I've been diagnosed with Alpha-1. Anti trypsim deficiency which has lead to me having Emphysema/COPD Gold stage 4 MRC breathless score 4 my FEV1 is 0.48, 20% predicted. I was a smoker but I've now stopped 8 months ago. My consultant was going to put me forward for a lung transplant but is now saying that because of my Osteoporosis he doesn't think that I would be a suitable candidate for this procedure. So he's now sending me to be assessed by a professor with regard for suitability for endobronchial valves or lung reduction surgery. He's also told me that if I were to get a chest infection it would probably kill me.
My GP has advised me to sign a DNR as resuscitation is brutal and he doesn't think I would survive it anyway.
I'm angry because my condition was treated as asthma for my entire life from age of 5 onwards. I always had problems with chest infections, Bronchitis, etc....I feel that my GP should of sent me for further investigations as I got older.... I just feel that I've been let down by my GP.
I'm so depressed. More importantly, I don't want to die and I've told my GP that I don't want to die. I won't sign the DNR.
I'm also wanting my consultant that I at least want to be assessed for a lung transplant.
Written by
Diddy2017
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You sound thoroughly miserable, and I don’t blame you. You have been treated appallingly. It is too easy for doctors to make quick assumptions about lung disease and plump for the commonest options. Your Emphysema should have been picked up years ago. But you have come to the right place. There are others here with A1ATD who will give you loads of advice. The diagnosis is a shock, but you will adjust to it. Your consultant sounds good.
We have to be proactive regarding our own health. We have had to do all of our own research on alpha one. All of the Drs are reluctant to go into depth. My husband has had to get aggressive and tell his Drs I want you to tell me as best you know my life expectancy. My status as to the progression of my lung damage. My husband had formed a close relationship with one of his Drs and he asked him, if it were you would you pursue a lung transplant. This one dr. Told my husband I personally would not go under the knife if I were you. My husband thanked him for his honesty. Due to the several complicating illnesses my husband has, a lung transplant would most likely fail. Doubtful he would even be considered a candidate. Some people may not be at a point in their diagnosis to desire such blunt info, however we have been dealing with the shocking news over ten yrs. we want truth and facts as close as can be guessed.
Hi Diddy so sorry for what you are going through I hope you can be assessed for lung transplant or valve or LVRS whatever is going to help you most. You don't mention your age, having the osteoporosis can happen at a younger age and it would depend on the severeity of the bone thinning I guess and how well you are able to cope with the side affects of the drugs and recovery process from the op., taking into account the drugs you are taking already to help keep lung infections as bay.
Have you been referred to Bromptom Hospital in London? they are the specialist hospital for heart and lungs. If so I hope the assessments go well for you there.
Has any one mentioned that other members in your family should be check for AIAD? Check with BLF helpline for further information on this or the Alpha1 website:
Stopping smoking was a great achievement and the best thing you could do.
For sure you need to protect your lungs the best you can from any infection, and avoid others at all costs who may be infected with a cold or flu virus, hope you've had your annual flu shot and the pneumonia vaccine already.
Do chat with BLF helpline for more information and support.
Very best wishes for your assessments and op.
Take good care, keep in touch and feel supported here.
L&H Bkin
My hubby has alpha one geno type ZZ. He was diagnosed at age 47 ten yrs ago. At diagnosis Fev1 was 42%. Now it is 25% stage 4 at last PFT. He is just getting over a chest infection. He took zpak and prednisone. He has a constant supply at home. Has been on a 3x a week antibiotic regimen for a couple yrs now. He still works as he has a non physical job. His sats are ok so he’s not on oxygen. His pulmonologist asks all the time about transplant list. He says it’s his job too. But my husband has osteoporosis due to arthritis. The two illnesses aren’t compatible with each other medication wise. The anti rejection meds leach calcium out of bones. We are in the USA and the cost of going through the tests to see if he would even be accepted for a transplant is at least 125k. I’ve researched his illnesses many many hours over these past ten yrs. I know he would not be considered a candidate. We both know that he would be trading one set of problems for another. Facing possible rejection etc....transplant isn’t a walk in the park. My hubby can’t walk fast or far but he does walk daily. Alpha one reacts differently to everyone. Many have died that weren’t as advanced as my husband is. When he was first diagnosed they gave him 18 months. Now it’s been over ten yrs. There is no accurate way of knowing how long he has left. It’s just guessing. I don’t think he can work much longer though. It’s getting to that point. We’ve had ten yrs to deal and talk about this illness. Been married 33 yrs. went through all the emotions many times over. It is our situation. We have filled out a POLST form giving all the Drs instructions as to end of life care. He did not sign a DNR at this point. He has signed not to have mechanical ventilation and only non invasive methods. This was carefully thought out due to research. We read about having a good death or a bad death. Not trying to sound morbid just factual. Drs do methods so as to not lose a patient on their watch, however this is not always in the best interests of a terminal patient. My husband wants a good death calm at home! In the meantime he does everything he can to stay healthy! I will take care of him and we will both focus on keeping him as comfortable as possible. We have 8 grown children and have discussed this as a family . We are all on the same page.
Hi, I am sorry to hear your news. I am in a very similar situation as you and have been told by the Brompton that I cannot have either lung reduction or valves due to arthritis causing a curve in my spine (never even knew). To say I was shocked is an understatement the dr's feedback seemed so positive all along. Have to wait for January appointment, guess at least they haven't discharged me - yet ! Anyway hope you find a solution,keep in touch
I am so sorry to hear about what's happened to you. Sadly with Alpha 1 so very many sufferers are misdiagnosed for years with asthma while the underlying cause is missed. If you would like to be in touch with others who have similar experiences to you and who may have had lung transplant/evaluations and valve procedures there are Alpha 1 support groups online - both webpages and facebook groups. The uk ones are Alpha 1 Support group uk and Alpha 1 Awareness uk. They can advise on Alpha 1 clinics where they have the expertise to manage your condition. All the very best. Z
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