No Diagnosis Drama

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drama [drah-muh, dram-uh]

Any situation or series of events having vivid, emotional, conflicting, or striking interest or results.

My original title for this post was The No Diagnosis Depression. After mulling it over, I decided it really is not a depression. Rather, it is a drama. It feels like I have vivid, emotional, and conflicting feelings with no certain result. My heart aches for a diagnosis, a reason to understand what Piper is going through. A diagnosis of which we can relate to other families with similar fates. A crystal ball to see what the future holds for my girl. We thought when we joined TGen’s Center for Rare Childhood Disorders, that we would finally get a diagnoses, an answer. God blessed us with being accepted into their research, and we are thankful.

After Darrin, Piper and I had our blood drawn, we waited. Then we waited some more. Finally, I got a phone call that had words I did not want to hear. It was the patient care coordinator from TGen. She said that the geneticists had tried exhaustively to find a diagnosis for Piper. Unfortunately, there are no known syndromes that match Piper’s phenotype. Phenotype means the symptoms she presents with like hypotonia, no speech, severe developmental delays, unable to potty train, unsteady gate/walk, often holding hands at a 90 degree angle, etc… I was told that her information would remain in their “super computer” until someon, somewhere, finds children similar to Piper and conducts a candidate gene study. Until that time, we are back at square one.

My feelings were unsettled. I wrestled with our conversation. They told me not to get my hopes up, but I expected at the very least something small. My mom urged me to call back and see if we could meet with the neurologist. There must be something overlooked or some more information that would be helpful. I explained my concerns and fears. A meeting was set up and Darrin was to join us. At the last minute, he was unable to attend because he had to cover at work. So, I took Quinn and Piper by myself.

It was a meeting of spoken words that I did not understand. My history of teaching biology helped me to understand some of their vocabulary. However, there were so many numbers and gene sequences mentioned that I could not keep up. I tried to write notes, but keeping Piper entertained and sitting in her chair was a distractor. She continually wanted me to walk her. The doctor and patient care coordinator shared they thought they had found Piper’s syndrome, but it was ruled out because I had the same mutated gene with a shank deletion. That meant that it could not be Piper’s syndrome. I felt so alone.

At home, I decided to look into this syndrome, thinking maybe they made a mistake. On the syndrome’s website there were children that reminded me of Piper. It is affectionately called the “no speech syndrome”. This described my Piper to a tee. I decided to contact the lead of the family support group just to ask a couple more questions. She willingly offered to forward our test results on to their main geneticists. Once I got the information from TGen, it was sent to a doctor in Paris and one in Mt. Sinai. They both concurred that Piper and I had such a small deletion it could not be diagnosed as this syndrome. So this truly was our dead end. It was our go back to start card.

Square one again can seem like a dead end. I can look at it like a hopeless street coming to an end with no way out, or I can choose to think of this dead end as a cul-de-sac. We will maneuver our way around the curve and head back out. The journey will continue.

Lord, I am disappointed but not hopeless. You are the one that decides the information we learn. Thank you for opening my eyes to see the pearl created by all these years of sand grinding, irritating, forming something within the oyster of my life. She is your child and that is what makes her special. Amen.

 

 

 

 

Pain

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[ payn ] 1. unpleasant physical sensation: the acutely unpleasant physical discomfort experienced by somebody who is violently struck, injured, or ill                                                                     

2. feeling of discomfort: a sensation of pain in a particular part of the body
3. emotional distress: severe emotional or mental distress

It is a humorous thing to my husband and others, that I am not able to handle pain at all. At the first sign of blood, I usually throw up or pass out. That is why it amazed them that I wanted to have a drug-free delivery. My husband just smiled and humored me. He knew that I would not make it past an hour without drugs. Well, he was right!

My contractions were horribly long and awful! I am sure that veteran birth mothers would find it nothing at all. The nurse said she would give me something to help me manage the pain. We were thinking something like extra strength tylenol. Actually, she injected morphine into the IV and then ten minutes later injected another dose. I passed out cold. When I awoke to be admitted, the labor had stopped.  We were worried about the baby and why the morphine had stopped my labor. After a conversation, the OB on call decided to administer pitocin. It was only three days before my due date.

A couple hours later, my water was broken, and I requested an epidural. My dialation went from 2 to 10cm very quickly. Our baby’s heart rate dropped and nurses rushed in. I amazingly pushed her out in only a couple pushes. We were going to meet our baby! But, that did not happen. Our baby girl was slow to respond to resuscitation and had decreased muscle tone. Her apgar scores were 6 and then 8. Nurses scurried, a neonatologist was called, she needed oxygen, and was then taken to an intermediate care nursery. I sent Darrin with her and watched them exit the room. My body hurt, my heart hurt, and I was so confused. I was supposed to be holding my baby on my chest, cooing over her beauty. Our little Piper Amanda was finally here but not with me.

That night was very traumatic. Nurses continued to force me to try and nurse her. Piper could hardly suck and would scream as I tried to feed her. She also made a loud vibrating sound as she breathed. Later I was told it was called strydor. I could not feed my baby or comfort her. The breast pump was introduced to me. It became a close friend, because it was the only way I could feed my girl.

Piper had fluid in her lungs, thought to be pneumonia or aspiration, and the doctors started antibiotics. We had to put our little girl through a broncoscopy to determine why her lungs were filling with fluid. She was diagnosed to have laryngomalacia. Her larynx was floppy and did not cover her windpipe when she drank. The milk would go straight into her lungs and turn into pneumonia. This meant she needed to have a nasogastric tube. So now she had a tube up her nose in addition to a heart rate monitor, oxygen monitor, and an IV. The IV happened to be in her head because the other places on her body would not allow it.

Doctors were still unsure of what was wrong with her. An echocardiogram was done, because in her chest x-ray the heart looked abnormally shaped. Some nurses thought they saw seizure activity, so ordered an EEG. All came back normal. I look back at my journal from this time and see that my heart felt like it was being ripped out. My daughter had tubes shoved down her throat into the lungs, needles poking everywhere to find a vein, and the insesant beep of the oxygen monitor yet I could not stop any of it. I could not fix it. I could not save her.

We finally were released after seventeen days. Seventeen days of visiting the hospital and praying we could bring our girl home. So we headed home with the apnea monitor, extra nasogastric tubes, tape to adhere the tube to her face, and bags to put my milk in to gravity feed her through the tube. We were so happy!

My husband still cringes when he hears a whirring sound like the breast pump. I was surprised I did not wear the motor out. It was pumping many hours in the day. Piper’s feeding times were a challenge. I would pump for 20-30 minutes, then put the milk into a bag that looked like an IV bag, hung it on a pole, and then let gravity do its work to feed our girl through her ng tube. That would take another 30-45 minutes. Then we would start all over again in a few hours.

A developmental nurse was to come to our house and check Piper’s progress in a few weeks. That was the only help the hospital offered. We were sent home feeling scared about how to care for our baby. Being a teacher, pictures of bullies, teasing, and special education classes were in my nightmares. How could they send us home so unprepared? Where was my lesson plan? Where was my list to check off of things to do for her?

That nurse did come, and noticed that Piper had gotten floppier. Her head always tilted to the left, and that side of her head had become flat. I explained that Piper would not look at me. She did not respond to faces or toys in front of her. We were sent to a pediatric opthamologist. He diagnosed Piper with Cortical Visual Impairment. There was nothing structurally wrong with her eyes, but the pathway from the eye to the cortex in her brain was blocked. The doctor gave me a script that changed our life. On it he had written the phone number for the Foundation for Blind Children. My body seemed to implode with pain. How could she survive? It was already bad enough that she could not drink on her own, could not hold her head up, but now she was also blind. God please help me to understand why this was happening I prayed.

After a week or so of wallowing, I made a phone call. It was one of the best phone calls I have ever made. A woman from the foundation came out to evaluate Piper. She set us up with an Early Invention Therapist that came to the house once a week. We were able to go to the foundation on Fridays for music time and a parent support group. This was the lesson plan I needed, the steps to follow, and a huge blessing. I was able to get her physical therapy, feeding therapy, and occupational therapy. They taught me how to apply to the Department of Developmental Disabilites (DDD) to receive these services and help pay for the many doctor appointments. I made some good friends and saw there were other families struggling in similar situations.

My maternity leave from teaching had to be extended a month. Piper was not ready to be left with my mom yet. During that time, we were working to get Piper off of the ng tube. She had a Modified Barium Swallow at four months that showed she was controlling her swallows better. If we wanted to get rid of the tube, we had to sit her in her carrier at an exact angle, use thickener, a special nipple, and regulate her swallows. We agreed!

So, I went back to work full time along with my friend – the breast pump. I officially felt like a cow. But I felt thankful that I was able to produce so much milk. My mother was fabulous and watched Piper during the day. Grams, as we call her, helped with Piper’s many therapies and intricate feedings. She accompanied me to doctor visits, held me when I cried, and kept me from having a nervous breakdown. While praying for Piper, God let her know that Piper is our little tortuga (turtle in Spanish). Her race would be slow and steady, but she would finish the race. My mom repeats this to me over and over. She stands by it and has a strong faith that it is true.

There is a fabulous poem called “Welcome to Holland” that I think everyone should read. It summarizes the excitement of preparing for a child, and the pain of that child being different than expected. As a parent you want the world for you little ones. Sometimes they are not capable to handle the world. Or rather, the world is not tolerant enough to handle them. We adore Piper and would never wish for someone different. But we also grieve that there are experiences she will never have, traditions that will never be, and communication between us that will never be easy. She may have to live with us for the rest of her life. So we will give her a life that is happy, loving, and safe. She is our Holland.

 

Welcome to Holland
by Emily Perl Kingsley

I am often asked to describe the experience of raising a child with a disability- to try to help people who have not shared that unique experience to understand it, to imagine how it would feel. It’s like this…

When you’re going to have a baby, it’s like planning a fabulous vacation trip -to Italy. You buy a bunch of guide books and make your wonderful plans. The Coliseum. The Michelangelo David. The gondolas in Venice. You may learn some handy phrases in Italian. It’s all very exciting.

After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The stewardess comes in and says, “Welcome to Holland.” ” Holland?!?” you say. “What do you mean Holland?? I signed up for Italy! I’m supposed to be in Italy. All my life I’ve dreamed of going to Italy.”
But there’s been a change in the flight plan. They’ve landed in Holland and there you must stay. The important thing is they haven’t taken you to a horrible, disgusting, filthy place, full of pestilence, famine and disease. It’s just a different place.

So you must go out and buy new guidebooks. And you must learn a whole new language. And you will meet a whole new group of people you would never have met.
It’s just a different place.

It’s slower-paced than Italy, less flashy than Italy. But after you’ve been there for a while and you catch your breath, you look around… and you begin to notice that Holland has windmills… and Holland has tulips. Holland even has Rembrandts.

But everyone you know is busy coming and going from Italy…and they’re all bragging about what a wonderful time they had there. And for the rest of your life, you will say “Yes, that’s where I was supposed to go. That’s what I had planned.”

And the pain of that will never, ever, ever, ever go away… because the loss of that dream is a very, very significant loss.

But… if you spend your life mourning the fact that you didn’t get to Italy, you may never be free to enjoy the very special, the very lovely things …about Holland.

Lord, thank you for having our plane land in Holland. Piper is a gift and a joy every day. Yes, I am tired most days from waking early and meeting her self-care needs. No, I do not feel anger toward her for this. You gave me this pearl to care for and treasure. Please help me to always remember the pearl she is amidst the sand and struggle.

 

Infertility

INFERTILE
1. not fertile or productive: incapable of or unsuccessful in achieving pregnancy

I thought with my fabulous ability to control my future,  getting pregnant would happen in a snap. God once again, had other plans. We spent four years trying, going to infertility doctors, getting painful fertility shots, insemination, and more. I became increasingly aware that I was the problem. My hormones were not right, my uterus lacked a lining, and my ovaries did not want to release the eggs. Had my years of abusing my body caused this? It probably was a contributor. My heart sank as I offered to divorce my husband, so that another woman could give him the child that he dreamed of. He thankfully declined!

During these years, one of my dearest friends got pregnant. She was afraid to tell me because of our unsuccessful tries. I was so happy for her, but so pained for us. Why was God blessing others and skipping over me? She was blessed to grow a baby. I was not. It is embarrassing for me that I actually cried my way through her baby shower. I look back and think how selfish I was. At the time, my emotions were so raw and hard to control. I hope that she understands and forgives me. Then another friend got pregnant and another.

Darrin and I had resigned ourselves to take a break. We were beaten and tired from the emotional roller coaster. If God did not bless us with our own child, we would adopt. I was actually at peace with this and looked forward to no more guilt. While you are trying to get pregnant, you get sick of so many people telling you to just relax and it will happen. Well, that is exactly what happened. I let go and let God! It was as if he were next to me saying, “Now is the time. You are ready for my special gift.”

I could not believe it. The pregnancy test showed two lines! Out of the hundreds, probably more like thousands, of dollars spent on pregnancy tests, I had never actually seen two lines! Instantly, I went to the store to buy a different brand of pregnancy test. Surely, this was a faulty brand. I took it to my parent’s house and promptly peed on it. It also said I was pregnant! Yippeee! The next step was to call my husband, who did not believe it either. He needed to see the pee sticks, then the blood test results, and finally an ultrasound. Men are so wrapped up in the factual aspect of things. My heart knew that God had finally blessed us.

We went through all of the doctor appointments and birthing classes. When asked about doing the genetic test for abnormalities or syndromes, I declined. My belief was that we went through so many challenges trying to get pregnant, surely God would give us a healthy baby. I ended up being wrong, but we would not have aborted anyway. My sister’s life was taken prematurely by cancer, and I could never take a child’s life because he or she was not perfect. It is rather ironic that I do not expect perfection from anyone but myself.

It was a few weeks from my due date, and some of my students had been out with the stomach flu. I was not immune to the virus and became very ill. It is difficult to run to the bathroom and actually vomit “in” the toilet with such a large belly. My respect goes out to those women who suffer morning sickness throughout their entire pregnancy. I went to the OB triage and was sent home to drink more fluids. That did not work, and I returned after losing six pounds. Finally, they admitted me for IV and potassium. My husband could not stay with me in the hospital, as he had been struck by the virus as well. It was a very scary night of blood tests, fetal heart rate monitoring, and being assessed every couple hours. The doctor released me the next day, thanks to the wonders of Zofran. It is a fabulous anti-nausea drug. I went home and awaited the arrival of my perfect daughter.

Lord, you knew the gift you were about to bring into our lives. You must have felt we were strong enough to handle a life with Piper. She was a long awaited miracle in a very painful, sandblasted life. Please help us to be the parents you desire.

Shields

piper afo

human (adj) having human form or attributes as opposed to those of animals or divine beings

Today, Piper and I walked at the train park to get her exercise before it was too hot. As I helped Piper meander up the play structure, a little girl looked at her, ran to her mom, and asked loudly, “Is it human?” This was a question I had never heard before. It took me off guard. As a parent of a child challenged with special needs, you learn to arm yourself with shields. Shields to deflect the stares or looks of pity. Shields to only hear the positive and drown out the words of people being thankful they have a “normal” child. Shields to stop the pain that pricks your heart each time you realize that life is harder and different for your child.

So after I put up my shields and quickly diverted Piper away from the area, I started to chuckle. This little girl had seen Piper’s enormous AFO’s (braces) that help her to walk steady, and focused on the metal and plastic. She could not see the inquisitive human girl inside. This is probably because there are currently many kid movies about robots living life like humans. She wondered if I walked my robot to the park!

I am blessed that my Piper is in human form. Some of that form is flawed and works slowly. But she has never known life to be different. Her struggle has always existed, and she has had to rely on her “robotic” aides to function. If we are honest with ourselves, we could realize that we are all in a flawed human form. We just put up our shields to hide the flaws, divert attention, and hear only what makes us feel good.

He has made many parts for our bodies and has put each part just where he wants it. What a strange thing a body would be if it had only one part!  So he has made many parts, but still there is only one body.  1Corinthians 18-20

God picked each part of Piper when he was forming her. He picked each part of you when he was forming you. There is a reason we are all here. We each have a part or role that God plans us to play in the lives of others.

Currently, my back and arm parts give me the strength to assist Piper with her mobility. My brain part gives me the ability to help my struggling learners learn. My heart part helps me to understand the struggle some parents face and equip them with knowledge to help their struggling child. If I do not take down my shields, these God given gifts will be unused.

Perhaps, the little girl was able to glimpse the honest, flawed, intricate design Piper has from her divine being/creator. In the absence of a shield, she may have wondered if Piper could be a human.

Lord, please help me to embrace my flaws and not hide behind shields. I know there will be hurt and anger at times. Allow me to use those to become the person you created me to be. Amen.

 

Words

In the 13 years of my daughter’s life, she has never spoken a word. Not one single word.Easter 2011 002Words [wûrdz] A sound or a combination of sounds, or its representation in writing or printing that symbolizes and communicates a meaning

People often ask me how we communicate with Piper. It is difficult to explain. She has no words, but will make high pitched squeals or short vowel sounds. Sometimes, it can sound like she is saying, “yeah” or “uh-uh”. Her facial expressions and tone of sound or cry can communicate her needs.

When Piper was younger, we tried to teach her sign language. She did not consistently understand the differences in each sign, so when asking for what she wanted, it was like watching the coach in a baseball game with arms waving, tummy patting, ear pulling, clapping, and chaos. She became very frustrated with us! Why could we not understand what she wanted?

Next we tried to give her “words” with picture cards and had her point to what she wanted. Piper became proficient at pointing to what she wanted and very good at pushing away the picture or item that she did not. Then the iPad came into our life. We loaded it with a communication program that we are slowly working to utilize on a daily basis. Piper will use it to request a certain food, tell me she “needs a break”, or let us know that she wants to go to school. The struggle is in her lack of motivation. She will use it to communicate if prompted, but not in a reliable manner. I often wonder if she finds it easier for us to anticipate her every need rather than struggle to translate thoughts into the push of a button.

It is quite an eye opener when you ask people to communicate without words. They struggle to make themselves heard when there is silence. Yet, their body language and facial expressions are so telling. We take for granted that we have a voice. We take for granted that we have words. We have the ability to choose positive or negative words, kind or hurtful words, uplifting or cutting words.

“A word of encouragement heals the one who receives it, but a deceitful word breaks the spirit.”    Proverbs 15:4 The Voice

Perhaps God’s plan in blessing me with Piper is that I learn the truth about words. My girl has no words, but seems totally content in life and feels love. Why do I put my self-worth in approving words from others? Why do I seek words of affirmation to prove that I am worthy? My Holy Yoga journey is one of finding the quiet. I am working to find peace in myself and knowledge deep down in my soul that He loves me. God knows I am worthy. I will not have the right words or ability to speak life into others until I fully rely on my Lord. It’s time to shut my mouth and listen.

Lord, I am seeking to hear your words and plan for my life. My flesh focuses on my desire to hear Piper say a word or tell me that she loves me. Her life is a pure example of how I should live my life. I want to have happiness and joy because I have your Truth within as my foundation. Help me to grow. Amen.