Hi ,
Well,  as result of my finger in the door accident and the resulting therapists appt. Bryn is being far more responsible. We have let him decide when to go to bed - he is going at 12:30 on a school night!! I am not happy with that and tell him I don't agree but I don't change the time. And he says to me "I know you don't like it" CALMLY! Six hours of sleep?! But he desperately needs to have the choice in this  - I see that.

Our therapist says that he is too bonded. When she says that I feel guilty or angry at her. I don't think he is too bonded. I think what happened is that becasue of the trauma of ALPS and the diagnosis he was unable to take normal responsibility for his age. He would go nuts at the drop of a pin. So in this way he has reached 17 and he is delayed in his development. He is normal slothful 17 year old boy, too, but I do think he is delayed also. He is crying for freedom when what he really needs is the ability to take responsibility so that he can be more independent and trust that he will be able to manage on his own. Somehow he knows instinctively that he hasn't developed that and he feels trapped by it. He NEEDS these skils at this point. And now when we try to teach them to him he rebels because that is normal for 17 year old individuating and gaining independence. So his rebellion traps him in the corner where he is stuck with no responsibility skills. The rebellion is normal too.

We broke that trap last time at the therapist. As hurt as I was that she didn't address his nastiness to me, or even offer a kind word to me - she brilliantly got him over the hump into accepting responsibility for more freedom at bedtime. I know she couldn't be seen to ally with mom and she couldn't move him off his teenage crazy head but I wish she had said to me something like "that was a tough session. Good work mom. Are you OK?" even in private. However, nonetheless, she got him over a very important hump. He is doing things that he should be  doing around the house much more readily and demanding to do things by himself rather than tossing them at me all the time and saying he is too tired or busy or whatever. He WANTS to do things. Brilliant!! Now if he would get some sleep that'd be great..... all in time.

:-)
Cathy

Hi,

Last night Bryn had what is becoming thankfully very rare,  - a meltdown. We are asking him to be off his video games and Skype 40 minutes before bed so he can wind down. He is not happy about this - all his friend are on until they go to bed and he wants to do the same. Also he made a deal with me to stay up late last Friday night if he got off games at 10:40 p.m. this week. He doesn't like to keep his deals.  When I told him we would turn the Internet off if he didn't get off he blew up.

He started slamming his bedroom door which is awful -we live in a townhouse and it is very disturbing to the neighbors. I put my hands on the door to stop the slam from being so hard and stupidly got my fingers caught in the door as it slammed. One nail was pulled right off and the other is very bruised. Bryn didn't mean to do this, but these are the things that can happen when someone goes ballistic.

Bryn was very upset and this became a good moment, after I had stopped writhing in pain for him to look at himself some more. Why is he so attached to the games and being on multi-player until bedtime that he would go off the deep end? What is going on deep inside that he is this desperate? I know he uses the games as a kind soothing mechanism for his anxieties around feeling different and afraid because of ALPS. I am not happy about this but am hoping the therapy he is doing will eventually move him to get off this by himself, as he heals emotionally.

Now I have been to emerg. and had an x-ray and my finger tip is fractured. I have to give up my clarinet playing for the time being, my orchestra concert on Dec. 1 which happens to include a piece that I fell in love with at the age of 9 or 10, the Grand Canyon Suite, which led to my love of orchestral music - it felt so full circle. My quintet is starting up again after 6 weeks off and I can't do that. These are my passions, loves and where my friends are, the things that I do. Teaching will be difficult too, without being able to play my clarient It's a real bummer.

Well, no one said parenting is easy. PArenting my son with the complications of ALPS is even harder. This was certainly an accident. I really love this boy. Damn it! Life....

Cathy

A Breakthrough

"Hi,
We took Bryn to the therapist last night to work out things around my injured finger. He was very upset. she asked what happened and I explained what led to the slamming doors. And from there she led to why he was so angry that he was slamming doors. He said because his mom "always", "never", "totally" "thinks she knows everything" "will never change" "is a control freak" keeps him in a glass bowl and mocks him"-- all very black and white exaggerated comments that hurt me quite a bit because they are just teen drama and not true. Eva worked with that and eventually moved him towards a version of Freedom, which is what he was crying for more of (esp. not to have a bedtime on school nights) which included taking more responsibility. He thought that was garbage at first but suddenly changed his mind. He agreed to get himself up in the morning, take his dishes to the kitchen, turn lights of, clean his own room etc, all of which he has steadfastly refused to do even having been asked a LOT, like maybe a million times. His  condition left him so stressed and upset that anything extra has been too much, including responsibilities - in this way he is less mature than his counterparts at school. Well, he stayed up until 12:30 a.m. which is way too late and that upset me, but I had agreed to this so I had to stick to the plan....I woke up in the night and was really upset, couldn't sleep. But he DID get himself up for the first time ever. He made his breakfast, cleaned up, brought the paper in and got to school all by himself. We actually stayed in bed until after he left having slept not very well for agitating about his late bedtime. Hallelujia!!

Right after the appt. with the therapist we went out for pizza. I was really hurt that Eva had not called him on his hyper-exaggerated sense of victimization - he was really in his teen hormone mind...But he came up right away and said "I'm sorry Mom. I love you" I asked him why didn't you say that up in the therapy room. He said he didn't know - then after awhile,  that he felt pushed into a corner and really scared - I think he means in general. I was hurt still that Eva had not called him on this but in retrospect I believe she had to work with what she had and she did get us around to an agreement to him having this freedom at bedtime which is so precious to him, for living up to his responsibilities which is so necessary to us and him. It worked!! Fingers crossed that it holds!

This has been really hard. He didn't learn this responsibility stuff between 10 and 17 yrs. because of the pain and trauma of the ALPS and diagnosis. He couldn't add anything else with out major tantrums. So he is not mature in this way and he cannot take it from me or his dad at this age now as he is needing space and to separate. But with Eva, he can take it. We are making headway... ahhhhh!  :-)... now it just has to stick! I think it will be a bit bumpy here and there, realistically, but it is definitely going in the right direction.

Cathy

Hi !
I worry about  my son. He is 17 this weekend and there is so much to deal with this grade 12 year all by itself. so much to deal with to grow up and become an adult. And then we add ALPS on top of all of that normal teenage stuff and nothing is normal anymore. ALPS seems to magnify everything, especially because Bryn has been so emotionally traumatized by the process of diagnosis and and the existence of his massive spleen.

Bryn has been working with the therapist my husband and I have seen off and on for the past 23 years. She is wonderful. I am so happy they have hit it off and he is finding her work with him very beneficial. In some ways Bryn is wiser than his years because of all he has been through and all he has learned as a result. In other ways he is behind his peers in maturity because of all he has been through. He doesn't have the energy to work AND go to school., where friends are gaining work experience. He hasn't been able to fully experience his capabilities and actualize them at school because he misses so much school when he gets sick. This year, because he is feeling somewhat better he is having some very positive experiences and doing really well in several classes. This is great! But he needs to have more of this building of positive experience and seeing what he is capable of - he has missed that these past 7 years when he has set his sights on passing and that is all - he manages his time to get 50%! :-/. He is really bright and talented at music and film editing and cinematography. He's a really good writer and his comprehension in reading is above average. He's got so much going for him! He does procrastinate like crazy, and his work habits and organizational skills are horrible. This year he is really getting some great marks and comments from teachers. They have always thought he is bright but now they and he are seeing it happen!

He will  be on his own path to the future - it won't look like his friends. It is hard for him to not be very  stressed sometimes - he wants to be like everyone else, but I don't think he will be ready for College in Sept. He is just taking these first steps to success. Of course there is no one way, one path, the path is wavy, up and down for everyone. The schools make them feel there is one way and it is hard for he and I and his Dad to know what will happen, what life will look like. Can he manage a full-time job? He's going to be better at project based work where he can have some rest time. He wants to work in film so that may work out well. It is just hard to see and hard to know. What are the options, the possibilities? 

Has anyone out there been here themselves or with a youth of there own? How'd it go? How is it going? I hope to hear from some of you about your creative solutions and paths to independence and success.

:-)
Cathy

It hasn't been all bad

Hi,
I want to let you know that as difficult as my very first post makes our journey seem, life hasn't been all bad.

This difficulty has pulled us closer together and we know each other on a very deep level. Our son has gained a lot of wisdom, too young, but still it is wisdom. There has been lots of grief and pain but lots of love and understanding between us, too. We support each other, forgive each other and love each other very much, Brian, Bryn and I.

We have travelled to Spain and Italy, a month in each place, nervous about medical care and extra insurance. On one of the trips, after I had explained Bryn's condition as an enlarged spleen to the person on the other end of the line, our policy came in the mail and said Bryn had "an enlarged sleep". Too funny!

We have had lots of fun and good times, too.  Lots!!

There are so many things to talk about. Today I wanted to make sure you know it has not all been bad, at all, as difficult as it has been.

So much more to talk about, I hope you will share with me, too.

Cathy

A hell of a journey - 7 years with ALPS (autoimmunelypmphoproliferative syndrome)

Hi!
 After 7 years of my son, living with Autoimmunelymphoproliferative syndrome or ALPS, I am tired of being alone, falling through cracks and not sure what is normal for my son given his VERY RARE condition. I am hoping you will share your experiences with me and maybe I will see that we are not so alone and maybe I will come to understand better where we are, what Bryn's normal is, what is real and what is not. Maybe you will find something here for yourself, TOO, in a journey with a youth with an AI condition or another limiting condition. The medical community, while providing a diagnosis after 5 years of searching,  has not been a big help in letting us know about what Bryn is or will facing daily. We fall through cracks all the time medically, in the schools, and HAVE NEVER MET ANYONE WTIH ALPS. So we don't know if he is more tired than "normal", what he will face as he gets older, can he hold a job if he is getting viruses all the time, if he runs out of energy and just needs to rest for awhile - he's ALREADY getting older - he's 17 next weekend!! He tells us he is more tired than his friends and that should be enough for us to know, but I want to know from others what their condition is like, what they feel, how has their experience been. I can't tell he is more tired because he keeps up with his friends and is even more sparky in his personality than they are. BUT he is just coming off 3 weeks sick and away from school - this is his grade 12 year, a really big year. He is often sick once virus season starts. .......And this is a long and complicated story.

When Bryn was 10 he had a long viral illness and was very tired. Eventually we went to the doctor and he found that Bryn's spleen palpated quite large. He sent us to a pediatrician immediately - she found Bryn's spleen even larger - right down into his groin - 21 cms. long! It is the size of a football - instead of a fist. The Pediatrician took some blood, pulled me aside, and said she suspected leukemia, and that the blood test would be done by late evening and we would hear from her by bedtime that night. He would be in Childrens' Hospital the next morning to begin treatment if he tested positive for leukemia.

 I WILL NEVER FORGET STANDING WITH MY YOUNG SON IN THE LAB WAITING FOR HIS TURN, PUTTING ON A BRAVE FACE (I thought), AND BRYN ASKING ME "WHAT'S WRONG MOMMY?" I TOLD HIM VERY BRIGHTLY THAT NOTHING WAS WRONG. AND HE ASKED"THEN WHY DO YOU LOOK LIKE THAT?"

Well, this was the beginning of a 5 year journey to figure out what was wrong and causing a massive spleen. Bryn tested negative for leukemia, thank god, and for 5 years he was followed at our local Childrens' Hospital, tested for everything under the sun - cancers, rare leukemias, liver disease, virus, rare viruses. When they weren't testing to figure it out, he was followed every second week wtih blood tests, x-rays, CT scans, etc. His platelets are low, his hemoglobin, neutrophils, and white blood cells, too. Blood levels went up and down as he caught illnesses but were always low. But other than being sick more often with viruses and having to give up any kind of sport that would threaten a rupture of his now vulnerable spleen (it was no longer protected by the rib cage and much more vulnerable to blows), and missing more school because of regular viral illnesses, he was quite normal. Certainly from outside no one could tell he had an illness of any kind.

Fast forward - 5  years of this. Our son has been seeming to do well outside the home, he is very bright, charming, lively, handsome and social, does well in school, but he is violent at home, volatile as hell, and we are dealing with constant abuse. He says the process of testing is not bothering him but but he HATES HIS SPLEEN AND HE WANTS TO BE NORMAL, sometimes he cries and says "I want to go home" but he cannot explain what he means by home (something we are to hear countless times) and  he is acting out at home constantly - holes in the wall, a broken window, a broken lamp,  things regularly thrown AND violence. We enter family therapy, and he does art therapy, Virginia Satir therapy, to try to deal with his emotions, to fix what we parents are doing wrong, but nothing really helps. When we ask to see a therapist at the hospital we are told "those are for the really sick kids." Well, not knowing what is wrong with you, being poked and prodded all the time and waiting for results of tests with NO BLOODY ANSWERS is a horrible kind of TORTURE unrecognized at our hospital or anywhere really. I don't think even my husband or I knew what we felt or how hard this was, which in retrospect seems crazy since our son was off the wall several times a day. We were alone with no other "sufferers"to bounce off of and no real understanding from the medical community. We received no help the medical system and continued paying large sums of our own money, desperately trying to help our son and ourselves. Meanwhile the neighbors thought we were abusing our son and called Children and Family Services.  This govt. agency came and did a home visit (I can't tell you how rude I was to her when she called!! I was shocked that they wanted to come to our house). They spoke with me, with our son, with my husband on separate days, and gave us a clean and even admiring bill of health for attempting to deal with our problems. No we are not perfect, yes, we have problems and are having a difficult time, but we are dealing with a very difficult situation with all the therapists for our son and ourselves that we can muster (she said, unlike many people who don't deal with their problems at all). I still can't understand how a neighbor could have done that to us - though I do know it was very loud and horrible sounding from inside our home. I was nauseous for 2 weeks after each visit of this very kind govt. worker, terrified they would take our son.

Well, 5 years after the first leukemia test our hematologist calls and says she thinks she knows what he has. She wants to do some blood tests and send Bryn to the National Institute of Health, NIH, in Bethesda, Maryland where they have a study on ALPS. The NIH will pay for Bryns' and a parent's plane fare and we can stay for free in the Chidlrens' INN on the campus of the NIH. Our doc eagerly and with our thanks, pulls together a lot of reports, data, files, tests and bloods, to send to the NIH and off we go.

Bryn and I went without his Dad which seemed a big deal at the time. Bryn had 18 viles of blood taken and  genetic testing with his genes read, and after a month wait we have a diagnosis - he has ALPS. He has had a genetic mutation on a single cell in his immune system - the double-negative T-cell. We have a page showing how the gene is written correctly for awhile and then the spelling on it goes haywire - ON ONE BLOODY CELL - and it has changed his life. His body no longer recognizes that cell or gets rid of it when it is no longer needed after an illness - it lodges in his spleen, creating this massive spleen. There are approx. 200 known cases of ALPS in the world. 190 of those are inherited from parents with ALPS who have not been diagnosed or have remained asymptomatic. There are only 10 like my son, who have had a spontaneous mutation during his growth in the womb, in MY womb. Is this my fault? I don't often think that but as I write I wonder if I think it more than I know.... more later on that....

So now my son is, as he says himself, 1/700,000,000 in the world. I am just happy he is going to live, not have a degenerating illness. But Bryn is still mad and upset, he wants to be like everyone else and he hates his spleen. His is a mild case, the most mild case of the NIH ALPS team - lucky right? Some kids are very very ill. He's lucky right? Not to Bryn. He wants to be "normal", he hates his spleen and the volatility continues - only now he is 15 and bigger and it is really tough.

I'm going to leave it there for today. It's a long story. Bryn is doing therapy again the last few months and the volatility has stopped - he is finally old enough to begin to deal with the trauma of what he has been through. I'll tell more about that another day. I am afraid for his future, of the unknown, for his schooling, which is going much better this year because he is feeling better, of what he will do, what can he do, after Gr. 12 with his gifts especially a passion for cinematography and film editing. I don't know whether anyone will comment or be interested or share their story. I hope so. I don't want to be alone with this any more. I don't want to keep the secret of our family difficulties that have arisen from this anymore. ALPS is rare, it is invisible in Bryn's case, we are tired of being in the cracks in the medical system, of trying to figure this out alone with no one to bounce off, no landmarks in sight, making all new landmarks and not even knowing sometimes whether we are in the right place or any place, and of no one else knowing or understanding. I would like to compare stories, to see how and what others are doing, parents of youth, youth themselves, people who were youth once in similar situations.To know we are not so bloody different and alone. Maybe we can help each other figure out the unknowns. Maybe we can support each other. Please share your story with me. From here on I am going to post mostly daily - thought, worries, situations, wonderings as they arise. Bryn is on the cusp of the rest of his life and there are so many questions, so much to think about and not very many answers. Maybe we can share answers or hammier them out together. If nothing else, maybe we can listen to each other.


From my heart to yours,
Cathy