Cyber Knife, number 2

My almost 18 year-old daughter completed her second Cyber Knife radiation treatment today. She will receive 5 total treatments for her brain stem cavernous angioma.

I am so proud of my girl! She was very brave--- no complaining whatsoever. The treatment is pain- free. The only difficulty she is having is sleep. Dexamethasone (Dex) really makes rest difficult.

My daughter is taking a total of 8 m.g. of Dex, along with a nausea medication (Ondansetron HCL) and another medication to help her stomach tolerate the Dex (Ranitidine). So far, so good!

We are still waiting to hear from Valley Radiology to find out when my 15 year-old son will have his spine MRI. This will be the second MRI for my son. Mr. D. was diagnosed with several brain lesions, but they are not symptomatic. He has none in the brain stem, thank the Lord! The brain lesions will just need to be monitored, unless he develops seizures or headaches. Unfortunately, upon neurological exam, my son's reflexes were "brisk." This might indicate a possible spine lesion. I do understand that the reflex exam can appear abnormal if the patient is anxious--- I so pray that anxiety is the reason his test was abnormal!

Regarding anxiety, mine is pretty elevated! I am very grateful that I have a church (Scottsdale Bible) that offers a Stephen Ministry. I am meeting with a counselor tomorrow, and am so looking forward to praying and talking with someone. Chronic illness kind of burns out your friends... (sorry guys).

Rescue Me, Rescue Her

For a long time, I could not even pray for healing. I was so overwhelmed by the trial and the emotion of all that a diagnosis of cavernous angioma entails. But when my daughter was diagnosed with the same disease, I had renewed motivation to be well. I needed to persevere. I wanted to be able to care for her and to encourage her in her walk through potential misery and ongoing trial.

My daughter starts radiation tomorrow. This will start a series of 5 sessions that will, God-willing, cauterize the lesion and prevent future bleeding. We would appreciate prayers for her, and for our family.

Psalm 31

For the director of music. A psalm of David.

1 In you, Lord, I have taken refuge;
    let me never be put to shame;
    deliver me in your righteousness.
2 Turn your ear to me,
    come quickly to my rescue;
be my rock of refuge,
    a strong fortress to save me.
3 Since you are my rock and my fortress,
    for the sake of your name lead and guide me.
4 Keep me free from the trap that is set for me,
    for you are my refuge.
5 Into your hands I commit my spirit;
    deliver me, Lord, my faithful God.

6 I hate those who cling to worthless idols;
    as for me, I trust in the Lord.
7 I will be glad and rejoice in your love,
    for you saw my affliction
    and knew the anguish of my soul.
8 You have not given me into the hands of the enemy
    but have set my feet in a spacious place.

9 Be merciful to me, Lord, for I am in distress;
    my eyes grow weak with sorrow,
    my soul and body with grief.
10 My life is consumed by anguish
    and my years by groaning;
my strength fails because of my affliction,[b]
    and my bones grow weak.
11 Because of all my enemies,
    I am the utter contempt of my neighbors
and an object of dread to my closest friends—
    those who see me on the street flee from me.
12 I am forgotten as though I were dead;
    I have become like broken pottery.
13 For I hear many whispering,
    “Terror on every side!”
They conspire against me
    and plot to take my life.

14 But I trust in you, Lord;
    I say, “You are my God.”
15 My times are in your hands;
    deliver me from the hands of my enemies,
    from those who pursue me.
16 Let your face shine on your servant;
    save me in your unfailing love.
17 Let me not be put to shame, Lord,
    for I have cried out to you;
but let the wicked be put to shame
    and be silent in the realm of the dead.
18 Let their lying lips be silenced,
    for with pride and contempt
    they speak arrogantly against the righteous.

19 How abundant are the good things
    that you have stored up for those who fear you,
that you bestow in the sight of all,
    on those who take refuge in you.
20 In the shelter of your presence you hide them
    from all human intrigues;
you keep them safe in your dwelling
    from accusing tongues.

21 Praise be to the Lord,
    for he showed me the wonders of his love
    when I was in a city under siege.
22 In my alarm I said,
    “I am cut off from your sight!”
Yet you heard my cry for mercy
    when I called to you for help.

²³ Love the Lord, all his faithful people!
    The Lord preserves those who are true to him,
    but the proud he pays back in full.
²⁴ Be strong and take heart,
    all you who hope in the Lord.

Of Anniversaries and Angiomas

Last night, my husband and I went out to dinner (thanks, Mom) to celebrate our 21st anniversary. How amazing that we haven't killed each other by now! But in the course of dinner and conversation, we inevitably talked of this lovely disease--- cavernous angioma-- and how is frustrates me to to end. I expressed my desire to find an expert in the field who could answer questions, and give me some perspective to the disease.

That's when my husband made an interesting comment that echoed in my mind all through the night. He said, "This isn't a disease to understand or manage. There's not much to do, and Dr. Willis has answered your questions. All that can be done is crisis intervention."

Crisis intervention. And I guess that's right. A doctor can give you seizure medications if needed. A doctor can track progression or regression of the disease. You can take steroids to reduce edema. A patient can opt for surgical resection or radio surgery. But really, there is not much to do but crisis intervention. There is no cure; there is nothing that can prevent the disease. For a person who likes answers---concrete answers-- this is difficult to swallow.

I have said to another friend that this disease is feels like you're waiting for the other shoe to drop. You just never know when the next wave of crisis is going to threaten you. You try to relax and trust the Lord, but your body defies you--- you're on constant alert.

Because the brain is plastic or dynamic, symptoms constantly change. One day feels like perhaps you're making some overall progress; the next day feels like the numbness has worsened. For emotion-driven people, like myself, not focusing on how you feel is very difficult. Telling yourself that you can not trust your symptoms seems so counter intuitive. How do you go on with the day, acting like nothing is wrong when in reality, you're treading water, emotionally-speaking?

The only way I can try to understand life right now is by viewing this whole dilemma through the Word. We are told to walk by faith, not by sight. We are told not to fear. We know we are never going to really understand all that we want to understand--- and that has to be okay. It has to be okay.

But this does bring me to another point of this post. If you do have cavernous angioma, it is imperative that you sign up on the international registry (angioma.patientcrossroads.org) and get genetic testing. That way, you can participate in the gathering of information and on going research to aid in the hopeful discovery of a cure or treatment for this dreaded disease.

Until medical progress is made, and definitive treatments are discovered, I (we) must remember the only true hope we have (or really ever need) is the Lord. He must be my hope, even if a cure is found. So I wait. He will give me what I need.

Psalm 130

A song of ascents.

1 Out of the depths I cry to you, Lord;
2     Lord, hear my voice.
Let your ears be attentive
    to my cry for mercy.

3 If you, Lord, kept a record of sins,
    Lord, who could stand?
4 But with you there is forgiveness,
    so that we can, with reverence, serve you.

5 I wait for the Lord, my whole being waits,
    and in his word I put my hope.
6 I wait for the Lord
    more than watchmen wait for the morning,
    more than watchmen wait for the morning.

7 Israel, put your hope in the Lord,
    for with the Lord is unfailing love
    and with him is full redemption.
8 He himself will redeem Israel
    from all their sins.

Frustration

I have heard it said that if you feel frustrated, it is because you are butting heads with God's will. If that is the case, I am so thankful He is a forgiving and understanding God! I sure need Him today (and everyday). I am frustrated, there is no denying it.

Our family arrived home yesterday, after a very pleasant 8 day impromptu California vacation. As usual, we heaped a bunch of activity into the time--- 2 days at Legoland, 2 days at the beach, and lots of family dinners. For the most part, the time was wonderful. We only had one bike wreck and a sprained ankle to complicate our days. Additionally, the house was nice and clean upon our return, thanks to a very good house sitter. The frustration entered the picture yesterday afternoon.

As other cavernous angioma sufferers know, symptoms come and go with no apparent reason. I was feeling just fine, perhaps a little tired from all of the activity, but really pretty okay. But after I got out of the car, my head just didn't feel right. I attributed the imbalance to fatigue, to the motion of the car, and to dehydration. And truly, perhaps those all contributed to the feeling. Yet, even this morning, I am just "off." A bit dizzy--- not quite disoriented. A very mild headache--- not even bad enough to desire Tylenol. Of course, whenever I don't feel absolutely normal, the thoughts start flying: Have I had another bleed? Am I just tired? Are all these symptoms psychosomatic?

You may say, "Well, why not go to the doctor?" That sounds like an appropriate response, but in reality it is not practical. Twice in the past, I have called the doctor, complaining of symptoms such as the symptoms I'm having. And twice, the doctor gave me an MRI. And twice, that MRI was clear: no sign of a new bleed. In hindsight, I understand that steroid-tapering can cause an increase of neurological symptoms. I am still on Prednisone, so steroids still may be an issue. Sigh. Even my physician husband basically told me to ignore my symptoms--- that the brainstem is dynamic-- and that symptoms will change day-to-day.

To those worriers out there, I do have a follow-up appointment in a month. Unless I have an obvious or "major health challenge" that would prompt me go to the hospital, a month is about a quick as a patient can even get in to see a neurologist. It is crazy.

And therein lies the frustration of having cavernous angiomas. Symptoms change--- sometimes daily... sometimes hourly. You never know when to call the doctor or go to the doctor. Sometimes your numbness and weakness is not a huge issue. And then there are times that the symptoms dampen your spirit and douse your enthusiasm for living. Sometimes it is difficult to remain focused and optimistic; it might be easier to just go back to bed and hopefully wake up feeling better.

Bible verse memorization was never a real strength of mine, but early on I did manage to memorize Proverbs 3:5-6 (NIV):

Trust in the Lord with all your heart

    and lean not on your own understanding;
 in all your ways submit to him,
    and he will make your paths straight.

Fighting this disease it not a matter of choosing optimism; it is more a matter of surrendering yourself to God and trusting that He will allow you to experience life as He desires. I don't claim to understand much of life right now, but I do know that if I continue to submit my frustrations to Him, He will direct my path. He will show me what I need to do, and He will lift my spirits. I am in a battle, or sorts. Much of the battle is in my mind--- my thoughts and symptoms are my foes. I depend upon you, Oh Lord, to lift my head!

Psalm 3

A psalm of David. When he fled from his son Absalom.

1 Lord, how many are my foes!
    How many rise up against me!
2 Many are saying of me,
    “God will not deliver him.”[b]

3 But you, Lord, are a shield around me,
    my glory, the One who lifts my head high.
4 I call out to the Lord,
    and he answers me from his holy mountain.

5 I lie down and sleep;
    I wake again, because the Lord sustains me.
6 I will not fear though tens of thousands
    assail me on every side.

7 Arise, Lord!
    Deliver me, my God!
Strike all my enemies on the jaw;
    break the teeth of the wicked.

8 From the Lord comes deliverance.
    May your blessing be on your people.

Blessings in the Madness

Even though life has been quite a trial, I must say there have been a number of blessings along the way. Even the most difficult day is replete with God's goodness and love for us. We do need to have eyes to see this goodness, as well as an open heart to accept what He does for us is always with our good in mind. It is also essential to keep eternity at the forefront of your thoughts. Our ways are not His ways.

Because a keep a journal (which has grown tremendously in length since November), it is easy to see many answered prayers and examples of God's goodness. The most obvious blessing is just that I am alive. The bleed stopped--- if it had been larger, or had bled quickly I would most likely not be here singing the praises of God. For a while, I was honestly disappointed that I could not just go to heaven and be with Jesus. Longing of heaven caused considerable sadness in me for a while, and I had to come to grips with the fact that it was apparently not my time. Of course, then I struggled with guilt that I wanted to leave.... Oh, the emotions I went through in just a few weeks! I am so thankful that I have a Big God who can deal with me, and all my emotions and thoughts.

The second blessing is that I am only nominally disabled. Had the bleed been larger, I could have had serious and significant disabilities. As it is, the only thing I simply can not do is run. The body will not cooperate at all. I am numb and weak on my left side, but I can still do most things--- just a bit more slowly. I am so grateful I am not sports-oriented; not being able to run does not ruin my day. The only issue that has become a bit more difficult is walking up and down the stairs of our new house (we moved in 2 weeks before my bleed). We haven't been here long enough to sell it, so I just need to deal with the hardship. But looking at the positive: I am sure the stairs have made my weak side stronger.

A third blessing I can think of is that the ordeal brought my husband and I closer. When we did not know if I was going to live, my husband and I spent many sweet times together. He would kneel at my bedside and weep--- verbalizing his struggle. My husband cried out to the Lord to save me, but he also surrendered his desires to the Lord's. I have never been more proud of my husband than in that moment when he finally surrendered me to God. It is such a difficult thing for a Type A physician to admit there was nothing he could do--- he had to trust God and let me go, it that was God's plan.

A fourth blessing relates to the third blessing. For years, I have prayed, "Lord, do whatever You need to do to bring my husband closer to You." My husband is a good man and a faithful church goer, but he has not always had a passionate relationship with Jesus Christ. Now, the relationship is so much stronger and more trusting. My husband reads the Word everyday now, and he has even led all of us in a family Bible study based on the book by David Jeremiah,"A Bend in the Road."So much growth can take place during crisis if we allow the Holy Spirit to minister to us. (By the way, Jeremiah's book is quite good and helpful.)

A fifth blessing is for my bleed itself. If I had not had a bleed, we never would have thought to have our biological kids tested. The MRI for my daughter could have very well saved her life. Sure, she will have medical issues to monitor throughout life, but this should not destroy her life. I also have 2 siblings, with a total of 6 nieces and nephews. They will all need to be tested, as well. Who knows who else will benefit from the crisis God allowed me suffer? Sometimes, what we perceive as bad is truly for our good. God works all things together for our good and for His purpose, if we belong to Him.

The final blessing that comes to mind is still tough to handle. This is not popular knowledge, but my husband and I had been in process to adopt a little girl from China. In fact, she was most likely due to come home this spring or early summer. All of our fees were paid, and we were just awaiting a travel date.

If is interesting to me that before and throughout the China process, I prayed, "Lord, if this little girl (we had named her Mia) is not meant to be in our family, do whatever You need to do to interfere with the process, because You know I'd adopt everyone, if I could."Soon after my stroke, it was very apparent to me that the Lord had interfered with this adoption process. We struggled with making a decision with whether or not to continue in the adoption process. We made the decision over a 3 month period---we had no peace continuing the process. In fact, the longer we waited, the more anxiety and discomfort we experienced while praying for wisdom. I had to accept that I now had limitations. Life was not the same; I was not the same person who initiated all the paperwork. It was another item to grieve and to let go of.

When we signed papers to relinquish our little girl, we found out that another family stepped forward almost immediately, asking for her paperwork. Could it be that we just stood in the gap for her? Perhaps we weren't every meant to be her forever family, but we had basically tied up her paperwork for two years--- was that time needed for her real family to step forward? I don't know. Perhaps the Lord was just testing us to see if we would actually proceed with what we thought was His call to adopt? Was He just seeing if we were willing?

So much of life we don't understand. Like the adoption journey. Was that really necessary, God? I still don't really understand why we had to go through all the financial strain and paperwork, etc--- and then not end up with a child. I just have to trust that this little one was not meant for our family, and that God has another plan for her--- she is to be a blessing for someone else. I have to cling to that trust---even when it seems so unreasonable. There must be a blessing somewhere....

Whatever crisis you may be in, remember to look up. Is there a lesson God is trying to teach you? Is there a blessing tucked away in the apparent nightmare? God may not reveal His purposes in every situation, so I consider myself very blessed that I am able to perceive a few blessings in the madness. I am just so thankful that this world is not all there is.

Attachment Issues Rear Their Ugly Heads

Most members of our family have gone through pretty normal ups and downs. Even with a good relationship with the Lord, everyone has gone through moments of anger, sadness, depression, or grief. It's just part of the process--- learning to live a new normal.

But for adopted children who were already having some puberty-triggered attachment issues, the grieving and adjusting process was pretty ugly. Life is pretty good now, 7 months post bleed. But those early weeks and months made every fear and anxiety come busting out. We had not experienced such negative behavior in our adopted kids since they came home. Stealing and lying again became common behavior. We had lots of yelling, fighting, bullying, and back talking. Fortunately, we were in family therapy already for attachment issues; this just gave the therapist more to work with.

In hindsight, it is very clear why our kids went ballistic for a few months. They had already lost so much: a mom and/or dad, a country, and everything they had ever known. Where they about to lose everything again? We tried to be sensitive about their fears, but in reality it is difficult to calm someone down if you are not calm yourself. My husband and I each had some really down times--- it is impossible to hide your deep struggles from your kids. They are much more perceptive than you give them credit for. So while they saw Mommy struggle, they pulled away. They didn't want to get hurt again; the thought of losing another mom was just too much to consider.

Unfortunately, the kids could never really talk about all that was going on. They mouthed that "Mom is sick; we are fine." The kids pretty much went back to trying to take care of themselves, as if preparing to be left again. We just prayed for healing and for patience,  and I tried to cry in private. I had to try and ignore the rejection and seemingly inconsiderate ways; I knew they were just trying to reject me first--- thinking that I would be rejecting them if I died. This was a very difficult time in our family. I truly felt attacked by the Enemy. The older kids were angry at the adoptive kids--- how could they treat Mom so meanly when she's sick? At least the older, bio kids could talk about all the issues and try to understand the root of all the negative behavior swirling around them.

I imagine that at every negative or challenging event in life, our adoptive kids will have a tendency to revert back to old behaviors. As if life weren't difficult enough--- then they become their own worst enemies.

Once the kids could see I was smiling again and that I was regaining my odd sense of humor, they began acting normally. Indeed, one of my kids is considerably more affectionate and loving now that we have all gone through this rough spot. (and this kid was the one with the worst behavior.) Pain does have a way of making you strong--- if you can just manage to endure the pain long enough to get to the good part.

When it Rains, it Pours

Did I mention that this horrible condition is hereditary? Unfortunately, having familial cavernous angiomas tends to run in Hispanic families (I am 50% Mexican). There are thought to be 3 different genes that cause the condition, and they are autosomal dominate. With a parent with multiple lesions, the offspring have a 50% chance of developing this disease. Symptoms can develop at any age, but the highest percentage of people develop symptoms in their 20's and 30's, if I remember my facts.

We waited until I was finished with radio surgery before battling with insurance to get our biological children tested. We finally got the test results just this last month. It was not good.

My eldest son got a clear test. Praise God! But even with a clear test, he will need to be scanned every year or two for the rest of his life unless he chooses to have genetic testing. Angiomas can grow at any point in life.

My youngest biological son has multiple angiomas scattered throughout the brain. None of them, however, are in an eloquent area of the brain. He will need to be followed by a neurologist and scanned with some frequency. The new was upsetting to us, but we realized it was not necessarily horrible news.

Then comes my daughter--- my sweet little girl. She just finished high school and has her entire life ahead of her. Her MRI revealed a brain stem angioma and multiple other angiomas throughout the brain. We had another crisis to face.

I have never become hysterical over anything in life. I tend go under react to bad news. But this time was indeed different. When I read the report, all I could do was to sob--- deep, wretched sobs. I tried to call my husband, but he was unavailable. My poor mother had to take my call--- just thinking about it makes me cry again. It was a moment I will not forget.

My daughter is scheduled for radio surgery on June 23, 2014.

Did I tell you you better be in the Word before crisis hits? Please take my word for it.

What Got Me Through

The only thing that got me through these difficult day was my faith in Jesus--- pure and simple. When I was so tired and literally could not read, all I could do is cry out to Jesus for mercy.

The only two books I could read on a good day were

I realize there have been many positive and negative comments about the first book--- but all I can say about it is that many of the devotions were exactly what I needed to hear at the moment. I realize Sarah Young wrote this book while in the midst of her own health crisis, and the readings definitely ministered to me during my health problems. It was like having a counselor--- someone could relate to the pain of life and give you the appropriate medicine (Scripture).

And the second book? I haven't read a negative comment about it. Streams of Desert is right on, theologically-speaking, and provides tremendous encouragement and insight to a person in the midst of suffering. Thank you to my dear friend who sent it.

These books might be great, but bottom-line is that if you don't have the Word of God buried in your heart BEFORE tragedy strikes (and it will strike at some point), you will flounder. I have been a believer and follower of Christ for almost 20 years, and this crisis still threw me. I can't imagine what I would have done without knowing the Truth.

Stupid Steroids

I finished Cyber Knife radio surgery on April 4, but needed to continue steroids to counteract any possible edema from the radiation.

I had been on steroids (Dexamethasone or Dex) since December, and they were taking a toll on my body and on my family. I expected to gain weight, so that wasn't the most wretched part of the medication. I lost hair--- patches of it--- and even that wasn't the worst thing. What was difficult were the psychological effects--- depression, anxiety, and apathy. I literally could not think. My head felt like it was literally spinning. I could not multi-task (I started making dinner at 3:00 p.m. just to have it ready by 6:30 p.m.!) I developed Cushing's Syndrome (odd weight gain--- round face, hump on upper back, stomach bloating) and had horrible insomnia. I did not dare drive, and didn't for 7 months. The only positive thing I can think of over these months is that it gave me hours of free time, starting at about 2:30 a.m.! Getting through the rest of the home schooling day was difficult--- I was so tired. It is only by the grace of God that I could continue taking care of my family.

I should not have needed to be on Dex for as long as I was--- but it wasn't the doctor's fault. I do have a crazy system. I have never been one to do well with medication, and avoid it whenever possible. If anyone can have a paradoxical effect with medication, it will be me! Every time! Unfortunately, every time Dr. Kresl tried to lower my dose of steroid, I would develop neurologic symptoms. Twice, I went in for repeat MRIs, thinking I had experienced another bleed. But it was not the case--- and I was very embarrassed. I felt like a hypochondriac. It wasn't until later that I understood that for some people, if tapering isn't done very slowly, they can develop what seem to be neurological symptoms--- but they aren't. The symptoms are caused by the body reacting to a reduction of steroids. It is almost like your body going though a type of withdrawal. It is wretched. All of this may have been further aggravated by the fact that I am hypo thyroid, and on medications for that condition.

One horrible day, I had an appointment with my endocrinologist for routine thyroid follow-up. I remember sitting in my endocrinologist's office saying, "I feel like I'm losing my mind!" That was the last day of Dexamethasone for me. This doctor knew me well enough--- she immediately knew something was amiss. Dr. Bailey told me that some people become psychotic on Dex. Great. I sure didn't want to become that person, but I could sense a dramatic change in my personality. Dr. Bailey, after consulting with Dr. Kresl, switched me to Prednisone, and started a new tapering regime. From then on, Dr. Bailey handled my medication. Thank you, Jesus. That change in medication started my healing.

Although tapering on Prednisone was still not easy, I felt significantly better just getting off "Demon Dex" as I now like to call it. Within 2 days of being on Prednisone, I felt like I had been "unplugged." I could now rest and sleep. To this day, I am on 5 m.g. of Prednisone, waiting for my adrenal system to repair itself. And I am feeling much better! I haven't lost a bit of weight and that upsets me, but I figure I'd rather be alive and fat than dead and thin!  Dex may have been necessary, but it was the worst part of my entire treatment.

Cyber Knife Radio Surgery

In total, I had 7 radio surgical procedures at Phoenix Cyber Knife. The procedures are actually a bit anti-climactic... which I guess is good! All of the time and energy goes into planning the surgery; the day of the surgery is just a matter of "pushing a button," so to speak. You have to love technology!

As a patient, this is the best type of surgery possible. All I felt during the procedures was a bit of burning--- probably not from the radiation, but from my neck muscles being very tight-- as I was literally locked into a particular position for 45 minutes or more at a time! I simply got on the treatment table, and the tech fitted the face mask to the table. (this would be the only part of the procedure that might cause a bit of anxiety for someone claustrophobic--- you literally can not move). The locking mask ensures that I stay exactly where I am supposed to be. Thankfully, I am not too squeamish, although due to a steroid-induced high heart rate, I did use some Ativan during a few of the procedures.  Sometimes an elevated heart rate is not a issue of "mind over matter."

The treatment table itself adjusts throughout the radiation treatment. The table senses if you have moved at all--- it automatically repositions you into the perfect position, and will not permit the machines to treat unless you are in that precise position. The "robots" (machines that emit the radiation) move around you.You can hear them making their weird robot sounds--- moving into position.  I chose to close my eyes because it was just a bit disconcerting to open your eyes and see a large white machine within a couple feet of you--- aiming radiation right at you!

It is an amazing procedure, honestly. The patient feels no pain during the procedure, and no pain or time lost in recovery. I did feel a bit dizzy getting off the treatment table--- but literally, that was all. No down time to brain surgery. Go figure.

If I were a wealthy woman, I would invest in this amazing medical technology!

This is a photo taken from the Phoenix Cyber Knife Facebook page. Imagine several of these machines aimed at you!

Dr. Kresl of Phoenix Cyber Knife

First, I want to make one thing very clear: I really like my doctors. I have complete trust and confidence in their skills and abilities. Dr. Kresl and Dr. Willis both have excellent communication skills and caring support staffs. The doctors are not condescending; they treated me with complete kindness and honesty. They admitted that my treatment was no guarantee of healing; I appreciated the frank discussion. Thankfully, they are also very experienced in the treatment of cavernous angioma bleeds. I felt very comfortable with our decision to move forward with Cyber Knife radio surgery for my brain stem lesion. My husband and I knew there were no guarantees to the outcome of radiation, but we both agreed the treatment was better than doing nothing.

My treatment would include steroid use and 5 sessions of high-intensity radiation used around the perimeter of the lesion. To prepare for the treatment, I needed to be on steroids until the edema surrounding the lesion was reduced. It was necessary to get the lesion down to the smallest possible target area.

After a few weeks of steroid therapy, I needed a planning MRI. This scan is used in conjunction with a CT simulation to design an exact plan for radio surgery. I needed to have a plastic mask made---a mask specially fitted for my face-- that was also used in the planning portion of the surgery. The mask also has holes on the side so that during the radiation, your head is held in the mask.

I am not claustrophobic, thankfully. The plastic mask is quite fitted, but because it has holes in it I was not too uncomfortable.

Unfortunately, my brain stem treatment had to be postponed because the planning scan showed I had experienced 3 additional bleeds. I had 2 new bleeds in the supratentorial region of the brain; I also had a second bleed in the brain stem. I was completely unaware of these other bleeds, as I had no significant symptoms from any of them. It is by God's grace and mercy that I had enough steroids in my system--- I theoretically should not have survived another brain stem bleed.

On January 23, 2014 and on February 12, 2014, I had both left and right supratentorial lesions treated with Cyber Knife radiation. I remained on steroids during this time.

It is my understanding that Cyber Knife is generally not recommended for treatment of cavernous angiomas. Dr. Willis says that regular (invasive) surgery is generally recommended for accessible, symptomatic lesions. In my case, because all of my lesions are inoperable (too deep to access), Cyber Knife is used to cut off the blood supply to the lesion so that it shrink over time. In the case of a brain stem lesion, the radiation treatment serves to cauterize the lesion so that it theoretically reduces the risk of re-bleed. Since these are fairly new procedures, not a lot of information is available on re-bleed rates.

Neurosurgical Evaluation

After many phone calls and extensive research, my husband I a decided to consult with Byron Willis, M.D. of Arizona Neurology and Spine.

Because of the delicate location of the lesion, Dr. Willis told us the cavernous angioma could not be surgically removed without causing neurological damage. The only options for my case were either no treatment or radio surgery. The radio surgery (high intensity radiation) would act to cauterize the perimeter of the lesion, theoretically reducing the incidence of another bleed.  I was told brain stem lesions have a propensity to re bleed, and that if my lesion bled again, it would most likely be fatal. As it was, the lesion was so large that it occupied the entire Pons of the brain.

Dr. Willis did put me on Dexamethasone--- a heavy-duty steroid-- to reduce the edema surrounding the lesion. I did feel significantly better going on the steroid. The "falling backwards" feeling stopped within a few doses of the medication.

Dr. Willis did advise us to get all of our biological children screened for this genetic disorder.

Dr. Willis also referred me to Dr. Kresl of Phoenix Cyber Knife. 

Angioma Alliance

The best information I have found on Cavernous Angiomas is the website Angioma Alliance. This site contains a wealth of information on the disease, and also has links to helpful resources for the newly diagnosed patient.

The following is copied directly from the AngiomaAlliance.org

About Cavernous Angioma

Cavernous angiomas are vascular lesions comprised of clusters of abnormally dilated blood vessels.  These lesions can be found in the brain, spinal cord, and, rarely, in other areas of the body including the skin and retina. Multiple names refer to this condition:

• cavernous angioma 
• cavernous hemangioma 
• cerebral cavernous malformation (CCM) 
• cavernoma

Cavernous angiomas are typically described as having a raspberry-like appearance due to their composition of multiple bubble-like structures called caverns.   Each cavern is filled with blood and lined by a specialized cell layer called the endothelium.  Endothelial cells are the basic building blocks that work in conjunction with other cell types to form blood vessels.  In the case of cavernous angioma, the bubble-like caverns are grossly dilated vessels that leak due to defects in the endothelial cells and due to the loss of other structural components that are required for normal vessel walls.  Patients may present with a single or multiple cavernous angioma lesions.  Lesion size is variable, ranging from microscopic to a few inches in diameter and the lesions may cause a wide variety of symptoms including seizures, stroke symptoms, hemorrhages, and headaches.

Incidence

Cavernous angiomas are estimated to occur in approximately one out of every 500-600 people, that is approximately 0.2% of the general population.  While presentation of cavernous angioma is not uncommon in children, individuals often show the first sign of symptoms in their 20’s or 30’s.  Cavernous angiomas can continue to form later in life; therefore incidence rates and number of angiomas per person are higher among adults.  Generally, more than 30% of those with cavernous angioma eventually will develop symptoms.

Familial Cavernous Angioma

For at least 20% of those with the illness, cavernous angioma is hereditary. This form of the illness is often associated with multiple cavernous angiomas.  While familial cavernous angioma can happen in any family, it occurs at a higher rate among Hispanic-American families who trace their heritage to New Mexico.  This prevalence in Southwestern Hispanic-American families is due to a specific genetic mutation which has been passed through as many as 17 generations within this cultural group. 

Each child of someone with the familial form has a 50% chance of inheriting the illness.  Recent research has shown that there are at least three genes that cause the familial form of cavernous angioma; inheritance of a causative mutation or deletion in any one of these genes can lead to the illness.

Sporadic Cavernous Angioma

In addition to the familial form, cavernous angioma may arise sporadically.  Under this condition, there is no associated inherited genetic mutation.  The sporadic form typically presents as a solitary cavernous angioma that may be present at birth or may develop later in life.  Because sporadic lesions do not arise following the same genetic inheritance as with familial cases, related family members will not have a predisposition for the condition.  Additionally, children of those with sporadic cavernous angioma may have no greater chance of having cavernous angioma than anyone else in the general public (one out of 500-600 individuals).

Associated Venous Angioma

Up to 40% of solitary cavernous angiomas may develop in the vicinity of another vascular anomaly called a venous angioma. The venous angioma, also known as venous malformation or developmental venous anomaly, usually does not create problems unless it is associated with a cavernous angioma. It may make surgery more difficult; the goal is not to disturb the venous angioma while removing the cavernous angioma.In some people, a venous angioma may lead to the development of more than one cavernous angioma.

Other Vascular Malformations

The cavernous angioma is part of a spectrum of lesions known as "angiographically occult vascular malformations" related to the fact that they are not visible on an angiogram. Cavernous angiomas can not be seen on angiogram because they are low-flow anomalies in which blood flows through the lesion slowly. This is one quality that makes cavernous angiomas different from arteriovenous malformations which are high blood flow lesions that are readily visible on angiogram.

Symptoms  

Cavernous angioma symptoms are highly variable among individuals; in some cases no symptoms may be present.  However, when symptoms do manifest they often depend on the location of the angioma and on the strength of the angioma walls and their propensity for bleeding.  Cavernous angiomas can cause seizures.  A person who suffers from seizures is said to have epilepsy. There are many types of seizure including mild absence seizures and dramatic tonic-clonic seizures.  Seizures tend to worsen with age and frequency. Most cases of epilepsy can be well controlled with medications.  The type of seizure a person experiences depends, in part, on the location of the cavernous angioma. If a person has seizures and more than one cavernous angioma, it may be difficult to pinpoint which cavernous angioma is the cause of the seizures.

In addition to seizures, cavernous angiomas can cause neurological deficits such as weaknesses in arms or legs, vision problems, balance problems, and/or memory and attention problems. As with seizure, the type of deficit is associated with which part of the brain or spinal cord the cavernous angioma is located. Symptoms may come and go as the cavernous angioma changes in size with bleeding and reabsorption of blood.

Cavernous angiomas can bleed in a number of different ways:

• Angiomas can bleed slowly within the walls of the angioma and remain quite small.  A small hemorrhage may not require surgery and may be reabsorbed by the body.  However, continued small hemorrhages in the same cavernous angioma often cause deterioration in function.
• Angiomas can bleed more profusely within the walls of the angioma. This can cause them to increase in size and to put pressure on the surrounding brain tissue.
• Finally, angiomas may bleed through a weak spot in the angioma wall into the surrounding brain tissue. This is called an overt hemorrhage.

The risk of hemorrhage is dependent on the number of angiomas.  The higher the number, the greater the chance of one or more hemorrhages occurring sometime over a lifetime.  On average, cavernous angiomas that have bled in the past are those that are the most likely to bleed again, particularly in the first two years after their initial bleed.  It is also important to note that a hemorrhage in a cavernous angioma in the brain stem can be life-threatening, as the brainstem is responsible for regulating critical life processes including breathing and heartbeat.

Finally, those with cavernous angioma may experience headache. This seems to be true particularly when a lesion has undergone recent bleed activity.

Cavernous Angioma Statistics

1. 1 in 500-600 people have at least one cavernous angioma.
2. At least 30% of those with a cavernous angioma eventually will develop symptoms.
3. At least 20% of those with cavernous angioma have the familial form of the illness.
4. Up to 40% of solitary cavernous angiomas may have an associated venous angioma.
5. Age at first diagnosis:

Under 20: 25-30%

Age 20-40: 60%

Over 40: 10-15%

1. Primary symptom:

• Seizure – 30%
• Neurological deficit – 25%
• Hemorrhage – 15%
• Headache – 5%

1. Odds of your child having cavernous angioma:

• If you have sporadic cavernous angioma, your child is at no greater risk for developing a cavernous angioma than anyone in the general population; that is a one in 500 chance (0.2%).
• If you have familial cavernous angioma, your child may have a 1 in 2 chance (50%) of inheriting the causative gene change and developing cavernous angioma. 

Diagnosis and Treatment

Cavernous angiomas are diagnosed most often when they become symptomatic. Although cavernous angiomas have been known since the 1930’s, they have not been reliably diagnosed until the advent of the MRI (magnetic resonance imaging) in the 1980’s. Previously, the illness may have been misdiagnosed as multiple sclerosis or as a seizure disorder with no known cause. Cavernous angiomas are not visible on angiogram and were only inconsistently visible on CAT scans. An MRI scan, with and without contrast and with gradient echo sequences or susceptibility weighted imaging, read by an experienced physician remains the best means of diagnosing this illness. The MRI scan may need to be repeated to assess change in the size of a cavernous angioma, recent bleeding, or the appearance of new lesions.

Most cavernous angiomas are observed for change in appearance, recent hemorrhage or clinical symptoms. Medications are available to treat seizures and headaches caused by cavernous angiomas. Surgery is advocated for cavernous angiomas with recent hemorrhage, those which are expanding in size, and in some cases, those which are causing seizures. Radiosurgery, by gamma knife, linear accelerator or new shaped beam techniques, is a controversial treatment that has been used on cavernous angiomas too dangerous to reach through traditional surgery.

For familial cases of cavernous angioma, genetic testing is another option for diagnosis.

Surgery

Cerebral cavernous angiomas are surgically removed (resected) using a craniotomy, or opening the skull. This is usually performed under general anesthesia, except in cases where mapping of the brain while awake is needed. Cavernous angiomas in the spine are removed using laminectomy or unroofing of the vertebrae.

Surgery for cavernous angioma has been made safer using the operating microscope (microsurgery) and image guided surgical navigation (also known as computer-assisted or frameless stereotaxy) to reach the cavernous angioma with as little disruption to normal brain or spinal cord as possible.

Risks of any surgery, including cavernous angioma, include stroke, paralysis, coma or death, although these complications are rare with modern surgery performed by expert neurosurgeons. Surgery on cavernous angioma in the brain stem and spinal cord is more risky, but these cavernous angiomas are more dangerous if left alone. While recovery is different for everyone, many patients leave the hospital within a few days and resume normal life within a few weeks of surgery. However, people with neurological deficits may require a prolonged period of rehabilitation.

What We Don't Know about Cavernous Angioma

While researchers continue to make discoveries about cavernous angioma every day, many important research questions remain.

• Genetic researchers and a growing number of other researchers are working to determine the cause of the illness and the mechanisms by which the defective blood vessels are formed.
  
   
• We don't know most of the factors that lead to angioma bleeding and re-bleeding. Efforts to uncover what causes a particular cavernous angioma to bleed will help us to be able to reduce the risk of bleeding.
  
   
• We don't know how to remove a cavernous angioma without brain surgery or how to prevent them from becoming symptomatic. Less intrusive treatment methods may allow for control of more angiomas before they become problematic.