Resting Place?

On Sunday afternoon we visited Leah’s grave. We had been at a church retreat about a half-hour from Waukesha, so the timing seemed right to go. As soon as we left the retreat grounds and began our journey I felt enveloped by a wave of pain and sadness. We got a little bit lost on the way there (thanks Wisconsin for the lame detour signs), and it looked like it would start raining at any time, However, much like the day that we buried Leah, by the time we arrived at the cemetery the sun was shining warmly from a clear blue sky.

My biggest fear was quickly allayed when I saw that her headstone turned out perfectly. They spelled everything right and we loved how the flower and cross design turned out.

I could see the lines in the dirt from where they cut the sod and replaced it after they buried her. The grass hasn’t grown enough to cover the scars in the earth.

I cleared the dead leaves from her grave. I know the wind will cover it back up soon, but I didn’t want to leave it cluttered.

It was hard to be there, and weird. It was such a contrast from the last time we were there, for her funeral. This time it was just me and Jeff. I think we may have been the only people in the whole place. Where there was once a tent filled with many friends and family, there were now only leaves. It was very quiet, calm, even beautiful, but not peaceful.

There is no peace in my baby being in the ground.



Philippians 3:20-21 (New Living Translation)

²⁰ But we are citizens of heaven, where the Lord Jesus Christ lives. And we are eagerly waiting for him to return as our Savior. ²¹ He will take our weak mortal bodies and change them into glorious bodies like his own, using the same power with which he will bring everything under his control.

Installed

Tomorrow we will be going to Waukesha to visit Leah’s grave. We recently received notice that her headstone was installed, so we want to see that too (it was actually more of a bill than a notification, but we got the information we wanted).

I am apprehensive about going. It has been a hard week. This week marked three months since Leah’s birth, and we are fast approaching her original due date. Although many good things have been happening lately that have lifted our spirits, we continue to mourn our precious baby. Please pray for us as we make our pilgrimage.

I know that it is only Leah’s body there in the ground. She is in the arms of her Heavenly Father. Nevertheless it still hurts to think about her little body in that tiny casketin the ground, when I want her in my arms. She should be in my arms.

Three Months Today

Mountains of Paperwork

Since having another child the old-fashioned way carries so many risks for us, we have decided to pursue adoption. We decided to get started now since adoptions take so long to complete, so I began doing some internet research. At first the results were very discouraging. The first agency I called wouldn’t consider us because of our income, so I gave up, assuming that all agencies would have the same standards. After a few friends encouraged me to keep looking into it I found an agency that was willing to take into account our imminent graduations, and therefore soon to be rising income (hopefully anyway), when considering us as candidates for adoption. We meet state requirements (125% of poverty level), so it is really a matter of convincing an agency that we will be able to meet a child’s needs and pay the adoption fees. It appears we may be succeeding on that front.

Today we met with a social worker from the agency we want to use for our home study (the certification from the state of Illinois). She was very helpful, and I think she will be great to work with. Now we have many forms to fill out, and decisions to make.

I also got a call from our international agency. As of right now it looks like we will be adopting from Uganda. We will have a better idea of that once the international agency can review our home study and make sure that we are good candidates from the perspective of the Ugandan government.

One nice thing about the length of the adoption process is that it will give us plenty of time to continue to grieve for Leah. I know that it will also be frustrating to wait, but I am working on trusting in God’s timing.

Breathing Room

This last week Jeff and I were in Estes Park, Colorado for the fifteenth International Workshop on Oxide Electronics. Jeff had to go to present a poster on his research, and I tagged along for fun. Needless to say, in order to have that fun I did not attend any part of the workshop except for the meals. Instead I slept in, read books, took walks, went on hikes, soaked in the hot tub, did a tour, and went to museums. The time away was really helpful for me. I feel like God really used the time to give me some space from my grief, and to give me strength to move forward.

As I hiked in Rocky Mountain National Park, amidst the grandeur of incredibly high mountains, pristine lakes, roaring rivers, and golden aspens, I felt God’s amazing care for me in my pain. Throughout this time Jeff and I have continuously felt his care through the love and provision of our community of friends and their support, but this was a different feeling. God directly created the Rockies, the rivers, the waterfalls, the lakes, the trees, the critters, the colors and the scents that comforted me. As I climbed the mountain I was struck by God’s perfect planning. He knew I would need this time and space to heal long before I did. When the IWOE planning committee met two years ago to choose a location, God already knew that I would need this. He knew the benefits before Jeff’s advisors told him he should go. I am so thankful for our time in Estes Park.

Things went well for Jeff too, and he claims that the workshop was very interesting. He got some good feedback on his research and made some important connections, including one that may lead to a collaboration in the future. He came back feeling excited about his work and energized to finish his project.

We didn’t have a chance to celebrate our anniversary this year (five years), as our anniversary occurred within a month of Leah’s birth and I was not up for going anywhere at that point. So we took an extra day in Estes Park to go hiking and sight-seeing together. It was fun to celebrate and just enjoy each other’s company.

Mom's Journal

My Mom wrote down her perspective of our experiences, and she recently sent them my way. I thought I would share her view point with all of you.

Tuesday morning Amy called about 6:30. She was dilated to 7. I dressed and Bill dropped me off. The Cook catheter did its job and she was dilated to 7. She was not fully effaced yet. The doctor came in and gave the epidural early because of the special circumstances. The pitocin and the epidural were begun. The process speed up a bit and Amy was in no pain and still very cognizant. When she was ready, the doctor was in a C-section so we waited until 2:00. Then they began to prepare everything. Amy’s medication had been lightened up bit so could feel to push. With two or three pushes Leah was born.

She moved her arms a little and opened one eye.

She was beautiful, not perfect but beautiful. She had 7 fingers on one hand, 6 toes on one foot. One foot was perfect with the little crease line on the bottom and pudgy. She had new baby color and her heart beat for about 45 minutes. She was a fighter. Her head was perfect. She weighed 1 pound 6 oz and was 9 and ½ inches long. Her hands were not formed well and her arms were short. Her legs were short but had great form.

The last nurse we had was Sarah. She said she was an Orthodox Jew. She told me this when I had asked to carry Leah to the room where she would wait to be taken for the autopsy. She said that being kind to her (Leah) in death was the last great thing you could do for a person.

I placed Leah in a nice white bag and she zipped her and put her in the refrigerator. She was wrapped in her cute white, blue and pink hospital baby blanket and I carefully placed her in the bag. I got to carry her down the hallway cradled in my arms. I said goodbye and kissed her one last time and went back to take care of my very first baby, Amy. My Amy who in spite of a horrific experience was brave and gave the gift of life to Leah and an opportunity to Jeff to hold his little girl.

On our nurses:
Lori was our first nurse who stayed with us all day and who was our nurse the full second day. When I told her that she was a gift from God to us she teared up and said “Thank you and I needed to hear that” She said she would be praying for all of us.

I get the sense that this young woman was sensing the same presence of God that we all knew was surrounding us.

Amanda the night nurse very talkative. She came in all night and checked on Amy as she slept. When I thanked her the morning of Leah’s birth, She said ”that she had attended many difficult births but she felt a spirit in the experience that was amazing. She said ”All the nurses at the station were talking about it.”

Confirmed: Short-rib Polydactyly

Last week we got the call from our geneticist saying that Cedars confirmed our diagnosis of Short-rib Polydactyly Syndrome (SRPS). I was really surprised to hear from her so soon, because we were originally told that confirmation would take 3 to 6 months. Finding out that the experts agreed with the diagnosis of SRPS really upset me, which took me by surprise because I thought that I had accepted the original diagnosis. I didn’t realize that I still had hope that they were wrong.

The doctors at Cedars also assigned us a type, Type 1- Saldino-Noonan. The medical literature on SRPS debates the accuracy of typing cases because SRPS has so many possible variations. Right now doctors have identified four types of SRPS, and each case is assigned to one of these sub-categories. However, many of the case studies (which is what most of the medical literature consists of) demonstrate that often the babies diagnosed with SRPS don't fit very well in any of the types. This leads scientists to believe that SRPS might have a spectrum of expression.

If there are indeed types, and SRPS is caused by different variations in whatever gene it effects, then Jeff and I could expect future pregnancies affected by SRPS to work out very similarly to Leah's. We would expect our gene combination to result in many of the same problems next time around. The timeline on the pregnancy might differ, based on the progression of the hydrops, but we would expect to see the rest of her problems repeated.

However, if SRPS has a spectrum of expression then we would have no idea what to expect. Not all babies with SRPS have cystic kidneys that cause oligohydramnios (lack of fluid), in fact often skeletal dysplasia results in hydramnios (an excess of fluid). SRPS babies can have other internal anomalies, as well as cleft lips and palates, ear deformities, and sex reversal. Some babies with SRPS even have a normal number of fingers and toes, although most have extra. No babies with SRPS survive.

We are hoping that they discover that typing is accurate, although it appears that the consensus is leaning towards spectrum. I would rather have a defined set of things to expect should we choose to get pregnant again, especially now that I know how many bad things can happen with SRPS, thanks to all my research.

I mentioned earlier that Cedars gave us our diagnosis more quickly than we expected. Apparently they worked to get it done quickly because they are actively researching SRPS right now. They believe they are very close to identifying the gene, in fact it may be only months before they find it. Jeff and I have been accepted into their research study, so soon we will be signing more consent forms and sending them our blood.

Scrapbooking is a Man's Work

After Leah's funeral, and when all the family except Amy's mom went back home to California, one of the first things we started working on was a scrapbook for Leah. Now, neither of us is in to scrapbooking, really at all, but we both felt that it was important, not just for our grieving process, but that it was something we needed to give to our daughter. Leah deserved it.

Amy and her mom spent two entire days driving all over the north shore picking up all sorts of scrapbooking supplies from the very basic necessities (e.g. fancy scissors, paper) to the more baby-girl-centric stickers and such, and of course an appropriately pink album. We printed out copies of our favorite snapshots from the time when the pregnancy was a secret between the two of us to the reception following Leah's memorial service. It would be some time before we had our pictures from Now I Lay Me Down to Sleep, but with our personal photos printed out we set to work.

We started on July 4^th and worked on Leah's scrapbook all long weekend while parked in front of the TV watching Prime Suspect, the Inspector Lynley Mysteries, a few movies I don't recall, and various other British television series all checked out from the Evanston library. Amy asserts that in this initial period, which I will call Scrapbooking Phase 1 or SP1, she became "the scrapbooking queen," a true feat since she has always loathed the activity. In my recollection, however, we both worked at more or less the same pace. We would each work on a separate page simultaneously, although we both contributed to the artistic direction of all of the pages.

Activity during SP1 naturally slowed somewhat during the week, as I had gone back to work at that point, but we still spent several hours each evening working on Leah's scrapbook. We continued steadily putting together pages with our snapshots (as well as other items such as Amy's and Leah's hospital bracelets, Melody's letter, and the program from Leah's memorial service) until we ran out of photos to work with. At that point we decided to put scrapbooking on hold until we received our Now I Lay Me Down to Sleep photos.

It has been almost a month since we got our NILMDTS photos from Deanna, and a little over a week since we started Scrapbooking Phase 2, or SP2 (there was some out-of-state travel in between). A strange thing has happened between SP1 and SP2. It turns out that my meticulous nature lends itself to scrapbooking quite well. A little too well perhaps, as somehow, somewhere along the way I became the primary scrapbooker in our family. Amy assures me that if not for her loathing of scrapbooking she would not let my meticulousness get between her and working on the scrapbook. As it stands, she is happy to give artistic input and leave the cutting, pasting, and more cutting and pasting to me. In the Klug family, scrapbooking is a man’s work.

Information for Our Relatives

Now that Jeff and I have been identified as carriers of a lethal genetic mutation, you may be wondering what this means about your genes. Some of you will also be carriers of this gene. At this point we have no information about which side of our family the gene came from, and currently no test exists to identify carriers. So what are the odds of you carrying the gene?

If one of our mutual grandparents was the carrier, then your parent has a 50% chance of having the gene. If they did inherit it, then there is a 50% chance that they passed it to you. If you inherited it, there is a 50% chance you will pass it on to your children. Because the gene is recessive, it will not affect you or your carrier children. The only way it becomes a problem is if you or your children marry someone who also carries the gene. If both parents carry the gene there is a 25% chance, for each pregnancy, of having a child born with short-rib polydactyly.

There is no way to tell if you carry the gene. However, even if you do it is very unlikely that you will marry another carrier, provided you avoid marrying your relatives, which you should probably do anyway. Research is being done at Cedars-Sinai Medical Center to identify the gene for short-rib polydactyly. We have been told that it is one of their most active research endeavors, and that they expect to identify the gene within five years, but possibly much sooner. At that point my parents will be tested to find out which one of them carries the gene. Based on that we can determine which side of the family this came from, and those who wish to can undergo testing.

Until we know which type of short-rib poydactyly Leah had, it is impossible to know the incidence rate and the rate of carriers in the general population. Our genetic counselor has indicated to us that all types are extremely rare, and that it is possible that outside of our relatives we do not know any other carriers. When we know more we will pass on the information.