Dear PHACE families,
I hope this note finds everyone well! I am writing on behalf of the PHACE Registry with hope that you might consider enrolling your child. Registry data is proving to be an invaluable tool for PHACE research....we are about to begin a parent-inspired study to look at the migraine-like headaches known to occur in PHACE. We would love to include your child's information in this and other studies!
We do our absolute best to ensure that your child's identity is kept strictly confidential.
Enrollment in the Registry is straightforward and involves signing the consent form, completing a questionnaire and submitting a photograph of your child's hemangioma. We can send you the information over email or by mail with an enclosed paid return envelope, whichever is most convenient for you.
Thank you so much for considering. If you can please respond with your preferred email or mailing address, or let me know if you would prefer not to enroll your child at this time, I would be most appreciative. If you have any questions whatsoever about the registry, please don't hesitate to ask.
Warmest regards,
Denise Metry, MD
Associate Professor of Dermatology and Pediatrics
Baylor College of Medicine
Chief, Dermatology Service
Texas Children's Hospital
Office: 832-822-3718
http://www.texaschildrens.org/carecenters/Dermatology/Phace.aspx
PHACE BROCHURE
Friday, November 12, 2010
Wednesday, July 14, 2010
PHACE Syndrome: the Families, the Physicians, and the Facts... 2010 CONFERENCE
September 16th & 17th, 2010
Milwaukee, WI
This conference is sponsored by:
Children’s Hospital of Wisconsin, Medical College of Wisconsin, the National Organization of Vascular Anomalies, and HIG (Hemangioma Investigator Group
Schedule for the Family Conference
• Thursday, September 16, 2010
o 12:30 PM Registration/Event Check-In
o 1:00-5:00 PM Family Conference
o Following the conference: Family dinner and social event
• Friday, September 17, 2010
o 8:30 AM Continental breakfast
o 9:30 AM-3:30 PM Family Conference (includes luncheon)
Event Highlights
2 day Educational Conference:
• Overview and update on PHACE syndrome
• Radiology imaging in PHACE syndrome
• Neurological issues that can be a challenge to individuals with PHACE
• Treatment and management guidelines
• Social implications of facial differences
• Laser therapy for hemangiomas
• Research update
Social Event Thursday Evening with dinner
(Reception, Dinner and Entertainment tentatively set at Betty Brinn Children’s Museum)
Registration
• Cost: Free for PHACE families and includes everything listed in this section
• How to register: To register electronically E-mail jlehman@mcw.edu and request a registration form or call Jennefer at 414-955-2818 and you can register over the phone. Registration deadline is August 13th, 2010.
• All educational sessions on Thursday & Friday
• Written educational materials about PHACE syndrome
• Childcare for children 2-8 years old during educational sessions
• Reception, dinner and entertainment on Thursday evening
• Light breakfast on Friday morning from 8:30-9:30 AM
• Lunch on Friday
• There is no charge to the families for anything listed in this section
Why Attend?
There are many reasons to attend the PHACE conference. Here are just a few of them:
• Attendees will leave with a wealth of knowledge about PHACE syndrome
• Opportunities to meet other families facing similar challenges
• Exchange ideas, support and advice
Who Should Attend?
This conference is designed for families of children with PHACE syndrome or anyone that is interested in the care of children with PHACE syndrome from newly diagnosed to the latest research available.
Speakers & Panelists
Denise Adams, MD - University of Cincinnati College of Medicine, Cincinnati Children’s Hospital
Beth Drolet, MD – Medical College of Wisconsin, Children’s Hospital of Wisconsin
Jen Duncan, Director/Founder at PHACE Syndrome Community
Ilona Frieden, MD - University of California, San Francisco
Maria Garzon, MD – Columbia University, Morgan Stanley Hospital
Karla Hall – Executive Director NOVA
Christopher Hess, MD, PhD – University of California, San Francisco
Jennifer Hoag, PhD - Medical College of Wisconsin, Children’s Hospital of Wisconsin
Jensen, John, MD - Medical College of Wisconsin, Children’s Hospital of Wisconsin
Craig Johnson, DO – Medical College of Wisconsin, Children’s Hospital of Wisconsin
Patricia Marik, Psy D - Medical College of Wisconsin, Children’s Hospital of Wisconsin
Denise Metry, MD - Baylor College of Medicine, Texas Children’s Hospital
Orbach, Darren, MD, PhD – Harvard Medical School, Children’s Hospital Boston
Dawn Siegel, MD – Medical College of Wisconsin, Children’s Hospital of Wisconsin
Other Speakers TBD
Conference location
The conference takes place at the Children's Health Education Center located at
1533 N. RiverCenter Dr.
Milwaukee, WI 53212
(414)765-9355 phone
Map available at this website
http://www.chw.org/display/PPF/DocID/20530/router.asp
Where to Stay?
Comfort Inn Downtown Lakeshore: Rooms $79 plus tax.
Has a shuttle to/from Children’s Health Education Center for families.
No pool.
There is a block of rooms set aside.
Address: 916 East State Street, Milwaukee, WI, US, 53202
Phone: (414) 276-8800.
Mention PHACE conference for discount room rate.
Deadline for this rate is August 15, 2010.
There are many other hotels in the area also.
We have also secured discounted passes for the Paradise Landing Indoor Waterpark for $10 each, at the nearby Hilton Milwaukee City Center downtown.
The passes are available for use that Friday from 3-10pm and Saturday from 9am-10pm.
Just mention that you are with the PHACE/Children’s Hospital Group when you purchase the passes at the water park.
Milwaukee, WI
This conference is sponsored by:
Children’s Hospital of Wisconsin, Medical College of Wisconsin, the National Organization of Vascular Anomalies, and HIG (Hemangioma Investigator Group
Schedule for the Family Conference
• Thursday, September 16, 2010
o 12:30 PM Registration/Event Check-In
o 1:00-5:00 PM Family Conference
o Following the conference: Family dinner and social event
• Friday, September 17, 2010
o 8:30 AM Continental breakfast
o 9:30 AM-3:30 PM Family Conference (includes luncheon)
Event Highlights
2 day Educational Conference:
• Overview and update on PHACE syndrome
• Radiology imaging in PHACE syndrome
• Neurological issues that can be a challenge to individuals with PHACE
• Treatment and management guidelines
• Social implications of facial differences
• Laser therapy for hemangiomas
• Research update
Social Event Thursday Evening with dinner
(Reception, Dinner and Entertainment tentatively set at Betty Brinn Children’s Museum)
Registration
• Cost: Free for PHACE families and includes everything listed in this section
• How to register: To register electronically E-mail jlehman@mcw.edu and request a registration form or call Jennefer at 414-955-2818 and you can register over the phone. Registration deadline is August 13th, 2010.
• All educational sessions on Thursday & Friday
• Written educational materials about PHACE syndrome
• Childcare for children 2-8 years old during educational sessions
• Reception, dinner and entertainment on Thursday evening
• Light breakfast on Friday morning from 8:30-9:30 AM
• Lunch on Friday
• There is no charge to the families for anything listed in this section
Why Attend?
There are many reasons to attend the PHACE conference. Here are just a few of them:
• Attendees will leave with a wealth of knowledge about PHACE syndrome
• Opportunities to meet other families facing similar challenges
• Exchange ideas, support and advice
Who Should Attend?
This conference is designed for families of children with PHACE syndrome or anyone that is interested in the care of children with PHACE syndrome from newly diagnosed to the latest research available.
Speakers & Panelists
Denise Adams, MD - University of Cincinnati College of Medicine, Cincinnati Children’s Hospital
Beth Drolet, MD – Medical College of Wisconsin, Children’s Hospital of Wisconsin
Jen Duncan, Director/Founder at PHACE Syndrome Community
Ilona Frieden, MD - University of California, San Francisco
Maria Garzon, MD – Columbia University, Morgan Stanley Hospital
Karla Hall – Executive Director NOVA
Christopher Hess, MD, PhD – University of California, San Francisco
Jennifer Hoag, PhD - Medical College of Wisconsin, Children’s Hospital of Wisconsin
Jensen, John, MD - Medical College of Wisconsin, Children’s Hospital of Wisconsin
Craig Johnson, DO – Medical College of Wisconsin, Children’s Hospital of Wisconsin
Patricia Marik, Psy D - Medical College of Wisconsin, Children’s Hospital of Wisconsin
Denise Metry, MD - Baylor College of Medicine, Texas Children’s Hospital
Orbach, Darren, MD, PhD – Harvard Medical School, Children’s Hospital Boston
Dawn Siegel, MD – Medical College of Wisconsin, Children’s Hospital of Wisconsin
Other Speakers TBD
Conference location
The conference takes place at the Children's Health Education Center located at
1533 N. RiverCenter Dr.
Milwaukee, WI 53212
(414)765-9355 phone
Map available at this website
http://www.chw.org/display/PPF/DocID/20530/router.asp
Where to Stay?
Comfort Inn Downtown Lakeshore: Rooms $79 plus tax.
Has a shuttle to/from Children’s Health Education Center for families.
No pool.
There is a block of rooms set aside.
Address: 916 East State Street, Milwaukee, WI, US, 53202
Phone: (414) 276-8800.
Mention PHACE conference for discount room rate.
Deadline for this rate is August 15, 2010.
There are many other hotels in the area also.
We have also secured discounted passes for the Paradise Landing Indoor Waterpark for $10 each, at the nearby Hilton Milwaukee City Center downtown.
The passes are available for use that Friday from 3-10pm and Saturday from 9am-10pm.
Just mention that you are with the PHACE/Children’s Hospital Group when you purchase the passes at the water park.
Saturday, April 4, 2009
Mighty Quinn In the News
Okay I know I am biased but I wanted to share the article about Quinn that was in our local paper regarding the upcoming surgery in New York.
Mighty Quinn is a brave little boy who now needs our help
Updated 4 days ago
The Mighty Quinn is headed to the Big Apple for surgery.
Two-year-old Quinn Michael Duncan is one of only an estimated 200 persons worldwide diagnosed with Phaces syndrome.
The syndrome's most visible sign is a hemangioma, or tumour, on the face or neck and other health problems such as abnormalities of the brain and eye.
The surgeries, to be done April 16 and June 6, will help ease "significant scarring" near his mouth caused by a hemangioma on the left side of the Sault Ste. Marie youngster's face.
His right eyelid and upper lip will be reconstructed.
His mother, Jennifer, describes the operations as "complex." She has struggled with agreeing for the surgeries to go ahead.
"To me he's Quinn the way he is," she said in a recent interview.
"It's been a long road trying to decide whether or not we wanted to do this."
Jennifer knows the scarring has bothered her son when he looks in a mirror.
She acknowledges some people have made rude comments about her son's appearance, recalling one experience when he was called a "monster" to his face.
"We've had interesting situations which we learned to deal with long ago," said Jennifer.
"We don't care. We take him everywhere. It doesn't bother us one bit. We don't even see it. I actually think that his scars are kind of beautiful because they tell his story."
Two doctors are waiving their fees for the operations through the Waner Children’s Vascular Anomaly Foundation. That includes foundation president Dr. Milton Waner.
"It's absolutely amazing," said Jennifer.
"When I found out I couldn't believe it. It just blew me away."
A fundraising pasta dinner to help cover the family's airfare, accommodation and meal expenses will be held at Brody's Sports Bar and Grill April 1 at 6 p.m. Admission is $10 For tickets, call Rochelle at 949-8846.
Donations can also be made Northern Credit Union account number 210653.
There have been some improvements, and some setbacks, with Duncan's health since The Sault Star profiled the youngster in February 2007.
There's no change with a tumour in the child's brain. That's good.
Two congential heart defects have healed, but Duncan now suffers from migraines.
The right side of his cerebellum is still smaller than the left. That affects his balance, co-ordination and, his mother suggests, was a factor in his being diagnosed with autism in 2008. He started walking at age 30 months.
Duncan earned his nickname for enduring so many health concerns at such a young age. The handle still fits.
"He is really complacent for a child who has a diagnosis of autism," said Jennifer.
"He's really happy-go-lucky. He's bonded with us. He's doing well."
He visits Children's Rehabilitation Centre of Algoma once a week. Mother and son spend three hours each morning on tasks as music and play therapy, making eye contact and socializing.
"I'm doing what I can," she said.
"He's made humongous gains since his regression (with autism)."
On the web: www.wanervascularanomaly.org
Thursday, March 5, 2009
NOVA's New Statement on Propanolol And The Treatment of Hemangiomas
Recently, there have been two reports of the use of Propranolol in the treatment of infantile hemangiomas. In both studies, the number of patients evaluated was small and most children were older at the start of therapy. Careful monitoring of side effects was not specifically outlined but initial improvement in the size and color of the hemangiomas was noted. The effect was proposed to be secondary to the factors that effect blood vessel growth or “angiogenesis”.
Propranolol is a medication known as a non-selective beta adrenergic blocker. The drug is used in children with heart (cardiac) problems such as an abnormal heart rate or rhythm. Propranolol has been proven to be safe in this population of patients. There have been some reports of patients who become ill and have symptoms of low blood sugar (hypoglycemia), low body temperature (hypothermia) and low heart rate (bradycardia) while on this medication. These are known side effects of this medication. Most of these reports are rare but still can cause significant issues.
Many physicians have read these reports and are starting to use propranolol in patients with hemangiomas. There have been unpublished reports of patients needing to be hospitalized for episodes of non-symptomatic (no preceding illness) hypoglycemia, hypothermia and bradycardia.Because these studies are small, the information is limited on the side effects and effectiveness of this drug in patients with hemangiomas. In some situations, such as patients with PHACE syndrome this drug may be dangerous and cause more complications.
It is very important that this new medication be studied in a controlled manner. There is presently a large study being proposed that will open at various centers in about 6 months.
If a child is put on propranolol it is important that they are followed in a vascular anomalies center or by a physician with experience taking care of these patients. A plan of care that specifically outlines the evaluations needed prior to the medication and during the medication administration to follow toxicity should be outlined.Most importantly, a documented consent process is advisable.
Leaute-Labreze C, Dumas de la Roque E, Hubiche T, Boralevi F, Thambo J-B, Taieb A. Letter to the Editor: Propranolol for Severe Hemangiomas of Infancy. N Engl J Med. 358;24;2649-2651.
Beta Adrenergic Blocking Agents in: Oxford Textbook of Clinical Pharmacology 1st Edition, eds. Graham-Smith DG and Aronson JK. Pg 64.
Drugs that Inhibit Adrenergic Nerves and Block Adrenergic Receptors in: Goodman and Gillman: Pharmacological Basis of Therapy, 6th Edition. Pg 192-4.
Chavez H, Ozolins D, Losek J. Hypoglycemia and Propranolol in Pediatric Behavioral Disorders. Pediatrics. 1999;103;1290-1291.
McBride JT, McBride MC, Viles PH. Hypoglycemia Associated with Propranolol. Pediatrics 1973;51;1085-1087
Artman M, Grayson M, Boerth RC. Propranolol in Children: Safety – Toxicity. Pediatrics 1982;70;30-31.
Kallen RJ, Mohler JH, Lin HL. Hypoglycemia. Clinical Pediatrics;19;8;567-568.
Here is the link to the article NOVA put out on its site.
http://www.novanews.org/Propranolol.htm
Propranolol is a medication known as a non-selective beta adrenergic blocker. The drug is used in children with heart (cardiac) problems such as an abnormal heart rate or rhythm. Propranolol has been proven to be safe in this population of patients. There have been some reports of patients who become ill and have symptoms of low blood sugar (hypoglycemia), low body temperature (hypothermia) and low heart rate (bradycardia) while on this medication. These are known side effects of this medication. Most of these reports are rare but still can cause significant issues.
Many physicians have read these reports and are starting to use propranolol in patients with hemangiomas. There have been unpublished reports of patients needing to be hospitalized for episodes of non-symptomatic (no preceding illness) hypoglycemia, hypothermia and bradycardia.Because these studies are small, the information is limited on the side effects and effectiveness of this drug in patients with hemangiomas. In some situations, such as patients with PHACE syndrome this drug may be dangerous and cause more complications.
It is very important that this new medication be studied in a controlled manner. There is presently a large study being proposed that will open at various centers in about 6 months.
If a child is put on propranolol it is important that they are followed in a vascular anomalies center or by a physician with experience taking care of these patients. A plan of care that specifically outlines the evaluations needed prior to the medication and during the medication administration to follow toxicity should be outlined.Most importantly, a documented consent process is advisable.
Leaute-Labreze C, Dumas de la Roque E, Hubiche T, Boralevi F, Thambo J-B, Taieb A. Letter to the Editor: Propranolol for Severe Hemangiomas of Infancy. N Engl J Med. 358;24;2649-2651.
Beta Adrenergic Blocking Agents in: Oxford Textbook of Clinical Pharmacology 1st Edition, eds. Graham-Smith DG and Aronson JK. Pg 64.
Drugs that Inhibit Adrenergic Nerves and Block Adrenergic Receptors in: Goodman and Gillman: Pharmacological Basis of Therapy, 6th Edition. Pg 192-4.
Chavez H, Ozolins D, Losek J. Hypoglycemia and Propranolol in Pediatric Behavioral Disorders. Pediatrics. 1999;103;1290-1291.
McBride JT, McBride MC, Viles PH. Hypoglycemia Associated with Propranolol. Pediatrics 1973;51;1085-1087
Artman M, Grayson M, Boerth RC. Propranolol in Children: Safety – Toxicity. Pediatrics 1982;70;30-31.
Kallen RJ, Mohler JH, Lin HL. Hypoglycemia. Clinical Pediatrics;19;8;567-568.
Here is the link to the article NOVA put out on its site.
http://www.novanews.org/Propranolol.htm
Thursday, February 19, 2009
Ask Google for a Childhood Stroke Awareness Day Doodle
1 in 4,000 newborns and 6 in 100,000 children are diagnosed with childhood stroke which typically results in hemiplegic cerebral palsy. An undetermined number of unborn babies are also diagnosed. Many go undiagnosed, have delay in diagnosis or are misdiagnosed. Help us spread the word...so we may find better treatments and preventions to help families all over the world affected by Childhood Stroke.
We kindly request that Google light their home page doodle in purple with the purple childhood stroke awareness ribbon to raise awareness for National Childhood Stroke Awareness Day this coming May 2, 2009.
Please add your name to this petition and tell as many people as you can about it.
P.S. You can see examples of previous doodles on this page:
http://www.google.com/holidayl...
CLICK HERE TO SIGN THE PETITION
Petition sponsor
The Childhood Stroke Awareness Campaign is sponsoring this petition in an effort to raise awareness that Kids Have Strokes, Too! It is sad to hear people say "I didn't know infants, children and unborn babies could have a stroke!"...Help us spread the word so we can find better treatments and prevention to this life altering disorder.
To learn more about the Childhood Stroke Awareness Campaign visit http://www.childhoodstrokeawar... and www.KidsHaveStrokes2.org in addition to the sites listed under the links.
Links
www.chasa.org
http://www.KidsHaveStrokes.org...
www.hemikids.org
http://www.childhoodstrokeawar...
http://www.KidsHaveStrokes2.or...
We kindly request that Google light their home page doodle in purple with the purple childhood stroke awareness ribbon to raise awareness for National Childhood Stroke Awareness Day this coming May 2, 2009.
Please add your name to this petition and tell as many people as you can about it.
P.S. You can see examples of previous doodles on this page:
http://www.google.com/holidayl...
CLICK HERE TO SIGN THE PETITION
Petition sponsor
The Childhood Stroke Awareness Campaign is sponsoring this petition in an effort to raise awareness that Kids Have Strokes, Too! It is sad to hear people say "I didn't know infants, children and unborn babies could have a stroke!"...Help us spread the word so we can find better treatments and prevention to this life altering disorder.
To learn more about the Childhood Stroke Awareness Campaign visit http://www.childhoodstrokeawar... and www.KidsHaveStrokes2.org in addition to the sites listed under the links.
Links
www.chasa.org
http://www.KidsHaveStrokes.org...
www.hemikids.org
http://www.childhoodstrokeawar...
http://www.KidsHaveStrokes2.or...
Tuesday, February 17, 2009
A NOTE ABOUT PHACE AWARENESS AND EDUCATION
Jennifer Duncan:
Mom to Quinn
NOVA PHACE Patient Advocate
PHACE Community Director
PHACE SYNDROME is a neuro-cutaneous association of anomalies, first coined by Dr Ilona Freidan in 1996. The acronym
PHACE refers to the combination of large segmental hemangiomas (the most prevalent being on the face though there are cases of children who do not present with the hemangioma) and one or more of the following:
· posterior fossa other structural malformations of the brain
· arterial anomalies
· cardiac defects
· eye abnormalities
· Ventral developmental defects.
PHACE is thought to occur mainly in females but there have been a number of cases of males being diagnosed with the disorder. A diagnosis of PHACE is made in the presence of a segmental facial hemangioma in association with one or more of the above anomalies. The majority of infants and children do not have the complete PHACE spectrum of anomalies. Every case of PHACES is different as are the anomalies and medical issues faced by each child. Some cases are mild and some are much more severe.
PHACE was once thought to be a rare disorder. It is now thought to be simply uncommon and often unheard of. PHACE is now thought to be more common than Sturge-Weber Syndrome, with which PHACE is at times confused. Sturge-Weber is associated with a vascular birthmark called a port-wine stain, which unlike a hemangioma (as associated with PHACE) is a capillary -like vascular malformation. It is present at birth (most hemangiomas develop shortly after birth) and it does not show signs of growth during infancy (hemangiomas go through a rapid growth stage called proliferation) Port wine stains undergo minimal expansion and do not regress. (Hemangiomas begin to regress and undergo a process called involution)
Many PHACE families have children who were at one time thought to have had a Port Wine Stain and were at risk for Sturge-Weber Syndrome. A few of our own community members have children who were actually diagnosed with Sturge Weber initially. It was only after referral to pediatric dermatologists that these children were shown to actually have hemangiomas. The two often appear similar shortly after birth, but a hemangioma quickly distinguishes itself as it begins to grow at a rapid pace. It is extremely important that all children who are thought to have (or adults/older children who had a birthmark as a child and present with anomalies associated with PHACE a facial birthmark are evaluated by a pediatric dermatologist or Vascular Anomalies Centre.
These children should undergo neurologic, ophthalmologic and cardiac investigations to determine the medical needs of each child. It is the hope of the families involved in the PHACE community, that we can bring awareness to this often complicated and not well-understood disorder. We hope that we can increase this awareness by educating the public and providing support, resources and love to other families who are facing the same diagnosis. It is highly recommended that each child with PHACE be followed by a specialized multi-disciplinary Vascular Anomaly Clinic, familiar with PHACE Syndrome.
(Sources)
1. Denise W. Metry, Christopher F. Dowd, A. James Barkovich and Ilona J.
Frieden, The Many Faces of PHACE Syndrome, The Journal of Pediatrics, July, 2001
2. Denise W. Metry, A Newborn Girl With a Large Red Plaque on Her Face,
Pediatric Annals, June 2006
Mom to Quinn
NOVA PHACE Patient Advocate
PHACE Community Director
PHACE SYNDROME is a neuro-cutaneous association of anomalies, first coined by Dr Ilona Freidan in 1996. The acronym
PHACE refers to the combination of large segmental hemangiomas (the most prevalent being on the face though there are cases of children who do not present with the hemangioma) and one or more of the following:
· posterior fossa other structural malformations of the brain
· arterial anomalies
· cardiac defects
· eye abnormalities
· Ventral developmental defects.
PHACE is thought to occur mainly in females but there have been a number of cases of males being diagnosed with the disorder. A diagnosis of PHACE is made in the presence of a segmental facial hemangioma in association with one or more of the above anomalies. The majority of infants and children do not have the complete PHACE spectrum of anomalies. Every case of PHACES is different as are the anomalies and medical issues faced by each child. Some cases are mild and some are much more severe.
PHACE was once thought to be a rare disorder. It is now thought to be simply uncommon and often unheard of. PHACE is now thought to be more common than Sturge-Weber Syndrome, with which PHACE is at times confused. Sturge-Weber is associated with a vascular birthmark called a port-wine stain, which unlike a hemangioma (as associated with PHACE) is a capillary -like vascular malformation. It is present at birth (most hemangiomas develop shortly after birth) and it does not show signs of growth during infancy (hemangiomas go through a rapid growth stage called proliferation) Port wine stains undergo minimal expansion and do not regress. (Hemangiomas begin to regress and undergo a process called involution)
Many PHACE families have children who were at one time thought to have had a Port Wine Stain and were at risk for Sturge-Weber Syndrome. A few of our own community members have children who were actually diagnosed with Sturge Weber initially. It was only after referral to pediatric dermatologists that these children were shown to actually have hemangiomas. The two often appear similar shortly after birth, but a hemangioma quickly distinguishes itself as it begins to grow at a rapid pace. It is extremely important that all children who are thought to have (or adults/older children who had a birthmark as a child and present with anomalies associated with PHACE a facial birthmark are evaluated by a pediatric dermatologist or Vascular Anomalies Centre.
These children should undergo neurologic, ophthalmologic and cardiac investigations to determine the medical needs of each child. It is the hope of the families involved in the PHACE community, that we can bring awareness to this often complicated and not well-understood disorder. We hope that we can increase this awareness by educating the public and providing support, resources and love to other families who are facing the same diagnosis. It is highly recommended that each child with PHACE be followed by a specialized multi-disciplinary Vascular Anomaly Clinic, familiar with PHACE Syndrome.
(Sources)
1. Denise W. Metry, Christopher F. Dowd, A. James Barkovich and Ilona J.
Frieden, The Many Faces of PHACE Syndrome, The Journal of Pediatrics, July, 2001
2. Denise W. Metry, A Newborn Girl With a Large Red Plaque on Her Face,
Pediatric Annals, June 2006
Monday, February 9, 2009
Olivia's Heart Journey
The following is written as Olivia's heart journey happened in the words of her mom Karen.
OLIVIA'S HEART JOURNEY
Part 1
We took Olivia to have a heart murmur evaluated. I was totally expecting that we would be told that many baby's have heart murmurs and for them to tell us that we were over cautious and to go home. We even had our plans for what we were going to do that afternoon (being in Omaha) after her visit.
But while they were doing her echo (Terry and I were able to be in the same room) I could tell (despite their ability to carefully choose their words) that something was not right. I did not get too worried, because Olivia looks so good. But after the Dr. and the very experienced Echo technician came back and spent quite a time trying to figure out what they were actually seeing and me hearing them having difficulty even identifying the main blood vessels coming off of her heart- that is when I began to realize the seriousness of all of this- or so I thought. I knew it would mean surgery, but that was still not even close to what I was thinking could be possible.
But, despite all of this, I could feel God's peace on me (it was almost unreal, knowing what I knew without having heard any diagnosis from the Dr. yet-- this is usually the time where your mind races and you think of all of the terrible things that could happen (that is especially true with nurses- we tend to know too much, but yet not enough, that it gets us in trouble)) So, here we were and I remember thinking - This makes no sense that I have this calmness- now that does not mean that I was not scared and didn't cry- because I did. But I know what God's promises are for those who are His children and walk in His ways- and I also know that it is our soul that is saved and not our emotions or thoughts. It is our job to "take captive every thought" that does not line up with the Word of God. And I know that we have to renew our minds daily. (I had to renew my mind sometimes every few minutes).
This is where it proves true that you need to "hide God's Word in your heart" I was able to think of all of these scriptures in the Bible and I was able to replace those scary thoughts with God thoughts. God's Word is true - it is black and white and there is no ambiguity. God made it so simple that no one could say that they did not know or were not given the opportunity to make that choice to become a member of His family. So, I was able to see the benefits of knowing what it says in the bible. Can you imagine if I was sitting in that office only having what I knew and was waiting to hear from the Dr., how worked up I could have gotten. How fear would have totally consumed me. This was just the beginnings of how amazingly God showed himself at work in this situation.
So, the Dr. came back and told us that Olivia has what is called coarctation of the aorta- basically a mis-shaping of the main blood vessel that comes off of the heart to feed all of the body (except for the lungs). And that main large blood vessel branches off into 3 and sometimes 4 different branches and goes to the R arm, R brain, L arm and L brain and then, one that goes to all of the rest of the body. Based on what he saw (and he consulted with the chief cardiologist) they wanted to admit her right away and get further testing.
So, we were then gong to have an MRI of the heart called a cardiac MRI, which they say is a fairly new technique used. They needed to be able to identify where all of these vessels were going. We had been in the cardiology clinic for 5 hours. So, we were taken over to the hosp and were admitted and stayed there over the weekend. It was to late in the day to get the MRI and it would have to wait until Monday.
Our Cardiologist said if he really wanted to do the MRI, he could do it on Friday, but he wanted to wait so that he could have the technician who takes the best pictures and who he feels has the best experiences with little, little ones. He also wanted to have a cardiac anesthesiologist (not just a regular anesthesiologist) in the room (we thought that was just precautionary and did not realize that it is fairly routine to anesthetize and intubate little ones for this procedure). And Dr. Kutty (our cardiologist) wanted to be there to see the pictures and be able to tell the technician if and where he wanted to take more pictures and look at thing from different angles. I respected this and preferred this also.
This was the beginning of us seeing how well these Dr.'s work together and how they totally do what seems unrealistic in the medical world- they did this continually - being what was best for her. So, you can imagine how hard it was to hand over our little perfect girl to be put to sleep and intubated for this test. In the mean time, they had a whole group of Dr.'s on hand to evaluate her for a condition that call PHACES syndrome. Highly rare and very highly under diagnosed. And just read this---- this really will display God's hand at work.
Our cardiologist just came to Children's hospital from Milwaukee (in June) and he had begun to work closely with a pediatric dermatologist (which there are not too many of) who was doing a study on kids who present without any problems, but a hemangioma on the face- which is basically a purple discoloration on the face and almost always covers part of one eyelid and is usually only on one side of the face. Many kids have discolorations like this on their faces and it goes away over time and they can refer to it as a stork bite or a portwine stain, etc. Anyway, this dermatologist would immediately send these kids to Dr. Kutty's office to have echo's and many times they would find what Olivia has (hardly ever as severe as Olivia's). And these kids would have surgery before it would ever really become a problem- because this can be lethal if not found. Actually the way most of these baby's are diagnosed is because they come in the hospital in heart failure and a lot of trouble breathing and then they have to be intubated and off to surgery. Isn't that again a blessing that God allowed for us to discover this in Olivia, yet have her still be in such good condition. (her only symptoms were the hemangioma- which most people don't even begin to associate with a malformation of blood vessels coming off of the heart.......and she was not gaining weight like they wanted. she had some trouble with the suck, swallow breathe mechanism and she had a heart murmur (which a lot of baby's have and then they go away).
I know this is a lot of info- I hope you are following without too much trouble. We have been overwhelmed with information and I have been running on lack of sleep for the past month, esp after my exciting C-section where my blood pressure dropped extremely low, along with me heart rate and emergency drugs had to be used- I went unresponsive (when normally you are completely awake during the whole procedure) and Olivia had to be delivered immediately because those emergency drugs shut off the blood flow to the uterus and her. (anyway, that is a whole other story).
So we had a pediatric opthamologist (eye specialist) come evaluate her eye- because there can be eye problems with this syndrome- hers is essentially normal- just needs follow up for lesser problems and monitoring for no signs or symptoms of bigger problems. We have seen a genetics Dr. (so nice and down to earth). She did a full panel of genetics readings (which this has to be done before you receive any blood products- because that would alter the results. Just think if Olivia had been one of those sick baby's that they usually see and they had to rush into surgery (which we know she has to have blood for) she may not have had the opportunity for the genetics testing. And with this condition- which is not well known, baby's can have problems with the blood vessels of the head and many times this is not evaluated - because it is not known to even be a potential issue- any way many of these baby's have been taken to surgery and have later had bleeding or infarct (lack of oxygen to the brain, resulting in a stroke). So our Dr. knew that this also had to be looked at.
So, along with her heart MRI, they also did a brain MRI and an MRI of the area around the eyes to ensure the blood vessels there were OK. This was all coordinated to take place at the same time with just one sedation and intubation. That alone is a blessing. Praise GOD!!! Olivia's blood vessels in her brain and around the eyes are fine. Well, even with this cardiac MRI, the Dr.'s could not determine the layout of Olivia's blood vessels. There was no question of surgery, the question was how can this surgery be completed in a safe manner. IT absolutely had to be determined where all of the major blood vessels were and how effective they were at perfusing her body- especially to the brain. They could not determine the origin, pathway or effectiveness of the blood vessels coming off of the heart before they went to the brain (they knew somehow the blood was getting there, because of the brain MRI, but the road map had to be determined, because of how they clamp off arteries and bypass others during the surgery. There could be a potential of completely cutting off all blood flow to the brain during the surgery. So they needed to know where these vessels originated from (esp because of her uniqueness and level of abnormality). Again, Praise God that we had this time to get all of these tests done and allow for the Dr.'s to gather all of the information needed to get the best results and avoid any potential for error.
So, the next step was to do a heart catheterization (another sedation and intubation) another time of handing over what appeared to be a perfect baby to have these scary tests done. But, we stood on what we know to be true- God's Word. And instead of being so sad (but don't think for a minute it was not hard- it was so hard, you could feel the physical ache in your heart, it would be hard to breathe or even feel like you could still stand on you own two feet ; due to this emotional pull. But again, I knew that was my emotions- and with going over scriptures and reminding myself of what kind of a God we have, I was able to reduce those feelings quite a bit. But we waited anxiously for the updates as the procedures were being carried out. Psalms 91 was reviewed several times. The ink might even be somewhat rubbed off by now.
This heart cath gave them the essential information they needed. Now our awesome cardiothorasic surgeon was able to determine an initial plan. He chose to postpone surgery for 1 week because as he said, he wanted his best team available to him and that he had also been covering for the head heart transplant Dr. at UNMC and that he has had an unusually intense string of surgeries in the NICU. And he admitted he was a bit exhausted. He said he wanted to be rested, have his best team and also have time to research out this PHACES syndrome and also contact other surgeons who have worked on PHACES babies, because of the potential for re narrowing of the arteries in the future. He also wanted to do a lot of measuring and reviewing of the tests. All of this would be considered when he developed a plan for Olivia's surgery.
Part 2
We came down to Omaha on Tuesday to have Olivia's pre-op physical and lab work and also a chest X-ray. The X-ray tech said they couldn't believe how still Olivia was and how she was so good as they had to secure her legs down and hold her arms up next to her head, and basically stretch her- she didn't even seem to care.
Everything checked out well. We also saw the cardiac anesthesiologist. She sent us with a couple of doses of steroids to give her, one that evening and then again at 3 in the morning. We then stayed at the rainbow house (a place for parents to stay while their children are at the hosp.
It is free and has a lot of things to offer- a very neat thing for many of the people who come so far for treatment. This is where we (well probably only Terry- I am sure I will stay at the bedside.
On Wednesday, we got to the hosp at 5:15 in the morning. We only got about 3 and 1/2 hours of sleep- which I didn't mind too much. It seems you are much easier going because of peer exhaustion-- and the brain synapses are firing a little slow. We had to give Olivia 2 bathes the
night before surgery- according to our Dr. instructions. There is a bed and a sink only in our room at the rainbow house, so I was glad we brought her baby bathtub with us. We had a laundry basket in our room (you are to wash your own towels and linen after staying in the room and also must clean the sink and vacuum the floor- not a bad trade for a free room!)
Anyway, we tipped the laundry basket over and used it to put the bathtub on, to then give her a bath. It worked pretty well.
So-- back to Wednesday ( I am going on very little sleep for the past couple of days- so this may sound a bit choppy, sorry)
At a little before 7 in the morning we walked Olivia to the surgery doors and handed her over to the surgical nurse. I think that was the hardest part of it all! She still looks so perfect on the outside and it just doesn't quite seem right to be voluntarily handing her over to have all of
those things happen to her. --We had noticed that she had begun to be a little more wheezy and did have a bit of a stridor, so at least in a small way we had something that made us feel like it was time to get this surgery done and over with. We were glad to have that week in between so
that we could prepare for the surgery, find a place for the other kids (Thanks Aunts Jen, Laura and Krissa, and also to the grandparents for helping out!!) It also gave Olivia time to recover from the last hospitalization. She had been intubated twice and received dye and also
barium for her swallow study. It took her about that full amount of time to get back to normal.
So, (again back to Wed :) ) Once Olivia was off to surgery, we went to out own little private waiting area (which was so nice to have- it is such an intense time- we really appreciated being able to go about the waiting process on our own. Dr. Hamel came in and gave us the final
details of how he anticipated the surgery to go. Of course, he still was aware that there might be some small surprises. Through out the surgery, we had a nurse keep us updated - this seemed to break the time up pretty well. We were really surprised that Olivia only had to be on the heart lung, bypass machine for only 1 and 1/2 hours. And, they did not have to cool her body much at all. And, they did not have to stop her heart during surgery as he had planned. Every time we would get some news, I kept finding my self surprised at how much better, than expected, everything went. He had planned on using organ donor tissue or a synthetic graft to reconstruct her aortic arch. Afterwards, he told us he did not have to use either one, but instead was able to use all of her own tissue. Again- amazement. It just goes to show how you should never put God in a box and think you know what He can do- He really exceeded our expectations. When we talked with Dr. Hamel, I said to him that it seemed like everything went almost as well as could ever be expected. He began to say something and then hesitated. He said that he never really says this, but that it was near perfect. Praise God!! ....I was so amazed at how calm and how much peace we had. I think people who could have seen us would have thought we were just not really "getting it" as if we did not understand what was really all occurring. But we were very much aware of it all and Dr. Hamel had to go through all of the possible things that
could happen to Olivia (and you can all imagine the things we heard) But we knew this was part of his job and probably the hardest part, besides when we actually handed Olivia over to the nurses) And we talked with Dr. Hamel about this. He did tell us that he really felt overall that there was a very good chance Olivia would be the same little baby girl after the surgery, as she was coming into the surgery. That was nice to hear.
They spent the first 1 and 1/2 to 2 hours establishing her arterial lines, IV's and intubation. Then it took a short while to get her established on the heart/lung bypass machine, but the actual surgery took about 2 1/2 to 3 hours- much less that we anticipated. Then it was time to go upstairs and see our little Miss Olivia. I really thought this was going to be hard. We had been given a tour of the PICU before and one of Dr. Hamel's nurse practitioners had reviewed a book of photos to let us know what we should expect to see. I was very surprised at how well she looked and we didn't have a hard time at all seeing her intubated and tubes and wires everywhere. We could look past all of that and see her, see how relaxed her face looked - she just really looked at peace. She had much less for IV fluids (despite the 7 IV pumps working away for her arterial lines and IV medications and blood products). She was really doing well.
Terry and I actually, both went to one of the 2 sleep rooms that they offer to the parents of the most critical kids- who have also come from a distance). It was only a single bed up against the wall and had a bathroom. Terry usually sleeps on the one side, but I took that side, so I would be up against the wall- It wasn't going to be me who fell out of the bed. We were both so tired that I don't think either one of us moved. I woke up and thought it had to be about 5 in the morning and went to her room. I was in her room for a short while and then noticed it was only midnight.
I guess I felt like I had a full night's rest after 4 hours of sleep- That was quite a rare thing to get 4 hours in a row! I then, stayed in her room and did get a couple of short naps in.
This morning, Olivia continued to be so very stable that Terry and I both went back to the
rainbow house to shower ( I thought the only way I would leave her room was if someone drug me out- so it was so nice to just have that peace and calmness to be able to do that) the peace that only God can give you, that passes all understanding (as it says in the Bible).
As far as what the Dr's had to say: Instead of taking out the breathing tube this morning, they wanted to give her 1 extra day, due to her uniqueness with her abnormal blood vessels and also because of her hemangioma near her airway. They wanted to give her 24 hours of steroids
to prevent inflammation in her airway and avoid the possibility of reintubation. (she was a hard intubation) Also she had developed in the later morning- purple coloration to her fingers and feet and her face was also that color some. They just wanted to allow the blood vessels to get used to this new good blood flow (as it almost double to most of her body) They did another echo cardiogram of her heart to see what her heart function was. Her heart function decreased- which is normal after surgery. They, then added a drug to help with the pumping mechanism,
since her'e is temporarily less than half of what it should be. It is just trying to find a new "normal" since now it does not have to go against any resistance. Within 1 hour of being on this IV medication, her hands and feet became warmer and the color improved quite a bit- It was
really amazing to see. (I later found out that almost all kids with heart surgery are on this medication when they come back from surgery, but because her heart function was so good in surgery, they did not need it-- this made me feel better about it all) We still cont to be amazingly calm and -just at peace with this all. We are so thankful to God for this- I can't imagine trying to do this wihtout Him.
Part 3
We had a little (OK- it was a very difficult) day yesterday. It came time to remove Olivia's breathing tube. We had waited until she was more awake. They had taken off her sedative and pain medication that morning. Well they took the tube out and she was having trouble breathing- not so abnormal. But, it did not stop. It lasted 10 hours!! It was horrible to
watch and not be able to do anything about. They kept a close eye on her, but did not want to have to put the tube back in. Several times, we had a room full of medical people and the intensivist ( a Dr. that is constantly in the ICU). They would do a few things to try to make it better and just watch her and try to make that difficult decision- do we put the breathing tube back in or not. It is really something that absolutely do not want to do, unless there was no other alternative. They gave her a reversal medication, to basically wipe out any pain medication that may have been in her system to see if that was suppressing her respiratory system (something the Dr. told us earlier he did not want to do because he felt it was "mean" because then they did not have any pain control)
Olivia struggled for 10 hours this way- it was terrible to watch. She was right on that borderline the whole time of working really hard and just doing enough to maintain her oxygen levels, and tiring out and completely stopping her breathing all together. And as they told us- she is in the right place, we have all of our equipment ready, so we will continue to watch her very closely. At one point they were drawing her blood (out of an IV line) every 15 minutes to make sure she was still getting enough oxygen in her blood. The intensivist also, at one point stood in the room and held her lower jaw forward to optimize her airway. This did help and after he left I spent quite a bit of time doing this for her (at least there was some small thing I could then do for her).
Anyway- I know this email report is much different that the others, but it is what occured. She finally began to improve at about 11 pm (they took the tube out at a little before 1pm). She still would stop breathing periodically, struggle quite a bit and then finally be able to take a deep
breath (just about the time you were ready to run out in the hallway and drag the nearest nurse in the room) -- and they she would take such a fast deep breath, it would cause her to hurt (because, they had wiped out all of her pain medication before with the reversal agent and they did not want to give her anymore to risk her having more trouble with breathing) Anyway-- she then would have her weak little cry because it hurt her- let me tell you, that was so hard (it actually brings tears to my eyes, now, just thinking about it). At around 11pm they decided to give her some pain medication because (after trying all other options) they thought that
maybe she was holding her breath due to the pain and that was why she would then struggle and have those harsh deep breathes. This did seem to help (which we were glad to have something to help, but very sad to think that she was in that much pain).
Some good news: Olivia then began to greatly improve and this morning looked like a completely different baby- you would think you were in the wrong room. She is definatley a strong kido!
So, for today, they had the ENT (ear, nose and throat) Dr. look at her throat and nose to see if there were any of those hemangiomas (group of blood vessels - like the one on her face) that may be obstructing her airway some- leading to the difficulty she had to breathing. That all
looked pretty good, some small issues- mostly related to having the tube in her airway. She does have one of those hemangiomas in there, but it is flat and not causing a problem, but something to keep in mind and watch for possible complications down the road (as these can grow and no longer be flat).
They also are doing some vibration treatments on her lungs because the Right upper area dows not want to inflate properly- not too uncommon with a surgery and breathing tube) They are doing some breathing treatments and giving her more steroids - all to help the breathing issue. They will do another X-ray tonight at 6pm and see if the right upper lung has improved, if not, they will use a mask that has some positive pressure with each breath to help to gently push that lung area open. Then after all of the lung/breathing issues are settled, we will be able to put a tube in her nose, down her throat and into her stomache (-- a VERY small tube, for all of you who are kringing (sp) ). This will then allow us to get some nutrition into her for the first time.
I tell you, this little girl is strong! She continues to amaze all of us. And she is so mellow. She really does't complain- like when they do the vibration treatments for her lungs/ or even when the ENT Dr. had to put that camera tube into her nose and her thraot to take a look.
Also, through all of this Terry and I have really grown strong together as a unit- something we decided very early on had to happen and had to be something we specifically paid attention to and had to put effort towards. We had issues, things we totally did not intend on having to deal with, that tried to divide us- we recognized it and had to make a decision. --- it really makes a difference when you actually verbally agree to stay strong together. So, despite this difficult and trying time, good thigs are continuing to happen- praise GOD!
Part 4
Things are great today (Tuesday - almost a week since surgery). We got the go-ahead to be moved to the 5th floor, we are just waiting on a room. Sunday night and Monday nigths were not the best for Miss "Livie Lou" as Josh has always called her (even before she was born). For some reason she just could not settle. Her breathing was really fast and Junky sounding (from all of the junk she couldn't quite cough up) she would squirm aroud and given out a little weak cry every once in a while ( the weak cry was from the breathing tube- but it just made her seem all the more...I guess you could say ..sad) It was hard, because you just couldn't figure out what was bothering her.
Monday was probably the worst night. She only slept for 20 minutes in 2 differnt segments, the whole night. They gave her Morphine (just in case it was pain) and also a relaxing medication- thinking she was just so overstimulated from all of the very frequent Respiratory treatments she was getting to try to get the right upper lung reinflated. Last night was much better- only up half of the night and this time the Morphine and Ativan seemed to work. They also changed her eating to allowing her to have a larger volume at one time - to allow her stomache to actually get to a "full" state (which I think was probably the best medicine for her) - something (actually one of the only things) I was pushing for. Her being restless at night , kind of had a little correlation with her previous (before surgery) pattern. So- now that we have changed,again, with allowing her to now eat when ever she wants and however much she wants, I am expecting her to have a good night tonight.
AS far as her lung- that is much improved. She is now only on 2 medications (one we will no longer be on when she gets home) So- just a blood pressure medication (which is very common for babies with the surgery she had)
The Doctors are very open to using the propanolol for Olivia's hemangioma. Propanolol is the heart drug that has just recently been shown to greatly reduce the hemangioma (birthmark looking thing on her face) and therefore, greatly reduce the possiblity of having eye and/or airway problems for Olivia. We are really excited about how open they have been to this possibility. They have even been excited to read the articles I printed off the internet on a study done in France. Our cardilogist is also contacting other Dr's in the US who have some experience with this drug being used for hemangiomas - he is checking with them on any possible
complications or special things they would have to watch for in Olivia's case. (Sorry- for those of you who feel this is way to much info- just skim over it- I know there are a few detailed people---somewhere out there-- who like all of this detail).
I guess I forgot to mention they pulled out her arterial and large venous IV lines that were stitched in place in her groin area of her leg (used to have a continous blood pressure reading)
Today (Wed SEPT 10 --I plan on keeping these emails for Olivia when she is older, or for answering Dr. questions, that is part of the reason for all of the detail and specific dates---juuuust in case you were wondering) .
So, today... we got rid of the feeding tube that was in her nose and traveled into her stomache. They did not want to feed her by mouth until she was stronger and all of her breathing issues were settled. So, she is much stronger and breathing good, most of the time- except when she gets herself worked up. She has been able to eat really well and almost more than she did before surgery. I guess she is making up for lost time. At this rate, she will be growing like a weed. (thanks for the tubs of clothes, Jade-- at this rate she will be in and out of those sizes pretty
quick!) Now all she has is one IV port (or saline lock- for those who know what that is) in her hand. The plan is to make some changes in her blood pressure medication and hopefully home on Friday!!! Can you believe that she could go through so much and be so good! It is really amazing.
We have grown to really like most of the nurses and also Dr.'s here. They are all an amazing team! I just love the way all aspects of a patients care is managed here at this hosp. It is quite different (but, how I have always tried to do for patients in the adult world-- probably why I never got off work on time) It has made me reconsider pediatrics.
Mikala got to come down here and spend the afternoon with me- she is still her now (Thanks to Krissa, who brought her down) She will stay with Walter and Melinda, here in Omaha. She is quite excited about that. She is very excited to play with the boys and also that beautiful, blue-eyed girl--Maggie). I hear there may even be plans for her to get to go shopping with Melinda and Maggie (time for the next stage of baby clothes for her.. she is really growing). Mikala is very excited about this.
WE ARE HOME!!!!
We got home last night (Friday) at a little after 8pm. It was a little scary taking her home- even though I couldn't say I was worried about anything in particular. Probably just the change of worlds and a little of the general unknown... and also knowing that we have to establish a new
level of "normal" in our house/schedule... I hope that makes sense.
We are really excited to have been able to start the beta blocker, Propanlol, for her hamangioma (birthmark-like thing on the side of her face ----which is due to blood vessels being told to over grow---from a genetic mutation that occured at some point of her very, very early developement-- This is what the genetics Dr. has informed us of). It is not a gene that is passed from one parent to the baby. -- This diagnosis is still so new and there is very limited information on this condition. Our genetics Dr. is wonderful! She has spent a lot of time talking with me and also has spent time researching all of this. She has access to a lot of articles and will print them out and mail them to me. She also assisted me in completing some of the details of being a part of a study. I had sent information and pictures to a Professor of Dermatology and
Pediatrics at Baylor College of Medicine and Houston Pediatrics Hospital before comming back to the hospital for her surgery. We also signed a conscent form for them to use Olivia's information and records in studying PHACE / PHACES syndrome. Our genetics Dr. will now help us to get
Olivia's genetic information and possibly her stored blood to them for the purpose of this study. She even took time to contact the Dr.'s in Houston to see how then preferred to go about this. She was very encouraging and thankful to us for considering- being part of a study. I am all for it, as long as she does not have to have any invasive studies - the most I would be willing to do is to give them a sample of her blood for DNA/genetic study (and even then I would do my best to corrolate that with a blood draw she would need for her own benefit).
Our Pediatric Dermatologist has also been very good! She is quite busy, because of her highly specialized practice. I guess there are not a lot of PEDIATRIC dematologists out there. Anyway, she has been in contact with the Pediatric dermatologist in Milwaukee, WI (who is beginning a study on the use of Propanolol for hemagiomas) to get her recommended dosaging on the
Propanolol, for the treatment of the hemangioma. She then gave that information to me and the Cardiologist. So, then it is up to the cardiologist to decide what dosaging they feel comfortable with (since this is primarily only used for cardiac reasons and effects the blood pressure and heart rate). Since Olivia has been started on this medication, her blood pressure was on the low side of normal (right on that border of almost too low) and her heart rate was also doing the same (almost on the border of being too low). The plan was to increase the medication by 50% and send her home. I wasn't too excited about this, especially since there really are no signs and symptoms to watch for in infants, to know when the blood pressure and heart rate were too low. And knowing that her hands already get purple in color and clammy at times - this was not going to be something we could rely on. So, I talked with the cardiologist and expressed my concerns and discussed the fact that the Dermatologist explained to me that the increase in the Propanolol is not necessarily something that has to be done- and that the recommended dose was what they (DR. in WI) believed worked the best, based on limited information, study and results. Since we had already seen some improvement in Olivia's hemangioma, I asked the Dr. if he thought that
maybe this was an appropriate dosage for Olivia in regards to the hemangioma and also a dosage that she is just barely safe with- is it worth the risk of her blood pressure and heart rate dropping to a dangerous level (since she was already on that borderline). He did agree with this and we went back to her normal dosage. This gave me a lot of relief- I know I would have been pretty nervous taking her home and starting that first bigger dose at home and not have anyway of knowingwhat her BP was doing.
Now, we have her aorta fixed and we were able to get her started on the Propanolol prior to discharge (this was exactly what we were hoping for!!) I am so thankful to the Dr's being open and even supportive of the idea of using the Propanolol- and for there hard work to research out the details on how to use it to treat her hemangioma.
Our dermatologist told us that we are one of the first to be using the Propanolol for this reason- I asked her if this gave her any concern, considering the fact that this drug has been around for years and has already been used in babies for heart reasons, alone. She really had no concern, but was excited for us and the fact that we could avoid the use of long term steroids and even possibly the use of chemo therapy to treat her hemangioma (which is what the other children have had to go through- it is hard to look at those babies who have changed so much from being on steroids, and think that would possibly be what we would have to do for
Olivia.) Steroids and Chemo have such terrible side effects and we are so ecited for a much safer alternative for our precious little Olivia. You can't help but be amazed in God's work in all of this. We have been in contact, AND gotten responses from Dr.'s in Omaha, Housto, Milwaukee, and
even France (France is where most of the info has come from--it was funny when I sent my firstmessage to the French Dr. and got an aotomated respone in french, letting me know the message had been received) I was glad for a reply in English!
+++This one is probably more for the detail- oriented people. Sorry if I
lost you in this one. +++
The bigges thing is --we are HOME!!!
We both (Olivia and I) slept the best we have, so far. Every night she gets better and better. Not staying up all night and being so restless/uncomfortable. We are very thankful to God for all of the amazing things we have been able to see Him in. Not only the big things, but the many small details. We have heard from different nurses, how well Olivia has done and we have also heard some surprise at how quickly she has recovered and been able to go home. Now we have Dr. follow up appts ahead of us. (dermatology, cardiac, opthamology, cardiothorasic surgery, genetics, and primary care). And also keeping up on all details for the purpose of her case study.
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