Sophia with her "melt your heart" smile 9/26/01

Name: Sophia Maria Litynski

Form of EB: Junctional probable Herlitz

Born: May 7, 2001

Died: February 9, 2002

About Sophia:

     On Monday night May 7, 2001 at 9:52 PM one of the most beautiful children in the world entered this earth weighing in at 7 pounds 13 ounces. After a seemingly normal pregnancy and much anticipation Sophia Maria Litynski blessed us with her presence and officially became a member of our little family.
Sophia Maria just moments after birth and Mama protecting as she would her whole short life 5/7/01

When the DR held her up and pronounced that we had a little girl my heart overflowed with joy and love for this beautiful baby. Our 2 1/2 year old daughter Amelia was certain the whole pregnancy that she was having a baby sister, and her dream came true.

   Sophia was so gorgeous and took our hearts the moment we saw her. However, immediately after birth the doctor and nurses immediately noticed that something was not "right" with her. She had open blisters on all of her fingernails, a few of her toenails, and open wounds on the back of her wrists. She had her umbilical cord wrapped around her neck, which luckily caused her no damage and waited a little while before taking her first breath with a little assistance from oxygen. While the nurses were washing her up I looked across the room and saw her open fingertips and asked what was wrong and no one had an answer but said they would have a specialist come up later and have a look at her. That night Sophia and I slept together in bed snuggling as tight as we could and who would have known that from that moment on our lives had changed forever as we knew it.

     The following morning her pediatrician came to the hospital for her first checkup and after looking her over commented that he thought she may have Epidermolysis Bullosa (EB), but would need to send us to a specialist to confirm. I had no idea what this term meant and how it would turn our world upside down with the battle we would face for our beautiful daughter's life.

So instead of a typical happy homecoming, 36 hours after Sophia's birth we were discharged and sent over to Children's Hospital of Milwaukee to meet with a team of ermatologists.

     Soon it was confirmed that yes indeed she had this dreaded disease, but the severity of it would be unknown until a skin biopsy was complete. They took her biopsy, provided us with some EB literature and informed us that in a worst case scenario the disease would attack her respiratory system and take her life. From that instant I felt like a deer in headlights and could not believe what I was hearing. I grabbed her and hugged her as tight as I could and stated that a disease I had never heard of was not going to take my child and we would fight this disease with every ounce of our strength and energy. How could this happen to my daughter? I had Amelia and she was perfectly healthy and my husband and I knew of no one in the family history that had ever had this genetic disease, let alone heard of it.       

     Sophia's first month was pretty wonderful. She had gained almost 2 pounds and had only a few new blisters here and there, but nothing too severe. She was a very alert and attentive little girl and was already tracking things at just a few days old. She loved to be held, kissed, and cuddled; she was a very calm baby and so sweet. She loved to be around her big sister and would instantly stop crying the second she heard Amelia's voice and turn to whatever direction she heard her. On the 12th of June I had to take her to the pediatrician because she had a cold for a few days and was just not feeling "right." She was a little congested and was having a hard time eating and breathing at the same time and would end up spitting and coughing a few seconds after she ate.

Sophia 1 1/2 months old and starting to lose weight and show signs of anemia 6/24/01
She had lost 1/2 pound, which I was not surprised to hear, but everything else seemed okay. Her lungs sounded clear and she had been running no fever. So I had her sleep sitting up in her car seat or elevated in her crib, the same thing that I had done for her older sister as an infant, but not knowing all the while that this was the beginning of the relentless battle against this evil disease. Two weeks later we went back to the doctor because she was still not gaining weight, congested and coughing after her feeds. We tried changing her nipples on the bottles, increased her caloric intake and monitored her weight for the next three weeks.     

      On July 22, 2001 we had Sophia christened and she looked like a sweet and precious Angel in her christening gown. She was so beautiful and we were all so in love with her. I knew then that something dreadful was coming and not really sure what it was. Eight days later we had to take Sophia to the emergency room because she was so lethargic and not eating anything and had this horrible cough that just did not sound "right." The staff ran some chest x-rays on her and noticed that her right upper lobe on her lung was collapsed and said that it was possible pneumonia. Sophia was instantly admitted and iv antibiotics were started. My husband and I were almost relieved to hear that it could be something "fixable" and thought everything would be better in a few days.

     The next morning the infectious disease specialist came in and explained to us that he felt it was not pneumonia, but rather aspiration and that she may need a feeding tube (g-tube) placed to help her. he next week in the hospital was so tiring and emotional and every question led to another test which led to more decisions for Sophia. By the end of the week it was decided after a barium swallow study that she had severe aspiration and was burning her calories faster than she could swallow them. After discussing it at length with her specialists we decided to have her g-tube placed three days later.    

  We scheduled a care conference with all of her specialists and reviewed at length how Sophia's tube was to be placed in the least evasive way to prevent further skin and internal damage based on her fragility and current condition. We stressed how Sophia was a very precious child and that there was no room for error with her. When the staff wheeled her away for surgery I was a mess; for the first and only time Sophia was out of my control and this drove me crazy. We were Sophia's number one advocates and for the next few hours we had to trust the doctors and pray that all would go smoothly for Sophia.

     Sophia came through surgery okay, a few extra blisters here and there, but overall all went well. They wheeled her in her room and I ran next to her bed and she opened her eyes for a second, looked at me and smiled as if to tell me that things were going to be okay. Oh, how I loved to look into those gorgeous eyes of hers. Her eyes were the window to so much and spoke volumes. Everyone who met her always commented on how beautiful her eyes were.

Sophia after her first formal pictures in her christening gown 9/6/01, 1 month after g-tube placed

     After a few days of observation we went home and tried to have as "normal" of a life as we could again. Sophia started to gain weight slowly and was trying her hardest to regain the strength that she once had. She had her first three teeth cut through on the 19th of September and was a champ! She smiled that morning as if she was trying to show them off and was starting again to grab her toys and have a little extra energy.

     We were very optimistic even though it had been confirmed that she was diagnosed with Junctional EB after a second biopsy and just loved her with every breath that we had. She had even started to coo and would carry on numerous "conversations" with anyone that would listen. She was a very content and happy baby despite all that she was dealing with. Sophia endured quite a bit of damage during her hospital stay and as hard as we tried to heal her the more her skin just seem to break down.

     Late in the evening on September 22 I heard Sophia "breathing" in a very loud and unusual fashion and ran to her bedside to find her struggling for air. This terrified me to the bone and I awoke my husband to call 911 and explain to them Sophia's disease and the problems she was enduring. Within minuets two ambulances, two police units and plenty of lights in the middle of the night arrived at our home. Before the medical technicians could even touch her I had to explain as quickly as I could what EB was and what could/could not be done to Sophia. I was so scared and shaking as I held her and tried to help calm her through her tough bouts.    

     Through it all Sophia always continued to amaze me. She remained so calm and just looked at me as if to tell me she was trying to work through it and not worry me. She will always be one of the most amazing people I will ever meet and am so proud to be known as her Mother. We went on to the emergency room and this time it was thought that she had croup due to her breathing condition and her positive response to steroid treatments. We were sent home after explaining very firmly that the hospital was the worst place for Sophia due to potential infection and comfort issues; the staff agreed and discharged us eight hours after admission.

     The next month went okay. Sophia's breathing was a constant up and down and on the 21st of October we were once again admitted to PICU for a few days with no official diagnosis except for a guess that this hideous disease had entered our daughter's airway. Two days after being discharged from the hospital we were back again in the middle of the night and Sophia was fighting her hardest to just relax and work through whatever was annoying her airway. Her ENT doctor confirmed by x-rays that her airway had narrowed and we needed to discuss the option of a tachometry.

     I was once again heartbroken that no matter what we did and how hard we fought this hideous disease it did not care and was laughing at us as we tried our hardest to save our sweet little Sophia.

Sophia 5 months old so proud to be in her high chair and out of pajamas! 10/3/01

The next few days were a constant extreme between down and further down. My husband and I, along with her specialists, discussed at length what would be the best decision for Sophia. I wanted to help her in every way that we could, but after praying and discussing we opted that a tachometry was not the best thing for Sophia. We all knew that Sophia was very weak and fragile to begin with and just had a really bad feeling that she would not make it through the surgery.

     There were many rough nights and many hours of crying over why this was attacking such a sweet little Angel and all we could do was love her and make her as comfortable as possible. Infections continuously attacked her both internally and externally. If we thought we had cleared an infection up it was only for a short time and would reappear a few days later stronger and more immune to our current antibiotic schedules. We tried numerous antibiotics and were admitted a few times to the hospital to help clear things up, but as soon as one aspect of this disease was being treated there were four-five evils waiting in the wings laughing at us.

     Sophia was so strong through everything and fought as hard as she did because she knew she was so loved and so much a part of our lives. Amelia told me that she had an idea on how to help Sophia - we should put Sophia back in my tummy where she was safe. Oh how I would have jumped at such an option. Amelia was so amazing with her and loved her with a passion that is indescribable. She was her protector and the sister's shared a bond that I will never be able to express in words. Sophia loved to hear her sister and Amelia loved to help her sister.

     December was another tough month and all we could do was keep ourselves focused on Sophia's comfort. Her doctors, nurses, and specialists were so wonderful and loving with her and our family. They always listened to what we wanted and helped us work through tough issues. We tried numerous blood transfusions to help bring up Sophia's iron levels, but as soon as we brought it up it started its slide down.

We developed numerous pain medication schedules to keep her comfortable and it seemed that every week her pain elevated and we increased her morphine and methadone intake to keep her comfortable.

     EB is evil and not only did it attack our daughters skin and her internal organs, but it also attempted to strip her of her beauty and cheerful personality. EB robbed her of so much and the only thing that we could "control" was her pain discomfort. She use to have the brightest look in her eyes and all you could see now was discomfort and sadness. Sophia could no longer smile and was sleeping more hours in a day than awake and it wasn't from her medications, but from the exhaustion of fighting and trying to live with EB.

     We made it through the holidays and I was so thankful that Sophia was there to share them with us. She tried her hardest to be happy and would never complain. Diaper changers were now a 45 minute procedure, bath time was 2 1/2 hours long, and just picking her up to love her was a very slow action. Her skin was over 85% broken down and shedding off whenever healing tried to start. You never saw her get mad or cry over all of this though, she would just look at me with intense concentration in those big beautiful eyes and try her hardest not to cry. She was being brave for me - what an amazing little girl.     

This is New Years Day 2002, Sophia is almost 8 months old and sitting in her favorite position on Mama's shoulder. What a sweet Angel! 1/1/02

 The last week in January I knew that Sophia's condition was somehow turning worse. She now had various infections and was on antibiotics for three weeks and things were moving no where in the positive. She was sleeping more and just not herself. On the 29th of January we had our Priest perform confirmation rights with Sophia and say a few prayers with us; I could not believe it had come to this. We fought so hard for her and did all that we could for her, but EB did not care, it just continued to torture my beautiful little girl.

     The morning of February 9, 2002, was like any other morning. Sophia stayed up a little longer in the morning before her nap and was taking in so much of Amelia talking to her. I laid her down for a nap around noon and kissed her and hugged her and told my Angel that I loved her and was so proud of her. I set her feeding machine up while she fell asleep and left her home with my husband so that Amelia and I could go out for a few hours for the first time in months.

     Her feeding was complete about an hour later and Ernie ran in her room to turn off the beeping alarm so it would not wake her up. As he was shutting the machine off she awoke for a second and looked at him like she always did when she heard someone in the room and immediately went back to sleep. A few minutes later I returned home and ran upstairs to check on Sophia and as I entered her doorway I knew at that moment she was gone. She looked so gorgeous and was the most relaxed I had ever seen her. I ran to her and picked her up and hugged her so tight and kissed her all over, she hurt no more and no one could hurt her ever again.

     Her pediatrician and home nurse, as promised months before, immediately came over to confirm her passing. We all held her and hugged her and thanked God for easing her pain and struggle and taking her while she slept. We made clay prints of her hands and feet and kept her at home with us for a few hours to just love her and say our good-byes to her. Amelia held her and said to me "Look Mama she's not crying anymore when I hold her."

     Sophia was and always will be the bravest person I have ever known. She was and always will be my second born and baby girl. She had the most incredible eyes and sweetest smile. Anyone that got to meet her or know her was lucky. EB is rare, but a child as sweet and beautiful as Sophia is even more rare. She was loved intensely and taught us and so many others so much about life. The world is a much better place after Sophia's presence. We will forever love and miss her and know that she knew we will forever be proud of her and her fight was so intense because she was loved so much.      

As I sit here four months later and reflect on all that has happened in the past year I am so thankful for Sophia and all that she shared with us. There isn't a day that goes by that we don't talk about her or think of her. She will always be a part of our family and Amelia's baby sister. Although Sophia went through so much I know that her life with us was happy because we loved her so much and did everything for her and not our own selfish reasons.

Sophia our sleeping beauty 2/4/02 9 months old

I take solace in knowing that one day we will be joined together in heaven and look forward to that joyful reunion.

We have set up a fund in Sophia's name at Thomas Jefferson University to help support DNA testing in DR Pfender's lab. This testing helps to identify the gene that carries the mutation. If you are interested in helping support our cause please make your donation to:
Sophia Litynski Fund
and mail to:
Jefferson Development Office
Suite 100
925 Chestnut Street
Philadelphia, PA 19107-4216

The whole family 1 day before another hospital stay. Sophia was 7 months old. I am so happy that we had a family picture taken and I knew at that time I had just created a memory that I would cherish forever with Sophia.

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