PRS Update~Nearly One Year Post-Revision Surgery

Many have asked about Tatya's recovery from her revision surgery which she underwent last June. How is she doing? Has the swelling subsided? Will she need any more surgeries? How is her health? Thank you to so many of you who have prayed for her this past year, shared encouraging words, and asked questions which show your concern for Tatya. We have been in communication with her surgeon, Dr. John Seibert, and we were happy to receive his recent comments on her recovery and his prescription for the coming year.

Tayta's revision surgery was intended to be her final facial surgery, a follow up cosmetic surgery to remove excess tissue present from her free-flap tissue transplant surgery which took place in August 2012, and to correct the atrophy in her left lip. The surgery was successful, but Tayta has experienced residual swelling (left side) which, though decreasing, is still present.

After viewing the last round of photos of Tayta's face, her surgeon is overall happy with the results. Tatya will continue to massage the affected area a few times a day and may do a couple more steroid shots over the next year. We'll re-evaluate  her condition at two-years post-op, which will be next summer. Tayta was thrilled with this decision and prescription; she is happy with the results of her surgery, the condition of her face, and strongly felt that she didn't want to undergo another surgery at this time--not because it was painful or uncomfortable, but because she feels that her face is still healing and needs more time to recover fully from the last surgery.

 Tayta and friend 

We are all thankful that Tayta has not experienced any other symptoms of her disease, Parry Romberg Syndrome, which is believed to be an autoimmune disease, and thus has no cure. We pray and hope that her PRS is in permanent remission. Meanwhile, we are trying to educate ourselves about autoimmune diseases, as many who suffer from one autoimmune disease seem to be more susceptible to other  autoimmune diseases. Tayta has been working on establishing consistent, healthy life habits: exercising regularly, getting enough sleep, and eating good foods. I was surprised at how willingly she decided to drastically cut back her sugar consumption. Tayta has developed her repertoire of desert recipes which contain no sugar and no flour, and we are all enjoying the benefits.

Thanks you to those who have cared for us these past two years by your prayers and words of encouragement. God has blessed us with so much love and support through your care.

New Friends

It has been one week since Tayta's revision surgery and tonight we are packing our bags, preparing for our drive to Chicago in the morning and our flight to Jordan  tomorrow afternoon. Kristen had her post-op appointment with Dr. Siebert yesterday; he removed her stitches and told her that she looks great. I agree. Residual swelling should subside over the next six weeks.

At our last dinner in the Madison Ronald McDonald House, we met the Melendez family. Kristen, with her natural social astuteness, noticed them first, noting that their son appeared to suffer from Parry-Romberg Syndrome. We introduced ourselves and asked if they were here to see Dr. Siebert. When they understood the connection and we explained that Tayta had just undergone her second surgery with Dr. Siebert, an instant bond was formed. They were so happy to meet us. Though they had been in contact with other PRS families, they had never met another PRS patient in person, let alone anyone who had undergone surgery with Dr. Seibert.
Tayta talked with Diego--he was concerned about the pain he would feel and Tayta explained how all the nerves in his face would be cut. He would experience discomfort, but hopefully no pain.

Diego's dad and I took pictures of our PRS kids together. Tayta and I were touched by their family's story: Diego showed signs of PRS when he was two years old, and soon after Diego's father moved their family from their home in Puerto Rico to Orlando, Florida, so that they could find a treatment for Diego's PRS. Their long and persistent search and research ultimately led them to Dr. Siebert, and they are filled with hopeful anticipation as they prepare for Diego's first surgery on Monday. Diego's bone and tissue atrophy are quite severe so he'll likely have to undergo a few surgeries; he will need bone reconstruction as well as the free-flap tissue transplant that Tayta had.

Please take a moment to visit the blog which Diego's family has set up on his behalf. You can read more of his story and meet his family (in Spanish). While God has provided for most of the expense of Tayta's surgery via our insurance coverage, not all families enjoy this benefit and must raise money via donations to cover the cost of these expensive surgeries. Please consider donating and praying for Diego and his family on Monday as he undergoes his first surgery. We will be praying and cheering for him back in Mafraq, Jordan!

Not Just Another Pretty Face

For nearly a year, Tayta has been sporting the look of someone who just had her wisdom teeth removed on the left side of  her mouth, and she has done it with grace and confidence; confidence that came not only with knowing that the extra tissue in her face would eventually be removed, but confidence that God has called her His child and has given her a purpose for living, a purpose which did not depend on the symmetry of her face.

Many of you know of Tayta's diagnosis with Parry Romberg Syndrome, a rare autoimmune disease which causes the tissue, and sometimes the bones, on one side of the face to atrophy. I have written a few posts about Tayta's journey with this disease and about her free-flap tissue transplant surgery which took place last August. You can read those here, beginning at the bottom of the page. (I include these again as these posts have put me in touch with others who have children with PRS and I'd like to share information about Tayta's treatment which might be helpful to others.)

Tayta expected that she would have to wait until next summer, our regularly scheduled time to visit the States, to have the revision surgery, in which the surgeon would complete the cosmetic aspects of her treatment, but God provided the means for Tayta and I to travel to Madison, Wisconsin this week so that she could have the surgery sooner than later. An extra bonus of our trip was a visit with Oldest Daughter, living in Evanston. She treated us to a wonderful performance of the Chicago Symphony Orchestra, but the best part was spending time together and being there to help her move out of her apartment for the summer. God has been very generous with us!

Here we are, packed and ready to fly out of Amman's new international airport. We're also trying our hand at taking pictures with my tablet--I like my camera better.

Tayta's surgery took place yesterday morning: it was a three hour surgery in which her surgeon "de-bulked"  her cheek, took  a couple stitches above her left eye to correct the pulled skin, and filled in her left lip, using tissue from her right lip. The picture below shows just how great Tayta is looking several hours post-op.

One day post-op, the eye and lip swelling have increased and there is more discoloration of Tayta's skin, i.e. bruising, but that is to be expected with such a procedure. Her surgeon predicts a six-week recovery period. While this surgical intervention isn't technically a cure for PRS, it has arrested the affects of PRS in every person who has undergone a free-flap live tissue transplant.

Our hearts are overwhelmed with gratitude to God for providing this surgical option for Tayta and for the many friends, old and new who have helped us, cared for us, and prayed for us along the way. Thank you!

I will leave you with some wise advice from Tayta, given to me this afternoon: "Don't ever have plastic surgery unless you have to."

Surgery Scheduled

We've been praying and planning, and this week we confirmed the date for Tayta's revision surgery--June 20th, in Madison, Wisconsin.  Last night I hit the "confirm" button, booking our airline tickets to Chicago. Since Madison just happens to be an only three hour drive from Chicago, we're able to fly to Chicago and stay a few days with our favorite grad student, Oldest Daughter. We're already planning a trip to the Chicago Art Institute and a visit to Chinatown.

This recent picture of Tayta shows how well the tissue transplant is doing--the only problem is that there is too much tissue. But this was the plan from the beginning, and thus, the revision surgery. The surgeon, Dr. John Siebert, will remove the extra tissue, sculpting it (that's the best word I could think of but I don't know if it's the right term to use) to normal proportions. He also plans to build up her lower left lip, where she has experienced tissue loss. We've been told by the mom of another PRS patient that this second surgery is a piece of cake compared to the first surgery, a free-flap tissue transplant. This surgery will take only a couple hours, compared to ten hours for the first surgery. It may be outpatient or Tayta may stay one night in the hospital. We'll remain in Madison for the week for Tayta's post-op appointment.

Meanwhile, Tayta has a number of entertaining episodes to recount of  how well-meaning people in Jordan have reacted to her oversized cheek. Strangers are not shy about asking, "What happened??" I've observed Tayta handle these situations with aplomb, always trying to put the the inquirer at ease. Hmm, that would make a good blog-post when we have some time.

13 Weeks

The healing continues and the swelling in Tayta's face continues to subside, little by little. I sent this photo and  a few others to Tayta's surgeon, Dr. John Siebert, and his assistant, and these are their encouraging comments:

She's  looking great! The skin discoloration will continue to improve over the next 2-3 years as the skin layers thicken over time.  The eye is great.... just pulled down a bit due to the weight of the flap.  She will just need debulking and rearranging of the flap at her revision We are very pleased with how she looks so far! 

Quotidian Fall

What has kept me from posting on my blog these past weeks? I'm not sure. Some autumnal puttering, maybe. I've adjusted to the subtle cadence of fall in Mafraq; until yesterday the temperatures remained summer-like, leaving only the changing afternoon sunlight to remind us that fall and then quickly, winter would be here.

We had a nice cloudburst last week, a brief deluge of precipitation strong enough to wash layers of summer dust from the leaves. Though it has been too warm to be out in the garden doing the fall work of planting and clean-up, I've admired the fresh greenery from the windows. It's funny, I hardly even notice the iron bars on my windows until I take a picture of them. I've a view of a lemon tree from one kitchen window, and and two olive trees from the other.

From my bedroom window, I enjoy a view of the pomegranate tree, preparing to drop its leaves, lemon trees, a few remaining flowers. The top of the tall pines which guard the garden wall provide refuge to many birds whose songs reveal their presence even as they hide themselves from sight in the amongst the needles and branches. The weather is cooling this weekend, so I plan to get all my fall bulbs in this week. Olives harvest will begin as well.

A plant nerd, I delight in discovering the botanical name of my garden plants. This is not always easy as garden plants sold in Jordan are not marked. I've had this plant in my garden for several years and always referred to it as a bee balm, as it looked like other bee balms I has seen in family Lamiaceae (mint family). Thanks to an Amman garden shop whose Facebook page I recently discovered, I now know that this drought resistant plant, native to South Africa and southern Africa, is  Leonotis leonurus, also know as Lion's Tail and Wild Dagga. Its orange blooms provide a bit of fall color, such as I am used to.

 Leonotis leonurus, Lion's Tail 

Tulbaghia violacea

My "lillies" are still blooming and it turns out that they are actually in in family Alliaceae, which explains why their leaves smell like garlic when I handle them. I've discovered that I can use them in salads, perhaps like chives, though I've not yet tried them.

Tayta's face continues to heal (11 weeks post-op here). The swelling is still subsiding, though at a slower pace than before. We hope to schedule her revision surgery this summer.

Lavender Nursery

Along with the bulbs I hope to plant a few more lavender bushes in my garden, and another Russian sage bush.

Dear Husband has been having knee problems this fall and had to give up his/our morning running routine. He purchased this new step machine in Amman last week as a substitute and we've all been trying out it. It seems effective and we hope it will be a good substitute for running, at least for the time being. Dear Husband moves it to the sun room, facing out into the garden, when he uses it so that he can at least have the sensation of being outside while he exercises. I joked with him that if we were in the States, we could probably buy something like this at a garage sale for a song as so many people sell exercise equipment at garage sales. (I noticed my reflection in the mirror in this image--not quite as artistic a portrayal of the artist as in van Eyck's Arnolfini Portrait, and completely unintended.) At least it is compact, only taking up about 1.5 square feet of floor space.

In the kitchen, we're enjoying  a new fall soup, Red Lentil Coconut Soup, and I've finally mastered the basic sourdough bread recipe, using a San Francisco Sourdough starter gifted to me by a friend. Sourdough bread from San Francisco is a fond food memory from my childhood and I have a particular association of sourdough bread and visits to Fisherman's Wharf. 

Forget not all his benefits

When we actually managed to meet together for family devotions this summer, Psalm 103 was the psalm we reviewed together, having memorized it for the first time many moons ago:

"Bless the Lord,

O my soul,

and all that is within me,

bless his holy name!

Bless the Lord, O my soul,

and forget not all his

benefits,

who forgives all your 

iniquity,

who heals all your diseases,

who redeems your life

from the pit,

who crowns you with

steadfast love and

mercy,

who satisfies you with good

so that your youth is

renewed like the

eagle's..."

I do not want to forget any of the benefits that the Lord made very real to our family this summer. First, I would like to remember all the Lord's goodness to us in regards to Tayta and her facial surgery. God has been so good to us!

I wrote here about Tayta's rare tissue disease and the plans for surgery, and if you've read along you've already read some of the ways God has provided for Tayta and our family. The later, August 15th surgery date turned out for the best in so many ways. For one, Tayta was able to enjoy, and participate in to the fullest, all the family trips and  summer outings, including our church's annual family camp (that's biannual to us, of course). 

As Tayta professed Jesus Christ as her Savior some years ago, we had talked with her about baptism, leaving the decision to her. On the last day of family camp, Tayta told Dear Husband that she would like to take the step of baptism, publicly declaring her faith and trust in Jesus. It was an occasion for much rejoicing. My parents were thrilled to be present for her baptism, a first for them as our other children were baptized in Jordan. Had Tayta's surgery happened on the earlier, "preferred" date, she would have missed family camp altogether.

On August 14, Tayta and I flew into Madison, Wisconsin, in preparation for her August 15th surgery. We were transported to what would become our perfect home-away-from-home in Madison, the Ronald McDonald House, by a RMH volunteer.

 

The Ronald McDonald House, which provides housing and services for out-of-town families with children in the hospital, is a wonderful community charity. In part supported by McDonald's, it is also funded by many local businesses and run by volunteers as well as paid staff. The RMH provided us with very comfortable accommodations just a couple of blocks from the hospital, and volunteers from the community brought in a home-cooked dinner each evening. We were thankful that they had room for us the three times we needed a place to stay: the night before surgery, the five days after surgery and release from the hospital, and a final night before Tayta's second post-op check-up. The house had full occupancy each night we were there.

Tayta and I decided that it was better that we had to travel away from home for her surgery as her recovery time at the RMH passed peacefully and comfortably, and there were no distractions for me. I was able to give my full attention to Tayta and her recovery needs. I'm not sure I can say enough good things about the Ronald McDonald House and its staff and volunteers!

Nor can I say enough good things about all the kind and competent health care workers who cared for Tayta during her surgery and hospital stay. We were very impressed with all the staff at The American Family Children's Hospital, where Tayta had her surgery...

 

...though I have to admit, Tayta and I were very thankful to be released as soon as possible. Once the tubes and monitors are no longer necessary, hospitals, no matter how nice, are just not the most relaxing and comfortable places to recover. Not to mention the poor food choices for someone who must sip through a straw. 

And now for the post-op/recovery pictures: below is Tatya in PediatricICU soon after her surgery. They provided a couch/bed for me so that I could stay with her at night. I thought the room pretty cheery for ICU.

The nursing staff was great and tried to let Tayta rest as peacefully as possible, despite needing to regulalry check her vitals and test the live tissue connection with a Dopler instrument.

Tayta was glad to move to a gliding rocker  the next day and,below, is how she looked for two days following her surgery: sad and pitiful. Her pain was minimal (the nerves in her face were severed during the surgery but will slowly regenerate) but she experienced discomfort from the swelling, which peaked a few days post-surgery, and from the tissue donor site under her left arm. She's sipping a chocolate milkshake. The first one was good, the fourth and fifth, not so much.

Two days post-surgery and still no smiles from Tayta. She is still looking pretty sad and pitiful but says she is fine. I made this picture smaller for any squeamish readers. Tayta wanted me to include it, but then she likes dissections and is thinking of pursuing nursing studies. For my readers with medical interest, or just curiosity, since many have asked, the head drain is for the one incision the surgeon made on Tayta's head, just in front of her left ear.(She had a 6 inch incision under her arm/back as it was the donor site for the tissue transplant). The yellow "popcorn" sutures held the transplanted tissue in place. Medical wonders, for sure!

Life suddenly improved when Tayta could leave the hospital, wear her glasses, and manage at least a half smile.

Five days post-op

At one week, Tayta's surgeon removed the sutures and gauze from her face, along with her head drain. Her smile is  growing.

 Dr. John Siebert, Tayta, and Dr. Seibert's PA

The pictures below were taken three weeks post-op, just before flying back to Jordan. As you can see, Tayta's kept her sense of humor through all this, an important quality for coping with looking "ridiculous" for some weeks. I like her spunk.

Two days ago,  back in Jordan, I took these pictures of Tayta, now four weeks post-op. The swelling continues to slowly subside, her skin tone is returning to normal...

...and best of all, her smile is back in full force. It will be weeks, even months, until the swelling subsides completely. Tayta will need a second, minor revision surgery anytime after six months from her first surgery. In this outpatient surgery the surgeon will correct the atrophy in her left lower lip, sculpt any residual tissue bulkiness from her left cheek/chin, and tuck the scar just inside her ear. Officially, there is no cure for PRS, however Dr. Seibert's patients have not experienced further tissue loss after undergoing this free-flap tissue transplant surgery. This is our hope and prayer for Tayta. Again, I say, medical wonders! And again, I give thanks to God for all the goodness with which he has blessed Tayta and our family as we've dealt with this disease and surgery. 

We are so very grateful for all the people who have prayed for and offered words of encouragement to Tayta as she underwent surgery, and now as she recovers. Thank you!

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.