More Courage Through Sharing

For the monthly blog carnival, Finding Courage Through Sharing, the carnival host Michelle gave a topic of sharing something that you wish other people understood about your life with a child or children facing medical challenges. Well as a parent to two former premature babies who also happen to have a liver genetic disorder called Alpha-1 Antitrypsin Deficiency, I could go on and on about this subject. Nonetheless, I decided to focus on their former prematurity for this entry.

Prematurity does not end when a baby is discharged from the neonatal intensive care unit (NICU). Preemies do not catch up by the time they are 2 or 3…perhaps in size, but not in the way their brains have been shaped. Prematurity is life long. Premature babies have brains which are wired differently that full term children. This is because they have to deal with sensory input long before their brains are ready to deal with the information they are processing after birth. You may have heard about NICUs that keep the lights down low, encourage a quiet tone of voice, and do such things as placing bags of IV saline next to the babies to make it feel like they are in the womb. While I applaud these efforts, they aren’t the same as a good, old fashioned womb.

There are many sources of misinformation to contend with as a parent of two former preemies such as urban legends, media, or your neighbor down the street. Former preemies are not the same or “just like” their full term counterparts.

Both Grace and Meghan contend with the invisible scars left from prematurity. As their mommy, my job is to help them with learning coping strategies for their preemie battle scars.

Grace’s early birth brought on sensory processing disorder. While some of her sensory seeking behaviors may be very appropriate for her age group, Grace’s go further. How many six year olds do you know that actively still seek out exploring the world using their mouths? Poor dear husband, Charlie, was on the receiving end of a juicy lick to the upper arm the other day. She also is a bit like Dino from the Flintstones. When she runs up to you, she doesn’t slow on her approach. She comes on in ramming speed motion, and has knocked me over several different times. Then, there is the ever-so-fun need to approach you to say something, and then without warning, jump head first into your mother’s or father’s chin. Ouch!


Grace needs a very active sensory diet through which she can subdue her need to touch, feel, and experience things through her senses. My husband should have gotten the “husband of the year” award last year when he brought home a swing set complete with a slide. Grace needs to swing, run, jump, and play like an addict needs his/her next fix. This activity calms and soothes her brain’s needs for sensory input. Yes, I do know that this sounds like an average case of attention deficit disorder, but it is not. Grace can pay attention for hours if she wants.


Meghan’s early birth provided her with general low tone throughout her body. For many years, we were in the “watch and wait” dance to determine if Meghan had a mild case of cerebral palsy. The left side of her body actively “drooped” compared to her right side. Many, many sessions of physical therapy later, her strength has improved. Hallelujah! We were even told that the cerebral palsy issue was resolved. It is another reason for us to rejoice. In any case, I do pay homage to Grace’s contribution to Meghan’s low muscle tone and poor reflexes. Since Grace is so sensory seeking, Meghan now has lightning fast reflexes. She has to. Grace will literally bowl Meghan over otherwise.


Prematurity has consequences that can’t be erased or ignored. Many individuals do not understand this. Now that I’ve said this, I want to make it crystal clear that I do consider my children to be miracles, and I wouldn’t change them. They are my heart. They are my pride and joy. They have boundless love, and I return that to them everyday.

Yes, preemies are medical miracles, but you should not expect any preemie to come away from their early birth unscathed. Prematurity leaves battles scars. Some that can be seen, and many others that are unseen. My daughters have some physical scars, but the majority are unseen scars.

I’ll leave you with a final picture of my beauties.

Here I Am

I finally took a new picture of myself. I'm not overly fond of having photo shoots, but the Alpha-1 Association wanted a picture of me for an upcoming conference. I took these myself in my dining room. haha

Yep, you can laugh at me

...but I was so excited that David Cook won American Idol. I hope to buy his CD someday.

Appointment Changed

Grace's ENT appointment on Friday was canceled. I'm struggling with the wait, but Grace is quite unaware, which is good.

We've got a new appointment for 4:30pm next Tuesday.

The cyst now appears to be slightly larger, but nothing shocking. C'mon Tuesday! Get here soon!

This mama needs some answers.

Celebrate!

Alpha-1 Foundation, Association Celebrate GINA signing; Plans Under Way to Discuss Implications, Opportunities for Alpha-1 Detection

MIAMI – The Alpha-1 Foundation and Alpha-1 Association today applauded President Bush’s signing into law the Genetic Information Nondiscrimination Act (GINA), and began making plans to take action based on the new federal legal protections.

Under GINA, companies would no longer be able to use genetic information such as a person's predisposition for lung disease, breast cancer, sickle cell or diabetes to make insurance or job decisions.

“Our community has been involved for more than a decade in the effort to bring this essential legislation into law,” said John W. Walsh, President and CEO of the Alpha-1 Foundation and President of the COPD Foundation. “We’re all celebrating a giant achievement today.”

According to Jan Petersen, Chair of the Alpha-1 Association, “the Alpha-1 community has played a significant role in advocating for the passage of this legislation and now celebrates the progress we have made. I thank all of those in the Alpha-1 Community who helped us to get to this point."

“Great news on a hard-fought battle,” said Greg Hules, Board member and Immediate Past Chair of the Alpha-1 Foundation. “The efforts of the many people involved will make a huge difference in the quality of life of those affected with COPD. You know you’re alive when you have the ability to make a positive difference in this world.”

Dennis Pollock, an Alpha-1 Association Support Group Leader from Oklahoma, was at the White House to represent the Alpha-1 community as President Bush signed GINA into law.

The Foundation’s Ethical, Legal and Social Issues Working Group, with representation from its Medical and Scientific Advisory Committee, will be meeting in the next two months to discuss the implications and opportunities that this legislation may present for Alpha-1 Detection, said Marcia Ritchie, Foundation Vice President and Chief Operating Officer.

The working group will also discuss the development of a birth screening workshop to be held in the fall of 2008. This will be a follow-up to the Foundation’s Gordon L. Snider Critical Issues Workshop Series No. 8, “The Impact of Genetic Testing: Ethical, Legal, and Social Issues”, held in October, 2000.

Partners with the Foundation and Association on the initiative to have GINA become law included the Congressional COPD Caucus, co-chaired by Sens. Mike Crapo, Blanche Lincoln and Reps. Cliff Stearns and John Lewis; the American Association for Respiratory Care; and the Genetic Alliance.

The House voted 414-1 for the legislation a week after it passed the Senate on a 95-0 vote. The bill would bar health insurance companies from using genetic information to set premiums or determine enrollment eligibility. Similarly, employers could not use genetic information in hiring, firing or promotion decisions.

Each person probably has six or more genetic mutations that place them at risk for some disease, according to the National Human Genome Research Institute. That does not means that a disease will develop, researchers said, just that the person is more likely to get it than someone without the genetic mutation.

Congressional efforts to set federal standards to protect people from genetic discrimination go back more than a decade, to a time when there were only a small number of genetic tests.

But now, with the mapping of the human genome in 2003, people have access to far more information about their hereditary disposition to potentially life-threatening afflictions such as Alpha-1, cystic fibrosis, Huntington's disease or Lou Gehrig's disease.

# # #

About the Alpha-1 Foundation:
The mission of the Alpha-1 Foundation is to provide the leadership and resources that will result in increased research, improved health, worldwide detection, and a cure for Alpha-1 Antitrypsin Deficiency. For more information, please visit: www.alphaone.org.

About the Alpha-1 Association:
The mission of the Alpha-1 Association is to identify those affected by Alpha-1 Antitrypsin Deficiency (Alpha-1) and to improve the quality of their lives through support, education, advocacy, and research. For more information, please visit: www.alpha1.org.

Genetic Information Nondiscrimination Act will become law tomorrow!

President Bush will sign the Genetic Information Nondiscrimination Act (GINA) into law tomorrow. One of my Alpha-1 pals, Dennis, who actively lobbied for this historic legislation will be in Washington D.C. to celebrate the day!

Woohoo!

Grace's Lump

We're visiting a ear, nose, and throat specialist next Friday at 1:30pm at our local Children's Hospital.

Pictures of Grace's lump:

Happy 60th Dad!

Gracie Has a Lump

Two days ago, Grace was sitting at the dinner table and I suddenly noticed that she had what appeared to be a lump in the same place a male's adam's apple would be. She has never had that before so I asked her if it hurt. "Nope. What lump Mommy?"

It isn't red or inflamed, which is good. The thing is the size of an aggie marble, and is becoming more noticeable now. I took her to the pedi today for her 6 year old checkup and to show him. He wants us to see an ENT as soon as possible.

He was trying to talk to me about it, but Grace & Meghan were in the room so I could tell he was trying to choose his words carefully. Essentially, he thinks that an ENT will want to do some imaging, potential biopsy, and he suggested removal. Of course, I googled and that made me feel worse. It could be as simple as a fluid filled cyst...really it could. It could go away on its own. We might just watch it. Yada yada yada. I'm trying not to freak out about the worst case scenario here. The pedi wasn't alarmist, but he seemed to convey a genuine amount of concern, while managing to not alarm Grace or Meghan.

So I guess I'm going to have to "look up" my old friend Denial for the time being.

And, I'm going to ask for prayers and positive thoughts. Gracie is angry that she has to go to yet another doctor for a different reason, but she was on cloud 9 because she came away from a doctor's visit without any shots or tests. I suppose that is something for which to be thankful.

Thanks,
Jen

In the Home Stretch

The following article will appear in an upcoming issue of the Alpha-1 News. Please enjoy this advance copy. Alpha-1 Association copyrights apply.

On two recent beautiful spring days, the "Ayes" went up against the "Nays." On their home fields, fans all around the country waited to hear word. Some trolled the Internet for any bit of news. A few monitored C-SPAN to see it for themselves. Some witnessed the events in person. Others went about their day unaware of the goings on. This mother went about her normal work day, but remained distracted hoping for the future of all Alphas. Eventually word began to spread.

In Congressional Stadium, history unfolded as rookies and veterans alike took a swing at passing the Genetic Information Nondiscrimination Act, known simply as GINA. At home plate, each of them answered with an aye or nay. Gradually, nearly all hit a home run to enact this historic legislation. Cheers erupted from fans all around the U.S. The “Ayes” had won the pennant.

Thirteen years after its first introduction, GINA was passed by both the Senate on April 24th and the House on May 1st. We’re in the home stretch now. President Bush is expected hit a grand slam by signing GINA into law. The goal of GINA is to protect individuals against discrimination based on their genetic information when it comes to health insurance and employment. Alphas and those, who have yet to be diagnosed, will be able to enjoy new protections for health insurance and employment.

The hope of GINA is to protect all Americans by prohibiting insurers in both the group and individual health insurance market from 1) “requesting or requiring” genetic testing of an individual or his family or 2) using genetic information to determine eligibility or establish premiums. It further prohibits employers, including employment agencies and labor organizations, from 1) “requesting or requiring” genetic testing of an individual or his family or 2) using genetic information to make hiring or promotional decisions, or when determining eligibility for training programs.

My young Alphas, Gracie and Meghan, don't understand the significance of this historic legislation yet. What they know is they've spent their whole lives growing up with Alpha-1. They understand that support group meetings, blood tests, ultrasounds, examinations, and simple protections for their lung health are normal. They love their adult and child "Alpha friends" alike. They know no other reality. GINA is a beginning for them...a beginning free from a lifetime of possible genetic discrimination. GINA is a beginning for all Alphas…and all Americans. We still have work to do, but Alpha-1 Association members are united together. Go Team Alpha-1! We’re in the home stretch now.

Ouch

At the end of March, I came down with a very nasty respiratory flu. (Yes, I got the flu shot like I do every year.) You know the kind. The flu that makes your whole person hurt in the strangest of places and comes with a nasty initial high fever followed by an annoying, lingering low-grade fever. I was miserable for nearly a week with fevers.

After that, I dealt with the after effects of the flu including running nose and coughing. I believe it was the most I've coughed in my entire life within the span of three weeks. My lungs physically hurt and I went to the doctor twice for assistance with how horrible I was feeling.

Anyway, you may have noticed that I've not been posting as often. The flu was the reason. Unfortunately, I am still dealing with horrible side effects. Now, I have coughed my way into a nasty case of "rib pain" along with "chest wall pain." Last Thursday, it was so bad that I actually went to the ER to get some more effective pain management.

When asked to rate my pain, I answered with this: "Right now, it is a 9. When I cough, it is a ten." My doctor pretty much brushed me off and said, "Take Advil or Aleve." Well, my body just sort of laughed at the Advil and kept right on hurting and hurting and hurting, which is why I headed to the urgent care. Sigh.

In any case, the ER doctor was so kind and willing to listen. After several chest x-rays and a d dimer test for potential pulmonary embolism, she diagnosed me with the same thing my primary doctor had diagnosed. Unlike he, she provided me with hydrocodone for the pain and a genuine amount of attention to what I was actually telling her. :) The blessing in that drug is that it is an opiate, which interupts pain receptors plus it acts like a cough suppressant at the same time. Essentially, it knocked me out for nearly 2 days and then I lowered the dosage to 1 pill ever six hours.

Now, the pain is subsiding although still there with movement and coughing. I'm amazed by how much one little muscle between two ribs can reek so much havoc on my body. I also suffer from fibromyalgia, and this has not been good for my other muscles. I'll get through, but I hope and pray this particularly nasty muscle pain and spasm go away soon. The kind ER doctor told me that it could take a few weeks since it took weeks to create the issue.

I guess I'm feeling old lately, but hopefully with Spring in full bloom now in Wisconsin, I'll be back to my old self soon...at least the me that doesn't require narcotics for pain.

4 Years! Already?

Flashbacks

Four years ago, I was on full bedrest in our hospital's antepartum unit. It was day 8 of bedrest, and I had just passed my initial goal of making it to May 1st. We already had 2 April birthdays in our family, and I was hoping that there wouldn't be another arrival...an arrival way too soon for my liking.

My blood pressure was still climbing as severe preeclampsia was deepening its hold on me. I spent most of my time in a darkened room because I had had some uncontrolled twitching where my head jerked to my left side. My doctors didn't want me to have visitors except for my DH, and I couldn't have phone calls since the ringing would set me into twitching mode.

I was resolved to stick it out as long as I could, but I'll be honest. I was secretly hoping that the doctors would say, "it's time." It didn't happen that day 3 years ago, and yes, I did know that more time in utero is better. I knew that, but this was my 2nd horrible experience with severe preeclampisa. I was slowly losing my grip on my sanity as I stayed indoors in the dark, and this was day 8. My next goal was to make it to Cinco de Mayo, the 5th of May, and it looked like I might make it.

I had noticed that my baby was moving around less and less so I took it upon myself to poke him/her as much as I could to keep up the stimulation. This was beginning to bother me and the nurses were beginning to record decels on my non-stress tests and BPPs. My amniotic fluid was dropping off, and at the end of May 1st, 2004, I noticed that my vision was suddenly changing. Straight lines appeared squiggly to me, and I thought, "Oh no, it is happening again. This is what happened when I was pregnant with Gracie 2 years ago."

Gosh I was stressed out, but calm all at the same time. It was sort of a calm before the storm of Meghan's birth on May 3rd. Hind sight is always 20 20, but I hope that I did my best to take care of Miss Meghan that day.

Thank You

Click here to help me reach my goal!

Historic Day

I'm amazed. I'm proud. I'm relieved. I'm wondering what the future holds.

The Senate passed the Genetic Nondiscrimination Act (GINA) today. It has been years in the making, and it is finally here. I'm still absorbing all the possibilities that this presents. I'm on cloud 9.

My children won't face a future filled with genetic discrimination. Praise the Lord!

From the Genetic Alliance

FOR IMMEDIATE RELEASE

Senate Passes Historic Bill on Genetic Information Nondiscrimination
Americans Can Take Advantage of Health Advances without Fearing Discrimination

Washington DC– April 24, 2008 – With overwhelming support the Senate today passed by a vote of 95-0 the Genetic Information Nondiscrimination Act (S. 358). With sponsors, the bill is a testament to a strong bipartisan effort. The Coalition for Genetic Fairness commends the members of the Senate for its commitment to affording comprehensive protections against genetic discrimination.

The Genetic Information Nondiscrimination Act (GINA) paves the way for the responsible use of genetic information while protecting against discrimination with respect to health insurance and employment.

"We are grateful for the bipartisan efforts of our sponsors in the Senate – Senators Edward Kennedy (D—MA) and Olympia Snowe (R—ME) as well as the tremendous support of Senator Michael Enzi (R—WY). They are our champions and are making history today," said Sharon Terry, President and CEO of Genetic Alliance, and President of the Coalition. "Fears that genetic information could be misused hurts individuals, researchers, clinicians, and associated industries. Today, our fears have been addressed."

Marla Gilson, Director of the Washington Action Office of Hadassah, said, "Just 10 years ago, only 100 genetic tests existed. Today, that number has grown to over 1,000 and everyday these tests are helping diagnose thousands of health conditions. Given the Jewish community's historical experiences with genetic issues, we worked hard to see that this bill was passed."

The Coalition has worked for thirteen years toward the passage of legislation to eliminate the misuse of genetic information. Discrimination on the basis of genetic information had led individuals to shy away from genetic testing that could help them manage their health proactively. It also has caused many to opt out of clinical trials for fear that their genetic information would be used against them. This lack of participation has slowed the research and development of treatments and beneficial drugs.

"We now have a huge task ahead of us." said Kathy Hudson, director of the Genetics and Public Policy Center at Johns Hopkins University, "to make sure that doctors, researchers, and the public are aware of the new protections GINA provides."
Just as the House of Representatives did when it passed GINA in April 2007, with 224 cosponsors, S.358 protects Americans from discrimination by health insurers or employers based on genetic information by:

* Prohibiting group health plans and issuers offering coverage on the group or individual market from basing eligibility determinations or adjusting premiums or contributions on the basis of genetic information. They cannot request, require or purchase the results of genetic tests, or disclose genetic information.

* Prohibiting issuers of Medigap policies from adjusting pricing or conditioning eligibility on the basis of genetic information. They cannot request, require or purchase the results of genetic tests, or disclose genetic information.

* Prohibiting employers from firing, refusing to hire, or otherwise discriminating with respect to compensation, terms, conditions or privileges of employment. Employers may not request, require or purchase genetic information, and may not disclose genetic information. Similar provisions apply to employment agencies and labor organizations.

The bill goes to the House, and is assured of passage there as early as next week. The White House has signaled its willingness to sign GINA into law.

###

The Coalition for Genetic Fairness is an alliance of advocacy organizations, health professionals, and industry leaders working to educate Congressional policymakers about the importance of legal protections for genetic information and ensure passage of meaningful genetic information nondiscrimination legislation.
The Coalition for Genetic Fairness is led by: Genetic Alliance, Affymetrix, American Academy of Pediatrics, American Heart Association, American Society of Human Genetics, Brown University, Hadassah, National Society of Genetic Counselors, the National Workrights Institute and the PKD Foundation.

Coalition for Genetic Fairness • http://www.geneticfairness.org • 4301 Connecticut Ave. NW #404, Washington DC • 20008-2369 • Phone: 202.

I'm Finding Courage Through Sharing

As an avid reader of blogs, I've discovered a gem of a writer in Michelle at In the Life of a Child. She began a blog carnival called Finding Courage Through Sharing. It is aimed at a large community of parents struggling through extreme medical challenges with their children. The purpose of the carnival is to give these parents a voice, a place to connect, to grieve a loss or a setback, to share a concern, a frustration, or to share those rare and wonderful triumphs… and to begin trying to heal. It is meant for any parent who has been through any form of medical challenge with their child, and also for those who are still in the midst of facing those challenges.

This post introduces my fellow participants of that carnival to my story.




At my 20 weeks visit, the ultrasound technician pressed down on my stomach and rolled the wand through the warm jelly. I was excited and straining my eyes to see the grainy images coming across on the monitor. Every so often, I was able to identify my baby's body parts. Colleen, the technician, would say things like, "Here's the heart. That is what we want to see. Nice blood flow. Nice strong beat." My husband, Charlie, looked elated to see our baby growing inside of me. "How big is the baby?"

Colleen replied, "I'll let the doctor go over that with you in just a little while." When it was all finished, she handed me two black and white pictures. We made our way back to Dr. C's office and sat down on the other side of his cherry desk. Charlie inquired, "What do you think?"

I replied, "I'm not sure. I'm nauseous from her pressing on my stomach so much." The pregnancy was really weighing on me. I had always thought that I’d begin to feel better by the midpoint, but it hadn't come yet. I was disappointed. I wanted to be one of those glowing mamas-to-be.

Just then, Dr. C came into his office and sat down behind the desk. He picked up my chart, and began to review the images one-by-one. I noticed that he stopped on one and carefully inspected it, but then moved along to review the others. I was looking for clues to how he felt my baby was doing. His body language revealed something wasn't quite right.

"The baby looks pretty good. Organs are developing appropriately, but the baby's size is small. Also, the overall tummy measurements are too big. You have a low placenta, and we'll need to keep watching this. I'm going to have you come back in eight weeks to recheck the baby's size and the position of your placenta."

I fumbled with my words but finally stuttered, "Oh. Oh. Okay. Is there anything different I should be doing?"

"We've found that women who rest on their left side can improve the blood flow to the placenta. So when you are resting or sleeping, use your left side. Just eat well and take it easy. We'll see what happens in eight weeks."

There it was. It was the beginning of our journey. We had no idea what was about to unfold for our baby.

For the next eight weeks, I did as instructed. I often wondered if I was actually helping my baby to grow. I hoped so. At the next ultrasound, the results again were not great on the baby's growth, but my placenta had grown up and away from my cervix. I also shared that I was now swelling off and on. My hands were chubby. My face was puffy, and, well, I no longer had ankles. Dr. C said, "Lots of women swell when pregnant. Your blood pressure is normal, and you don’t have any protein in your urine."

I'd have to come back in four weeks to recheck the baby's progress. Meanwhile, I began to have headaches off and on. The swelling worsened, but finally I didn't feel nauseous anymore. That was a blessing as all of the rest was unraveling. I prayed and hoped for the best.

At 32 weeks, my stomach had begun to pop out a bit. I waddled a little, and my doctor said that the baby's growth was worse. The baby was approaching falling below the third percentile for weight. "Why do you think this is happening Dr. C?" I inquired.

"Your placenta is not working well. We call this intrauterine growth restriction. This happens sometimes. I’d like you to come back every 3-4 days now. We’re going to do an ultrasound and biophysical profile at each visit."

As I heard this, I wondered when I would really feel what he was telling me. It occurred to me that I should probably feel like crying, but all that I felt was numb. Charlie looked very concerned, but he remained very quiet. I could tell he was pleased with a plan of action to watch the baby. Having grown up with an obstetrical nurse for a mother, I knew what this meant. Dr C just wasn't saying it yet. Our baby was getting into trouble and would likely come early. When I returned home that day, I laid down to my left side and prayed for the health of my baby. I was tired, swollen, hot, and generally uncomfortable.

Within two sets of ultrasounds, I was placed on complete bed rest. The swelling was throughout my body and into my brain. My teeth felt very tight inside my gums, and I could feel my pulse against my teeth. Light was painful to my eyes, and straight lines appeared wavy. At one point, small little fireballs raced across my field of vision. I thought, "What the heck was that?" I blinked in response, but I could see the fireballs even with my eyes closed. As fast as they came, they were gone.

A few times, an uncontrollable twitch jerked my head to the left. I had no idea what had happened, but it stopped. My mom had begun calling me daily from Chicago where she lived. When I told her what happened to my head, she replied, "Jen, I think you are getting preeclamptic. I want you to tell Dr. C what happened. Please hang up now and call him." I argued with her, "Mom, he never said he suspects preeclampsia. My blood pressure is fine, and I have no protein." She calmly replied with love in her voice, "Hon, I know he didn't say those words, but you're having some issues. Tell him. It can't hurt to tell him." My emotions were reminiscent of being a teenager again. I felt rage as my mom's concern became my reality. Why couldn't I just have a normal experience? Why? Why my baby? I didn't understand that my uncontrollable emotions were also a sign of my central nervous system faltering. I didn't get that I had been experiencing small seizures. I couldn't process information as my normal self. I had no inhibitions. Whatever came to mind is whatever came out of my mouth.

As I got sicker, so did my baby. Eventually, it was time to remove the baby from the comfort of my womb to begin the process of ending my preeclampsia. My health was failing. My kidneys were shutting down, and my blood pressures were stroke worthy. I had been hooked up to an IV of magnesium sulfate, was wearing an oxygen saturation monitor on my left index finger, and had an oxygen mask strapped to my face. They were inducing me except that my body wasn't responding well. After nearly 24 hours, I had only dilated to 1 centimeter. Charlie, my mom, and my good friend, Shanna, were with me. Suddenly, my fetal heart monitor began to alarm. My mom instructed me to flip over onto my right side. The alarm kept ringing. I wondered if my baby would die inside of me.

I was wrong. My beautiful Grace Ann was delivered by c-section soon after the alarms began to ring. She was small for 34 weeks gestation but she cried and wailed. I was so relieved to hear her cry. The neonatologist assessed our precious miracle, and briefly allowed my mom and Charlie to bring Grace to my place on the OR table. She was wrapped in a blanket and already had a hat on her head. She squinted under the lamps of the operating room table. As fast as I kissed her forehead, Charlie tucked her inside his arms and rushed off with the team to the NICU.



Nearly three days later, I was finally able to visit Grace for the first time in the NICU. My health status prohibited moving me outside of a dark room and an IV of magnesium sulfate prevented eclamptic seizures. By the time I got to the NICU, Grace’s color was pumpkin orange. She was hooked up to a CPAP machine to help her breathing, and she fretted and squirmed inside her incubator. Unfortunately, I couldn't see her. My vision had been impaired due to hypertensive retinopathy. Essentially, my blood pressure had gotten so high that blood vessels had burst inside my eyes. Disappointed, I held her tiny hand, and tears streamed down my cheeks.

It was just the beginning of our journey as parents of premature babies and of children with a genetic disorder called Alpha-1 Antitrypsin Deficiency. I’ll share more of our journey along the way with you. In the meantime, I'm Jen. I survived severe preeclampsia and temporary blindness twice. I've been a NICU mom twice.

My children are miracles. Their lives are not guaranteed. Their presence in my life reminds me to enjoy life's little interactions. In those spaces is where God's grace shines.

My Grace is now six years old. She has a younger sister, Meghan, who is nearly four. Meghan was born a micropreemie. Both of my daughters have Alpha-1. I'm mom to the Alpha Girls. :)

March for Babies

Most of you are aware that both of our daughters, Grace and Meghan, were born prematurely. Gracie was our "biggest" baby born at 34 weeks weighing 3 pounds, 14.5 ounces. Meghan was our littlest peanut born at 27 weeks weighing in at 1 pound, 9.5 ounces. Severe preeclampsia caused their early births. Every day 1 in 8 babies born in the U.S. arrives too soon. Premature birth can happen to any pregnant woman. It is a serious, common, and costly problem. The March of Dimes is leading the campaign to reduce premature birth by supporting research and by educating the public and health care providers.


















The effects of prematurity last longer than past hospital discharge, and both of my daughters experience the under recognized side effects of prematurity every day. This Saturday, April 26, 2008, my husband, Charlie, and I will be proudly walking with our miracles in the March of Dimes March of Babies walk. I would be honored if you would consider a donation to our fundraising efforts. Thanks for your consideration. We appreciate it more than you could ever know.

Please help by donating today!

www.marchforbabies.org/alphagirls

Jen

When I look at this child, I can say without a doubt that I'm filled with emotion. I guess I'm most overwhelmed by the delicate beauty I see. Her skin has a soft, peachy, glowing color, and her light-red hair hugs the contours of her faces and falls gently to her shoulders.

Upon closer look, I see large violet-colored eyes with black eyelashes, which seem almost artificial. Above her eyes are the most distinctly arched dark eyebrows I've seen on one so young. I think of all the time I spend trying to achieve what this child has naturally. Her nose is small on a thin little face. The mouth is small too, with thin pink lips that turn up quickly into a smile as I speak to her. As she lifts her head, I see a distinctly square chin, which seems to point outward as she gives me an impish grin. I look beyond her smile as she jumps and runs on thin long legs. She lifts her arms up and down as she runs and relishs the fact she can snap her long fingers.

As this child grows and develops, I see more than physical changes. A budding personality is emerging. She has qualities of both her parents. I'd like to say only their good qualities, but this would be deceitful. I can say she projects a truly genuine personality that's all her own. There is nothing tomboyish about this little girl. She loves adorning herself with jewlery, sweet smells, and frilly dresses. Of course, she will not accept just anything. Her dresses must be red, with ties in the back, and especially not itchy. The curt denial of anything but her desires might result in a freeze over Brazil. She can stand by the mirror for hours combing her hair and admiring pigtails one day and the next refuse a combing altogether.

She thinks ballet classes are just great, but loves rough-housing with her daddy (as long as it's not too rough). Her quick change from bubbling giggles to torrents of tears always signals the end to rough games for her. How she loves to play silly, but her intense pride in doing projects well can be almost overwhelming. The look on her face will tell if too casual a reply about them has been given.

Her sensitivity to others is so mature. A quick kiss to her sister's hurt knee or a gentle "It's all right." come so easily from her, but if called to her attention, she lowers her head in quick embarassment. A moment later, though, she lifts her head and shows a wide grin, and I know everything's all right. She has appreciated my noticing.

I could go on and on about this little beauty, but that might be bragging. For you see, my mother love is showing through because this little girl is my firstborn, Jen.

Guest Writer: Sherry, dated 11/23/1977

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I guess my writing abilities came from my mother's side. :) I found this description inside my baby book today. I was 5 when she wrote it. I hope my writings inspire my girls when they are old enough to appreciate them.

Has it really been 6 years?

Contracting

Short of breath, I huffed my way down the carpeted, hospital hallway. My toes and ankles ached. My husband's flip flops adorned my feet, and the swollen skin on the top of my feet painfully stretched with each step. I felt quite awkward, but the stoic side of me forced a smile to mask my terror.

Charlie snapped my picture. I cringed on the inside. Why in the world of all that is good would I want to remember this day? My body was slowly failing me and my baby. I didn't want to capture the moments of my body's failure. Charlie made small talk with the nurse who was guiding me to my labor and delivery room. He seemed a bit nervous too.

Upon entering the room, the baby made a lumbering slow roll under my rib cage. I thought, "Aha, there you are. I've been waiting for you to move. You had me worried." It had been seven days of kick counts, and time spent on my left side. I was obsessed with the kick counting, and this was the most I had been upright in over a week. Wooziness was washing over me. My blood pressure was 160/110, and I no longer felt the urge to use the bathroom. All of the fluid in my body was slowly leaching out of my blood vessels and into me.

The swelling had reached my brain and was now affecting my vision. Straight lines now appeared wavy, and the light of day was blisteringly painful to my eyes.

Today was the day to be induced. The baby had stopped growing, my blood pressure was rising, and my kidneys were shutting down. The process of bringing my precious cargo into the world was about to commence. Today was the day that I would begin the process of delivering my baby too early. IV lines were placed. Bags of pitocin and magnesium sulfate were hung. I crawled into bed again, and found my left side. A long sigh emerged from within me.

When the mag hit my inside, I immediately felt hot. It was as if someone turned on a blast furnace from the inside. From there, the details all glide together into a commingled yet disjointed experience. The sequence of events eludes me anymore. That, in and of itself, brings immense disappointment within me. This was my first pregnancy. I want to remember what happened, but the disease of severe preeclampsia stole it from me.

The fuzzy memories I have usually center around the pain I was feeling with each pitocin induced contraction or how the wrinkled sheets underneath my body felt as if I was laying on match sticks.

Each year on April 15th, those fuzzy memories contract and contract and contract within me again. I clamor for the relaxing break between the emotional pain, but the contractions keep coming and coming and coming. I take cleansing, slow breaths in hope they will help. The routine of my day and the preparation for my first baby's birth date don't suppress the contracting. Nothing helps except for the end of the day.

Sleep lulls me now. Hopefully, it will be a sleep so deep and sound that I don't dream of those contractions again at least until next year.

Boston Marathon Update

In January, Grace and Meghan were matched with Jen Davis, a runner in the Boston Marathon, through a patient match program in use at the American Liver Foundation. Jen is participating in the Run for Research, and we are incredibly honored that she runs in honor of Grace & Meghan, who live with Alpha-1 Antitrypsin Deficiency every day.

Please consider making a donation to Jen! She is doing such a great job running for anyone affecting by liver issues/diseases. Thank you Jen!

http://www.active.com/donate/liverteam08/alfJDavis



Here is her latest update:

The marathon is 2 weeks from today and the the hard training is now over. We had our 21 mile run last Saturday and I am thrilled to say that I got through it. I have been nursing a quad injury and had not run for 2 weeks prior. I ran a nice steady pace and was able to complete the whole run. Although I had trouble walking for the next 2 days. From now until the marathon we are in taper mode. Giving our muscles time to heal. My fundraising is going great. I am at $6,100 and plan on hitting my goal of $7,500. I am attaching a picture form our run last weekend. This was taken before we set off on the 21 miles. Of course that is why I have the smile on my face. The picture at the end isn't so pretty.

A big hello to Grace and Meghan and please let them know that their names will be written on my marathon shirt in huge letters! ~ Jen

Sensations

Nearing dawn, I awoke with the sensation that my chest felt heavy. The weight of my upper half felt as if it was pressing down from the inside onto the muscles of my upper back. It was odd, but strangely relaxing. The usually knotted muscles within me relaxed in response.

Was this sensation a remnant of that space between awake and asleep?

A virus had wrapped itself inside the cells of my being. The words "sick and tired" paled in comparison to how I felt. Dark circles grew larger under my eyes. My chest inhaled and exhaled rapidly over and over. My heaving breaths seemed to further extinguish my energy.

I shifted in the space of my bed, and pain rushed through the nerves of my lower neck, reached an intersection, and flowed up both sides of my face to the pinnacles of my temples. The ibuprofen had worn off over night, and I needed another dose. I didn't like relying on the pain reliever, but the alternative reminded me to ginger myself.

A tickling sensation grew near my vocal cords. I swallowed to tame the tickle. It didn't work. Coughing, that seemed to originate deep inside my lungs, sputtered from my lips. Over and over, I coughed. My oxygen supply was diminished, which encouraged my lungs to inhale deeply. I choked on the air, and sputtered some more.

The pounding of my heart startled me, and I sat up in response. I gasped for air again, and reminded myself to try a shallow breath. That helped, but it was followed by a fast 1-2-3 sequence of breaths. Suddenly a scratchy sensation welled up within my throat.

Choking coughs emerged, and my vocal cords throbbed in pain. My heart raced in response, and the din of my sputtering faded away. I sighed and grabbed my forehead. A chill cascaded within me, and I wondered about my temperature.

Was it 102.5 again?

My chest rose and fell over and over again in rapid fire sequence. My friends and family with Alpha-1 Antitrypsin Deficiency ran through my thoughts.

Is this how they feel?

Is this how their breaths feel?

Is this how Grace and Meghan will feel in the future?

Choking coughs thrust forth again, and throbbing within my throat induced a swallow. Tears pooled within my eye lids, and my thoughts failed to calm my fever induced anxiety.

A virus was ravaging my insides, and I reminded myself to rest again. I gulped two more pills, and poured the water into the desert of my mouth and throat. I no longer had the energy to sit up. The mattress enveloped me with warmth, and my eyes closed under their own weight. Charlie rolled over and slid his sleep heavy hand onto my hip. Unfortunately, the muscles surrounding my hip knotted in response. More pain flowed.

A tear slid down my cheek toward my ear, self pity tried to emerge, but was quieted by the coughing within me.

A new sensation quelled. Itchiness inside the lower portions of my lungs insulted my sanity. I rubbed the middle of my back near the itching but found it futile. The itch was inside me, nowhere near anything I could scratch.

Fortunately, sleep found me again. Rest was what I needed. Rest would help with the battle against this virus.

Rest.