Why Hemophilia Isn't Stopping Us from Having More Children

Ana 2010

Soon after Matt and I got married, we agreed that we would have as many children as God gave us.  We put our faith in God, trust in Jesus and believe the Bible.  God always refers to children as a positive thing: gift, blessing, treasure, reward, inheritance, etc.  We were both uncomfortable with any form of birth control.  It just didn't seem right to us to try to prevent a possible gift, blessing, treasure, reward, or inheritance that God might have for us.

So, we were very delighted to find out we were expecting our first little blessing right before Thanksgiving, just 5 months after our wedding.  Then, we were filled with grief when we buried that sweet baby just a month later.  I have given birth to 5 healthy babies since then, and I can tell you that the pain of miscarriage is the pain of death.  The pain of birth is life, and so much easier.

Malakai, Eli, Isaiah, and Ana 2011

God filled our hearts with joy once again when we found out our little Isaiah was on the way.  We actually heard his heartbeat on our first baby's due date, an unexpected gift from God.  That day would've been very hard otherwise.  Then, after Isaiah was a year old, my womb was full again.  This time with Ana.  Isaiah got in the pool for her birth.  I caught her myself.  So priceless.  Then, our not so little Elijah came (10lbs 14oz!) less than 2 years later.  His birth was the most spiritual I've ever experienced.  It gives me chills to this day.

It was when Eli was 4 months old that we were introduced to the world of hemophilia.  He had a couple hematomas along his waistline.  Blood tests showed that he had less than 1% of factor 8, severe hemophilia A.  A few months after that, I was tested.  I thought he probably had a spontaneous mutation.  I didn't have any family history of hemophilia at all.  Well, I remember getting that call like it was yesterday.  The test came back, and I was a carrier.  My heart sank.  Was this my fault?  Could I have more children in good conscience now? What does this mean for my home births? All these questions and more weighed me down immediately.

Our family of 7 with Matt's Mom and Grandma 2011

We reexamined our original decision:  We trust that God would give us as many children as we should have, and He would give us the specific children we should have.  Had all that changed since hemophilia?  The truth was that if it had changed, then we'd never truly believed it.

So, we stuck to our plan.  A few months later, we were blessed again.  This time, birth planning was a lot different.  We chose to get an ultrasound to find out if the baby was a girl or a boy:  BOY.  Then, we started interviewing midwives.  It definitely puts a twist in the interview when you mention, "Oh, by the way, our baby has a 50% chance of having a severe bleeding disorder. . . "  It wasn't so easy finding a caregiver this time.

Then, my husband lost his job, and we moved clear across the country from California to New York where he got a new job.  Homebirth just wasn't working out this time, but we had a great midwife who had privileges at the Women's and Children's Hospital.  I insisted on a water birth to give my baby the most gentle birth I could.  They'd never had a water birth, but in the end, they approved my request.  We used our pool, and they moved the bed out to make room for it.  Malakai was born, making hospital history!  His cord blood test came back that he did not have hemophilia.

Malakai and Silas 2012

Malakai is now 2 1/2 years old, and his little brother, Silas was born last September.  Silas is 5 1/2 months old now.  We were able to have a totally awesome home birth with him, and coordinated with our new (moved again!) HTC to get the cord blood tested.  Silas does have severe hemophilia A like his older brother, Eli.  He has shown little signs of his hemophilia so far.

Matt and I are very excited to look forward to the future.  Our beautiful family is growing, and although they keep us hopping, we just love our little crowd!  If God wants us to have more children with hemophilia, then it is what is best for our family.  If not, then that's what's best.  We choose to trust Him.

Matt and I have lots of friends and family whom we highly respect that do not share our beliefs on childbearing.  We believe it is a personal choice for each couple to decide on their own.  I know that I may have lost some people when I mentioned God, but I wanted to share our heart with you, and I can't do that without mentioning God.

Anyway, isn't having any child a risk?  People who don't carry a genetic disorder are not guaranteed a perfectly "healthy" child every time.  Elijah and Silas are just as much a blessing to our family as Isaiah, Ana, and Malakai are.  I believe with all my heart that God has a special plan for each one of our children.  I believe that everything in their lives is there for that purpose, to make them exactly who they are meant to be.  No matter what happens, God always loves us and we can always trust Him.

Meet Craig Wright

Craig Wright

Over the past few months, I've been learning a lot about a man named Craig Wright.  Craig was born in August of 1953.  After he was born, he was diagnosed with severe hemophilia when he cut his lip and it wouldn't stop bleeding.  He spent a lot of time in the hospital because of his hemophilia and received gallons of blood from numerous donors.  Life for a hemophiliac was much harder back then.

Craig grew up on the family farm in Prince Albert, Saskatchewan Canada.  At a young age he expressed a desire for farming.  He was told that he should give up that dream because a life of farming would be too dangerous for a hemophiliac.  That did not stop Craig from following his dream.  He grew up to take over the family farm.  He married, and he and his beautiful wife were blessed with 3 precious children.

Craig with his family

However, the Wright's lives were turned upside down one day when 32 year old Craig learned that he had contracted AIDS from bad blood.  His daughter, Ashley recalls:  "I was quite small (maybe 5-7 years old).  I remember seeing one (commercial on HIV/AIDS) and saying, 'Hey Dad, that's you, right?' and immediately after running to my room because I thought I would be in trouble for speaking about it. We all knew, it was NOT something you brought up!"

Ashley remembers telling her best friend about her dad's condition.  "It was the middle of winter and recess time and I asked her to sit with me on a little snow hill and told her."  Her friend didn't understand the reality of the situation, but knew she was sworn to secrecy until Ashley decided to tell others.  It was three years later in 6th grade that Ashley decided to share the situation with her classmates.  She never imagined that her dad would be laid to rest before she would finish 7th grade.

Craig's daughter, Ashley with her son, Parker

As Ashley shared her memories with me, I was deeply impressed with her parents.  Ashley remembers, "They came into the school and did presentations for the class as well as the parents, and I know they had to go through a whole bunch of red tape to even think about discussing such a topic with children.  I remember being scared but yet so proud of my dad for standing in front of the class and explaining to them in children terms what was going on in his body and why.  Needless to say, the children took it better than the parents.  I distinctly remember at the parent info meeting, the parents of the children in my sister's class saying, 'We do not want your daughter playing with our children.'  How do you even respond to that?!  Being a mom now, I have no idea how they handled that without tears!"

Ashley remembers that her dad missed being in his band.  She also remembers the "countless hours and hours he spent on the phone with lawyers and conference calls as well as the many trips to the lawyer's office or the lawyer coming to the house."

Craig was driven by truth and justice.  When he was infected with a deadly virus as a result of others' mistakes, he wanted those responsible to be held accountable.  He dedicated the remainder of his life to fighting for compensation to the families affected by bad blood and holding the responsible parties accountable.  He was deeply involved on "The Krever Commission".  This commission fought for better blood screening, safety of blood products, but mostly for the compensation for the affected families of bad blood.

Craig's grandaughter, Charlie

The Globe & Mail June 1, 1996 states,

"The mandate of the Krever inquiry is twofold:

-Determine how and why more than 1,200 hemophiliacs and transfusion recipients contracted between 1980 and 1985 the virus linked to acquired immune deficiency syndrome and why another 12,000 Canadians contracted hepatitis C from blood and blood products between 1980 and 1990;

-Recommend ways to revamp the blood system so a similar disaster is not repeated."

From studying the documents that Ashley sent to me for this post, I learned how the organizations responsible for the bad blood epidemic were quite upset about the Krever commission.  They thought that the notices of "potential misconduct" should be quashed because they unfairly stigmatized them. 

In 1993, Craig was interviewed by The Leader-Post.  He stated, "The knowledge we are getting now is the governments knew in 1982 and didn't act until 1985.  The time frame is so bizarre, . . .  How do you justify not letting hemophiliacs at the grassroots level know?  How can they justify not publicly putting out a news blurb saying:  'Hemophiliacs, be careful,'?"

In the end, Craig was not completely satisfied with what the courts decided the compensation should be, but he was told that it would be another 7 years before it would make it in front of a judge again.  Since he knew he wouldn't live long enough to see it go to court again, he agreed with what would be given.

It seemed that as soon as Craig knew that his family would be provided for, his body started shutting down.  Then on May 9th, 1996 in a Saskatoon hospital, 42 year old Craig Wright passed away.

Craig's grandson, Parker

I am so inspired by the life and legacy of Craig Wright, and I am so very saddened for his life that was lost.  We can all learn from this man's example.  From the moment he learned of his hemophilia, he decided that it would not keep him down.  Craig lived his life to the fullest.  He was loved and respected by his family, friends, and even people from his local and national government.  When carelessness led to his contracting the AIDS virus, he did not roll up in a ball and sulk or give up.  He let it fan the flames in his heart.  He stood up and fought for his family and many others.  When AIDS ultimately claimed his life, many honored him.

One of the saddest parts of this story is the grandchildren who never got to meet him.  Craig's daughter, Ashley, is brokenhearted that her little ones, will never know their grandfather who was such a hero.  No, Charlie and Parker will never look into their grandfather's eyes as they grow up, ride on the tractor with him on the family farm, listen to their grandpa's singing and guitar playing, or hear his stories from his own lips.  But, I believe they will know their grandpa.  He lives in the hearts and memories of so many people.  I believe Charlie and Parker will be inspired by their very special grandpa's legacy.  Parker's blood inherited the mutated gene carrying hemophilia, but that blood has a legacy of much love, determination, and strength in it from his grandfather, Craig Wright.

Boo-boos

Lately, I've been giving a lot of thought to my parenting.  We have 5 children:  2 boys without hemophilia, 2 boys with hemophilia, and one daughter who is a carrier and bruises often.  Things are going much better with the jealousy issue.  I am learning a lot. 

Now, let's talk about boo-boos.

One of the best ways to show your children they are special is in how you care for them when they are sick or hurt.  In a family where half of the children have a severe bleeding disorder, it would be easy to give our children the impression that it's not so bad if one of the "non-affected" children gets hurt.  If Malakai falls and hits his head, I scoop him up, cuddle him, comfort him, get an ice pack, kiss it and make it "all better".  However, if Eli falls and hits his head I do all the above, plus call the HTC, set up for factor treatment, call Matt at work, treat Eli, and Matt comes home and takes him in to the ER for a CT scan.  I've caught myself and my husband saying things like, "I'm so glad it was (non-affected child) and not (hemophiliac child)!"  Although it's true that it is less dangerous for a child without hemophilia to get an injury,  this shouldn't be the attitude, much less spoken.

Whether our child has a bleeding disorder or not, they need to be shown compassion, love, and loving care when they are hurt.  Pain is pain even without a bleed. 

Some great tools for boo-boo "tlc":
-snuggles
-kisses
-ice pack
-basic first aid
-band-aids (even when not truly necessary.  Besides, if you have prophy supplies, you have plenty of band-aids!!)
-breastfeeding
-special blanket or toy
-singing
-reading a book
-You know your child.  What would be on your list?

I am not saying that we should baby every boo-boo.  Obviously, there are levels of severity.  My point is that each child needs to feel like Mommy and Daddy are sorry that THEY got hurt.  They should never feel like it is a relief that it was "just them" and not their brother. 

I do not see a problem with this in our family yet.  Hopefully, seeing this now will prevent it from ever being a problem in the future, and be a good example to Ana if she has a family of some hemos/some non-hemos someday.

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

To My Husband with Much Love

Thank you, Honey. 

Thank you for being my best friend.  Thank you for your love and support through everything these last 9+ years. 

When we first found out about Faith, and then couldn't keep her, you held me together through all of that.  The worry, the confirmation that our tiny baby was no longer living, the pain, the hospital, the incredible grief. . .  We came through that together with God as our strength.

Thank you for 7 1/2 years of successful breastfeeding.  I couldn't have done it without you.  When Isaiah was born, and had trouble latching on, you never suggested I give up and just give him a bottle.  Those first nights of pumping colostrum and spoon feeding him every 2 hours were so hard, but you were so strong right with me.  You even rocked him between feedings so I could sleep.  A few days later when the roof of his mouth smoothed out and he nursed with no problem at all, it was a victory for all three of us.  We all worked very hard for that.  Also, you've always been very supportive through all the (often difficult) tandem nursing months and years with each of our 4 children. 

You love me through all my hormonal mood swings.  You always look at me like I'm the most beautiful girl in the world no matter if I'm a size 4 or I'm 8 months pregnant and no longer fitting into my maternity clothes because I've gained 50 or 60 lbs!  You are an excellent birth assistant.  If you are with me, I know everything will be ok.  Our births have been so amazing mostly because of you.  I could never have been so relaxed and at ease if you had not set that tone for me. 

You are such a great daddy.  I am so thankful our children have you.  No matter how busy you are, you make time for them.  You are a patient teacher and love them with the love of Jesus.  I love seeing you wear each of our babies in the sling or baby wrap.  You have become just as much of a breastfeeding, baby-wearing, gentle birthing advocate as I am, and sometimes even more than I am!   

I am so proud of you.  When Eli was diagnosed with severe hemophilia, a seed was planted in your heart, and now you are working very hard to earn your nursing degree so that you can help other children and families with bleeding disorders. 

I love how you love being a daddy.  You are even more baby crazy than I am!!  It doesn't even matter to you if we have more children with hemophilia.  On the possibility of having multiple little hemophiliacs, you just say, "Line 'em up!" (for infusions).  You just smile on the idea of caring for each child with their own unique needs. 

 As we anticipate the arrival of our fifth child any day now, I wanted you to have the spot light.  You are the greatest husband and daddy I've ever known.  I love you.  Thank you so much for everything. 

Love,                    

Me                  

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.

Tater (Ben): Liz and Bubba (James) Purvis's Story

I met Liz Purvis on facebook.  I was searching for hemophilia birth stories specifically ones where the baby suffered a bleed.  I just wept when Liz shared her story with me.  Since then, she has shared pictures of little Tater on BWB's facebook page.  This post is dedicated to Tater and his loving parents, Liz and Bubba.  The following is her written story and the captions to the pics are in her words.

~Our Story~

I would like to introduce myself. I’m Elizabeth and I’m married to James. We have a beautiful son named Benjamin. He has severe hemophilia A with inhibitors.. I’m a nurse (but I stay at home with Ben) and my husband is in the army. Here is our story.

After 6 days of my water being broke. 3 internal monitors placed.
Finally a csection due to failure to decend. 5 weeks early

subgaleal bleed from 3 internal
monitors being placed

We decided to get pregnant. Nothing ran in our families so we were expecting no complications. My pregnancy was wonderful. Then at 34 weeks my water broke. I was admitted to the hospital and 6 long days later Benjamin came in to the world at 35 weeks via c-section because he failed to descend. I kept asking what his apgar score was (a score that basically says how well the baby is after birth) they would not tell me. They put a hat on his head. Let me kiss him and my husband held him for a brief second. And they whisked him away. When I was in the recovery room they told me that he was in the nicu on oxygen. I finally made it to the floor and my husband said they would not let him see the baby. We called up there and they said to come pick up some paper work and supplies for breast milk. My husband came back and told me there was something wrong with Bens head. The next morning the doctor came to talk to us and told us that Ben had a subgaeal hematoma. That is a bleed in between the scalp and skull. It looked horrible. They had no idea what caused it. They needed our consent to give blood transfusions. They ran a clotting test called a Prothromin Time (PT) but every sample clotted before the lab could run the test. After a week they said we could take him home. We roomed in with him. They did a heal stick on him and he bleed thru a band aid paper towel house shoe and six blankets. He no longer could maintain his temperature. They took him back to the nicu and ran tests. The next morning they did a ct scan and found a subdural bleed. This is a bleed between the brain and skull. They wanted consents to start cryoparticiapate which is packed blood cells and give him a drug called Novo7. They were consulting with a neurosurgeon. They told us the baby would need brain surgery to evacuate the blood. We went to see him. We were waiting for the next step and all of a sudden they said it was time for the surgery. We had a priest come and baptize him. 

Right before the surgery.
Subdural bleed causing stroke and seizures.

3 internal monitors placed. They "screw"
into the scalp. And they "came out"

And he went down. The surgery took about 2 hours. When the surgeon removed the skull the hematoma expelled itself because of all the pressure. He also had some clots in his brain that they removed those clots caused him to have a stroke. They inserted a Picc line in his head. So they wouldn’t have to keep doing ivs in his hands. They kept him on a ventilator and in a medically induced coma. After a few days we were transferred to a large city where we immediately got a diagnosis of hemophilia a. His level was 16%. They started advate and weaned him off the ventilator. Ben did so well. He started eating on his own so they could remove the feeding tube. He held his own temperature. It was wonderful. Then his IV accesses stopped working, even the Picc line. We were then bombarded with people who wanted to put in a central line. I asked for a central line for days. We consented for one and they decided to put in a picc line at the other hospital. This whole time a hematologist only spoke to us to give us the diagnosis. NO other time. We kept asking. SO after a hematologist spoke to us and told us he had to have the broviac (central line) to go home. He did very well with that surgery.

Healing at surgical site

We were soon able to take him home. The only thing we knew is we had to give advate three times a week and they told us he would never get to be a normal boy. We had lots of questions that they would not answer. Like can he play baseball or ride a bike. Do we get extra advate in case he falls? You know stuff like that. They said our questions would be answered later on his life. I got tested and I am a carrier at 12%. And we retested Ben and his level was <1% so he is a severe. Lots have happened since all of this. He now has a port. He also has an inhibitor. That complicates the situation even more. I have met some awesome people thru all of this. He is doing really well. We attend Physical therapy and occupation therapy and many many more appointments.

Tater's scar 17 months later

"Normal" by Amber Webb

I read this the other day, and was deeply moved.  This beautiful note was written by my cousin's wife.  They, like us, have a new normal.  I think you will be blessed by these words spoken straight from her heart. . .

~Normal~

It's funny how that word takes on new meanings as we walk through life. Normal.
I remember being pregnant and talking to Micah about what it was going to be like to have a little boy: all the things we would do; where we would send him to school; what his room would be like; who he would look like. I remember holding him in the hospital room when he was born and thinking, "This has to be the most beautiful baby ever born." The night the nurse told us Ben had Down Syndrome is also one that I'll never forget. The most heartbreaking moment for me was hearing Micah say, "That means I can't teach him to fly." His airplane themed nursery suddenly didn't seem to fit. It was almost as if we were mourning the loss of the child we planned for. Everything changed in that moment. This "normal" we had planned suddenly vanished.
The days and weeks after that have had their ups and downs. Some days I wonder where the balance is between acknowledging that Ben is different and ignoring it completely.
Here's what I've learned so far...
My son is different than yours. SO WHAT. My version of normal has changed. SO WHAT. I'm thankful for that pesky extra chromosome and I'm thankful the Designer decided to bless us with such wonderful little boy.
Micah is back overseas working hard and my time is consumed by a precious little boy with Designer genes.

God is still good and that's MY normal. What's yours?