2 months old!

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Merry Christmas

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Seizures

I realized this morning that many of you probably don't know that Elianna is having seizures because I haven't said it.
Seizures are a common feature of Zellwegers. Elianna has been having them for sure since we came home from the hospital (and likely before, just no one saw them, it is hard to distinguish in babies, though I did once see what I thought was one).
Sometimes the seizures seem to coincide with the blue episodes/apnea (where she stops breathing), other times not. Her seizures have changed a lot over time too. The current ones have a few different flavours, some where she just looks like she's having a big stretch, some where she flinches repeatedly, and some where they make her cry involuntarily (these are the worst to witness because even though she isn't aware it's still hard to hear and there's not much I can do).
This morning she had a crying seizure at 5am (first time that's happened during sleeping hours) and by 8am she'd had a total of 7 of these crying seizures. That's how I got onto this subject in the first place.
I often go through the day is such a haze I don't have the ability to count how many she's having, because every day blurs together and the rhythm of each day (pump/feed, do a few things before the next one, pump/feed, wash pump dishes and repeat) makes it hard to keep track.
So, today I got a picture of what is going on, since one woke me up and I looked at the clock and then had my alarm for getting up give me a definite time frame to count through. She's had another one as I type this blog post.
She is on an anti seizure medication but we need to give it a couple of weeks to take full effect. So far it doesn't seem to be doing much but we are hopeful it will help.


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roller coaster

As the days go by and I am learning more about Zellwegers through the experiences of other parents, I'm learning one thing. This is a disease that is incredibly unpredictable. We don't know what will happen or when. Peroxisomes are structures within cells that perform part of a cells metabolism processes. This is what makes the condition incurable, it's a not chemical problem, it's structural. There is a host of things that are common in this disease, demyelination of nerves which can effect almost anything, often hearing and vision are impaired, muscle tone and motor development, adrenal insufficiency, seizures and more I can't think of at the moment. The brain was effected in utero and doesn't have all the folds it should. Kids can gain skills and reach developmental milestones and lose them, some even go up and down with skills. It just depends on which nerves are effected and how much and when more demyelination happens.

Most kids with the disease die from respiratory insufficiency/failure.










Elianna's death is no longer as imminent as we first thought it was, but it is still the expected eventual outcome. For the time being she is growing (by that I mean physically, gaining weight) and stable. We are for the time being leading a relatively normal life (if tube feeding and the realities of her low muscle tone, which means she has little to no head control at 10 weeks and can barely move her arms can be called normal), knowing that we will experience a big disruption to this normal at some point in the future. It's kinda like the plans one makes in the last days of pregnancy (only our time frame is unknown instead of a couple of weeks), where every plan is made with the contingency that it could be disrupted by labour and delivery, only instead of anticipation it's dread that we face, because we know it will be hard and incredibly sad when our disruption happens.





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one month old!

Mama guilt

Mama guilt.  

This phenomenon has been the absolute most difficult part of everything that is going on.  I think it would be there regardless of the circumstances surrounding the addition to our family, and I certainly had it even when I was mama to only one kid.  But in this current situation the guilt swallows me up frequently.  

If you are of the praying persuasion, this is something you can pray for.  I feel torn in half about caring for both my kids, especially because their needs seem so big amidst all this stress.  

This is the thing I keep working to release.  I am doing my best, that is all I can do.  I can not and will not get it right every time.  I'm learning that this is how it has to be, that it is ok to not get it right, to struggle with the balance of meeting multiple people's needs.  But it should not be something that drowns me in despair.  When I mess up or get is wrong, it doesn't mean I don't love my kids, and it doesn't mean they won't know that I love them.  

Good bye to mama guilt.  The task master I don't need and was not meant to have.  I will try each day to look toward God as my example of a parent and recognize that He doesn't want me to live under the weight of this guilt.

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11 days old - photo shoot

Thankfulness

From Jesus Calling:

November 24

Thankfulness takes the sting out of adversity. That is why I have instructed you to give thanks for everything. There is an element of mystery in this transaction: You give Me thanks (regardless of your feelings), and I give you Joy (regardless of your circumstances). This is a spiritual act of obedience—at times, blind obedience. To people who don’t know Me intimately, it can seem irrational and even impossible to thank Me for heartrending hardships. Nonetheless, those who obey Me in this way are invariably blessed, even though difficulties may remain.

Thankfulness opens your heart to My Presence and your mind to My thoughts. You may still be in the same place, with the same set of circumstances, but it is as if a light has been switched on, enabling you to see from My perspective. It is this Light of My Presence that removes the sting from adversity.

Always giving thanks to God the Father for everything, in the name of our Lord Jesus Christ.
—Ephesians 5:20

Give thanks to the Lord, for he is good; his love endures forever.
—Psalm 118:1

Blessed are those who have learned to acclaim you, who walk in the light of your presence, O Lord.
—Psalm 89:15


This devotional book has had a lot of impact on me lately. I'm not sure I've been able to be 'thankful' in our current circumstances, it feels more like a calm acceptance and willingness to walk this road set before me. I can say there's been moments of joy and a general sense of well being. I will say that we do feel covered in peace and carried in His arms during this difficult time. God has made sure His people are surrounding us and caring for us in many different ways, practically and otherwise. For this, I am so incredibly grateful. This road is one I could not walk alone.

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Cancelled

I had a post that got lost. Sigh. Here's the short version. God is certainly at work and orchestrating events for us. Tuesday was our meeting with the anesthetist to review if Elianna could have surgery successfully. That Doctor also happens to be a pediatric palliative care doctor. So, he was able to speak with us on a big picture level and give me a real sense of peace over our decisions for Elianna's care. Perhaps I will write more about that again later.
For now, we are postponing surgery because Elianna's breathing is potentially too weak. I'm following my gut on this, because I'm learning all the time that it has some value and I was really unsettled after meeting the surgeon.










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Surgery scheduled

So, today was a pre op appointment for E to schedule a surgery for the insertion of the GT (permanent feeding tube) tube. 

Tuesday is a meeting with anesthetist. We aren't sure if they will consider her fit for surgery but will have to see. We are hoping for a yes but would ask for wisdom in the person making that call.

Surgery will be this Thursday at 7:45am. 

Praying for peace and that E doesn't experience much pain. It is always a question with how much pain she is experiencing. It breaks their hearts not to know. 

From me,

I pray for my friends and try to imagine what it's like to love someone knowing they could be taken from you any second. 

We all face that question some days. But, it's much more real for my dear friends. 

And Milena, let's not forget about sweet, beautiful, amazing and brave Milena. 

May your prayers continue to carry her. 

Shalom 

Most know that the Hebrew word shalom is understood around the world to mean "peace." However, "peace" is only one small part of the meaning. "Shalom" is used to both greet people and to bid them farewell, and it means much more than "peace, hello or goodbye".... Hebrew words go beyond their spoken pronunciation. Each Hebrew word conveys feeling, intent and emotion. Shalom is more then just simply peace; it is a complete peace. It is a feeling of contentment, completeness, wholeness, well being and harmony. According to Strong's Concordance 7965 Shalom means completeness, wholeness, health, peace, welfare, safety soundness, tranquility, prosperity, perfectness, fullness, rest, harmony, the absence of agitation or discord. Shalom comes from the root verb shalom meaning to be complete, perfect and full. In modern Hebrew the obviously related word Shelemmeans to pay for, and Shulam means to be fully paid. Of course, there is only one way to find TRUE shalom - and that is in the Word of YHWH. Many search for fulfillment, happiness and contentment in material possessions, money, sex, entertainment, etc. But those things do nothing to fill "that little hole in our soul" that only GOD can fill! Those things only serve to distract and prevent us from finding true peace...the shalom that can only come from Him who created and put all things into place. If you'll recall, Yeshua is called Sar shalom, Prince of Peace, which perfectly describes the ministry and personality of our Messiah (Isaiah 9:6). Luke 2: 8. At this time shepherds were there in that region were they were lodging and keeping watch there at night over their flocks. 9. And behold a Messenger of Elohim came to them. And the glory of Master YHWH shone upon them and they feared with a great fear! 10. And the Messenger said to them, "Do not have fear, for behold I announce hope to you! A great joy which will be to the whole world. 11. For today is born to you in the city of Dawid the Savior who is Master YHWH, the Mashiyach.[1] 12. And this is a sign to you; you will find an infant who is wrapped in swaddling clothes and lying in a manger." 13. And at that instant the great host of heaven appeared with the Messenger, glorifying Elohim and saying, "Glory to Elohim in the highest, and on earth peace and good hope to the sons of men. (AENT) [1] One of the most powerful statements about YHWH and His Mashiyach in the entire Aramaic NT. YHWH is the real Mashiyach, who chose the vessel of Y'shua the man. However, within Y'shua the man is an occurrence of the One Divine Nature of YHWH, also known as the Ruach haKodesh (Holy Spirit). This is the literal meaning of the fullness of YHWH dwelling inside Mashiyach, and in accordance with Tanakh prophecy (Isaiah 53:1, Zechariah 12:10). The divine and human natures exist separately yet side-by-side within Y'shua. In other words, the word shalom is a mighty blessing on several levels! Proverbs 18:21 tells us there is life or death in the power of the tongue. Therefore, whenever you employ the word "shalom" you are speaking into someone's life all the wonderful things that shalom means! So, from us at The Refiner's Fire to you: Numbers 6:24-26: YHWH bless you and keep you. YHWH make His face shine upon you and be gracious to you. YHWH lift up His face upon you and give you SHALOM. In the name of Yeshua haMashiyach SAR SHALOM - the Prince of Peace. And take a look at these tidbits! Each of the 22 letters of the Hebrew alphabet has a special meaning and a story to tell. The Hebrew letters spelling the word "shalom" are Shin Lamed Vav Mem sofit. Taken together, and reading from right to left, they spell SHALOM. SHIN is the 21st letter of the Hebrew alphabet and it has a numerical value of 300. SHIN means TOOTH, YEAR, REPEAT, TEACH. SHIN makes the sound SHHHHHH when the DOT is on the right, and SSSS when the DOT is on he left. Shin is one of 7 letters which may be adorned with Tagin, or little crowns, in the Torah, Tefillin or Mezzuzah scroll. LAMED is the 12th letter of the Hebrew alphabet and has a numerical value of 30. Lamed makes a sound like the L in Like. Lamed means both TO LEARN and TO TEACH. The Lamed is the tallest and most majestic of all the letters. Since LAMED means LEARNING, it would seem that this would constitute a direct connection between LAMED, LEARNING and YHWH. VAV is the 6th letter, has a numerical value of 6, and is constructed of a single vertical line. Vav means HOOK and makes a sound like the V in Victory. When used as a vowel, Vav can sound like a long o or a long u. Translated it means AND. It is the GREAT CONNECTOR. Vav appears in the Four Letter name of YHWH our Elohim - Yud Hey Vav Hey. MEM is the 13th letter of the Hebrew alphabet and has a numerical value of 40. Mem makes a sound like the M in Middle. But according to the ancient kabbalistic text Sefer Yetzirah, the MEM hummmmmms! Mem is usually associated with the word mayim - WATER or Eemah - MOTHER. Mem is composed of a Caf (20) and a Vav (6) = 26, which have the same GEMATRIA as the Yud (10) Hey (5) Vav (6) Hey (5) = 26, name of God - the holiest of Name of God. The first word in Torah is Bereshit, meaning: A BEGINNING. If you divide Bereshit into bara and sheet, it means HE CREATED SIX (sheet is 6 in Aramaic) Six what? The 6 days in which creation was completed (Zohar 1:3b. 13)....