Hemophilia Awareness Month Daily Facts (posted thoughout March on my facebook profile page)

1. Hemophilia is an X chromosome genetic mutation. That's why girls are carriers. We have another X to make up for the affected X. (There are exceptions, but it's complicated) Boys don't. So, a mom that carries the gene (ie: me!) has a 50% chance of passing on the gene with every pregnancy. Dads who have hemophilia will never pass it on to their sons since their sons get their Y chromosome. However, all their daughters will be carriers since they get his X chromosome, and the affected X is the only one he has to offer.

2. It is very nice for a hemophilia mom to spend the evening at the salon (via birthday money) after an exhausting day at clinic with all the children. . . ;o)

3. The most common hemophilia gene mutation is "22 inversion". All the right information is there, but inverted in a section of the X chromosome. Therefore, the information that is there cannot communicate to get the job done (in this case: make factor 8!). This is the gene mutation in our family.


4.  When you have a child with hemophilia, everyone is affected. We are not just a family of 7 with 2 hemophiliac boys. Our family has hemophilia. We may not all have it in our bodies, but we have it in our family, and we deal with it everyday. We are all in this together! :o)

5. When my Dad was a young boy in the 1940s and 1950s, most hemophiliacs didn't make it through their teen years because the only treatment was whole blood transfusions. Their was no preventative treatments. The few that did survive, suffered major bodily harm from their bleeds, and their bodies are mangled, but they're probably some of the strongest men you'll ever meet. The most impacting thing for me is to hear these men speak of the different life their grandsons have now that they have factor. (Based on the film,"An Unexpected Life. Living with Hemophilia." Presented by the Alta Bates Summit HTC)

6. To make a clot, our bodies make a platelet plug, then a fibrin clot to hold it all together. Hemophiliacs make the platelet plug, but not the fibrin clot. With little cuts and scrapes, the platelet plug is sufficient, but sometimes not when they are in the mouth. Saliva breaks down the platelet plug, and makes a lot of trouble! So, there is a liquid medicine called "Amicar" that is available to help. Amicar prevents the saliva from breaking up the platelet plug. Sometimes, this is so effective, factor is not needed! Yay for Amicar!!! :o)

7. What are the leading causes of bleeds for a little hemophiliac during childbirth? forceps, vacuum, internal monitor

8. How does factor work during a joint bleed? Blood starts entering the joint capsule. It fills up with blood. The factor helps the bleeding stop. Digestive enzymes eat away the blood, but also eat away at the cartilage and bone. Once a joint has had a bleed, it will always be more susceptible to getting more bleeds.

9. One of the greatest threats to hemophiliacs today is an inhibitor. An inhibitor is when their body starts rejecting the factor it needs so badly. When this happens to a factor 8 deficient person (hemophilia A), they are treated with factor 7 until the inhibitor is resolved. Clotting factors work like dominoes, and factor 7 is a good plan b for factor 8 deficient hemophiliacs because it allows clotting to skip a step. It is not easy. You have to infuse the factor 7 a lot more often than factor 8. Sometimes it takes a very long time for an inhibitor to go away.

10. Did you know that the worst position to give birth is lying on your back*? This position reduces the size of the pelvis by up to 30%!! This is especially dangerous for babies with hemophilia since that would threaten the use of vacuum or forceps if the baby appeared to be "stuck", and those interventions are the leading cause of headbleeds for newborns with hemophilia. Get off your backs, ladies! ♥
*There are exceptions to this.  Sometimes, a woman's pelvis is shaped in a way that consistently causes back labor, and lying on her back is the only way to get the baby to turn.

11. Carriers of hemophilia have varying factor levels. Sometimes their factor level is well within the normal range. Sometimes it is the low end of normal. Sometimes it is so low they qualify as mild hemophiliacs. Rarely, it is low enough to qualify them as severe hemophiliacs. I thought this wasn't possible until I met a girl with severe hemophilia. The explanation for this is in how the chromosomes split and multiply soon after conception. Another interesting fact is that carriers typically have elevated factor levels while nursing or pregnant. Since I have been nursing and/or pregnant for the last 8 years, I don't know what my regular factor level is. When I was tested while nursing Eli and again while pregnant with Malakai, it was 90% (within normal range). When I was in my 3rd trimester with Silas, it was 190% (high end of normal).  Ana was tested and her level was 44%.  Normal factor level is anywhere within 40%-200%.  I can tell you that I used to bruise a lot more than I do now!

12. When a person is having a bleed it feels like burning, swelling, sharp pain, or bubbles/tingling. Other descriptions include: "when a joint swells up with blood it is like having cement in there causing you not to be able to move"

13. It is super fun to launch caps off of empty syringes after prophy. . . ;o)

14. When you have a child with hemophilia, you no longer take it for granted when you actually can just "kiss it and make it all better" with a non hemo child or a minor injury with a hemo child. ♥

15.  "Hemophilia is a genetic bleeding disorder that prevents the blood from clotting normally. The main symptom is uncontrolled, often spontaneous bleeding. Internal bleeding into the joints can result in pain, swelling, and, if left untreated, can cause permanent damage.'  -Jennifer Grizzard Smith

Why I Love Water Birth: part 2

Before Ana could start walking, we were looking at two happy pink lines again!  We had been in IL for Isaiah's birth, MI for Ana's birth, and now we were in CA looking for a midwife.  We couldn't possibly have more than one baby in any state, could we?  We found the best midwife for us, and began planning another home water birth. 

I knew I was in labor at about 1:30am.  I labored in bed for a while on my side.  I got on my knees and leaned over a chair, all the time moaning through contractions.  By now, Isaiah was about 1 month from being 4, and Ana was about 2 months from being 2.  I had practiced these birth noises with them so they wouldn't be scared when the time came.  It worked.  They weren't scared at all.  I had nice breaks between contractions too, which really helped.  Isaiah wanted to help me so badly, but kept getting in people's way.  So, I asked him if he could hug me through my contractions.  He was my doula for this birth!   

The pushing stage for this birth was way more intense than it had been with Ana's birth.  For the first time during active labor, I called out to God, thanked Him for this baby, and that "I can do all things through Christ who strengthens me" (Phil 4:13).  When it was time to push, I pushed like I had never pushed before.  His head came out.  There was a small break before the next contraction (seemed like forever!).  Then, I was pushing again.  He was so huge that I thought he was completely out when he was only out to his waist!  Elijah was 10lbs 14oz!  I couldn't believe it!  His birth was so gentle, and he was a beautiful chubby newborn.  Four months later, we found out he had severe hemophilia.  I am so thankful that we had such a gentle birth for him.  I believe the water birth was a major reason why he was so big and did not have any bleeds or bruising, and I did not tear at all. 

By the time we were expecting our fourth little one, we knew that I was a carrier of hemophilia.  I have written all these stories before, and have recently posted the story of "Our Journey".  Please read it to get the full story of how we began birth planning with the knowledge of hemophilia being a possiblility.  We moved from CA to NY when I was 6 months pregnant.  I found a midwife, and we started planning a homebirth with the goal of drawing cord blood and transporting the specimen from home to a lab.  That plan did not work out.  We had to consider a hospital birth.  I met with the perinataltologist.  I explained to her that water birth was the safest, most gentle way I knew how to birth, our large hemophiliac baby had been born in water at home with no problems at all, and I insisted that our fourth baby's birth was just as gentle.  Malakai was the first water birth ever for this hospital.  His birth was so gentle that his water never broke until his shoulders were born. 

I know I've shared all these stories in length before, but I wanted to give a fresh review to illustrate where I'm coming from with water birth.  Based on my experience, I love water birth because:

• It calms my pain and discomfort.
• It relaxes my body.
• It softens my perineum, and helps me not tear.
• Because I am so relaxed, I can get in the best possible position for my pelvis to be at maximum size.
• I can easily move to different positions at will.
• Because I am so relaxed, my whole body opens to birth my baby as gently as possible.
• It gives me my own space, and I feel safe. 
• Being in the water helps me feel covered, and I don't feel so exposed and vulnerable.

I am now 13 1/2 weeks pregnant with our fifth baby.  Malakai is 16 months old, Eli is 3, Ana is 5, and Isaiah is 7.  In about 5 weeks we will find out if this baby is a boy or a girl.  There is no question that we will be planning another water birth.  We hope to have a home birth, but if we must consider a hospital, we will plan a water birth there.  Hemophilia babies need a gentle birth even more than other babies do, and I am so thankful that we already practiced such a gentle way of birthing even before we knew hemophilia was a part of our lives.

Why I Love Water Birth: part 1

I first heard of water birth when I was a teenager.  My first thought was "That's crazy!  Won't the baby drown?!".  I really didn't think of it much more until I was married and we were expecting our firstborn.  The first midwife we interviewed told us that she almost exclusively did water births.  She told us that water birth was a lot gentler on both the baby and the mom.  "For the baby, the difference is like waking up at your own leisure on a Saturday morning vs. being woken up with an alarm clock on Monday morning."  The baby is born from warm fluid into warm fluid, and is gently lifted up to the air, and the baby is not stimulated to breathe until he/she comes in contact with the air.  The warm water helps mom relax, and her body opens up more.  The warm water is also great pain relief, and allows the mom to move to different positions effortlessly.  We were impressed with all the benefits she shared with us, and decided to go with a water birth.

Our first birth was at home.  My husband (Matt) and I were in the birth pool together.  Isaiah was crowning for an hour.  Our midwife kept close watch on his heart rate and skin color which was fine, and Matt played with his hair.  After 4 hours of pushing, Isaiah was born into his daddy's hands.  Matt passed him to me, and I lifted him up out of the water.  He was big!  9lbs 8oz, broad shoulders and big chest.  No wonder he took so long to come out!

Baby #2 was also born at home.  Things went a lot faster this time.  I knew I was in labor around 11:00pm and she was born at 1:30am.  My mom called our midwife, Matt got the pool ready, and I was laboring on the toilet, tailorsitting on the floor, hanging on people. . .  I couldn't wait for the pool to be ready!!  Finally, it was warm enough and full enough for me to get in.  Ahhhhhhhhh. . . .   instant relief!  My sister acted as my doula, and my sis-in-law was there to take care of Isaiah (then 2 years old).  Matt and I were in the pool.  My midwife would not tell me when to push.  She said I would tell them when it was time to push.  Suddenly, one contraction caused me to bear down!  I told my sis-in-law to wake up Isaiah.  He wanted us to wake him up for the birth.  She did.  She told him that the baby was coming.  He got up, unzipped his pajamas, and started taking off his diaper, and climbing in the pool!  My midwife said it was fine.  So, we let him in.  Within 6 minutes of the first pushing contraction, Ana was born into my own hands.  Matt was holding Isaiah, my sister was right outside the pool next to me.  With one hand on my sister's shoulder, and one hand on Ana's head, I told everyone when she was crowning, when her head was out, and brought her up out of the water when she was born.  She was pink and looked so little at 7lbs 4oz (2lbs less than her brother was!)

Our Journey

I was an avid natural birth advocate way before I knew what the word hemophilia meant.  After our third child was diagnosed with severe hemophilia and I found out I was a carrier, I began to ask people a lot of questions.  I asked other hemo moms, doctors, nurses, midwives, anyone!!  I wanted to know if my beautiful home birth days were over, and I wanted to know the real risks posed to baby hemophiliacs at birth.  It was then that my Bubble Wrapped Birth journey began. 

I have heard many stories, read the studies, looked at medical reports, and read our recommendations published in Hemaware magazine.  I have never once read a birth story where a baby had a gentle birth with no drugs or interventions of any kind, and ended up with a bleed.  In fact, all the birth bleed stories I've read have been births with drugs or interventions such as vacuum or forceps. 

I am committed to planning safe deliveries for each of my children.  I've learned a lot from other hemo families, midwives, and my own experience.  My husband and I have learned what works best for us and allows us to have the most gentle birth as possible.  We use water birth.  It allows me to fully relax, move effortlessly, and be in the greatest positions that open my pelvis to maximum size.  There are some situations that we have decided would mean the safest delivery would be a c-section.  These include placenta previa and a breech boy (maybe girl, not decided yet). 

The bottom line is this.  We believe that a gentle vaginal birth is most beneficial to our children unless one of the above complications become evident.  In this Bubble Wrapped Birth journey, I may come across other reasons for scheduling a c-section.  My concern is what is best for my babies.  Period.

As far as home birth, I birth at home when I believe the hospital situation threatens dangerous interventions and does not support our birth plan.  One hard thing we've come across is how to get the cord blood tested from a home birth.  It was for this reason our fourth child was born in the hospital. 

His birth was perfect, and just as gentle as a home birth.  After he was born, he had a meconium issue, and needed suction and breaths.  That was a first, and not fun.  He was ok, and I thought the staff did a fine job taking care of the situation.  If we had been home, we would've been set up right at the side of the pool.  He would've been able to be suctioned, and get breaths on a flat warm surface without cutting the cord or leaving me.  The baby care at the hospital was also something new for us.  Home is much gentler on the baby, and we are in full control of what happens with our baby.  In a hospital, we dealt with required procedures, sometimes dangerous for a baby with a bleeding disorder (ie:  rough personnel and the heel prick).  We were there for one reason, and that was the cord blood testing.  He did not have hemophilia.

Now, we are 12 weeks along with baby #5.  We will find out around Easter time if baby is a boy or a girl.  Plan A is a home birth with cord blood testing and a trained professional with emergency equipment.  Plan B is a gentle water birth in a hospital with a midwife where our birth plan is respected and we can get the cord blood tested for factor 8 level.  We will continue to grow and learn, and we will never compromise our baby's safety.  If we are having a girl, we will probably plan a home birth. 

Bubble Wrapped Birth is a passion of my heart.  Thank you for joining me on this amazing and precious journey!

So much to be thankful for. . .

Today, I heard a story of a symptomatic carrier who nearly lost her life during the birth of her first child.  I also heard from a woman who's daughter has severe hemophilia and was badly bruised at birth.  My heart broke for these families, and I ached as I imagined what they went through at that time.

If you've read my birth stories on here, you know I've never gone through anything like that.  So many people deal with so much more than we do.  Something like that could happen to us at any time.  We need to thank God for his loving mercy in sparing us from such hardships as these.  We should never take anything for granted.  Every moment is a gift.

I also heard from some other moms today.  What stuck out to me about them was their ability to look back and have total confidence that they did what was best for their baby.  That has never meant more to me than it does now. 

In the past, I've met moms who were not given the best care, and their baby suffered much for it.  It is hard for those moms not to personalize the blame, even when they too were also a victim. 

These may be somewhat random thoughts.  I just want to pay tribute to this amazing community we've been a part of for almost 3 years now.  I learn so much from all of you.  I really believe that we are much stronger when we have each other.  Thank you so much for sharing your stories and insights.  I thank God for you!

Back Labor

So, today we were talking about back labor on facebook.  Well, it didn't start out about back labor.  I started by making this statement: 

"The worst position to give birth is lying on your back. This position reduces the size of the pelvis by up to 30%! This is especially dangerous for babies with hemophilia since that would threaten the use of vacuum or forceps if the baby appeared to be stuck, and those interventions are the leading cause of headbleeds for newborns with hemophilia. Get off your backs, ladies!"

One mom commented that she strongly disagreed, and said that she had suffered terrible back labor for all 4 of her births, and the only position that worked for her was on her back, pulling her feet up (in a frog position).  She said that pulling her legs up also allowed her tailbone to move.  I asked my BWB fb page readers if any of them had a similar experience with back labor.  One mom agreed and said that this position was also the only one that worked for her.  However, it seems that it only worked for the pain because her baby got stuck, and they used a vacuum on her. 

So, my question is:  What is the safest way to deal with back labor?  I've never had back labor, but I want to know what I could do if I ever were dealing with the pain of back labor.  Another mom commented and said that her husband pushed his fists into her lower back as hard as he could for counter pressure while she squatted or leaned forward.  To me, that seems like the ideal choice.   Although, it may not be feasable for all back laborers, it is currently my plan A if I were ever in that situation.

Get Off Your Backs, Ladies!!!

http://www.youtube.com/watch?v=OD88c9dbiZg

The worst position to give birth is lying on your back. This position reduces the size of the pelvis by up to 30%! This is especially dangerous for babies with hemophilia since that would threaten the use of vacuum or forceps if the baby appeared to be stuck, and those interventions are the leading cause of headbleeds for newborns with hemophilia. Get off your backs, ladies!  Stand up, squat, get on all fours, even lay on your side. Just get off that tailbone so it can move!  This 5 minute video shows the dangers of your baby getting "stuck".  Please watch!

Our Newest Addition. . .

If you are on the facebook page, you know already that we are expecting our 5th child!  My EDD is September 29th.  We are so exited!!  We will find out at Easter time if we are having a boy or girl, and make the safest plan from there.  I'll be 12 weeks on Thursday!! 

I'm Failing as a Blogger! :o/

I am so sorry.  It is hard for me to keep up with this blog, but I will try to do better.  I will post whatever I put on my fb page on here.  Please like the fb page if you'd like to keep up with me!  "Bubble Wrapped Birth:  How to Safely Birth a Hemophiliac"  Meanwhile, I will work on a new post!  :o)