Sweet Sammie – a smile beyond the struggle

sammie-1This is Sammie.

After having the below conversation with her mom, I enter this week with a fresh perspective on fatherhood and life.

To see the world from the eyes of another, and take that view with us on life’s journey, is to reach greater heights as a parent and as an evolving human being in general.

That’s why this week, I’m excited to share an inspiring Q&A interview we did with family, friend and mother of three, Angela Knop.

Her youngest, Sammie, has an extremely rare medical condition, known as Poland’s Syndrome. This summer, as part of her ongoing treatment, Sammie is set to have two surgeries to correct an over-curving of her spine.

Treating a child with a medical condition is tough. Treating a child with a rare medical condition, as Angela admits in greater detail below, it to say the least, extremely frustrating.

But thank heavens for the Angelas of the world, and for all those who give a part of themselves, so others may benefit.

Here, Angela has given us her time and full attention to tell us about Sammie and what it’s like having a great kid with a tough battle.

This is her story.

sammie-6Q: Can we start off with an introduction to who Sammie is, how you’d describe her?

A: Wow…describe Sammie… Sammie is the type of child who’s smile lights up a room, not just because she is mine, but really: She is full of energy, enthusiasm, and spunk. She is a force of nature. She is so aware of how much love is showered on her and she generously returns that love to everyone she knows.
She is bright and cheerful, always singing and has an infectious giggle. She, even in 4th grade, kisses and hugs us both goodbye in the morning and waves and blows kisses from the bus window until we are out of site.

Like any little girl, she loves her American Girl Dolls, arts and crafts, polar bears etc. She also is a caretaker; a little mother hen to her younger relatives and our pets. She is amazing!

sammie-2Q: Most people aren’t familiar with her condition. Can you summarize what Poland’s Syndrome is and to what degree does it affect Sammie now, and how it might affect her in the future?

A: (From the Poland Syndrome Website) Named after Sir Alfred Poland, it is described as an absence or underdevelopment of the chest muscle on one side of the body and webbing of the fingers of the hand on the same side. Sometimes referred to as “Poland anomaly,” it is a rare condition present at birth. For people born with Poland’s Syndrome, the breastbone portion of the chest muscle is also missing. Poland’s Syndrome is often not diagnosed or reported. Poland’s Syndrome is three times more common in boys than girls, and affects the right side of the body twice as often as the left.

Samantha is a rare case of a rare syndrome. She is a girl and affected on her left side.

Her involvement includes a missing pectoral muscle, several ribs, and breast. Her hands are not affected. Because of the deficiency on her left side, she has scoliosis. At her first X-ray at two months old, her curve [in her spine] was 18 degrees. By the time she was eight, it had increased to 27. However in the last two years it has rapidly increased to now being 47 degrees. This causes her a fair amount of pain. She is determined to not let it slow her down though.

This is why she will be having surgery this summer. She will be having both anterior and posterior spinal fusions. Long term, she will need more surgeries for rib replacement and breast construction. This will wait until she is mature.

Q: What is the extent to her treatment thus far?

A: So far, she has only had two MRI’s and X-rays every six to 12 months. She gets massage therapy also, and takes Motrin for pain and uses a heating pad.

sammie-3Q: What are some of the challenges associated with treating rare conditions such as this?

A: I think the most frustrating thing is that it is so rare that a lot of doctors haven’t even heard of it, let alone ever treated a PS child. It is also hard to get accurate statistics because of the rarity.

Q: Can you describe some of the research you did on it, what you found, i.e. the extent or lack of medical knowledge about it, and if you know how many others are affected?

A: Gosh, I have read everything I could get my hands on. http://www.polands-syndrome.com/ is a great site. PS occurs in 1 out of every 100,000 births.

Q: What advice would you give to other parents whose children might have a rare condition? Can you recommend any resources?

A: I guess my advice would be to be vigilant – keep looking. The Internet is a wonderful thing. Also remember, try not to get paranoid and freaked out by what you read. Like with anything, everything you read may not pertain to your child. Use the info as a resource not the rule. Try NORD (National Organization for Rare Diseases). Also, any children’s hospital can direct you in the right direction.

Q: You’re a nurse. Can you summarize your professional medical credentials?

A: I am an LPN and I worked for years in both Hospice and Alzhiemers.

Q: When did you learn that she had it, and what were some of yours and Jacek’s (husband) initial reactions?

A: Shock. And the fact it was called “Polands Syndrome.” My husband is from Poland and I remember us looking at each other when they called it Poland’s Syndrome. As we quickly learned “Poland” was the name of the doctor who discovered it. Sammie was not diagnosed until she was two months old; although it was evident something was not right at birth. The hardest part was that “it” had a name and our precious baby girl had a “syndrome.” It was terrifying and heartbreaking.

Q: Can you describe what it’s like as a parent to live with it? Where do you and Jacek draw your strength?

A: From the very beginning, Jacek and I made the decision to treat Sammie exactly like our two older children. We have never held her back from anything. She has played sports, rode horseback, swam, ect. As a matter of fact, people who have always known her are surprised to hear that she has this. We put her Poland’s Syndrome on the back burner most of the time – after all, this doesn’t define who Sammie is. We deal with it as we need to and when she is symptomatic. Until the last year or so with her spine curve increase, she has never had issues.

sammie-4Q: I’m curious about your marriage dynamics. In what ways does your deep and close relationship with Jacek factor into getting through the rough days?

A: Thank God for our marriage. It comes down to the basic trust, love, and the fact that we are great parents. We sat across from a doctor this week who was telling us our baby had to have two consecutive surgeries this summer. As I felt the world fall away, my husband reached out and held my hand. I instantly found my footing. We both keep lines of communication open. We both know that we can be scared, worried, whatever with one another. We realize we are the only two people in the world feeling the same thing about our child. Going through this together has only bonded us more.

Q: How does Sammie feel about it? Where does she draw her strength?

A: Samantha is aware of her PS. She however is not aware of the extent of her surgeries to come. We will explain much closer to the date. Sammie is delightful. She knows she is beautiful and perfect. She knows that like everyone, she is different in some ways too. Her best friend has had open-heart surgery, so she knows that everyone has differences. Hers just happens to be PS. She did one night call me into her room. She was in tears and asked why does she have PS, why her? (a question I have asked myself a million times). We just reassure her. I also was blessed enough to find a girl in Canada that is four months younger than Sammie with PS. They are pen pals now. It helps her to realize that there are other kids out there.

sammie-51Q: How do her brother and sister approach it? Is it a topic of frequent discussion? Why, do you think, or why not?

A: Both Jonathan and Alex are very loving toward her. She is also their pesky, pain-in-the-butt baby sister. It is usually not a huge topic here, but has been recently due to the pending surgeries. All three kids know if they have questions we will answer them honestly. It is not a subject we avoid, but not one we dwell on either. Both kids are worried. I went upstairs last night after we had told them about the surgeries. There they all were, piled into a one bed – my 18, 13, and 10 year old – Sammie, of course, in the middle.

Q: What has it taught you as a parent?

A: You know you always hear, “a child is a blessing,” and believe me, with my first two children it was love at first site, and I felt blessed. But to have Sammie, and get to be the parent of this amazing young lady who just simply loves life, you learn very quickly what a miraculous blessing they are.

Q: Health care change is a hot topic in today’s discussions. What changes to the system would you like to see?

A: I think there needs to be a more central information center with doctor and specialties listed, especially for rare diseases.

sammie-7Q: Lastly, it’s clear that you absolutely love kids, and not just your own. Many times, you’ve opened your home up to your friends and family’s kids. You’ve invited your nieces and nephews along on your family vacations, and you’re usually first in line for the long-duration babysitting gigs. What is it about children that you love so much? Why?

A: Goodness, what is not to love? The innocence, the fact that the simplest thing can bring a huge smile. That they look at you with such trust and honesty. That you can act ridiculous just to hear a good belly laugh. The way there hair feels against your cheek when you hold them, the feeling of a little hand clasped in yours. It is the ability to see everything again, for the first time, through their eyes 🙂

– Thanks Angela.

Now, I’ve always considered myself an optimist – and I believe if you throw enough positive energy at something, good things start to happen. So many thanks to Angela for her great outlook, to her fantastic family, and to her sweet trooper, Sammie.

And thank you too – the readers of On Fatherhood – for joining us in sending out those positive thoughts in her direction.

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23 Responses to “Sweet Sammie – a smile beyond the struggle”

  1. Petrina Meyers Says:

    The Knops are one of the most loving families I have ever met. They exemplify the meaning of “good neighbor” in every aspect. The children are delightful, courteous, bright, energetic and affectionate. Sammie’s sister, Alexandra– Allie as we know her, is a friend of my granddaughter Rebekah. Rebekah had a brother, Nathan, who was handicapped and challenging. Allie always showed him kindness and understanding and had no fear of him like Rebekah’s other playmates had, including him in their play. I will always be grateful to her for that. My prayers will truly be with this beautiful family, especially Sammie and the doctors who are treating her.

  2. Angela Knop Says:

    Luke,
    Words cannot express the gratefulness of letting other people get a glimpse of Samantha…and selfishly…the more prayers the better. You will never know what this has meant to us…to me…and as you already know…you hold a special place in my heart…and this reminds me why. Thank you…and I love you beyond words….
    AA

  3. Tracy Says:

    What a great interview and article. Our thoughts are with you all, Ang. We love and miss you.

  4. The Kallin Family Says:

    Angie and Jacek – Sammie and your entire family are in our thoughts and prayers. If there’s anything that WE CAN DO, please don’t hestiate to contact us. Love – Chris, Kristen, Kendra, and Makenna

  5. sarah Says:

    Please send Sammie my love and let her know she’s be ok. I’m a rare case too – female, left-handed and it affects my left side. I’ve several ribs missing and an under-developed breast. At 20 I opted for surgery to reduce my right breast (I wanted to feel ‘all me.’) which was a lot larger. I kept sugery a secret from the guy I’d been dating as I barely knew him – the difference had always effected my confidence with boys! The surgery was great and I’m glad I did it – for me – as it really gave me confidence. Whether or not Sammie ever has a similar op – make sure she does it for herself. I dont regret it – but turns out 8 yrs later Im marrying the same boy (now man) and he wouldn’t have loved me any less pre-surgery.
    Im a writer and theatre actress now – P.S. HASNT HELD ME BACK ONE BIT! So good luck Sammie in all you choose to do – I’m sending lots of love xxxx

    • Angela Knop Says:

      Sarah,
      What a beautiful and unselfish thing to do…share your story, with us. It gave both Sammie (an I, her Mommy) a small sense of relief (which is rare these days with the surgery coming up). Your words are appreciated more than you know. If you would like me to add you to her hospital care page when we are admitted you can email your email address Angknop@gmail.com. If you are comfortable with that. Again…thank you…and God Bless you!!!
      Angie Knop

  6. lukepinneo Says:

    Great dialog here folks. This is exactly what On Fatherhood was intended to be.

    Many thanks to everyone for sending along such messages of hope to Sammie and her family.

    The goodness of people is seen best during trying times.

    I am inspired and deeply moved by the power of the people on the web who can foster such a nurturing community.

    It’s long been said, it takes a village to raise a man. In the 21st century, it takes a community to raise decent, honest and healthy children.

    Thank you all for helping to shape that community.

    Best,
    Luke

  7. James Sagriff Says:

    Hi Angela, it’s funny you know i don’t think we have ever talked.MY wife has told me so much about you and Sammie, that i feel i already know you.When she told me that she gave Alex her own e-mail address i was wondering what for. Because as a father and having a little girl ten . I didn’t want her to have an e-mail adress, until she told me why. It has been great to watch her exchange the e-mails with Sammie her face lights up everytime she gets mail from Sammie.We have explained to Alex what is going on with Sammie she is very concerned and hopes everything goes well. Our prayers are with you and your daughter. God bless and your family.

    James Sagriff

  8. Julie Harvey Says:

    Sammie i will be praying for you all the way, Jesus Christ is with you every step of the way, and so are my prayers, god bless you and keep that beautiful smile always.

  9. Kristie Says:

    My prayers are with Sammie’s parents as I too have a 4yr.old lil boy with PS…He is left side affected with hand anomaly. Thank you for sharing your story and educating people about PS.

  10. Beth Steinzor Says:

    Hello,
    I’m 44 with Polands I had no idea that this even existed until about a year ago, I had surgery when I was 16 just for breast reduction on one side. I want to wish Sammie luck this summer, I’m sure she will be more comfortable after her spine is straighter. Its great you have found this info out for her, she will need to know through her teens years that she is not the only one going through this. Good luck
    Beth

  11. Nico Says:

    I honestly have to say this has to be the best article I have ever read.
    I am a 20 year old male and currently have PS.
    My right side of chest is missing. I dont have any problems with my hands. It really first hit me once i turned 15 16. I would go to the beach with my family and friends and I was embarressed to take my shirt off.
    I am a very athletic person. Play soccer every day of my life.
    Its funny because when you stop and think “wow, Why me?” DONT! I tell you everything in life has a reason to it at the very end.
    I Now look at the big picture and say wow I am 20 years old and i am HEalthly! I can see, breathe, walk. So we have it alot better than other people.
    We have to be thankful for the family we have that support us.
    I recently told my friend of 9 years that I had PS. Me thinking that when i was going to show him, he would be like woah whats wrong.
    He looked at me and said Nico, What does that change. And i said nothing but i feel uncomfortable all the time.? He said you are a great person and trust me everyone has defects. But its how you live with them and react to them who makes you YOU!
    I am currently a very intelligent Adult.
    I have my real estate license, 440 license and I am going to keep moving up.
    The reason why i share what i do is to show everything that has PS.
    The people who have PS have a very difficult life with already feeling comfortable.
    Once you get over that you can stand up to anything.
    So I end this messege by saying thank you for story and keep your head up!!! we have our whole life in front of us!! =]
    God Bless!!
    -Nico

    • Angela Knop Says:

      Hi, This is Sammie’s Mommy…I am so grateful for all the kind words of encouragement and hope. If you would like to follow Sammies surgery journey on her hospital Care Page please email me @ Angknop@gmail.com and I will send you an invite. God Bless you all, and again please accept my heartfelt thanks!

  12. Tracy Kelly Says:

    Sammie,
    As the surgery date draws near, I sit back and think of my life with your Mother as well as you. You are truly blessed to have such a loving family, as they are to have you.
    I feel honored to be a part of your life and to know the beautiful person that you have become. We will always have a place for you and your family in our hearts. I love you Sweetie!
    Love,
    Aunt Tracy and Tyler

  13. Jodi Lynn Bellinger Says:

    Sammie, my long lost little friend!! Such a beautiful young lady you’ve become!! I cannot begin to tell you how your family, and you particularly, have effected me throught the passt few years. Throughout my career as an Occupational Therapist, I’ve had the opportunity and pleasure to provide services to many many young people, but few who have effected me as you and your family did. I have faced several health challenges over the last few years – breast cancer, blood dysfunctions requiring transfusions, the loss of a baby from medical complications – and honestly, throughout all of it I’ve kept in the back of my mind the struggles you’ve fearlessly faced with your PS and the unwavering support you receive from your family. I feel so fortunate that the paths of our journey crossed, Sammie, and want you to know that you were/are a blessing in my life. I’m sending you positive energy, prayers and good karma for a smooth surgery, rapid recovery and quick return to the active lifestyle you so enjoy. Angie and Jacek, thank you so much for the gift of Sammie as well as the gift of your friendship. Sending you peace during this scary time, but rest easy…..Sammie’s light shines so bright that I’m confident she’ll be on the mend and back at it in no time…….

    • Angela Knop Says:

      Jody,
      It is so wonderful to hear from you. Thank you for all your kind words and prayers. You were also a blessing to us. When we get home from Boston, we need to be sure to catch back up in person. Wishing you the best of everything!
      Much Love,
      Ang

  14. Pip Davies Says:

    Thank you Angela for sharing your story. My daughter is also affected by Poland Syndrome – I suppose it makes them both rare little gifts. It is great to read of someone who is further along in this journey and who is so positive about it. God sends kids to parents for a reason and it is obvious from your story that he made the right choice sending Sammy to you.

  15. Lisa Cohn Says:

    Hi Angela and Sammie,
    Every once in a while I google PS to see if there is anything new. And there you were! Such a beautiful young girl! The joy of my life is my 9 year old daughter. She has PS too. I always hoped we would meet someone else who Sanni could get to know. Sanni is pretty shy and quiet, but she loves to read, draw, play, and to spend lots of time outside. Though she is missing several muscles she is strong and can do anything. I bet you can do anything too.

    I am really happy that you and your mom were willing to share your story with us.

    We hope you are doing great after your surgery last summer.

    Lisa and Sanni

  16. Jeanniebopp Says:

    This is a very inspiring story my daughter turned 12 in Nov. and was just diagnoised with PS in Aug. she just had surgery on Tues. of this week. But it sounds like her PS is not as bad as your daughters. But this article has inspired me to a great extent. And I will let Nicole (my daughter read it). If you have any questions feel free to email me!

  17. Angie Knop Says:

    It brings me great happiness to know that Sammie’s story can bring comfort to others. Samantha is doing amazingly well. We just celebrated the one year anniversaries of her surgeries. She is still her bubbly, smiley self. She is back to all activities and is enjoying lots of swimming this summer. She has only grown stronger both in mind and body since last summer. She is an amazing young lady. I feel so blessed to be her Mommy. My email is Angknop@gmail.com to any of you who have questions or concerns or just need a sounding board. All the best to all of you. And to Luke, thank you again so much for giving Sammie, PS, and me a voice.
    Love,
    Angie

  18. Fernando Says:

    Hey Sammy and all!
    As I wrote about a couple months ago I am a 21 yr old male who also has Poland syndrome .
    It has been so great being able to go on here and read everyones thoughts whenever I am down .
    Thank you all for all your help! I am scheduled for a diep flap surgery at the end of this month to fix my chest.. Which is the only part that Poland syndrome affected.
    I’m so excited for this since this has been what I have wanted now for a Longtime..
    I will jeep you all updated after the surgery! Thank you all! Take care 🙂

  19. Lavonne Says:

    Thank you for writing – my now 6 year old daughter adopted from China also has PS and is left side affected as well.
    When we first started out on this adoption – it was unknow what she had and finally found a great doctor in Seattle that led us to PS and we quickly learned there was little information about this condition. Since there is much more information out and great sites to connect with others with PS. We recently found a little boy only 2 miles from us that also has PS, which is wonderful to have another person for my daughter to connect with that shares this with her. A book was recently published by Cynthia Sharp who also has PS in CA and the book shares stories of other PS survivors who live with this daily.
    My daughter does not have the curved spine, but just had the VEPTR Titanium Bar placed in her chest to support the left side that has no ribs in the front. But originally this was used to reverse the curving of the spine – Dr. Robert Campbell designed this in the late 80’s and it has done wonders saving lives. I believe he is now working at CHOP in PA – you may want to look into this for Sammie.

  20. Angie (Sammie's Momma) Says:

    Lavonnne,
    Small world…Sammie’s story is also included in Cynthia Sharps book. I actually showed my husband the article on you daughter because of the VEPTR involvement. Sammie had her anterior/posterior spinal fusions in July of ’09. It was a very extensive, rough surgery. Sammie was such a brave girl. She is doing amazing now 1+ year post op, with no restrictions. I wish you all the luck with your daughter. If you ever need to “talk” you know where to find me.
    Angie

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