Sweet Sammie – a smile beyond the struggle

This 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.

Q: 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!

Q: 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.

Q: 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.

Q: 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.

Q: 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.

Q: 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.

Tags: poland's syndrome