Pregnant AGAIN with Sickle Cell Disease
One thing that my good friends and family know about me is that I am amazingly stubborn. I like to think that this is a particularly amazing characteristic that I have which has enabled me to remain motivated and tackle obstacles against all odds.
And those odds are definitely stacked against me.
I am currently 24 weeks pregnant with my second child 😨 I know what you’re thinking. Didn’t I learn my lesson the first time? The short answer is yes...and no. So maybe there is no short answer. But I do know that individuals with sickle cell disease and other chronic illnesses have the same human urges to procreate. I'm not saying everyone desires to be a parent, but generally, we have similar wants, needs, goals and dreams as healthy people. I've accomplished a lot in my short time on this Earth and I am so proud of me for hanging in there through some tough situations. But maybe you don't know about those? Okay, I'll tell you.
I wasn't diagnosed with Sickle Cell Disease until I was 13 years old. I took a ski trip to Monarch, CO which boasts an elevation of about 10,500ft. As you can imagine, that didn't go over well with my body and I found myself with what I thought was an upset stomach in the ski lodge for the majority of the day. I'm sure you're wondering why it took 13 years to be diagnosed? The accurate name of the type of Sickle Cell Disease I have is called hemoglobin SC disease (HbSC) or Sickle C disease. This distinction indicates that I have a sickle cell trait (HbS) combined with an abnormal hemoglobin trait (HbC) which created a subtype of Sickle Cell Disease. Although I went through newborn screening when I was born, this subtype was not as prevalent or as well known as traditional Sickle Cell Disease (HbSS) and my parents never knew I carried a variation of the disease. The good news is that this subtype, along with other variations, are commonly included in the newborn screening now. Also, a simple blood test called an electrophoresis can be done which will also give you information on whether you carry any of the traits or subtypes such as HbSC.
HbSC manifests in similar ways as HbSS, however there are some pretty unique ways that they are not similar. For instance, my hemoglobin levels tend to remain higher and can be interpreted as me being slightly anemic. I don't necessarily have the "typical" indicators of a sickle cell crises which means that the amount of pain I am in is not usually represented and/or observed by a decrease in hemoglobin values. Instead, I am more prone to vascular occlusive crises which mean my blood cells (whether they are shaped in the form of a sickle or just sticky) get clogged within my veins, arteries, joints, organs, tissues, etc., and restrict the amount of oxygen going to those areas. My body then responds to the lack of oxygen available and sirens go off in the form of pain. As providers are looking for clues as to why my excruciating pain has increased, this prime differentiation in the manifestation of HbSC vs HbSS often gives the impression that I am without pain and/or there is another underlying issue. Also, my blood counts were lower throughout pregnancy while many of my friends with HbSS felt much better with higher red blood counts.
Another difference I have experienced with HbSC is that I tend to have prolonged pain. Period. I've had many surgeries and it is now commonly known amongst my providers that my pain tends to linger longer than those without this disease. I'm unsure of why this is, but I can imagine that with the backup and delays of my blood to oxygenate the areas affected by surgery, my body needs more time. It also appears that there are certain incidences throughout life that trigger more pain than normal. For me, pregnancy is one of them.
When I was pregnant with my first child, my pain was centered around my hips and spleen. There were other areas that were affected, but the hip and spleen pain dominantly presented itself and other sites of pain were mostly ignored. As my blood volume increased during the pregnancy, more blood was being filtered through my spleen. As a result, my red blood cells that were sickled and/or sticky were trapped preventing the flow of oxygen within the spleen. This caused me extreme pain during that pregnancy as my spleen expanded to a size of 18 cm and wrapped around to the back of my left side. I also experienced more drops in my red blood count and my body wasn't producing enough red blood cells to keep up with the demand. Once I had my spleen removed a few months after my first child was born, I was told that it was riddled with areas of avascular necrosis (AVN) which is a fancy word for bone and tissue death due to lack of oxygen in the area. Also, my hips had been severely affected by AVN as well which has resulted in me having multiple surgeries on my hips, including having two total hip replacements on the same hip.
With the amount of issues triggered primarily by my pregnancy, I was reluctant to face those odds again. My son is 8 years old, thriving, and is a giant even though he was born prematurely at 33 weeks via emergency c-section. During this time I also faced more surgeries, two strokes, seizures, and many, many hospitalizations for sickle cell crises and its complications. However, I have had a strong desire for a second child even though I was afraid of the consequences. Ultimately, I felt that I had gotten myself to a place in my health where I felt I could carry another child. My health was a bit stabilized, I'm still solid in my marriage, my son is doing fantastic and I was well aware of the complications that could arise in the event that HbSC was triggered by a repeat pregnancy. Based on the past incidences, I didn't want to take any blind chances and wanted to assemble the best team possible with a plan of action for this pregnancy.
The final note was to make sure I was mentally prepared to face the pain again and had a plan of action. As a woman with such complex health issues, I have several specialists on board. My team consists of my primary doctor (whom I only see about normal issues), OB/GYN, maternal fetal medicine (high risk specialist or perinatologist), and two wonderful hematologists. One of the hematologists specializes in blood diseases and disorders with significant research in Sickle Cell Disease. Knowing the complications that arose during the pregnancy with my son prompted me to assemble a team, discuss every detail, and be better prepared the second time around.
This pregnancy is not without its issues, but baby is doing great and I'm taking it one day at a time. Pain is still a significant issue. If I am to be honest, what hurts the most during this pregnancy is that there is still a huge misconception of what Sickle Cell Disease is. The complications to a mother and her unborn child aren't as well known. Treatment for those complications that may arise are also unknown for most of the individuals that I have interacted with. This can cause extreme unease by providers less familiar with me as a person, and unfamilar with sickle cell as a disease. Unfortunately, I have experienced the result of many provider's lack of knowledge which manifests as disrespect, disregard, discrimination, judgement, unfair assumptions, negative attitudes, them being argumentative, and harmfully callous in the opinions that they voice.
This is why I open up a part of my life and write about my experiences. Perhaps there is someone out there that will benefit from hearing a mother's perspective on living life with this disease and desiring to expand her family. Or maybe someone who knows absolutely nothing about the disease will come across this post and learn something new about the 100,000 of us that are living in the United States with Sickle Cell Disease.
And those odds are definitely stacked against me.
I am currently 24 weeks pregnant with my second child 😨 I know what you’re thinking. Didn’t I learn my lesson the first time? The short answer is yes...and no. So maybe there is no short answer. But I do know that individuals with sickle cell disease and other chronic illnesses have the same human urges to procreate. I'm not saying everyone desires to be a parent, but generally, we have similar wants, needs, goals and dreams as healthy people. I've accomplished a lot in my short time on this Earth and I am so proud of me for hanging in there through some tough situations. But maybe you don't know about those? Okay, I'll tell you.
I wasn't diagnosed with Sickle Cell Disease until I was 13 years old. I took a ski trip to Monarch, CO which boasts an elevation of about 10,500ft. As you can imagine, that didn't go over well with my body and I found myself with what I thought was an upset stomach in the ski lodge for the majority of the day. I'm sure you're wondering why it took 13 years to be diagnosed? The accurate name of the type of Sickle Cell Disease I have is called hemoglobin SC disease (HbSC) or Sickle C disease. This distinction indicates that I have a sickle cell trait (HbS) combined with an abnormal hemoglobin trait (HbC) which created a subtype of Sickle Cell Disease. Although I went through newborn screening when I was born, this subtype was not as prevalent or as well known as traditional Sickle Cell Disease (HbSS) and my parents never knew I carried a variation of the disease. The good news is that this subtype, along with other variations, are commonly included in the newborn screening now. Also, a simple blood test called an electrophoresis can be done which will also give you information on whether you carry any of the traits or subtypes such as HbSC.
HbSC manifests in similar ways as HbSS, however there are some pretty unique ways that they are not similar. For instance, my hemoglobin levels tend to remain higher and can be interpreted as me being slightly anemic. I don't necessarily have the "typical" indicators of a sickle cell crises which means that the amount of pain I am in is not usually represented and/or observed by a decrease in hemoglobin values. Instead, I am more prone to vascular occlusive crises which mean my blood cells (whether they are shaped in the form of a sickle or just sticky) get clogged within my veins, arteries, joints, organs, tissues, etc., and restrict the amount of oxygen going to those areas. My body then responds to the lack of oxygen available and sirens go off in the form of pain. As providers are looking for clues as to why my excruciating pain has increased, this prime differentiation in the manifestation of HbSC vs HbSS often gives the impression that I am without pain and/or there is another underlying issue. Also, my blood counts were lower throughout pregnancy while many of my friends with HbSS felt much better with higher red blood counts.
Another difference I have experienced with HbSC is that I tend to have prolonged pain. Period. I've had many surgeries and it is now commonly known amongst my providers that my pain tends to linger longer than those without this disease. I'm unsure of why this is, but I can imagine that with the backup and delays of my blood to oxygenate the areas affected by surgery, my body needs more time. It also appears that there are certain incidences throughout life that trigger more pain than normal. For me, pregnancy is one of them.
When I was pregnant with my first child, my pain was centered around my hips and spleen. There were other areas that were affected, but the hip and spleen pain dominantly presented itself and other sites of pain were mostly ignored. As my blood volume increased during the pregnancy, more blood was being filtered through my spleen. As a result, my red blood cells that were sickled and/or sticky were trapped preventing the flow of oxygen within the spleen. This caused me extreme pain during that pregnancy as my spleen expanded to a size of 18 cm and wrapped around to the back of my left side. I also experienced more drops in my red blood count and my body wasn't producing enough red blood cells to keep up with the demand. Once I had my spleen removed a few months after my first child was born, I was told that it was riddled with areas of avascular necrosis (AVN) which is a fancy word for bone and tissue death due to lack of oxygen in the area. Also, my hips had been severely affected by AVN as well which has resulted in me having multiple surgeries on my hips, including having two total hip replacements on the same hip.
With the amount of issues triggered primarily by my pregnancy, I was reluctant to face those odds again. My son is 8 years old, thriving, and is a giant even though he was born prematurely at 33 weeks via emergency c-section. During this time I also faced more surgeries, two strokes, seizures, and many, many hospitalizations for sickle cell crises and its complications. However, I have had a strong desire for a second child even though I was afraid of the consequences. Ultimately, I felt that I had gotten myself to a place in my health where I felt I could carry another child. My health was a bit stabilized, I'm still solid in my marriage, my son is doing fantastic and I was well aware of the complications that could arise in the event that HbSC was triggered by a repeat pregnancy. Based on the past incidences, I didn't want to take any blind chances and wanted to assemble the best team possible with a plan of action for this pregnancy.
The final note was to make sure I was mentally prepared to face the pain again and had a plan of action. As a woman with such complex health issues, I have several specialists on board. My team consists of my primary doctor (whom I only see about normal issues), OB/GYN, maternal fetal medicine (high risk specialist or perinatologist), and two wonderful hematologists. One of the hematologists specializes in blood diseases and disorders with significant research in Sickle Cell Disease. Knowing the complications that arose during the pregnancy with my son prompted me to assemble a team, discuss every detail, and be better prepared the second time around.
This pregnancy is not without its issues, but baby is doing great and I'm taking it one day at a time. Pain is still a significant issue. If I am to be honest, what hurts the most during this pregnancy is that there is still a huge misconception of what Sickle Cell Disease is. The complications to a mother and her unborn child aren't as well known. Treatment for those complications that may arise are also unknown for most of the individuals that I have interacted with. This can cause extreme unease by providers less familiar with me as a person, and unfamilar with sickle cell as a disease. Unfortunately, I have experienced the result of many provider's lack of knowledge which manifests as disrespect, disregard, discrimination, judgement, unfair assumptions, negative attitudes, them being argumentative, and harmfully callous in the opinions that they voice.
This is why I open up a part of my life and write about my experiences. Perhaps there is someone out there that will benefit from hearing a mother's perspective on living life with this disease and desiring to expand her family. Or maybe someone who knows absolutely nothing about the disease will come across this post and learn something new about the 100,000 of us that are living in the United States with Sickle Cell Disease.
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