Need your advice please

[chynz]

hello everyone, i have been a member for some time but havn't posted in some time. I do have a question or a dilemma depending on your view so here it is.. Ive recently visited a few hemotologist a couple months ago and the conclusion was not what i was expecting. i was hoping for a regimented transfusion because my hemoglobin has been dropping slowly for the past few years. i was hovering around mid 10's, then high 9's, and now been bouncing around a high 7 to mid 8's. but my fatigue seems to feel worse than the RBC's indicate and the bone pain or fatigue pain is worse too. Being tired is not so bad it's the weakness in my whole body, particularly my legs that really bother me because theres an overwhelming feeling to need to sit or lay down. Also, theres been some heart palpatations or arrythmias going on that really kicks my butt. All this has been discussed with my doctors and his prognosis is that my condition is spiraling fast and that i need to have a BMT. This was a huge surprise to me and to be honest it felt good that somebody said something unexpected and compassionate. For once I didnt feel like i was a fraud trying to convince drs. that what i was really feeling was legit. Finally, this hemotologist took the time to study my labs and diagnosed me as having the more severe type of alpha thal H disease known as the Constant Spring variant. However, he expected my iron would be in the 1,000's but to his and my surprise it was 225. He believes that my history of bleeding ulcerative cholitis and dark urine is the culprit for excreating my ferritin levels. So depite all that, i saw a transplant doctor and his opinion is that transplanting at my age(31) is too risky and advises against it. However, he wants to see me in two weeks to go over hla typing with my two siblings to see if there is a good match. Blood typing is 1,500$ US each so 3,000$ in all, which, is very expensive if he's not even going to approve the procedure. I also told him that i have four cord bloods from each of my nephews in storage for this occasion but he said they were not viable for some reason i didnt understand. i thought cord blood was the best option and every article i found on-line states the same. My question is should i go through the blood typing and hope a good match will change his mind? And has anybody known of anyone who used cord blood at my age and was it successful? I know the risks of GVHD and the chemo and all the other good stuff, but to be honest, the past 12 years of my life is a repetitive hell with no quality of life, and getting worse. Should i fight for this transplant and even see another doctor if this one wont budge? This disease is cruel in that i dont really fit the criteria of thal major yet serious enough that im having hemolytic crisis's every so often which required blood transfusions. I would really appreciate it if you had any imput and help me decide my fate.. Thanks Ryan H.

[marisa_c]

Hi Ryan, if I was you I'd be seeking as much advice as I can, and getting opinions from as many doctors. If your quality of life is suffering and want it to improve, do as much as you can to get it back! :)
I'm not sure about cord blood transplants sorry.
I hope you get some answers.
Keep us updated on how you get on.
Marisa

[chynz]

Thanks Marisa C, I appreciate the reply and hope you are doing well. As for the transplant I think I'll forego the procedure and hope I can get on a transfusion regiment bc my iron is so so low and it would be less of a pain and burden for everyone. It's just hard to accept that doctors differ alot in their differientials and it's the patient that has to fight for what he or she needs. One doc fears the risk while the other believes in the benefits in quality of life. I really don't know what I want, just not to feel this way anymore no matter the means..

[aki:)]

Hey Chynz,

I can relate to the weakness, body aches and the palpatations you've mentioned here. I am a B Thal Maj and try to maintain my Hb above 9 pre transfusion. But I do feel these symptoms when my BT is due and I have had a long hard day at work.
But even I think that you should consult as many doctors as possible. There's so much of development happening everywhere and innovative treatment is what everyone is talking about.
But in the meanwhile, try to make sure you sleep for at least 8hrs a day and consume a healthy and a high calorie diet. It will help you fight the fatigue.

Take care
Akhil

[chynz]

thank you Aki for your imput and experience. The one thing that is hard to gauge around here is pre transfusion status and criteria. May I ask you, I know you said your hemo is 9 pre trans and prob goes up to 10 or 11 hopefully. What would happen to yor body or hemo if you don't get transfused? Would it go down slowly and to what number do you think? What symptoms do you begin to exhibit like pain, weakness, mood swings, or even speech problems? I know there are differences in alphas and betas but can't quite seem to differentiate they on a clinical or physiological level. I've been tested couple times and my hemo was 7.4 gml even when I was feelin quite well. But when I feel really weak and tired it's hard to get to a dr. To test my blood in time before I know it will bounce back up to mid 8's or 9. Do you think it's possible that hemo levels can drop sporadically due to oxident stress and then back up when I make it to a dr.? Each time I try the doc says my hemo is back to the steady 8's and therfore the criteria for transfusion is just over 7, of course when I get a chance to be tested it's early in the morning and I'm quite rested and feeling like an 8 or 9. So the system here in my area is flawed bc the few times I have been transfused I happened to keep a low enough hemo for the process to begin. Do you have that prob and or is it just understood that you need transfusion bc you have thal major? I'd really appreciate the feedback. Thanks Ryan

[aki:)]

Ryan,

Well whenever you get transfused, the rise in your Hb is dependent on several factors such as:
1. Your own body weight (as Hb is a relative value)
2. The donor's Hb
3. The freshness of the blood
4. Any antibodies present in the donor's blood that may affect you, etc.

The medical standards are different here than what you mentioned. I have seen Thal Carriers who are refused transfusion even when their Hb falls to as low as 5-6. The doctors here are very conservative when it comes to transfusing blood. In fact, I know a lady here whose Hb was around 3, for over 2 yrs and who didn't even feel a thing.
But then yes, it is observed that for Thal Majors, the pre transfusion Hb ought to be above 8.5. And considering you are transfused with 15-18ml/kg body weight of 60-65% PCVs, your Hb will rise by around 3.

The first sign of low Hb (around 9) for me is the difficulty and stress in the legs while climbing the staircase followed by sleepiness and restlessness. You can even tell your Hb looking at your finger-nails and the paleness in your eyes. Symptons of really low Hb are breathlessness, lack of will to talk, etc.

FYI, if one doesn't get transfused, his Hb will fall and continue to fall to a point where the body cant take it anymore which would eventually be fatal.

Best Regards

[kermit]

Chynz, Please contact the Cooley's Anemia Foundation at 1-800-522-7222 ask for Eileen the Patient Service coordinator.

You need to get on a transfusion schedule and chelation. You also need to visit a Thal Center for your annual comprehensive care - ie: echo cardiogram, ekg, T2*, bone density etc: these centers will work with your hematologist to coordinate your care. It sounds like your doctor needs some guidance.

A BMT is quite serious, especially in an adult. As others have stated start a transfusion regimen you will feel better. And remember ask questions, be pro active in your health, knowledge is power.
If you want PM me.

[chynz]

thanks for your replies Kermit and Akhil. I actually have seen my hematologist and a few weeks ago and disscussed my options. He is addiment about getting a BMT to cure my disease because my file for the past 12-15 years has shown that I've been progressively getting worse and that nobody had been anything but reluctant to treat the root of my disease. However, a BMT is a big commitment that you am willing to explore however it's a burden I wouldn't want to put on anyone in my family and friends because of hard times and I need to work to help support my families business. So that being said, I opted to take the transfusion route to see hiw my symptoms respond. It's been two week since I was given 2 units of blood and to my surprise my levels have raised from 8.2 to 10.7 hb. I have people telling me I have more color in my face and my eyes are less jaundiced for once, a benefit well worth it alone. I didn't notice a sudden change in energy, infact the first three days I was more relaxed and could finally fall asleep without any sleep aides. About a week later I began to notice the blood kick in and now I'm not stronger yet but am less tired and do have more stamina in my legs and mentally in a better mood. I wonder if this is what others feel too? Anyways, my dr. Said it should take a few more regiments to get the full effects of transfusions and hopefully back to my 'normal' self. It's been so long I don't know if I'd recognise that person, lol. Anyways, I feel better and my heart is racing less so I think my body is not workin harder to commpensate. My only question is is now how long will this feelin last before I need another and does anyone here believe I'll feel more energetic with a course of traetments as opposed to how I feel now? The only downside is that I have noticed a bit more joint pains and mid-day drowsiness, plus the sudden urge to smoke cigarettes, which I never really was into.. Thank you all who have posted here and gave advice as it has helped. Any experiences you have gone through would be nice if you could share so I could get a baseline of what I'm going through and what to expect. I see my dr. In 6 weeks for a follow-up to explore this new option to see if my body can handle it or if it compromises my future chances for the ulitimate goal of a BMT. Thanks again and hope you all are well...Chynz