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

Interview with Liz Purvis

Two weeks ago, Liz Purvis shared her heartbreaking story about the birth of her son, Ben (Tater).  She allowed me to ask her questions about her birth.  My hope is that this mom's message will touch your hearts and make you think.  If you are touched by her story in any way, please leave a comment for her below.  I know it would mean a lot to her to read that their family's suffering is not in vain, and she is helping others by sharing her story.

BWB:  Why is it so important to you to share your story and is it emotionally difficult to do so?
Liz:  It is important because I had no idea that I was pregnant with a son who had hemophilia. I feel like everything went wrong. I want to share my story with other moms who are in the same situation.  Hopefully, they will take away something from this and be prepared. It is very emotional. For 6 days I tried to bring him into the world safely, and it didn't happen.  It's hard to see all the beautiful and safe births, and ours went so wrong.

BWB:  Do you know why your water broke at 34 weeks?

Liz:  To this day, I have no idea. I remember telling some friends and the doctor that my belly had dropped.  I remember feeling the urge to clean the night before. My husband had literally just walked in the door from being out in the field for a week. I was starting a load of clothes, and it just happened.

3 months pregnant

BWB:  How was your nutrition and hydration during your pregnancy?

Liz:  Great. I only gained 20 pounds (though, I only made it 34 weeks! lol) I ate well, drank plenty, and took my vitamins.


BWB:  Do you think the progress of your labor was hindered in any way due to the care you recieved at the hospital?

Liz:  Yes.  I was on my back the entire time.  I wasn't allowed to eat.  I had fluids going in at 200 cc an hour.  I was forced to be in the bed all the time and use the bed pan.  I would get up and use the restroom when the nurses left because it seemed like I had to go every 15 minutes.   I wasn't allowed to walk.  I was allowed to be up for one hour a day when I would play cards with my husband.  I was very uncomfortable with different people in my room all the time.  I was very uncomfortable.  We had no clue what was going on.  We were away from home.  I was always taught (in school) that the baby needs to come out within 24-72 hours after water breaking.

BWB:  "failure to descend" - By any chance, were you on your back?

Liz:  Yes I was. I also had cervical cancer and had lots of biopsies done on my cervix and cancer removed.  So, I never thought I'd be able to delivery naturally with all the scar tissue.  No one listened.  I saw a different male OBGYN every visit, and I was transferred to a different hospital.

BWB:  What drugs were involved in your delivery?

Liz:  They hung pitocin after the contractions starting coming on their own. I was very upset with that. I didn't want that stuff at all. I said, "I waited for 6 days!  Now you can wait!", but they hung it anyway.  It was the worst thing ever.  I had an epidural that the doctor turned off while I was trying to push.  He said if I could feel the contractions maybe I could push him out.  I was also getting iv pain meds: Stadol, I think.

BWB:  Do you believe the Pitocin made your contractions too severe for Tater?

Liz:  I know it made it very hard on me.  So, I imagine they were too severe for him.


BWB:  Did the Pitocin make the contractions so severe that it made the epidural essential for you to endure the pain?

Liz:  Yes ma'am. The last thing I wanted was someone messing with my spinal fluid and putting a needle in my spine. 

BWB:  What is your opinion of them using an internal monitor on Tater?

Liz:  I was ok with the first one.  Tater kept moving around.  So, the external monitors couldn't read. 
*Liz went on to say that she was not ok with the second set that was put on Tater.  It made her very uncomfortable as a mom, and she questioned the medical staff about it being too much for him.


BWB:  How was your strength level when you were induced?

Liz:  I was tired, and recieving so much iv fluid that I was up and down every 15 minutes.  I couldn't sleep because of all the monitors on my belly.  I hadn't had a meal.  I was finally upped to a liquid diet after 2 days.


BWB:  Had you been able to keep up your strength by eating and drinking for the 6 days you were in the hospital?

Liz:  No ma'am.

BWB: How long were you contracting before the c-section?
Liz: about 9 hours


BWB:  How do you feel things were handled after the cesarean?

Liz:  Horribly.  My stomach was growing, not going down.  I had horrible back spasms. My legs were swollen my ankles and feet were so swollen that I couldn't even wear a house shoe.  The pain was horrible.  They wouldn't tell me what was going on.  I had to climb from the surgery bed to my own bed.  They wouldn't even let Bubba help me transfer beds.  I wasn't allowed food or water.  I went to the ER after being discharged for 2 days.  They rushed me back.  I had high blood pressure.  I was so swollen.  They did ultrasounds of my thigh and leg because they thought it was a blood clot.  They stuck me over 6 times to try and get blood.  The amount of blood I had left was too low and they wanted to transfuse me.  When I was admitted back, they gave me iron pills and 3 doses of blood pressure meds, and discharged me again.


BWB: What caused his intercranial hemorrhage?
Liz: We really don't know if it was from all the trauma or if it was spontaneous.

BWB:  What have you learned from this experience?
Liz:  To not have another baby. I don't know how all these people were involved and let things go so wrong.

BWB:  What is your advice to pregnant women who are reading your story?

Liz:  Speak up! If you're not comfortable, talk with the staff and get comfortable.  Don't do anything you don't want to do.  Watch for signs of a brain bleed.

BWB:  What would you like others in the hemophilia community to learn from your story?

Liz:  My story is not normal.  Most people don't go through all of this.  Don't be scared.  Work with your hematologist.  Let your family and friends support you.  Make sure everything is ready just in case.

BWB:  As a nurse, how do you think the medical staff should've communicated with you during and after Tater's birth?

Liz:  They could have done much better.  The doctor was always too busy to talk with me.  The nurses just did what they were told.  The NICU staff was amazing.

_________________________________________________________________________________

Liz's story has made a huge impression on my life.  I am grateful to have had the honor of meeting her online and speaking to her over the phone.  I believe Liz's birth was stolen from her.  She had plans of a gentle birth.  She took care of herself during her pregnancy, and she and her husband were looking forward to having a large family.  For whatever reason, her water broke at 34 weeks.  Her birth was horribly mismanaged, and resulted in lifelong injuries for her sweet little boy.  Liz cares for Tater everyday with her whole heart.  She and Bubba want the very best for him.  Please comment below for Liz.  She will receive anything helpful and encouraging you can share. 

Love and Guilt

Eli, Malakai, Isaiah, and Ana

I know that it is not my fault that 2 (maybe 3) of my children are affected by hemophilia.  I know that genes are more than just  a mutation, and that only those particular X chromosomes would've made each one of our children.   If they had recieved any other X, they would be a completely different child.  Looking at it like that, it makes it easy to be thankful for everything that comes with or does not come with my children's genes because I want THEM.  If hemophilia comes with the package, so be it.  However, last night when Ana showed us a mysterious bruise that she discovered on her hip, I felt an unusual weight of guilt. 


It doesn't make much sense.  I know all the facts.  Maybe it's just because I'm extra emotional with Silas's birth coming so soon.  I don't know, but when I looked at that deep purple bruise on my little girl, I felt like I did that to her.  She is a little ballerina.  She loves all things to do with homemaking and babies.  She is the spunkiest little girl I've ever met.  I love her so much. 

I don't need to feel guilty.  If carrying hemophilia is part of what God has for her life just like it is part of what He had for mine, I praise God for it.  That sweet little princess is going to take the world by storm!  She will have opportunities to reach people that she wouldn't have otherwise. 

I am concerned for some things like:  Will she have any bleeding issues when she hits puberty?  Will she have any issues with childbirth?  Will her factor level ever go lower?  I don't know the answers to these things, but God does.  He loves her even more than I do, and I trust Him.

Baby Carrier Issues with a Little Bruiser

If you follow this blog at all, you know that I am just a BIT excited about the new addition to our family expected very soon (38 weeks tomorrow!!).  There are so many things to thing about.  We are baby wearers.  Whenever we are out with baby, either I or my husband wears the baby in a sling/wrap.  We don't even invest in infant carseats.  We just start with the convertable carseat right away.  Carseats are for the CAR anyway, right?  ;o) 

Baby Eli in Maya Wrap

Eli all padded up in stroller

Well, when Eli (who has severe hemo A) was a baby, this wasn't so easy.  He would get bruises behind his knees where the edge of the sling pushed against his legs.  We had a wrap as well.  That worked better, but was so inconvenient to use.  Everytime we took him out, we had to totally rewrap it before we put him back in.  It was made of a heavy t-shirt material.  As a result, little Eli spent most outings in a stroller with sheepskin behind his knees.  If he wanted us, we just held him. 

I asked my other hemo mom friends about this on my facebook page for Bubble Wrapped Birth.  I got the same recommendation from a few different moms: The Sleepy Wrap!!   It is the same as the Moby Wrap we already have, but the material is lighter, softer, and a lot stronger.  Apparently, it doesn't stretch out, making rewrapping unecessary when you get baby out then back in.  I ordered one, and can't wait to try it out with Silas!  I don't know if he has hemophilia or not, of course, but either way I will post a review here in the next couple months. 

I can't wait! 

I can't wait!! 

I can't wait!!!