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May 15th, 2009
10:14 AM ET

Remembering two precious brothers and all who suffer MPS

By Beth Karas
In Session Correspondent

A week after the 9/11 attacks, I was reporting from ground zero for Court TV, now In Session, when I received a call from my father that my older brother, Joseph, had taken a turn for the worse. Joe had had many brushes with death but this would be his final one. The next day, I was at his hospital bedside in Massachusetts when he passed away. I spent another hour with him after he died, saying goodbye, and contemplating his life and that of my younger brother, Jonathan, who passed away eight years earlier. While they are in my thoughts daily, today is special because it’s International MPS Awareness Day.

Beth's brother Joe used a wheelchair in the last years of his life.
Beth's brother Joe used a wheelchair in the last years of his life.

My brothers suffered from a rare metabolic disorder called mucolipidosis type III (or ML-III), which they unknowingly inherited from both my parents. ML-III is linked to other disorders called mucopolysaccharidoses (or MPS) which are characterized by a missing or defective enzyme needed to break down and recycle materials in the cells. The body stores these materials, causing the cells not to perform properly. The progressive damage wreaks havoc throughout the body.

Through the years, my mother would tell me that the first time she held Joe in the hospital, she knew something “wasn’t right.” As for Jonathan, his birth was so complicated that he and my mother were hospitalized for several days—my mother recalling time and again her near-death experience. Little did we know what lay ahead. It would be decades before the diagnosis of ML-III would be made, but not for lack of effort on the part of my brothers’ physicians.

Early signs of Joe’s ML-III were his late development in walking and the stiffening of the joints in his hands by the time he was 5. His unbearable headaches at 6 led my parents to specialists two hours from home—at Boston’s Children’s Hospital. Joe’s craniotomy began his, and ultimately Jonathan’s, journey in and out of hospitals for the rest of their lives as doctors struggled to reconstruct joints, alleviate pain, create more mobility and generally improve their diminishing quality of life.

Doctors were mystified about my brothers’ disorder. My mother used to tell me that they came from around the world to examine Joe and Jon in those early years and that my brothers were among the earliest reported cases. As my parents, sister and I watched their disease progress, we knew that some day they would no longer walk without aid. Indeed, by the end of their lives, they both used wheelchairs.

Characteristics of MPS and ML vary depending upon how severely one is afflicted. It can cause mental retardation, hearing loss, diminished eyesight, and damage to the heart and respiratory system. One of the most common characteristics is skeletal, such as short stature and stiff joints. My older brother used to tell me that one of his goals in life was to know what it felt like to have his palm on a table. His stiffened and curled fingers prevented it. Both brothers had their hips replaced while in their 20s and lived with chronic pain from every joint in their bodies.

There is no cure for ML or MPS though there are treatments today for some of the MPS disorders. Those treatments can be prohibitively expensive but they improve the quality of life and extend the life expectancy of those lucky enough to receive them. It is the mission of the National MPS Society, of which I am an active member, that there will be a cure someday for these horrible diseases. Until then, we seek to raise awareness of these devastating diseases and offer hope and support to affected individuals and their families.

For more information, visit  www.mpssociety.org .

“In Session’s” Beth Karas is a journalist and former Manhattan assistant district attorney who has covered some of the country’s highest-profile trials over the last 15 years.

Editor's Note: Medical news is a popular but sensitive subject rooted in science. We receive many comments on this blog each day; not all are posted. Our hope is that much will be learned from the sharing of useful information and personal experiences based on the medical and health topics of the blog. We encourage you to focus your comments on those medical and health topics and we appreciate your input. Thank you for your participation.


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soundoff (43 Responses)
  1. Melissa

    Thank you Beth for sharing this.

    May 15, 2009 at 10:50 | Report abuse | Reply
  2. Chris

    Thank you for creating awareness.

    May 15, 2009 at 13:21 | Report abuse | Reply
  3. Jeff

    My brother is currently diagnosed with MPS and it's very tough on him. I hope new discoveries advance the MPS Society's mission to find better techniques in curing and treating MPS.

    May 15, 2009 at 13:25 | Report abuse | Reply
  4. Amy

    Beth, what a moving story. Thank you for sharing it with us.

    May 15, 2009 at 13:36 | Report abuse | Reply
  5. S Callahan

    What a great article...and a new awareness for me. Godspeed in opening the door for more and better treatments!

    May 15, 2009 at 13:39 | Report abuse | Reply
  6. Shazia Arain

    They wil be with you in memories for ever. My prayers ae with you and them

    May 15, 2009 at 14:08 | Report abuse | Reply
  7. Marie M. Merzon

    Dear Beth, I have watched and admired you on Court TV. Our daughter is a Manhattan ADA , actually she is now the unit chief for Idenity Theft. So I understand how hard you have worked whilst sharing your time and love with your brothers. Your family's story is such a sad one and I pray that you will see a cure for this rare and deadly disease. Thank you and much good luck with your future professional aspirations. Marie Montone Merzon

    May 15, 2009 at 14:08 | Report abuse | Reply
  8. tony

    Beth,
    Upon reading about your brother's illness and the pain they were in and the emotional pain you and you parents must have been in I reflect on my life and how lucky I and the rest of us are to be healthy and with out pain. We take it for granted that we are in good condition. I had my own bout with RSD which causes severe pain in joints that can last a life time for some. But it won’t kill you. I had it for 20 years and it still comes and goes. But it pails in comparison to what your brothers went through. So many of us get self absorbed in our own issues and miss out on enjoying life. I’m sorry for what your family had to go through.

    May 15, 2009 at 14:10 | Report abuse | Reply
  9. K Nelson

    Thank you for continuing to help raise awareness for these heartbreaking diseases.

    My daughter also has a related disease, MPS 1. You can read about her here: http://tinyurl.com/ouq34p

    May 15, 2009 at 14:30 | Report abuse | Reply
  10. Kelly Waddell

    Thank you Beth, for sharing your insight on MPS/ML with the world, and spreading the word for all of those with MPS/ML. As a mom of an MPS child, I cannot count the number of times I have had to explain my son's disease to someone..... even doctors.

    God Bless.

    May 15, 2009 at 14:34 | Report abuse | Reply
  11. Eric

    Beth, your story and the lives of your brothers give hope and courage to all who suffer from any disabling disease. thank you so much for sharing such personal information.

    May 15, 2009 at 14:50 | Report abuse | Reply
  12. Alicia Bennett

    THank you so much for sharing your brothers with us all. I am the mother of three children with MPS-III. Ciara is 13, Hunter is 11 and Tommy passed away five years ago when he was 4. God bless all MPS/ML children and our angels in Heaven!

    Hugs,
    Alicia Bennett – http://www.caringbridge.org/ca/bennettboys

    May 15, 2009 at 15:35 | Report abuse | Reply
  13. Jennifer Restemayer

    Thank you Beth for sharing such a personal story, it is so nice to see that people are getting out there to raise awareness. My 8 year old daughter, Allison, was diagnosed with MPS 1 at the age of two. Here is a link to a vidoe that both of my daughters worked on for MPS/ML Awareness Day. http://www.youtube.com/watch?v=jTdfZA1LRyo

    May 15, 2009 at 16:33 | Report abuse | Reply
  14. Mieke

    Thanks, Beth, for sharing your story and helping to create awareness of these terrible disorders! My son has MPS-IIIA, Sanfilippo Syndrome. Luke will turn 10 next month, and we've learned to live in the present and enjoy each and every day. Life expectancy for children like Luke suffering with Sanfilippo Syndrome is 10-14 years.

    We wore our PURPLE today and passed out purple ribbons to Luke's dentist and his staff this morning, as well as sharing MPS information with his school. Thanks, again for sharing your brothers' stories!

    May 15, 2009 at 16:53 | Report abuse | Reply
  15. Phyllis

    Thanks to Beth Karas for writing and to CNN for sharing this story on MPS Awareness Day. My prayer is that increased awareness may hasten the day when the children who're affected can be restored to health; when they and their families can look forward to long and joyous lives!

    May 15, 2009 at 18:21 | Report abuse | Reply
  16. Pam Umstead

    Thank you, Beth, for your thoughtful remembrance. Our older son Scott went to Heaven last October, at the age of 36, from the effects of MPS III-A...much longer than most MPS III-A patients live. He began life as a healthy baby; thrived as a toddler with a head full of blond curls, bottomless blue eyes, and a mischievous grin; and then his disease progressed. He lost the ability to keep up with his peers mentally, to run, to talk, and to see. The journey was difficult but God was faithful to carry us through it and show us blessings in the midst of the pain...and to reveal how Scott blessed others along the way. Now Jesus has healed him, and we know we will be together again...that is the promise and the hope with which we live each day.

    May 15, 2009 at 20:14 | Report abuse | Reply
  17. Barbara Hays

    Thanks for sharing this, Beth. Our grand-daughter was diagnosed with MPS-1 (Hurlers) shortly after her first birthday. While it explained the health ups and downs of her first year that had kept us "wondering", it was quite devastating to learn of this rare metabolic condition, that we had never heard of before, and many related lysomal disorders like that of your two brothers. We could say more about her cord blood transplant treatment and the one year check-up she is going through this very week. But today, we are remembering the special courage of all individuals and families who have been affected by this disease, the resarch and medical teams that are working so diligently to find cures, and the wonderful network of support offered by the National MPS Society.

    May 15, 2009 at 20:30 | Report abuse | Reply
  18. Brenda

    this is really touching and revealing about your brothers..You are Great! Brenda

    May 15, 2009 at 22:14 | Report abuse | Reply
  19. Jenn

    Thank you Beth for sharing the story of your family and how you live with MPS. Our daughter has MPS 1 and was diagnosed early – we hope that treatment will continue to help keep some of the symptoms at bay. Wonderful picture of your brother.

    May 15, 2009 at 22:30 | Report abuse | Reply
  20. Barbara Pryor

    Thank you Beth for sharing your story on International MPS Awareness Day. My nephew has MPS II or Hunters Syndrome and is 11 yrs old. When Andrew was 3 1/2yrs old he was diagnosed with MPS II and we were told that he wouldn't live past the age of 5yr. Due to the National MPS Society raising money and awareness, Andrew is one of the MPS children that get the Enzyme Replacement Therapy or ERT. When we first learned about MPS there were no treatments for any MPS children and now there is ERT. One day we hope that all of the MPS/ML syndromes will have treatment.

    May 15, 2009 at 22:48 | Report abuse | Reply
  21. Shanna

    Than you Beth for sharing this with the world.

    May 15, 2009 at 23:49 | Report abuse | Reply
  22. Adene Balzer

    Thank You, Dear Beth for sharing your story with the world. I'm a sister, mother, grandmother and nurse and as a member of the human community, we each have a special place in sharing our individual experiences. Someone is the better for that. Someone is stronger for the knowledge that they are not alone in the battle that they are experiencing. Life offers so may challenges that none of us knows the answers to but, at least there are wonderful people out in this universe that can offer some solice, care, and kindness, as I offer to you. Stay strong and keep telling your story. Many of us need each other to stay strong. Namaste. Adene

    May 16, 2009 at 01:30 | Report abuse | Reply
  23. Kevin and Andrea Gates

    Thanks Beth for sharing. As with many rare diseases we need to get the word out to as many people as possible about Mucolipidosis. My 12 year old son Spencer suffers everyday from this disease. His hips are the main source of his pain and are so bad now he can hardly walk. Seems like your experience with your brothers is exactly what we are going through now.

    May 16, 2009 at 02:51 | Report abuse | Reply
  24. Priscilla Raftery

    Beth, thank you for honoring your beautiful brother's by sharing their story...their courage lives on through your words and your love ~

    mom to Joey, MPSIIIB

    May 16, 2009 at 07:33 | Report abuse | Reply
  25. Pat

    Thanks for sharing about your bothers. We too travel the road of MPS and ML with MPS Type III. Again as you say there is no cure and not even a treatment to help with MPS III yet. We are trying n experimental therapy so to speak that is recognized in Poland with genistein and we have seen a slow in progression of the disease. Will it be enough to sustain our child until a cure??? I doubt it, but it gives us a longer tme and better quality of life with our son.
    Thanks for publically sharing your story with others!

    May 16, 2009 at 08:24 | Report abuse | Reply
  26. Misty Luthcke

    Thank you for this article. My son Will, is 6 years old and suffers from MPSlllA. So little is know about these this orders. Our job as parents and family members of these individuals is at least to raise awareness. Beth, thank you for raising awareness and I am so very sorry about the death of your brothers, Misty

    May 16, 2009 at 08:34 | Report abuse | Reply
  27. Melissa Hogan

    Thank you for your heartfelt story. My son has MPS II and until his diagnosis, no one I know had ever heard of it. In fact, my mother (a nurse) diagnosed him after seeing an episode of Mystery Diagnosis. That alone proves the need for more publicity surrounding MPS and other rare disorders. Thank you again for publicizing MPS Awareness Day!!

    May 16, 2009 at 10:07 | Report abuse | Reply
  28. Cynthia Anderson

    My son died at the age of thirteen in 2004 of MPS – Sanflilippo. He was a normal child , laughed, smiled and ran around the house, climbed steps then at the age of 4 when his speech was not going in the right direction, test were done and we found out that most sanflilippo victums dont live past the age of 14 – 21.
    That is because of a enzyme that we was born without, wouldnt be there to eat the mucus that our organs make and this mucus attacks the muscles and everything else.
    MPS – is making head way, maybe finding a treatment so kids that our born today with MPS they just might have a chance. May 15th is the day we remember the ones that have lost the battle but there might be a future for someone with MPS.

    I thankyou for sharing this rare disorder

    May 16, 2009 at 14:04 | Report abuse | Reply
  29. Erica Thiel

    Thank you, so much for raising awareness of these truelly devastating diseases! As an adult with MPS I I am thankful there are so many people working to bring more awareness!

    Erica http://www.caringbridge.org/wi/ericathiel

    May 16, 2009 at 23:56 | Report abuse | Reply
  30. jennifer yard

    thank you for sharing your story my daughter kaylin hafner is almost 3 and has mps-1 hurler's disease. she has had a cord blood transplant and has been recieving ert since dec 2007 we are very grateful that she doesnt have any of the serious health problems right now and fortunate for the research and care that has been made available to kids like her. it is true we need to make more people aware of this disease so that a cure can be found.

    May 17, 2009 at 00:18 | Report abuse | Reply
  31. AJ

    Bethie:

    Your family story has touched many. Your brothers suffered so much, but were so very fortunate in one remarkable way–they knew the caring and adoration of a sister who loved them unconditionally. Much was taken from them because of this wretched disease, but God saw fit to give them the undeliable gift of you. And in the shadow of their valiant struggle, emerged your bravery and tenderness.

    Their story and yours are an inspiration.

    May 17, 2009 at 01:50 | Report abuse | Reply
  32. kelli Busey

    My heart goes out to you and your family Beth. Always with best wishes your friend,
    kelli

    May 17, 2009 at 05:17 | Report abuse | Reply
  33. Lisa

    Beth,
    Thank you so much for sharing your story and shining a light on ML and MPS. My 4 year old son was diagnosed in Oct. 2008 with MPS II and we are praying for a cure in his lifetime. The National MPS Society will play such a huge role in funding research to reach that goal.
    Lisa

    May 17, 2009 at 09:20 | Report abuse | Reply
  34. Liz Provo

    Beth. Thank you for sharing this part of your life in order to help others. You are a true inspiration. I've been watching your career progress for years and I'm a huge fan. Take care.
    liz Provo

    May 17, 2009 at 11:56 | Report abuse | Reply
  35. Denise Crompton

    Hello Beth, Glad to see this post by you. We spoke on the phone a few times a number of years ago, when we connected through the MPS Society. Our daughter, Kelley, did very well to make it to the age of 45 with ML III. She died two months ago... pretty memorable that someone named Kelley (my maiden name) happened to leave us on St. Patrick's Day.

    We appreciate the many advances in medical science and some really wonderful doctors who performed the necessary operations to keep her functioning as long as she did. However, the last six months of her life were pretty difficult, mostly with breathing problems. She is at peace, but her positive spirit and happy smile will remain with us forever!

    May 17, 2009 at 18:08 | Report abuse | Reply
  36. A.J. Miller

    What a moving story, Beth.

    As a jeweler in my first career with a very hands-on intensive second career, as well, I just can't imagine what I would do or how I would feel if I did not have the use of my hands. Thank you for sharing your story and reminding me to be grateful every day for the blessings of good health that I enjoy.

    May 17, 2009 at 21:55 | Report abuse | Reply
  37. Mary Ellen Morell

    Dear Beth, You are well known and respected and your beautiful story has touched many hearts. I live in MA and know the many fine hospitals that work so hard to treat and try and prevent so many different afflictions. You have also made us pause and look at how treatment helps the family and caregivers of the patients. Your love shines through–keep up your wonderful reporting on Tru TV. God bless you and your family.

    May 17, 2009 at 22:17 | Report abuse | Reply
  38. Geraldine Smyth- Ireland

    I was sorry to read about your brothers disease- my son has MPSII- Hunter Syndrome. You have done a great thing here, writing about your own sad experience with these horrible disease, your brothers would be proud, i am sure.
    Thank you for this piece on your brothers you have helped by sharing your heart breaking story,
    May they both rest in peace.
    Go Raibh Mile Maith Agat!- (thank you in Irish)
    God Bless

    May 18, 2009 at 16:25 | Report abuse | Reply
  39. Jon Bresnahan

    My name is Jon i am 28 years old and living with MPS II Hunter's Syndrome. I've been getting the treatment for over 5 years now and it has helped me so much. i was one of the first 10 in the Enzyme Replacement Therapy study they had in North Carolina. Thanks putting this article out to help with awareness.

    Jon

    May 21, 2009 at 12:46 | Report abuse | Reply
  40. glenn miller

    Hi beth my name is glenn miller me and my wife lost our son little glenn he just turned 18 years old.he had mps hunters.we lost glenn october 31 . 08. i just cant tell you how hard this mps is glenn lived every day to fullest . i just wish we could get this out there more. me and my wife are going to do the best we can to keep working to get the word out. and i am sorry for your lost.we have a hard road ahead and even how hard it is for us there many folks out there doing the same thing we did.we cannot forget them .thank you so much for this story this means alot to me and my wife.we miss you little glenn.love your dad

    May 27, 2009 at 21:46 | Report abuse | Reply
  41. Stacey Montgomery

    Thank you for sharing your story and spreading awareness.
    Our son suffers from MPS III Sanfilippo syndrome and we hosts fundraisers for the MPS Society in hopes that eventually there will be a treatment to stop the progression in all of the MPS/ML disorders .
    http://www.caringbridge.org/visit/hopeforlucas
    Here is a link of a slide show of other chilren with MPS. I created it for our Awareness Event http://secure.smilebox.com/ecom/openTheBox?sendevent=4f544d314d7a67794d513d3d0d0a&sb=1&campaign=email_receipt_directurl_makeyourown

    June 3, 2009 at 13:03 | Report abuse | Reply
  42. Susan Kester

    Beth
    As a mother of a 21 year old boy with ML III, I can't thank you enough for writing this article and sharing your story. Awareness is so important. Thanks again.

    June 17, 2009 at 14:06 | Report abuse | Reply
  43. Johanna

    Beth,
    Just wanted to say say your article was beautiful and touched my heart. My niece, (just turned six), has ML II (after being misdiagnosed and going through unnecessary procedures because of it). I try everyday to make at least one more person aware of MPS/ML. On the 15th, everyone I knew wore purple and we all (u=including my three children), handed out purple ribbons.

    June 17, 2009 at 14:07 | Report abuse | Reply

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