For those less familiar with our story, you can visit Jayson's blog- Little J's Journey. Because our story is about to change in a very big and exciting way, we saw it fitting to start another blog. This blog will focus less on Jayson's medical journey and more on our new family of four.
When Mike and I married and discussed our plan for the future, we used to say we planned on having four children with the possibility of an accidental fifth. Mike comes from a large family of nine children and I had two other sisters, so four to five kids seeemed somewhere in the middle. But when we struggled to conceive our first and rely on modern medicine to be blessed with one miracle, that number quickly dropped. But when we saw the challenges our only son had to face and would continue to battle his entire life, we no longer talked about the future of our family. We lived in the moment. We focused on today, because there were times we feared today might be the only time we have. As our warrior boy battled on and conquered everything in his path, life began to calm down and we established our new normal. Dozens of hospital stays a year turned into a couple. Life became more manageable and we began talking about the future again. But there was still a couple of very large challenges in discussing the possibility of growing our family. We still had fertility concerns and clearly there were concerns about how we could possibly manage another child when our days were filled with caring for Jayson's many complex needs. But our biggest concern was the possibility that we could pass Jayson's undiagnosed genetic syndrome on to another child. We had no idea before, during, or immediately after pregnancy that Jayson had a genetic mutation. But now that we know that he does and all of the many organ systems it affects, there was no way we could ever take the chance of passing that on to another child. So we were stuck. We were open to adoption, but that was so complicated and expensive. LDS family services dissolved its adoption program. Many foreign adoption agencies eliminated us as potential adoptive parents simply because I've been on antidepressants to cope with the difficulties of the past couple of years. I had read many horror stories of shady local adoption agencies, and even more stories about legitimate ones that cost far more money than we could ever raise. I joined support groups for adoptive parents where I learned more often than not a family goes through a failed adoption before they encounter a successful one. With that comes the loss of thousands to tens of thousands of dollars and intense emotional scarring. Our journey was already so emotional, I didn't think I could bear the pain of a failed adoption. And due to medical expenses, we were already in debt and had no idea how we could fund an adoption or recover from a failed one. We looked into the Foster to Adopt program with social services. They came to our home to meet with us and start the process. It was then we discovered this was not an option for our unique family circumstances, despite how badly we wanted it. They needed us to be open to more than one child and explained that these children would likely come to us behind or completely unvaccinated and would have a long list of medical needs. We would need to be available to take them to regular doctor and dentist appointments for the first few months they were with us. We would also need to be available for several hours every week to take them to their family visits. What would I do with Jayson during all of these doctor appointments and home visits? He has to be in the care of a nurse, which is expensive to leave him. How would I continue to work and provide for our family if we add dozens of doctors appointments to our already full schedule with Jayson's doctors? Can I honestly bring other children in our home who may carry illnesses that could threaten Jayson's health and well being? There were too many concerns. This was not the right fit for our family. So we found ourselves without options. It was painful to accept, but Mike and I clung to each other and began to process and accept the idea that Jayson would likely be our only child...
In April of last year, 2016, I was sharing these details with my closest friend, a fellow special needs mother to twins who were born prematurely, which caused a multitude of health conditions between the two of them. We had never had an in-depth conversation about why we hadn't had more children and what challenges we faced in trying to grow our family. It was during that conversation that my friend Jessica introduced an idea that would change the course of our lives, forever. It was something we had never heard of, discussed nor considered by either of us. It was inspired, and my heart knew the words she was going to say before she even said them. I was overcome with a comforting spirit and with emotion. Jessica had tragically lost her husband two years before and she knew very well the heartache of infertility. She and her husband TJ became parents through the miracle of invitro, and they still had four frozen embryos she had been storing all of these years later. She shared how it's expensive to continue storing them, but every time they call to see if she wanted to dispose of them or donate them to science, she just couldn't do it. One time she agreed to donate them for research purposes and she called them back because she couldn't live with the idea that her and TJ's babies would be experimented on. But she knew very well that she didn't want to give life to those babies herself. Her hands were already incredibly full with her medically complex twins and she was still trying to figure out life as a single, widowed mother. She didn't know what to do with her embryos, but she knew she couldn't just dispose of them. They were a part of her and TJ. So our conversation inspired her to ask if we would ever consider carrying someone else's child? Of course we would. We hadn't considered it before, but why not? We were open to adoption! This idea was fairly similar! She explained that she had four frozen embryos, two of great quality, and she wanted to look into donating them to me and Mike. She wanted US to be the parents of her and TJ's babes. I will never forget the spiritual and emotional feelings of that day. Before I even knew if this could medically and scientifically be a possibility, I knew in my heart this was our destiny. This was God's plan for our family. And my beautiful friend Jessica would become part of our family.
Mike and I went to our initial appointment at the fertility clinic. Truth be told, we were not that impressed with our doctor because he struggled to pay attention and maintain a conversation. But later in his office when he was looking something up on his computer, he fell asleep mid-sentence. That's when we knew he must have had a rough night. We know alllll about rough nights in our world, so it made us much more sympathetic and understanding. He explained to us that I was a compatible match for these embryos!!! Due to the fact they had been frozen for 7 years using the older method AND that I was no 32 which is an older maternal age, we only had a 50% chance of having a viable pregnancy. Not spectacular odds. And he was considering those odds with THREE embryos transplanted!!! But I remember not caring about the statistics. I tuned them out completely. No, it wasn't due to ignorance or inability to handle the truth. It was because I had received the spiritual confirmation that I was to be a mother of at least one of these embryos. I knew it in my heart. This was our path and our journey, and the statistics didn't matter. We went home with so much hope and excitement. We were going to receive a calendar via email letting us know when I would start particular hormone treatments. We were wanting to plan for a June baby since I was just starting a new job and would have summers off. That meant the embryos would be transferred in September. It suddenly became real. We were going to be parents.
I had to have a couple of tests done to make sure my uterus was in good shape to carry a baby. I was told the procedure could be very painful. I brought my sister for emotional support, particularly because she is an ultrasound tech and was familiar with this procedure. I was surprised at how easily I handled them expanding my uterus with saline for the scan. The doctor told me my uterus was in excellent shape, even with my endometriosis. However, my ovaries had several cysts and potential tumors. I have had several removed in the past. He informed me that if we were to try and get pregnant naturally, it wouldn't likely happen due to the progress of my endometriosis. But with an embryo transfer, it shouldn't be a problem. Again, another confirmation that we were on the right path.
I spent the summer weaning off of several medications I was on, so I could give this baby the best chance at having a healthy life. I was taking a migraine preventative and an antidepressant. I'll be honest, weaning off of these two medications was one of the hardest things I've endured. The brain zaps, brain fog, joint and muscles aches, extreme fatigue, and mood swings were horrific. They lasted about 4-6 weeks, but I was at last free from all medications that had held me hostage for years. I will never forget the pride I felt in getting through this weaning process and I hope to never put my body through that again.
I started lupron injections in August. This was particularly a challenge for me because I have experienced lupron before. When I was 20 I was diagnosed with severe endometriosis, the worst case possible. I had a very large tumor and several cysts removed from my ovaries and uterus. As treatment so I could potentially have children in the future, I was put on Lupron injections for 9 months. This was an older form of lupron which has since been found unsafe. It caused me to experience severe mood swings, depression and even made me suicidal. It's possible it has contributed to my severe migraines I've experienced since. Needless to say, I'm not a fan of this drug. And back then, I just had monthly injections. Now, they would be daily. I've also done daily injections in the past of vitamin B12. I'd been diagnosed with a severe deficiency several years ago and doctors found my body wouldn't absorb it through my GI tract, so I had to give myself daily injections in the stomach. I hate needles, and it triggered small daily panic attacks each time I had to give myself one. I feared this would be the case again with these daily injections in my stomach. Fortunately, my husband agreed to give them. He has since become comfortable with the medical world thanks to Jayson's health struggles and he felt he could give my injections. So we began, and we endured! Weeks later, we added the daily progesterone shots, which went into the hip muscle like my lupron shots. Again, the trauma was real because of my previous bad experiences, but we endured. And it wasn't so bad. I had minor allergic reactions to both shots, but doctors felt it was due to the serum mixed with the hormones, so I just coped with the itching and hives surrounding the injection sites. It would all be worth it. I knew it. I also took an oral estrogen supplement daily. The combination of the hormones only made me mildly crazy and I gained quite a bit of weight fairly quickly. But again, I knew it would all be worth it.
Transfer day came so quickly. We couldn't believe it. We were about to be parents, again... a dream we didn't know would ever become a reality. The transfer was very quick! I was nervous it wouldn't happen because I had come down with a very serious cold just two days before. But my exam went well and we moved forward as planned. I prayed that my illness would in no way affect the embryos. We were told that when they thawed the four embryos, only two survived and those would be the two they would transfer. Due to my inability to maintain a full bladder, the ultrasound was a challenge during the transfer process. In fact, the doctor could not even find my uterus on the scan. It made me a little nervous to see them struggle to see where to put the syringe with the embryos, and then the doc suddenly said, "Well, we'll hope that did it!" That was it. It was done. They couldn't see where they put the embryos exactly and we couldn't get an ultrasound pic of the little ones entering their new environment, but they reassured us that they have done this transfer process for many years before ultrasound technology was available. I just held onto the truth that I knew this would work.
It was hard to concentrate the next few days. We had to wait until day 11 to do a beta blood test to see if the embryos implanted. I looked for any signs I could that these little ones implanted, but I knew in my heart it was going to work. I started to feel some signs of pregnancy around day 7 and I decided to test. I was not surprised to see a faint line. And I knew it would get darker the next day. And then the next. WE WERE PREGNANT!!!! We went in for a beta test and they called us a couple of hours later to confirm. We were pregnant. Not just pregnant. VERY pregnant. Our beta number was in the 600's, almost 700. Anything about 100 would be a clear positive. They told us to prepare for twins. We were elated. It seemed all of the trials, pain and suffering for the previous years led to this moment, and it was all so worth it.
We repeated the beta two days later to see if it would double. It nearly quadrupled. Again, we planned for twins which was honestly terrifying. At this point, it had started to sink in that we were going to be parents again. Parents of a typical child or two, in addition to the medically complex child we have. In addition to our jobs, our service callings, and all of our responsibilities. I started to wonder what I was thinking?! Life was already hard enough and I hadn't managed to figure out a good routine over the past five years. Why did I think I was ready to grow our family?? Then I reminded myself that this was all part of a plan. I knew that. This was the right thing for our family. God would bless us and give us the support we needed. I tried to keep my anxiety low, but I was still panicking.
A couple of weeks later, the vomiting started. Severe vomiting. Vomiting that resulted in cramping, diarrhea, and even vomiting blood. I called my fertility specialist since I hadn't yet made it to my 10 week appointment to be transferred to my OB. I had been vomiting for about 12 hours straight and they told me to come in immediately. I vomited in the car on the way there, and twice in the building's bathroom. They got me hooked up to iv fluids and prepared for an ultrasound. I had to prepare myself mentally that I might be losing the babies. I was in such pain, and vomiting blood is a bad sign. Thankfully, our very first scan revealed we were still expecting... ONE baby. I almost felt guilty, but I was so relieved to see there was just one. Part of me wanted to mourn the loss of the other baby, but I reminded myself that God was directing us on this path. He knew what was best, and one baby was it. I focused on the positive. There was one healthy baby in there with a strong heart beat. We were still going to be parents again.
Unfortunately, this was just the beginning of a very hard, long journey through pregnancy. I continued to vomit, a lot. So much it made my esophagus bleed and made me cramp regularly. I couldn't keep anything down. I vomited all morning, a few times in the afternoon, and uncontrollably at night. Everything was a trigger. Good smells, bad smells, movement, car rides, looking at my phone or computer, watching tv, eating, not eating, drinking, everything. My doctor got me connected with home health and I soon started getting daily iv infusions of fluid and iv vitamins. I tried the medication Diclegis, but it made me more nauseous. I was wanting to avoid Zofran due to the latest research linking it to birth defects. We were going through pretty extreme measures to increase our chances of having a healthy baby; I didn't want to decrease those chances by taking a medication if I could avoid it. When I was well hydrated, I was able to keep a couple of foods down. Drinking was still not an option. We realized that iv fluids on there own were a reasonable preventative and treatment for my nausea and vomiting. I was officially diagnosed with Hyperemesis Gravidarum, a condition I had never before heard of. I was not ill with Jayson, so this was completely new to me. Doctors thought it could be related to the hormone treatments for invitro or perhaps due to me carrying a baby that wasn't biologically mine. Even with iv fluids, my body was dehydrated from the vomiting. I was blowing iv's daily and my home health nurse said I needed a PICC line placed. I am familiar with PICC lines. I live in a special needs world and many kids depend on them. They are also in and out of the hospital with line infections. This terrified me. My doctors reassured me that the risks are minimal for adults and that this was the best option.
Getting my PICC line placed was a very difficult experience. Mike had to work, so my sister went with me. I was told and had read it was a fairly simple procedure. I was not prepared for the emotional trauma of entering an OR in a very sterile environment, with everyone masked and gowned. It brought back so many memories of Jayson and his surgical experiences. I felt my anxiety rise. I was also not prepared for the pain I felt. I don't know if my situation was different from others or if I was having a low pain tolerance, but it hurt! BAD. The pain made me vomit and as I went home I wondered if I had made the right choice. My vomiting escalated over the next week due to the pain I felt from my picc line. It felt like a severe bruise combined with the stinging of an open wound. I found it difficult to sleep through the pain and function during the day. As I got used to my new PICC, my husband and I also made a rookie mistake in preparing my iv fluids at home. Somehow, 6 inches of air got into my line. SIX INCHES. I didn't feel the effects for about 3 minutes. But then I felt pain and pressure in my chest, which led to an uncontrollable coughing attack where I was gasping and gasping for air and couldn't breathe. Mike had ran to the gas station and I called him and couldn't get any words out. He knew that I thought I saw air in my line, so when he heard me coughing on the phone he turned around and headed home. The coughing lasted somewhere between 5 and 10 minutes, but it felt like much longer. Over the next twenty minutes I slowly felt the pressure ease out of my chest. I called my home health nurse and she felt I was okay to monitor myself at home now that I got through the worst of it. She explained I basically gave myself a pulmonary embolism that passed through. It hurts like a mother and makes you fear you're going to die, but I was okay now. We learned a valuable lesson about air in my line that day...
These next few words I feel guilty for saying, but I feel they need to be said. My illness was severe. It was life-threatening. I had a PICC line and was dependent on two liters of iv fluids a day to survive. I felt pain in my kidneys, GI tract and all over my body as a result. I could literally feel my organs shutting down. I couldn't eat, drink, sleep, function. I feared every moment of every day for several weeks that I would die from carrying this baby. There were days where I wondered if the best thing would be not to carry this baby. I feared for what my husband and son would do without me. My husband saw how sick I was and he had the same concerns. I knew with my severe dehydration and malnutrition that the baby was at risk anyway; I prepared myself mentally that maybe a miscarriage was the best thing for all of us. I know, it's awful. But not many people can understand those emotions unless you've been there. My body was literally shutting down. My labs were very very scary. I was in ketosis with high levels of ketones in my urine. All of my vitamins and levels were completely out of normal ranges. I had lost 17 pounds in just a couple of weeks and every time I stood on a scale I was filled with guilt from losing more and more weight. I could feel my kidneys shutting down, I was constantly having heart palpitations and arrhythmias, and I literally felt myself dying a little more each day. I hope to shortly forget the horror of those few weeks, but somehow I think they will live on in my worst nightmares.
We stopped my hormone injections and pills in November and I noticed a subtle improvement in my nausea, which gave me hope that maybe things would get better in second trimester. Many women with hyperemesis suffered the entire pregnancy, but I could hope that I would be one of the lucky ones who would become "fluffy" in the following trimesters. I had a couple of good weeks in December! I weaned down my iv fluids to once a day and even tried three days in a row with drinking and no iv fluids. To my dismay, it resulted in me backsliding big time and having one of my worst weeks, just before Christmas. I went back onto two bags of fluids, and my labs still showed I wasn't staying hydrated enough. It was at this time I started to accept that perhaps I would be suffering from HG and be dependent on my PICC and fluids my entire pregnancy.
My sister and I have the fun experience of being pregnant together!! That has been a positive in this pregnancy, even just having someone to commiserate with! We have the same obgyn too! She is due just one week ahead of me, but due to my HG my appointments have been off of the typical schedule. I was beyond disappointed to find out my sister was finding out the gender during her 15-16 week scan (which is when we found out with Jayson) yet I had to wait until my appointment at 18-19 weeks. I desperately needed something to lift my spirits and keep me going! I needed to bond with this baby! I needed to feel connected to the child that was causing me to be so ill!! So we decided to splurge and go to Fetal Fotos to have the baby scanned, get photos and find out the gender just before Christmas at about 17 weeks! We had the perfect idea too, and I was soooo excited I could carry it out! It went perfectly! We had Jessica join us and they had a big bed for me to lay on, couches for Mike and Jessica and a big screen tv to watch the scan. We got to see very clear images of the baby, see it moving and active. It brought tears to our eyes!! It was real! It was so very real! We then turned our heads while they scanned for the gender. Baby was incredibly active and wiggly which made it hard to tell, but Jessica and the tech soon knew the gender with 90% certainty!! We then waited another three days until our gender reveal party. We had Jessica's family, my family, Jayson's nurse, and a few family members on Skype to announce. We had a beautiful white tree decorated in both pink and blue lights and ornaments. Both sets of lights were plugged in. We did a count down from 10 and when we got to zero, Jessica unplugged one set of lights leaving one bright color glowing----- PINK!!!!!!!! I screamed!!! I was in disbelief!! Mike and I both very much wanted a girl and I couldn't believe it was happening!!! What's even more fun?? My sister Tessa is having a girl too! They are going to be the best of friends! There are girl cousins her age on Mike's side of the family too, and more girl cousins being born on my side. It was time to buy all things pink!!!!
I started to adjust to a new normal. I got a pump from home health that would pump my fluids into me while I was working or wherever I was! Due to this convenient factor, my mental health started to improve. I could get out and do some grocery shopping. I could get fluids while at work or driving instead of constantly being connected to an iv pole at home. I was able to start doing laundry and things around the house again. I felt like I was establishing a new routine. I was still very sick, but the fluids were helping me eat and keep a lot down. I had found my safe foods-- Rumbi's kids rice bowls, Zupas BBQ chicken salads, and Costa Vida Salads. I could not cook or warm up anything in my home because of the smells. I had to only eat out or take out, and only at these restaurants. As I tried other foods, I always threw them up. I was able to start drinking some apple juice and cranberry grape juice, but never enough to stay hydrated.
Throughout December and January, I had noticed that my allergies were flaring up. This was a common health problem I had dealt with for years without adequate treatment or explanation. It would always start with my right eye itching and watering for days, then the right eye and side of my face swells, then my hands swell and my joints ache. Eventually, it ends up with an anaphylactic attack. I had been experiencing 3-4 of these attacks a year the past 3-4 years. My allergist tested me for every allergy under the sun. I wasn't allergic to anything, even though my body was having an allergic response. My allergies don't respond to epi-pens, I've learned, and only sometimes respond to Benadryl and steroids. I became nervous after having a couple of weeks in a row of a watery, swollen eye and swollen hands. At just 18 weeks pregnant, my hands were far too swollen to wear any type of ring on my fingers. I had always been concerned about this undiagnosed medical condition of mine, but I started to get an intense feeling that I needed to try and figure it out again because it could affect the baby. As I spent a weekend doing my own research, I had found a couple of potential conditions to ask my doctor about and each one of them posed risks to the baby during pregnancy. Not just risks, but high statistics of still births and miscarriages. The research also mentioned a connection between these conditions and hyperemesis during pregnancy. I knew it was urgent I get seen.
I got into my allergist quickly. Upon seeing me and hearing an update that I had still been dealing with these attacks and that they've escalated during pregnancy, he became very serious and a little panicked. He immediately put me on high doses of three different anithistamines and said I needed to start taking them now. He said I couldn't go off of them for any reason, even allergy testing. He left the room to make a call and came back. He mentioned the condition "Hereditary Angioedema" and said it was rare, complicated and well beyond his scope and ability to treat and I needed to see the best doctor in the state right away. He mentioned that the best doctor was very experienced and old, and had just had hip surgery, so it was likely I would see his new partner Dr. K. He told me Dr. K was familiar with all of the newest and latest research and with the help of his older colleague, they could diagnose and treat me. He had me call the office right there and remind the office he had just called Dr. K himself to get me in the next day. They set up an appointment for 8:20 the next morning. I felt such relief in knowing I may be getting answers that would hopefully keep baby safe.
I was immediately impressed by Dr. K. He was professional, asked good questions, listened, took notes, and admitted he would need help in treating my case. He told me after looking at my previous swelling pictures and my current swollen, watery eye that I fit the symptomology for Hereditary Angioedema. The problem is that I don't know anyone in my family who has it and my labs testing my C1 inhibitor levels were normal. We tested them again, yet they were still normal. That rules out Type I and Type 2 of HAE. There are those like me who fit into a third category, where they believe there is a genetic component to the HAE with mutations that have and have not been discovered. There is also a connection with HAE Type 3 and estrogen levels. As he asked me questions, we recognized that my allergies flared after I stopped taking my estrogen pills and progesterone shots. He felt there was a connection. Most with Type 3 are women and their symptoms escalate with periods, pregnancy and menopause. I fit the description. All but the feet! He said usually people with HAE also have feet swells. I never have. He confirmed that I needed to stay on the high doses of the three different antihistamines. He explained that epi-pens don't work with this condition, which I had already experienced. Since I had responded in the past to steroids he called in a script for me to use as my rescue medication. He hoped the antihistamines would help keep this at bay and that the past couple of weeks of minor swelling would not result in an anaphylactic reaction like I've seen in the past. But he emphasized that the minute I feel my airway swelling, I should take the steroids and head to the hospital urgently. This condition goes from bad to worse, quickly. I learned there is a 30% mortality rate in the 2nd and 3rd decade of a HAE sufferer's life. Not something to mess around with. I asked if there were risks to the baby and he said, "Yes, great. Your airway swells, you can't breathe, you die and baby dies." I could tell he wasn't messing around. He told me he would work with my obgyn to come up with a birth plan since my swelling would likely get worse after birth. I had experienced that with Jayson, so I assured him that was a good idea. He said we'd follow up on a monthly basis, but let me know if I had any concerns in the meantime. I left feeling both scared and relieved that it looked like I had a diagnosis and treatment plan.
For the next two weeks, I felt wonderful! The antihistamines treated my allergies and eye swelling and miraculously, they also treated my hyperemesis!!! The knot in my throat I had felt since October that resulted in me vomiting at any given moment was GONE. The night nausea that was debilitating, GONE. I could eat a couple of new foods and in larger quantities. I started gaining weight!! I felt hope again and had energy! I had thought the worst was behind me and that in getting help from my doctor for the HAE I was treating the hyperemesis, and life would be great! Unfortunately, I experienced too much hope, too soon.
One morning in February I awoke to a slightly swollen face and hands and pretty swollen feet. As I went throughout my day, my feet got larger and larger. By the end of the day I found it comical how large they were. I could push my finger into my foot and the indent would stay there for minutes!! I was entertained, so I took a picture of my blimp feet and shared the picture with some friends in a pregnancy group online. Their response quickly taught me that this was no laughing matter. I text the picture to my home health nurse who said I needed to get to my obgyn immediately the next morning to be tested for preeclampsia. I went in and my blood pressure was surprisingly low, not high, and my blood and urine labs were fine... beside suggesting dehydration, STILL. My obgyn's office advised me to contact my new immunologist right away about a possible HAE attack. I called Dr. K's office and he was out. I talked to his nurse who told me I needed to go to the ER right away. I just laughed. I have big puffy feet! What are they going to do for me in the ER??? I don't even know what to tell them to do? The nurse called Dr. K on his cell and called me back. The orders still stood. Go to the ER and take my emergency steroids. Sigh. I felt silly. I had a day full of doctor appointments for Jayson, so I finished those up, took him home so my mom could watch him, and I drove myself to the ER. Sure enough, they had no idea what to do with me. They ended up contacting Dr. K and letting him know my airway was in now way affected, just my feet, and they agreed to let me know home without needing to start the steroids.
My blimp feet stuck by me for another couple of days. We wondered if maybe this was just early pregnancy swelling and not HAE. Otherwise, I was feeling pretty well! But we spoke too soon. On a Saturday morning I was excited to wake and see my regular, boney, skinny feet... but I had no voice. My airway was swollen. It was HAE. I emailed my doctor and was told to go to the ER right away. I took my steroids and my mom took me to our local ER. We lucked out to have an attending who was relatively familiar with HAE, which is shocking because it exists in only 1:20,000-50,000 people and my type may be as rare as 1:150.000. They were on top of it. A crash cart was brought by. I was taken back immediately and a delivery nurse was waiting in my room to check on the baby. The doctor examined my airway and was relieved to see it was only mildly inflamed. Since I had taken my Benadryl and Steroids they said they were just going to monitor me for a couple of hours and make sure it doesn't progress. After about 3 hours, I started to get my voice back and we felt comfortable going back home. I emailed my doctor and sent him pictures of my feet and the hospital notes. He called me at home on a Sunday night and we talked for a while. He said we were hoping for the best, but it looks like we were going to need to come up with a treatment plan during pregnancy that may involve meds that may or may not be "safe". There wasn't enough research to know. He informed me of how dangerous this condition is, that we can take no risks, and that the pregnancy really complicates things. He reassured me he would be researching more about HAE Type 3 in pregnancy, treatment options, and consulting with his colleague and other national colleagues looking for help and guidance. He told me he would be there for me, and we'd get us through this safely. I was so grateful for his support.
A couple of days later, my blimp feet returned with a vengeance and several hours later at about 1am I began to feel pressure in my chest while sleeping. I woke up and tried to say something to my husband to wake him. Nothing. I had no voice. My airway was swollen, again. I really didn't want to journey to the ER at 1am in the morning and I had important work meetings in the morning I really didn't want to miss. I hooked myself up to our back up pulse oximeter and sat myself up with pillows in a seated position to try and doze through the night until morning. My O2 hung out around 90 most of the night which kept me out of the hospital, but definitely showed there was some swelling. By morning I had a little voice back so I thought I was doing better. I started driving to work and felt the pressure in my chest again. Dang it! I tried to talk out loud to myself. Nope. I was losing my voice again. I quickly called my doctor's office before I would completely lose my voice. They told me he and his partner were out of the office all week!! When I got to work I sent an email to my doctor and hoped for a quick response. I was able to sit through my many work meetings that day and pretend I had lost my voice to a cold, but when I was still without at voice around 3PM and hadn't heard from my doctor, I worried I needed to go into the hospital to be checked out. Sleeping another night with a swollen airway made me feel very uncomfortable. I don't know what the hospital could do because technically we had exhausted my treatment plan, but I needed to try something. So I drove myself to our local ER where I very unluckily got a doctor this time who is not so familiar with HAE. I wasn't hooked to any monitors. I didn't see a nurse or get my vitals taken. He looked at my airway and said it was mildly inflamed and he would try calling my immunologist to get a treatment plan. An hour and a half later, my immunologist called me. No, the ER doc never called. He suggested we go up on the dose of steroids instead of tapering down. Clearly this was a longer attack and was not resolving. The steroids may help just enough to keep my airway open since this airway attack happened as soon as I started tapering. He also feared I may need the rescue med treatment for HAE which is a plasma infusion. This treatment has not been proven safe in pregnancy, but he feared our lives may be in jeopardy without it. He said the problem is that many insurances won't cover it for Type 3. Not all research institutions agree there is a Type 3 and this treatment only works 50% of the time in Type 3 and no one can explain why it works. It's a several thousand dollar treatment. He said he would work on the pre-authorization. In the meantime, I was to take Benadryl around the clock, increase my steroids dose, and I would see him in a couple of days at my next appointment.
Fortunately, the steroid increase seemed to help with my airway. Not so much for my puffy feet. They seemed to just get bigger and bigger. The pain was great. I was finding myself in tears daily from the intense pain from the swelling in different parts of my body. I was back to throwing up off and on due to the pain I was experiencing. I was having GI issues from the swelling in my intestines. I hated looking in the mirror because my face was so puffy. And I had gained 15 lbs in a month which I knew was mostly water weight. I felt like I needed to spend some more time over the weekend before my appointment doing more research on HAE. Maybe if I understood it better I could help my doctor in treating it. I had so many unanswered questions. Why did these attacks suddenly start 3-4 years ago? Why did they get worse during pregnancy? Why were they not as bad during first trimester? I read a few research papers on Type 3 that really emphasized that there is a relation between hormones and these attacks. Some women got the attacks during their periods. I can't remember if this was the case for me? Some women got much worse during pregnancy. That was definitely my case. By why wasn't it this bad first trimester? One article was great and listed different treatments that were tried and their effectiveness. Sure enough, out of 200+ women in the study, ZERO responded to prednisone and antihistamines to resolve a bad attack. It became pretty clear at that moment that my attack was not just going to resolve. I needed a rescue med, but which one? The Berinert which is the plasma infusion medication had some good success in a lot of the women. But it didn't specifically study pregnant women who were on it. I am still concerned about the safety of that med for my baby and also the infection risks that come from infusing live plasma. I saw another option, but learned it wasn't used much in the US and due to my kidney concerns it would not be an option. Lastly, there were 8 women who were treated with progesterone out of the 200+. Not many, in fact the smallest research group. But guess how many responded positively? All 8. All 8 women's attacks were completely resolved with progesterone treatments. That's when it clicked!!!!! I was on progesterone shots in first trimester!!!!! The Type 3 attacks are linked to estrogen imbalances. I had been on estrogen supplements for invitro, which should have made things worse, but the progesterone shots balanced them out! I didn't start to flare with my swelling until after I stopped the progesterone! But why oh why did these attacks start suddenly 3-4 years ago??? What does THAT have to do with progesterone?? I strained my brain for hours to remember what birth control I was on after I had Jayson. It finally came to me. When I looked it up, it was straight progesterone. It was a mini-pill. I didn't have attacks during that time, and that was the same birth control I was on all through my 20's to control my endometriosis. Then I remembered at a obgyn check up my doc/nurse said I needed to switch birth controls. I don't remember why? Maybe they didn't think the mini-pill was strong enough? Maybe my periods were irregular? I forget. The new one they put me on---- straight estrogen. NO progesterone. That was about the time I started having these swelling attacks. Oh my goodness. I think I figured it out!! My attacks are strictly hormone based!!! As I read more research, this was great news for after the baby! My swelling can be controlled for YEARS by just taking progesterone based birth control. But until then, it's very very complicated. But I had some new questions. I wanted to ask my doctor at the appointment if I could potentially go back on progesterone shots or suppositories?? Would that help? Would it be worth the try? What are the risks involved? I knew progesterone was safe at this point in my pregnancy because I know many people to take it to avoid pre-term labor. I knew insurance wouldn't approve it for this purpose, but if I paid for it myself could we try it?
I asked these questions at my appointment. He said he had never heard of progesterone as a treatment beyond birth control. I shared the article with him and he was equally as impressed with the 8/8 women who responded positively. He said he would talk to my obgyn about the safety and risks to the baby to see if it's something we can try. He looked at my feet and my swelling and was literally speechless. The kept touching, poking, pushing, stroking, and saying, "OMG, OMG, OMG." I told him it was actually a good day. He said we have no choice but to treat this attack. It's not responding to anything so far and it's progressing. When I asked if it poses risks to my health beyond just the intense pain? He said, "Oh, yes! You are at high risk of developing blood clots, heart failure, kidney failure and many other problems. This is a very big concern." Wow. He said he would be consulting with his experienced colleague to see if at this point we really try to get me Berinert the plasma infusion medication to treat these acute attacks. Then he would contact my obgyn to see if we can use progesterone as a preventative. He reminded me my insurance may not approve the Berinert and by the time we get through an appeals process the baby could be here. That made me feel a little discouraged. He told me he would work on these things and we would be in close contact. In the meantime, he wanted me to go up on my steroid dose and not come down. It was clear the steroids weren't treating the attack but they may be keeping my airway open, which was worth increasing the dose.
So that's where we are. Since increasing the steroid dose I've experienced less pain and I'm in better spirits. That has made this more tolerable. But my feet are still incredibly blimpy, my airway has stayed open but has gotten mildly hoarse a couple of times, particularly in the mornings when my face swells the most. I finally got compression socks that go up to my thighs and they have made a very big difference in my swelling! My feet fit in shoes and I can stand to walk on them without crying! However, just a couple of hours after removing them, the swelling returns. Insurance surprisingly approved my Berinert!!!! But the logistics of having the medicine made, sent here and to have me trained in administering it via my PICC line have been complicated. I am hopeful that perhaps next week we can try this medication and see if it works. I have not heard an update about the progesterone as a preventative. I know right now the focus is on getting my acute attack handled.
So that's been my pregnancy in a nutshell. Uncomfortable, scary, painful, and even life-threatening. Not an all what I expected when we started this journey. Pretty unique, right? But it is still so very beautiful. Baby girl is healthy, active and right on track. I am doing everything I possibly can to keep her safe and growing. We are over the moon excited for her entering our family and are busy making preparations! We have a name picked out we are pretty set on and we are still working on the middle name.
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