M281

*Several years ago I wrote a blog post called Anti-Kell Antibodies: Dr. Moise’s New Treatment and I promised to update the blog when there were new developments. This is the update 3 years in the making!

*Disclaimer: I am not a medical professional. This is what I understand about M281 and the phase 2 trial currently underway.

I like to think in pictures and stories, so I’ll share the scenario that comes to mind when I think of M281, a new drug currently being tested to treat severe alloimmunization and HDFN in pregnancy. Imagine a busy train track with trains coming and going every hour or so. Now imagine a toddler walking around exploring the train tracks, picking up rocks, stumbling and righting himself, following a passing airplane overhead with wide eyes. I’ll use a picture of my 20 month old, Callum just for a cute visual.

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You hear a train approaching in the distance. What is your first reaction? Go get that baby off the tracks, right? It seems logical to protect the toddler from being hurt or killed before the injury happens. What if someone nearby said, “Oh, it’s fine. Let him do his thing and if he gets hit by the train we have medical professionals who can treat his wounds. There is a possibility of death but hopefully we can save him after he gets hit by the train.” No one in their right mind would stand by and let that scenario play out. Why not remove the baby from the area or at least put up a fence or other barrier to keep the baby away from the train track?

Sometimes it feels like the methods used at the moment to treat alloimmunization during pregnancy/HDFN are similar to the train track scenario. Instead of protecting the baby from the antibodies to begin with, the protocol is to wait until the baby is attacked by the antibodies and becomes anemic before stepping in to help. But this way of treating the disease has proven dangerous far too many times. Sometimes the baby dies before the doctors detect the fetal anemia or the anemia is so severe by the time they discover it that the baby can’t be saved (like in Lucy’s case.) Sometimes the fetal anemia is discovered but the doctors wait too long to do a transfusion and the baby dies. There can be complications from the IUT which can lead to premature delivery or death. Another complication is hyperbilirubinemia after birth which can lead to permanent brain damage if not treated quickly and aggressively. All of these tragedies could be avoided if we could figure out a way to “put up a fence” between the antibodies and the baby BEFORE the baby is attacked by the antibodies. What if there was a way to protect the baby from the injury in the first place instead of trying to treat the wounds afterwards? Well, Dr. Moise and some other brilliant people might have figured it out!

M281 is a drug that can potentially block a mother’s antibodies from going through the placenta to her baby during pregnancy. If the antibodies don’t get to the baby then there should be no fetal anemia, no IUTs, no fetal hydrops, no hyperbilirubinemia after birth, no death. This drug is still in the trial phase but it looks very promising and I am so excited to see how it might save lives in the future.

M281 is FDA approved and since my original blog post in 2016, researchers have done drug studies on pregnant primates and non-pregnant humans to test for safety and efficacy of the treatment. They have studied the drug in human placentas in the lab too. To learn more about these studies you can check out these links:

Human Ex Vivo Placental Perfusion Model:

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http://ir.momentapharma.com/static-files/71c4376f-4984-419e-a0b1-430326783e99

First Human Study:

firstinhuman.pdf

Right now the phase 2 trial for pregnant women is open and they are accepting 15 trial participants worldwide. This trial is the first one to test M281 on pregnant women with severe HDFN/alloimmunization. Here is the link to the study details: https://clinicaltrials.gov/ct2/show/NCT03842189?cond=m281&rank=3

I wanted to answer some of the most common questions I have received about M281 here so that the information is easy to find. If you have other questions about the new treatment or about the drug trial you can come join our M281 Discussion Group on Facebook or you can email me at bethanysk55@yahoo.com or just ask your question here in the comments section. If you have specific questions about the trial here is the contact information for Momenta Pharmaceuticals:

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT03842189

 Momenta General Queries +1 617-491-9700 ClinicalTrialInfo@momentapharma.com

Here are some of the most commonly asked questions:

How does the drug protect the baby from the antibodies?

M281 works in two ways to protect baby from the mother’s antibodies while in the womb. It lowers the mother’s overall antibody count and it blocks the antibodies from getting through the placenta to the baby.

How is the drug administered?

M281 is given via IV infusion once every week and the infusion takes about two hours.

Does the drug work for any antibody or just for certain ones?

The drug trial is only for women with anti-D or anti-Kell antibodies since those two are usually the most aggressive ones when it comes to HDFN and the study wants to specifically target the worst case scenarios before declaring that it works. But the drug does block all red cell antibodies from going through the placenta so if it does get approved and go on the market it will work for anyone with a red cell antibody.

Does the drug cause birth defects or dangerous side effects?

This is part of what they are trying to determine with the phase 2 trial on pregnant women but the previous studies did not find any evidence of birth defects or major negative side effects. The drug will be administered once the woman is in her second trimester so that alone is safer in regards to birth defects since those typically happen in the first trimester. Also, previous studies show little to no transfer of the drug to the fetus.

How is M281 different from IVIG?

If you read my post about Titers, Antigens and Sharks you might remember that the “shark cage” was IVIG which can be used in severe cases to protect the baby in utero from the antibodies early in pregnancy before IUTs are possible. IVIG stands for “intravenous immunoglobulin” and it is made up of thousands of people’s antibodies concentrated into a liquid given intravenously. Doctors still aren’t sure exactly how IVIG works to protect the baby but most doctors think the IVIG protects the baby in one or more of these three ways:

  1. It lowers the titer so that there are fewer antibodies present in the mother’s blood to attack the baby.
  2. It slows/prevents the antibodies from going through the placenta and attacking the baby’s red blood cells.
  3. It helps the baby fight off the antibodies that are attacking his red blood cells.

IVIG doesn’t completely block the antibodies from getting to the baby like M281 could but it does delay the attack. With my second and third sensitized pregnancies I had plasmapheresis and IVIG treatments which delayed the antibodies’ attack and saved my babies’ lives. Even with those life saving treatments Nora had seven transfusions total and Callum had six transfusions total as well as other interventions and medications, NICU, etc. The antibodies still wreaked havoc on my babies both in the womb and in the weeks following birth. IVIG is also very expensive and most countries will not approve it as a treatment for our disorder. Another down side of IVIG is that it usually comes with some pretty severe side effects for the mother. I had debilitating migraines, vomiting, rapid heart rate, difficulty breathing, back pain, muscle weakness, eczema and more. I also had to take more medications (mostly class C drugs for pregnancy) to manage the side effects. For the people who were given M281 in the previous human studies (non-pregnant) there were no significant side effects.

When will the drug be ready for public use?

I’m not sure if or when the drug will be available to the public. The next step is to complete the phase 2 trial which means following 15 women through their pregnancies and for six months after birth. Only once the drug is deemed effective and safe will they consider making it available to the public. Right now the estimated trial completion date is 2022.

If the drug blocks all maternal antibodies from going through the placenta, how does the baby get the good antibodies from the mother? Is the baby born without an immune system?

The drug does block even the “good” maternal antibodies from getting to the baby but the doctors have a solution for this. The plan is to induce labor/deliver at 37 weeks, so at 35 weeks they will stop the weekly M281 infusions to allow maternal IgG antibodies to transfer to the baby. The woman will receive IVIG to boost her antibody count (NOT her red cell antibody count though.) During the two weeks without the M281 infusions the MFMs will watch baby very closely to make sure he isn’t getting anemic. If baby is showing signs of anemia they will deliver. The baby will get mom’s antibodies for two weeks before birth. Once baby is born the doctor will test the cord blood to make sure baby’s IgG antibody levels are high enough. If they aren’t, they will give baby IVIG infusions to boost the antibody count and baby will have an efficient immune system.

Who can be included in the trial?

The doctors have very specific requirements for trial participants, all for good reasons. If you are interested in joining the study, contact your OB or MFM and have them contact Momenta Pharmaceuticals at +1 617-491-9700 or have them email Dr. Ken Moise at kenneth.j.moise@uth.tmc.edu  Here are SOME of the requirements for trial participants:

  • Age >18
  • English speaking
  • Singleton pregnancy
  • Gestation 8-13 weeks
  • Anti-D alloimmunization with a titer of at least 32 or Kell with a titer of at least 4.
  • Previous pregnancy with one of the following at 24 weeks or earlier- severe anemia at time of transfusion with hemoglobin less than 0.55, Hydrops Fetalis with elevated MCA over 1.5 MoM, fetal demise with pathology of the placenta/fetus consistent with severe HDFN (need records)
  • Evidence of immunity to certain diseases
  • No history of a clinically significant medical complication that would jeopardize the safety of the patient or her fetus (ex: pre-eclampsia, pregnancy induced hypertension, drug or alcohol abuse, etc.)

I will try to keep everyone updated on the new drug’s progress here on the blog and definitely on the M281 Discussion Group on Facebook so come join us there if you’re interested in following along!

 

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Fetal Center Reunion

For the first time in my life, I have a 19 month old toddler and I am not pregnant. With all four of my other children by the time they were one and a half I was already pregnant with their younger sibling. As Callum grows it feels bizarre not to be growing as well with his younger sibling inside. There is a little bit of sadness but mostly a delicious freedom and ease to life, yes even with four kids. To be able to chase Callum or deal with a toddler melt down without fighting back nausea is such a relief. It makes this stage so much easier as a parent. I am enjoying my four kids so much and savoring the absence of emergency in our lives. I love having my body all to myself and being able to sleep through most nights without tending to a baby.

Our baby Callum is doing well and growing so fast. He was a late walker like Nora, but their personalities are more laid back so I’m guessing that is the reason they walked later than their big brothers. Liam walked at 11 months, Asher walked at 8-9 months, Nora walked at 14 months and Callum was 16 months. It’s fun to have all four kids now on their feet and somewhat independent. I am loving this stage of life with the chaos and the sweetness and the hilarity of young children.

In April we drove to Houston for the Fetal Center Reunion and it was a quick but wonderful trip. The last time we were in Houston was when Nora was a newborn. She was only about 4 days old when Josh and I woke up in the early morning hours in 2015, packed up our room at the Ronald McDonald House and drove out of the city towards Alabama. As we left, the sun was coming up and I looked in my rear view mirror at the city doused in morning light and I cried happy tears of disbelief. We had done it. God had done it. Nora was alive and I felt such a surge of love for the city of Houston, for all the miraculous things that had taken place there. So now to be back in Houston with bubbly Nora who never stopped talking the entire trip was very surreal and special. We stayed at an Air B&B in the medical district and visited some of our favorite places in the area. Again, it was surreal to be in these places where I had experienced such emotional turmoil four years prior, completely desperate for my baby girl’s survival. Now here she was playing with her brothers in the same spot, completely healthy and very much alive. I will never get over the miracle of her life and I will never stop telling of the wondrous things God did for us. He is such a faithful loving God, patient and kind and trustworthy. I truly have no good apart from Him.

Here are some pictures of Nora playing in the park in front of Children’s Memorial Hermann Hospital where she was born:

At the Fetal Center Reunion we got to see Dr. Moise and many of the other doctors and nurses who treated us during my pregnancy. I saw some of the women who coordinated my care during my pregnancy with Nora and some of the nurses and ultrasound technicians who monitored Nora every week in the womb and assisted during her intrauterine blood transfusions. All of these amazing people worked together to save Nora’s life and to see them all together again was so special to me. My gratitude overflowed. I’m actually tearing up right now typing this because these people mean so much to our family. It really did feel like a family reunion. They were all so sweet and happy to see Nora. Nora was excited to finally “meet” Dr. Moise (even though he had already met her when she was a baby obviously) and Callum loved Dr. Moise too even though he had no idea who he was. I loved seeing Callum with his namesake and Nora with her hero.

I also got to finally meet one of my most dearly loved fellow “ISO moms” Brittany Pineda. She reached out to me several years ago when she was pregnant with her son, Kristian, in her first known ISO pregnancy (anti-D.) I grieved and still grieve with her over the loss of her baby Kristian after his first IUT and encouraged her to try again for her rainbow baby. Long story short, she traveled to Houston for her treatment with her second son and Dr. Moise performed his earliest IUT on Brittany’s baby at 15 weeks. I think that week shaved about ten years off my life I was so anxious. Her miracle baby, Mikah Kristian Joseph was born after 9 IUTs and is now a healthy toddler. Brittany has always been such an inspiration to me and she encouraged me throughout my pregnancy with Callum too. Now our miracle boys share a middle name, Joseph, after our hero Dr. Moise. You can read more about Brittany’s story here in the Fetal Center Newsletter fetalnewsletter-1.pdf

After all of our history together, Brittany and I had never met in person outside of our cellphones. It was amazing to see her and her family face to face finally! Of course we both had to spend most of our time chasing after our toddlers but we got a little conversation in here and there. Here are a few pictures of our families together with Dr. Moise and at the Fetal Center Reunion:

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Callum kept waving at Dr. Moise

Rainbow babies Nora and Mikah, 14 IUTs between the two of them at the Fetal Center:

It was really hard to get a picture of these two together since they NEVER stopped moving! Our boys, Callum Joseph Thomas and Mikah Kristian Joseph:

And of course our favorite part of Houston is always the Korean food:

 

Titers, Antigens and Sharks

Anti-Kell antibodies, along with the other red cell antibodies during pregnancy, can be very confusing and scary, especially when you are newly diagnosed and trying to figure out what is going on. Since the disorder is very rare, medical professionals don’t encounter it often and sometimes have to rely on their memory of what they learned about it in med school. Some of the most common misconceptions revolve around the antigen blood results, titers and MCA scans. I have been trying for years to come up with an analogy to easily illustrate how these things work together and I have finally found a pretty good one. Obviously it isn’t perfect but it’s the clearest one I’ve been able to come up with to represent these specifics facts. In this post I will be using anti-Kell antibodies as the illustration but the analogy can also be applied to the other red cell antibodies such as E, D, c, Jka, Jkb, Fya, Fyb, etc. So, here is my shark analogy:

gray_reef_shark_fish

The sharks represent the mother’s antibodies. They have the potential to be very dangerous, they are designed to attack and destroy. But this doesn’t necessarily mean that the baby will be attacked by the shark. Many of us have been in the ocean with sharks and not been attacked by sharks. Just because they are there it doesn’t mean that they will attack. For most people, the thought of sharks is terrifying and the thought of these antibodies possibly harming our babies is terrifying as well.

Let’s talk about the antigen first. The antigen is hereditary and is passed down from a parent. It isn’t dangerous, just a protein in your blood, similar to a blood type. If the baby has the antigen, passed down from his father, then that puts the baby in the ocean with the sharks. Still not necessarily getting attacked by the sharks but in the water and possibly in danger. If the baby did not get the antigen from the father, then the baby is on the beach, far back from the shoreline, playing peacefully in the sand or maybe taking a nap. The sharks are still in the water with all their muscle and sharp teeth, but the baby is way up on the land, completely out of harms way.

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Ironic picture of my baby who certainly wasn’t “safe on the beach” during my pregnancy

This is why it’s important to have the baby’s father tested for the Kell antigen. It’s obviously necessary to know whether your baby is in the water with the sharks or up on the beach playing peacefully in the sand. Many times I’ve been approached by nervous parents who tell me that the nurse or even the MFM has told them the antigen status of the father has no effect on the baby. Or they have been told, after the father’s test showed he is negative for the Kell antigen, that the baby could still be attacked by the mother’s antibodies. This is simply not true. There is no way a shark can attack a baby who is way back away from the water, on the sand. And there is no possible way for the antibodies to hurt your baby if the baby is Kell antigen negative. By the way, the mother is always Kell antigen negative since it is impossible to produce anti-Kell antibodies if you have the Kell antigen. This is why we always focus on the father’s Kell antigen status. If he is negative for the antigen we know that two negative parents cannot possibly pass down a gene to their baby that they themselves don’t have.

Ok, so if your baby’s Daddy is Kell antigen negative, your baby is safely playing on the beach and there is no need to worry about any sharks. If your baby’s Daddy is Kell antigen positive then it’s possible that your baby might be positive for the antigen as well. You can find out at 14 weeks by doing a non-invasive maternal blood test if your baby is Kell antigen positive or negative. The blood test can also be used to test for the E antigen and the c antigen. There is an even earlier maternal blood test to check the baby for the D antigen (rh.) For all other antibodies an amniocentesis can be done at 16 weeks to check baby for the antigen. So, what if my baby has the antigen? That means your baby is in the water with the sharks. In my analogy we are standing on the shoreline looking out at the ocean and the baby is under the water swimming around, much like they do in the womb. It’s very scary as a mother to know that your baby is under the water with the sharks and you can only gaze out anxiously at the surface of the water, unable to really check on the baby and see how he is doing.

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This is how it feels during a sensitized pregnancy. It is such a helpless, scary experience to know you can’t physically see or help your baby stay safe from the sharks. Thankfully, there are ways that our heroes, the medical community, can check on our babies and protect them from the sharks.

First, let’s talk about the titer. Your titer shows the amount of antibodies that are in your blood. The titer does not tell us anything about how our baby is doing under the water. Is baby being attacked? Is he struggling? Is he ok? Titer does not answer any of those questions. The only question the titer answers is “How many sharks are in the water with my baby?” Through years of research, doctors have come up with a critical titer for each antibody. The critical titer says, “Once there are this many sharks in the water with your baby, your baby is actually in danger of being attacked.” For most antibodies the critical titer is 16 and for Kell the critical titer is 4. A recent study also showed that Kell in particular can affect the baby at any titer, even titers below the critical threshold. Regardless of the type of antibody, even with a low titer, there is still at least one shark down there swimming around with your baby. Titers are helpful though, because they give an idea of how closely the baby should be watched and whether baby needs a shark cage or not. Only the babies in the most dire circumstance need a shark cage, but I’ll get to that later.

So, a very high titer means there are a lot of sharks down there with your baby, which does raise the chances of baby being attacked. My titer was 1,024 and all of my babies happened to have the Kell antigen, so with each pregnancy I had a baby swimming in shark infested waters. As expected, all three of them were attacked at some point. If you have a low titer, there are fewer sharks and a lower chance of being attacked. But it is still important to be vigilant just like you would if your baby was swimming around in the ocean with a couple of sharks out there.

MCA scans measure how fast your baby’s blood is flowing through the middle cerebral artery in the brain. The MCA scan will give you a PSV number, which is the speed at which your baby’s blood is flowing and if it is too fast, the doctors can be pretty certain that your baby is anemic. The PSV number can be converted to an MoM using this calculator. An MoM above 1.5 signals an anemic baby that needs help. So in our analogy, the MCA scans are the doctor’s way of going down under the water and getting eyes on baby. In our analogy the MCA scan answers the questions, “Is my baby being attacked by the sharks?” “Is my baby’s life in danger?” “Does my baby need help fighting off the sharks?” The MCA scans answer the important questions that regular ultrasounds and titers cannot answer. The real life questions the MCA scan answers are: “Is my baby anemic?” “How anemic is my baby?” “Does my baby need intervention in order to survive?” “Is my baby’s life in danger?” This is why MCA scans are so important. Would you be satisfied just knowing how many sharks are in the water? Or would you also like to know exactly how your baby is doing in the water and whether your baby needs help or not? Women with critical titers should have weekly MCA scans starting between 16-18 weeks. Thankfully, fetal anemia is treatable, but only if it is caught in time.

So, what if my baby is being attacked by the “sharks” and is anemic? The doctors can help the baby fight off the sharks by giving the baby blood through an intrauterine blood transfusion which helps keep the baby safe. Another way that doctors keep the baby safe is by monitoring closely and removing them from the shark infested waters as soon as it is safer for baby to be on the outside.

What about the shark cage mentioned before? I’m using the shark cage to represent IVIG given early in pregnancy. IVIG is usually given (often only at the mother’s prompting) to women who have had a previous loss or previous severely affected pregnancy, since it is obvious that the mother’s antibodies are very aggressive in those cases. But I am also seeing IVIG being offered more frequently to women who have no previous loss or even previous severely affected pregnancy but have very high titers, usually 256 or 512 and above. I think (just my personal opinion here) every woman with titers in the hundreds or thousands should be encouraged to start IVIG treatments early in the pregnancy (10-12 weeks) to protect their baby from their antibodies. The IVIG does act like a shark cage in our analogy by protecting the baby from the sharks that are swimming all around. IVIG delays and/or prevents fetal anemia and it is a good way to keep the baby safe in extreme cases. The shark cage protects the baby from being attacked whereas the more common approach just watches and waits for the baby to be attacked before stepping in and trying to help the baby. With both Nora and Callum we used the IVIG “shark cage” to protect them while they were in shark infested waters and that is why they are alive today. My doctors refused to even consider the IVIG shark cage for Lucy and that is why she was attacked so early and so viciously.

So here is a quick recap of the shark analogy:

ANTIBODIES=SHARKS

TITER=NUMBER OF SHARKS IN THE WATER

ANTIGEN NEGATIVE=BABY IS ON THE LAND

ANTIGEN POSITIVE=BABY IS IN THE WATER

MCA SCAN=DOCTOR GOES UNDER THE WATER TO CHECK ON BABY

IVIG=SHARK CAGE

Hopefully my shark analogy has helped some of you and not confused you more. Thanks to my ISO Moms in our Facebook group for helping me clear up some of the details in my shark analogy!

 

2,191 Days

It has been six years since Lucy’s heart stopped. I have lived 2,191 days without my daughter. 2,191 times my eyelids flickered opened and I awoke to a world without Lucy in it. And I got up out of bed and I made it through the day. Over two thousand times. In all of those days I have missed small and intimate moments with her, mundane moments that we parents often take for granted, and important milestones. All the baby snuggles, reaching for her hand in a parking lot, painting tiny toenails and washing her hair in the bath. The first words and the Halloween costumes and teaching her how to read. Birthdays and Christmases and bedtime stories. I even miss potty training and disciplining her and taking her to the pediatrician for her vaccinations. The overwhelming weight of all the things we have missed while living our lives without Lucy seems like it’s too much to bear sometimes. At the moment my grief is so fresh and raw, it seems impossible that it has been six whole years since she died. The persistence of my grief is a testament to the depth of my love for her. Oh how I ache for my little girl. But the grief should not come as a surprise. Just as it is good and right to enjoy and celebrate the children we have with us, it is good and right to miss and mourn the children that wait for us in heaven. Liam just turned ten years old yesterday and we celebrated his life. We gave him presents and made his favorite breakfast and told him we loved him. We didn’t say, “Oh, that was ten years ago. I already celebrated him back then and I’ve moved on by now.” We acknowledge the joy of his life and the gift of having him here with us. Conversely, we grieve the absence of our precious daughter, Lucy, and we acknowledge the depth of our pain and how much we miss her.

In a sermon preached only days after the 9/11 attacks in New York City, Timothy Keller said that there are two mistakes people can make in the face of tragedy, suffering and death and they are warned against in 1 Thessalonians. The first mistake is to try to avoid grief and weeping. The second mistake is to grieve as those who have no hope. He says:

 “The Bible indicates that the love and hope of God and the love and hope that comes from one another has to be rubbed into our grief, the way you have to rub salt into meat in warm climates where it will go bad. Your grief is either going to make you bleaker and weaker or it could make you far more wise and good and tender, depending on what you rub into it, what you put in. And that’s what we’re here to do. We’re here not just to weep but to rub into our weeping, hope and love.”

So today I acknowledge my profound grief for my daughter, Lucy Dair. Her name means light, and when her heart stopped, a light went out inside me that will not be lit again until I am in heaven. I feel her absence every minute of every day. We all love you so much Lucy.

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Original photo by Wright Media, edited with permission by Siobhan Fisher Photography ❤

But I do not grieve as one who has no hope. I know that, just as He promised, God will redeem every one of the 2,191 days we have missed with Lucy, and He will redeem all of the missed days to come. Today, as always, I will continue to rub hope and love deep into my wounds. Lucy left me with many gifts that I have slowly been unwrapping over these past six years since she left. The way I look forward to heaven now is a gift. My ability to appreciate life in a way I never have before is a gift. The way my heart has been softened towards others who are hurting is a gift. Even the suffering itself can be viewed as a gift because it teaches us things no one else can teach us.

Sheryl Sandberg wrote a book called Option B after her husband died unexpectedly and she was left to raise her two young children on her own. In one section of her book she writes about Joe Kasper, a man who lost his son but then found healing when he realized that his actions could be a part of his son’s legacy.

“While studying for his Master’s degree, Joe created a therapeutic process called ‘co-destiny,’ which encourages bereaved parents to view their child’s life in a larger framework so that death does not become the end of the story. Parents who seek purpose and meaning from their tragedies can go on to do good, which then becomes part of their child’s impact on the world. As Joe explained, ‘I realized that my destiny was to live my life in a way that would make my son proud. The awareness that I could add goodness to my son’s life by doing good in his name motivates me to this day.’ It’s not surprising that so many trauma survivors end up helping others overcome the adversity that they have faced themselves. ‘There is nothing more gratifying than helping someone else escape this quagmire of despair,’ Joe told us. ‘I know this passion of mine is an area of personal growth related to my trauma. Helping others grow from their trauma reflects back to my son’s life.’ After undergoing a hardship, people have new knowledge to offer those who go through similar experiences. It is a unique source of meaning because it does not just give our lives purpose- it gives our suffering purpose. People help where they’ve been hurt so that their wounds are not in vain.”

I can attest to the healing power of adding goodness to Lucy’s life by doing good in her name. This is part of the rubbing in of love and hope. Very few things in my life are as gratifying as helping other women who are dealing with a high risk pregnancy or the loss of a baby. My trauma has given me unique experiences and insights that I can share with others. I’ve been able to learn the ins and outs of this high risk pregnancy disorder and I now have the tools to help other women advocate for the right medical care during their pregnancies.

I have also been on the receiving end of this hope sharing. When Lucy was stillborn I was in the hospital for two days and two nights. While I was there I encountered many doctors and nurses who were kind but I only remember one woman who was able to comfort me with her words. A nurse told me she had suffered a miscarriage in the past and was devastated by it. She told me about her rainbow baby who had brought her so much healing. “You just have to try again, honey. You just have to get back up on that horse and try again.” Obviously, her words weren’t perfect because I couldn’t just “get back up on that horse and try again” like she had said since my next baby would also face anti-Kell antibodies like Lucy. But her act of sharing even just that tiny glimpse into her own pain, and her acknowledgement of my desperate desire for a living baby gave me hope. It encouraged me to hear about someone else losing a baby and then finding hope and trying again afterwards. Since then many, many people have reached out and shared their own stories of suffering with me and those are the people who have made a huge impact on my life. Our ISO Moms group on Facebook is full of women who have been through traumatic losses and high risk pregnancies and they use their suffering to lift other women up. We share what we have learned from our own pregnancies and we help others protect their babies. In fact, if I had not found the women in the ISO Moms group I don’t think my own children, Nora and Callum, would be alive today. I was not a part of the group when I was pregnant with Lucy so I had to figure out that pregnancy on my own. What a difference these women made in my life by sharing what they gained from their trauma.

My friend, Melanie DeSimone, lost her son, Dominic, several years ago and since then she has started a blog, The Life I Didn’t Choose, and a Facebook group for bereaved parents, Heartache and Hope: Life After Losing a Child. She has used her suffering to encourage and support other families going through similar losses. The wisdom and empathy that Melanie shares with thousands of people daily is only available to her because of Dominic, and because Melanie has chosen to rub love and hope into her grief.

For those of you who have suffered a loss or gone through a traumatic event or faced discrimination, what do you know now that you didn’t know before your crisis? How has your perspective changed? These are your gifts to share with the world, to bring purpose to your suffering and to help others who are facing a similar tragedy. And I can tell you from personal experience that when you reach out and use these gifts to help others, it will end up benefiting you more than you can imagine.

Lucy’s life and legacy live on through the families who have been changed by her story.  Here are some of the gorgeous babies from the past year or two who have been helped because of Lucy’s story. Their parents are some of the bravest people I know. I cannot describe how much joy and fulfillment these babies bring to my life and I feel honored to be a part of their stories. Happy birthday, Lucy. Some of these babies would not be here if it weren’t for you. I hope you can see what a difference your life has made in the world. I am so proud to be your Mommy.

Maggie

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Bay

 Oliver

Jake

Hayes

Cameron

Alex Ray

Francesca and Vincenza aka Frankie and Vinnie

Mila

Cole

Nolan

 Harris

Paxon, Madon and baby sibling on the way

Brianna Anne

Kolt

Eliza

Rainbow baby girl Stevens on the way

Piper

Evangeline

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Nusayba

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Uwais

Rainbow baby girl Stacey

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Baby Allen

Meadow Mae

Ezra (baby sibling on the way)

Mattea

Crosby

Anysia

Grace

Emmi

Zylas

Baby Rusch #3

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Cameela

Laney Maree

And beautiful Liliana, who, like Lucy, would probably be alive today if she had received the right care.

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Liliana

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Liliana

Hard Conversations

Our little rainbow baby, Nora, is now almost three and a half years old. She is in that shifty space between baby and kid where one minute she is using words like “consequence” and “incredible” in complex sentences and the next minute she is shouting for Mommy to come wipe her butt after a good poo. It’s amazing to watch her grow and transform into the person she will be. She is so different from her brothers; another species entirely. At her age they were reading independently and constantly trying to run over the edges of tall things. The boys would spend hours every day constructing train tracks and creating deadly train crashes along the way. Nora doesn’t care about reading because she can just make up the story herself if there isn’t an adult around to read it to her. She spends most of her day thinking about princesses, making up dances and songs, and caring for her baby dolls. She loves being in charge, taking care of people’s needs and feeling romantic.

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Parenting a three year old is both fun and exhausting because your child can now verbalize her thoughts about the world which means there is an endless stream of words and questions flowing out of her mouth. We laugh every day at something hilarious and ridiculous that Nora has said and we die from the cuteness too. She recently started telling us that she loves us and she uses it to her advantage when she doesn’t like our decision about something. “Nora, I have to go to work now.” says her Daddy. “But Daddy, you can’t go to work because I love you.” “Nora, it’s time to take a nap.” “But, I love you so much. I can’t take a nap.”

Nora is also very aware that she has a sister named Lucy who isn’t here with us but is still a very important part of our family. For Liam and Asher it comes naturally to include Lucy in most things, which still amazes me and feels like balm to my heart. When counting how many girls vs. boys in the family, Lucy is always counted. For projects at school they include Lucy in their family description most of the time. At Christmas we always hang Lucy’s golden stocking right there where it should be between Asher and Nora’s stockings. And our family ornaments always include Lucy’s name. But this Christmas Nora asked a lot of new questions that were painful and difficult to answer. “But how is Lucy going to open her stocking on Christmas if it is here? Will she be here in our house on Christmas? I want her to have her stocking. Can we give it to her?” And none of my explanations felt adequate. Recently, Nora noticed a little drawing taped on the wall in her room. It has been there ever since she was a newborn. Soon after we brought her home from Houston, Asher drew a special family picture for Nora and insisted we tape it on her wall for her to look at.

It includes Daddy, Mommy, Liam and Asher holding his baby Nora. Up in the sky, is Lucy, looking out of the window of her mansion in heaven, smiling. It’s beautiful and heartbreaking. Lucy is included as a natural part of our family but the separation is clearly illustrated. All of us down here together. Lucy up there in heaven. So Nora asked about the picture on the wall and I pointed everyone out and explained it to her and she loved it. But of course, she had lots of hard questions.

Nora notices more and more how the big brothers play together so effortlessly without her in the scenario. I mean, it only makes sense that she would not want to be part of their conversations about Fortnite or the imaginary Civil War battle they are meticulously planning out, casualties and all. But she can’t understand why they don’t want to play Barbie princesses with her or act out the Cinderella ball scene over and over again. In those moments I ache so deeply for Nora to have her five year old sister here with her. I can see the hours of fun they would have playing together every day, an easy friendship built into the family. My whole childhood was a never ending play date with my sister Kristin, who is 22 months younger than me. We didn’t even call each other by name. We called each other “friend” because we somehow knew that being friends was extra special. A person doesn’t have a choice about who her sister is, but she does get to choose who her friends are. And Kristin and I wanted everyone to know that we had chosen to be friends. We weren’t just sisters. Nora would LOVE to have a five year old sister friend, even for just one day. How many days of joy and friendship are missed? Years of friendship gone. A lifetime with her sister gone. And it’s hard for me not to let the bitterness take over as I think about the doctors at UAB with their careless mistakes and I want to ask them, “Do you know how many years of friendship have been lost because of your pride? Nora’s only sister, dead, because you couldn’t take ten minutes out of your busy day to check Lucy for anemia when I begged you to.” I forgive the doctors at UAB multiple times a week, sometimes multiple times a day, and I ask God to give me compassion for them. Thank God for the power He gives me to forgive and to love the people who are hardest to love. Without Him I would be drowning in my bitterness.

This past week Nora randomly said to me, “Mommy, I really miss Lucy and I really want her to come here.” It stopped me in my tracks. “I know, I miss her too and I really wish she could be here too.” I said. “But I want her to be here in my house and play with me. And I miss Lucy and you miss Lucy but Callum doesn’t miss her and Asher doesn’t miss her and Liam doesn’t miss her and Daddy doesn’t miss her like we do.” she said. I knew what she was trying to express, that the boys don’t miss Lucy like we do because they have each other. I told her that the boys do miss Lucy as much as we do, except for maybe Callum because he doesn’t know about her yet. “Why doesn’t Callum know about her?” she asked. “Well, he’s too little to understand and it’s hard to explain it to him, but when he gets older he will know who Lucy is.” “Will she come here tomorrow? To our house? Or maybe the next day?” Nora asks hopefully, and my heart sinks. This is the worst. “No, she’s not coming here.” “But maybe she can come later? To our house? And she can stay at our house.” Nora pushes. “I’m sorry baby, but she can’t come to our house because she is in heaven. But we can see her when we go to heaven.” I say, trying to steady my voice. “Oh!” she says, “so how do we go there? Where do we go through? Where’s the door?” “Well, we can’t go there until after we die.” I say slowly. Nora instantly looks horrified. “I don’t want to die!” Oh, now we are spiraling into uncharted territory. I don’t know what to say to my sweet little three year about the horrors of this world we live in. But I have to tell the truth so I say, “Everyone is going to die one day, but if you love God, after you die you can live in heaven forever. And it is going to be so wonderful.” “But I don’t want to die.” she repeats, looking up at me with her giant worried eyes. “I know, and you don’t have to worry about that at all right now.” I say, not exactly sure how to reassure her. “Will Lucy show me her toys when I get to heaven?” she asks and I feel relieved that we are now onto toys as opposed to inescapable death. Finally she lets me change the subject but I am shaken, and the rest of the day I feel desperately sad.

A couple days later as I was getting Nora ready for preschool she nonchalantly asked me, “When am I going to die?” and here we go again. Even though Nora’s questions are heartbreaking for me and difficult to answer, they remind me of how thankful I am that I do have the answers, even if they might be too complex for Nora to understand at the moment.

People die and kids grow up and everything changes but God remains the same. He never changes. He is constant and trustworthy and His love for us never changes. He is our anchor. He even conquered death so really, for those who know God, death is only moving from this place to our real home. Lucy is home and I wonder if she asks God, “Can Nora come to our house and stay? Will she come here tomorrow? To our house? Or maybe the next day?” And He says that the wait will feel like the blink of an eye and before Lucy knows it her family will be home forever. Together. And the most beautiful, comforting thing is that God does not leave us here to flounder in grief while we wait. He is here with us, filling in the gaps where we ache, where dreams are unfulfilled and people are missing. God can be our fulfillment, our strength, our peace, our joy. So maybe Nora has to wait until heaven to have a sister, but she can have other meaningful relationships here and she can have peace, joy and fulfillment through the God who never leaves or changes.

Zephaniah 3:17 The Lord your God is in your midst, a mighty one who will save; He will rejoice over you with gladness; He will quiet you by His love; He will exult over you with loud singing.

Romans 8:38-39 For I am sure that neither death nor life, nor angels nor rulers, nor things present nor things to come, nor powers, nor height nor depth, nor anything else in all creation, will be able to separate us from the love of God in Christ Jesus our Lord.

Deuteronomy 33:27 The eternal God is your refuge, and underneath are the everlasting arms.

Donate Plasma, Save Lives

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Are any of you interested in donating plasma in order to help save lives? As an added bonus, you can make some money while doing it. If you have anti-D antibodies or have a negative blood type, you could qualify to become a donor for a company called Grifols. They use plasma to create an important immune globulin medication (Rhogam) that is given to Rh negative women during pregnancy to prevent them from creating antibodies. Most of you know my story, that I have anti-Kell antibodies which killed my daughter Lucy and threatened the lives of my two youngest children, Nora and Callum. Anti-D antibodies work in a similar way and are much more common than anti-Kell, putting many babies at risk for hemolytic disease of the fetus and newborn. The Rhogam medication is given during pregnancy to prevent the anti-D antibodies from developing. If there was a medication like that for anti-Kell, I probably never would have lost Lucy or faced high risk pregnancies. My sister has a negative blood type and her husband has a positive blood type, so she potentially could develop anti-D antibodies. If she did, she would face a very high risk pregnancy situation like I did with Lucy, Nora and Callum. But because Rhogam is available, she simply receives an injection during pregnancy and afterwards and has never developed anti-D antibodies. Her two year old son and the baby she is currently pregnant with are both safe because of the Rhogam medication.

The Rhogam medication is made from plasma containing anti-D antibodies, which would not be possible without donors. If you are interested in becoming a donor, check out the Grifols website and see if there is a donation center near you. Feel free to contact them with any questions you have. Also please share this blog post so we can reach lots of potential donors. Thank you!

If you have Rh-negative blood, you have the ability to produce anti-D antibodies, which are needed to create an important Rh immune globulin medication given to women who are Rh negative during and after pregnancy to prevent hemolytic disease of the newborn (HDN). A condition where the immune system of a mother who is Rh negative attacks her baby’s Rh positive blood often resulting in the death of the baby. To learn more about our anti-D program, details and locations to donate, visit:

https://www.grifolsplasma.com/en/specialty-plasma/anti-d-program

Anti-Kell Antibodies: Get Those Numbers!

As with all of my anti-Kell posts, this one also applies to the other red cell antibodies during pregnancy; anti-D, anti-c, anti-E, anti-FYA, anti-Jka, and so on.

One of the best ways you can protect your baby is to find out exactly how your baby is doing, and one of the best ways to know how your baby is doing is to know exact test results (assuming your MFM is performing the right tests.) Often, doctors do not automatically give out exact test results but give their interpretation of the results instead. “Your baby is doing fine” or “Your results are in the normal range” or “Baby’s numbers have increased a bit this week but we will just keep an eye on him and scan again next week.” With most conditions this is the norm; doctors interpreting the results into understandable, bite sized sentences that patients can grasp easily. But most conditions are more common and doctors encounter patients with those conditions on a regular basis. Our disorder is far more rare and MFMs encounter it less frequently than the other pregnancy complications they deal with. Your doctor might not be up to date with the best practices regarding monitoring and treatment for your baby; and yes, even at the best hospital in your state or the best hospital in your country. This is why it is so important to get your specific test results, even if you aren’t exactly sure what to be looking for at first. Numbers are concrete. You can find out specific information with your number in hand. You can use those numbers to help the doctors protect your child. But if you just have your doctor’s opinion, you can’t help advocate for your baby or make sure you are getting the correct treatment.

Sometimes it is awkward to ask for the exact number after your doctor has concluded, “Everything looks great!” or “The numbers are a bit elevated but baby should be ok for now.” Some doctors might take it as a challenge, an irritation in their busy day or as a simple question that they are happy to answer for you. Some doctors like it when their patients want to be educated and involved in their care decisions. Regardless of how your doctor responds to your inquiries, those are YOUR numbers. They belong to you and your baby and the medical professionals are required to give you what belongs to you if you want it. Don’t feel bad for asking. Think about when your baby is finally born and they put her on the scale and you anxiously wait to see how much your bundle of love weighs. Now imagine the nurse writing the number down on your chart and not mentioning it to you. You ask for baby’s weight and they say, “Oh, it’s in the normal range,” or “It’s a bit higher than average.” Would you be ok with that? No! You want to know how much your baby weighs! We expect to receive those numbers after baby is born, but sometimes we are willing to let these other, far more important numbers go. These numbers can determine whether your baby lives or dies, so GET YOUR NUMBERS and record them in some way (notebook or phone, etc.) Envision yourself standing in front of your baby ready to fight the antibodies that threaten your child. These numbers are the weapons in your hand, ready to defend and protect. Here is a list of the most important numbers you need to record during your ISO pregnancy:

Antibody Titer: Your antibody titer gives you an idea of the amount of antibodies present in your blood. The monitoring and treatment you receive is often based on your antibody titer, and these ultrasounds and treatments are very time sensitive (my daughter might be alive if we had done the scans and treatments in time.) As soon as you find out you’ve had a positive antibody screen ask your doctor for your antibody titer and write it down, or ask for access to your medical records so you can look for it yourself.

PSV or MoM Number: This is probably the very most important number you will get during your pregnancy because it shows if and to what extent your baby is being affected by your antibodies. This number is far more important than your titer even. At every MCA scan your doctor is looking at the PSV (peak systolic velocity) number and it will show on the screen when they are doing the scan. You can look on the screen and try to find it or ask for the number. You use the PSV number and your exact gestation to determine baby’s MoM (multiples of median) which shows if baby is anemic or not. An MoM of 1.5 or over shows that baby needs an intrauterine blood transfusion. I use this website to calculate my MoM https://medicinafetalbarcelona.org/calc/  Click on Anemia then click on Cerebral Doppler, then plug in your gestational age and your MCA PSV number and it will calculate your baby’s MoM. Or you can ask your MFM for baby’s MoM instead of the PSV if you prefer. I kept a notebook with all of Lucy, Nora and Callum’s weekly PSV and MoM numbers so that we could compare them to each other and track the numbers to see if there was an upward trend starting (those often lead to IUTs.)

Beginning and Ending Hematocrit/Hemoglobin: These numbers are only important for the women who need IUTs. The beginning and ending numbers are important because they show how anemic baby is before and after the IUT. It always helped me during the IUT procedure to focus on the numbers, so the doctor would call out the hematocrit once they checked baby’s blood and I tried to remember the number instead of focusing too much on what was happening. The doctor called out the ending hematocrit once they got the blood to baby. If the doctor didn’t call out the numbers, I asked and it always made me feel better. If you prefer not to think about numbers during your IUT, just ask the MFM what the beginning and ending hematocrit/hemoglobin (some doctors prefer to use one or the other) was once you are in recovery for monitoring after the procedure. Knowing how anemic baby was before and after the IUT can help you answer all kinds of questions like- “Did my baby actually need an IUT?” “How long can my baby wait until the next IUT?” “Did my baby get enough/too much blood during the procedure?” “Was my baby anemic enough to cause future problems?”

*Side note- It is important to remember that the MoM does not always correlate with an exact hematocrit in baby, so the doctors can’t know EXACTLY when the best time to transfuse is. The MCA scan gives an idea of how anemic baby is and the 1.5 MoM cutoff is the best tool they have for knowing when to transfuse. Some babies will be more anemic with an MoM of 1.5 than other babies with the same MoM. To give you an idea of how my babies’ MoMs correlated with their hematocrits here are their beginning numbers for their first IUTs:

Lucy- MoM=2.5 Beginning hematocrit=6

Nora- MoM=1.6 Beginning hematocrit=26

Callum- MoM=1.49 Beginning hematocrit=28

Bilirubin: This number is very important after your baby is born. The type of jaundice our babies deal with is different from normal newborn jaundice. Our babies’ bilirubin usually peaks between days 4-6 so make sure they continue checking bilirubin until baby is past the peak days. Hemolytic jaundice often has to be treated more aggressively than normal newborn jaundice so you have to make sure they are tracking baby’s bilirubin closely and treating it appropriately. It is very common for our babies to be discharged too early while bilirubin is in the “safe” zone but still rising and then the babies have to be brought back and readmitted with high bilirubin levels later. High bilirubin can sometimes cause permanent brain damage, but it is completely preventable so these numbers are very important. Also be sure to ask how old baby was at the time of the blood draw since that affects treatment levels. I think Callum’s bilirubin level at birth was 5, which would not be a big deal if he was a couple days old, but right at birth that is considered a high number. So get the bilirubin number and how many hours old baby was at the time of the blood draw. This is a good tool to use to find out if your baby’s bilirubin is high enough to need treatment: https://peditools.org/bili/ 

Hematocrit/Hemoglobin: These numbers are very important after baby is born. These numbers tell you if your baby is anemic and whether baby needs a blood transfusion or not. Transfusion levels vary depending on your specific circumstance so the ISO Moms Facebook group is a great place to ask questions about whether or not your baby might need a transfusion. You can also email me if you have questions about this. Of course, we cannot make medical decisions for you but we can share our insights and experiences and you can combine that information with your doctor’s suggestions. When Callum was in the NICU after his first post birth transfusion the doctors wanted to let him drop below a hematocrit of 25 before transfusing. That isn’t super low, but I felt uneasy about waiting too long to transfuse since he was dealing with so many other preemie issues on top of the anemia. He got to 26 and was having more oxygen desats and heart decels and wasn’t feeding as well as he had been. I had a suspicion that if we handled the anemia the other issues might improve as well. I spoke to his neonatologist about this and said that I felt strongly about going ahead with the transfusion. She heard me and agreed to let them transfuse even though he hadn’t dropped below 25. Callum improved so much after his blood transfusion. No more oxygen desats or heart decels, his feeding improved rapidly and he was discharged from the NICU two days later. Make sure that your baby’s hematocrit or hemoglobin is followed after discharge from the hospital. Delayed anemia is normal for our babies since our antibodies linger in their bloodstream and continue to destroy their red blood cells for weeks after birth. One of my friends lost her beautiful daughter because none of her doctors followed up with blood work after her healthy baby was discharged from the hospital. By the time they caught the delayed onset anemia weeks later, it was too late. Dr. Moise did not allow Nora to be discharged from the hospital after birth until we had an appointment set up with a pediatric hematologist the following week, even though her numbers were perfect and she wasn’t anemic at all at birth. With both Callum and Nora we had weekly blood draws to check hematocrit and hemoglobin and both babies needed transfusions a little over a month after birth.

Ferritin: Most of you will not have to worry about this number at all, but it can be very important if your doctor wants to give baby iron supplements after birth. If your baby had IUTs or was Coombs positive after birth, do not allow the doctors to give baby an iron supplement unless they have checked baby’s ferritin levels first. The ferritin levels show how much iron the body is storing. Doctors often mistakenly equate hemolytic anemia with iron deficiency anemia and want to treat babies with extra iron. When a baby is given a blood transfusion, in utero or after birth, they are given adult donor blood which has adult level iron content. This means our babies are usually overloaded with iron so adding more iron can be dangerous. Since our babies aren’t anemic from low iron (they are anemic because the antibodies have been destroying their red blood cells) adding more iron won’t help their anemia at all. When Callum was in the NICU, after four blood transfusions (3 in utero, 1 post birth) his neonatologist wanted to give him an iron supplement. I told him I would only allow it if they checked his ferritin levels first since he didn’t have iron deficiency anemia. They agreed to check his levels and they were 1,216! The normal range for a baby his age was 25-250. We had to consult a hematologist and do some liver function tests and even discussed possibly doing a liver biopsy to check for liver damage. I’m so glad we didn’t give him MORE iron, adding to his extremely high levels. So, if you have a Coombs positive baby (who might need a blood transfusion in the near future) or have a baby who has had a transfusion, only allow the doctors to give an iron supplement if they have checked the ferritin and baby has low levels.

Remember how useful these numbers can be in your fight to protect your baby from your antibodies. If you get your numbers and then realize that you aren’t sure what to do with them, feel free to email me with any questions you have, or come join our ISO Moms Facebook group for access to about a thousand other women who have been or are going through the same thing. It is a secret group so you won’t be able to search for it, but you can search for our public page, Maternal Isoimmunization Support. Just send me (Bethany Weathersby) a private message or send the Maternal Isoimmunization Support page a private message and we will get you added to the group!