*Warning: May contain some graphic details of medical procedures*
Aside from the 20 weeks of constant nausea, I had a great pregnancy! However, near the end, I got tremendously huge and my doctor ordered an ultrasound to check things out. After the longest ultrasound of my life (the tech returned three times to re-measure some things and do a brain scan), we discovered the baby was a girl. The tech estimated the baby’s weight to be between nine and ten pounds. I was 36.5 weeks pregnant. She told me to go home and wait by the phone, as the radiologist had called my doctor about some things from the ultrasound. Since the actual radiologist isn’t present for the ultrasound and the techs aren’t allowed to tell you ANYTHING about the condition of your baby, I left the office full of anxiety, but not overly concerned. As we were walking out, the tech said to me “You’ll be having a baby VERY soon…” This was Friday afternoon ~ October 10th, 2003.
Not knowing the full extent of what was going on, Rob returned to work and I headed home with Kira. I received a phone call from Dr. Conner about 12:30 that afternoon that changed my life forever. She informed me that not only did I have an extreme amount of amniotic fluid, but the baby also had an accumulation of fluid building up in her abdomen. Explaining that they had no answers, and that sometimes this fluid could be indicative of kidney issues, she said I needed to see the perinatologist IMMEDIATELY. I had an appointment already made for me for 2:30. I called Rob nearly hysterical and told him to hurry home… he never got there so fast. I called my mom at work to come and stay with Kira. We quickly left the house and headed for the unknown.
We arrived at the perinatologist’s office and they began a procedure called a non-stress test where they measure any contractions, baby’s movements, heart rate, etc. and it gives them an overall picture of how baby is doing. After being monitored for about 20 minutes, they determined I needed to head over to labor and delivery to be monitored. I was pretty miserable, but I had no idea I was technically in labor ~ with regular contractions. Dr. Conner came and met us at the hospital to offer a friendly face. After hours and hours of monitoring, we talked to the perinatologist on call. He reviewed the available information, ordered some blood tests, and did an ultrasound himself, pointing out the extreme amount of fluid in her abdomen. I would have to have a cesarean section to deliver the baby ~ she was just too big, and I had too much fluid. There was so much fluid in her abdomen that it was compressing her organs, and especially impeding the lungs. According to the ultrasound, the kidneys looked pretty good, but there was no way of knowing what was wrong. The thought was that it was probably a chromosomal defect. We would just have to wait and see. Although I was contracting, the baby was floating around in all that extra fluid and I was not dilating. I was allowed to go home, but with strict instructions to do NOTHING for the next 36 hours and return on Sunday morning for another NST. Following that, I was to call his office on Monday morning and schedule an appointment for an amniocentesis to check for chromosomal abnormalities.
Saturday was a long and stressful day. I was so uncomfortable it was hard to walk. Rob and Kira slept in the living room so I could have the bed to myself in hopes of making me more comfortable. Sunday morning, my mom showed up to stay with Kira, and Rob and I headed for the hospital, with the bags, just in case… When I arrived for the NST, the perinatologist on call that day, Dr. Zuidema, met me there. She informed me that she was pretty aggressive and saw no point in waiting. I was going to be admitted and I would be having an amniocentesis right then and there to check for lung maturity.
The amnio itself turned out to be less painful that I thought it would be. They made arrangements for a room for me on OB special care and there I made my home for the day, awaiting the results. Dana Boyd and Mikayla came to visit and my parents brought Kira up to see me. Dr. Shaw, a neonatologist, stopped by to discuss the potential seriousness of the baby’s health. He had no answers, and we had a lot of questions. He did leave us with the thought that the last baby he had seen with a lot of fluid didn’t survive. Scary to hear that, but we appreciated the realness he brought to the situation. The phone call finally came about 5:30 from Dr. Zuidema… the lungs were mature! Surgery was scheduled for the following morning and was to be performed by Dr. Cook. Rob and I hung out for the evening… he brought me KFC as my final meal… what a guy!
Most of the night I spent in misery… unable to get comfortable and contracting like crazy. I was anxious for morning to come. And while I knew I was having major surgery, I was more nervous about getting the IV… Monday morning finally arrived and it was a whirlwind of activity. Rob and I finalized a name for our soon-to-be-born baby girl, switching from Allison Kate to Allison Grace at the last minute. My parents and Kira arrived, as well as Rob’s dad and stepmom. They came to get me and off we went downstairs to begin the adventure.
My anesthesiologist was not only cute, but very reassuring and kind. Sometime after they walked me to the OR, Marikje arrived to wait with my family. The doc got me numb with a spinal and things started happening fast. They made their incision across my belly and then let Rob come in… the neonatal team was prepared and ready to my left. I felt pretty relaxed. At the point they were able to pop open my uterus and release the extra amniotic fluid I felt a great rush of relief as I was able to take a deep breath for the first time in weeks… it was one of the best sensations I’ve ever had. Within moments, Allison was born.
She was not able to breathe at delivery, as the fluid was severely compressing her lungs, but Allison managed to squeak out a small cry before being intubated. I wasn’t able to see her through all of the people they had working on her, but caught a 1/2 second glimpse as she was careened through the air and into the isolette. The neonatologist (who I later learned was Dr. Radecki) said “Here’s your baby, flying by!” They whisked her to the NICU where they “tapped” her abdomen and over the next hour she lost 2 lbs of fluid ~ a total of 3lbs in the next 24 hours.
It was a few hours before we were allowed to see her, and when we finally did, she was covered in tubes and wires and just looked so swollen. I was prepared, but it still took my breath away. I was able to hold her foot (I was still in my hospital bed and VERY sore from surgery) as that was the only appendage I could reach. Her nurses were Brenda and Tara. Allison was Tara’s first delivery.
It was testing upon endless testing, with still no answers… at one point they thought she may have Down’s syndrome, but that turned out to be wrong as well. After four long days, they finally discovered that she had a jejunal atresia, making part of her small intestine impassible. We considered this the best news we could ever hear ~ we had a reason and it was fixable. So on her fifth day of life, we sent our newborn baby girl into her first surgery.
Once they opened her up, they discovered even more complications, as she also had perforated (thus the cause for the fluid), a significant case of meconium ileus (the meconium in the bowel hardens and “plugs” the intestine), and volvulus (her intestines were twisted in an unusual manner). After removing the portion of her intestine that was encompassing the atresia, the surgeons felt they shouldn’t reattach her intestines at that point and created stomas ~ to be repaired in 4-8 weeks. It was then that Dr. Schlatter mentioned the ileus being highly suspect of Cystic Fibrosis. We were floored and adamant that our baby didn’t have CF! We don’t have anyone in our family with CF! This isn’t possible! They would do the gene probe, but it would need to wait until Monday to be drawn and then another week or so for the results. We waited and waited and waited. We ignored the cough. It couldn’t be true!
Needless to say, 18 days after Allison was born, it was confirmed that our baby did indeed have CF. The only thing I knew at the time was a vague recollection of “Alex” ~ after having read it as a young teen. All I knew is that Alex had it bad… and she died… young. Having that kind of knowledge is a dangerous thing as it allows your mind to run quickly in a direction that no parent wants to think. Fortunately, the neonatologist that broke the news (Dr. Shaw) called the pulmonologist (Dr. Schuen) from the CF clinic and he immediately came to talk to us. He basically painted a rather rosy picture of life with CF ~ it was sugar-coated and extremely optimistic. All we knew is that we would be needing to feed her these “enzyme” things at every meal and bring her to the “clinic” (a place I picture to be far more institutional than it really was) four times a year. He was also quite adamant to not believe ANYTHING we read on the net with the exception of the Cystic Fibrosis Foundation’s website. It was a few weeks after this time that she began her first lung infection (in CF terms it is called an exacerbation). No one really explained to us that she was “sick” ~ we assumed this was the natural progression of the disease and this is how she would always be. How ignorant we were! This illness delayed the surgery to reconnect her intestines by just over a week.
We eventually get the poor baby put back together and assume that we are on our way home. Ten days after surgery her wound dehisced and exposed her intestines. She got very sick and spent a lot of time on the ventilator. They were keeping her sedated to attempt to keep her still and her pain under control. Two days after her incision split open, they decided a surgical repair to the open wound would be the best way to treat it. She was in bad shape. She would go into coughing fits, trying to clear herself and would desat (her oxygen levels would drop to dangerously low levels) and turn horrible shades of blue while her heart rate would begin to plummet. They had to resuscitate her back several times. She eventually got past the worst of it and worked on recovering. They started drip feedings into her intestine (OJ) and although it took a while, she finally started pooping! It was a miraculous day ~ the nurse that night, Lani, made a sign for her crib and everything! We thought we were out of the woods. Once we could get her off the TPN (IV nutrition) and digesting, then we could get rid of the PICC (long-term IV) that had been in her poor little shaven head for weeks.
Then she started having more reflux problems than previously realized. Because of the risk of aspiration for her lungs, the consensus was that she needed another surgery for a Nissen (fundoplication) and a G-tube. This surgery takes your stomach, wraps it around the esophagus and tightens the invisible “valve” at the top of the stomach to prevent reflux. I was told it would be IMPOSSIBLE for her to vomit over it and that it would help this horrible cough she had. Sign here, please. Because of her history of poor healing, they worried about making yet another incision on her abdomen and we were fortunate that the surgeon who stood in for our regular surgeon (who was on Jury Duty) was all about doing it laparascopically. The only problem was trying to get through the scar tissue created by her previous surgeries.
They did encounter a few problems with her surgery (excessive, undetermined bleeding), but were able to complete it with the scopes. When I finally got to see her, I was horrified at how big the g-tube seemed (she came out with a button, instead of the “tube” we were told to expect and everyone thought we were so LUCKY!). That’s NOT how it was described to me… over the course of the next twenty four hours, it got much smaller in my eyes, and I had little problem with it. It was around this time that she also had a positive throat culture for pseudomonas (this is a dangerous bacteria for someone with CF ~ it can cause serious, chronic lung infections and once you get it, the thought is that you always have it).
The cough stayed, the three hours treatments (nebs and CPT) continued for probably 2 solid months. Toward the end of her stay, we got it down to every six hours. She never learned to eat orally, and developed a significant aversion to eating ~ all of her nutrition is via g-tube. We’re in feeding therapy, and working on getting her to eat, but it’s going to be a long, tough road. Like All Things Allison, she’s a fighter about EVERYTHING! Hopefully this bodes well for the future.
In total, Allison spent four months in the NICU (DeVos Children’s Hospital in Grand Rapids, MI) and came home the day after Valentine’s Day 2004. She was on oxygen and monitor and a drip feed (due to a leaking g-tube site) and several medications. Those first few months were overwhelming and incredibly difficult. We also had a two-year-old daughter and balancing a sick baby, a well toddler, finding a new house and moving was a bit much to take on. But take it on we did ~ and things are more settled now than they have ever been. She’s completely off oxygen and monitors, and we only use the pump for overnight feedings.
Allison has been readmitted to the hospital on four separate occasions following her release from the NICU. Two times in June of 2004 for unexplainable intestinal bleeding. Twice in September of 2004 ~ once for more bleeding, and once to resolve a possible bowel obstruction. The hospital will become a way of life for us ~ frequent doctor visits and intermittent hospital stays are the typical pattern for someone with CF. We have no idea what the future will hold for Allison, we have high hopes that she’s going to lead a very happy and fulfilling life… despite her disease. We love you, Allison!
Thank you for taking the time to read our story…
The Summer of 2005… and beyond!
Allison has had a rough summer. On the night of June 10th, 2005, Allison began coughing. We believed she was heading for a lung infection. By 10am the next morning, we had called the doctor and explained her symptoms. It almost seemed like a virus to her, but told us to watch her for increased fever, etc and to bring her into the ER if she was over 101. A few hours later, Allison was miserable, unable to tolerate any feedings, her fever was 103, and we began to get what appeared to be stool coming out of and around her g-tube. We rushed her to the hospital to be evaluated.
They were very concerned about her. Her oxygen saturation levels were hovering around 92-94%, so they added some blow-by supplemental oxygen. They started an IV to re-hydrate her, ordered a full blood panel, chest and belly x-rays. They considered a lot of different things (like meningitis, the need for a surgical consult to rule out obstructions/fistulas that may have formed) and knew that we would have to be admitted. She was put in the PICU overflow area, so that she could be closely monitored. Despite the Tylenol they kept giving her for the fever, it reached 105.3 at its highest. The blood tests came back with a lower white count, indicating that it was likely a virus. She simply needed time to get past it. Surgery looked at the x-rays and did not see anything that had formed that needed a surgical fix. So we waited. It took a few days, but eventually Allison was fever free and feeling better. As we started reintroducing feedings, Allison responded very negatively with an extremely distended abdomen (which x-rays revealed were full of air and they suspected a blockage) and severe discomfort. Things, again, weren’t looking great. It took a few more days, and some slow reintroductions of feedings, but after 9 days, we were finally able to bring Allison home, thinking the worst was behind us. She had lost some weight, but we were confident we could quickly put it back on her.
It only took four days for more trouble to start… Allison started having signs of belly pain and we called the doctor, asking for permission to try a laxative called Miralax (they had prescribed it for us, but told us to only use it with permission). We tried it, it didn’t work, and back to the hospital again… in the middle of the night. This was a short stay; as we were able to clear her out and get things moving again with some saline enemas. We were only in for about 36 hours. We were allowed to leave, knowing that we would be able to handle getting Allison back up to full feeds on our own; after all, we had lots of experience…
This was only the tip of the iceberg. By the end of July, she had lost over 6 pounds and was looking skeletal. She was constantly getting blocked up (something they attributed to DIOS ~ Distal Intestinal Obstructive Syndrome ~ that can be quite prevalent among people with CF) and was in serious pain. She vomited several times a day and was not tolerating feedings hardly at all. She needed help and we agreed to put her on IV nutrition ~ TPN (Total Parenteral Nutrition). The only problem was that she needed a central line in order to administer the live-saving solution. After carefully weighing all of our options (PICC, Broviac, or Port), we settled with a port. It would have to be placed surgically, but would give her a semi-permanent IV site that could be used for the next few years and would virtually eliminate the need for peripheral IV’s (in her arm or elsewhere). We happily sent her into surgery (on Rob’s 30th birthday!) and she came out, ready for TPN. After a few more days in the hospital and after we learned how do use her TPN pump for at home, we left the hospital. The next several weeks went fine, she was getting small g-tube feedings and a vast majority of her nutrition was coming from the TPN. (The drawback to using TPN is the fact that it is very hard on the liver, an organ that didn’t need any more abuse than it had already suffered.)
Then the infections began. She had port infection after port infection. She was on IV antibiotics (both at home and in the hospital) constantly, on top of the TPN and the g-tube feedings. It was a very grueling schedule. By mid-December, we finally declared her port “colonized” with a staph infection and it had to come out. The problem was, we didn’t really know if the constant IV antibiotics were now a major contributor to her belly issues, or if it was still a feeding intolerance issue. We agreed to try to go it with g-tube feedings only and chose not to place another central line until we could get a good sense of what was going on.
We came home on 24 hour drip feedings through her g-tube, and Allison did okay for a while, and then we would do tremendous backslides… very disheartening. We managed to make it through Christmas, but the Friday night before New Year’s I knew that we had to cry “uncle” and called the doctor. We had exhausted all of our efforts at home that were within my comfort zone and she was still continuing to deteriorate. She was vomiting, not pooping well, and was in a tremendous amount of pain. After a brief discussion with the Dr. I agreed to bring Allison in to be admitted.
The x-rays were suggestive of a blockage in the area of her intestines that they would commonly consider to be a DIOS episode. We gave her some Go-Lytely to help clean her out and then eventually started to try to feed her again. Within an hour of starting her feeding she was in pain and I knew that something else had to be done. So we gave her a gastrograffin enema that they can see on x-ray down in the radiology lab. They didn’t see anything they suspected they would and suddenly became puzzled. Over the next several days, we finally came to the conclusion that Allison needed to have exploratory surgery to determine the root cause of this problem. We’d never been able to capture anything on film that told us if anything was wrong, and it was a leap of faith for us, but we agreed to the surgery. SOMETHING had to be done to help this poor baby… The contingency plan was to go ahead and place a central line of some sort during surgery to start giving her TPN, as she had only had IV fluid for a week and pedialyte for a few days before that. The kind of central line (temporary or more permanent, but NOT a port) would be determined by the surgeon as a result of what he found on the inside.
The GI doctor said he thought that “maybe” they’d find a small kink or something… the surgeon was confident that he’d be able to fix it, whatever “it” was… the GI doctor said “Yeah, but he’s a surgeon ~ he thinks he can fix anything…” So, blindly, we handed her over to the surgery team and went to wait in the waiting room. They had slated about 3 hours for surgery, but the surgeon had assured us it likely wouldn’t take that long at all. After about two hours, they sent out the scrub nurse to fill us in on the progress.
She said they had found “a LOT” of adhesions and were finding the surgery difficult. It would be a few more hours yet. A few more hours passed by and she returned again… another few more hours. Still working on a ton of adhesions. About six hours after surgery began, we saw the surgeon walk in and he came back to us with astonishment all over his face. He was amazed that she had ever been able to pass anything through there at all. He found kinks, narrowings, and adhesions cemented EVERYWHERE. He had inadvertently injured her gallbladder while removing adhesions and had to repair that, so he put in a JP drain (looks like a grenade) to watch for any bile leakage. He was confident that the adhesions were the reason for her recent GI troubles, and so only placed a subclavian central line to allow for short term TPN usage. She had remained stable throughout surgery and would be returned to the regular peds floor after a short stay in the recovery room.
We were elated that surgery was over, he had been able to address her very severe GI issues and that this seemed to be the answer. We sent everyone who had come to wait with us home and we waited to be able to go see her in recovery. The anesthesiologist came out and let us know that she needed some extra help to get stable after surgery, but we should be able to see her in about 20-40 minutes. So we waited an hour and finally called PACU and they hesitantly agreed that we could come back. It had been two hours since surgery, and her blood pressure was finally UP to 40/30 and her heart rate was soaring around 200bpm. Allison’s respiratory rate was 12-16 breaths per minute and she was receiving several liters of oxygen by mask. She was rather unstable, needed blood, among other things, and was going to be admitted to the Pediatric Intensive Care Unit.
Hopefully you’ve never had to witness the well-oiled machine of an intensive care unit, but I can tell you that it was phenomenal to watch them all work as a team and quickly get her everything she needed. It was AWESOME! I felt so good knowing that she was getting the best possible care. After 24 hours, she was much more stable and in 48 most of the major concerns were behind us. She ended up spending 4 days in the PICU before being transferred back to the floor.
I’m ecstatic to report that she’s doing FANTASTIC since the surgery. No IV’s, no TPN, nothing other than her regular g-tube feeding schedule and overnight drip. She’s not in pain; she’s happy and even began eating orally (a little). She’s an entirely different child. I am so proud of her!
There’s nothing so frustrating as being a mother with a child who needs help, and not being able to find a reason behind the pain. I know that many people felt as though I should run away from her doctors as fast as possible and seek a second opinion, but I just wasn’t comfortable doing that. We are working with the best team of doctors where we live and they are fabulous. They all care, they work together wonderfully, and they all did the appropriate tests, etc., but nothing was ever “typical” diagnostically that would allow us to see what the issue was. Different doctors elsewhere would have run the same tests and seen the same results. The surgeon just told me the other day that the Textbook says there is around a 5% chance of adhesions causing issues such as Allison’s in someone with previous abdominal surgery. Well… Allison’s rules state that she MUST fall within the smallest percentage of possibilities at all times, or life just wouldn’t be interesting.
Yes, there is a chance (surgeon estimated perhaps around 20% lifetime) that this could happen again, and given that there is ANY chance at all, it likely will happen for Allison. This isn’t pessimism, just the reality. Next time, we will know what more to look for and hopefully she won’t have to suffer as long.
This is obviously the abbreviated version of the story, for more details, please feel free to ask!
December 2006 Update
What a year it has been! Allison has not been admitted to the hospital for nearly an entire year and we could not be more proud of how far she has come! We are still riding the roller coaster of CF, but it has been a blessing to find that her third year has been her healthiest thus far.
An ultrasound in February 2006 brought us some disconcerting news about her liver: she has something known as portal hypertension. Most generally this is found in older CF patients (and is not CF-specific), but does occur in the younger set as well. Her liver function is fine (high normal), and we are just watching for now. The thought in February was that her liver was still irritated from the nearly 100,000 mLs (think 3300 ounces) of TPN she received in 2005. Her spleen was enlarged and we were concerned about a bulging vein in her esophagus (side effect of portal hypertension called esophageal varices) that was discovered during some endoscopy when she received her port in July of ’05. Later (April 2006), another endoscopy showed no progression of the bulging vein, a good sign. However, an ultrasound done in November of ’06 showed that her liver was the same, indicating the portal hypertension was likely permanent. What this means for Allison: it could several years for this issue to progress to something we need to do something about. For now, we just keep tabs on what is going on and track any changes that may be occurring as a result. We hope to not have to deal with any more severe liver issues for many years to come.
We are also in the middle of tracking some sinus problems to see where they may lead us (CF’ers have a high likelihood of severe sinus issues). Allison does require sinus surgery in the near future and we are hopeful that this will not need to become a frequent occurence.
It is not all negative, I promise. Allison is very smart and knows her letters, colors and shapes. She is working on Spanish and sign language. She is now fully potty trained and doing very well at it. Allison loves to giggle and play. She is an absolute joy to be around!
Thank you for taking the time to check up on us!
January 2008 Update
My, what a difference a year makes! Allison continues to grow and thrive, not without some continuing issues, however. She did indeed have not only one, but two sinus surgeries in 2007. The first, in February of 2007, was a relative breeze for her and while she got some relief, it wasn’t apparent for very long. She ended up back in the OR for sinus surgery #2 in August ~ just six months later. The second surgery was a bit rougher on her and they removed her adenoids as well. The recovery was a bit slower, but we did see some positive improvements. However, only four short months later, we are seeing the potential for even more sinus surgeries in the near future.
She is slowly learning to eat more and more orally ~ and has mastered the art of enzymes and applesauce (dipped in oatmeal or bananas, of course). We have a tremendously long way to go, but she’s definitely getting the hang of it.
Recently (starting the week before Thanksgiving and continuing into the New Year), we’ve had some concerns over some pain she’s had in her tummy (contributing to decreased ability to tolerate her g-tube feedings, thus she lost weight ~ nearly 4 pounds) and some minor blockages. We had a consultation with her surgeon and ran a few tests. As of now, she remains stable, and overall doing well, but the whole medical team is now on high alert as we watch for potential signs of a returning adhesion issue for her. In an effort to avoid the prolonged trauma of the guessing game we played in 2005, we made a plan with the surgeon about what the next step would be if the pain were to return to previous levels ~ and we are comfortable with what has been laid out. Here’s to hoping that we don’t need to use it.
Overall, Allison continues to be a source of constant joy and continual amazement. Take a moment to be thankful for the blessings in your life ~ just as Allison has been such a blessing to ours!
January 2009 Update
As another year has passed, I find myself marveling at the changes we’ve encountered over the last twelve months. Allison continues to thrive and grow in her personality and we enjoy every moment of her hilarious antics. She is learning to read and write and loves to draw. Rob and I often joke about how good she is on the computer and with all the electronics… she’s obviously taking after Rob in that regard! Allison is stubborn, and adorable and breathtakingly sweet all in the same moment. She spent the most of the year in relative good health. We were able to get in some good camping trips and spend some time as a family and with friends. She absolutely loves being outdoors!
In mid-July, I took Allison in to see the Developmental Pediatrician that runs the local feeding program. Once again, she was approved to be a part of the program and was referred to Mary FreeBed to work with the outpatient program. We started out attending therapy three times a week and eventually went down to one, until eventually we were released from the program (as she had met all of her goals for oral motor skills — not for actual eating). We have been referred to the intensive feeding program and have been given a start date of sometime between April 30th and June 30th. This means that she will attend a behaviorally based program 5 days a week, 8 hours a day, for 6.5-8 weeks. We are hopeful that this will help Allison learn to chew and swallow a variety of foods to help her grow and develop like her peers. The underlying issue here with Allison is NOT that she WON’T eat… it is that she just doesn’t know HOW. Trust me, she asks for and gets whatever she wants in the way of food ~ she is MORE than interested. We just have to teach her how to chew and swallow in quantities that will sustain life.
As fall progressed, her sinuses became worse and worse, and her snoring was atrocious. We tried a number of things, to no avail, and it was apparent that she needed to have another sinus surgery. Before we could get to that, however, in mid-November, I took Allison to her regularly scheduled CF clinic appointment. While talking about things with the doctor, I mentioned that she had been suffering from some minor, intermittent back pain. Concerned that she may have kidney stones, the doctor ordered an ultrasound, and also added in her liver, gallbladder and spleen. The ultrasound was the next week and within 2 hours of the ultrasound, the doctor called me on the phone. It had been discovered that the main portal vein in her liver was blocked with a blood clot and she would need to be hospitalized immediately for assessment and treatment. After a multi-specialist consultation and a few tests, we agreed to start Allison on an injectable blood thinner that we would have to give her twice a day. This initially proved to be a heartbreaking challenge, but like with all difficult things, we all have become accustomed to the ritual and at this point, she doesn’t even cry anymore when we poke her. She is one amazing kid! This treatment will continue for 12-16 weeks with the hope that we will be able to entirely resolve the clot.
There remained a question in our minds about “why” this happened to begin with. A person with Cystic Fibrosis is more prone to naturally have “thinner” blood as they tend to have trouble absorbing key vitamins that regulate blood coagulation. The Hematology docs ordered a familial blood history and sent it to Mayo for analysis. It came back to show that Allison has a genetic blood condition called MTHFR (methylenetetrahydrofolate reductase) which means she is susceptible to higher levels of homocysteine in her blood which causes coronary artery disease, heart attack, stroke, and blood clots. However, her homocysteine level is only a 2, on the lowest end of low. The MTHFR played no role in the formation of this clot. Another logical conclusion was that her portal hypertension was getting worse. We did some research and looked a few hospitals to see what we could find out about getting a second opinion.
I took Allison to Children’s Memorial in Chicago. It was an interesting trip and I got a lot of my questions answered about the relationship of portal hypertension, cystic fibrosis related liver disease, cirrhosis and liver function. Without doing any invasive testing, the very experienced liver doctor there felt Allison’s abdomen and shared his opinion on what he thought her future might hold. He was very confident that she has cirrhosis, that her body has found ways to compensate for now, and that she would eventually need a liver transplant as a child. Now, I can understand that this information might overwhelm you as you read this, but please understand that this is information that on some level I already anticipated hearing, although we had never even broached the subject here. He suggested a few tests that could be done to more thoroughly assess the liver, but didn’t feel that anything was urgent. His opinion behind the blood clot was that as the pressure in the vein became great greater, the blood started to become stagnant, and formed a clot. It is just about the best answer we have. We do not know what this means for the future.
We recently did an endoscopy to check to see if the effects of her portal hypertension were becoming a problem in her esophagus, and I’m ecstatic to report that her stomach and esophagus looked better than they did two years ago. Our doctor here will be consulting with the Chicago doctor to plan out next steps, if any need to be taken, and meanwhile, we just enjoy living!
On December 29th, Allison had her much needed sinus surgery, the third in her lifetime and the 11th overall total surgery she’s ever had. Her recovery was quick and she’s doing very well overall. We are continuing to do her shots for the next several weeks and will be doing some follow up ultrasounds as well to check the blood flow through her liver.
December 2010 Update
What in the world have we been up to? Well… it’s a long story. You might as well pull up a chair.
We went into 2009 with the the never-ending blood thinner injections and while it seemed to be working very slightly, it was taking way too long! Early in March, Allison wasn’t feeling well, was pale, and then had a huge GI bleed (discovered in the bathroom), for which we rushed her to the hospital and had her admitted. This happened three more times in a few short weeks. We would stop her feedings when she would bleed, it would stop. We took her home, fed her, and within a few days she would be bleeding again. It was a very frustrating cycle. We could never pinpoint a source of the bleeding, despite valiant efforts made to get her through nuclear medicine scans and endoscopy at first signs of bleeding. These were not minor bleeding episodes… these were bleeds that required several blood transfusions. By the end of March, we were out of options, and the doctors made arrangements for us to take a medical flight out to Children’s Hospital Boston.
There are a bazillion details to this part of the story, all of which can be found by catching up on our CarePages for this time period. However, the Cliff Notes version goes something like: It was discovered that she never actually had a blood clot in her liver, her portal hypertension was off the chart SEVERE and it was causing the build-up of pressure in the veins of her lower intestinal tract, and it required some serious intervention. At the end of the day, we had to make a choice between a liver transplant or a liver shunt. For practical reasons, we chose to try the shunt surgery. It worked… and has continued to work for over a year now. We spent more than 6.5 weeks INPATIENT at CHB and finally arrived back home in May of 2009.
From then on, Ally’s health has been better, but not without its challenges. Because of her new liver blood flow, we have to be careful to keep her protein intake on the low end. We also had a few repeat bleeding episodes that led us to believe that she may have a milk allergy to her formula. The change in formula gave us a problem with hypoglycemia… her sugar levels would drop dangerously low (for those in the know… less than 15 at times!!!) and they dubbed it Dumping Syndrome. We ended up back on a 24-hour-a-day drip for several months. Her liver numbers started doing really crazy things, and this past June, we took her back to Boston for a 9-day hospital admission to evaluate her liver numbers and address the hypoglycemia issues…
Her liver was fine and the shunt is still working well… too well, in fact. But, she was getting too much protein, and this may be why her liver was doing weird things with the numbers. We changed her formula back to what she had been on previously, added cornstarch, and put her back on a bolus schedule… and voila! She is doing VERY well with it! However, we did do a follow-up appointment with the allergist here in Grand Rapids and discovered that while she has a sensitivity to milk, she is really allergic to eggs and we now have an Epipen for emergencies. If it’s not one thing…
She never did make it into the feeding program and we have decided for now that she will eat what she eats and go from there. There aren’t too many other choices for now. Allison recently celebrated her 7th birthday and is enjoying playing with our goats, our cats and kittens, and doing riding lessons on Festus the Pony! She is still being homeschooled, but is on the waiting list for a school near us, and when they have an opening… SHE IS GOING! LOL
Thank you for all of your support! I will check back in soon!
This is long overdue…and I apologize for that… but I find it hard to write about this stuff…. so I decided that
One of the most difficult questions ever posed to me is “How is Ally doing?” The easy, short answer is to say “she’s doing okay…” I mean… the reality is how long do people want stand there and listen? The dichotomous bigger picture is one of the hardest things to reconcile. She’s running around, playing with American Girl dolls and begging me to iron her latest perler bead creation while looking up Grumpy Cat memes on Pinterest and trying to talk like a minion. She’s crazy and fun and one of the shyest kids you’ll ever meet. But for 8 weeks this summer, she was also sporting a massive IV line in her upper right arm (called a PICC line – Peripherally Inserted Central Catheter) that we used to deliver high doses of antibiotics designed to kill the bacteria that live and thrive in her lungs. Now, “normally”… this process is a 2 week stint in the hospital and then life goes back to status quo…. but we all know this is Ally, right???
Over the last several years, Ally has been maintaining her own level of status quo. She does several rounds of oral antibiotics until we move on to the more intense, IV delivered ones; she’s no longer a 2-week and done sort of gal…. she’s a high achiever needing at least 3 or 4 weeks, usually (although this current round was excessively longer). We do several (3-4 breathing and vest) treatments a day on the regular. She’s still getting 17 medications a day, on average. She remains entirely dependent on g-tube feedings; some because of her hesitation to eat orally and some because of the stability offered by delivering a specially formulated diet that has the right ratio of protein to fat to help control the level of ammonia in her bloodstream that her liver is no longer able to process (from the shunt we had placed several years ago). Even with the special liquid diet, she struggles to gain and maintain weight (especially while sick) and remains on the
We had her PICC line pulled just yesterday, as 8 weeks is a long time to have one and it made her infectious disease docs very nervous. Also, trying to avoid forming a blood clot…. so while she’s not 100% better in terms of her lung health, the risks were starting to far outweigh the benefits. We switched her over to some oral antibiotics; the caveat being that they make her far more sensitive to the sun. Always seem to be trading one issue for another. But on the upside… she can now get in the pool (and avoid the sun?!??!?!).
There has been a lot of buzz about the latest and greatest CF drug – Orkambi. Orkambi is the 2nd in a class of drugs designed to help control CF at the cellular level. Kalydeco was the first, and has shown tremendous results in those with a particular mutation of the disease (about 4% of the CF population). Orkambi is a combination of Kalydeco with another drug that works on the most common mutations of the disease (of which Allison has), and while a very far cry from a cure, it’s the best thing we have to help control the hallmark symptoms of the disease. (Orkambi has not been shown to be as impressive as Kalydeco with symptom reduction, but has demonstrated a significant decrease in the number of pulmonary exacerbations – and that’s awesome). That said, there are a few parameters that qualify you to be able to take this “miracle” pill – you must be 12 years old. For Ally, that point is basically moot as she’s less than 3 months away from turning 12. You also should have good liver health. Well…. her docs are concerned, but still willing to forge ahead and try it, with lots of monitoring. Finally, it’s contraindicated with a few other drugs she’s currently taking to battle the war being fought in her lungs. So… we have months before we are even able to contemplate her taking Orkambi.
Well… and there’s that small matter of cost. Orkambi will cost $259,000… a YEAR! Yes…. it’s math time! Get out your calculators, folks… a typical dose is 4 pills a day – which comes out to $21,583 a month, approx $719 a day… and about $180 a pill (likely the most expensive breakfast you’ll ever have – $360 – before you’ve even gotten to the smoked salmon eggs benedict topped with caviar). Yes, insurance and patient assistance programs will cover the vast majority of this, but for those working in small business, the hit on pharma costs could be devastating.
This fall, Allison will start the 6th grade… I have two middle schoolers!!!! As unfathomable as this may be, we are so proud of her scholastic ability and not using her CF as an excuse to slack off. She’s a stellar student and a great friend.
It’s hard to decide what to share, or how specific on the details to be… there’s a fine line between providing information and sounding like we are complaining about the demands that CF has put on our lives, not to mention exposing the vulnerable parts of her/our life. There is no room for defeat, nor do we ask for pity. We simply do what we need to do to get done what we need to get done while we continue to live life to the best of our ability. There are molehills and there are mountains; we keep our hiking gear handy. Ally’s just like every other kid out there… with a few extras. So when you ask how Ally is doing… be prepared to pull up a chair – this is going to take a while.