Hematology appt- Von Willebrands Disease

We saw hematology on wed.  We discussed Hannah's Von Willebrands (VWD).  He said we are not using the DDAVP since they are sure that is what caused the drop in sodium and her seizure. 
  They are going to be giving her Von Willebrands factor as an IV as soon as she gets to the hospital the day of surgery and for at least a week after.  There is also a medicine called amicar that they will also start giving her before.   She has  had it before.
  they called yesterday and said her VWD levels were still the same, I was hoping different.  Her Vit K levels are low so they want us to start a special compound of Vit K.  Our pharmacy doesn't have it so they had to send it to Hills in Easton.  They need one ingredient to fill it so it won't be ready til Monday.  I told her I don't have a way to get it on monday especially since I hope to be in Va with my new grandson.  She said once it was ready she would call and deliver it herself!  I am not used to that :)  After 6 doses they want us to repeat the labs.
  She had low Vit K levels years ago and was on regular vit K for a long time.  the reason we found out before was she was bruising a lot.  When we went to hematology for the bruising and vit K, that's when they found she has VWD!
  the spinal fusion surgery is one of the most bloodiest I was told.  the ortho surgeon told her the greatest risk for Hannah is bleeding out since she is so small (only 38lbs and 40" tall)  So I am thankful they are being aggressive with the bleeding issues now.  We are only 4 weeks away from surgery now.

Hannah hematology appt

Hannah has an appt with hematology wed,  I hate appts that are late in the day, this one is at 1pm :(   gets us home really late and into rush hour traffic.   Years ago she was diagnosed with von Willebrand disease, she has a  mild case. The last time she had surgery (years ago)  they used the DDAP before.  The next day after surgery she had a major seizure that couldn't be stopped with anything...they finally found that her sodium levels were very low and when they gave a major dose of saline, it stopped.    What impresses me is that it was picked up when her pre surgery  things we being set up.  It shows me they are on the ball about things. So she is having bloodwork done and then we meet with a hematologist to discuss it.  I am glad about it since her dr is concerned about her bleeding out since she is so small.


http://www.nhlbi.nih.gov/health/dci/Diseases/vWD/vWD_WhatIs.html 

The Miracle of Mercy Land by River Jordan blog tour

Mercy Land has made some unexpected choices for a young woman in the 1930s. The sheltered daughter of a traveling preacher, she chooses to leave her rural community to move to nearby Bay City on the warm, gulf-waters of southern Alabama. There she finds a job at the local paper and spends seven years making herself indispensible to old Doc Philips, the publisher and editor. Then she gets a frantic call at dawn—it’s the biggest news story of her life, and she can’t print a word of it.
Doc has come into possession of a curious book that maps the lives of everyone in Bay City—decisions they’ve made in the past, and how those choices affect the future. Mercy and Doc are consumed by the mystery locked between the pages—Doc because he hopes to right a very old wrong, and Mercy because she wants to fulfill the book’s strange purpose. But when a mysterious stranger shows up, Mercy begins to understand she may have to choose between love and loneliness . . . or good and evil . . . for the rest of her life.

RIVER JORDAN began her writing career as a playwright with the Loblolly Theatre group. She teaches and speaks nationwide on ‘The Power of Story’, is a monthly contributor to the southern authors’ collective A Good Blog is Hard To Find, and produces and hosts the weekly radio program CLEARSTORY with River Jordan, in Nashville, where she and her husband live. She is the author of Saints in Limbo and this is her fourth novel.

This book really makes you examine the choices you have made in you life...how they affect your life and the lives of others that you come in contact with, now or in the future. A good book that keeps you on the edge of your seat wondering what is going to happen next! :)

Making friends Monday!

A good way to make friends today!

Join swagbucks today!

If you look on the right side of this blog, there is a swidget for swagbucks...click on the swagcode button and use that code to sign up and earn an extra 20 swagbucks...
use this link to sign up and we both win!
http://www.swagbucks.com/refer/joanjones
now is a great time to start, I am using my swagbucks to buy amazon.com gift cards to use for Christmas gifts! It's easy to earn swagbucks just for searching!

Hannah update

Hannah is still having tons of secretions, very thick almost plugging her trach..thankfully she still has a pretty good cough. For the last week she has been having more seizures than normal. :(
thursday we are going to dupont, she has an appt with GI for followup. And then we are seeing a hospitalist (for the first time) about her upcoming surgery. I just realized it's only a little over a month away now!
Jenn and Bob's baby is due in two weeks! so please pray that when she calls, the extra nursing (respite) will fall in place for us to be able to go!
thanks for the continued prayers for us!

Hannah's story

Chuck and I were married in June 77. In 3/81 our oldest daughter Becky (Rebecca Lynne) was born, 2/83 our middle daughter Jenn (Jennifer Michelle) was born. When they were 12 & 14yo we had Hannah Grace (7/95).
We were surprised when she was born with all the issues they found. I was 37yo when I had her and the whole pregnancy just knew there was going to be something wrong with the baby (didn't know the sex back then). I didn't tell anyone I felt that way, I just thought it was my age and you hear that the older you are, the greater chance for a child to have down syndrome.
At 21 weeks they did an ultrasound to check the due date. Because she was small, they said I was 17 weeks. I knew I was right, when it's your 3rd pregnancy you know when you are pg! LOL they tried to get a profile of her but she kept turning her face to follow the probe thing. If they had gotten a profile, we would have seen a very large obvious cleft lip. But we never saw that. My uterus measured a normal size so they never did another US. I had also refused all testing and they had me sign papers saying I refused it. About two weeks before she was born the dr asked how big our older girls were, I told him 6 lb 13 oz and 6 lb 10 oz. He said I think we are looking at 6 1/2 - 7 lbs here! He was totally shocked when she was born and was so small! She was 3lb 14oz and 16 1/2" long! Immediately they saw the small size, very large cleft lip and palate and realized there was a problem. Interestingly her APGAR score was a 9! Her lip was very ugly but she was beautiful. Becky was in the delivery room and her first words were "Mom, she is beautiful!"
they tried feeding her and she aspirated and got her first of many aspiration pneumonia's then. They came in and told me she had pneumonia and would have to go up to the NICU. When they said the helicopter would be there soon I was shocked, I didn't know they didn't have a NICU there and up to the NICU meant going to Baltimore MD! I thought they meant upstairs! I later found out that most parents didn't understand that either!
She was in the NICU for 3 weeks. While there her bloodwork showed she had a deletion on her first chromosome. She was missing bands 21 -25 on the long arm. (this summer 2010, we redid her blood work for her chromosomes and found that her deletion is actually 1q 24-31! That is a very large deletion that controls a lot of things. Testing had changed a lot in 15 years!
She was sent home from the NICU with an NG tube for feedings and an apnea monitor. I don't think they really expected her to live long and she didn't get her gtube surgery until she was 3 months old! She was still too small to have her cleft lip or palate surgery. She had a good suck, she loved her pacifier but looking back her problem was she couldn't swallow. they did a PH probe to test her for reflux and found that she had over 300 episodes in 24 hours. It was silent reflux, she rarely spit up but was constantly crying and miserable.
So at 3months, she got a nissen and gtube. She was a different baby then, hardly every crying. She didn't sleep much and drove her sisters crazy at night! Our room is down the hall so we didn't hear her but they were right across from her!
When she was around 11 months old she had her first status seizure. That started her with many aspirations and seizures that ended up with her flying to Baltimore to the hospital. At that time she was learning to sit alone and could do a few things like roll around and roll over. We also found out that she is profoundly deaf and has cortical vision (she is considered to be deaf/blind) We don't know what she sees or hears.
She had a lot of trouble breathing at times so it was suggested that she get a tracheostomy when she was 3 1/2 yo. I was a little shocked but did some research and decided it was the best thing for her then. She did well but was in the rehab hospital for 5 weeks waiting for nursing to be approved! Finally she qualified for a Medical Assistance waiver and was allowed to go home. That was in Jan 99.
She still had constant aspirations so in 2001 she had a surgery to stop aspritations. It was called a laryngotracheal separation and completely cut off her upper airway from her bottom airway (lungs). This surgery is permanent and means she will always have a trach. She can't breathe through her mouth and nose anymore. She also can't make sounds since no air goes past her vocal chords. That is why she is so quiet! :)
She hasn't had pneumonia since then! She still has a lot of respiratory problems and gets a lot of neb treatments around the clock. It was found that because of all the aspirations she acquired trachomalacia and bronchomalacia so she was put on CPAP. She uses a ventilator for the CPAP. She has nurses her with her at night for 12 hours. Two days a week she gets an extra 4 hours for me to go to the dr etc. She doesn't know not to pull on her trach, etc and if she pulls her trach out, she can't breathe, so she has to have "awake care" 24/7!
Hannah's seizures have gotten worse over the years and she has lost a lot of her "abilities" She is developmentally about 2 -3 months in age. She can't do anything for herself at all.
We always knew she had a neurogenic bladder but last year found that her brain doesn't tell her spinchter to open so we have to straight cath her 4 times a day for residuals. Sometimes we can get as much as 500cc's from her tiny body! At 15yo she is weighs 38lbs and is 40" tall. She wears a size 5 infant shoe and can wear slightly larger than newborn hats.
We had been watching her scoliosis for the last few years. In two years her curve went from 31 degrees to 75 degrees so in Nov 10 she is having spinal fusion surgery. We are not looking forward to that at all, but know it is necessary.
I know I have left out a lot. She has had multiple surgerys for her cleft lip and palate but at this time they are still not completely repaired, it's all cosmetic and we have always said we would only do what is the best for her to keep her comfortable.
Prayer is the one thing that has gotten us through all this. Hannah would not be where she is without it. I thank the Lord for the opportunity to be her Mom.
  

feeling better

Hannah seems to be feeling better today. The gagging etc are much better but she still has a lot of seceretions. Thanks for praying.
A nurse from general pediatrics at A I duPont called today to schedule Hannah an appt with a hospitalist for her upcoming surgery. Thankfully they fit it in next week on the same day she has an appt with GI.
I was looking around the hospital website and found this link about spinal fusion surgery that she is having.


http://kidshealth.org/parent/medical/bones/scoliosis.html#

Hannah is sick again

She started with a lot of extra secretions on saturday and had a few more seizures than usual. Sunday morning I noticed her face flushed for just a second and hoped it was a fluke. She was fine during Church, didn't even have to take her out and suction her like I usually do. After we got home, she started with the hiccups, face flushing and chewing, later she started gagging, she hadn't done that with the other episodes like this :(
Michele said she had a terrible night. Today was a little better. She has been trying to sleep but everything keeps waking her up. Gave her pedialite today to give her tummy a rest.
She has episodes like this every couple months and we don't know why!