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My Marfan Story

Heads up to anyone reading this...its a long story. Hi my name is Tiffany Wolfe I am a 26 year old Mother of one beautiful (adopted) daughter and I have Marfan syndrome. I am what the doctors call a spontaneous case.There is no family history, no one in my family has Marfan syndrome but I just happened to be born with it. When I was two years old my parents began to worry, I was the size of a average 4 year old (tall) I walked on my ankles (severely flat footed) and I would walk into furniture and was very clumsy ( legally blind). my parents sought out help for my vision problems, luckily the optometrist who had seen me immediately recognised some of the symptoms I was presenting and thought that I may have Marfan Syndrome. I was referred to a geneticist who agreed and I was diagnosed with Marfan Syndrome (although there were no genetic tests for Marfan at this time). I began seeing a cardiologist soon after my diagnosis. My parents struggled to take care of all my needs, it was a real struggle to get me into leg braces (but they managed and I wore them day and night). My younger years I had it pretty easy medically but with my tall stature, my thin glasses and my buck teeth (due to my cleft palate) I was teased relentlessly by my peers. when I was 7 years old I awoke in the night with severe eye pain my parents took me into the hospital and they found that my lenses had dislocated. The decision was made that the lenses would be removed. due to the weakness of my tissue the doctors decided that they would not be replacing my lenses, instead I would wear contact lenses. Thankfully contact lenses worked for me I had near 20/20 vision in my eyes. at the age of 8 I got braces (THANK GOD!) I could not even close my mouth because my teeth were pushed so far forward. I continued to do well for a few years but at the age of 11 my cardiologist discovered that my aortic valve was needing a repair he referred me to the hospital for sick children in Toronto Canada where I had my surgery. The surgeon had decided that instead of doing valve replacement he would "simply" repair the valve. 6 months later I had to go back under the knife because the valve sparing procedure didn't work. The next surgery was done and a mechanical valve was successfully put in. 4 months later I began presenting symptoms of a cold/ flu this lasted nearly a month and began to worsen. I was in renal failure and I was rushed into the children's hospital via air ambulance and had immediately undergone emergency heart surgery. The surgeon decided to do a bentol procedure ( a mechanical valve and a section of the aorta). 3rd times a charm! The surgery worked I recovered from the blows of having 3 open heart surgery inside of a year and went on with my life. the trauma done to my body hadn't gone unnoticed by my peers. I had scars and as a result of having my heart 4x its normal size. my breast bone was extreamly narly it was bumpy and jagged (protrusion of the sternum made worse by the surgery)...I hide during my teen years behind sweaters. At the age of 16 I got breast implants to help hide my ugly sternum..this just made it look like I had 3 breasts. At 17 years old I had my first lung collapse ( Pneumothorax) it was repaired by inserting a chest tube and a few nights in hospital. through my teen years and early twenties I sought out a surgeon who would dare fix my breast bone (given all the scar tissue) ...after 7 years of searching I found a surgeon who would do it, with the stipulation...a Heart surgeon needed to be present during the operation. after years of torment and thousands of days spent covered by baggy sweaters I finally had a surgery date (I was 21 years old). they literally broke my entire chest plate into 6 pieces and turned it inside out...and it was worth it..the biggest sigh of relief...I wore tank tops all summer long. I had a 2nd pheumothorax the summer following my breast bone surgery (22 years old), one lung had fully collapsed and the other was half collapsed they had to operate and fix the tear in order to re inflate my lungs. At 23 I was told that I could no longer wear contact lenses (they were suffering my eyes). I underwent a lens replacement (intraoccular lens implants) that year I was married to my boyfriend of 6 years, happy to begin our lives together the idea of children was on our minds. The decision to adopt wasn't easy, I think every woman longs to see what their biological child looks like..especially because it is a mix of yourself and the person you love most...I explored many different avenues, I knew that pregnancy would weaken my tissue and could cause more damage, I looked into surrogacy, but at the end of the day I could not knowingly pass on Marfan Syndrome to my child..I was a sick child..I seen my parents break down several times and I didn't want the heartache of "dealing" with a sick child...My sister offered to donate one of her eggs..I love my sister but I couldn't risk putting her in that position.. or the pain of loosing her first child to me...my husband and I had decided to try the adoption route we placed our names on the list and were picked within 1 month of applying our daughter was born on January 24 2011 and we brought her home on January 26th 2011. Our adoption journey was amazing, and we are totally in love with our daughter...and ironically she looks just like me (minus the height). On Dec 22 ,2011 I had severe pain in my back and chest (like a ripping) I was rushed to the hospital and it was found that I had a type b aortic dissection...the doctors tried to manage the dissection with blood pressure medication. On Dec 23 they found that the tissue had weakened and I had an aneurysm that was about to burst the emergency surgery took place on midnight of December 24th. The surgeon replaced the section from the top of my aortic arch to my diaphragm. I recovered fairly rapidly and was home from the hospital by new years where we celebrated the holidays with our families. on April 16 2012 I was admitted to hospital from back and chest pain. A c.t scan found that another aneurysm has been growing right at the point where the prosthetic aorta and the natural tissue meet...I will be having another surgery within the next few weeks to replace my aorta (from my diaphragm to my groin). this same c.t scan also uncovered a bulge (aneurysm) at the point of my aortic valve and the natural tissue (opposite side of the arch) this will also need to be repaired in the up coming months. Well like I warned you I had a long story, but I needed to share my story with others, not to scare them but to enlighten them..I also hope to find and communicate with other Marfan's people. thanks for reading ** update Oct 29 2013 ** My aorta surgery was successfully completed. The aorta is prosthetic ( Dacron) from my arch to my femoral arteries. My vocal cord surgery was also successfully completed and I sound similar to how I sounded prior to it being paralyzed.. For more information regarding Marfan syndrome or if you have questions for me. please email me at tiffany_wolfe@hotmail.ca.
twolfe85 twolfe85 26-30 14 Responses May 2, 2012

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Hello all, my daughter Elle has Marfan's and there are always crossroads to choose when it comes to this syndrome. Yesterday, her eye specialist told us that we have to make some quick decisions about her lens being dislocated. She can have them removed or get implants...I have been searching the internet for answers and can't find what is the best. She is 5 years old and I don't know if she will respond to removal and wear contacts, or just get the replacement and get it over with. I would appreciate your experience on the subject and maybe a little incite on the subject from someone who has had the similar problems. Any info would be great!!

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Hello, I to have Marfan Syndrome. I am 14 and currently 6 feet 7 inches tall. I am also spontaneous, no one in my family has Marfan but me. This past november i had. My aortic sparing surgery. Luckily they did not damage my aorta. I healed from that pretty well, but there was a some fluid after the surgery around the aorta that put me in the hospital another few days. As a result, i got another month off from school because they had to monitor it. I love basketball thats always been one of the hard parts of Marfan; not being able to play. My marfan was also discovered by my eye doctor when i was 6. He noticed my retinal detachment and i guess i was referred. Im quite fortunate and thankful however to be located only and hour away from Johns Hopkins so I've always been monitored well. Im still young and not too many health concerns have risen however I've found that my eyes have always been difficult. It hard generally to see far away but I've been told that i could cut diamonds without a microscope up close because of my nearsightedness. Sorry it's so long i tend to rant.

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Sorry my story is long as well.

Hi Tiffany,

After reading your post, I found that we have a lot in common. My Marfans was inherited. My mother and my younger brother had marfans, both passed away early in life. I am currently a 46 year old male. My wife and I also made the decision to adopt. We adopted our daughter in 2000. We tried to adopt again in 2004 unfortunately the adoption agency turned us down the second time because of my health problems. We are very grateful that we were able to adopt our daughter. I have had all of the symptoms repaired that you have experienced, with the exception of the dislocated lenses. My major heart surgeries started in 1993. I have had 2 mechanical valves for 20 years now. I am grateful for all of the new technology improvements. My first and second aneurysm repairs were difficult surgeries. I only found one surgeon would undertake the second surgery. The surgeon described the process like opening up a frozen orange Juice can in order to repair the Aorta. Technology has improved a lot since then. They can now go in through your groin area and repair it without major surgery. I think the cosmetic surgery I had at 12 and the collapsed lungs that followed have contributed to my current restrictive lung disease. I also have a problem with my heart beating erratically. I have had a couple of ablation on the top of the heart to fix some of the problems. The latest problem I have is in the bottom chambers of the heart. The doctors say they cannot fix it because of my mechanical heart valves. Thank god for new technology my pacemaker deliberator has saved my life more than 15 times in the last couple of years. I hope soon there will be some new technology that will allow them to fix the current problem. I had a very successful professional career as until I was 41. Since then I have not been able to work because I could not make adjustment that would allow me to continue my employment. Thank god for disability insurance I signed up for when I was 23. Good luck to you and your family.

Thank you for sharing your story with me. It isn't often that you meet other marfans. let Alone, another Marfan patient who has had many of the same struggles. My husband and I were also turned down by international adoption agencies. However, we were able to pursue a private adoption ( open adoption) through a local agency. I was worried that we would not be able to be approved for another open adoption, but luckily I was able to get my surgeon to write a recommendation to the agency. We are currently waiting on an adoption list. Hopefully, we will be able to adopt another child one day soon. I am currently on disability and am unsure when,or if I will be able to return to work. My husband has amazing benefits at his job. through him I am also insured. I have heard of stinting. This is the next plan for me, should my femoral arteries begin to dilate. May I ask when your first aorta surgery was?

My son, Harrison must be spontaneous mutation as well. He is currently 11 years old and stands at 6 ft 5 inches. He has dislocated lenses and has some scoliosis issues on his right side. His feet are terribly flat as well. He has 4.3 cm aortic dilation. One cardiologist recommends aortic replacement when he reaches 5.0 cm while the other 4.5. How long was your open chest recovery? I can understand your hesitation in becoming pregnant. What a loving gesture to consider adoption! I am in awe of all you have experienced and wish you continued good health and all of God's blessings!
Deena

Hi, my apologies for taking so long to write back ( I don't often come onto experience project). Is your sons aortic valve leaking? Or where on the aorta is the dilation? Luckily, the technology is available and aorta surgeries are being done successfully. Heart and aorta surgeries take about 8 weeks to fully recover from. I was out of hospital in 5-7 days post opp. The first 2-3 days post opp are the most difficult. Abdominal aorta surgery, the the incision runs lengthwise along the stomach. opening the chest wall ( open heart surgery) the incision runs lengthwise along the chest. They have to break open the sternum so this surgery is a little more painful but after day 2-3 people a generally feeling pretty good. The Thoracotomy ( incision on back running along the shoulder blade and across the side) this surgery has to cut and stretch muscle. The muscle pain is pretty painful, however with a light massage and using massage therapy throughout recovery it significantly helps. They have also began using stents. by going through the femoral arteries and stenting the aorta.they do not have to do any open procedure. However, as explained to me by my surgeon.. He did not recommend stenting on young patients and marfans because they have to hook the stent into place and marfinoid tissue may not support the stent long term. I hope everything goes well and your son can hold off having surgeries for a few more years.

i would love to send you a copy of my project but i dont know where to send it to. please resond soon because my class is almost over. how did your surgery go?

I just seen my surgeon the surgery went well. I should have a relatively normal life. You can email me at Tiffany_wood@hotmail.com

I am glad I have Inspired you. I actually had my last surgery this past December (the doctors took their sweet time planning) I am one
Of the only people in North America with a entirely prosthetic aorta. I am blessed that the technology was available. I look forward to the next chapter of my life... I have been given a clean bill of health and am hoping to adopt another child. I will be sure to continue posting updates :) good luck on your project :)

Did they have to make a special graft on your renal arterys to replace the entire Aorta? I have a couple of anurysms that they are watching. One is a patch of an old repair and the other involves an anurysm where the kidneys connect. Let me know if you would be willing to share this information. I would also like to know who performed the surgery.

No ,none of the connecting arteries are prosthetic. However, because there are so many connections to the spine only the main ones are connected. One of the main concerns is getting adequate blood supply to the spine. There is a risk of becoming a paralyzed due to complications and high spinal pressure after surgery. I am from manitoba Canada. There were 3 surgeries done. Though, had the first not been an emergency it would have only been two. Doctor Michael Moon, Dr. Pasco, and Dr. Guzman were the three who did my aorta operations. Dr. Glen van No ,none of the connecting arteries are prosthetic. However, because there are so many connections to the spine only the main ones are connected. One of the main concerns is getting adequate blood supply to the spine. There is a risk of becoming a paralyzed due to complications and high spinal pressure after surgery. I am from manitoba Canada. There were 3 surgeries done. Though, had the first not been an emergency it would have only been two. Doctor Michael Moon, Dr. Pasco, and Dr. Guzman did the aorta surgeries. Dr. Van Arsdell ( sick kids Toronto Canada) did my valve replacement and a small piece of my aortic arch.

<p>omg im doing a project for marfan's in school and im on a laptop at school, im in science class and everyone is lookingat me because i am bawling my eyes out. you never know how much you take your healthy life for granted when you have a simple cold. this story was so inspiring to me. thank you.</P>

my daughter has Marfans, was dianosed at 8 months old, though they suspected from birth due to her long fingers, legs and arms etc. She wore glasses at 3 months, had her dislocated lenses removed at age 2 after suffering glucoma (acute onset) due to the dislocation, a very scary time i might add. She has heart involvement and takes beta blocker medicine daily, She is 6 yrs old now, she is 4ft 10 and a british adult size 4 foot, im only a 5...she too was a spontaneous mutation of the gene. I am so scared for her future, i wonder what she has in store for her, surgery wise and socially, hope she doesnt get bullied. Thankyou for your posts, it helps me as a mother to read others that understand, not that i wish any sufferinfg on anybody.

I absolutely cannot imagine what my poor parents had gone through with me. I would much rather be the one laying on the table than have to watch my baby go through anything. The best thing you can do, Is be that constant support for your child. Though, I'm sure it is hard. teach her to look on the bright side and keep her positive. My dad was absolutely instrumental in helping me through all of the ups and downs of marfans. He taught me that their is always a bright side and that no matter how difficult I thought I had it..their was always someone who had it worse. Luckily, the advances in technology are amazing and things can be done to ensure that your child grows up happy and healthy and lives a long life. Have her monitored closely by a doctor. And make sure you have a cardiologist on board. As a child I had a MRI yearly to monitor my heart and aorta. Does your daughter have any vision? What are her doctors saying about her sight? Utilize the marfans associations they can be very helpful. And educate yourself and your daughter. Hopefully, she will have a mild case and won't be afflicted too severely. Thanks for posting :-)

It is really nice to read to be able to read your stories! Tiffany I also had my surgeries at Sick Kids and am now seen at TGH. Congratulations on your daughter :) I am in awe of your story and the many surgeries you have gone through! I can relate to some of them, but aside from the three surgeries I've been okay. I'm also a spontaneous mutation, they can't find a history of it in my family. I don't know anyone else with Marfan syndrome, although I am planning on attending the NMF conference in Chicago this summer, I'm excited but nervous. It would be really nice to chat sometime if you're up for it, I am especially interested to hear about your eye surgeries and your chest.. I've been thinking about them but have not initiated anything.

I have never been to a meeting with other Marfans either I think it would be nice to go and hear others stories. yeah I would be up for chat although I will be going for surgery on May 29th so I may not get back to you right away. what did you have your 3 surgery's on?

Absolutely :-) I'm on face book and you can email me any time at either tiffany_wood@hotmail.com or at tiffany_wolfe@ hotmail.ca. I love meeting people and sharing stories. Sometimes having a rare condition can feel isolating. I am always happy to meet and connect with others. Thanks for your message

I was born with the dislocated lens they told me when i turned 16 most likely my lens would completly fall and my vision would worsen but that never happened. Right after heart surgery my vision got worse an every 6 months its been getting worse mainly n my right eye thats the worst one. Im legally blind without contacts or glasses n the right my left is alot better i wear contacts everyday an recently been wearing glasses over them to drive it really helps an clears things up. Did they have a problem at all doing your implants? Did u have a problem at all with finding someone to do them?

I was very young when my lenses dislocated I was 8 years of age. initially they decided that they would not do lens implants (other Marfans had done the lens implant surgery and the artificial lenses didnt stay in place and these patients went blind) I had always been afraid to risk getting the intra occular lenses for this reason but I had come across a few marfans that had had iris fixated lenses with no problems...I reserched and found myself a doctor who had experience with other marfan patients I have no had both eyes implanted with lenses and I have 20/20 vision in both eyes (no glasses). to find a doctor start with your optometrist or family doctor..this surgery is exactly like surgery for Cataracts. good luck :)

I have short stature marfans syndrome, i am petite tiny features, i have to buy my clothes from the misses isle. I can only wear children s shoes. My ring size is a 5. I wore braces on my legs as well as a child, and i have the internal problems as well. We just have opposite problems. short stature is very uncommon, but many have it, like myself. i know one other female but thats it. it runs in my family, but i seem to be the only one it spread to. atleast in the members i know and associate with. many of my family live in different southern states that i dont know. nice to know you, my name is camie. my son has tall marfans, hes 17 and hes 6 foot tall. and counting lol

thank you for replying your story is very interesting I had never heard about small stature Marfans before.It is kind of neat that your son has tall Marfans..how tall are you if you dont mind me asking? is the elasticity of tissue still a issue with short stature Marfans patients?

Wow your case is similiar to mine except mine isnt as severe as yours yet and im sorry you have went through so many surgerys:( i will be praying for you ! I was born with dislocated lens an was seen by so many doctors when i was young but there were no tests to prove i had marfans. No one in my family has it either and alot of the symptons I do not have my height is only 5'7. I am 25 now but when i was 22 my aorta dissected all of a sudden so i had open heart surgery an have a mechanical valve now. I have the type A an type B dissection w the auesyrum. So now doctors are certain i have Marfans. I was actually searching to see if anyone has gotten breast implants with having the mechanical valve i am wanting the surgery done but wasnt sure if its possible. I see u have had it done. Did it affect anything? Im glad i came across your story ;)

yes I have breast implants I got them when I was 16 because of my breast bone. at first I got the saline but because I had little tissue (no boobs) the implant rippled...I eventually got the gel silicone and they are amazing...no problems at all and because they are gel they are less likely to leak. I am glad you replied I find comfort in knowing their are others who have went through similar battles as myself...may I ask what happened with your dislocated lenses? do u wear glasses?

hello Tiffany<br />
It's faith wiens Denise's mom I will pray for you and hope you get better. We love you. Faith and Denise Brown

thank you Faith :) ♥ love you guys too