Monday, June 25, 2012

One in a Million~ A Journey with Parry-Romberg Syndrome

I joked with Tayta after leaving the dermatologist's office in the fall of 2010 that we had always known she was one in a million, but now we had the diagnosis to support our opinion. Tayta smiled and laughed, as she often does, and did, even though she had just been diagnosed with a very rare autoimmune (probably) disease, Parry-Romberg Syndrome, for which there is no known cure. It is estimated that PRS affects one in one million people, though it could very well be that it is more common, but under-diagnosed. Since it took  visits to four dermatologists to receive a diagnosis for Tayta, I think that could be the case.


A short medical history: The onset of Tayta's PRS probably began with she was seven or eight years old; the first manifestation was a slight bluish area of skin on her left chin.  Her PRS progressed so slowly that we hardly noticed it, and it was a friend who encouraged me to take her to a dermatologist. After visits to a few dermatologists, some trials of expensive face creams, blood tests, and a skin biopsy, Tayta was finally introduced to a dermatologist who had heard of PRS and had seen a case or two in her residency many years previous. PRS can only be diagnosed by physical examination, which this dermatologist did.

Tayta's prognosis wasn't dire, nor was it encouraging: PRS affects each person who has it in a variety of ways and to different degrees, most noticeably the wasting or atrophying of the facial tissue on just one side of the face. Though Tayta's PRS was progressing, it was progressing slowly, and thankfully, was mild enough that it hadn't affected the growth of her face's skeletal structure, her jaw/teeth, or her vision. Nor has she experienced any pain. But, her PRS was progressing.

What to do? Though there is no cure for PRS,  there are some treatments which sometimes, though not always, help arrest the disease. Tayta took a mild anti-malarial for a year and a half and the next recommended step was a type of chemotherapy. The conventional wisdom is to wait until the disease arrests itself (though there is no knowing when that will happen) and then attempt reconstructive surgery. 

Since we didn't have a diagnosis until the Fall of 2010, and since some people have never noticed (or commented) the atrophy on Tayta face, we haven't  mentioned it to many people until recently. This past year however, we have been praying for Tayta's healing more earnestly, have asked others to pray for Tayta, and I began a new search  for possible treatment options. 

 I cautiously did another Google search last fall, afraid that the results would be more discouraging news and disturbing images of those who had suffered from more advanced cases of PRS. Instead, the first entry of the search was the The Romberg Connection, a portal of connections and resources for those who suffer from PRS. I believe this was God's perfect timing for Tayta and our family. I had done numerous searches in the years past but had never come across the Romberg Connection. 

I immediately joined the Yahoo email loop, made up of members from around the world who suffer from PRS or who have children who do. I posted an introduction and within hours a couple of parents wrote to recommend that we consider having Tayta seen by Dr. John Siebert, a plastic surgeon at the University of Wisconsin who had performed surgery on their children with great success.

A New Hope
I was very encouraged by what I read: Dr. Siebert had performed a number of innovative and successful surgeries on children with PRS:  he performs a very specialized microvascular free-flap tissue transfer surgery, which he calls an intervention rather than a reconstructive surgery. Instead of simply padding the atrophied facial areas with fat--one form of corrective treatment for PRS--Dr. Siebert takes live, healthy tissue from the patient's body and places it in the atrophied areas, connecting it via its blood vessels.  The healthy tissue "communicates" with the body, and it seems that by this means of intervention the biology of the disease is changed. This isn't officially a cure, but Dr.Siebert has seen a very high success rate of the new tissue living on, healthily, in those who receive this type of tissue transplant, halting any further wasting of facial tissue.

Moving Forward
Last month, just after attending Oldest Daughter's college graduation, we headed to Madison, Wisconsin, to meet and consult Dr. Siebert.  Dear Husband, Tayta, and I were very encouraged by the time we spent with him. He has a wonderful bed-side manner and immediately put us at ease with his knowledgeable comments about Tayta's PRS and explanation of the intervention surgery he recommends and performs. He was even able to identify some tissue atrophy near her nose and left eye that weren't yet visible to us, but when he pointed them out, we could see it and Tayta confirmed that, yes, she could tell the tissue was thinner in those areas. Dr. Seibert  gave us the encouraging news that Tatya's PRS had not affected her skeletal structure, making the reconstruction easier.


one of those clinical photos we've been taking to chronicle Tayta's PRS progress

Based on this consultation, recommendations from other parents, and research of alternative treatments, we decided that we should proceed with scheduling a surgery for Tayta this summer. We began to move forward in faith that God would provide, though not knowing how. Understandably, the costs for such a specialized and lengthy surgery, requiring a 3-5 day hospital stay, would be high. We still don't have all the numbers, but we are already experiencing God's hand of provision for Tayta and our family: on June 1st, our employer changed to a more comprehensive insurance policy. One inquiry from Dr. Siebert's administrative assistant yielded this good news: Both the physician and the hospital are in network (I've come to understand the importance of the phrase!) and the insurance company approved her surgery! (!!)

The Surgery
We are praying for a July 5 surgery date for Tayta, but won't know if there is an available operating room until June 28th or so. If she doesn't get this date, the surgery will be scheduled for August 15, preventing me from accompanying Artist Son and Dear Husband to take Artist Son to college.

Tayta and I will be in Madison for about 10 days once we travel.  The surgery will take approximately eight hours and Tayta will stay in the hospital for 3-5 days. We'll remain in Madison for several additional days for Tayta's post-operation examination. Tayta will need to have a minor revision, or "fine-tuning" surgery, anytime six months after her first surgery so that Dr. Seibert can correct the atrophy of her bottom left lip and smooth out any other areas which require it.


Please Pray
Please join us in praying for Tayta:
  • Thank God for all the good care he has provided for Tayta and for the good insurance coverage we have recently received.
  • Please pray for July 5 surgery date. I would sure like to accompany Artist Son to college, something Dear Husband and I have done, together, for our other two college kids.
  • Please pray for a successful surgery, and a good recovery for Tayta
I'll post an update when we get a surgery date.

So, what does Tayta think about all this?
Well, since I'm writing this, you'll hear my take on what Tayta thinks. The smile in the picture above is one that those of you who know Tayta are familiar with. She smiles broadly and confidently, and it is her joy to bring a smiles to the faces others. She was sad that she would be loosing her dimple, until she realized that it was caused by PRS. She also told me that if she knew her PRS would arrest at this point, she wouldn't feel the need for corrective surgery. However, her PRS continues to progress, so she agrees with us that surgery seems to be the best treatment option at this time.

Thank you for reading this far and for your care and concern for Tayta. Many of you have already prayed for her and I hope you were encouraged to read of how God has worked through your prayers. 

26 comments:

MagistraCarminum said...

What a sweet smile! praying now and through the coming weeks for God to do a marvelous work through all this. May He continue to be glorified in your family!

Pictoria said...

Thanks for sharing about your journey. Your trust in our Lord shines loud and clear. We are with you in prayer and hope! xoxo

Valda said...

her Smile is wonderful, any chance he will put in a corrected dimple for her when he does the reconstruction?

Carolyn said...

I'm so glad to hear the update -- and delighted that the consultation went well. We'll be praying the surgery goes smoothly and that recovery skips the adventure of complications.

Becky and Mark said...

Thank you for this great explanation. It helps us to know how to pray more specifically. We will be praying for the operating room to be available for the July surgery date.

Unknown said...

Amen! And keep smiling! We are, indeed, fearfully and wonderfully made.

Natalie said...

Beautiful smile! So thankful the Lord has led you to someone so knowledgeable about the syndrome. Praying for an early surgery date and great success for the doctor as he treats your daughter.

jlt said...

I can see why her smile blesses all those around her! I will be praying for the surgery to go smoothly and with no complications and also that you will be able to take Artist Son to college.

Alyse said...

Thank you for the update, and knowing how to pray.

Jodi said...

Yes!! You have our prayers. God bless.

Maida Hairabedian said...

Dear Manning Family.
Ill be praying for Kristen and for you all, ALL THE WAY! God is our healer, and He is going to be with Kristen during all these steps that she is going to be taking. Praying for JULY 5th! PRAYING for QUICK, FAST, SUPER SONIC recovery!!!!!!!!

Kristen, You are SO beautiful... inside and out. Your smile is too precious! I miss you! Praying hard core for sure. Us sisters must stick together! love ya!

my3girlz said...

Thanks so much for sharing. Tayta is a brave young woman. We will be praying for her, the doctors and your family.

thewierengas@msn.com said...

What a BEAUTIFUL creaton of our King is Kristen! Be assured of Iowa prayers. So thankful for how He has shown Himself faithful thus far. We don't need hindsight to see the blessings, do we? Looking forward with hope to future posts. Love you guys!

ElendilPickle said...

Thanks for the update. We will pray for you all!

Quotidian Life said...

Thank you all for your care, encouraging words, and prayers. They mean a lot to us and we are very thankful for our friends nearby and far flung.

Anonymous said...

Praying that all goes well and a July 5 surgery slot opens up.
Under His Wings,
Sheila

Laura A said...

Praying for Tatya's surgery, and I agree with everyone about her smile!

Anne said...

I just want to say that your daughter has the most beautiful smile! She - and you - will be in my prayers...

Heather said...

What a dear daughter you have been blessed with! Praying for this to happen as you desire. Christian love to you and your family!

k8tie davies said...

I have PRS, my condition started when I was 2 so my skeletal structure was affected. I have had over 10 facial reconstructive surgeries with Craniofacial Australia Prof. David David. He is amazing and has done amazing work on me. I am now 31 year old. I have never met anyone else with my condition. I volunteer my time at Craniofacial Australia helping their foundation.
You can find me and Craniofacial Australia on Facebook. If you have any questions I would be glad to answer them or talk to you.
My name is Katie Davies and my PRS took ten years to atrophy my face and I developed loss of muscle and vision in my left eye, loss of hair and epilepsy.

Anonymous said...

Would love to know how Tayta is doing now.

Unknown said...

I think I have the same condition. I'm going to my Dr Friday.

Anonymous said...

Hi Katie
I'm suffering from PRS from the last 2 years and really eager for some more information. I'm 29 years old.
Please let me know how I can contact you. My email is nt36@outlook.com. Please do let me know.
Thanks so much in advance..
Trish

Shannon Kendig said...

Thank you so much for sharing your story and journey. Do you care sharing if she has experienced any facial spasms with this? And if so, when did they begin?

Quotidian Life said...

Katie, My daughter hasn't had anything she's described as spasms, but she has had "sensations" in areas that have active PRS. One was more intense, like needle pricks, and it happened when she was ascending in an airplane. As far as I know, that was the only time she experienced it in that way/intensity.

Cheryl Rick Klein said...

I am the PRS patient that introduced Dr Seibert to The Romberg Connection. He performed a facial vascular flap on my face 15 years ago. Since every case of PRS is different the outcome of this very invasive major dangerous procedure seems to be Dr. Seiberts specialty. My case was more severe than your daughter's and I was 38 yrs old when he performed the 12 hour surgery, and I found out from another MD at NYU Hospital that Dr Seibert never completely the procedure because I was bleeding out when he accidentally cut a major artery near my ear. I had 5 blood transfusions and he forced to stop the procedure because I was going into shock. I know there are major risks involved in this kind of surgery buy Dr. Seibert with his very laid back charming beside matter seems to sell everyone who has PRS on this "miracle" procedure that cost 75k. My insurance paid me back only 16k and was not sent to me but to his office in NY. After several friendly calls my check was never sent to me. My brother had to go in person and confront him to get the check. After the surgery my face was held together by bolts and my husband tried to hid his repulsion the best he could. I had to have another surgery to fix what he never finished during the first surgery. My face was swollen on my right side 3x the size of the other side of my face. I hid for six months. Finally the swelling went down and yes I looked beautiful. The results however do not last that long. I needed and have had addition fat fillers added every few years. I saw Dr. Seibert Around June 2016 and he no longer had a medical license into practice in NY. I needed additional fat again by my chin. Dr. Seibert suggested another vascular flap procedure he would have tio perform in Wisconsin. I was like "No Way." I no longer recommend Dr, Seibert . He was no longer the charming "God" of plastic surgeons anymore. He seemed desperate to sell me a expensive surgery. I was so disappointed when I left the office. I wish him the best of luck.