Afton Jane

Day 25

Three weeks and 3 days later, Afton has come home.  We are all doing well.  Afton continues to work on her bottle feeding, but we are still giving her food and medicine  through her NG tube (feeding tube through the nose).  We have decided to do surgery to insert a G tube (feeding tube through the stomach).  Since she is currently on a blood thinner for her clot, so we need to wait to have surgery until she is off that medicine.  We are hoping this will happen in mid to late January.  The G tube is more common than  we thought, and will only leave a scar when she is older.  It helps us as parents be reassured that she is getting the calories and medicine she needs, which will hopefully make her first illness less serious.  

Thanks again to everyone for all your support.  We couldn’t have made it through the last few weeks without our friends and family.  

Day 19

We just wanted to reiterate our appreciation to everyone for all of your positive thoughts.

Afton is stable and continuing to work on her bottle feeding.  She definitely knows how to eat with a bottle but hates doing it.  She either pretends like she is sleeping (playing possum) or cries when we try to get her to eat.  The doctors believe that she is very uncomfortable when she swallows, so it could be reflux or the feeding tube is irritating her throat.  She will probably be in the hospital for at least another week if not two weeks.  Our hope would be to have her home for Christmas, but we need to just stay patient.  

Today was Afton’s first encounter with a celebrity and we have to admit that she has the biggest claim to fame of anyone in the family.  Will Smith was so great and positive.  He gave so many kids in the hospital such a good experience.  Everyone was smiling and most were speechless.  It gives us a lot of respect for him to take time out of his day to make these children forget about their illnesses for just a short amount of time.  

Day 15

Today Afton had a swallowing test.  They took an X-ray of her swallowing to ensure that all the liquid goes to her stomach and not down her airway.  If some of the liquid is leaking into her lungs it could cause an infection, which could be dangerous for her.  She did really well with the test and so they have let her start to practice bottle feedings with small amounts.  

The doctors have been starting to educate us more about Afton’s disease.  About 1 in 100,000 babies are born with this.  It is quite rare, so not only are we learning the medical professionals are leaning more every day as well.  

The most important part of keeping Afton healthy in the future is to ensure that her ammonia levels stay within normal ranges.  We can do this by giving her medicine and putting her on a special diet.  However, when Afton’s body creates white blood cells, her ammonia levels can go up as well.  So, this means that anytime she is starting to get sick, we need to be proactive and get her to the emergency room.  They will give her the nutrients and fluids that her body needs to lower the ammonia levels.  To begin with, we will need to go to the hospital for any of the following:  a cold, fever, loss of appetite, vomiting, flu, or exposure to people who have had the flu.  

This is very overwhelming for us to think about as parents, so we would like to ask for help from our closest friends and family in monitoring this.  If we are all together and you/your family has had or had exposure to the flu (or other illnesses), we ask that you let us know.  Additionally, if we are together and soon afterwards your family gets ill, we would appreciate a phone call letting us know that as well.  Lastly, as fun as babies are to hold and play with we ask that you refrain from doing this if you have been sick or around others that are sick.  As Afton gets older this shouldn’t be as hard for us to manage, but we need to be extra careful in these younger years.  

There is more talk about getting Afton ready to go home.  At this point we think that she will go home with a feeding tube.  She will probably be able to take a bottle, but the feeding tube helps us guarantee that she is getting the appropriate amount of calories as well as her medicine.  This is our preferred method because if we have direct access to her stomach, there is less risk of her ammonia level rising to dangerous levels with her first illness.  So, if she starts to vomit, we can ensure her body gets nutrients and the appropriate medicine by using the feeding tube rather than trying to get her to swallow it. 

We are open to visitors if you would like to come and meet Afton before she leaves the hospital.  Please just call one of our cell phones to arrange a time that works.  Again, we ask that you not visit at this time if you have been sick or even around someone who has been sick.  Thanks for all your support and prayers.  

Day 11

Afton has had a number of changes over the last few days. She was extabated (removed from her breathing tube) two days ago. This process went well and she is doing a good job breathing on her own.

Her legs are still very swollen due to the dialysis catheter. This created a blood clot that goes from her knee up into her groin. They will continue to monitor this but her body needs to auto-correct the blood flow around the clot, which may take some time. She will need to be given 2 shots a day for 3 months to help with this.

She had another echocardiogram done on her heart and from that standpoint everything has returned back to normal. So there are no major long term concerns with her heart function.

She had an MRI done on her head on Monday to help assess any damage from the high ammonia levels. Unfortunately, the neurologist believes that this traumatic experience has caused brain damage, but to what degree we will not know for months or possibly years. We understand that this brings many questions to mind and we want to express that we are feeling the same way. There are no definite answers at this time and that is hard for all of us to accept.  We are learning to take things one day at a time. Each time she reaches a new milestone (sitting, standing, walking, speaking, etc) it will help us understand where her limitations will or will not be.

We continue to be optimistic and hopefully her brain will do amazing things. She is only 11 days old and babies are very unpredictable. This is why they cannot tell us what to expect in the future. If she were an adult the impact would be much more predictable. As far as today, we look for signs that she can do things that babies would do which include: crying, pooping, sucking, breathing, looking around. She is able to do all these things. Today she has been crying quite a bit. She is still on a feeding tube, but has been sucking on a nook occasionally. They have turned her feeding tube off for three hours to encourage her to get hungry which will eventually let us try bottle feeding. If the feeding tube is constantly on, there is no motivation for her to suck on a bottle. This will be the next big step for her.

Today she was transferred out of intensive care. We have mixed feelings about this. We have definitely developed a comfort level with the doctors in the PICU, so the transition will be hard for us as parents. However, this is good step in getting Afton ready to come home. They are starting to educate us on her care for when she does return home. We are excited for this to happen, but obviously are dealing with a lot based on the neurological results. It has been a very emotional and difficult time for us both, but we are working on taking things one day at a time and not trying to predict the future. Thanks for all your thoughts and prayers. Please keep them coming.  

Day 5

Doctors continue to wean Afton off the breathing support, however this is something you don’t want to rush.  They tell us it’s common for newborns on respitory systems to develop lazy tendancies when it comes to breathing. We need to teach Afton to breathe on her own again, but aren’t sure how long this process will take her. 

Like most newborns, she slept most of the day, but she had periods of alertness. Her eyes would open and she would respond to touch. She was tightly swaddled to keep her from pulling out the breathing tube, as her physical movements increase. Throughout the day, she was not on any medication for pain or sedation, so she slept and awoke on her own schedule. 

She has swelling in her leg from the dialysis entry point in her femoral artery. The color has reduced from purple & red tones to normal skin colors. Today they did an ultrasound on the leg and found a large clot blocking the passage of blood. At this point, this isn’t a cause for great concern, because it’s not blocking the blood to the leg, rather the return of blood back to the heart. Her lower leg still has a pulse, and she can wiggle her feet and toes. The nurses/doctors have told us that the body will naturally reroute blood so it gets back to the heart. With this said, they are taking measures tonight to try and reduce the clot and open the passage, therefore helping to reduce the swelling. 

Throughout the day she was fed formula (through a feeding tube). It was a special formula prescribed by the metabolic specialist, that allows Afton to process the proteins within. 

While we feel encouraged about some of these progresses, the full impact of the high amonia is still unclear.  We want to thank everyone for all your thoughts and prayers, please keep them coming.  

On Saturday, November 22nd in the evening hours, Bridget went into labor and was admitted into Methodist Hospital. Labor progressed smoothly through birth, and Afton Jane was born at 12:45pm the following day. She was 7 pounds - 7 ounces, 21 inches, and seemingly healthy and well. The initial exam with the staffed pediatrician went well. Even in the capacity of her limited knowledge of Bridget’s pre-existing heart condition, her assessment was positive. This however, we were prepared to take with a grain of salt, because we had already lined up a pediatric cardiologist at the Children’s Hospital to assess the tests on Afton’s heart. Our orders were to have the specialist receive the test results as soon as possible after birth. Upon reviewing the data, the specialist was concerned enough to have Afton transferred to his hospital to be monitored. 

The first day at the Children’s Hospital things went well. However that evening Afton suffered a respiratory-arrest and would not start breathing again on her own. She lost consciousness, and the doctors were unclear what was happening to her, or how to awaken her.  She was immediately put on life-support systems and medication to regulate breathing, heart functions, and nutritional intake. At the same time, testing swiftly began to try and find a reason for her condition. The next morning, the doctors had test results showing unusually high levels of a couple things — one was an abnormally high ph level in her system, and the other was a high amount of ammonia. The ammonia lead the doctors to suspect a failure in the metabolic system, and it was decided to have Afton transferred again, this time to the Children’s Hospital at the University of Minnesota, where an area renown specialist in metabolic systems was located. 

Upon arriving at the new location, we were greeted by an amazing group of doctors and staff. The first step, as immediately as possible, was to lower the levels of ammonia in her body. For this they had introduced medication, however the only proper way to make a true attempt at lowering the levels as fast as possible was to use a process called continual-dialysis. In this process, the blood is continuously syphoned from the body, scrubbed, and put back into her. She would continue on this process until her ammonia levels dropped to a normal level, and we were sure would remain stable and at healthy levels. That night, the dialysis worked very well at removing the toxins, and by morning, her ammonia level was within the confines of normalcy. This was key, because the faster the toxins are removed from the system the less damage it can do to the body. Throughout this process, she continued on life-support machines and medications to regulate her bodily systems.

Now that her ammonia levels were seemingly stabilized, the focus shifted. We needed to find the reason why her body had produced  toxic levels of ammonia in the first place. This was the task of a brilliant specialist in the area of metabolic processes. She had already initiated many labs that she would use to find the root of the problem. With the specialist’s direct contacts at the Mayo Clinic, and the Department of Health, the labs were fast-tracked, to expedite the finding of the solution.

In the meantime, Afton’s body started to show some light signs of recovery, as she started showing physical reactions to some touches, and involuntary movements in her extremities. Her responses to stimuli and her physical activity seemed to be slowly increasing, which is very positive.

The metabolic specialist then told us that she had determined the diagnosis. Afton possessed a rare genetic defect in her metabolic system that, when breaking down certain kinds of proteins, the process went awry at the last phase of metabolism where ammonia is converted into urea (urine). When the human body is sick or under stress, it looks to protein stores in the body to metabolize into food to use as fuel to combat its condition and heal. Afton’s body cannot (by itself) successfully break down those proteins. The result was a build up of toxins (ammonia), causing more stress, and the cycle snowballs. This condition can be mediated, with a specific diet and medication, and with that knowledge her body has recently began to be intravenously supplemented with proteins that her body can process.

At this point, we are unclear how much damage her body has suffered. But, with the factors that cause the damage eliminated, we have reached a phase of healing. We don’t know how long this process will take, nor do we know to what extent it will progress.  Our main concerns are for a full recovery of her heart and brain functions.  Slowly, specific life-support systems are being weaned, hopefully allowing her to breath without the help of machines, and let hear heart function without the help of regulating medicine.  Unfortunately, at this point Afton needs time, so we will need to be patient and wait to learn more.