back in the hospital 2 additional times for high ammonia. She would normalize for a few days then go into a crisis mode. It was scary from our perspective and the doctor’s also because she didn’t show us any symptoms. It was after the third time that we elected to have a port put in her. This is a simple surgery that puts a port into a central vein close to the heart. Each time Afton was in the hospital they needed to put an IV in her, which is very difficult on a baby. She would often times get poked 3-4 times before it was successful. They also needed to check her ammonia every couple of hours, so they would need to poke her elsewhere. After two weeks of this, almost every vein was bruised and damaged to the point where we couldn’t draw blood, not to mention that she is probably traumatized by the experience. Having a port will save her from experiencing that again. An IV, blood draw, or central line can easily be administered on her through her port.

It was around that time that the doctors started talking to us about a liver transplant. While this sounded scary at first, we eventually learned that there are also a lot of benefits. First a liver transplant cures her disease. So, we would no longer have to worry about high levels of ammonia, which cause brain damage. Also, she would be able to eat anything she wants and there would be no food goals as long as she gains weight. We feel that the chances of further innovation in her lifetime is more likely with a liver transplant because her disease is so rare.

It has been a couple of weeks and Afton has stayed stable. They have reduced her protein significantly. This seems to help, but it’s not great for helping her grow. We are not able to bottle feed her. She is on 24 hour food drips through her G tube, which the doctors seem to think helps her stay stable. The good news is that she is hovering around the 50th percentile for weight and height.

With all this being said, we are putting her on the transplant list on Monday this week. Our prayers are that she stays stable until a liver is available, that the family who loses their child to help save Afton is comforted and rewarded some other way in life, and that Afton survives and is stable after the surgery. Once we get the call, we will need to be mentally prepared to live in the hospital for months. They tell us the first year is the hardest, and after that life gets better. Typically by year two, she will probably be on one medicine and have a doctors appointment every couple months….eventually the appointments become annual. She will have no limitations: physically or diet wise.

We strongly feel that Afton has been through too much to continue her life like this, and a new liver is her chance at being given a better life. Since she is young, we hope that she overcomes any damage that has already occurred to her brain. Developmentally, babies with these disorders have done much better after having a transplant. We pray for that for Afton.

So, in conclusion we ask that everyone pray that Afton gets a liver in the appropriate amount of time, and that it is a perfect fit for her body and blood type. We also ask that you do not forget the family that this is coming from. Their pain will unfortunately be our miracle. We’ll do our best to keep everyone up-to-date.

Peace from the Fruits.

Happy (sometimes) and Growing

First we would like to apologize for taking so long to post another blog.  Life has been so hectic, next thing we knew weeks had past.  We have also updated Afton’s photo library so be sure to check it out.  

The last month or so has had its ups and downs.  The week after the surgery Afton got an infection in her belly button, which is where they did an incision.  We brought her into the hospital, just to be safe.  They gave her antibiotics through an IV, and two days later sent her home.  The next day we got a phone call saying that her blood sample (which was drawn while in the hospital) contained a bad bacteria.  They asked us to bring her back in because they were expecting her to get extremely ill.  After spending 3 more days in the hospital with a pretty happy and healthy baby they decided that the blood sample had been contaminated.  We were then sent home again.  So, we spent a week in the hospital for no reason at all, except a topical infection on her belly button.

Two weeks after the surgery Afton started being very fussy especially when it was time for the bottle.  At one point she would cry all day and even harder when you come near her with a bottle.  After talking with the doctors, they think that the surgery has caused her to have bad reflux.  So we started her on a medicine called Prevacid.  We know various other babies who have been on this and have had successful results.  After a week and a half of being on the medicine, Afton was still having a hard time….but we have to admit it was slightly better.  Since we were more confident in the diagnosis of reflux, the doctors doubled her dose.  They say we should see results by Monday, so we’re keeping our fingers crossed.  

Otherwise Afton is doing quite well.  She likes to smile, stand (with some support) look at toys and books, and listen to music.  She can’t take her eyes off of the guitar when Dad strums a few tunes.  She even got to go on a couple of trips.  We went to Tomah to spend a weekend with her Fruit cousins.  She also visited Great Grandma (GG is the preferred nickname as she’ll explain to you) Lundorff.  So many people have been coming over to visit her as well.  

We did hire a nanny.  Her name is Brittany, and she is so wonderful.  She’ll make a great addition to our family.  Afton got to play with her all day yesterday.  They were friends from the start.  

Thanks again to all of you for your thoughts and prayers.  Let’s all hope that things stay calm for a while.  As parents, we really enjoy and need these times of normalcy.  Love and kisses to you all!  Happy Valentines Day.

Post Surgery

We are happy to report that Afton had her surgery this week to insert a feeding tube.  It was a long and hard week, but now that we are all home again things are going well.  She seems much more happy without the NG tube (nose).  She is still a little sore, but nothing that a little Tylenol cannot handle.  

Afton is eating all of her food through the bottle, and is starting to have a good appetite.  Her weight gain has been great throughout her first month home.  This week she has her 2 month appointment, which will be filled with not-so-fun shots.  She is a pretty happy baby in her most alert times.  She smiles a lot which is so cute.  She is also starting to play with her voice.  She tries so hard to talk that she usually ends up with the hiccups. Occasionally, she gets a little baby babble out of her mouth, which surprises her and makes her smile.  When in the hospital, we realized that Afton really loves a good bouncy chair.  Now that we have purchased one for at home, she spends most of her time there.

Post-Christmas

Afton has now been home two weeks and is doing great.  We were able to travel a little for Christmas.  Our visiting time was limited, but Afton was happy to meet many family members (especially great grandma L).  

She has transitioned almost completely to bottle feeding, but still requires the tube for medicine.  In the past she would drink around 1/2 ounce to one ounce per feeding and we would supplement the remaining calories through her tube.  Currently, she is eating about 3 to 4 ounces per feeding.  

At her appointment today she weighed 9.25 pounds, so she is gaining weight well.  You can definitely tell when you look at her cheeks.  She continues to be very alert and explore the world around her.  Her eyes are getting better at tracking sounds and objects.  

Despite the fact that she is doing well with the bottle we are looking at doing the surgery to inplant the feeding tube into her stomach in January.  She is still on the blood thinner but the doctors have decided that her clot would not be at risk of getting worse if we take her off the medicine for a couple of days to do the surgery.  Then she will go back on the blood thinner for an extended time that is yet to be determined.  We feel that having direct access to her stomach could be life saving for her in the future.  Hopefully we won’t need to use it on a daily basis.  Right now we still use it to give her medicine.  

Thanks to everyone for all your help as we have returned home.  We can’t state how thankful we are for all the support.  For those of you who have not met Afton and would like to, we are more than happy to have visitors to our house.  Again, please hold off on visiting if you have been ill.