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.