The People Behind the Scenes

I hear the words "super mom" quite often.  People wonder how I juggle work, Eli's bandage changes, preparing to leave for Minnesota for 4 months, the fundraising we have been doing, and basic every day stuff.  I post updates on Eli almost daily and those updates sometimes include pictures of the two of us or videos that I have taken of him or talk about appointments that I take him to.  That is what people see-mom does it all. 

But that is not the truth. Mom does not do it all.  Mom has a team of people who help her juggle it all.  That team includes FIVE home health care nurses who adore Eli and understand his condition as well as we do.  They change more diapers than we do and do more of his feedings than we do-because while mom and dad are at work-they are at home with Eli making sure he is fed, clean, happy, and his pain is under control.  Sometimes when I don't have to work, they even let me get an extra hour or two of sleep after Eli wakes up. They set up and almost always clean up after bandage changes which save us a hour out of our day.  They have seen us lose our ever-loving minds when our son is screaming, twisting his wrists or kicking his legs preventing us from bandaging him back up.  One of our nurses spends hours every week working the graveyard shift to cut bandages while the rest of us are tucked away in our bed and she was up all day with her own toddler. She used to attend to Eli's every hour or two wakings on the nights she would work but for the most part we just let him sleep in bed with us now.  Our nurses have gone to doctor appointments with me, have helped us clean up bloody vomit from our hands, sheets, and towels, and have sprayed poop out of Eli's clothes.  They sometimes drive me crazy-because I am a particular person about some things-but I know that feeling is very mutual between all of us.  Without their help and love for Eli our life would be far more difficult to manage! Part of me is dreading the thought of not having them to talk to or help me while we are in Minnesota. 

Beyond the help of our nurses, I have to toot my husband's horn.  He hates being on camera so I don't get to share pictures of him and Eli too often.  He works 40 hours a week, comes home two nights a week to do evening bandage changes with Eli (which take half the time that mine do because Eli isn't as attention seeking during bandage changes with dad nor is dad AS particular as me), and on the other days he does not have bandage changes he is remodeling our home after a 9 hour day at work.  On days like today, when mom is at work and dad is at home with no nurse he is making sure Eli is fed, takes a nap, changes his diapers and/or sits him on the potty if Eli requests it, and is still nailing down flooring in our living room to keep the remodeling project going forward. 

We have owned our house for 5 years now and have experienced one miscarriage, adoption of a kitten, one failed IVF, one successful IVF, 9 months of pregnancy, and 30 months of parenting a high needs medically fragile child in our home.  We have also experienced a septic tank back flowing into our basement, a new sewer line being improperly installed to then be reinstalled, the city storm sewer flooding our basement at least 10 times, installing four sump pump pits in our basement to keep the water out, our furnace and a/c going out on us requiring replacement, our dryer breaking numerous times requiring replacement, our dishwasher breaking on our son's birthday requiring replacement, I think I remember our microwave blowing up too which required replacement, tearing down three bedrooms, one bathroom, a living room, a dining room, a hallway, and a stairway to the studs due to having poor plastered walls, poor insulation, poor to no ventilation in areas of our home, and knob and tube wiring throughout the home, and have worked for the past 5 years to make our home safe, clean, and modern.

And when I say "we"-I really mean "he." I don't lift a finger when it comes to installing new appliances or tearing down walls or anything else that needs to be done. I just pick out what I want and usually my husband trusts my design judgment.   Most of the work done to our house has involved minimal contracting and a whole lot of labor from my husbands own hands.  He hears me complain a lot, about how much time it takes away from being involved in family activities and about the mess I can't stand living in at times or a sink full of dishes when I was working all weekend and he was home (working on the house of course-just not things that I would have done), but truthfully-I love the finished product when it is all done and I know deep down he wants to make our house as clean, organized, and safe for Eli in the end and he is just trying to do that as quick as he can.  Every year he uses his annual bonus that he works his tail off for-taking phone calls and emails on his days off and even on our vacations-to continuously improve our home. 

Beyond our home-my husband manages our bills.  After Eli was born, this was part of our life that I handed over to him.  I trust that as a banker he knows how to manage our finances and will let me know if I need to be aware of something important.  Occasionally I log into our bank account-like 10 times in the past two years. He does our own taxes every year and has been the one to set up our fundraising accounts and met with lawyers to make sure what we are doing is legal. He has been the one to get our power of attorney paperwork and living wills taken care of during this process of preparing for MN. 

And yet....he still participates in our community more than I do.  He is involved in numerous clubs and heavily involved with the Journeyman's Club where he has played a vital roll in the annual Easter Egg Hunt and Oktoberfest Parade the past several years since Eli was born. 

So if anyone deserves to be called "super" anything....it is these people.  The ones who are behind the scenes of every post about Eli and our life.  And if anyone is the worst at expressing these feelings to the people who are most deserving of this acknowledgement it is me. Sometimes, ....most of the time, I focus on what wasn't done the way I wanted it done or how I would have done things and I get agitated and snippy instead of expressing appreciation for the help and the hard work that goes into our son and our home.

We each share a piece in Eli's puzzle. Not one of us more than the other. We are a "super team" who strive our best to ensure Eli has a good quality of life that is filled with love and laughter and a feeling of safety and security.  We also keep each other balanced and without the roles that each of us play-I personally would not have the little bit of sanity that I do have or the time that I can devote to being the mother that I am.

 

 

 

 

 

 

 

 

New Year; New Page; New Journey

Happy New Year from Alex, Lindsay, and Eli-or better known as "Baby M" in the pregnancy and fertility world!

It's been a long time since I have shared anything on our blog...since September 16, 2016 to be exact. Time flies when you're having fun I guess.  If you can call parenting a toddler fun, not to mention a toddler with medical needs😉 Let do a little recap of our journey and then I will catch you all up to speed on our current happenings.

I started blogging in January 2014, at the start of our first fertility cycle to express my emotions and in hopes of helping other women not feel so alone.  My husband and I met in 2006.  We lost a daughter to stillbirth at 24 weeks on March 1st, 2008.  I had an early miscarriage the following year in September.  We fostered a 16 year old with Reactive Attachment Disorder in 2011.  2012 was super adventurous between being diagnosed with a rare genetic disorder, Homocystinuria (a 1 in 400,000 disease), having five emergency eye surgeries, graduating with my masters degree, getting married, and conceiving another baby.  On Martin Luther King Day in 2013, at 11 weeks our baby no longer had a beating heart and the following week I had a D&C.  Fast forward 9 more months and we had another miscarriage, a blighted ovum. 


Through all of our losses we were told a variety of different causes.  Our daughter was born with her cord wrapped around her neck so her death was considered to be cord strangulation.  The first miscarriage...well these things just happen.  Once I was diagnosed with Homocystinuria we were told I was at high risk of blood clots during pregnancy which leads to miscarriages and stillbirths.  This seemed like a better explanation to us...something we could treat at least. So when we fell pregnant at the end of 2012 I started Lovenox injections immediately to try to prevent blood clots and more heartbreak.  Unfortunately, until March 2013, we did not know that two of my chromosomes are fused together which will result in a 25% chance of having a child with Down Syndrome and a 25% chance of having a child with Trisomy 13.  Both of these medical issues can be fatal during pregnancy or after birth.  Our last two miscarriages both had Down Syndrome.


The most logical plan of action at that point was "treat" the things we knew were "wrong" with me.  Blood thinners for pregnancy-check. But the chromosome issue?  That's when we invested in in-vitro fertilization with pre-implantation genetic screening.  I hated the idea of "choosing" a baby based on rather or not they were healthy.  I always thought that saying when people ask you what do you hope you are having when your pregnant-"it doesn't matter as long as it's healthy"- is kinda a crock of crap. I believe as a parent you love what you are given.  Boy, girl, extra/missing fingers and toes, chromosome or genetic conditions that cause various organ problems....I am pretty sure that when it is your own child you can't help but to love them.  Of course nobody wishes or hopes for a medically fragile child.  And in our case it felt as if we being selfish by trying to have a baby that we knew for a fact would likely either die during my pregnancy or would have severe medical problems.  It didn't make sense for us to keep putting ourselves through heart ache or to bring a child into the world that would have to fight for its life. 


God, of course, doesn't always have the same plan that you do.  Sometimes that is a hard thing to accept.  Our first IVF cycle failed.  We had 12 eggs collected during my non-medicated extremely painful egg retrieval, only 7 of those were mature enough to fertilize, and only four continued to grow long enough to biopsy and test for chromosome abnormalities.  Among those four, we had three with confirmed chromosome issues-only one of which was related to my fused chromosomes.  The fourth embryo had an unconfirmed result so we blindly decided to give it some faith and prayed for the best.  After our negative pregnancy test we were left with $20,000 deeper in debt and questioning what was next for us. 


After a long and bumpy ride we ended up pregnant from my sister's donor eggs 9 months later.  We took every precaution under the sun to ensure our baby would be "healthy" and our pregnancy would last another 9 months.  Lovenox injections daily to thin my blood. Progesterone shots daily for the first several weeks to keep my level up. Metformin daily for my insulin resistance.  Synthroid daily for my slightly elevated thyroid hormone. Aspirin daily for extra blood thinning. Intralipid infusions every 2 weeks for 14 weeks to treat any potential auto-immune problems. And a whole slew of vitamins specific for my Homocystinuria. 


I was the happiest I had ever been.  I was pregnant with our Rainbow Baby.  I was anxious, yes.  But I really enjoyed every moment of being pregnant and was obsessed with hearing "Baby M's" heart beat and watching him grow.  Despite the risk of growth restriction due to having a Velementous Cord Insertion in the placenta, which occurs in only 1% of pregnancies of course, our son was born a near 8 lbs on August 8th, 2015.  "Baby M" was named Eli Francis Monnier and was immediately wisked away to the NICU due to having "a problem with his skin." 

Recessive Dystrophic Epidermolysis Bullosa. RDEB for short.  It is a rare, incurable, progressive condition that affects the largest organ of the body, the organ that protects the rest of the body from bacteria, regulates the body's temperature and moisture levels, and contains receptors for recognizing pain.  It is caused by two mutations in the Collagen VII gene and results in body being unable to produce the protein that "glues" the layers of skin together.  Any slight trauma or friction can tear the skin off or shift it enough that a large blister forms. This protein is also vital to the mucosal tissues which are found in the eyes, nose, mouth, esophagus, trachea, lungs, stomach, intestines, ureter, urethra, and bladder. Life expectancy with RDEB is around 25 years or less. Cause of death is typically infection, skin cancer, or heart failure.

 

I have questions that verse so many times.  I would be lying if I said I haven't.  But I have say two and a half years later that God has given us a child who is brilliant, funny, loving, strong-willed, and puts a smile on our faces all the time. 

It has not been an easy life so far, and I know our future will have it's own hurdles to jump.  We have treated bacteria infected wounds every day, have held our son while screaming in excruciating pain from bowel movements that rip apart his rectum despite his stool being soft, have applied antibiotic cream to our son's eyes from multiple corneal abrasions, and have held handfuls of blood from blisters rupturing in our son's esophagus.  We have gone to work more days than I can count with only two hours of sporadic sleep.  We have battled feeding issues and have had to learn how to care for a gtube.  We have purchased around 100 different creams/gels/lotions hoping one of them will be the miracle that heal a wound overnight.  And we administer around 10 medications a day to keep our son as healthy as we can and treat his pain. 



Every day we pray for a cure. We pray for hope. We pray for guidance. 

That prayer has led us to our next journey.  Eli has been approved for a bone marrow transplant at the University of Minnesota. It isn't a cure.  But it is the only systemic treatment option for severe cases of Epidermolysis Bullosa. Eli is considered to have severe generalized EB, meaning both of his gene mutations have been seen in other "severe" cases and his condition will only progressively get worse to the point where he will have no areas that have been untouched by blisters.  His fingers and toes will fuse together in the future.  His toes are already fusing due to the recurrent blistering and scaring that has occurred so far in his short life.  Internal issues will also progressively worsen as we have already seen over the past 8 months. As a systemic treatment, the goal of the bone marrow transplant is to help Eli's body create enough Collegen VII that the condition will not be AS SEVERE.  It could help him produce enough of the protein that he should not blister as often or as large and should heal faster.  It should give his skin enough strength that it shouldn't rip off or blister with every minor fall or accident (example-a ball hit his chin the other week when tossing it back and forth with me and it tore the skin off instantly). With these potential benefits come very scary potential risks which includes death related to infection and organ failure due to killing off his own bone marrow (immune system) so he can accept someone else's. 


That "someone else's" is his father's. Eli's dad will be his bone marrow donor.  After discussing it with the doctors and geneticist in Minnesota this was the most logical choice.  The current protocol for transplant is to use a half-matched related donor, which is a sibling or parent.  Eli has no siblings and I am technically his aunt....plus my own genetics are terrible so even if I were his biological mother I would not be able to be his donor.  That leaves my husband and my sister.  Both are in general good health and have no crazy medical issues.  However, this is a time consuming process and my sister has her own child to take care of and life responsibilities including a demanding work schedule.  Part of the protocol, using a related donor, allows for cell boosts up to five times post transplant which has been proving to be more successful than previous protocols where full matched non-related or 8/10 related matches were used but no additional cell boosts were given. 


Despite the scary negative side effects that can occur, there is a lot that gives us hope.  This past year they started testing for antibodies against the donor.  This gives them an idea of how likely the patient is to reject the transplant or develop graft vs host disease.  With this new knowledge, they now know that if a patient has antibodies against their donor they either need to select a different donor or get rid of the antibodies.  Eli DOES have antibodies against his dad.  They are a low enough level that the doctors are comfortable still using his dad as his donor but we will retest them the week before we go to MN to see if we need to get rid of the antibodies as his level can fluctuate.  Another thing that gives us hope is that even older children who have had more years of "damage" from EB are having successful transplants over this past year.  Eli is overall in good health and his labs are always excellent which makes us believe his body will be able to withstand the difficulties of the transplant process.  He has a strong personality also and is a little fighter. 


So in this new year I will be preparing to "move" to Minnesota from March-August (potentially longer) to be with Eli while he receives his transplant.  We will continue to share our journey on my personal facebook page and our family page which has been renamed from Alex and Lindsay's Baby Quest to "Rainbow Baby; Butterfly Boy" and can be found at www.facebook.com/rainbowbabybutterflyboy .  Our new page name is more fitting to our current journey as we are 100% focused on Eli and 0% focused on extending our family at this time.  If you aren't a follower on our page yet, join us for our 2018 Journey through Transplant and become another prayer warrior and supporter in Eli's Fight Against Epidermolysis Bullosa!