Zero is a Great Number

Haley's shunt revision surgery went well on Tuesday. Dr Henderson was pleased and said that her spinal fluid was flowing briskly, indicating that her pressure had been too high. Haley had a good bit of pain after surgery and passed out twice in the immediate post op period. Since then she has not passed out at all!!! None on Wednesday and none so far today (Thursday). That's none, nada, zilch, ZERO fainting spells!!!!! After passing out 50-80 times a day for months, this is fantastic news and we are grateful to God for giving Dr H the skills to help people like Haley.

She is still in pain, which Dr H thinks is from tethered cord. We will discuss this on Monday at her post op appointment. If he thinks she's a candidate for surgical "de-tethering" we trust that her remaining pain will decrease (or even disappear!)

The staff at this hospital has been great. They are so knowledgable and kind. They are so used to taking care of people with this disease (EDS), so it is refreshing not to have to constantly explain things. One of the people told Haley yesterday, "other places you are considered a zebra, here you are a horse!"  It's nice to be a horse for a change!!

The PA, Bob came in a while ago and walked Haley down the hall. No faintiness at all!! She's as weak as a kitten from being in bed for so long, but it's just a wonderful thing to see her up and moving. Hopefully she will soon be able to do all the things a 22-year-old young lady should be doing!!

We are hoping that she will be discharged later this afternoon and I will post a picture of her walking in the hallway with Bob when we get home. I'm blogging from my phone and can't figure out how to get a picture on the blog if I'm not on my computer. Haha. At least I finally figured out how to finally blog!! Thank you all so much for your prayers and messages of encouragement to us. They mean the world to us.

Sometimes It's Good to Fail a Test

I thought it was time to give you all an update on Haley. She has continued to suffer from severe pain from her head to her toes (specifically head, neck, back and leg pain.) Also, her "fainting" has picked up again. She had a decrease in it after Dr. H took some fluid out of her spinal column, but it's back. As you can imagine, she is pretty discouraged. She knows that many are praying for her and she is encouraged by cards, visits and some gifts she has received. Such kind and thoughtful things have made a big difference. Thank you.
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Yesterday was a big day. We traveled to Maryland again. This time, it was to see a urologist who Dr. H referred her to so that she could have some testing done on her bladder. Apparently, if the bladder is not functioning correctly, it points to possible "tethered cord." Well, as you can imagine, the tests were no fun. They had to fill her bladder and take measurement while it emptied. (I held her hand and sang to her-- "Fill me up Buttercup!" She wasn't impressed.) Between the embarrassment and pain, she persevered and got through it. (So proud!) Anyway, there was some sort of problem with the office, so the doctor was delayed. (Side note: what is it with doctors' offices in that area that stay open so late?) Anyway, we waited 2.5 hours in a small room for the doctor to review the test with us! Thankfully, our dear friend, Kendra, was with us. I could never have taken that trip without her! When the doctor finally came in, he asked Haley several questions and showed us the printout of her test. Bottom line: she failed. (Yay!) This points to the possibility of tethered cord. I have long suspected that this was the reason for her pain and fainting. If it is, it can be surgically corrected and, hopefully, both can be alleviated. Please pray for this.

Today she has her pre-op physical and blood work in preparation for her shunt revision surgery on Tuesday. She also needs to go to the hospital and have an x-ray taken of her back so that Dr. H can see the shunt placement before surgery. Finally, we have an appointment with the Social Services department to see if she qualifies for any type of disability coverage. Hopefully, she will get a little money each month, as she is not able to work and has no discretionary spending money. If it's more than a little, she can take care of some of her medical expenses.

If you're reading this early Friday, will you pray that she can make it through all of these appointments today? I can't tell you how difficult yesterday was for her. For someone with virtually no activity level to be out for 11 hours (we got home at 10:30 pm!) and constantly sitting up, was rough. At times, she faints continually, one after the other, which she did several times yesterday. Again, Sweet Kendra, rode in the back of the van and revived her with alcohol preps while I drove like a lunatic on those crazy Washington, DC-area roads. I'm glad to be back home where traffic is virtually unheard of.

I will update this blog after her surgery on the 17th. We do covet your prayers for her and for us as we care for her. I continue to have hope that the situation will improve, and I constantly remind her that "it's not forever." We are praying fervently that that is the case. Thanks in advance, all.

Another Surgery

Thought I write another update:  Haley had a tough time recovering from her lumbar puncture (or LP, commonly known as a spinal tap.) She could barely move without excruciating pain for about 4 days. She couldn't even get out of bed to use the bathroom, so we resorted, again, to the bedpan. Dr. H called on Friday to check on her and he specifically asked about the fainting episodes. We realized that they were quite less frequent--about 5-10 times a day versus about 40-60+ times a day before the LP. He had checked the pressure around her spinal cord during the LP and told us that it was high, so he took off some fluid. The fact that her pressure was high, combined with the fact that her fainting was made better when the pressure was lowered, made him realize that 1) her shunt is not working and 2) he needs to do surgery to repair the shunt. So, he has booked her for  surgery on 2/17. It seems a long way off, but I'm sure the days will go by fast.

Dr. H also suspects something called "tethered cord," which is where the spinal cord is pulled down too tightly at the lower spine. This is not uncommon in folks with EDS, and gives symptoms like Haley has been experiencing. In order to diagnose this, there are some tests that must be done, especially bladder tests. Once these are done, Dr. H will decide if she needs her cord "untethered." The tests have been scheduled for 2/12 in Maryland with a urologist who Dr. H uses for many of his patients. I understand that this is not a "fun" test, so prayers would be appreciated.

Based on some conversations I have had with others with similar conditions, I believe that Haley has tethered cord and that if it is corrected, she will have a great improvement in her symptoms. Will you pray that this is the case? We all want to see Haley return to her "normal life" as she is tired of living in a hospital bed and needing constant care.

I have had some wonderful people come and stay with her so that I can continue with my schooling. I have dropped my part-time hours at the hospital to prn (as needed), so I will be working even less. This will ensure that I get my practicum hours in so that I can graduate in May.

Thanks to so many of you for your encouraging messages to Haley and to our family. I especially appreciate all of the sympathy cards we received following the loss of my mother. They are precious reminders that we don't walk through this dark valley alone. We are grateful.

Back to Dr. H

Things have gone from bad to worse for Haley. She has had several episodes of her "fainting" spells coming one on top of the other. She has been taken by ambulance twice to the ER, only to be sent home after getting IV fluids. Sometimes she can go a pretty long time (close to an hour) without passing out; other times I've timed the passing out at every 30-50 seconds. This is so difficult on her and on us. She cannot be left alone at all. Yesterday, she began to vomit while she was passed out. Even though I am a nurse, I was ill-equipped (literally) to make sure her airway was secure. It is really easy to aspirate liquid into the lungs when consciousness is altered.

Phone calls were made to Dr. Henderson and he would like to see her at the hospital in Maryland to do a procedure to check her spinal fluid to try to find a cause for these spells. We will travel to see him on Wed. Jan 21. He will likely keep her overnight and do other tests. Please pray for an answer and a remedy for her.

Our family has also suffered another difficult trial in the past few days. My mother passed away in her sleep on January 5th and we all (except Haley) traveled to Long Island for the funeral. This was a big shock, and very, very difficult for me, especially. I have postponed the start of my semester of grad school until things calm down at home, but I am anxious to get back, as this is my final semester and I am hoping to graduate on May 7th. For now, we all live moment-to-moment, which is a challenge. We are relying on friends to come and stay with Haley so that we can run out to do a quick errand here and there, and we will be hiring folks to stay with her so that we can work and so that I can be at my practicum site.

Will you pray for us? Haley feels so isolated and discouraged. Just last evening, she had several friends stop by for a visit, and it really boosted her spirits. Please pray for:

• Dr. H to figure this thing out and correct it
• For Haley to tolerate the procedure and the travel to and from Maryland
• For Larry and me to have the resources and stamina to care for her
• For friends/family who will offer to help us care for her
• For us to see God's hand in all of this, even when things are so bleak.

As always, thank you for your concern, support and prayers. They are keeping us afloat!

Dr. H, the thinker

Our visit with Dr. Henderson yesterday was a long one. The appointment was for 3 pm, but we waited a long time in the waiting room (he was running behind). We got out of there at 7 pm!  Haley passed out about 6 times in the waiting room in her wheelchair. (Once, I timed her as being out for 12 minutes!) Needless to say, it was a long, tiring day for all.

Dr. H was really stumped by her. He had the disc with the MRI of her head and neck up on the big screen when we got into the exam room. He saw no evidence of anything on the scan that would explain her symptoms. After much thought, including him leaving to go back into his office for about 30 minutes to think (per his NP, we were the only people still there, and he was "thinking"). He believes that she has two things going on:

1. He thinks that these episodes are not fainting at all, but are non-epileptic seizures (also known as "drop attacks"), where she loses muscle tone everywhere. This would explain why she can often hear during these attacks and can quote what was said while she was "out".  He thinks that the cause is due to mast cells activation syndrome. We are not exactly sure what that means, but I know that mast cells are important in immune function and it seems that they can go haywire to varying degrees. He speculates that her mast cells are attacking her brain stem, (his words) and he feels that this can be turned around with medications and a special diet.  He said that he sees this a lot in patients with Ehlers-Danlos Syndrome, which she has, and that he currently has about 100 patients with this. He prescribed many medications to "stabilize" her mast cells and he wants her to follow a low histamine diet (or even a ketogenic diet). This is a lot of information for us to process. I asked where the specialist for this condition is, and he said the best doc is in New York--(road trip, anyone?) We will plan to go up there as soon as possible, but the doctor likes people to be on the meds/diet for at least a month before she sees them.

2. He thinks Haley has "tethered cord syndrome", which is also not uncommon in EDS patients. She has chronic leg pain, neck and back pain, and some odd urinary symptoms. He poked her with a big safety pin, which she barely felt in her lower extremities, and checked her reflexes and muscle strength. He would like some further testing, such as an MRI of her low back and some special tests of her urinary system before he considers surgery to "untether" her cord.

This news was very difficult for Haley, as she was hoping that he could do surgery to help her to stop fainting, just like last time. Her life is so hard now, and she misses being able to do what she wants. To be dependent on others for everything (including bathing) is very demoralizing.

My thoughts are that this condition has been present for a long time. Dr. H listed the "triggers" for mast cells misbehaving, and one of them was structural problems. Haley definitely had a big structural problem before he corrected it, so I think that maybe her previous fainting was due to mast cell problems. Other triggers are stress, some foods, some medications, and environmental factors.

Even though I am in the medical profession, I must admit that I have some research to do on this condition, but for now, I am just trying to be her mom and support her in whatever way I can. We are grateful to you for your support as well. As we were in the exam room waiting for Dr. H to come back, I knew for certain that there was an army of folks praying for wisdom for this man. Our phones were blowing up while he was in with us, as many were calling and texting to find out what he said. We have had many Facebook messages from people saying they are praying for Haley and us. Thank you all for that. We are truly grateful.

Please continue to pray as follows:

• For the "fainting" to go away quickly so Haley can resume her normal life and get out of the wheelchair and the hospital bed in the living room.
• For her to be able to follow the special diet and for it to make a big difference.
• For Dr. H to continue to think about her condition (I understand from his staff that he will continue to think and research long after his patients have left the office).
• For Haley's pain to decrease.
• For our family to know how best to love and support Haley through this difficult time.
• For us to all know God's kindness in a new and clear way.

A Fun Surprise from Haley and Some Other Sweet Ladies

She said that there would be a surprise for me when I got home. She knew that I was worn out from studying for my final exam and working and taking care of her. And she wanted to encourage me. When I pulled into the driveway as I got home from work on Tuesday evening, I noticed the two small Christmas trees on either side of the front door, all lit up. My first thought was that Larry had decided to get a start on the decorating. (I had made everyone aware that I was feeling stressed about having done nothing to get ready for Christmas, and this small gesture was so appreciated by me, as much for the decorating itself as for what it represented.) When I got inside, though, I saw it. There was a fully decorated live Christmas tree in my living room, lit up in all its glory! As it turned out, Haley had masterminded a plot along with our friends Donna, Rebecca and Kendra, to spend the entire day decorating our house. I immediately burst into tears!  These four had worked all day getting bins down from the garage shelf, unwrapping ornaments, connecting lights and putting various decorations around the house. They did all this as Haley sat and directed them. Even the mantel was decorated!

This past month has been incredibly difficult. As you can imagine, to go from having Haley fully functional, driving, working and titrating off of her pain meds like a champ, to her current state of health is more than discouraging. Even in the past few days, she has continued to go down hill. She is fainting about 20 times a day, sometimes even when she's just sitting in her bed. Today, she fainted and fell out of the bed. She woke with her wheelchair on her and in a ton of pain in her head.








We continue to covet your prayers for perseverance, healing and wisdom for Dr. Henderson. We will travel up to MD on Thursday (of maybe Wednesday night) for a special MRI on Thursday at 8 am and will see Dr. H at 3 pm. I know this will be hard on Haley, as she has little endurance. Please pray for that, too. We are hopeful that Dr. H can once again fix whatever is wrong in her neck. I will keep you all posted. Thanks in advance, all.

Seventeen Days

Haley has had a rough several days. Her fainting has gotten more and more frequent. She had another trip to the ER on Wednesday night (into early Thursday morning) for severe headache and continued fainting. It's so hard for her and so stressful for those of us who are around her when she faints. She is black and blue all over from falling on the hard ground. Can you imagine seeing your child constantly hurting herself and not being able to do anything to prevent it? Larry and I are on eggshells whenever she tries to get up.  My nephew, Michael, who is living with us, has been such a help to us.

We spoke with Dr. Henderson on Thanksgiving day, and he said he wants to see her "in an expedited fashion." I got an email this morning from his scheduler, and her appointment with him is December 18th. Seventeen days away. It seems like a long time. Hopefully it will fly by. Dr. H thinks that it's likely that her neck bones have shifted and that's the reason for the fainting, but he also said that it's more than likely two or three things all related to her Ehlers-Danlos syndrome.

We have a wheelchair in the house again, but it's emotionally hard for Haley to use it much. She says it feels like she's going backward to a very sad place. (I sure understand.) We have talked about putting a bed in the living room again, but she flatly refuses. It's too depressing for her. So, we have to escort her upstairs when it's time to go to bed at night.

We have been in touch with the leadership at our church, and are planning to ask for volunteers (again) to check in on her while we are at work.  Boy, it is so humbling and also such a blessing to be cared for like this. But we absolutely can't let her be alone. It's too risky. If she faints with her collar on, her face usually lands with her nose and mouth pressed against the floor making breathing difficult (or impossible), as the neck brace makes it impossible to turn her head to the left or the right.

Thanks, again for your love, concern and prayers for our girl and for us!