living with sacral agenesis complications

This is part 9 of a series of posts by Julie Hemker, a young woman born with sacral agenesis, and her Mom, Charlotte Schild. You will find links to Julie and Charlotte’s prior posts at the end of this one. Much thanks to Julie for sharing her insights about navigating the silos of pediatric and adult medicine. Pat


Growing up with sacral agenesis (SA), a rare congenital spinal birth defect, is associated with many difficulties including physical manifestations and social acceptance. I am lucky that I innately have had the strength, fortitude and optimism to overcome the challenges of SA. What is challenging to accept and manage is the transition from the world of pediatric healthcare to adult healthcare.

Having been born in 1974, I have experienced firsthand many of the relatively recent changes in the healthcare system ranging from the length of stays post-surgery that have decreased significantly to cut costs to patient care issues, such as being able to get clean sheets daily or even a simple sponge bath.

I have also witnessed the impact of these changes on increasingly over-worked nurses. Now, it seems to me, nurses are assigned so many patients that they lack the time to give the one-on-one care that I know they would like to give or that I have received in the past. They are often exhausted from working 12 hour shifts.

These changes are “manageable” for me, as the patient. Out of necessity, I’ve learned to always be cognizant of my surroundings to ensure that I receive things that I need, such as ice water when I am thirsty or receiving scheduled pain medications on time—a shocking ordeal to endure.

What has been most disheartening and frightening to me is gaining access to appropriate medical care for my medical condition, sacral agenesis. I am now of the age where my original doctors and surgeons who took care of me in my youth have retired.

Many of my usual doctors do not treat their patients once they reach age 18 and are considered adults because of hospital “red tape.” It is only if the pediatric surgeon has operating rights with an associated adult hospital that they may be able to treat and perform the delicate and necessary surgeries of a patient, like me, an adult with a congenital condition.

Often it feels like I have been “thrown to the wolves,” left to find someone of the same caliber to treat me in the adult arena as I had in pediatrics.

Most adult physicians, who could potentially treat someone like me, an adult with sacral agenesis, are simply not as familiar with my type of birth defects even though they are commonly seen and treated by pediatric physicians.

Physicians in the pediatric world more frequently see childhood birth defects and the care is of a higher standard because of the simple fact that they see more children with these anomalies.  Therefore, they have a larger experience treating patients with rare birth defects.

It’s as if because we hit a certain age, the children’s hospitals “transition” us to the adult hospital—not necessarily to a physician who has experience or is the best fit given the disorder. It is very frightening to be thrust into a world where the physicians don’t know how to spell sacral agenesis let alone know the associated problems and treatment.

I have learned to take control of my own healthcare and search for the best doctors to treat me—this search has even taken me out of state. There is nothing I won’t do to obtain the healthcare I deserve.  Rare birth defects do not disappear or heal on their own just because someone turns 18.

In fact, as I’ve been aging—I am now 37—the rate at which I need surgery has not decreased although I’m well over 18. Aging is a “process” for everyone and my manifestation of sacral agenesis is degenerative with regards to my spine.

Now that I am facing a possible 12th spinal surgery, it’s increasingly obvious to me that the fight to find physicians who are a good fit for me medically or have the knowledge that my pediatric physicians had is difficult.

Yes, I have skills to cope with the chronic pain and remain optimistic.  Attitude is essential and aids in the problem-solving process of seeking outside help when my current health care options may not be the best for me—perhaps a physical liability.

All 47 of my surgeries have been necessary and have improved my life significantly. Looking forward, it actually frightens me that physicians won’t treat, because of lack of knowledge, the adults with rare birth defects. I have had doctors who have worked around the red tape to ensure my care was met.

There are physicians aware of this situation and who, against all odds, have found a way to treat me which is why I had surgeries in adulthood to keep me walking. But, what about those who aren’t as proactive as I am? I worry for those who are passive and accept what they are told by one physician even if it doesn’t feel right to them.

It’s exhausting to be in an acute medical crisis and also fight to receive the care from physicians who have the knowledge to treat you. I have said it before and I’ll say it again, I will crawl to get the healthcare I need. I am very proactive and empowered as an individual to receive what I deserve. I have methods for staying focused and overcoming obstacles. My whole life has taught me to be adaptive and how to work within whatever structure at a given time.  I’m lucky in that way.

I’ve always had a “fight song” (I called it) that helped me get fired up to win swim meets as a youth; I was a very competitive swimmer. This powerful song is “Eye of The Tiger,” by Survivor. When I need a burst of energy to make another phone call or fax more records, endure more pain in the waiting process I listen to the words,

“Don’t lose your grip on the dreams of the past you must fight just to keep them alive…just a man and his will to survive, he’s watching us all with the eye of the tiger.” 

This song charges me to take on whatever system I have to know I’ll come out on top.  It seems with age I listen to this song more. Perhaps I need the energy boost or it’s the memories which do keep me moving—I know what kind of care I had and will fight to have the same access as an adult.


Click here to read other stories in this series.

8 COMMENTS

  1. Here here Julie!
    I too am a woman born in 1974 with Sacral Agenesis ( as you know). I have been reading your blogs with great interest today and can relate so much. This blog is so VERY important for all people with a chronic illness. I found the switch from pediatric health care to adult very challenging, and for virtually the same reasons you’ve touched on. After just spending 9 days in the hospital I was asked to fill out a patient survey about the care I received. Hopefully my comments will be read and considered as I mentioned that during this visit I did the most self-care I have ever done. I am well aware that as a patient I am an integral member of my healthcare team, and am required to do as much as I can to get well. Not all people in the hospital are able to get out of bed, be proactive in having their needs met and realize that they need to know the hows and whys of all treatments. Good on you Julie for bringing this to light. You are a fantastic ambassador of what it means to LIVE a life with meaning …. with or without a chronic illness! Sending you much respect and positivity <3

    • Nicole,

      Wow, you are so very articulate and wonderful. Perhaps it’s the age and SA thing :) I’m so glad to have a partner in this challenge and glad others embrace the positivity necessary to navigate life, like you said, with or without a chronic illness! We’re like soul sisters. Please feel free to email me at hemkerjulie@gmail.com if you’d like to talk more. It’s nice that you are reading this blog. It means a lot to me. I thought I was so alone with this, not like in a pity way. I just thought I had these solitary thoughts. Cheers and best of luck!
      Best,
      Jules

  2. Dear Sandy,

    Hi! I know this is a bit different than the others in this series. It’s relevant and something about which I’m passionate. I’ll keep blogging. I never had blogged before but really enjoy it and being able to talk medically and personally. It’s a wonderful combination. Hopefully it impacts someone and the issues are heard as well.
    Wishing you all the happy heartbeats friend.

    Best,
    Jules

  3. Julie, your wisdom in writing this blog is most thought provoking.
    Yes, we are hearing and seeing more and more from people that have achieved incredible gains in the pediatric health care provider world experiencing little to no direction to sustain those gains once becoming adults.
    Then leaders like you appear and make others aware of these gaps to improve and make things better.
    Keep up the great blogging so that others may learn and apply better solutions.

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