How do you know when it is end stage IPF?

My husband was just diagnosed a month ago, but was told he has had IPF for a long time and has been misdiagnosed for quite some time. In the past week he has had to increase his resting oxygen to 4 1/2 -5 liters and he uses 6 when he is up moving around but still gets quite winded and often s to stop and catch his breath while walking just a short distance. The past 3 days he has had severe pain in his joints and muscles and has had recurring problems with constipation, severe fatigue and chills. when I check his pulse ox resting today it was 95 percent. His color does not look good to me, but there are so many issues with this awful disease I don't know if he is having bad days or if this a progression to something worse.

I know a pulse ox of 95 is good but am concerned about all the other symptoms that are getting worse. His chest and back pains seem to get worse each day. I am going to talk to his dr about pain meds to make him more comfortable. And does anyone know if hospice is most always needed at the end? Many situations I have read about this seems to be the situation. This such a scary disease and the fear of the unknown is even worse. Thanks in advance for all your help

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My heartfelt prayers go out to you, gapeach1. While my diagnosis (dx) is PF, it is due to my scleroderma lung issues and the methotrexate my rheumy-doc prescribed. I have experienced the passing of many SD/PF patients and I do not recall those other symptoms expecially when your husband's O2 levels are so high.
Please remember that we are not immune to other sicknesses on planet earth especially summer colds or the stomach flu which almost sounds like what your husband is experiencing. But I am not a doctor or even in the medical field. I just know that by having scleroderma for 13 years and PF for 3 that those 2 chronic illnesses cannot be blamed for everything.
You are doing the right thing by putting a call into his doctor. But the question is, which doctor? I would certainly call the sub-specialty doc that knows him well and you both consider to be his "team coach".
It is extremely paramount for all patients who have a chronic illness to have 1 coach who knows you well, easy to communicate with, doesn't rush you and answers all your questions/concerns over a doctor who is thinking about his next patient while looking at you. Remember, he's working for you and isn't suffering from the illness. You are. Having said that, it doesn't mean you can go into his office or exam room with an "attitude". You need him. So keep your tone, attitude, language in check. Doctors can "fire" patients and it's happening more and more. Another thing, if the office staff/nurses are cordial I would work on cultivating your relationship with them too. To me, it is the staff who can work the miracles when it comes to the doctor. They work with him/er everyday and know what he'll respond to. If a patient acts like a "pill" then your message or question can get put on the bottom of the stack or marked with a lesser priority by the very people you see before walking in the exam room. If you have questions, "ask" not "demand" the answers. You can ask in a manner to which the doc is willing to take a little bit longer to make you feel comfortable. It's a fine art of "balance", don't be a wimpy victim but don't be so angry he'll think you need a psych exam or threaten to call Security. Don't go there....

As for hospice, in my humble opinion (IMHO) once a patient is admitted into Hospice care it is basically to make them feel comfortable and pain free. So meds will continue to be administered until the end. However, a patient can "graduate" from hospice, if you will. There are numerous cases where the patient rallied back and end up leaving Hospice. For more specifics,
I would call your local hospice and/or the hospital social worker for a detailed time line process of the last steps.
This is also a good time to talk about advance directives, end of life decisions, DNR (do not resuscitate), etc. I urge you to do it while your husband is of sound mind and can verbalize. I look at it as part of the circle of life and regardless of whether a person is sick or not, it's so much easier to have this talk and set up arrangements way ahead of the time. That way, you know for sure what your loved one/patient wants.

I apologize for the novelette, I get to typing and don't look up until I get it all out. The main thing is, figure out w/ your husband how you both can live "well" with IPF even though you have to prepare for the inevitable. The mind is a very strong part of the body and use this time to learn all you can about IPF, make amends with whomever, plant positive, uplifting memories and take the time to really observe all that is around you. It's a beautiful world out there. Attitude is everything. I wish you well.

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CJ, thanks so much for your reply. I have been in the medical field for many years and yes I agree you get a lot further with the doctors if you are not too demanding and make an allie (sp) of the staff. We had to go out of state to get a definitive diagnosis because his lung dr said his symptoms were not his sarcoidodis which he has had since 1993, and he was right, but was his heart and the cardiologists said it was not his heart but his lungs and we went back and forth like this for 14 months with 10 hospitalizations and nobody could find anything definitive and day by day my husband was getting sicker and sicker.

I don't think he has a virus or a cold as he has not been running any fever. I have an early morning appointment for him on Wednesday and hopefully the pulmomologist will be able to answer some of these questions. Just never thought this is how we would be spending our days of retirement, him suffering and me eventually planning a life without him. He is only 60 and I am 55.
It is so difficult to stay positive when I am so angry, sad, and confused. I never let him see these feelings I keep them to myself and only show him a positive front. He never sees me crying in the pillow at night when he is sleeping or knows that I stay awake for hours at night just listening to him breathe because I am afraid to go to sleep because I am afraid he may stop breathing. I don't really have anyone to talk to about my feelings so that makes this so much harder. sorry to ramble, just having an especially emotional night, and sleep escapes me once again as I listen to his shallow breathing. Hang in there yourself and I pray that your health stays stable for many years to come.

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You don't know!

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gapeach1 - First of all, I'd give you a big, warm hug if I could see and chat with you in person. Since that is not possible, picture my cyber-hug coming your way! You sound very lonely and I want you to know that you don't have to walk this journey alone. Surely there is a PF support group located near you or perhaps other PF patients' spouses that you see in the doctor's waiting room?
A burden such as this is much better carried with others who understand your feelings since they too are walking that same journey and most likely are experiencing the same sleepless nights as you!

Believe it or not, your husband does know your sadness, anger, helplessness, etc. He's not saying anything because this is a problem that he cannot "fix". Men are geared that way! In addition, he's probably going through the exact same emotions that you are experiencing. You're both on the same team and try to be each others' cheerleader!

Do your best not to "focus" on the illness but focus on your husband. This is the season in which your marriage vows really kick in. Especially the "for better or for worse" part. Ask for help in doing the things he used to do around the house. Perhaps a friend of his would drop by for a visit and skillfully ask if he can help with "such and such" and before hubby can reply "no", you can interrupt with "Why thank you, Joe. How nice of you to assist!"

Surround yourself with non-toxic friends but not all at once!! LOL Play it by ear but for right now, you (the caregiver) need support too. There is a website with wonderful information for caregivers: http://caregiver.com/ Please check it out!

I wish you the best for today. And do remember to take it "one day at a time" that's all any of us can do! Don't miss the present because you're thinking about the future or looking at the "woulda, coulda, shoulda" of the past. He needs you right at this very moment and is grateful that you are his life's partner.

Hope this helps. Be blessed today!

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To coldwatercreek - not sure what you mean by "you don't know" and if it's directed to me and/or gapeach1.

FYI, Team Inspire's primarily focus is that of "inspiration" and many share their trials/tribulations plus advice based on their personal journey.

IMHO (in my humble opinion) "You don't know" is like lukewarm water.

Be blessed today!

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My comment was not directed at you. You made a great point about focusing on the husband and not the illness. Non of us really know until we get there what the last stage is. For some the last stage is almost at the same time as the dx. Others like myself have had a long haul with PF (11 years).

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Gapeach: your questions are:
1. And does anyone know if hospice is most always needed at the end?

2. How do you know when it's the end stage IPF.

Only a biopsy can tell you in what stage the lungs are. Hospice is almost not needed at the end.

If he is getting worse, the best for you is looking for a transplant for him. Always there is hope.

Don't think in the end; think in a new beginning for him.

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There are pleanty of PF patients on other sites using hospice. PF is a very PERSONAL disease. No two patients are the same, so there are No pat answers.

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Of course coldwatercreek you always are right, and with a lot of time to jump from site to site. lol

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I have the time because I am bed ridden with this disease. I don't even pretend to be right. You are the one giving the advice.

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I find with my o2 if I am on 2 to resting then I need to go to 4 and 5 while walking.
you said he is at 4 to 5 lpm resting.
I am not a doctor but I would think he would need 8 to 10 LPM while walking ? not 6
In-fat sometimes I don't need any o2 at rest and when I start walking I start at 2 and I need go to 4 lpm in 2 min.

AS FOR HOSPICE
I think that is something only your Husband can answer and you should ask him when the time is right.

For me, at the end I would want Hospice, my angel of a wife who has God's Private cell phone #. I really believe that in my heart.
My mom and dad and sister.

How ever, The last time I was in the hospital with chest pains in the emergence ward, DAD stood at my bed and said if I lived better meaning food and not smoking, I would not be here and had to have him thrown out.
His timing is not the greatest........lol

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Has your husband been checked for hypercapnia? The pulse oximeter cannot distinguish between oxygen and carbon dioxide in the blood. The latter can rise to dangerous levels with lung disease while the pulse oximeter gives you to think his oxygen is great. Your husband may need to be hospitalized for this. My heart goes out to you!

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We are all on Inspire to support and share with each other as a family and team. I have seen some get short with others and I feel it is because of the Nasty, Ugly disease that is robbing our bodies and shortening our lives. It's tough not to be angry but lets also know that we are all in this struggle together. We struggle to breathe, We fight to live. I pray for all of us and hope that they will find a cure for this pulmonary fibrosis and pulmonary hypertention someday. Maybe not in our time, but miracles do happen and God answers prayers. I hope I have not offended anyone and I apologize if I did in any way. God bless you all.

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You know when it's all said and done, whether you're walking around with O2 POC, look great with impaired insides (an invisible illness) and/or bedridden due to the progression of the disease---every day you (the patient) simple wants to be acknowledged, affirmed, feel valued and loved. Don't all human beings? Sure the meds help you get through but it's the other stuff (listed above) that costs nothing and yet it "fills your cup" each day to where you (the patient) can make it through to see another day.

I do like to be equipped with knowledge of scleroderma, PF and all the other stuff that comes with a this illness. The difference is, I don't let information control my life. Like PF, no 2 patients are alike and since this has no known cause, it has no known cure. The extra caveat is the lung transplant decision is scrutinized even more due to our GERD problems. Who wants to grant a transplant when the patient will aspirate and ruin it? Makes better sense to give it to someone whose life will be saved and can live longer because of it. If said transplant is granted to a sclero pt, chances are you will be eating through a feeding tube for the rest of your life. Again this depends on the patient, it's a complicated illness.

So for today (and each day) since God granted it and I opened my eyes to find I'm still breathing....I thank Him and go about my daily routine noticing all the wonderful things, take care of my needs or allow my caregiver to assist, keep my attitude in check and be grateful for everything around me. I say thank you alot, show my appreciation for any concern and say "I love you" several times a day.

We don't know when the time will come but you know what? I don't want to leave this earth without the people in my life (or have touched my life) knowing how much they meant to me and how their very existence contributed to who I am and what
I may have accomplished while on planet earth. My plan is to die graciously not angry. It is what it is.

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Knowing when the end is near, is a very difficult thing. As the lungs decline, and your oxygen needs increase, these may be indicators that time may be running out. Is your husband a transplant candidate??

My sister had PF for 10 yrs., went on oxygen in Oct., (2 L), then increased to 4L, and eventually 8L with exertion. She was denied for her transplant, due to other health issues, in March. When I saw her at Easter, she looked good, but seemed sad, and resigned. We discussed hospice, and I told her that she would know when it was time to call them. When talking about going to our family reunion in June, she stated that she "wasn't going to make it until June". She started hospice on April 10th, went to inpatient on the 11th, and passed on the 17th. None of this is within our control, except how we live, manage, and cope. It is a very difficult, heartbreaking, sad, and emotional journey. My mother, and brother, also died from PF, and I am now in my third year. So many unknowns, variables, and gray areas. Try not to drive yourself crazy, make the best of the time you have, do the things you, and your husband like, make memories, love each other, and enjoy whatever you are able.

Currently, I am not on oxygen, and feeling pretty good, so I am trying to follow my own advice. It is difficult, as I am pretty much in this alone. No more primary family members, few distant cousins, adult children busy with their lives, adult grandchildren, also busy with their lives, and no partner (except my 18 yr. old dog). Spend a lot of time thinking about what is to come, but do go out with friends dancing, exercising, movies, etc.

God bless each of you, and give you strength.

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Today we had a dr visit with my husband's regular pulmonologist. First appt since we returned from ohio and he received his awful and devastating diagnosis. Did not get good news here either. He said he had discussed my husbands previous ct studies from previous years to the current one and discussed with specialist in ohio and feels like he has had IPF since at least 2007 and at this point is in advanced stage and most likely in end stages. Would not give us a time period but gave us information on advance directive, gave him pain meds, and told us to enjoy the time we have left together and be sure to call them if we have an questions that alarm us. My husband is taking this very well, but I am a mess. How do I get past the illness and worry and just enjoy being with him while I can? I am so scared.

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Your fears, and feelings, are well founded, and certainly to be expected. Are you sure your husband is "taking it well", or just "being strong, like a man is supposed to be". Try sharing your feelings, and fears, support each other, as you are in this together. Make sure finances, wishes, etc. are in order. Think hard about the living will, and advance directives, and make sure he has a will. All of these documents, and putting things on paper, can make the aftermath easier, as well as ensure that his wishes are honored.

At no time in life have we ever known when our time would be up. In some ways having a time line gives us the opportunity to plan, and do things which we might have put off.

Hospice care can be a great support for your husband, as well as yourself. They will provide physical, emotional, and bereavement care. It is not giving up hope, but rather living well, and comfortable until the end.

While I am not married, my recent experience was with my sister. She had IPF for ten years, and went into hospice in April. Although I knew very well that the day would come, I kept thinking we would have more time together. It was still a shock. I remained at her bedside, day, and night, for six days, and it was very difficult, and emotional. We were very different, and often did not see eye to eye, but we were sisters, and the bond was deep. I am left with many good memories, and am still working through the sadness.

There are no rules, no easy answers, and this is an individual journey for each of us. I pray that each of you find a level of comfort, and don't be afraid to cry, hug, talk, share, and do whatever it takes to reach a place of peace. Good luck, and God bless!!

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GREAT REPLY!

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I am fairly new to this group, so I don't have a lot of info to share, but I just wanted to give you a hug, and just want to tell you I am thinking of you both and will keep you in prayer.

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I am fairly new to this group, so I don't have a lot of info to share, but I just wanted to give you a hug, and just want to tell you I am thinking of you both and will keep you in prayer.

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