Sarah Flukinger
USA
“Ma’am, ma’am, wake up. We need to talk to you. Ma’am. Ma’am.”
I woke up and looked at the clock across the hospital room. It was 2:30 in the morning. I was still very out of it from all the anesthetic. I had to be put under general anesthesia because my epidural was crooked and I could feel everything. I had only been awake a little while earlier, around 10:30, to get a first glimpse at my new baby boy and to try and breastfeed him. He looked entirely perfect to me at the time. He fed terribly, but he was also groggy since the medicine did a number on him as well.
But now it was 2:30, and the neonatologist’s voice was very urgent. “Ma’am, we need you and your husband to sign these papers to give us permission to move your son to the Neonatal Intensive Care Unit to begin blood transfusions.” WHAT?
The neonatologist started talking about how when my son come out via c-section, his head had several large bruises on it (I didn’t see them since he had on one of those stocking caps) and that he was covered in these red and purple spots called Petechiae, which I would later learn means there is bleeding under the skin. They ran a CBC (Complete Blood Count), which they don’t normally do, but due to his appearance the doctors felt it was necessary, and discovered that he had a really low count of platelets; the white cells that help you clot blood and keep you from hemorrhaging. His count the first time was only 8,000 and the doctor thought it was a bad reading, so they took another vile of blood and sent it off, to discover it had dropped quickly to 6,000. Now, they were frantic to get him moved and start platelet transfusions. Of course, we immediately signed the consent forms, but we mainly wanted to know why this had happened in the first place.
The next few days were a blur to us. Jay, our new baby boy, would receive a transfusion, his counts would come up a little bit, but never over 50,000, only to plummet later in the day. The neonatologist was desperate to get him over 50,000, because as we learned, later, he was at a major risk to start bleeding out in his brain. He contacted the Pediatric Hematology/Oncology clinic at Texas Children’s Hospital in Houston. They suggested running platelets that were straight from aphaeresis (in other words, they were donated as only platelets and not taken from a whole blood donation.) They also ran a medicine to him called IVIG (Intravenous Immune Globulins.) That seemed to work much better, and on discharge day, he had gone up to just at 116,000 platelets. This was still a little low, as a healthy range is considered 150,000 to 450,000 but it was much better than 6,000!! Jay also had an ultrasound on his head to rule out any damage to his brain and he checked clear, thank goodness. But the main problem is we still had no clue what had caused this whole thing in the first place!!
We went home on a brilliantly hot, sunny Sunday in August. We had to go to the Pediatrician in town to have him looked at and run another CBC that Tuesday. To our dismay, his counts were back down to 65,000. We were shocked, and scared. We were new parents and there was something really wrong with our baby. Why couldn’t we just enjoy having a new baby? What was this life threatening condition? The next day was Wednesday and we had an appointment at the Hematology/Oncology clinic at Texas Children’s Hospital in Houston.
No one up until this point could tell us exactly why Jay had such low platelets. They said it could’ve been infection, which they had ruled out. They also said it could have been something from me, because I also had a low platelet count after delivery. But they were confused because even though my platelets were low, they weren’t so severe, and how could that have caused his to be low? But then we met Dr. Yee and he had an explanation. He said it was a genetic disorder called Neonatal Alloimmune Thrombocytopenia. He said that basically, my husband and I were incompatible on our platelets. It was rare, and wasn’t seen very often, but that before we decided to have any more children, we would need to have our bloods tested to see if this was in deed the cause. But he thought that maybe since I also had gestational Thrombocytopenia, this could’ve been the reason for Jay’s low counts and so we just went with that answer, that it was an anomaly. My delivering OB even went so far as to tell me that the idea that we had NAIT was absurd and that I could go on to have a healthy second pregnancy and just be closely monitored.
So we went on with our life, and around 9 months after his birth, we started getting the “itch” again. We enjoyed having a baby, he was over the difficult newborn period and gosh, he was so cute!! But I told my husband, “Remember what Dr. Yee said? He said we should get that test! Honey, I can’t handle if something really bad happens to the next baby. I can’t just hope for the best.” So I started reading up again on NAIT. I scoured the internet for answers, and to my surprise, there wasn’t much out there on the subject. It seemed like it was so rare. I dug out the old paper work from Dr. Yee and gave him a call. By this time, my oldest was 15 months old. I told him that we wanted to add to our family and that we were planning to try after Jay turned 2, but we wanted to get the testing. After trying to get the testing coordinated out closer to where I live and not having much success, Dr. Yee helped me get in contact with a Maternal Fetal Medicine doctor at Baylor College of Medicine named Dr. Kenneth Moise. He’s often referred to as the Blood Man.
We went to see him for the first time I February of 2009. Looking at our history, he told us that he was about 99.9% sure that the cause was NAIT. What he didn’t know was the likelihood of it occurring again. All of that was dependent on my husband. I, more than likely, lacked a certain antigen, or protein, on one of my platelets. If my husband had both proteins, that meant we would have a 100% chance of it occurring. If he had only one of the proteins, that meant we had about a 50% chance of it occurring. We would need an amniocentesis if we were in this group when, and if, we decided to have another baby. They took blood from both my husband and myself and sent it off to the
Wisconsin Blood Center. Then it was a waiting game. Two weeks passed and we heard nothing. Then Dr. Moise called and said we had to go back in because they lost my husband’s blood!!! So we went all the way back to the Med Center for another draw.
Finally, two weeks later, the results were in. I was homozygous HPA 1B/1B. Only 2% of the world’s population is estimated to have this rare typing. Hubby was homozygous as well. He was HPA 1A/1A. He had both proteins. This meant that without a doubt, if we decided to have another child, this baby would be in danger from the moment of conception.
With such a close call with our first child, many people told us to be done. “Count your blessings. You have a healthy baby. Move on. One and done.” Oh God, how that hurt us, especially me. I would make Pro/Con lists as to whether we should have another baby. There were lots of pros and a whole bunch of cons. It felt like I had lost the dream of having another baby. It just weighed so heavy on my heart. Around the time of getting our test results, I found solace in an online support group. It was such a relief to talk to other parents that knew EXACTLY what we were going through and who had gone through treated pregnancies. I finally felt a glimmer of hope, that yes; we would conceive and have a healthy baby, but that I would have to literally put my body through Hell to achieve it. It was just a question of how badly did I want to do it? Was I really ready to mentally go through this, knowing the end results could be catastrophic?
Well, we decided to lean on our faith and to let God decide if we would have another baby. Jay turned two on August 15, 2009. On September 4, 2009, I took a pregnancy test and it was positive!! My elation quickly turned to fear and uncertainty. There was no way out of this now. I was either going to welcome a child into the world, or I was going to lose a child to my body’s own devices.
People were overjoyed for us, but were cautious as well. However, in their minds, the first few weeks were the scary period. (I guess since people think of miscarriage in the first 12 weeks of a pregnancy.) But all of us NAIT mothers know the truth. There is no safe point in a NAIT pregnancy. Your baby can appear fine on the ultrasound one minute, and quickly bleed out in their brain that day and die. I literally held my breath for 37 weeks.
The first twenty weeks went by with a feeling of uncertainty and little control. I wanted to start these IVIG treatments that I heard all about. I wanted to feel like I was doing SOMETHING to protect my baby. Finally, at 19 weeks, we saw the anatomy scan. We knew we were having another boy and that so far, he wasn’t showing any signs of a bleed. We started treatments at exactly 21 weeks on Friday, January 1, 2010, New Years Day. I had the first two treatments in the hospital back to back and reacted terribly. I had the worst headache I’ve ever experienced in my life, nausea, vomiting, the works! Dr. Moise prescribed my treatment to be two IVIG transfusions a week and at 32 weeks, to add Prednisone on top of all of that. IVIG is a blood product, made from over 1,000 blood donors’ plasma. So you can imagine when you are getting that much blood product that often, it really makes you feel horrible. Also, starting at 19 weeks, I went in to Dr. Moise every two weeks to have a major ultrasound on the baby to check his brain, abdomen and spine for any signs of bleeding. It was always reassuring to see him, but also nerve wracking wondering if they would find something bad.
As the weeks ticked by and my family and friends started to feel more confident, I felt even more terrified. I distinctly remember having a meltdown about 10 days away from delivery when my doctor’s office was readying everything. They were trying to determine if they could harvest my platelets or if they would need to find a donor. My doctor’s wife and nurse, Karen, called to tell me that they couldn’t collect my platelets because I had again, as I had with Jay, developed Thrombocytopenia. I freaked. If IVIG was supposed to help maintain platelet counts and mine were plummeting, what was happening to the baby? Trying to maintain my calm for those next few days was really hard.
I was scheduled to have an amniocentesis on Friday, April 23rd at 7 am to determine if the baby had mature lungs. I would be 37 weeks to the day. If he had mature lungs, they would schedule a c-section delivery for the following Monday, April 26th. However, they made sure that they had donor platelets on hand in case something went wrong during the amnio and we had to get him out then.
The doctors had to perform the amnio in the Labor and Delivery Triage area because all of the Operating Rooms were booked. It was really full, between it already being a tight space, and then adding a Maternal Fetal Medicine doctor, two nurses, an OB/GYN, my husband and my very pregnant self. Tension was high as they were looking for a good pocket of fluid to insert the needle into. Dr. Moise offered to perform the amnio since it was such a tight fit, being as that I was 37 weeks pregnant. Karen helped me control my breathing so that I wouldn’t flinch and cause Dr. Moise to hit the baby. He quickly inserted the needle and drew out the fluid. It actually didn’t hurt a bit, just a bit of a strong contraction. He immediately announced the fluid was mature, but sent it off to the lab to get the exact number. I looked at him and said, “Well, if it’s mature, can we just have him today? I can’t stand it anymore. I want him here. I want him safe.” Dr. Moise said he would talk to all the doctors, make sure we still had fresh platelets and see if we could get into an OR.
At about 10:00, Dr. Ivey, my OB/GYN came into the triage and asked if April 23rd sounded like a good day to have a baby!! We were thrilled that he would be born that afternoon!! I was starving to death, as I had not eaten since midnight the night before and finally at around 2:00, they came to get me ready for surgery. The OR was so cold. I was shivering and of course, my nerves didn’t help. The nurses were so busy getting me all prepped, they almost forgot to get my husband in the OR!! As my husband entered, they were already opening me up. Dr. Ivey was busily instructing an intern on the do’s and don’ts of c-sections and apparently, my previous c-section left much to be desired.
At 3:42, I heard the most incredible sound. Dr. Moise was up by my shoulders, talking to Jeff and I the whole time and then came this loud cry!! He was crying before he was even out of my uterus!! He was so mad to be out of me!! He looked incredible!! Very small, since he was a wee bit early, but gorgeous all together!! Dr. Moise said, “Oh, he’s going to have great counts!! Look how great he looks!” I cried a cry I’ve never felt before. It was so much emotion wrapped into one moment. The joy, the relief, the fear, the pain, all of it rolled into one. It was THE most intense moment of my life.
They showed him to me and then quickly whisked him away to get his blood for a count. Dr. Moise went back with them to see what his APGAR was and find out the platelets. He had been teasing me earlier, telling me that I would be pregnant again within two years and going through it all again. I laughed and told him I doubted it. I really felt complete, but then again…..if the baby had good counts, maybe I could go through it all.
A few minutes later, I was joined by my husband and Dr. Moise. The counts weren’t good. Not by a long shot. After twice weekly IVIG and heavy Prednisone, the baby only had 37K platelets from his cord and 21K from his actual blood. He was in the severe range and at a high risk for a brain bleed, if one hadn’t already occurred. They brought him to me in the incubator so I could touch his little hand and say good bye until I could go to the NICU after I recovered and they took him to start his transfusion to get him out of the danger zone and to perform a head ultrasound.
Dr. Moise sat down by me again and said, “Well, that’s that. Don’t have any more babies. Treatments didn’t work as well as they should have.” I thought to myself, ‘Well, it’s nice when God can give you a nice, clear sign without hurting you too deeply.’
The baby was in the Level 3 NICU at Texas Children’s Hospital. That is the highest NICU in Houston, probably even Texas. The nurses were in contact with me the entire evening. By 11:00, his count had risen to 187,000 and his head ultrasound came back clear of any bleeds. He was totally okay and out of the danger zone. At that point, they were just going to monitor him and make sure he could maintain his counts.
Over the next few days, his platelets went up and down, but never went below the danger zone. It was a rough hospital stay, but nothing compared to Jay. We were out by the time the baby was four days old, and that last night, I even got to experience what normal mothers experience – rooming in!! They had discharged him from the NICU and moved him to the regular nursery. It was nice to have that normal experience, even if it were only for a day.
At the end of my experience, I felt stronger and wiser. I had put my own health and life at risk and knew, going into it, that I was putting an innocent baby in danger as well. Unless you’ve walked a mile in the shoes of a NAIT parent, you can never understand it. It’s a unique situation. Even more unique than other disorders, because even when you’ve had a genetically abnormal baby, the chances are good that the next baby will be fine. With NAIT, your only guarantee is that your baby will be fighting from minute one. We make hard decisions throughout our pregnancy in regards to treatment that may or may not put our babies in greater danger. But we, the mothers, are strong. We are fighters, and so are our children. We were chosen for this, for better or for worse, and I am better for knowing these mothers and for being a part of this journey.
Sarah