I have diffuse scleroderma with Interstial Lung Disease and Pulmonary Hypertension as well. I also have a kidney transplant, esophageal dismotility, GI issues and have been seeking a lung transplant but was turned down at two centers due to how complicated my disease is. I sleep elevated and try not to eat 3-4 hours before bedtime. I use oxygen for exertion. Years ago they tried cyclophosphamide to stop the progression of the lung fibrosis but I had a toxic reaction to it and it was stopped. I take about 25-30 pills a day.
I hope you are seeing a scleroderma specialist and a pulmonary hypertension specialist. My PH is managed with oral medication right now. The gold standard for diagnosis is a right heart catherization. Have you had one? Do you know what your pressure was? Mine was 45 but is now 23 with the medication, which is normal. A 6 Minute Walk Test and Pulmonary Function Tests should also be done and if you are dropping below 87% on the walk test you should be using supplemental oxygen.
We all have our down moments and the best thing I can recommend for that is to find a good therapist. An objective viewpoint can help you deal with both the disease and your family. Perhaps your husband will even be willing to go to a session or two with you. There were times I have wanted to give up, too, but then I think of how hard it would be on my husband without me. I am sure you think of the same things about your family.
I try not to let my illness stop me. Even on dialysis I traveled. Since my kidney transplant I go even more places for longer periods of time. I have a portable oxygen converter that charges in the car and is approved for air travel. I use wheelchair assistance at airports. I go to the beach every day and when the ocean is calm I can even go in it. We try to go on as many trips as we can. The next two months we have week long car trips planned and early next year we will be going out of the country for a month.
My nephew is diabetic and just went through surgeries on his feet to prevent amputation. He went to a theme park and rented a scooter so he could do everything he wanted to. I know the thought of using assisted devices like a scooter, wheelchair and oxygen are not appealing but if it means continue to have the kind of life you and your family want it is well worth the stares you get.
Another thing I do is plan downtime. I know that I will need time to rest after a couple of days of activity and work it into my trips and for when I return. I also need it if I have a couple of busy family days. Try to explain to your family that there will be times when you need to regroup. It may mean sleeping all day or just laying on the couch reading a book. Granted, I don't have children so this is easier for me. My husband also does most of the housework and cooking so that is a big help. I worry about how all this affects him as well.
This group is extremely supportive and is where I come when I have my down moments. Everyone understands because they have been there. Please keep posting and let us know how you are doing. Sending you healing thoughts,