You may be wondering. . .

Silas - 1 month old

My sister, Julie, called me yesterday and wanted to know what would be happening with Silas now that we know he has hemophilia.  She asked some really good questions, and I thought some of you may be wondering about some things too. 

Q:  Is Silas getting bruises or bleeds yet?
A:  No.  Silas has no bruises.  Some of you may remember that Eli didn't get any bruises until those 2 hematomas showed up when he was 4 months old.  It is normal for hemophiliacs to have bruises (most always hematomas) all over just from normal life when they get more mobile.  These "superficial" bleeds do not need to be treated with factor, but bruising on a joint needs to be watched carefully as it may go into the joint. 

Q:  Is there ever any difference in severity levels?
A:  There are different levels of severity between hemophiliacs, but not within the same family.  My gene mutation is called 22 inversion.  That gene mutation is always severe hemophilia A.  Ana is a carrier like I am.  She bruises more easily, but she and I have another X that makes up for the hemo X.  Eli and Silas do not have another X.  So, they have severe hemophilia A, which means their bodies make <1% of clotting factor 8. 

Q:  When will he need to get factor?
A:  For the first year or year and a half of Silas's life, he will be receiving factor as needed.  For Eli, the first year included soft tissue bleeds in his cheeks (both sets ;o)  ) and worrisome head bumps (none turned out to be a head bleed). 

Q:  Will he be getting a port? and when?
A:  A port is not always a necessity; it is a choice.  We chose to get a port for Eli because we wanted to preserve his veins, and have an easy access in case of an emergency and a vein would be hard to find (dehydration, etc.)  We will probably get a port for Silas since it has worked out so well for Eli, but we will be making that final decision later.  He would get a port when he needs to begin prophylaxis.

Q:  When will he start prophylaxis?
A:  He will begin "prophy" when he is 18 months old or when he gets his first joint bleed.  Whichever happens first.

Q:  Are we doing anything extra to prevent bruises and bleeds?
A:  Yes.  He will start wearing a comfy cap when he starts spending more time on the floor (for 4 obvious reasons ;o)  ).  We bought a sleepy wrap because it is stronger and softer on him than a Maya Wrap or Moby Wrap.  We were not planning on letting him use the jumperoo, but some of my online hemo mom friends told me that they did not have any problems with the jumperoo at all and in fact, it helped their boys' legs get stronger.  Another mom suggested a product called Hugga-Bebe.  It is extra padding made for jumperoos and exersaucers.  It looks excellent, and Matt and I are looking into getting one and trying the jumperoo.  We will use knee pads and elbow pads to protect his joints, and pad his clothing.  By the way, an excellent company that makes padded clothing is Bruz Wear.  We have one pair of pants from them, and they're adorable!


I added a few more questions than Julie asked, but you may even have more.  Please ask anything in the comments section below.  We love to help people gain a better understanding of hemophilia.  Sometimes Blogger doesn't let people comment.  If that happens to you, let me know.  You can comment on facebook or email me at bubblewrappedbirth@yahoo.com

"Why Home Birth?" by Stacey Rainer

The following was written by a hemophilia mom who worked as an OB nurse.  With her permission, I have republished it here from where she originally posted it on "cafe' mom" back in June 2007. 

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So, someone asked me why I choose home birth, so I decided to write a journal entry to explain it, as I think it's too long for a reply to a previous entry.

First, my birth history. My first three pregnancies were induced because I had high blood pressure. The first was the worst, but the other two were not far behind. I was over 39 weeks each time, so no preemies or anything. I was never sick enough to need meds for my blood pressure. The first two times, I had Pitocin to start my labors. The third time, I did not want it, so I requested to be induced by rupture of membranes. By this point, I had been an OB nurse for a year, so I had a pretty decent working knowledge of the birth process from more than just a participant. During my pregnancy, I began to question circumcision. I talked to several doctors about it in the course of my work, and decided I'd not do it to my children. I had all girls up to this point. After that third baby was born, during my foray into the anti-circ world, I became acquainted with things like not vaccinating your children and home birth. When that child was 9 months old, we made the decision to stop vaccinations. I also decided that my future children would be born at home.

Two years later, I was pregnant again. I could not find a midwife in my new state, Illinois. So, I called a midwife I knew back home in Kansas, and asked her if she would attend my birth. I would travel there at 37 weeks or so, and hang out with my sister until I went into labor. It was all arranged and working well, until 7 weeks along. A sonogram showed twins. I immediately dismissed my home birth plans and while I hoped desperately for a vaginal birth, I knew my chances were slim. I was right. C-section for "unfavorable" position. After that, I'd had it. No matter what happened, I was having a home birth next time. Of course, I still have not found a midwife. Not to be one to wait until I needed, I got on the internet and began searching shortly after my boys were born. Didn't find a midwife, but became acquainted with something else new. Unassisted home birth. This was cool! By this point, I'd been working in OB for 4 years. I felt confident that we could do this. So, two years later, when I was pregnant again, this was the plan. I opted for an unassisted pregnancy as well. I did my own prenatal care. I did finally meet a midwife, just 2 weeks before she was born, but I had already made my plans, so stuck with them. I had a fantastic, 2 1/2 hour birth, in a pool in my family room. It was the most amazing thing ever! I knew then, that I'd never have another baby in the hospital again. So, two years later again (yeah, I know. I have a pattern), when I became pregnant, we didn't even discuss it. We knew we'd be having another unassisted home birth. The time came when labor started, and it was a long, grueling, 52 hour labor. I required a bit of assistance this time. I was ready to throw in the towel and head to the hospital at 50 hours. A midwife friend came over, broke my water and left. Two hours later, I was holding my screaming baby boy.

That's the birth history. Now for the whys of it all. During my career as an RN, I began to see how much stuff is done to women simply because it's what's easiest for the care providers (OB and RN). It's much easier to watch a continuous feed of fetal monitoring at the nurse's desk than it is to hunt down the walking mom every 15 min. to listen and count with a doppler. It's certainly more convenient to break mom's water or give her Pitocin when things are going a bit slower than the OB would like so he can "get that mom delivered" by a decent hour. Those late nights are hard, after all. I began to realize that birth is so much less traumatic both for mom and baby when it's left alone. However, it's nearly impossible for birth to be left alone in the hospital. Everyone's got to get their hands in it, literally. That's not something I want. My first baby was taken to the nursery for the first 6 hours of her life. I needed to rest, you see, because of my blood pressure. It was the longest 6 hours of my life (longer than that labor had been, actually). I hated it. My second was given for adoption. My third, the took her from me, put her in a baby warmer where I could see her. She was messed with, poke with a vit. k needle and a hep b needle. Junk was put into her eyes... she was exhausted by the time I finally got to hold her. She was way too tired to breastfeed. Fortunately, it didn't hamper future attempts and she nursed fine. My twins were taken from me since I had to go to recovery. But, by then, I knew to request no needles, no goop. I wanted them alert when I got them back, so they would nurse and so I could meet them. That was fine. I only had to wait an hour to finally hold my babies. It was better than before, but still not ideal. When my twins were 5 months old, one of them was diagnosed with hemophilia. His blood does not clot properly. So, things like a circumcision, or an intramuscular injection can cause bleeding that won't stop. Thankfully, we'd avoided that. However, the question then became, is it safe to have a home birth when this is a possibility for future children? Well, the next baby was a girl and I knew she was before she was born, so no worries. Then came Ian. There is a 5% risk of bleeding on the brain with vaginal birth. After some reading, I discovered that the risk of this bleeding without the use of instruments to deliver goes down to less than 1%. Well, that's a no brainer. I don't own forceps or a vacuum extractor so there was no concern about an instrument delivery (however much I thought I wanted one by the third day of labor). Well, I knew this baby was a boy, and I knew he had hemophilia. I did not know this through any tests of any kind. I just have a great gut instinct for this kind of thing. Well, he was born, I drew blood from the placenta as we'd arranged, and my husband took it to the clinic for testing. I was right, but my baby was fine. Some would suggest I got lucky. I would say I planned things in such as way as to be sure my baby would be born gently so that there was no risk of bleeding of any kind. Let's talk about risk of bleeding, since this was a question asked of me. Besides that less than 1% risk of brain bleed jumping to 5% in the hospital, there are many other opportunities for a hospital birth to actually cause bleeding. Artificial rupture of membranes - bleeding from the big scrape that is now on the scalp. How about that deep suctioning they do after birth? GI bleed. Rough handling, flicking of the feet, vigorously rubbing the back all to stimulate crying - muscle bleeds. IM injections - muscle bleeds. Of course, there is ample opportunity in all of this for big ugly bruising to be left behind by uneducated staff. This all just addresses the hemophilia. But why home birth at all?

Ultimately, I have a very strong faith in my Maker and the way He created me. I am a strong, healthy woman. My body was designed to conceive, grow, carry and finally birth a baby all on it's own. I also believe that a vast majority of fetal distress and failure to progress cases are caused by hospital intervention. The use of continuous fetal monitoring has NOT improved outcomes when it comes to fetal mortality. It has, however, increased the incidence of c-sections 3 fold. Pitocin has become a drug of convenience rather than emergencies. Docs going out of town? No problem We'll induce before she goes. Mom is only here to help you for one more week? Ok. Let's induce. You're due tomorrow? Well, let's just get you scheduled. Something like 90% of all inductions are not medically necessary. Labor's going to slow? We'll augment with Pit. Why? Oh, just to get it over and done with for you. Sheesh! No one even talks about the RISKS!! Yes! That's right! There are RISKS to this drug! In fact, it's not even approved by the FDA to use for augmentation! But, who needs to follow labels? Let's use this great new blood pressure drug, Cytotec to stimulate labor! So what if it causes ruptured uteruses? That doesn't happen to every one. Think of all the time that can be saved by getting labor started on a time table. Obstetrics is the only medical specialty that is not evidenced based. It's tradition based. There have been multiple studies that have proven the old ways are wrong but it's still going on! Why? Why is there such a need to manage something that works just fine on it's own? Pregnancy is not a disease. It's not a sickness. It's not an emergency waiting to happen. It's a normal function of life. We go to the doctor when normal functions aren't happening normally. Your hearts not working right? See a cardiologist. Blood not working right, as in our family? See a hematologist. Birth IS working right? See an OB anyway. Do you get it? Do you understand why someone might choose to flee as far and as fast as possible? Because what I DON'T get, is the American practice of obstetrics. I don't get it, and I won't have it. Home, where there is peace and gentleness surrounding the entrance of my baby into this world. There's enough violence throughout life. It doesn't need to start with the first breath of life.

"He has it." I said to my midwife. . .

It was evening.  Matt had taken the cord blood up to the hospital, and had been home for a few hours.  We'd taken our herb bath and were now resting and breastfeeding in bed.  Now, Matt was on the phone with our hemotologist getting the results of the blood test.  He was on the phone for a long time in the next room.  That was my first clue.  Then, I heard him say, "No, we haven't seen any bruising at all." 

"He has it."  I said to my midwife. 

Matt got off the phone and confirmed that I was right.  The first thing I felt was a heaviness.  Here's this perfect baby boy nursing in my arms, and now we know that he has a severe bleeding disorder.  Of course, I was not shocked or suprised.  We knew he had a 50% chance of inheriting my gene mutation.  However, knowing it's a possibility and finding out it's a fact are 2 very different things.  I didn't cry.  I just felt some heaviness in my heart as I thought about what was ahead for this sweet baby. 

Our midwife left.  Then, Matt called Eli into the room.  Both of us wanted to tell him first.  When he heard that Silas had hemophilia just like he did, his little face softened in a way that I'd not seen before.  He moved over to Silas, and warmly said, "Aw, Silas!".  He had the most loving smile on his face. 

"Do you want to hold him?"  I asked.  He did, and climbed up on the bed to hold his new baby brother. 

Perhaps even sweeter than Eli's reaction was Isaiah's.  When we told him that Silas had hemophilia, his face lit up with excitement.  "Really?!"  he exclaimed, and he jumped up and ran to Eli.  (He was unaware that Eli already knew, and ran out before we could tell him.)  "Eli, guess what?!  You have a little buddy now.  Silas has hemophilia just like you!"

Of course, Silas didn't have to have hemophilia to be Eli's buddy, but we knew what he meant.  It was so precious!

I had my cry later that week when I checked facebook and read the tragic story of a little boy with hemophilia in the Philippines who recently died because he couldn't get the factor he needed so badly.  It was too much!  "That's our boys, honey! If we ever couldn't get factor, this could be them!"  I cried to Matt.  I really cried.  It's been a while since I cried that hard.  We talked for a while.  I asked Matt, "Is it ever hard for you?"

He replied, "No, it isn't.  I just look at it as something God has put in our lives for a reason, and we just need to make the best of it.  That's why I am going to nursing school to be a nurse and help."  Matt wants to be a nurse manager in pediatric hematology.  He wants to help other families the way our nurse helped us.
 
"Why did God have to mess up my X chromosome?!"  I sobbed.

Matt said, "I don't know, but honey, look at what you are able to do because of this (BWB) and all the people you've been able to meet."

I said, "But, honey, I would give it up in a heartbeat in exchange for my children's health!" 

After more crying, talking, and hugging, I started seeing something I didn't see so well before.  God gave this to us as a gift.  He put us right in the middle of a special group of amazing people.  It is our blessing to know them and love them. 

No, hemophilia does not define Eli or Silas or our family for that matter, but it is a part of us.  It's a very special part because it is not just a bleeding disorder, it is a new family into which we were welcomed with open arms.  God bless you, dear hemophilia family.  You are a gift from God to us, and I pray that we can be as much a blessing in your lives as you are in ours. 

Our Fifth Birth - Silas

17 weeks

31 weeks

After a very busy pregnancy with my husband's career change, a major move, many blog and facebook posts, and much prayer, the time finally came for Silas to come out and join us. . .

On Wednesday, September 21st, I started to feel like "I really want to have this baby soon!!!"  My sporadic contractions had been picking up in frequency and intensity that week.  The day before (20th), I had really begun nesting, and on the 21st I was insanely energetic.  There was crazy hyper nesting going on, and the house was looking great!!  I hit 39 weeks on the 22nd.  That weekend, I started feeling like "This baby is never going to come!  I will be pregnant forever!!"  That is significant because I always feel that way a couple of days before the baby is born. 

Grandma "Kat" helping with bedtime

Isaiah beginning to record
my contractions

Sunday night, the 25th, my contractions were getting stronger, thanks to some alone time with my husband and nursing our toddler.  In the morning, I had some bloody show and more mucus plug, but my contractions were weaker.  I was really discouraged!  I told my husband, "I feel like a car in the winter time that you keep trying to start again and again, but the engine just won't turn over!"  He sweetly and firmly replied, "No, that is not a good comparison.  When a car does that, there is something wrong with the car.  There is nothing wrong with you.  Your body is doing exactly what it's supposed to do.  Don't be discouraged.  You are making progress, and getting ready to have this baby."  Well, that put me right at ease.  I love him.

Birth pool filling up next to Isaiah's bed

Matt went off to work that day ready to come home in an instant if needed.  I kept him updated throughout the day.  When we were on the porch waving to him, our oldest, Isaiah (7yrs) said, "Don't worry, Mom.  If Silas comes, I'm here."  My contractions started getting stronger that afternoon, but were 20 minutes apart.  As the day went on, they got more frequent.  Isaiah started tracking them on a paper.  That evening, I called my mother in law, and asked if she could help me with bedtime.  She came over and was a big help.  My contractions were between 5 and 8 minutes apart.  I was putting sheets on a couple of the kids beds while my mother in law fed them and got them ready for bed including reading books to them.  I would've gone crazy if she hadn't helped me through that evening!

Laboring in my bed with my phone
(I was keeping my fb crowd updated)

Matt came home as quickly as he could after he got off at 10:00, and our midwife arrived soon after that. Or maybe she got there first. . . I can't remember.  She brought her 2 children with her, and right away, her kids and ours were piled on Isaiah's bed playing with Isaiah's game boy!  She got her kids to bed quickly, and Matt got ours back to bed.  Then, I tried to get some sleep. 

Malakai came in to check on me

My contractions were getting more intense.  We decided to at least fill the pool halfway, but I was getting such strong contractions, I ended up telling him to just fill it up all the way because I wanted to get in!  However, when I got in the pool, my contractions slowed down a lot.  We called our doula at 11:30.  She got there when I was lying in Isaiah's bed next to the pool.  I was so tired!
Back in my own bed, I tried to sleep, but lying on my side brought on great contractions.  I just focused on relaxing, knowing that those more difficult ones were the ones that were helping Silas get to my arms faster.  When they were extra hard and I just wanted to survive through it, I would totally surrender my body to the contraction and remove my mind and emotions from the pain.  I would think about my baby, and how the contraction was opening my body for him.  It really helped.

Back in the pool

Whenever I got back into the pool, my contractions slowed down.  This was a very weird labor for me.  My midwife and Matt made sure I was eating and drinking well throughout my labor which helped my energy and endurance level a lot.  My midwife gave me some Valerian herb in tincture form to help me rest.  I did rest, and continued to contract all night.  Our doula had to leave to get back to her baby at home around 8:00am.  She said to call her if things picked up.

Silas, right when I brought
him out of the water

One of the times that I got back in the pool during the early morning hours, Eli walked in the room.  His first words were, "See, told ya I would wake up myself!"  I had told him that someone would wake him up if Silas was coming during the night.  He was very excited that the pool was filled.  Then he said, "So. . . is your belly gonna crack open like an egg?"  I proceeded to give him a basic understanding of where babies come from and the special way that God made mommies to birth them.  He was satisfied enough.  We chatted for a while.  It was so nice to have one of our children in the room.  It made me feel so comfortable, and the atmosphere felt more normal.

Instant Joy after lots of hard work!

While Indian style on the bed, my contractions came every 5 minutes and their severity was very good.  Then, during one contraction around 11:30, I felt Silas move down.  It was pretty cool!  When my legs got tired of that position, I tried the birthing ball.  While I was lunging on the birthing ball, Matt said, "I feel like it's Christmas, and we're trying to squeeze Santa down the chimney." It was really funny, and we both laughed!

After the birth ball, I got down on the floor.  There was a short time (just a few minutes) that I was alone.  I tried to sing "Edelweiss" to Silas, rocking and rubbing my belly.  I couldn't.  I was so overcome with emotion on the lines, "bloom and grow" and "you look happy to meet me", I could not sing!

I was eating and drinking during this whole time. Silas's heart rate was very good.  My midwife suggested that I start walking around.  That was very effective.  My contractions' intensity increased noticeably.  They were more difficult to get through, but I stayed on top of them.  My midwife told me to get in the pool, walk around in the water, then squat down for contractions to ease the pain.  That plan worked very well, and that time ended up being the sweetest and most personal part of my labor.

Our younger 4. Isaiah was busy playing with the midwife's son!
He did peek in from the hallway though.

Matt came in the room with me.  We closed the door for privacy.  Sunlight was pouring in the windows, and our birth play list was playing softly in the background.  I felt so silly walking around in the pool with Matt sitting there watching.  So, I asked him if he'd like to walk with me.  He held my hand, and walked with me around the pool (he was outside).  When I felt a contraction coming, I would squat down, and relax.  Then, I got back up to walk.  Well, our walk became a dance, like a tango or something silly like that.  We laughed so much!  Matt said, "OK, we are not supposed to be having this much fun during a birth!!"  It was such a sweet, funny, and romantic time between the two of us.  

When my contractions were well established, still 5 minutes apart, I stayed down in the water.  Apparently, some were 3 minutes apart because that is what is written in my Labor Record for 1:15pm.

At 1:28, I checked myself, and felt my sweet baby's head!  He was 2 knuckles in, and I felt the molding of his head.

Beautiful pic of Silas and Daddy
(Stacey Rainer Photography)

Gradually, my contractions changed to pushing contractions.  It was NOT easy like Malakai's birth because he had never tucked his chin! So, I did push with my body.

I had asked my midwife to call the children in when I started pushing.  When the children were coming, I pushed his head out.  His bag of waters were still intact until his head came out!  Remember, in Malakai's birth, when I softly stated, "Yes, the head is out." Not so this time!  I exclaimed with exhaustion "HIS HEAD'S OUT!"  After that, there was a brief rest period before the next contraction.  I was caressing his little head.  My midwife came around behind me.  She said his color was good, and said she would check for a cord.  "There is a cord." I told her.  It was loosely around his neck once.  Then, at 1:50pm while Chris Tomlin's "Indescribable" was playing on Matt's iTouch, Silas slowly came out with the next contraction.  I was carefully getting him unwrapped from the cord.  Matt told me to bring him up.  So, I started moving faster.  My midwife said, "Wait, let's get it off his shoulder."  I did with her help, and brought him up.  My midwife stripped Malakai's clothes off and put him right in the pool.  He loved Silas immensely right from the start.  It was so sweet!  Eli told her, "I do not want to get in the pool!"

After our happy relaxed time rejoicing over our newborn and phone calls to our moms and our doula (My labor was so weird that we never knew when to call her until it was too late!), it was time to think about that cord blood.  I carefully got out of the pool, and sat on the birth stool.  Almost immediately, Silas's placenta was born into the bowl below me.  Then, our midwife drew the cord blood.  Silas was still attached to his placenta.  So, she wrapped it in a chuck pad, and put it in a plastic bag.  We chose to do physiological cord clamping for Silas.  We researched the risks and benefits, and decided to let him receive all of his own precious blood and stem cells.  It would take a whole separate post to express all the protection and benefits that newborns receive when they are allowed to get all their own blood and stem cells!  Our midwife was able to get plenty of blood without cutting the cord.  His cord was not clamped or cut for 4 hours.  We did learn that the physiological cord clamping doesn't work the same with a hemophiliac.  First, his cord was cut very long, and didn't need any clamping at all.  However, later that night, when we cut it shorter with no clamps, it did seep a bit, and we had to clamp it.  Next time, we will wait a full 24 hours before cutting the cord short.  With help, I walked to my bed to lie down with Silas.  Matt was soon out the door to run the cord blood to the hospital.

When Matt got back, it was time for our herb bath.  That's always a nice time, and we got lots of beautiful pictures of Silas in the bath.  I think I might make a whole post full of herb bath pics!

A few hours later, we got the call from our hematologist about the blood test results, and learned that Silas does have hemophilia.  I will write more about that later since this post is already so long!

Silas taught me that just because a baby is small, it doesn't mean that the birth is easy.  Silas was only 6# at birth, and his birth was one of my most intense (and I've had a 10#14oz baby!).  I really had to work for him!!  No, size is not the issue, it's all about position.  Silas did not tuck his chin probably because his cord was right there and it was uncomfortable for him.  He came out in an anterior brow position.  However, it was another amazing and beautiful birth, and it was so nice to be back home and share the experience with our children.  Welcome, Silas Martin Reeves!!

Little Hemos are Little Stinkers Too!!

I am dying to get Silas's birth story written and video edited, but until then, here's a funny little story that happened this week:

A couple days ago, the kids were playing in the backyard.  Eli tripped and fell.  He got a little scrape that didn't even bleed, and I cleaned it up and bandaged it.  Then, I put him down to go back outside.  He wouldn't stand on that leg.  He was having a knee bleed.  So, I set up and gave him some factor, and his older brother and sister (Isaiah and Ana) made him a little bed on the couch and got a movie playing for him.  That night, I made a little bed for him on my floor so he would stay off of his knee during the night.

Eli and his new baby brother, Silas

Yesterday, I was still keeping him off of it.  As all of you with hemophilia in the family know, this gets pretty boring very quickly for a little guy!  Last night, I put him up on my bed while I was taking care of the other kids.  I said, "You can stay here, and when it's time for bed, I'll tuck you in (in the little bed on my floor)."  "Ok!" he said with a smile. 

After a short while, I went back to check on him.  He and his little brother, Malakai were jumping and dancing all over the bed!  Eli was watching himself in the mirror and singing, "You have to stay off your knee.  Off your knee.  Off your knee!"  As soon as he saw me, he dropped to the bed.  I could not say a word.  I just looked at him (who was looking at me with a very guilty face), and walked back to the living room.  I told my mom what happened and we both just cracked up! 

This morning at breakfast, I told the kids what he did, and everybody laughed.  Little stinker!!!