End of the Road - 8 weeks from Diagnosis to No Hope

[Neil Cuadra]

Chirley,

It might be worth checking up on any doctor you consider, using the Medical Board of Australia website, to help you avoid doctors that are likely to skip town!

Here in California we can refer to the Medical Board of California website to check a doctor's education, certifications, and disciplinary history.

[Chirley]

I did that Neil. We also have a site called AHPRA which tells us where they Registered, how long they have been Registered and any conditions they have had on their right to Practice. This last Doctor was a Specialist in General Practice..he actually did extra exams in General Practice, he had been doing it for 16 years, had never had a complaint. He had been head of a large NHS Practice in England before he emigrated to Australia. I grilled him thoroughly on my first visit that he had all the qualities that I wanted because I needed someone to be my advocate. He was so good my parents changed GPs to be with him. He went the extra mile. I'm completely confused.

He was a devout Muslim who even donated his time to provide free medical care to small villages in Yemen where he his life was in danger on many occasions. I will miss him. He was a pacifist who abhorred any type of violence.

[Alcof]

Glaxosmithkline have approved my Dad the supply of Eltrombopag. They have responded to a rather emotive letter I wrote - and working with my Dad's doctor, have given approval to supply my Dad with this little bit of hope in a tablet. Something we could never have afforded ourselves and is currently not subsidised in NZ.

Hurrah!

Now don't worry - we are totally realistic of where our hopes must be with regards to a) staying infection free with Zero Neutrophils long enough for the drug to have a chance to work and b) the effectiveness of the drug % wise.
But for those who have been following my posts will know that two weeks ago we had no further options. Now we have possibilities.

I am now going to put all my Dad's info and the eltrombopag dosage that he is on in an email to NIH to see if they can approve the dosage and how compatible it is with Danazol and Cyclosporine which it is intended he will continue to take.

I have learnt on this road, that it pays to be knowledgeable and the more heads working on a problem the better. My Dad's doctors are good. I am happy with what they are doing. Even if I have had to push and ask all the questions and make all the suggestions. Just because we put our Dad in a hospital, doesn't mean he stops being ours.

So keep up the good work, marrowforums. Thanks so much for being there to help me advocate for my Dad. Today we are in a much more hopeful place.

[Alcof]

Originally Posted by Chirley

Alcof, I'm so pleased to hear everything is coming together and that the Doctor is filling out the forms. I'm just a bit disappointed that you had to start the ball rolling rather than the Doctor initiating this himself. Thank goodness you are a very good advocate for your Dad. But what about other patients of this Doctor who don't have advocates such as yourself? Do you ever wonder why sometimes they just don't mention these options themselves and you have to do the work? I was in that position a lot for myself and now for my mother and father...it's frustrating isn't it?

Yes, Chirley I agree. In an ideal world we could just relax and just provide the moral support. It is so exhausting, constantly being on top of what is happening - and even knowing what to ask for.

I guess the way I've been rationalising it is that in NZ with rare diseases there is so little experience and knowledge - and also nearly all medical treatment is government funded. Public health in New Zealand is by and large very good. But it is a largely a thankless task that I do not think I would have the patience nor inclination to do.

But yes I do agree. it would be lovely if everyone that got diagnosed with a scary disease had a loving advocate with the time and energy to put into researching everything for them and standing by their side the whole time. I used to be an advocate carer for intellectually disabled people and that is what we were taught. Be an advocate for the person. I guess it is forgotten sometimes in the medical world that sick people need advocates too. That they sometimes don't have the strength, inclination or will power it takes to fight their own corner and ask WHY all the time.

[Chirley]

I was in a position a couple of years ago when I was too sick to voice my wishes and no one advocated for me and I almost ended up in an aged care facility at the age of 55. Luckily I recovered enough function to state my wish for rehab even though I was told it was futile.

I,deep down, can never forgive my friends and family for not advocating for me. It is a hurt that won't ever go away. That is why I am such a vocal advocate for the rights of patients now. For the need for Doctors to be aware that patients have rights and feelings and don't have to agree with everything the Doctor says. I advocate for my elderly parents to the point where I am considered a thorn in the side of the health services but I don't care as long as they get the best treatment available.....I'm damned if I will let their care be compromised because I know how absolutely vulnerable you are when you can't speak for yourself.

[Marlene]

It may be worthwhile to have a consult set up with your doctor and NIH. I can't think of a good reason why your doc would oppose one unless his ego won't let him.

I agree that it's a good idea to make sure the combination you have him on is appropriate as well as the dosages.

Good luck.

[Alcof]

Today is not a good day. My Dad has really taken a turn for the worse. He has burning fever, his head is covered in a red large lump rash, he has large ulcers through his mouth and his face is swollen. His tongue and gums are so swollen that he can hardly talk and is struggling to eat at all. He is really struggling.

The doctors are quite at a loss. They are looking for infection. They are growing cultures off everything we can find. They have done MRI, ECG and all sorts and cannot find the source of infection. It will take a few days for the cultures to grow anything. In the meantime Dad seems to be declining. He's trying so hard and we can't do anything.

Goes to show that with Neutrophils of Zero its just so verydifficult to stay infection free.

I'm now going through the process of considering all his medications and what might be causing such a reaction in case its not an infection...

[Marlene]

So sorry to hear this. Is he still on cyclosporine? May be worth considering stopping it for now since it suppresses the immune system. And are they treating the infection? If he's on antibiotics, then the cultures may not show much. I assume they did a blood culture also. Good that you are also looking into possible drug allergy/interactions.

[Hopeful]

I am also sorry to hear this. My thoughts are with you and your dad.

[Cheryl C]

I'm also very sorry to hear this news about your dad and hope a solution can be found quickly.

In the mean time, I don't know if you're aware of the wonderful NZ product Medihoney. I find it very soothing to swish a spoonful around in my mouth if I have ulcers.

See http://www.express.co.uk/life-style/...-survive-chemo

[aussie danielle]

Just a little hello from across the Tasman,i was sad to read your post .I dont know what to say to help, other than to say i am thinking of you,and I hope things improve for you and yours .

Life can deal us some hard knocks at times ..cant it??my prayers are with you all.
I recently had a bone marrow done to see if i would qualify to take Revlimid in Australia,which could have, hopefully, seen my red cell count improve..and make me transfusion independant. BUT the test came back negative...i was very dissapointed ... simply an another curved ball to handle....

Keep your chin up and know that this is a very helpfull forum and there are lots of information and very kind people who may be able to help you....aussie danielle

[Alcof]

Thank you so much for all the support. Its so easy to get discouraged when I see my Dad trying to be brave when he is in so much discomfort. We are not out of trouble yet, but Dad is definitely improving.

They have isolated an infection in his mouth and a targeted treatment is starting to work. He still has difficulty eating and swallowing - which has caused him to start putting his kidneys under stress - which is aggravated further by the necessary nature of cyclosporine and the other medicines. But the doctors kicked it all up a gear, got a team of renal specialist in and put Dad on IV fluids and now they're happy with him, an Oral Specialist has joined in now and is working on getting his mouth right and a whole team of Gastro doctors looked in his gullet today and the treatment is working. The large nasty rash and swelling on his head has completely gone. His tongue is starting to shed the sores, and I believe I actually saw my Dad's teeth today!LOL!

We aren't giving up. And he isn't either.

[Cheryl C]

Good news at last! Thanks for sharing it with us all. So pleased that there is improvement in your dad's condition. He's very fortunate to have such faithful, loving support in you guys too.

[Alcof]

Well what a difference. Dad is getting over this infection in leaps and bounds. He almost looks normal again! Mouth is going to take a while to get completely over. He still has sores and pain and is unable to eat anything solid. But the swelling is right down and each day it improves. Temperature is stable. He is even getting mobile again, thank God.

I have presented information to the doctors with regards to the G-CSF and they have agreed to stop this now after 3 weeks with no response. Took a while for them to get on the same page, but they listened and now today they have stopped the injections.

Also today they have stopped cyclosporine which I have mixed feelings about. There was no taper. Straight from 300mg per day to Zero. Any thoughts about this? I assume as there is no response to cyclosporine there is no real reason to taper gently.

Also have not found any information that suggests that either Danazol or Eltrombopag may be more or less effective with cyclosporine. Any ideas here?

In any case, Dad is delighted to have kicked the cyclosporine as it should help his gums recover even more rapidly - we hope! Thanks to those who have posted in this forum in this regards!! And he always had difficulty taking them. Horrid smelly things!

Tomorrow is Dad's birthday. He's dreaming of eating Ciabatta bread rolls with salad and ham! Shame he can't have that until that mouth is better. Its just protein formula until he can swallow properly. But great he has an appetite!

So a good news day. We are quietly hopeful that we may even get to bring him home soon - maybe in another week. Which will be a great relief to my 74 year old mother who stays every night in the hospital and is actively nursing him due to the over worked and slim staffing they have in our public hospitals. And 5 weeks of night shifts is hard going for anyone.

So fingers crossed now. No more infections. And we continue to pray that he has a response in his bloods.

[Hopeful]

Alcof,

I am glad that he is doing so much better! To my knowledge, there is no risk with stopping cyclosporine abruptly. It isn't like prednisone, for example.

I must warn you though that it can cause intense arthritic-like pain in the joints for some people when the dosage decreases (even slightly). So, look out for this and know that it will pass within a few days/weeks.

[Alcof]

Thanks Hopeful! Its always hard double guessing what some new symptom is. Nice to be ahead of the game for once...hope he just eases off it without too many issues...just happy gums...!

[Sally C]

Hi Alcof,
I have been keeping up with your posts. So thankful your Dad is doing better. You are a great advocate for him and obviously love him very much.
My husband had great success with Eltrombopag without being on Cyclosporin. I do have to add that he had no response with the Cyclosporin but don't know if that makes a difference.
If you would like, please read my posts about his journey. He came from being on our Oncologist's "death list" and over 125 blood/platelet transfusions to being transfusion independent for over 3 years post Eltrombopag. He stopped the med in 2012 due to platelets going over 100,000 and has yet to lose his reponse.
While I know every case is different, I hope this helps. By the way, he has MDS.
Best wishes and God Bless,
Sally

[Alcof]

Well at last Dad is infection free. And home from the hospital after 6 weeks of constant battling to get clean of so many infections including 5 hospital bugs. He is so well its amazing. Unfortunately this is due to a magical balance of anti-virals, anti-biotics, and anti-fungals and not because his blood has improved at all.

However it won't be until Monday next week that he goes on to the full 150mg Eltrombopag dose. Then we will really start to be on tenterhooks. Waiting and hoping.

We just need to get Dad's strength back now and muscle mass. A long slow, taxing and emotionally draining process for him. And pray and hope we don't get more infections.

Sally your husbands story is so inspiring and uplifting. Its what keeps us all going - knowing that people have lived through it and come out with SUCCESS! Only for Marrowforum and I would never have heard of Eltrombopag, would never read your story and never found the courage to hope and pursue the stuff.

[Marlene]

Couple of things to keep in mind. It's very difficult for any on the blood lines to improve when fighting infections. Fevers chew through platelets and red cells. And any white cells will have been called to the areas of infection/inflammation with very little left in the blood. All of this, plus the meds, are taxing on the bone marrow.

John went 84 days without a white count. He too was on all the "anti" meds. As soon as his ANC hit .300, they would pull one med at a time. If he did not spike a fever they kept going. If he got a fever, they would resume the med.

At this point, he was on G-CSF (neupogen).

Keep an eye out for GI problems, specifically, very smelly stools. Could indicate a C-Diff infection which can happen being on the other meds.

Lastly, good sources of protein will help build the body back up and start off with mild exercise. If he's on Danazol, that may help too.

[curlygirl]

I know a lot of people with autoimmune disorders eschew dairy, but milk was great when my son got a mouth full of sores along with fevers along for two weeks after going of his prednisone following ATG (It could have been serum sickness, or a virus, it was hard to tell). The sores made it hurt too much to eat, the coolness of the milk helped his sores, and clearer liquids made him feel like would throw up. It's also high in calories, protein, Vitamin D, and vitamin B. Just a suggestion. If you dad has any kind of congestion it can make it worse so you'll have to see if it agrees with him.

[Sally C]

Hi Alcof,
Your post warmed my heart. So thankful that Don's story has helped you and your Father on his journey. Please keep us posted.
Best wishes and God Bless,
Sally

[Alcof]

Its really tough staying optimistic. No movement in bloods across the board. And we are fighting infections, in and out of hospital. Near death so many times that we have to ask how often can he come back? Its the repeated flare ups of the ESBL and their friends that do the damage. And we are in week 8 of Eltrombopag and praying for a miracle to happen. Its a battle between the blood recovery clock and the antibiotic resistant bugs.

The neutrophil count is 0.05....so zero. Consistently. Never changing. Only the Hb and platelets change as they fall between transfusions.

I struggle to avoid the question what if. What if eltrombopag doesn't work? What then?

Such a cruel condition as Dad fully comes back in between bouts and he seems next to normal for a day or two. And then the doctors remind us that he has little or no hope. To say they aren't optimistic is an understatement.

Anyhow, we keep praying and hoping and making the trips to the hospital and not sleeping. Its far more unpleasant for Dad.

Keep the fingers and toes crossed for us...

[Chirley]

I don't know if it's any comfort but my Mum has had ESBL and VRE for two and a half years and still doing okay.

[Alcof]

My father passed away peacefully at home on Wednesday 29th. It was 18 weeks from diagnosis to the true end of the road.

Thanks to the Marrowforum community for all your great stories and support and thoughts and prayers over the last 4 months.

[Neil Cuadra]

Alcof,

I'm very sorry you've lost your father. From your posts I've had the impression that both you and your dad were positive people and I wish he could have won this fight. I'm saddened at the short time he had after his VSAA diagnosis.

You told us that you had been an advocate carer for other people, and it's clear you were there when your father needed you. He must have drawn strength and comfort from having you as a medical advocate. I'm glad to hear that the end was peaceful and at home, but I know this is anything but peaceful for you. You have my best wishes at this sad time.