On the outside, Hallie looks like a normal, energetic 8 year old. However, on the inside, it is a completely different story. She was born December 20, 2005 at 37 weeks gestation, and while technically being considered full term, she looked more like a preemie, weighing in at 4 lbs 8 oz and 17 inches long. I was only able to hold my baby for about half an hour, when they took her from me to try and determine what was wrong with her. Hallie was transferred by a special team to the children's hospital in Salt Lake (where she was born) within hours of being born. By the next afternoon, they had determined she had a bowel obstruction and began surgery to fix it. What they found was worse than they had imagined. She was diagnosed with a jejuna atresia-an apple core atresia to be exact. There had been a disruption to her blood supply to her intestines and her intestines completely separated in utero. Her small intestine end sealed off and because she kept drinking the amniotic fluid, it stretched out like a balloon. Her large intestine end had no blood supply, so it withered up and died. They ended up removing half of her small intestine and half of her large intestine when she was 1 day old. She remained in the NICU for 25 days, during which time the nurses noticed a heart murmur and tests were ordered in which it was determined that she had an atrial septal defect, commonly referred to as an ASD.
We were home only 2 weeks before she was admitted with a respiratory virus known as RSV. Hallie spent 11 days in the hospital, during which time she contracted an intestinal infection commonly referred to as C-Dif. Basically, the antibiotics they were using to fight her ear infections, killed off the good bacteria as well as the bad in her intestines. This is a big problem for her because she is missing so much of her intestine, that it is very easy to disrupt the "flora" (the balance of good and bad bacteria) of her intestines.
Shortly after returning home with our baby, we noticed a bulge from time to time as we were changing her diaper. If we pushed on it, it went back in, so I took her in and she was diagnosed with an inguinal hernia. We scheduled surgery to repair it with a new surgeon because we had heard her original surgeon did not give her much hope of surviving without being TPN (nutrition through an IV) dependent. We needed someone who would give her at least a fighting change. Just 3 days before her scheduled surgery, Hallie's intestines stopped working and she was rushed in for emergency surgery, this time with the surgeon who happened to be on call. Dr. Eric Scaife found that a section of her small intestine was flopping over onto her large intestine and had created a lot of scarring. He removed 5 inches more of her large intestine, although he wasn't hopeful that it was going to solve her problem.
During this 2 week hospitalization, Hallie was scheduled for a cardiologist appointment and since they were booked so far out, I didn't want to reschedule. I took her for a little walk and met with the cardiologist and was given shocking news. Based on the fact that she had a unique heart defect called an interrupted inferior vena cava with an azyguous continuation, he diagnosed her with polysplenia, a condition in which the patient has multiple spleens. He also informed us that she had an underdeveloped right lung. As I started to research polysplenia, I was devastated. Only 4 in 1,000,000 children are born with it. The survival rate is not great&emdash;1 in 3 will not make it to the age of 1. Half of those that survive that long, will not make it to the age of 15. Major birth defects are associated with polysplenia. Basically the spleens take up too much room while developing, and so none of the other organs develop properly. Also, while one would think that having more than 1 spleen would be great (if 1 is good, multiple is better), the majority of polysplenic kids don't have a functional spleen, which greatly effects their immune system.
Armed with these statistics, we took our daughter home, only to remain there for 2 days before her intestines stopped working again. Hallie was admitted to the hospital again, where her doctors, led by Dr. Scaife, determined that her best chance of survival was a very new surgery, that had only been performed a few dozen times in the nation, called a Serial Transverse Enteroplasty (STEP). They had no statistics to give us, but we felt this was her best chance and chose to go ahead with this option. However, because Hallie's last surgery had been just 2 weeks before, the risk of causing more damage by operating again that soon was too high. They surgically implanted a central line into her chest and got her started on TPN. We took her home a week later, with her only being able to eat 2/3 of an ounce of formula every 3 hours. It was all her intestines could tolerate. The rest of her nutrition consisted of me starting at 5:00 at night, mixing up her TPN and lipids in a sterile environment, programming pumps and hooking her up by 6:00. By 12:00 the next day, she would be done and we would get a small break from the pumps. We did this for 6 weeks to buy her time to recover, in order to do her STEP procedure. It was a very difficult period of time for us, with Hallie crying a great portion of the time.
A week before she was scheduled for surgery, she ended up with a line infection and we wound up back in the hospital, on antibiotics, trying to save her central line. We finally ended pulling the line a few days before surgery because they would not operate without the infection being under control. On May 2, 2006, Dr. Scaife took Hallie from our arms and began the STEP procedure. I had asked him to look at her spleens while he was in there and he found that she has 5 spleens. Her pancreas is wrapped around her intestines, her liver is very flat and malformed. All of this is attributed to her polysplenia. He ended up cutting her intestines apart in 30 different places and stapling them all back together again. In essence, he completely rebuilt her small intestines, giving her back about half of the amount she had originally lost. It was a very hard surgery on her and caused extreme amounts of pain. She required blood during surgery and they gave her a morphine drip to try and control the pain. Several hours after surgery, she started itching and jerking around. They couldn't figure out why until she stopped breathing. She was having an allergic reaction to the morphine. What a scary day that was! Once they got her breathing again, they gave Hallie some Benadryl and we quickly switched her to different pain meds. After that, she amazed all of us. Just 4 short days after surgery, her intestines recovered enough for her to start eating. We left the hospital one week after surgery and she took off from there.
Hallie was still on TPN for another month, while her intestines completely recovered. She had another line infection, which required another week in the hospital, but on June 1, 2006, they removed her central line. From an intestinal stand point, she had about 2 1/2 years that she did really well. She was hospitalized in January 2007 for a respiratory virus, and then again in February of the same year for another respiratory virus. In March 2007, we took her in and had tubes put in her ears to help with her ear infections. We moved to Reno in September of 2007 due to my husband's job. In January 2008, she ended up with RSV and pneumonia and was in the hospital here for a few days on oxygen. We were hopeful that we would only be dealing with her immune issues and the aftermath of her intestinal surgeries.
Shortly after Christmas 2008, Hallie started experiencing a lot of pain in her belly. We watched her for a few weeks, wondering if she didn't have an intestinal virus causing the pain. After she started waking up at night in pain, I took her to her pediatrician. We did x-rays and Hallie was diagnosed with an ileus. Basically, for whatever reason, her intestines just stopped working. They put her on a liquid diet for the weekend, but by the next week, we ended up in the emergency room, with her in immense pain. They gave her enemas to get things moving again, but I could still see that things were not normal for her. Because she has so little of her intestines, she should have frequent, loose bowel movements. She started going 48 hours in between movements. At this point, I contacted her surgeon and GI doctor in Salt Lake. They put her on a laxative to try and get things moving and they did for a few weeks, but by the end of February, she started going 48 hours in between bowel movements, even on the laxative. I took her to Salt Lake in March, where she was diagnosed with a partial obstruction. We had to wait to have surgery because she had a cold and was having an asthma-like reaction to it. Finally, on March 23, we took her in for surgery. Dr. Scaife went in and removed about 7 inches more of her intestines, leaving her with only 130 cm of small intestine, about half of normal. The day after surgery, she started coughing and was quickly diagnosed with influenza and become so weak that she couldn't even blow bubbles from a wand. She remained in the hospital for 9 days, and then we stayed with family for a few more days before flying back to Reno.
Hallie had only been home for 24 hours, when her intestines stopped working. She was admitted to the local hospital, where they contacted Salt Lake and decided it was in her best interest to send her back. She was Emergency Airlifted back to Salt Lake the next day, where they took her off all food and ran a bunch of tests. After a week in the hospital, we determined that she could not absorb enough nutrients out of her intestines, so we placed a feeding tube in her nose, got her stable and released her. We stayed with family through the Easter weekend to make sure that she really was stable and then flew home to be with the rest of our family.
Two short days after returning home, she started having a lot of pain and throwing up, which are classic signs of obstructions. She was admitted, only to find that she had contracted the rotavirus. Because she was so malnourished from her ordeal of the last few months, she was there for 4 days while they balanced out some of her nutrition levels. Shortly after she got home, her baby brother was admitted for the same virus, however he only had to stay 24 hours.
In June, we took Hallie in and had a permanent feeding tube placed, in order to make her life easier. She started having problems again in July. We did more tests in Salt Lake and they found that a section of her intestines is slightly malrotated. In and of itself, that is not a huge problem, but for whatever reason, her intestines just don't want to move things through. In September, we surgically placed a central line (permanent IV) and began doing supplemental nutrition intravenously. We began with just a few days a week and then slowly increased her to doing it daily.
November 2009 took us to Stanford to try and find better help closer to us. We only went there a few times before they told us that there was nothing more they could do for her. They wanted to put her on 100% IV nutrition (TPN) and not allow her to eat. The problem with this is that the TPN will eventually damage her liver. The more of it she is on and the less she eats, the quicker this damage happens. Once the liver is damaged, there really isn't much more that they can do. We quickly decided to seek help elsewhere.
We headed to Cincinnati, Ohio in December to spend Christmas with Grandma and Grandpa. Since the children's hospital there has the number 1 GI department in the nation, we made arrangements to take Hallie there. They admitted her for a few days for specialized tests.
In March 2010, Hallie came running in will blood spurting out of her central line. It had broken in half. They were able to put a temporary patch on it and back to Salt Lake we went to get a new one put in. We made the decision to put port in. It basically goes completely underneath her skin and is accessed by a needle when needed. The biggest reason for this was to give her more of a normal life. With her previous central line, she couldn't swim, she couldn't bathe, she couldn't get it wet. She also had developed an allergy to her dressings that covered the central line. Hallie started to get these open wounds under the dressing. We tried several of them, but she continued having reactions. We hoped that by only accessing the port when needed and when it wasn't in use, her not having to have a dressing or anything on her skin would help the situation. It meant us having to learn to poke her with a needle, which I hated doing, but in all, we have been very pleased with the port.
April took us back to Cincinnati where they ran even more tests. She was diagnosed with reflux and a mechanical defect. They felt like no amount of diet would help her and started her in their bowel management program. It involved playing around with different medicines and getting an x-ray to see how much it helped clear her intestines out. Over April and May, Hallie had probably 50 abdominal x-rays. She had also struggled all winter with asthma and we were just starting to make the connection that it was directly related to her reflux.
May brought an amazing adventure for our family. Hallie was granted a wish through Make A Wish and chose to go to Walt Disney World. It was an amazing time for our family to reconnect and put aside as much of the medical problems as possible for a week. We stayed at the Give Kids the World Village, which is specially designed for wish kids. They had so many fun things for the kids to do aside from the amusement parks. We were given VIP treatment everywhere we went and it was good to be able to meet families that were dealing with health problems like us.
In July, Hallie had 2 pseudo obstructions. We spent most of the month trying to rehab these, but finally ended up taking her back to the hospital in Salt Lake. She spent a few days there while they tried to figure out what she was doing. We took the opportunity while we were in Salt Lake for a few weeks to run more tests. We headed back home, only to have Hallie wake up with a fever of 105 the first part of August. She was quickly admitted to the hospital in Reno, where we spent 4 days fighting a line infection before it became critical and she was airlifted back to Salt Lake. We spent 5 more days fighting it before they decided to just remove the port. They put a PICC line in her arm and discharged her. We headed back in September and they surgically placed a new port.
We received a job transfer in October and moved our family in November to Chicago, Illinois. We had prayed for a long time for a way to get better help for Hallie that didn't require our family to be apart as much. When we agreed to move, we quickly began praying that Hallie would stay stable enough to get us out here. Amazingly, her body went into a remission of sorts and she did phenomenally until January 2011. She gained weight, didn't take any meds and was pretty much pain free. It was an amazing few months and the peace we felt was almost tangible. But through it all, we felt like it was only a matter of time before it started up again. We were hoping to build up her reserves to help Hallie through whatever came next. The doctors even started weaning her off the TPN.
But as we expected, Hallie relapsed the end of January. She ended up with a partial bowel obstruction that landed her in the hospital. We spent 3 days there, during the blizzard that hit Chicago. Thank goodness Grandpa was able to come up and be with the boys so that I could focus on taking care of Hallie. Since this hospital visit, Hallie has continued to go downhill. She rarely has good days any more. We are grateful for 4-5 hours a day. Hallie has been diagnosed with migraines. She has had them for about a year. They are coming more and more frequently and last longer and longer. Her abdominal pain is almost daily. And her legs are hurting her a lot. Her bowels seem to be functioning better than they have in a while, which is a huge blessing, but her fight is slowly draining out of her. She is giving in more and more often to the pain, missing school frequently. I have always been impressed by her determined ways and her refusal to let her illness define her life. To see her giving in and saying she just can't do this anymore breaks my heart. Her doctors think she is depressed (who can blame her?) and want to start her on some medicine.
In April, at my insistence after having several specialists tell me that she might have a tethered spinal cord, Hallie underwent an MRI. Not only did they find the suspected tethered cord (which means she has a hole at the end of her spine that let her nerves go through and connect to her tailbone and a fatty tumor pressing on her spine) they found that her cervical spine is completely malformed. She has 2 vertebrae that are completely fused together.
We spent the summer running test after test after test to determine the extent of her cervical spine problems and whether they need to be fixed. At this point, her neck is stable so we will just continue monitoring it every 6 months. Her neurosurgeon tells me that the pressure her vertebrae are putting on her discs is huge and he would expect her to have degenerative discs in the next 10 years or so. We attempted surgery to repair her tethered cord, only to have it cancelled as anesthesia was hooking her up because the doctor was afraid she might stroke out during surgery. And so we got to do more tests. She finally was able to have that surgery in October, where she stayed in the hospital for a week. She will continue to be out of school for at least another week, perhaps longer as her body slowly recovers.
During all of the other testing, we managed to fit in some upper GI tests as well. Her esophagus is being slowly fried because her acid levels are so high. When she was sleeping, her acid levels are somewhere between hydrochloric acid and battery acid. Hallie was on IV meds for a year and a half for her reflux. We made the decision to do a surgery called a Nissen. They wrapped the top of her stomach around the bottom of her esophagus to prevent her from refluxing. The surgery in and of itself was very difficult. This is something that can typically been done laproscopically, but for Hallie, they had to open her up completely, from one side to the other, and from just below her sternum to below her belly button. She had so much scar tissue that it took hours for the surgeon to cut through it all. Her organs are all malformed and positioned incorrectly, so it took him hours to work around them to do what needed to be done. Hallie went into liver and kidney failure after surgery due to the trauma. Recovery was very slow.
Even when she managed to make it home, she was fraught with setbacks. She ended up losing the blood supply to her spleen and lost her main spleen. Her smaller accessory spleens were quick to follow. This left her without a place to store her platelets so her platelets are in her bloodstream. She has about 4x the number of platelets in her blood as a normal person. She also is no longer able to filter out encapsulated bacteria. She was put on a daily antibiotic to try and protect her from things like staph, pneumonia, and meningitis. Hallie had a bowel obstruction a few weeks later, which she was admitted for. We were able to rehab it at the hospital. On Easter Sunday, Hallie came running out of the bathroom screaming for me. I went in to find a toilet filled with blood. I took her to the local hospital where they stabilized her. Her heart rate was sky high and her blood pressure was very low due to the large blood loss. They transferred her by ambulance down to the Children’s Hospital. She spent several days in the hospital while they scoped her and ran other tests. It was determined that she had an ulcer at the anastomoses (place where they sewed her intestines back together) from her last resection.
They sent her home and a month later, Hallie went back in for an encapsulated endoscopy (a pill cam). Since a part of her intestines have been completely restructured, it is almost impossible to get a traditional scope into that section. It was confirmed that she had a large ulcer.
For the last 2 years, Hallie has bled off and on from the ulcer. She has also had unexplained bleeding from her kidneys. We have battled low blood counts, high heart rates, and low blood pressures. She goes in every two weeks for iron infusions to try and combat that ongoing blood loss. She is given injections three times a week to encourage her bone marrow to produce more blood cells. She has required two blood transfusions. It has been a constant battle to keep her blood counts in an acceptable range. She has battled with ongoing fatigue and pain. When her ulcer flares up, the pain doubles her over, and then the bleeding starts. Hallie gets very tired and has a hard time functioning throughout the day. It has been a hard couple of years.
During this time, her migraines have also gotten worse. She has been admitted for them and taken to the ER several times. It is unclear whether there is an association between the migraines and the ongoing blood loss. Another theory is that the smaller blood vessels to her brain are abnormal, as they are everywhere else in her body. Whatever the cause, they have become a big problem in her life. She has been put under anesthesia so many times that is starting to affect her brain. She has ADHD that is hard to manage due to her many heart defects. Hallie also has post-traumatic stress disorder and anxiety from all she has been through. Her mental health has become a very large part of her everyday life.
Hallie is currently scheduled for her 4th bowel resection. This will be the 7th time that they will cut across her abdominal organs. This surgery is very risky. They will have to cut through all of the scar tissue from her previous surgeries. Once they separate her intestines, they will run the scope up into her restructured section. They will look for additional ulcers that could be contributing to her ongoing blood loss. If they find more than one ulcer, they would have to perform multiple resections. This is on ongoing nightmare. I want so much to keep her from having to go through this again. However, the risks of her continuing on with the ulcer as it is have become much greater than removing the ulcer. It is a tough position to be in.
Hallie is a full time job. I never imagined anything this difficult when I thought about being a mom. There are so many days that I wonder how in the world I will make it through and so many sleepless nights worrying about how to get Hallie the care she needs and whether we will be able to keep her body going. But I wouldn't trade it for anything. Hallie has such an amazing spirit. She just seems to be able to find joy in the smallest things. It doesn't seem to matter how hard her day has been, the hug and "I love you Mommy" makes it all worth it.